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Table of Contents
     Preface CARDIOVASCULAR Congestive Heart Failure Pediatric Hypertension Kawasaki Disease Acute Rheumatic Fever GENERAL PEDIATRICS AND PHARMOCOLOGY         Bite Wounds Inborn Errors of Metabolism and Metabolic Diseases Pediatric Nutrition Dysmetabolic Syndrome General Approach to Poisonings/Ingestions Pediatric Antibiotics Radiologic Studies and Basic Indications Henoch-Schonlein Purpura GASTROINTESTINAL    Gastrointestinal Hemorrhage Gastroesophageal Reflux Disease Neonatal Hyperbilirubinemia RESPIRATORY AND ALLERGY      Acute Airway Obstruction/Stridor Parapneumonic Effusion and Empyema Anaphylaxis Asthma Cystic Fibrosis NEUROLOGY    Seizure Disorders Stroke in Childhood Ataxia 83 86 88 65 69 72 73 76 53 56 58 23 26 31 35 38 44 46 48 7 10 13 17 3

2 ENDOCRINE     Diabetes Mellitus Adrenal Crisis Hypoglycemia Puberty FLUIDS AND RENAL         Acute Renal Failure Fluids and Electrolytes Rickets Renal Tubular Acidosis Hematuria Nephrology Cocktails Proteinuria Hydronephrosis INFECTIOUS DISEASE       Meningitis Shunt Infections Infant with fever of uncertain source Urinary Tract Infections Catheter Related Sepsis MRSA Infections in Children HEMATOLOGY/ONCOLOGY       Emergency Treatment of Bleeding Disorders Fever in Immunocompromised Children Thrombotic Disorders Sickle Cell Disease Oncologic Emergencies Transfusions APPENDICES    Quick Calculations IVIG Administration Protocol Phone Directory 179 180 181 153 158 160 165 173 175 135 139 140 143 147 149 107 111 115 118 122 125 126 130 93 97 99 101


This handbook is designed to assist housestaff with common pediatric problems. This edition represents the fourth major revision. Changes to this edition include new chapters on Henoch-Schönlein Purpura, Neonatal Hyperbilirubinemia, Ataxia, Delayed/Precocious Puberty, Rickets, Renal Tubular Acidosis, Hydronephrosis, and MRSA infections. In addition, some chapters have been condensed, revised, or expanded to provide a more accurate resource. Finally, every attempt has been made to provide an up-to-date reference, yet medicine is rapidly changing and what is standard of care today, may be ―archaic‖ in a few years. Thus, if in doubt, always consult other sources or your attending. We would like to thank the following people, in addition to those who have previously contributed to this handbook, for their assistance with this edition: Kathryn Stephenson, M.D. Michael Gonzalez, M.D. Carla Roberts, M.D. Matt Wienecke, M.D. Ozzie Shuler, M.D. Kelly Lewis, R.D. Rathna Amarnath, M.D. David Schwartz, M.D. Trey Brown, M.D. David Amrol, M.D. Kevin McRedmond, M.D. Tim Livingston, M.D. Rob Holleman, M.D. Laura Pirich, M.D. Ron Neuberg, M.D. Mark McDonald, M.D. Erika Clark, PharmD Malaka Jackson, M.D. Jennifer Bair, PharmD Matthew Garber, M.D.

Editors Caughman Taylor, M.D. James Stallworth, M.D. Sara Lindsey, M.D. Jason Hawn, M.D. Joseph Delaney, M.D.






Congestive Heart Failure
ETIOLOGY CHF may result from congenital or acquired heart diseases with volume and/or pressure overload or from myocardial dysfunction.      Congenital Heart Disease: most common cause in pediatric age group. Acquired Heart Disease: Rheumatic Fever, Collage Vascular Disorders, Vitamin deficiencies, Kawasaki Disease Myocardial Dysfunction: Myocarditis, Endocardial fibroelastosis, Metabolic abnormalities, Cardiomyopathy Arrhythmia: Tachyarrhythmias (SVT), Bradyarrhythmias (AV Block) Miscellaneous: Acute Hypertension, Severe anemia, Volume overload, Sepsis CLINICAL MANIFESTATIONS Diagnosis is based on clinical judgement, relying on history, physical examination, chest x-ray, ECG, and echocardiogram. Decompensated CHF (inadequate cardiac output) can present with end organ dysfunction/failure. History  Tachypnea and grunting  Poor feeding (sweating, increased RR, tiring with feeds)  Poor weight gain Physical Exam  Tachycardia and tachypnea  Gallop rhythm  Heart murmur  Cyanosis (most often cyanosis in the face of CHF will be peripheral although occasionally it can be central)  Weak and thready pulses  Cardiomegaly, Hepatomegaly  Peripheral edema rarely seen in infants with CHF Left-sided Failure  Tachypnea  Orthopnea  Wheezing and pulmonary crackles  Pulmonary edema, Pleural effusion Right-sided Failure  Hepatosplenomegaly  Pleural effusion, Ascites  Edema: eyelid, sacral, less frequently lower extremity (in pediatric population)  Jugular venous distention (rarely seen in infants)

7 Chest X-ray  Cardiomegaly and increased vascular markings ECG  Signs of LVH and RVH may be present, as well as signs of strain with ST and Twave changes Echocardiography  May be diagnostic for a congenital or acquired heart defect  Can be used to evaluate systolic and diastolic cardiac function MANAGEMENT       Elevate head of bed Oxygen Salt restriction in older children to less than 0.5gm/day Fluid restriction to 2/3 maintenance (NPO initially) Correct anemia (slowly if severe), reduce fever, and treat underlying infection Treat underlying causes (hypertension, arrhythmias) and correct electrolyte imbalances DRUG THERAPY Management involves preload as well as afterload reduction and inotropic agents. For acute management of decompensated CHF consider a combination of dopamine, dobutamine, milrinone, and/or Lasix. If CHF is due to duct dependant lesions or severe coarctation consider PGE1. Less commonly, epinephrine is required. Management of ―Chronic‖ CHF includes one or more of the following: Diuretics  Acts through preload reduction  Use may preclude need for digoxin  Lasix 1-4 mg/kg/day, po in 1 to 4 divided doses (typically dose does not exceed 2mg/kg/dose)  Spironolactone 1-3.3mg/kg/day po in 2-4 divided doses, may be used in conjunction with Lasix for its potassium-sparing effect (most often used if lasix dose exceeds 2mg/kg/day)  Side effects of diuretics, which predispose to digoxin toxicity, include hypokalemia, hypochloremia, alkalosis, and dehydration Digitalis  Should not be used in children with IHSS, complete heart block, WPW, or cardiac tamponade  Digoxin/Lanoxin is preferred and po route should be used. Digitalizing dose typically is not used for treatment of CHF. Maintenance dose is variable depending on the age of the patient. See Harriet Lane Handbook for dosing.

8 Common ECG effects are shortening of QTc (earliest sign), sagging ST segment, diminished amplitude of T-wave, and slowing of heart rate Digitalis Toxicity  Non-cardiac symptoms include nausea, vomiting, diarrhea, restlessness, drowsiness, fatigue, and visual disturbances in older children  ECG signs are probably more reliable and appear earlier. These include prolonged PR interval, profound bradycardia or SA block, and arrhythmias.  Factors that exacerbate toxicity include hypokalemia, hypomagnesemia, low T4, and hypercalcemia  For management of digitalis toxicity see Poisondex or Harriet Lane Handbook Oral Angiotensin-Converting Enzyme Inhibitors (ACE inhibition)  Afterload reduction  Captopril and Enalpril are the most commonly used ACE inhibitors  May cause neutropenia, angioedema, cough (more common with captopril), azotemia, hyperkalemia (use with caution in combination with potassium sparing diuretics), rash  AVOID use with dialysis with high-flux membranes because anaphylactoid reactions have been reported NOTES 

altered mental status May progress to seizures Congestive heart failure may be present depending upon duration and severity. vomiting. Consider fasting lipid panel and insulin if pt is obese. Lupus.9 Pediatric Hypertension DEFINITION Hypertension is defined as average systolic and/or diastolic blood pressure that is ≥ 95th percentile for gender. and height on 3 or more separate occasions. sex. Other studies as indicated by history. HSP Chronic pyelonephritis Renal vascular disease (renal artery stenosis (RAS)) Pheochromocytoma Chronic renal failure Steroids and miscellaneous drugs Essential hypertension Coarctation CNS. This is more common in infants. lethargy. age. ANA  ANCA . or severity of hypertension:  MRI or CT Head  Plasma renin and aldosterone  Urine for VMA and metanephrines  ASO. Secondary hypertension is more common in younger children with renal parenchymal disease being the most common cause. and height  Pre-HTN: ≥90th percentile. and echo. COMMON CAUSES OF HYPERTENSIVE CRISIS IN CHILDHOOD           Acute post-streptococcal or post-infectious glomerulonephritis Other forms of nephritis: HUS. age of patient.increased ICP. renal ultrasound. (>120/80 in teens)  HTN(stage I): ≥95th percentile ≤ 99th  HTN(stage II): >99th percentile + 5 mmHg  Malignant HTN: Findings of target organ disease SIGNS OF HYPERTENSIVE CRISIS    Headache. U/A and urine culture. Essential hypertension is now the most common form in pediatrics when all ages are included. mass.  Normal BP: <90th percentile for age. tumor DIAGNOSIS AND WORK-UP   Initial evaluation for all children includes BMP. visual disturbances. <95th percentile. complement levels.

bone marrow suppression. max 50mg BID) Diuretics  Most commonly used as second or third agents (except patients with Na sensitive primary hypertension)  Drugs: HCTZ (1-2mg/kg/day qAM-BID). Nifedipine (0. max start 100mg BID)     . mostly for infants) Calcium Channel Blockers  Good first choice. DM  Drugs: Atenolol (0.5 mg/kg/dose q8hrs. in addition to calorie limits. or solitary kidney with RAS  Adverse affects: cough. Bradyarrhythmia. ↓GFR  Drugs: Enalapril. max 40 mg/day).Spironolactone (13mg/kg/day qam-BID) Beta Blockers  Less well tolerated. Lisinopril (start 0. Captopril (0.08 mg/kg dose qam-bid. include avoidance of caffeine and reduction of sodium intake by limiting consumption of high salt processed foods and increasing fresh vegetables and fruits. Heart Block. BLOOD PRESSURE MEDICATION FOR CHILDREN  ACE Inhibitors  Good first choice if suspect renovascular HTN. proteinuria. start 30mg qam. Labetalol (4mg/kg/day divided BID.RAD. bilateral RAS. angioedema. Stage II hypertension generally requires referral to pediatric nephrology and management with medication. gingival hypertrophy  Drugs:  Acute: Isradipine (0.1mg/kg/dose q6 hrs prn. Amlodipine (0.3mg/kg/dose qam-BID) Angiotensin Receptor Blockers  Used primarily when cannot tolerate ACE-I or for added anti-proteinuric effect  Drugs: Losartan (start 0. Recommended diet changes. DM. comes in 10mg cap)  Chronic: Nifedipine XL (for teens.10  Thyroid levels  VCUG  Renal biopsy TREATMENT OF HYPERTENSION IN CHILDREN Therapeutic lifestyle changes are recommended for all children with hypertension and pre-hypertension. K-sparing.0 mg/kg/day qam-BID).pregnancy. so recommended mainly for difficult to treat patients  Contraindications.5-1. HA.1-0. very well tolerated and safe  Adverse effects: flushing.25 mg/kg/dose q 4hr prn.1-0. suspension can be made). ↑ K+. edema. may need BID).8mg/kg/day qam. Weight reduction is the primary therapy for obesity related hypertension. Chronic Kidney Disease  Contraindications .

Lasix Autosomal dominant pattern of HTN with hypokalemia (Liddle’s syndrome) Amiloride ADHD and HTN with compliance issues .Calcium channel blocker NOTES .ACE-I Acute GN with HTN from volume overload . proteinuria. mircroalbuminuria .11 CHOICE OF DRUG FOR SPECIFIC CIRCUMSTANCES      Renovascular HTN.Clonidine patch Work up in progress but need to start med on young child . DM.

5 cm  Usually single and unilateral adenopathy  Generalized lymphadenopathy is not seen . It can result in coronary artery abnormalities in up to 20% of untreated children. Adenovirus. Specifically. systemic vasculitis of unknown etiology. DIFFERENTIAL DIAGNOSIS           Scarlet Fever Toxic Shock Syndrome Staphylococcal Scalded Skin Stevens-Johnson Syndrome/Drug Reactions Collagen Vascular Disease Rheumatic Fever/SBE Serum Sickness Leptospirosis RMSF/Other Rickettsial Disease Viral Syndromes: EBV. Diagnostic Criteria Criteria Comments Bilateral Non-Exudative Conjunctivitis  Usually more prominent in the bulbar than palpebral conjunctiva with sparing of the limbus  Associated eye features include anterior uveitis and acute iridocyclitis in >80% Polymorphous Exanthem  Usually generalized and erythematous  Can be morbilliform. The diagnosis of KD is based on clinical criteria as established by the CDC. or erythema multiforme  Generally not bullous or vesicular  Typically worse in the GU area Cervical Lymphadenopathy  Nodes should be > 1. Diagnosis is based on exclusion of other etiologies and the fulfillment of clinical criteria. scarletiniform. Measles DIAGNOSIS No diagnostic test exists for KD. Hepatitis B. diagnosis requires fever lasting over 5 days and at least four of five other criteria. maculopapular. Influenza.12 Kawasaki Disease Kawasaki Disease (KD) is an acute febrile.

and bilirubin Thrombocytosis after 10-14 days (subacute phase) Sterile pyuria in 70% INCOMPLETE KAWASKAI DISEASE      Some patients do not fulfill the above criteria and are subsequently diagnosed with ―incomplete‖ or ―atypical‖ Kawasaki Disease Incomplete KD is more common in young infants. and urine ≥ 10 wbc/hpf See algorithm for workup of children with suspected incomplete KD . ALT. even if the infants have no clinical criteria Supplemental lab criteria include albumin ≤ 3.000/mm³. an echo.13 Changes in the Hands or Feet       Edema of the hands and feet Palm or sole erythema Periungal desquamation in convalescent phase Fissured. if evidence of systemic inflammation is found.0g/dL. iridocyclitis in 80% Arthritis or arthralgia in 35% Hydrops of gallbladder in 10% Pericardial effusion or arrhythmia in 20% Aseptic meningitis or carditis in 5% Irritability       Laboratory Findings Leukocytosis Anemia Increased CRP and ESR Increased AST. Infants ≤ 6 months old or on day ≥ 7 of fever without other explanation should undergo laboratory testing and. Accurate diagnosis is especially important in this age group as they are at higher risk of developing coronary artery abnormalities. platelets after 7days ≥ 450. anemia for age. wbc ≥ 15. red lips Strawberry tongue Diffuse erythema oropharyngeal mucosa Changes of the Oropharynx Associated Features       Clinical Findings Anterior uveitis.000/mm³. elevations of ALT.

14 Evaluation of Suspected Incomplete Kawasaki Disease (KD) Fewer ≥ 5 days and 2 or 3 clinical criteria Assess Patient Characteristics Persistent Fever Consistent with KD Inconsistent with KD Assess Laboratory Tests KD unlikely CRP <3.0 mg/DL and ESR <40 mm/hr CRP ≥3.0 mg/DL and/or ESR ≥40 mm/hr Follow Daily <3 Supplemental Laboratory Criteria ≥3 Supplemental Laboratory Criteria Fever Continues for 2 days Fever Resolves Echo Treat and Echo No Peeling Typical Peeling Echo + Echo Fever Abates Fever Persists No f/u Echo Treat Repeat Echo Consult KD Expert KD Unlikely .

Patients with documented coronary artery disease need prolonged low-dose aspirin therapy If the patient does not respond within 48 hours of IVIG administration. Patients with a pre-IVIG treatment CRP over 10 mg/DL. Live virus vaccines should be delayed 11 months after receiving IVIG to avoid suppression of immunologic response to the vaccine NOTES    . Aneurysms can occur anytime during the acute and subacute phase of illness. the dose can be repeated. Echocardiogram may also reveal pericardial effusion. Steroids are dosed at 30mg/kg/day for 3 days. LDH over 590U/L. MANAGEMENT AND THERAPY     Echocardiogram should be performed at time of diagnosis and repeated 6-8 weeks later Intravenous immunoglobulin initiated within 10 days of the onset of fever in conjunction with ASA reduces the incidence of coronary artery aneurysm and causes fever resolution See Appendices for IVIG Administration Protocol Aspirin therapy is given in the acute and subacute phase. Long term therapy is aimed at preventing MI or ischemia. then 3-5 mg/kg/day (maximum 4080mg/day) given as a single dose until the platelet count and sedimentation rate are normal. The use of IVIG within the first 10days of illness has been shown to decrease the incidence of transient coronary artery dilation to 5% and that of giant aneurysms to 1%. and /or HgB less than 10gm/dL are at increased risk for IVIG failure. Cardiac involvement ranges from carditis to coronary aneurysms. Initial dose is 80-100 mg/kg/days divided QID until fever resolves. mitral regurgitation. Treatment in the acute phase is aimed at reducing inflammation in the coronary artery wall and preventing thrombosis. Steroids can be tried if the patient fails to respond to 2 doses of IVIG.15 CARDIAC INVOLVEMENT Cardiac pathology represents the major cause of morbidity and mortality in patients with KD. or left ventricular dysfunction.

It is a delayed (usually 2-4weeks) nonsuppurative sequela of pharyngeal infection (not skin) with group A streptococcus (GAS). up to 2. persist for weeks  Symmetric distribution on extensor surfaces of joints. pericarditis. Diagnosis of ARF requires two major criteria or one major plus two minor criteria to be present as well as evidence of a GAS infection. or signs of CHF Erythema Marginatum  Occurs in 5-10%  Rash is evanescent. and erythematous in a serpiginous or annular pattern  Occurs on trunk and proximal extremities (never on the face)  Rarely seen in other diseases Subcutaneous Nodules  Occurs in less than 10%  Hard. painless. nonpruritic. other criteria may be absent and ASO and ESR may be normal. nonpruritic.  Manifests as emotional lability that progresses to motor incoordination (may initially see handwriting deterioration) then to spontaneous purposeless movements Minor Criteria Fever  Fever is usually low grade and present early in the course of ARF . usually extremities  Extremely painful but responsive to NSAIDs  Migratory involvement: two or more joints involved Carditis  Occurs in 40-50%  Signs of carditis include: tachycardia. and spine Sydenham Chorea (St. The first episode typically occurs between 5 and 15 years of age (rarely seen before the age of 3 and after 15 years of age). cardiomegaly. chorea may stand alone as a diagnostic criteria for ARF. new heart murmur. JONES CRITERIA FOR ARF Major Criteria Polyarthritis  Occurs in 60-85% and is often the earliest manifestation  Involves the large joints. therefore. scalp.0cm in diameter. It is a clinical diagnosis based on the Jones Criteria. freely mobile. Vitus Dance)  Occurs in up to 15%  Due to late onset (often 3 month or longer latency).16 Acute Rheumatic Fever Acute Rheumatic Fever (ARF) is one of the primary causes of acquired heart disease worldwide.

such as streptozyme. Other antibody tests. can also be used.17 Arthralgia  Cannot be counted towards diagnosis if arthritis present Elevated Acute Phase Reactants  CRP and ESR (ESR may be spuriously low in the face of CHF. or NSAIDs have been administered Prolonged PR interval  Cannot be counted towards diagnosis if carditis is present Previous Rheumatic Fever PRECEDING GAS INFECTION Evidence of GAS infection is required for the diagnosis of ARF. or sodium valproate may be used. This can be drawn at presentation and then in 3 weeks. Only 25% of patients with ARF will have a positive throat culture or rapid antigen test. Haldol. Joint pain should disappear within 24-36 hours and if not the diagnosis should be questioned. There is evidence that steroids may shorten the course of chorea. Valium. eradicate the streptococcal infection. Begin aspirin at doses 50-100mg/kg/day divided in 4 doses. ASA. a single ASO titer greater than the 85th percentile of the normal population is strong evidence for recent infection. Carditis may require treatment with steroids if high dose NSAIDs are not effective. and prevent recurrence Patients should limit their activity if actively symptomatic with arthritis. Chorea is usually self-limited but may last several months. or chorea ALL patients should receive appropriate antibiotic therapy Each patient’s anti-inflammatory treatment should be individually tailored depending on the severity of arthritis. carditis. Add ASA when taper begins and continue until CRP is normal and the ESR is falling. Overall 95% of patients with ARF will have at least one antibody test indicating preceding GAS infection. Anti-DNase B is the next best test. Thorazine. ASO is considered significantly elevated if the acute titer is 2 or more times greater than the convalescent titer. treat CHF if present. anti-streptokinase and anti-hyaluronidase. then taper. Dose prednisone 2mg/kg/day divided BID for 2 weeks. If severe. CRP is unaffected by CHF)  May be normal in patients with only chorea or when steroids. A recent history of scarlet fever without laboratory confirmation of GAS is inadequate evidence.   . Other NSAIDs can also be used at anti-inflammatory doses as alternatives to aspirin. TREATMENT     Treatment of ARF is designed to reduce inflammation. Likewise. The most frequently used tests are the anti-streptolysin O (ASO) and anti-DNase B.

NOTES . See AAP Red Book for prophylactic regimens.18  Length of secondary prophylaxis is variable.

19 NOTES .

20 General Pediatrics and Pharmacology .

21 .

22 Bite Wounds The most common bites seen are those of dogs (~80% of animal bites). The most common complication associated with these injuries is infection. systemic illness. muscle. Incidence of infection in cats is higher— approaching 30 to 50%. Immobilize and elevate affected limb(s) when possible. Obtain an x-ray if foreign body is suspected or if there is apparent bone or joint disruption. and an immunocompromised patient ANTIBIOTIC THERAPY  Antibiotic therapy is indicated in all of the following circumstances:  Moderate to severe injuries  Significant crush injuries (such as those seen with some dog bites)  Deep puncture wounds  Facial wounds  Wounds on the hands or feet  Wounds on the genitals  Immunocompromised patient  Bites >8 hours old at first evaluation  Any signs of infection        . Most can be treated on an outpatient basis. Indications for admission/inpatient treatment include: failure of outpatient oral antibiotics. Incidence of infection in dog bites is between 5 and 10 % (higher risk with bites to hands or feet). Do not suture puncture wounds or wounds on the hand or feet—these wounds have the highest incidence of infectious complications. tendon. Patient specific past medical history should also be gathered including any underlying medical disease process. DHEC should be alerted and attempts should be made to collect the animal for observation if possible Initial wound management should include washing with soap and water and irrigation with sterile saline under moderate pressure as well as debridement of devitalized tissue Local anesthesia can be used to facilitate wound cleansing if needed Examine for vascular. or nerve damage. Follow up frequently to monitor for infection. and humans. Suturing of wounds that are more than 8 hours old should be avoided. MANAGEMENT  Initial history should include information surrounding bite (provoked or unprovoked) as well as type of animal and immunization history of the animal. joint. Suturing: Large wounds or wounds that are on the face (cosmesis-low incidence of infection) may be sutured. cats. concerns for infection and delay in seeking medical care.

23  Most cat bites  Human bites Prophylactic antibiotic treatment is not indicated for most dog bite wounds with the exception of wounds to the hand. 2Also consider cat scratch disease and tularemia in cat bites. azithromycin. 3More prone to infection than cat or dog bites. Fusobacterium. TMP-SMX for penicillin allergic patient) *Note that single drug therapy may not be adequate in penicillin-allergic patients. (Usual prophylactic course of treatment is 5 days. Peptostreptococcus. .4 Organisms of Infection1 Common Pathogens Staph aureus α-hemolytic Strep (most common in dogs) Pasteurella multicoda (most common in cats) Capnocytophaga canimorsus (severe sepsis in asplenic or alcoholic patients) Other Staph and Strep species Klebsiella Bacillus subtilis Pseudomonas Enterobacteriaceae Anaerobes (Bacteroides. 4Must also consider Hepatitis B and HIV in any human bite. and Actinomyces) Staph aureus Eikenella corrodens Haemophilus influenza Strep species Anaerobes 1No single organism is responsible for > 30% of infections.) Possible choices of antibiotics include: 1)amoxicillin-clavulanic acid (40 mg/kg/day) for dog or cat bites as well as 2)dicloxacillin or 3)cephalexin for dog bites (erythromycin or TMP-SMX for penicillin allergic patient) 4)cefuroxime or 5)dicloxacillin + penicillin for cat bites (levofloxacin or moxifloxacin. Consider wound culture if the injury is more than 8 hours old or if there are signs of infection    Species Dogs and Cats2 Humans3. There is evidence to support prophylactic antibiotic treatment of cat bite wounds.

14. or Rabbit Bite Generally not considered rabid. 7. This is an IM vaccine given on days 3. and 28. Woodchuck. Cat. and tetanus prophylaxis must be addressed if needed based on the following guidelines. if it has been >5 years since the last dose. give Td. Bat. Rodent. Livestock. When to Give Rabies Prophylaxis Clinical Scenario Approach Dog. give DTaP unless otherwise contraindicated. The dose is 20 IU/kg. Cat. Consult DHEC. Administer immunization and RIG1. Clean/Minor Wounds TIG Td1 Other Wounds TIG Previous number of adsorbed tetanus toxoid Td1 doses Yes No Yes Yes Status unknown or number received <3 No No ≥ 3 previous doses No2 No3 1For children <7 yo. Dog. Dog. if it has been >10 years since the last dose. with half being infiltrated into the wound and the other half given IM. 3Yes. Raccoon. The patient should also be given the primary rabies vaccine (2 types: HDCVhuman diploid cell vaccine or RVA-rabies vaccine adsorbed). or Ferret Bite Rabies prophylaxis if the animal develops Animal is healthy and available for symptoms of rabies observation. Fox. 1RIG is not recommended in previously vaccinated individuals NOTES . Cat. For persons ≥ 7 yo. RABIES PROPHYLAXIS   Prophylaxis for rabies consists of Rabies Immune Globulin (RIG) which is given on the first day of treatment.24 TETANUS PROPHYLAXIS Bite wounds are considered dirty. or Ferret Bite Consult DHEC Unknown status of animal Skunk. or Consider the animal rabid unless proven other Carnivore Bite otherwise or in an area free of rabies. or Ferret Bite Administer immunization and RIG1 Animal is rabid or rabies is suspected. 2Yes.

Pyruvate dehydrogenase deficiency. urea cycle defects. and storage disorders. Nonketotic. hyperammonemia. Proprionic acidemia.e. Pyruvate carboxylase deficiency) . Most are caused by a single enzyme deficiency or cofactor that disrupts one step of the metabolic pathway. Symptoms worse with fasting Mitochondrial Disorders (includes Elevated lactate and pyruvate MELAS. poor feeding. Homocystinuria) gap. No transcarbamylase deficiency.25 Inborn Errors of Metabolism and Metabolic Diseases Inborn errors of metabolism are a diverse collection of disorders including organic acidemias. No ketonuria. Majority are autosomal recessive. Variable respiratory alkalosis. metabolic acidosis. Citrullinemia) metabolic acidosis. Amino acidopathies (includes PKU. Elevated acylcarnitine. apnea. tachypnea Acute encephalopathy or seizures Unexplained jaundice. Early detection is vital to prognosis. vomiting. fatty acid metabolism defects. COMMON SIGNS AND SYMPTOMS       Symptoms of lethargy. Metabolic acidosis with increased anion Maple Syrup disease. Neutropenia and thrombocytopenia can be present. i. Variably elevated plasma ammonia and lactate. metabolic acidosis. Abnormal plasma amino acids Fatty Acid Oxidation (includes Medium Illness or stress increase metabolic chain acyl-CoA dehydrogenase deficiency) demands leading to hypoglycemia. Elevated orotic acid in OTCD. Often presents when meals are more spread out. the infant is sleeping more. liver failure. Elevated plasma amino acids Fasting hypoglycemia and ketosis. Elevated plasma and urine ketones.. Urea Cycle Defects (includes Ornithine Markedly elevated plasma ammonia. or hypoglycemia Dysmorphic features or unusual odors Myopathy Failure to thrive IEM PRESENTATIONS AND ASSOCIATED DISORDERS Class of Disorders Organic Acidemias (includes Methylmalonic acidemia. +/Glycogen Storage Diseases hepatomegaly. Abnormal urine organic acids. Isovaleric acidemia) Characteristic Lab Findings Metabolic acidosis with increased anion gap.

and diabetes mellitus presenting before the stroke. Lactic Acidosis. No hyperammonemia. No metabolic acidosis. FIRST TIER EVALUATION          ABG CMP. cardiomyopathy. Look for myopathy. ataxia. because metabolites of certain disorders are not detectable when the patient is asymptomatic. mental retardation. deafness. All disorders of mitochondrial DNA are maternally derived. laboratory evaluation should be obtained while the patient is symptomatic. PRESENTATIONS OF MITOCHONDRIAL DEFECTS Organ System Possible Involvement Central Nervous System Encephalopathy. ataxia.26 Nonketotic Hyperglycinemia Acute encephalopathy. virtually any organ system or presentation is possible. strokes. and Stroke Like Episodes) can appear at any age but usually present as ―stroke of the young‖. seizures . MELAS (Mitochondrial Encephalomyopathy. consider mitochondrial defects. Abnormal plasma amino acids LABORATORY EVALUATION FOR IEM If possible.calculate anion gap CBC Ammonia Urinalysis Urine and plasma amino acids Urine organic acids Urine reducing substances Plasma lactate and pyruvate SECOND TIER EVALUATION        Plasma total carnitine and acetylcarnitine CSF lactate and pyruvate Urine orotic acid Plasma citrulline Chromosome analysis Plasma long chain fatty acids Muscle biopsy MITOCHONDRIAL DEFECTS When complex neurological or multisystem involvement is present.

pancreatic insufficiency. hypoglycemia Myopathy Renal tubular acidosis Pancytopenia. villous atrophy Diabetes mellitus. or anemia NEONATAL HYPERAMMONEMIA Symptoms in first 24 hours of life Symptoms after 24 hours of life Premature Full-Term Acidosis No acidosis THAN INBORN ERRORS OF METABOLISM (i. neutropenia. organic academia or PC deficiency) ORGANIC ACIDEMIAS UREA CYCLE DEFECT PLASMA AMINO ACIDS Absent citrulline Citrulline moderately elevated. no ASA THAN-Transient Hyperammonemia of Newborn . diabetes insipidus. ASA present Urine orotic acid Citrullinemia Argininosuccinic aciduria Low CPS deficiency Elevated OTC deficiency Citrulline markedly elevated.27 Cardiovascular Gastrointestinal Endocrine Musculoskeletal Renal Heme Cardiomyopathy. heart block Liver disease.e.

The first goal is the removal of accumulating metabolites such as organic acid intermediates or ammonia. IV lipids can be given to infants with urea cycle defects and other disorders in which dietary fat plays no role. PYRUVATE CARBOXYLASE DEFICIENCY PYRUVATE DEHYDROGENASE DEFICIENCY. MULTIPLE CARBOXLYASE DEFICIENCY. FRUCTOSE 1.28 METABOLIC ACIDOSIS WITH INCREASED ANION GAP Elevated lactate Normal lactate Abnormal organic acidsccidsac ids ORGANIC ACIDEMIA Abnormal organic acids Hypoglyemic METHYLMALONIC ACIDEMIA. . IV glucose should be administered to provide as many calories as possible. normal L-P ratio Normal or low pyruvate elevated L-P ratio No hypoglycemia Dicarboxylic aciduria FATTY ACID OXIDATION DEFECTS GSD TYPE 1. If an organic acidemia is suspected vitamin B12 and Biotin can be given to improve enzymatic activity. PYRUVATE CARBOXYLASE DEFICENCY EMERGENT THERAPY FOR POSSIBLE IEM Appropriate and aggressive treatment before the confirmation of a diagnosis may be lifesaving and may avert or reduce the neurologic sequelae of some of these disorders. Critically ill infants with hyperammonemia may need hemodialysis. An IV arginine preparation is available commercially and a protocol is described below. PEP CARBOXYKINASE DEFICIENCY RESPIRATORY CHAIN DEFECTS. If a there is a suspicion of a disorder with protein intolerance. The second goal of therapy is to prevent catabolism. OTHERS Normal organic acids Elevated pyruvate. PROPIONIC ACIDEMIA. an infusion of arginine can be given. 6-DP DEFICIENCY. In patients suspected of having a urea cycle defect. protein intake should be discontinued immediately.

QS to 2450 ml total volume of Dextrose 10%. To run over 2 hours. admit to hospital for IV therapy: If lethargic or stuporous.5 x maintenance fluid rate.5 x maintenance fluids) for 24 hours. QS to 700 ml total volume of Dextrose 10%.95m2 Bolus dose: Ammonul® 52 ml mixed with Arginine 10% 168 ml. NOTES . Bolus Medication To infuse over 90 – 120 minutes. Example: Current: weight 28kg and BSA 0. Maintenance dose: Ammonul® 52 ml mixed with Arginine 10% 168ml. admit to PICU. Ammonul® (sodium phenylacetate and sodium benzoate) 55 ml / m2 Mixed with Arginine 10% 6 ml / kg and Dextrose 10% to a total volume of 1. To run at 102 ml / hour (1. If not lethargic. Maintenance medication To infuse over 24 hours at 1.29 SAMPLE PROTOCOL FOR TREATMENT OF HYPERAMMONEMIA If ammonia level is significantly elevated.5 x maintenance fluids. Ammonul® (sodium phenylacetate and sodium benzoate) 55 ml / m2 Mixed with Arginine 10% 6 ml / kg and Dextrose 10% to a total volume of 25 ml / kg. admit to general pediatrics floor.

3 1.8 3.4 1.8 3.1 3.7 7.4 7.4 Lactose/milk protein intolerance Lactose/milk protein intolerance Lactose/milk protein intolerance Milk/Soy protein sensitivity No MCT Sucrose/Lactose Free Malabsorption Neonatal Biliary Stasis 33% MCT Malabsorption Biliary Stasis 55% MCT Sucrose/Lactose Free Free amino acids 20 Casein Hydrolysates 1.9 6.5 Formula Calories (cal/oz) 20 20 20 20 Fat (gm/100cc) 3.6 3. partially hydrolyzed Lactose free Lactose free 20 20 1.9 6.3 7.0 7.6 3.5 3.5 20 Soy Protein Based 1.9 3.7 7.5 Alimentum 20 1.7 Neocate 20 Elemental Based 2.2 7.7 7.5 7.5 3.7 Goodstart DHA/ARA Supreme Soy Nutramigen 20 1.2 1.4 7.9 3.5 Isomil 20 1.3 3.9 3.0 3.1 7.30 Pediatric Nutrition Infant Formulas Protein Carbs (gm/100cc) (gm/100cc) Milk Protein Based 1.9 6.0 .4 1.4 Uses Breastmilk Similac Advance Enfamil Lipil Goodstart DHA/ARA Supreme Similac Sensitive Enfamil Lactofree¹ Prosobee Preferred Basic Formula Basic Formula 100% whey protein.7 Pregestimil 20 1.

7 4 3.2 Free amino acids Unflavored <1 year of age 33% MCT Increased protein/vitamins/ minerals Catch up growth Can be used to 1year of age Can be used into toddler years Neosure Enfacare 22 22 Preterm Infants 2.9 3.5 Nutren 2. ³ Goodstart Natural Cultures contains Bifidus BL cultures.0 45 60 6.5 87% MCT oil. 25% less lactose.0 430 745 .0 Formulas for Children over 6years Calories Protein Osmolality (cal/oz) (gm/100cc) 30 4.1 7. ² For infants with reflux.8 ¹ Enfamil Gentlease also available.0 7.5 6.2 3.0 Nutren 1.4 345 30 3.5 610 Free amino acids 30 45 3.31 Elecare 20 2.0 8. consider use of Enfamil AR (added rice starch) or Similac Sensitive RS (rice starch).0 4.9 Sim 60/40 20 Renal Formulas 1. risk of essential fatty acid deficiency Cow milk based Formula Nutren 1.5 Portagen 30 Fat Modified 3.0 430 30 3.0 300 Uses Basic Substitute for Ensure Fluid restriction CF Substitute for Deliver 2. Similac Sensitive is also lactose free.6 2.2 260 390 Elemental Formula Pediasure Kindercal Nutren Jr Neocate One Plus Peptamen Jr Resource Just for Kids 1.0 350 50%casein/50% whey Gtube/Oral formula 30 2. Formulas for Children over 1 year Calories Protein Osmolality Uses (cal/oz) (gm/100cc) 30 3.1 7.

9/tbsp 6.6 − − (mg) Calcium 40/scoop 1. Whey protein concentrate Powder 30/scoop 6/scoop 0.3 − − (mg) ¹ Better to use microlipid in formula because MCT oil separates out.9 − − (mg) Chloride 13. Used often with Cystic Fibrosis and Biliary disease.6 − Sodium 7.42 0.32 Propass Description Protein Supplement Modular Additives MCT oil¹ Microlipid¹ Fat Source used for Calorie Supplement Medium Chain Triglycerides Liquid 7.7 Source Form Liquid Powder Calories 4.42 1.8 − − (mg) Phosphorus 0.8 mEq of sodium MCT (medium chain triglycerides) oil is easier to absorb compared to other fats.42 0.2/tbsp 1.5/scoop 1/scoop OTHER INFORMATION         Increase total daily fluid by 25% if a child is on a fiber formula Use fiber containing formulas if diarrhea present (not constipation) One salt packet (1/8 tsp) gives 12.7 0.51 − CHO (g) 5.8 − (mg) Potassium 0.7/cc − 0.5/cc 23/tbsp Protein (g) − − Fat (g) 0. infants should double their birth weight by 6months and triple it by 1 year Rule of thumb: Infants should be taking about 2 ½ oz of formula per pound up to a maximum of 32-40 oz All exclusively breastfeed infants (taking less than 16oz of formula/day) should be started on 200 IU of Vitamin D by 2months of life .93 − − 50% fat emulsion for oral/TF Safflower Oil Polycose Powder CHO Calorie Supplement Glucose Polymers Duocal Fat and CHO Source Hydrolyzed corn starch MCT oil Powder 42/tbsp − 1. Goat’s milk is not indicated in children secondary to folic acid deficiency Typically.

33 FORMULA RECIPES Similac Advance 22cal  1tsp powder to 120 cc ready to feed (hospital) OR  3 scoops powder to 5 ½ oz of water Goodstart DHA/ARA Supreme 22cal  2 ½ scoops powder to 4.5oz of water Goodstart DHA/ARA Supreme 24 cal  1tsp powder to 75cc ready to feed (hospital) OR  3 scoops powder to 142 cc water Breastmilk 22cal  1 tsp Similac powder to 120cc Breastmilk OR  1 tsp Enfamil powder to 150cc Breastmilk Breastmilk 22cal (preterm infant)  1 tsp Neosure with 120cc Breastmilk OR  ½ tsp Enfacare with 90cc Breastmilk Breastmilk 24cal  1 tsp Similac powder to 60cc Breastmilk OR  1 tsp Enfamil powder to 75cc Breastmilk Neosure/Enfacare 24cal  3 scoops powder to 5 ½ ounces of water (home) NOTES .

wt loss. Hypothyroidism  Weight gain. papilledema. gallstones  Hip or knee pain. glucose intolerance) have the metabolic syndrome.34 Dysmetabolic Syndrome Obesity is currently the single most prevalent chronic disease in childhood. consider bone age for short obese children where genetic abnormalities may need investigating RECOMMENDED LABS BMI 85-94th percentile without risk factors  Fasting lipid panel BMI 85-94th percentile with risk factors  Fasting lipid panel  Fasting glucose  ALT and AST . dry skin. Obese children with any two of the following abnormal findings (hypertension.Hypertension and Pseudotumor cerebri  Snoring.Obstructive sleep apnea  Abdominal pain.Type II DM  SOB. polyphagia.most children with exogenous obesity are ≥ 75% for age.PCOD.Nonalcoholic fatty liver. blurry vision. nausea/vomiting. acanthosis nigricans. daytime somnolence. Defining the obese Youth (2-20 years old): BMI percentile for age  Underweight: < 5th percentile  Healthy weight: 5-84th percentile  Overweight: 85-94th percentile  Obesityt: ≥ 95th percentile  95% BMI estimate after 9 years of age  Boys: Age in years + 13  Girls: Age in years + 14 HISTORY AND PHYSICAL ESSENTIALS Children who meet the diagnostic guidelines for Dysmetabolic Syndrome should be assessed for the following comorbidities of obesity:  Headaches. cold intolerance. chest pain. dyslipidemia. DOE.Asthma  Psychosocial-Depression.Cushing Syndrome  Abdominal tenderness-Gallbladder disease  Stature.SCFE  Menstrual irregularities. hyperinsulinemia.Hypothyroidism  Polyuria. eating disorders  Acanthosis nigricans-hyperinsulinemia  Hirsutism-PCOS  Violaceous striae.

U/A. etc. Keep TV out of the children’s bedrooms.35 BMI ≥ 95th percentile  Fasting lipid panel  Fasting glucose  ALT and AST  Other tests as indicated by Health Risks such as sleep study. echo. FURTHER LABS TO CONSIDER   Fasting insulin  HOMA. thyroid studies. Urine microalbumin/creatinine ratio (if hypertensive). bone age. whichever is less Stage I hypertension Systolic or diastolic BP >95th percentile Stage II hypertension Systolic or diastolic BP >99th percentile Glucose(fasting) ≥126 mg/dl Glucose tolerance test ≥140 mg/dl Insulin ≥ 20 microIU/ml Urine microalbumin/cr ratio >30 mg/g LIPID PARAMETERS High ≥200mg/dl ≥110mg/dl ≥130mg/dl <40mg/dl Borderline 170-199mg/dl 100-130mg/dl Desirable < 170mg/dl <110mg/dl < 100mg/dl Cholesterol Triglycerides LDL HDL EVIDENCE BASED TREATMENT STRATEGIES         Limit sugar sweetened beverages Consume recommended fruits and vegetables Eat daily breakfast Limit fast foods Use appropriate portion sizes Eat meals together as a family Limit TV time and video games to <2 hours per day. Encourage moderately vigorous physical activity of 60 minutes a day or more .5 CMP.homeostasis model assessment of insulin resistance (Fasting insulin)(Fasting plasma glucose) ÷ 22. HbA1c LABORATORY PARAMETERS Prehypertension Systolic or diastolic BP > 90th percentile for age or 120/80. testosterone level.

36  Motivational interviewing – patient centered communication MEDICATIONS  Weight loss – used in conjunction with behavior modification  Sibutramine 10mg – approved for children ≥ 16 years  Orlistat – approved for children ≥ 12 years COMORBID TREATMENT  Hypertriglyceridemia/Hyperlipidemia  Lifestyle changes and dietary changes  Weight management and increased physical activity  Fiber dose of age (years) + 5 gm/d up to 25 gm/d  Lipitor has been approved for children > 12 years of age with an abnormal lipid panel who have not responded to an adequate trial of diet and lifestyle modification – consider multivitamin for all children on Lipitor Insulin resistance  Exercise/nutritional changes to cause weight loss  Glucophage (metformin):  <12 year old. increase every week up to 15001800 mg/day divided BID  ≥12 years old-start with 500-850 mg qhs.start with 500 mg qhs. increase every week up to 1600-2000 mg/day  Check LFT before and after starting therapy NOTES  .

gastrointestinal symptoms. and vital signs Initial lab data include BMP. The hospital computers have Poisondex which can be found by accessing Micromedex. include cardiac arrhythmia. The major toxic signs of poisonings. chocolate or fruit syrup to make more palatable. Do not need to give a cathartic to any pediatric patient for the initial dose of charcoal. Consider aspirin and Tylenol levels in teenagers. lithium. intubation) before attempting GI decontamination. in general. CMP (for a baseline). and treatments attempted at home as well as any pertinent past medical history Take steps toward further ―decontamination‖ with administration of activated charcoal. cyanides. amount of ingestion. and/or whole bowel irrigation. Give charcoal and remove tube. seizure activity or unusual behavior. boric acid   . such as chemicals on the patient’s clothing and/or skin as well as flushing eyes as appropriate Obtaining as much history and details of the encounter as possible to aid in identification of substance ingested. etc. mineral acids/bases. ingestion of hydrocarbon or caustic agent.37 General Approach to Poisonings/Ingestions Poisoning should be suspected when a patient shows signs of altered consciousness. papillary response. and CNS symptoms. metabolic acidosis..e. or cardiac arrhythmias and any other stabilizing measures Decontamination measures to remove further hazards. alcohols. Can add coca-cola. Drop NG tube if decreased mental status. Can use multiple doses with ½ the initial dose repeated every 2-4 hours — important for substances with enterohepatic circulation. Remember that if the airway is compromised it must be secured (i. osmolality. Only one dose typically used. all heavy metals Absorbs toxins and prevents systemic absorption Dose is 1 g/kg with adult dose being 50 to 100g per dose given in a slurry. ACTIVATED CHARCOAL        Substances that charcoal does not bind or binds poorly—iron. quantitative specific drug levels. Most pertinent physical exam findings include mental status. insuring adequate ventilation/oxygenation. ileus or intestinal obstruction. and urine drug screen on all patients. treating hypotension. See Harriet Lane Handbook for antidotes to specific toxins. coma. Can consider cathartic (Charcoal with sorbitol) for multiple dose charcoal. time of ingestion. including securing compromised airways. solvents. Contraindications include signs/symptoms concerning for possible aspiration with absent gag reflex. CT of head or LP(altered MS). shock. STEPS IN EMERGENCY MANAGEMENT     Management of ABCs. ECG. gastric lavage.

increase until results. GASTRIC LAVAGE      No longer recommended in most ingestion cases since you recover only ~30% of stomach contents and force the rest across pylorus for absorption May be considered in patients who have ingested a large quantity of potentially lethal pills if lavage can be performed within 1 hour Typically. phenobarbital. and alcohols URINE ALKALINIZATION     Use for elimination of weak acids like salicylates.5 to 2 times maintenance rate) Goal urine pH of 7 to 8 Must monitor serum electrolytes to watch for disturbances IPECAC  No longer recommended by AAP for home use due to risk of prolonged emesis limiting other interventions (charcoal) and has limited use in ED or hospital setting. for 4-6 hours. ileus or in situations where the airway is compromised. in adolescents or adults. hemorrhage. 1 to 2 L/hour for 4-6 hours Contraindications include intestinal obstruction. barbiturates.38 WHOLE BOWEL IRRIGATION     Helpful with substances that are poorly absorbed by activated charcoal and with ingestions of substances that have delayed or sustained release formulations Generally. lithium. . methotrexate Use sodium bicarbonate by bolus (1 to 2 mEq/kg) or continuous infusion (D5W with 150 mEq/L NaHCO3 at 1. use polyethylene glycol solution as continuous infusion through an NG tube For pediatric patients: 25 to 40 mL/hour. use 15 mL/kg of normal saline per cycle to max of 200 mL/cycle Contraindications include caustic ingestions or ingestions of hydrocarbons and in patients who are seizing Need to intubate patients with depressed level of consciousness or absent gag reflex to protect against aspiration HEMODIALYSIS   Indicated for low-molecular weight substances with low volume of distribution and low capacity to bind to plasma proteins Use in ingestions of aspirin. theophylline.

Repeat level at 8 hours with extended release formulations. excessive sweating. Plot level on Rumack-Matthew nomogram in Harriet Lane to help identify potential toxicity. NAPQI removed by conjugation with glutathione. May give activated charcoal followed by NAC on initial dose Oral NAC dose is 140 mg/kg then 70 mg/kg every 4 hours for a total of 17 doses. then 100 mg/kg IV x 1 (over 16 hours) Do NOT wait to initiate treatment with NAC if the patient ingested staggered overdoses. and methyl salicylate (oil of wintergreen) Minimal potentially toxic dose is 150 mg/kg Causes uncoupling of oxidative phosphorylation leading to increased heat production.39 ACETAMINOPHEN INGESTION                The most common pediatric ingestion Toxic ingestions normally are more than 150 mg/kg in a child or 7.S. then 50 mg/kg IV x 1 (over 4 hours). bismuth subsalicylate (Pepto-Bismol®). if presentation is more than 6-8 hours after ingestion or if hepatotoxicity is suggested on baseline laboratory values (still obtain a level) It is more cost effective to treat with the IV formulation SALICYLATE INGESTION     Salicylates include aspirin.‖ Chronic Tylenol ingestion may also cause toxicity. Treat ―possible and probable hepatotoxicity. check level and hepatic panel. also interferes with glucose metabolism.5 g in an adult patient Acetaminophen is metabolized by the liver to toxic metabolite N-acetyl-pbenzoquinone imine (NAPQI). and dehydration. If considering. time of ingestion. No intervention is required if ingestion is less than 140-150 mg/kg and 4 hour level is less than 150 Activated charcoal should be given immediately up to 4 hours after ingestion and should be considered after 4 hours with an extended release formulation Treatment with N-acetylcysteine (NAC) should be initiated with plasma levels that indicate potential hepatic toxicity. and any signs of toxicity Lab workup should include a plasma acetaminophen level as soon as possible but at least 4 hours after the ingestion. Primary respiratory alkalosis and primary metabolic acidosis . Acetadote® is the IV formulation that is now available in the U. glutathione stores are exhausted in overdose.—the dose is 150 mg/kg IV x 1 (over 1 hour). Evidence of liver damage develops 24 to 48 hours after ingestion with peak damage after 72 to 96 hours Initial workup should include a thorough history to help clearly define amount of ingestion.

altered mental status.40         Leads to hyperventilation and respiratory alkalosis from stimulation of respiratory center. pulmonary edema. dopamine (D2). hypotonia Initial toxic effects within 30 to 90 minutes and may persist for up to 3 days Treatment is supportive—may need to support blood pressure Needs PICU or PIICU monitoring TRICYCLIC ANTIDEPRESSANTS    Exert effects on the reuptake of norepinephrine. respiratory depression. Supportive care for anti-cholinergic symptoms (Ativan) Benzodiazepines or barbiturates for seizures     . urinary retention. Symptoms: nausea/vomiting. widened QRS. Ach and serotonin Also influences histamine (H1). muscarinic (M1). coma Evaluation should include thorough history and physical as well as serum salicylate level initially and 6 hours after ingestion Use of Done nomogram is currently not recommended Repeat serum levels every 2 to 4 hours until patient is showing clinical improvement and has non-toxic level (<30 mg/dL) Activated charcoal or whole bowel irrigation should be used for gastric decontamination Urinary alkalinization also is used to reduce absorption in distal tubules (See above) Hemodialysis also an option in severe cases CLONIDINE INGESTION     Overdose leads to symptoms from alpha-2 receptor stimulation: decreased level of consciousness. hypotension (may have hypertension from alpha-1 cross stimulation). tachycardia  Dysrhythmias: most common sinus tachycardia. prolonged QTc (Blockade of sodium channels hallmark of TCA toxicity). tachypnea. decreased bowel activity. heart block. ventricular dysrhythmias. dry mucous membranes. and sympathetic α1 receptors Signs and symptoms include:  Anticholinergic signs: dilated pupils. conduction delays. ototoxicity leading to tinnitus. miosis.  Hypotension  Seizures  Altered mental status  Coma Initial altered mental status indicates a significant ingestion has occurred Treatment involves gastric decontamination with activated charcoal or gastric lavage. Late: V-tach and/or V-fib. bradycardia. Should perform gastric decontamination as far out as 12 hours from ingestion.

hemodialysis with methanol/ethylene glycol. ethylene glycol Variety of common substances (antifreeze. miosis Also can lead to seizures. respiratory depression.50. dry mucous membranes. bicarb. use Ethanol drip or fomepizole to inhibit alcohol dehydrogenase.1 mg/kg IV however 1/10 of the dose should be effective. stupor. coolants. TOXIC ALCOHOLS          Include ethanol. Coma. hypotension. sterno. coma. visual disturbances Ethylene glycol: Metabolized to oxalic acid. brake fluid. Osmolar gap evident. pulmonary edema. hypothermia. Do not give lidocaine (sodium channel blocker) Special note: class IA and IC antidysrhythmics are contraindicated due to effects on sodium channels AND phenytoin should be avoided for any related seizure activity because it may potentiate ventricular dysrhythmias OPIOID INGESTION     CNS depression. gastritis. Management: correction of acidosis. coma. moonshine. bicarb. apnea. decreased GI motility. contaminated homebrewed beverages—i. varnishes. rubbing alcohol) Can lead to CNS depression of variable degrees Must obtain serum osmolality. cyanosis. paints. But you may induce seizures and death if patient dependent on opioids. isopropanol NOTES . de-icing solutions. Initial dose is 0. see oxalate crystals with woods lamp to urine Isopropanol: 2-3x intoxicating effects of ethanol. hypotension. If V.e. Methanol and Ethylene glycol toxicity present with coma. hyporeflexia. bradycardia Mostly treatment is supportive: intubate and monitor Naloxone (competitive antagonist) may be considered. bicarb. Alkalinize serum not urine to Ph ~7. methanol. respiratory depression. isopropanol. Has short half-life so may need repeat dosing or a naloxone drip. bronchospasm.41     Volume and norepinephrine or phenylephrine for hypotension Sodium bicarbonate for signs of cardiac toxicity (prolonged QRS)—1 to 2 mEq/kg. metabolic acidosis and renal failure Methanol: metabolized into formaldehyde and then to formic acid leading to metabolic acidosis.tach or fib. No metabolic acidosis. solvents. facial flushing. (To help overcome sodium channel blockade and to alkalinize the serum and bind TCA to albumin). CNS depression. vomiting. wiper fluid.

42 NOTES .

obese). meningitis. choose the longer interval.. the scheduled doses should be held and serial levels followed to determine timing of the next dose  If the level is less than 2mcg/ml.  If the level is above the nomogram. or patients who are not responding to antibiotic therapy monitoring a peak will help determine the dose needed for maximal response  The peak should be drawn at the end of the one hour infusion. It is not indicated for infants <6months. the dosing interval should be every 24 hours  If length of therapy is greater than 5days. Caution when using neuromuscular blockades or calcium channel blockers. receiving Vancomycin and other nephrotoxic drugs. q36.. then the dosing interval should be every 24. burn patients.  Traditional Dosing  Peaks and troughs should be monitored with traditional dosing  The trough is drawn 30min prior to the next dose and the peak is drawn 30 min after a 30 min infusion  Gentamicin/Tobramycin peaks should be 5-10 mcg/ml and troughs < 2mcg/ml  Amikacin peaks should be 20-30mcg/ml and troughs < 10mcg/ml VANCOMYCIN     This is a time dependent bactericidal antibiotic The trough should be 5-15 mcg/ml and the peak between 25-40mcg/ml A peak level should be drawn 60mins after A 60min infusion. 36. If it is on the line. patients with altered volume of distribution (i. decreased development of adaptive resistance. obtain a random level weekly  In patients with altered body habitus (i.43 Pediatric Antibiotics AMINOGLYCOSIDES The concept of once daily aminoglycosides is based on the theories of enhanced concentration-dependent bactericidal activity. 48 hours respectively. edema). A trough is recommended if the patient is on dialysis. or endocarditis.e. The trough is drawn immediately prior to the next dose.  Plot on nomogram (See next page)  If the level falls in the area designated q24. and decreased toxicity due to aminoglycoside free interval with protection against bacterial regrowth due to a post-antibiotic effect. clinical deterioration in status . Monitoring Levels for Toxicity  Once Daily Dosing  Obtain a single random serum level 6-14 hours after the 1st dose.e. A clinical response of 90% is achieved when the serum peak concentration is 8-10 times the MIC of the organism. or q48. or meningitis. A peak is recommended if treating osteomyelitis. endocarditis. has rapidly changing renal function.

If within the normal range. a trough should be measured prior to dialysis. ONCE DAILY AMINOGLYCOSIDES DOSING NOMOGRAM  *Antimicrobial Agents and Chemotherapy 1995. 39(3): 650-655. In dialysis patients.) 13 14 NOTES .44 after 5 or more days of therapy. administer Vancomycin following dialysis. 14 12 10 Concentration (mcg/ml) q48h 8 q36h 6 q24h 4 2 0 6 7 8 9 10 11 12 Time Between Start of Infusion and Sample Draw (hrs. or treating an identified culture with susceptibilities.

The patient needs to be evaluated for peritoneal signs. inflammatory bowel disease. then proceed with study. non-stimulated state. small bowel masses. etc. AIR ENEMA    This study is used to diagnose and reduce an intussusception. Order an Upper GI if primary concern is beyond the esophagus (esophagus will still be visualized). An Upper GI with SBFT is ordered to evaluate for obstruction. BARIUM ENEMA. Hirschsprung disease Magnesium Citrate is often given as a laxative to prep the bowel Do not order colon prep when evaluating for Hirschsprung disease. If none are present. order an esophagram and patient does not need to be NPO. constipation. The duodenum and proximal jejunum are always included with the upper GI. The colon needs to be evaluated in the normal. in case of complications during the study. SOLID COLUMN    Solid column enema is used to evaluate strictures. There is no prep necessary Prior to the study. the patient needs IV access and a surgical consult. ULTRASOUND    For gallbladder evaluation the prep is the same for upper GI Ultrasound is the test of choice for pyloric stenosis For a pelvic ultrasound the bladder should be full   . so no need for SBFT if one is only interested in the duodenum If truly interested in only in the esophagus. Laxatives can distort the configuration of the rectum. AIR CONTRAST  This examination is performed to evaluate for inflammatory bowel disease or polyps. BARIUM ENEMA. This assures visualization of the duodenal C loop. which is necessary to assess for possible malrotation.45 Radiologic Studies and Basic Indications UPPER GI  In an infant or young child the first test ordered should always be the Upper GI.

46 COMPUTED TOMOGRAPHY SCANS    Non-contrast CT requires no prep unless sedation is to be used If sedation is to be used, the patient should be NPO at 4-6 hrs prior to sedation For a CT scan with contrast and without sedation, the infants and toddlers should be NPO for 4 hours prior to the study and children older than 6 years should be NPO after midnight VOIDING CYSTOURETHROGRAM   No prep or sedation is necessary However, mild sedation can be ordered if patient is older than 2 years old. Patient should be NPO as above if patient is to be sedated. NPO GUIDELINE FOR PEDIATRIC PATIENTS SCHEDULED FOR RADIOLOGY EXAM      CT: NPO for at lease 2 hours prior to any contrast given Nuclear Medicine: HIDA scans, Gastric emptying and Reflux (milk) scans NPO 4-6 hours prior MRI: MR cholangiogram/gallbladder- NPO 6 hours prior Other MRI: NPO status based on sedation criteria only Diagnostic  Barium Enema: NPO after MN. For constipation or Hirschsprung disease there is no prep.  Upper GI and Small Bowel Series:  0-6 months- NPO 3 hours prior  6mo-3 years- NPO 4 hours prior  3yrs and older- NPO after MN Ultrasound  Less than 1 yr- NPO 2hours prior  Over 1 yr- NPO 4 hours prior NOTES


Henoch-Schönlein Purpura
GENERAL INFORMATION       Most common systemic vasculitis affecting children IgA deposition found in small vessels Unknown etiology but often preceded by respiratory illness Male predominance with presentation more common in the winter Typical age is less than 10 with younger children having milder disease Course usually lasts 4-6weeks with 1/3rd having recurrences CLINICAL MANIFESTATIONS  Skin: Present in 100% of cases and is the most common presenting symptom. Nonthrombocytopenic purpura often begin as urticarial lesions with progression to petechiae/purpura. Symmetric distribution in gravity dependent areas such as the legs and buttocks. Infants may have lesions on the trunk/face. Subcutaneous edema prominent especially in ages less than 3. Arthritis: 2nd most common manifestation and can often precede the skin findings. Usually periarticular disease involving the ankles and knees. Not associated with joint effusion, erythema, warmth. No permanent deformity. Gastrointestinal: 3rd most common manifestation. Usually colicky abdominal pain but can range from mild symptoms to GI hemorrhage, ischemia, and perforation. 50% with heme positive stools. Intussusception is a rare complication. Its ileoileal location is better diagnosed with ultrasound. Nephritis: Important cause of morbidity/mortality. Most commonly microscopic hematuria but can have associated proteinuria. Rarely progresses to acute nephropathy and renal insufficiency. Can present up to 3months post diagnosis. Scrotal Pain/Swelling: rule out testicular torsion EVALUATION     Clinical diagnosis: Palpable purpura required with one of the following four features: Abdominal Pain, Biopsy with predominant IgA deposition, Arthritis/Arthralgia, Renal Involvement Blood pressure Laboratory tests: CBC with diff, PT/PTT, CMP, stool for heme, Urinalysis Follow urinalysis at diagnosis and with all recurrences. Repeat weekly-bimonthly up to 3months depending on degree of abnormality.

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48 MANAGEMENT    Supportive care Steroids are used for joint involvement and abdominal pain only. Does not prevent recurrences or shorten duration of acute illness. Dose 1-2mg/kg/day x 714 days. Nephrology consult needed for persistent proteinuria, nephrotic range proteinuria, hypertension, renal insufficiency NOTES


50 Gastrointestinal .

51 .

coughing. beets. red blood (BRB) or coffee grounds is associated with a lesion proximal to ligament of Treitz in the distal duodenum  Melena: Passage of black. may help localize bleeding to systems other than the GI tract  In a newborn or breastfeeding infant.) Liver disease. bismuth or spinach). A negative aspirate suggests a lower tract bleed. LOCALIZATION OF BLEED Distinction must be made between upper and lower tract bleeding. Gastric aspiration should be done in all patients with a GI bleed. red blood in stools is consistent with lesions in the ileum or colon  Blood streaks on the outside of stool suggests origin as anal canal or rectum  History of recent epistaxis. GERD End Stage Liver Disease. Indicative of bleeding proximal to the ileocecal valve as well as significant blood loss (over 50-100ml/24hours)  Hematochezia: BRB or dark. Hiatal Hernia. malnutrition. pylori infection. acquired (post surgical. Localization can be frequently made by history and clinical findings. consumptive coagulopathy Esophagitis Esophageal varices Mallory-Weiss Tears AV Malformations Coagulopathy Milk Protein Allergy . etc. Vitamin K deficiency. iron. drugs. Confirming the presence of blood is important.52 Gastrointestinal Hemorrhage Differential diagnosis of hematemesis and rectal bleeding varies with age.. hematochezia (food coloring. severe burns. DIC. tarry stools. etc. Portal Vein Thrombosis Acute Vomiting and retching Congenital. Kool Aid. ICU patients. consider a maternal source (Apt test) ETIOLOGIES OF UPPER GI BLEEDS Differential Diagnosis Duodenal/Gastric Ulcer/Gastritis Causative Factors H. The following characteristics help establish the most likely source:  Hematemesis: Vomiting of bright. NSAID use Erosive/Herpetic esophagitis. peroxidase-containing vegetables. Hemoccult can have false-positive results with ingestion of red meat. or melena (iron preparations. A number of substances may simulate hematemesis (red food dyes or coloring). Omnicef). Head Trauma. immunocompromised state.

53 Trauma Ingestion. Most accurate when done within 2448hours of the active bleed.) acute presentation usually ulcerative Anal Fissure Prematurity/Low Birth Weight Infectious Colitis Henoch-Schonlein Purpura Meckel’s diverticulum Intussusception Coagulopathy Hypersplenism. Lower GI Bleeds  Colonoscopy/Sigmoidoscopy: may be difficult in the case of active bleeding. CMV.  Air Contrast Enema: study of choice when intussusception is suspected. NEC Toddlers: MPI. sharp foreign bodies. May be diagnostic and therapeutic. Vitamin K deficiency. Shigella. difficile Viral Illness Post-viral illness Acute or Chronic Liver failure <5years: Retention polyps (benign. Contrast may hinder further studies so this must be considered carefully. Infectious colitis Constipation Malnutrition. Campylobacter. can detect bleeding at rates as low as 0. coli O157. active bleeding. Salmonella. C. Good for biopsy and diagnosis/treatment of polyps. non-recurring) >5 years: Juvenile Polyposis (adenoma.etc.  Scintigraphy Scan: useful for occult. DIC Polyps Malrotation/Volvulus Inflammatory Bowel Disease DIAGNOSTIC PROCEDURES Upper GI Bleeds  Endoscopy is the procedure of choice.1ml/min . Liver diseases. Infectious Colitis. Accidental injuries ETIOLOGIES OF LOWER GI BLEEDS Differential Diagnosis Colitis Causative Factors Infants: Milk Protein Allergy (MPI). Hirschsprung Disease.  Abdominal Ultrasound with doppler flow is helpful when considering portal hypertension or hepatobiliary lesions  UGI may reveal lesions beyond the reach of the endoscope. Hamartoma. MPI E.

Needs to be performed prior to any barium study. orthostatic changes. Angiography: rarely used in pediatric patients Barium Enema: NO role in lower GI bleeds MANAGEMENT  Assess hemodynamic stability. If tachycardia. or thready pulses are present. hypotension. volume expansion should be initiated until PRBCs NOTES .54    Meckel’s Scan: technetium pertechnetate concentrates in areas of gastric mucosa and bladder epithelium.

It can also be diagnostic when pH probe and UGI are normal. anemia. Gastroesophageal reflux disease (GERD): reflux associated with symptoms or complications Warning signs (suggestive of non-GER diagnosis): bilious/forceful emesis. determines association with symptoms. apnea.55 Gastroesophageal Reflux Disease    Gastroesophageal reflux (GER): physiologic in most infants. Enfamil AR (added rice starch) works only in acid medium (pH less than 5). hematemesis. Do not use with PPI or H2 blockers. evaluates esophageal clearance. pH probe: A probe is placed at the distal esophagus and the pH is recorded for 24hours. Consider 2week trial of hypoallergenic formula. irritability Esophageal: Esophagitis. frequent burping. Usually a ―second‖ line test because it requires hospitalization and is invasive in nature. chronic cough. recurrent pneumonia EVALUATION  UGI: most useful when anatomical abnormality suspected (visualizes through the duodenal C-loop). Histamine-2 receptor antagonists (Zantac). hoarseness. Also. TREATMENT     Reflux precautions: Upright posture with feedings. small feeds Thickened feeds: Addition of cereal to bottles (1/2-1 tsp/oz) will decrease spitting. Nuclear Medicine Milk Scan: useful in diagnosing non-acid reflux and aspiration Endoscopy: Can be useful for diagnosing complications of GERD such as esophagitis or strictures through visualization. abdominal distention /tenderness. onset >6months of age. microcephaly. macrocephaly. hematemesis. asymptomatic and self-limited. wheezing. fever. Multichannel impedance pH probes can detect brief acidic reflux episodes as well as non-acidic GER episodes and can be done when the patient is on medications. ALTE. diarrhea. dysphagia. Proton pump inhibitors (Prevacid)    . hematochezia. seizures COMPLICATIONS    Systemic: Failure to thrive. does not discriminate between physiologic and nonphysiologic GER episodes. stricture Respiratory: Stridor. It detects acid reflux. Formula: small percentage of infants with milk protein allergy. Due to short period of time involved with study. vomiting. Watch for added calories. hepatosplenomegaly. heartburn. It is brief. and assesses adequacy of therapy in unresponsive patients. Acid reduction therapy: Antacids. results can be misleading.

56    Prokinetic agents: i. Erythromycin. NOTES .e. No RTCs supporting their use. Bethanechol. Surface agents: Carafate (not as effective in an alkaline environment) Consider GI referral if symptoms still present after 18months of age or any of the warning signs exist. Reglan.

large hemangiomas  Extravasation of blood: Hematomas. stomatocytosis  Red cell enzyme deficiencies: G6PD. Glycogen Storage Disorders  Tyrosinemia  Hypermethioninemia  Metabolic  Hypothyroidism  Hypopituitarism .57 Neonatal Hyperbilirubinemia DIFFERENTIAL DIAGNOSIS Indirect Increased Production or Bilirubin Load  Hemolytic Disease  Immune-mediated  Rh alloimmunization. abdominal. pyropoikilocytosis. pulmonary. ABO and other blood group incompatibilities  Heritable  Red cell membrane defects: Hereditary spherocytosis. cerebral. consumptive coagulopathy. pyruvate kinase deficiency  Hemoglobinopathies: alpha or beta thalassemia  Unstable hemoglobins: Congenital Heinz body hemolytic anemia  Other causes of increased production  Sepsis  Disseminated intravascular coagulation. elliptocytosis. or other occult hemorrhage  Polycythemia  Macrosomia in infants of diabetic mothers  Increased Enterohepatic Circulation  Breast milk jaundice  Pyloric stenosis  Small or large bowel obstruction or ileus Decreased Clearance  Prematurity  Glucose-6-phosphate dehydrogenase deficiency  Inborn Errors of Metabolism (may also have direct component)  Crigler-Najjar Syndrome  Gilbert Syndrome  Galactosemia.

diaphragmatic hernia (right sided)  Spontaneous perforation of the bile duct  Mass: stone.58 Direct Obstructive  Extrahepatic biliary atresia  Choledochal cyst. Niemann-Pick disease. Hereditary fructose intolerance. annular pancreas. Glycogen storage disease  Bile acid metabolism and transport excretion  Zellweger syndrome and other peroxisomal metabolism  Bilirubin transport: Dubin-Johnson syndrome. prematurity  Disorders of the intrahepatic bile ducts  Alagille syndrome (paucity of the intrahepatic bile ducts)  Nonsyndromic paucity of the intrahepatic bile ducts  Caroli disease: congenital hepatic fibrosis with bile duct cysts  Metabolic disorders  Amino acid metabolism: Tyrosinemia  Lipid metabolism: Gaucher disease. Acyl-carnitine deficiency  Alpha-1-antitrypsin deficiency  Cystic fibrosis  Neonatal iron storage disease  Endocrine  Hypothyroid  Hypopituitarism and septo-optic dysplasia  Infectious  Sepsis  TORCH infections  Hepatitis B  HIV  Drugs and Toxins  TPN  Medications  Fetal Alcohol syndrome  Vascular anomalies  Budd-chiari syndrome  Hepatoendothelioma/hemangioma  Cardiac insufficiency and hypoperfusion  Chromosomal abnormalities  Trisomy 21 and 18 . Rotor syndrome  Mitochondrial disorders. tumor Hepatocellular  Idiopathic neonatal hepatitis. Cholesterol ester storage disease (Wolman disease)  Carbohydrate Metabolism: Galactosemia.

In infants receiving phototherapy. Liver biopsy may ultimately be needed. Hepatitis panel. Infants with jaundice at or beyond 3weeks should have a repeat fractionated bilirubin Check neonatal screens Additional workup is guided by the historical facts and physical exam. Infants should be on Phenobarb or Actigall for 48hours prior to HIDA scan.  All newborns discharged at less than 72hours of age should be seen within 2days or sooner if major risk factors. previous sibling requiring phototherapy. CBC with smear. TORCH titers. Repeat level in 3months if high suspicion. Evaluate infant for sepsis. MANAGEMENT AND PREVENTION The goal is to prevent severe neonatal hyperbilirubinemia and subsequently kernicterus (chronic bilirubin encephalopathy). Jaundice within the first 24hours of life is always abnormal. chromosomes.    . Rule out cholestasis with CMP and GGT. Breastmilk jaundice can cause prolonged jaundice (beyond 2-3weeks) in 20% of infants.  Phototherapy with elevated direct component may lead to the bronze baby syndrome or severe blistering  Discontinue phototherapy when bilirubin <14mg/dL. Significant rebound is rare.  Major risk factors for development of severe hyperbilirubinemia: bilirubin in the high-risk zone. Elevated direct component: Obtain urinalysis and urine culture. Blood group incompatibility with positive DAT. Advise frequent feedings (at least 8-12 times/day). Consider an abdominal ultrasound and a HIDA scan.59 EVALUATION      Fractionated bilirubin: Direct component > 2mg/dL or more than 20% of total is abnormal. significant bruising/cephalohematoma. early jaundice. alpha1-antitrypsin level. infant’s blood type should be obtained along with a Direct antibody test (DAT). East Asian race  Promote and support successful breastfeeding. Blood type and DAT.  Establish the risk zone: See Figure 1  Phototherapy remains the mainstay of treatment for indirect hyperbilirubinemia. Serum bilirubin levels generally peak at day 3-5. gestational age 35-36weeks. a fractionated bilirubin. exclusive breastfeeding.e. etc. Level may be falsely elevated during hemolysis. Breastfeeding jaundice occurs within the first 2-4 postnatal days and is from relative dehydration. Maternal ABO and Rh blood types: If mom is group O or Rh-negative.  Recognize visual estimation often leads to errors  Interpret bilirubin levels according to the infant’s age in hours. i. and reticulocyte count should be drawn Measure glucose-6-phosphate dehydrogenase level in all infants approaching exchange levels or if FH/ethnic origin suggests likelihood. See Figure 2 for guidelines for the initiation of phototherapy.

60   If total bilirubin level is >25mg/dL or lower if <72hours of age. FIGURE 1: ESTABLISHING RISK ZONE 25 20 High Risk Zone 95th %tile Serum Bilirubin (mg/dL) 75th High Intermediate Zone 15 40th %tile Low Intermediate Zone 10 5 Low Risk Zone 0 0 12 24 36 48 60 72 84 96 Postnatal Age (hours) 108 120 132 144 . obtain a type and cross and request blood for possible exchange transfusion. The dose can be repeated in 12 hours. In isoimmune hemolytic disease.5-1g/kg over 2hours) if the total bilirubin level continues to rise despite intensive phototherapy or if it is approaching exchange level. administer IVIG (0. Immediate exchange needed for infants manifesting signs of acute bilirubin encephalopathy.

+ risk factors) 5 days 6 days 7 days NOTES . and well) Infants @ higher risk (35-37 6/7 wk.38 wk + risk factors or 35-37 6/7 wk.61 FIGURE 2 Guidelines for Phototherapy in Hospitalized Infants >35 Weeks 25 Total Serum Bilirubin (mg/dL) 20 15 10 5 0 Birth 24hrs 48hrs 72hrs 96hrs Age Infants @ lower risk (>38 wk and well) Infants @ medium risk (.

62 Respiratory and Allergy .

63 .

expiratory. acute vs. CBC.  Wheezing can be present with lower airway disease. and blood culture if toxic  Esophagram (i. Adenovirus. previous intubations. or fluctuance  Position of comfort: neck hyperextension. hoarse)  Tonsillar size. tripoding  Hemangiomas  Cardiac murmur  Macroglossia. related to feeds. Influenza. chronic course  Recurrent episodes or present since birth  Presence of drooling  Ask about choking episodes. asymmetry. cardiac surgery or PDA ligation  Fever or URI symptoms  Pattern of stridor: inspiratory vs. position. Biphasic stridor suggests fixed lesion. possible FB aspiration. micrognathia DIAGNOSTIC PROCEDURES Evaluation will depend on clinical situation but workup may include:  AP and lateral inspiratory neck films (usually not helpful with chronic stridor)  CXR (inspiratory and expiratory): assess lung volume and r/o mass  ABG.64 Acute Airway Obstruction/Stridor CLINICAL ASSESSMENT History  Duration of symptoms. UGI): evaluate anatomy and for evidence of compression  Airway fluoroscopy: assess airway dynamics (normal study does not rule out pathology)  Echo: cardiac and vascular anatomy  CT or MRI of chest/neck/skull: for choanal stenosis or piriform aperture stenosis  Bronchoscopy/Laryngoscopy: direct visualization SPECIFIC ILLNESS AND MANAGEMENT Croup  Usually viral etiology: Parainfluenza.e. RSV .e. presence during sleep Physical Exam  ABCs/Vital signs  Pattern of stridor: Inspiratory stridor suggests extrathoraic origin while expiratory suggests intrathoraic origin. appropriate weight gain  History of prematurity. worse with position.  Quality of cry (i.

Omega epiglottis is a normal variant Visualize epiglottis in OR and place endotracheal tube Labwork and procedures should be performed after airway stabilized Begin antibiotics to cover typical pathogens (Group A Strep. i. flu Foreign Body       Most common under age 3 Onset can be sudden or have an indolent course ALWAYS consider FB. S. recurrent Generally occurs between age 6months to 3years Child will have a hoarse voice. and H. Watch for rebound in 3-4hours. Strep pneumoniae. and recurrent pneumonia . often no prodrome. rales. Heliox (available 70:30) to improve laminar flow Epiglottitis           Generally occurs 7months to 10years Decreased incidence with introduction of H flu immunization Onset is abrupt and the child is toxic Symptoms include dysphagia.6mg/kg IM/IV x1dose (half life 36hours). barking cough. and drooling Older children complain of severe sore throat but have normal exam May appear anxious and prefer to sit with hyperextended neck (tripod) Lateral neck films reveal the ―thumb‖ sign. aureus. Tracheitis        Can occur at any age during childhood Often superinfectious complication of croup Sudden onset of fever and deteriorates clinically Radiographs reveal irregular tracheal air column with some degree of subglottic narrowing Direct laryngoscopy reveals copious purulent secretions Secure airway and obtain tracheal and blood cultures Start antibiotics to cover S. Presentation depends on location. and audible inspiratory stridor May have low grade fever and viral prodrome Inspiratory lateral and AP neck film reveals ―steeple‖ sign Decadron 0.flu. If the FB is laryngeal. Consider the addition of clindamycin or vancomycin if concerned for MRSA. odynophagia.5ml.05ml/kg/dose diluted to 3ml with NS. 3rd generation cephalosporin. Maximum dosage 10mg.65         Spasmodic croup: Acute onset. pain.25% soln) 0.e. and stridor Tracheal FB will have brassy cough and wheezing If located distally. May repeat in 18-24hours. there are voice changes. Racemic epinephrine (2. aureus). Maximum dose is 0. H. GABHS. there will be wheezing.

Strep sp. >14mm at the 6th vertebrae. neck swelling. or the diameter greater than vertebral body diameter CT scan may be a better tool for diagnosis. improves with prone position Tracheomalacia: expiratory stridor. ―hot potato‖ voice. or symptoms fail to resolve in 48hours.g. Clindamycin with 3rd generation cephalosporin). Other causes           Angioedema Laryngomalacia: inspiratory stridor. aureus. trismus. Obtain ENT consult for I&D. such as Augmentin or Clindamycin. hospitalize and begin IV antibiotics. central/low-pitched wheeze GERD Vocal cord paralysis/dysfunction (Bilateral VCP should have CNS imaging to evaluate for tumors/lesions or structural abnormalities) Tumor/Papillomas/Hemangiomas Laryngeal webs or cysts Vascular rings Hypocalcemia Subglottic stenosis . Consider Decadron with severe airway obstruction or pain Retropharyngeal Abscess         Usually in children under age 4years Patient typically has a history of preceding sore throat and fever The patient may have difficulty breathing or swallowing. drooling Lateral inspiratory neck film shows increased retropharyngeal soft tissue which may be defined as an AP diameter >5-7mm at 2nd cervical vertebrae. and possibly otalgia Physical exam: Medially displaced tonsil with fluctuance. deviated uvula If no toxicity or fluctuance treat with antibiotics. Usually polymicrobial infection. a stiff neck. and anaerobes (e.66   Inspiratory/expiratory chest films as well as decubitus films aid diagnosis Laryngoscopy and/or rigid bronchoscopy is diagnostic and therapeutic Peritonsillar Abscess        Typically in adolescence History of sore throat and fever The patient may have pain. to cover Group A Strep. fluctuance. and anaerobes If toxicity. Consider presence of cellulitis (usually GABHS) if no abscess ENT consult for I&D Start antibiotics for Staph aureus. S.

extrinsic airway compression. aspiration. airway hemangioma. thryoglossal cysts. and failure to thrive Cyanotic episodes. tracheal stenosis. choanal atresia/stenosis. subglottic cyst/nodule. laryngeal web. micrognathia. vocal cord dysfunction Lower airway causes of chronic stridor: Subglottic stenosis. tracheal rings Who needs a bronch for chronic stridor: SPECSR and HIVE Severity: Parent’s subjective impression of severity Progression of the obstruction over time Eating or feeding difficulties. vallecular cysts. laryngeal papilloma. tracheomalacia. ALTEs Sleep: obstruction causing retractions even during sleep Radiology: specific abnormalities detected by radiographs OR H: I: V: E: Hemangioma Intubation Voice Expiratory or Biphasic NOTES   . vocal cord paralysis. laryngomalacia.67 CHRONIC STRIDOR  Upper airway causes of chronic stridor: Piriform aperture stenosis. macroglossia.

Lateral decubitus films may also be obtained. Pleural empyema is a serious complication and rarely resolves without appropriate medical therapy and drainage procedures. If a child remains febrile or unwell 48 hours after admission for pneumonia.68 Parapneumonic Effusion and Empyema Parapneumonic effusions (pleural fluids associated with pneumonia) may be uncomplicated. All children with parapneumonic effusion or empyema should be admitted to the hospital and blood cultures obtained. mediastinum (abdominal infections can also cause a reactive effusion) COMMON ORGANISMS      Streptococcus pneumoniae Staphylococcus aureus Streptococcus pyogenes Haemophilus influenzae Anaerobes DIAGNOSTIC WORKUP AND MICROBIOLOGY     Postero-anterior radiographs should be taken. The natural course of a complicated parapneumonic effusion is to develop into single or multiple loculations and to progress to an empyema cavity. or complicated effusions that require pleural space drainage. COMMON CAUSES OF PLEURAL EFFUSIONS         Pneumonia Trauma Malignancy Collagen Vascular Disease Congenital Heart Disease Renal disease Immunodeficiency disorders Adjacent infection involving the oropharynx. Ultrasound should be used to confirm the presence of a pleural fluid collection and is helpful in determining if loculations are present Patients with pleural effusions which have not been treated with antibiotics should be considered for a thoracentesis to obtain fluid for culture Ultrasound should be used to guide thoracentesis or drain placement if the effusion is not large or multiloculated . parapneumonic effusion/empyema must be excluded. free-flowing effusions that resolve spontaneously with antibiotic therapy. esophagus.

is a consideration. No indication for bronchoscopy. Decortication. 3. and drainage of pus from the pleural cavity under direct vision. etc. .Achieves debridement of fibrinous pyogenic material.Involves an open thoracotomy and excision of the thick fibrous pleural rind with evacuation of pyogenic material. It is a longer and more complicated procedure. MANAGEMENT PROTOCOL FOR PEDIATRIC EFFUSION Treatment Failure @ 48○ New Presentation Suspicion of Parapneumonic Effusion Chest Ultrasound Small.69     If ultrasound suggests a large loculated or multiloculated effusion then a CT scan of the chest to rule out intraparenchymal lung abscess or other anatomical lesions is usually required by pediatric surgery prior to proceeding with VATS. The algorithm is for presumed infectious effusion. Where possible. break down of loculations. Blood cultures should be performed in all patients with parapneumonic effusion All cases should be treated with intravenous antibiotics. Consult Pediatric Surgery for chest tube placement. antibiotic choice should be guided by microbiology results. free effusion no loculations Consider thoracentesis Small to moderate loculated effusion Insert chest tube Large loculated or multiloulated Chest CT Institute antibiotics and/or consider AB change for resistant pathogen Consider intrapleural fibrinolytics VATS or Thoracotomy 1. immediate thoracentesis should be done if malignancy. 2. There is no indication for diagnostic bronchoscopy SURGICAL PROCEDURES   VATS (video assisted thoracoscopic surgery) .

70 NOTES .

01ml/kg/dose (max single dose 0. Additional manifestations of immediate hypersensitivity include angioedema and urticaria. H1-blocker (Benadryl) 1-2mg/kg/dose po/IV/IM up to 75mg/dose q4-6hours Consider. Common causes include insect venoms. 15% of patients have no urticaria or angioedema. drugs. repeat bolus and consider adding pressor support. flushing. Trendelenburg position if hypotensive. obtain a serum tryptase level within 90 minutes. CLINICAL FEATURES      Sense of impending doom may proceed or accompany symptoms Cutaneous symptoms: feeling of warmth. hypotension. or dysphagia Gastrointestinal: nausea. Individuals with anaphylaxis warrant an allergy referral. eggs. It should be elevated with acute anaphylaxis. Children <30kg should get the Epi-Pen Jr. Delayed worsening can occur several hours later. Treat hypotension with NS bolus 20cc/kg. Respiratory: stridor. If hypotension persists. Once acute attack subsides.5mg/kg/dose (max 50mg) IV q8hours Methylprednisolone (Solumedrol) 2mg/kg/day IV divided q6hours. NOTES . milk. For patients with bronchospasm. These reactions usually occur within seconds to minutes in individuals with preexisting antibodies. Can be repeated up to 3 doses. Tourniquets are no longer advised. the epinephrine should be administered at the same site to minimize further absorption. A loading dose of 2mg/kg/dose may be given as the initial dose. If anaphylaxis results from a sting or injection. and diarrhea Cardiovascular: tachycardia. contrast. If the diagnosis is in question. bronchospasm. and latex. Discharge patient with an Epi-Pen. itching. shock. Maintain airway. and death MANAGEMENT             ABCs. and angioedema.5ml) IM every 10-15minutes. H-1 blocker should be continued for 48-72hours. Wean steroids according to severity of attack. vomiting. hoarseness. H2-blocker (Zantac) 1. seafood).71 Anaphylaxis Anaphylaxis is an immediate IGE-mediated hypersensitivity reaction. urticaria. They have limited shelf life (27months) and will lose their effectiveness. Administer epinephrine (1:1000) 0. foods (such as peanuts. administer nebulized beta-agonists Anaphylaxis is often biphasic.

congenital airway anomalies. It is characterized by the triad of smooth muscle spasm. or bronchitis DIFFERENTIAL DIAGNOSIS OF WHEEZING   Infectious: Bronchiolitis. cyanosis   . difficulty with completing full sentences. SCM). pulmonary edema. hospitalizations.  Wheezing  Dyspnea with little provocation  Cough  Exercise intolerance  Chest tightness/pain  Night awakenings  Recurrent croup. interstitial lung disease. allergens. exercise. smoke  Comorbid conditions: GERD. pneumonia.e. tumor/granulation tissue). wheezing. alpha-1-antitrypsin deficiency. bronchiectasis. primary ciliary dyskinesia. reactive airway disease. Wegener Cardiovascular: Vascular rings. retractions. allergic rhinitis. reversible airway obstruction. colds. chronic sinusitis. HISTORY  Previous history of asthma. PRESENTING SYMPTOMS It is important to understand that all wheezes are not asthma and all asthma does not present with wheezing. nasal flaring. grunting. The presentation can be subtle and varied. pneumonia. and medications  Previous PICU admissions or intubations are risk factors for death from asthma  Ask about frequency of steroid or Beta-agonist use as well as ED visits  Asthma triggers: weather changes. ABPA  Night cough or chronic cough  Exercise-induced symptoms Rule of Twos: Symptoms more than two times per week or more than two nights a month should be considered persistent asthma.72 Asthma Asthma is a chronic disease of recurrent. foreign body. intrabronchial lesions (i. hypersensitivity pneumonitis. cystic fibrosis. mucosal inflammation. agitation. ABPA. anxiety. viral croup Respiratory: Asthma. and mucus hypersecretion. mitral valve prolapse. congestive heart failure Gastrointestinal: Gastroesophageal reflux PHYSICAL EXAM  General appearance: accessory muscle use (esp.

SQ or IV drip of Terbutaline. An inhaled anticholinergic agent (Atrovent) works synergistically with Albuterol. Steroids do not need to be tapered if given less than 10days. polyphonic. monophonic vs. diffuse vs. Rales/rhonchi may be present. high vs. Morgan Dennie lines. Consider PICU admission if these therapies are required.e. PO and IV steroids are equally efficacious. Wheezing may not be heard with poor air exchange. It should be used acutely in the outpatient or ED setting but not for routine hospital use. However. Prolonged expiratory phase. chronic asthma or more often depending on symptoms. focal). Musculoskeletal: Clubbing LABORATORY EVALUATION     Blood gas: Evaluate PaCO2 as indication of patient’s ability to ventilate. conjunctival cobblestoning Respiratory: Inspiratory/expiratory wheezing. Usually given 2mg/kg/day divided BID as 5day course. PFTs should be obtained yearly in stable. PO/IV steroids (i. Solumedrol) depending on severity. Orapred. Look for pneumomediastinum or pneumothorax. Pulsus paradoxus (reduction of systolic BP more than >10mmHg during inspiration) HEENT: nasal polyps. Quality of wheeze (i.e. NOTES  . peak flows can be used as an objective measure. Interpret in light of respiratory rate. low pitch. Chest X-Ray: Hyperinflation. 20% will have infiltrates. Pulmonary Function Testing (PFTs) have no place in an acute attack.73    Vital signs: Tachycardia (from distress or Beta agonist use). leading to low serum levels. Normal PaCO2 may be an ominous sign in a tachypneic child. or Magnesium. TREATMENT      Oxygen as needed Inhaled beta2-agonists (albuterol) are the mainstay of acute treatment. Potassium: Beta-agonists drive potassium into the cells. atelectasis. MDI and spacer are equally effective if not superior to nebulization. Severe exacerbations may require continuous albuterol nebulization. allergic shiners. or a combination of the two.

74 NOTES .

False positives and negatives can occur. DIAGNOSIS OF CF SC currently screens all infants for CF with blood immunoreactive trypsinogen (IRT).  Mutation analysis with 2 characteristic alleles  Increased nasal transmembrane potential differences CLINICAL PRESENTATION CFPANCREAS Mnemonic: exacerbations/presentation C Cough worsening/chronic cough F Fever/FTT P Pulmonary Function Tests decreased/Pancreatic Insufficiency A Appetite decreased/Alkalosis N Nutritional failure/Neonatal meconium ileus or nasal polyps C CBC abnormality/Clubbing R Radiographic changes/Rectal prolapse E Exam (increased rales. This gene codes for a chloride channel named the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). repeat screen indicated. retractions)/Electrolyte abnormalities A Activity decrease/Absent vas deferens S Sputum changes/Sputum with Staph or Pseudomonas Pulmonary   Pulmonary disease is the major cause of morbidity and mortality Superimposed on chronic disease are episodes of acute exacerbations . Children with sweat values between 40 and 60 should be considered for genotyping if there is an elevated suspicion for CF. the following is needed for diagnosis: Requires one of the following  Presence of one or more characteristic clinical features  Family history of CF  Positive neonatal screening test AND evidence of CFTR abnormality  Sweat chloride ≥60mmol/L (mEq/L) on 2 occasions: quantitative pilocarpine iontophoresis collected by Gibson-Cooke procedure. which accounts for over 70% of CF cases. If elevated (≥105ng/ml). It is an autosomal recessive disorder with the genetic defect located on chromosome 7q31. The most common mutation is the ΔF508. wheezes. tachypnea.75 Cystic Fibrosis Cystic Fibrosis (CF) has an incidence of 1 in 3200 newborns in the United States. Requires collection of at least 100mg sweat in 30minutes. If continued elevation (≥70ng/ml).2.

flu. rusty sputum. hypoproteinemia with/without edema. neuropathies/spinocerebellar syndrome Impaired coagulation Gastrointestinal    Rectal prolapse GERD Several intestinal obstructive lesions can occur at different ages. or eosinophilia Other problems encountered include nasal polyps. such as abdominal pain and emesis. aureus.  Meconium Plug Syndrome is much less common and affects the large intestine. NTM Pneumothorax occurs in 3-5%. pulmonary infiltrates. there is partial or complete obstruction of the terminal ileum. This is also associated with Hirschsprung Disease. pansinusitis.  Distal Intestinal Obstruction Syndrome (DIOS) is equivalent to meconium ileus in the older child. RAD/asthma occurs in 25-50% Allergic Bronchopulmonary Aspergillosis occurs in 5-10% and typically presents as wheezing.76        Causative organisms include S. H.  Malabsorption may present as steatorrhea or FTT  Pancreatic insufficiency can lead to fat-soluble vitamin deficiencies. Stenotrophomonas. due to chronic hypoxemia. night blindness. Each of these will present with signs and symptoms of bowel obstruction. It is more common in pancreatic sufficient patients. Typically.  Abnormal glucose tolerance and diabetes can occur Vitamin Deficiencies Vitamin A: Vitamin D: Vitamin E: Vitamin K: Xerophthalmia. Those who are ―pancreatic sufficient‖ generally have less severe disease. and anemia  Pancreatitis is rare. There is a high incidence of recurrence.  Meconium Ileus is a small intestine obstruction. . is a common finding with end stage disease Gastrointestinal Pancreas  Pancreatic insufficiency affects 85% of patients and leads to malabsorption. B cepacia. Alcaligines. It is pathognomonic for CF. dry skin Osteopenia. Pseudomonas. rickets Hemolytic anemia. and hemoptysis Clubbing is found in almost 100% of patients and correlates with severity of lung disease Cor pulmonale.

Hypercalciuria. They should be performed on admission and every 5-7days during the exacerbation. . Give vitamin K if needed. It includes clubbing and may involve the distal ends of tubular bones.5mg.  Hypertonic saline may be used in both the acute and chronic settings to normalize airway surface liquid and to enhance mucous clearance. and microscopic hematuria MANAGEMENT Pulmonary The major goal is to delay and possibly prevent development of chronic lung disease. Emergent Pulmonology involvement if severe. hemoptysis. 2. nephrocalcinosis.  CPT should be done minimum of BID as an outpatient. Many devices such as the Vest or procedures are available. Response to therapy is indicated by improvement of FEV1 and FVC. portal HTN. and hoarseness.  Bronchodilator therapy has been controversial. and liver failure can develop Other Clinical Findings     Impaired fertility in males (absent vas deferens) and females Electrolyte abnormalities including hyponatremia. metabolic alkalosis Hypertrophic pulmonary osteoarthropathy occurs in about 15% of patients. check PT/PTT.  Airway clearance can be improved with chest physiotherapy (CPT). Β2-agonists are generally used BID-QID.  PFTs are the most accurate and objective measure of pulmonary status. Recommend pre and post testing prior to its initiation due to risk of bronchoconstriction.  If hemoptysis develops. Side effects include anaphylaxis. and more often with exacerbations  Pulmonary secretions can be thinned with Pulmozyme (one vial. nebulized qday-BID). Stop pulmozyme and CPT. hypokalemia. hypochloremia.  Overnight pulse oximetry should be used during hospitalizations to evaluate for hypoxemia. but up to 50% of patients have an RAD component. Strengths range from 310% and should be administered BID following Albuterol to prevent bronchoconstriction.77 Hepatobiliary    Clinically significant cholestasis is found in 5% of patients Fatty infiltrate of the liver is a common finding (steatohepatitis) Focal biliary cirrhosis.

TOBI (300mg tobramycin/5cc ampule) is dosed 1 ampule BID for 4weeks. Dose is 500mg M. Some patients require enteral feeding to ensure adequate calories. To evaluate.78 Infectious Disease          Antibiotics are the mainstay for treating pulmonary exacerbations. Fat-soluble vitamins should be supplemented Vitamin A. This also should not be done during acute exacerbations as many CF patients will have impaired glucose tolerance during illness. and E as well as PT/PTT (Vit K) should be performed annually Patients on aminoglycosides should be on twice weekly po Vit K regardless of PT. In adolescents consider yearly fasting glucose to evaluate for diabetes. Prealbumin and daily weights should be followed in the hospital Oral enzyme replacement therapy with all meals and snacks is crucial. and multi-drug resistant Pseudomonas (defined as resistance to all antibiotics within 2 out of the following 3 drug classes: beta lactams. HbA1C is not the most accurate test in CF but should be done yearly. aminoglycosides. Once daily aminoglycosides are frequently utilized. Diagnosis is made through oral glucose tolerance testing. Doses in excess of 10. These patients may need insulin during exacerbations. Azithromycin is given for kids >6years who have chronic Pseudomonas and no history of NTM infection.e. the sample should be sent for synergy studies if not performed within the past year Inhaled antibiotics are recommended for maintenance prophylactic therapy. Antibiotic choice is based on prior sputum cultures until a new sample can be obtained and cultured If a culture reveals a multidrug resistant organism. fluoroquinolones) Administer influenza vaccine annually Gastrointestinal and Nutrition       Optimal growth and nutrition is associated with improved long term outcome Caloric goals should be 130-150% of normal caloric intake for age Formulas with MCT oil may be easier to absorb (i. Acid suppression may be needed to enhance enzyme activity. obtain 72hour fecal fat. Typically antibiotics are administered IV and dosages are high in comparison to those used in other children. B. then off for 4weeks. Periodic evaluation of CMP and GGT should be performed      . Those with prolonged PT should have daily Vit K until PT normalizes. Contact precautions for children with MRSA. Combination therapy is used to slow the emergence of resistant organisms. but duration is based on PFTs and clinical course.W. Treatment for exacerbation generally includes 14-21 days of IV therapy. cepacia. Portagen. D.000 units of lipase/kg/day should be avoided as they have been associated with fibrosing colonopathy. but are typically not used in acute exacerbations. Alimentum) Dietary supplements such as shakes or glucose polymers should be considered.F for >40kg and 250mg for <40kg.

consider abdominal ultrasound and Actigall Guidelines for Initiation of Pancreatic Enzymes Infants: 2.000-4. 500 units lipase/kg/snack Children >4years: 500 units of lipase/kg/meal. 250 units lipase/kg/snack NOTES .79  For hepatomegaly or abnormal LFTs.000 units lipase/120cc of formula or breastmilk Children <4years: 1.000 units of lipase/kg/meal.

80 Neurology .

81 .

Partial seizures are divided into simple or complex. less than 15 minutes. Febrile Seizures  Age of onset between 6 months and 6 years  Temperature greater than 101F  No previous episodes of non-febrile seizures  Simple FS: generalized. Seizures involve both sides of the body from the onset of seizure. clonic. or semi-purposeful movements. or autonomic.82 Seizure Disorders TYPES OF SEIZURES Status Epilepticus  A prolonged or repetitive seizure lasting greater than 10minutes which is considered a medical emergency. typically occur in children 2-5 years old. sensory. involves staring (infrequently with automatisms) in a 4-12 year old. Classically.  Simple partial seizures may be motor. face. then midazolam infusion or pentobarbital drip if needed under the guidance of the ED or PICU) Generalized Seizures Loss of consciousness is usually the first manifestation. Partial Seizures Focal seizure activity is often seen clinically on one side of the body. There is a generalized loss of muscle tone causing collapse.  Complex partial seizures involve impairment of consciousness.  Atonic seizures. aura. longer than 15 minutes or more than 1 per 24 hours  Excellent long term prognosis (97-98% resolve by 5-6 years of age) . Different types include:  Tonic/clonic  Absence seizures have a brief impairment of consciousness. These are often provoked by hyperventilation. brief shock-like contractions of the child’s entire body. no more than 1 per 24 hours  Complex FS: partial/focal. The patient may have automatisms. myoclonic)  Non-convulsive (absence or partial)  Treat sequentially until controlled (every 10-20 minutes with lorazepam then fosphenytoin. tonic-clonic. EEG demonstrates generalized 3 Hz/sec spikes. tonic.  Myoclonic seizures are sudden.  Convulsive (generalized or focal. then Phenobarbital. déjà vu. often referred to drop attacks. so it is important to obtain history regarding the seizure’s first manifestation. There is no change in the patient’s mental status. or trunk. Partial seizures may generalize.

Also consider imaging in neonates and patients older than 16 years at onset of seizures. Laboratory evaluation (performed in historical context) to consider include CBC. However. ANA. Consider non-accidental trauma and ingestion. ―bicycling‖. These movements are characterized by repetitive flexor/extensor spasms. Magnesium. breath-holding spells. amino acid screen . generalized).     . Urinalysis. Neuroimaging has a low yield in evaluating children with a first. Give particular attention to the laboratory evaluation of the neonate. or delayed return to baseline. and serum ammonia. A full sepsis workup should be performed with consideration of HSV. MRI is the best imaging modality. but CT is often easier to obtain. night terrors. focal or prolonged seizure. Laboratory tests are of little value in evaluation of the first. and surrounding circumstances of the seizure. EEG is recommended as part of the neuro-diagnostic evaluation except for a healthy child presenting with a first typical febrile seizure. neurologic. unprovoked nonfocal seizure as well as with a first simple febrile seizure. drug screen. It is indicated in cases of trauma. and birth history. In children with febrile seizures. pseudoseizure. A thorough physical exam should be completed with attention to vital signs. Imitators of seizures include syncope. lead level. nature at onset (focal vs. Exceptions can be made if the treating physician feels otherwise. unprovoked seizure in children over 2 without prolonged post-ictal confusion. It should be strongly considered in children 12 18 months. tremor. trauma. GER.  EEG demonstrates hypsarrhythmia INITIAL SEIZURE WORKUP     A detailed history should be obtained of the duration. fundoscopic. and paroxysmal vertigo. abnormal neurologic exam.83 Neonatal Seizures  Seizures in the neonate can present with repetitive contractions. sustained posturing. The yield is repeated with subsequent routine studies or prolonged monitoring. tongue thrusting. Due to the neonate’s immature neurologic system. Infantile Spasms  Typically occurs 3months – 1 year of age  Typically presents as clusters of spasms on awakening. over-reliance on EEG should not be used to delay treatment and/or other testing in patients with a history clinically consistent with seizures. In a child older than 18 months. developmental. a lumbar puncture should be performed in children less than 1 year of age. Up to 50% of patients with new onset seizures may have a normal EEG initially. cardiac arrhythmia. head circumference. developmental delay. clinical skills should be used to determine the need for an LP. generalized seizures are rare. and cutaneous exams. Past medical history should focus on previous seizures. Screen for inborn errors of metabolism. or eye deviation. CMP.

84 NOTES .

Malignancy. encephalitis Subdural/Epidural hemorrhage Alternating hemiplegia of childhood ETIOLOGY               Cardiac Disease (accounts for 50% of ischemic stroke) Infection: Varicella (postinfectious). Takayasu arteritis Hemoglobinopathies: Sickle Cell Hematologic: Polycythemia. cranial nerve signs/symptoms DIFFERENTIAL DIAGNOSIS          Migraine Seizure +/. and/or multifocal/generalized neurologic dysfunction. anterior cerebral or middle cerebral distribution)  Language difficulty and right hemiparesis suggest left carotid or left MCA  Left hemiparesis suggests right carotid or right MCA Posterior circulation (vertebral and/or basilar distribution)  Vertigo. stroke arises from ischemia. Meningitis. Fabry disease Arterial Dissection: Marfan. and/or thrombosis.85 Stroke in Childhood Stroke in childhood usually presents with acute focal neurologic disturbance. Pathophysiologically. AVMs. Infrequently strokes may present with subacute symptoms/signs. PAN. Ehlers-Danlos Vasculitis: SLE. Thrombocytosis Coagulopathy/Thrombocytopenia Vascular Dysplasias: Moyamoya disease.Todd’s paralysis Hypertensive Encephalopathy Hypoglycemia ADEM/MS Brain Tumor Infection: abscess. hemorrhage. Cocaine use Diminished systemic perfusion: anaphylaxis. sepsis. Mitochondrial disorders. The most common clinical manifestation is acute hemiparesis. HIV Metabolic: Homocystinuria. LOCALIZATION   Anterior circulation (carotid. Intracranial aneurysm Head Trauma Vasospasm: Migraine. Kawasaki disease. Fibromuscular Dysplasia. chemotherapeutic agents Idiopathic (up to 1/3 with unidentified cause) . hypoxia/asphyxia Drugs: OCPs.

include imaging of cervical and proximal intracranial arterial vasculature CT if MRI not readily available Echocardiogram CBC. do not use in a hemorrhagic stroke or in those with sickle cell disease Consider the use of Aggrenox or Plavix in the older child (long term prevention) Consider anticoagulation for extracranial arterial dissection associated with arterial ischemic stroke. neurosurgery. PTT ESR. cerebral venous sinus thrombosis. Antiphospholipid profile Lipid profile Urinalysis and drug screen Blood Pressure Prothrombotic workup: see Thrombotic Disorders Metabolic workup: as directed by the Neurologist Varicella titers HIV ACUTE MANAGEMENT         ABCs Maintain normal temperatures and glucose homeostasis Do not treat hypertension acutely unless formally agreed upon by neurology.86 EVALUATION             MRI/MRA investigation of choice. and for those with a cardiac source of embolism If sickle cell disease: See Sickle Cell Disease CVAs NOTES . hematology. PT. and/or PICU (potentially can worsen ischemia) Early neurosurgical/neurology consult Aspirin 5mg/kg/day should be administered initially for arterial ischemic stroke. ANA.

often monosymptomatic. Astrocytomas. antihistamines. systemic symptoms present with widespread CNS disturbance . seizure. headache. paraesthesias. AMS. diplopia. hydrocephalus. brainstem Gliomas  Congenital malformation: Chiari malformation. etc.  Ingestion: anticonvulsants. ataxia. or neurologic disease LOCALIZATION Cerebellar Ataxia  Wide based gait  Truncal ataxia (with sitting and standing)  Titubation (head bobbing)  Dysmetria  Nystagmus Sensory Ataxia (involving position and vibration sense)  High stepping gait  Sensory changes (joint position sense/proprioception)  Diminished reflexes  Romberg sign  Abnormalities of fine finger movement DIFFERENTIAL DIAGNOSIS Consider age appropriate developmental skills before diagnosing ataxia. Dandy Walker. weakness. alcohol. photophobia. encephalitis. etc  Infection: meningitis. MRI with periventricular lesions  Acute Disseminated Encephalomyelitis (ADEM): monophasic demyelinating disease. vertigo. Acute Cerebellar Ataxia  Brain Tumor: Medulloblastomas. HISTORY      Onset and duration Fever.87 Ataxia Ataxia is defined as impaired balance and incoordination of intentional movement. or environmental exposures FH of migraine. vomiting. The most common presenting symptom is an abnormal gait or tremor. tremor Developmental screen Recent infection. Myelopathy (spinal cord). cerebellar abscess  Multiple Sclerosis (MS): recurrent demyelinating disease. head injury. heavy metals. myopathy and neuropathy can also present with gait disturbances.

ADEM. or spinal cord tumor. urine ketones. increased ICP.  Urine drug screen. post-infectious demyelinating disease. obtain pan cultures with lumbar puncture  Lumbar puncture: Elevated protein is found in Miller Fisher syndrome/GBS. ophthalmoplegia. and ACA. Complex or unclear exam and localization may need MRI of the spinal cord. CMP. specific medication levels. characterized by symmetrical ascending weakness. Triad of ataxia. and sensory complaints WORKUP This is guided by historical facts and physical exam supporting localization and suspected diagnosis  Neuroimaging: CT scan is essential for emergent imaging but MRI is more efficacious in visualizing the posterior fossa. areflexia. vasculitis  Trauma/Postconcussion Syndrome Recurrent Cerebellar Ataxia  Migraine variants  Basilar migraine  Benign Paroxysmal Vertigo  Dominant recurrent ataxias: episodic ataxias caused by ion channel defects. MS.  If concern for infection. age 2-6years most commonly.  Urine HVA/VMA and chest/abdomen images for neuroblastoma  Metabolic workup to include serum/urine amino acids. no systemic symptoms  Vascular Disorders: stroke.88 Miller Fisher Syndrome: variant of Guillain-Barre. Caution if patient has mass effect. postinfectious etiology with abrupt onset. plasma/CSF lactate and pyruvate NOTES  . CSF with elevated protein  Opsoclonus myoclonus ataxia: paraneoplastic syndrome associated with Neuroblastoma  Acute Cerebellar Ataxia (ACA): diagnosis of exclusion. often a positive family history  Metabolic disorders  Maple syrup urine disease  Hartnup disease  Pyruvate dehydrogenase deficiency Acute Sensory Ataxia  Guillain-Barre Syndrome (GBS): acute. Oligoclonal bands are also present with MS. and areflexia. With complex or unclear exam/localization should consider CPK to rule out myopathy. ammonia. urine organic acids.

89 NOTES .

90 Endocrine .

91 .

qhs.etc. polydipsia. weight loss Polyphagia (often not present in children and new onset Type I diabetes with ketosis) Vomiting. If they have an insulin pump. nocturia. Thyroid exam Skin: Acanthosis nigricans.  Blood glucose checked qac. Will vary with insulin plan. ICA 512 (IA-2) Ab. headache. anti-peroxidase and anti-thyroglobulin antibody  Test for celiac disease only if symptomatic (tissue transglutaminase IgA with concurrent total IgA for validation)  If obese consider insulin level or C-peptide level. abdominal pain If known diabetic. think celiac dx. ask insulin dose and regime (units/kg/day) and time of last insulin.g. ask about last site change and if any problems occurring. and 0200 (0200 may be omitted in established diabetics). striae. Rheumatoid Arthritis. Assess for symptoms or FH of autoimmune disease (e. look for hypotension or Cushings triad Assess hydration status Looks for signs of infection and evaluate neurologic status Plot on growth chart/BMI: Obesity see Dysmetabolic section. vitiligo Pubertal status    Laboratory  Fingerstick blood glucose  Stat BMP and urinalysis (dipstick okay). Lipids and urine for microalbumin should be checked as outpatient when sugars better controlled. or adrenal dx. Celiac disease.25 or HCO3 <15 mmol/L and presence of urinary or serum ketones EVALUATION History      Physical      Polyuria.Blood gas. DM. . +/. Insulin Autoantibodies  Thyroid functions (when out of DKA): TSH. Multiple Sclerosis. GAD 65 Ab.) ABCs With DKA. Total T4. thyroid dx. SLE. Vitiligo.  New onset diabetic: HbA1C. If short stature.92 Diabetes Mellitus DEFINITION    Fasting glucose >126 mg/dL Random glucose >200 mg/dL PLUS symptoms DKA defined as Glucose >250 mg/dL and pH <7. thyroid dx.

less than age 5 start 0.g.93   Urinary ketones should be checked for all sugars >240. NPH)  1/3rd as short or rapid acting insulin (i.  Long acting insulin (e.8 units/kg/day. Peak 1-2hours. *Sensitivity factor: If insulin sensitive divide 1800 by total daily dose of insulin. check urine for ketones with next blood sugar. The insulin:carb ratio is determined by dividing 500 by the total daily dose of insulin. Regular or Humalog/NovoLog/Apidra)  1/3rd total dose in PM  1/3rd as short or rapid acting insulin before dinner  2/3rd as intermediate acting insulin at Bedtime  Basal-Bolus (Multiple Daily Injection. Glulisine (Apidra).e. Short: 30 minute onset. Aspart (NovoLog).g. Consult Endocrinologist on call. Peak 6-10 hours.  Give a ―correction‖ formula: Blood sugar – target glucose divided by sensitivity factor. Peak 2-4hours. E.  2/3rd total dose before breakfast  2/3rd as intermediate acting insulin (i. Duration 10-18hours. *The target glucose is usually 120. Lantus. Usually start insulin at 1unit/kg/day.6-0. lispro (Humalog).e. Example: In a 50 kg child receiving 1unit/kg/day. Regular (Humulin R/Novolin R) Intermediate: 2 hour onset. This is for Regular insulin. If insulin insensitive divide 1500 by total daily dose of insulin. MDI): This method is diet flexible but requires more injections. Insulin regime:  Split-fixed (3 shot method): This method requires less injections but involves a fixed diet. they would receive 1 unit of rapid acting insulin per every 10 grams of carbs. If positive. then add 1-2 for every 50 above 200. Often used with insulin pumps and other basalbolus plans. THERAPY  Sliding Scale with Regular Insulin: Can use for mild-moderate DKA treated on the floor or if initiation of insulin is not immediately conducive  wt(kg)/8 = #units for BS 150-200.  You can give meal bolus and correction insulin concurrently Alternate insulin regimes may be used at the discretion of Endocrinology INSULIN PREPARATIONS    Rapid: 5-15minute onset. NPH (Humulin N/Novolin N)    . Levemir): 40-60% of total daily dose given at bedtime  Rapid acting based on carb counting with meals.

Adjust as rate as needed.  The goal is for the glucose to decrease at a rate of 75-100mg/dl/hour. DKA MANAGEMENT Initial Management  Place 2 large bore IVs  Bolus 10-20cc/kg NS over 1hour. (Hypernatremic dehydration over 48hours).94  Long: 1-2 hour onset with 24hour duration (peakless). need for insulin drip. Glargine (Lantus). give Glucagon kit IM/SQ (available on the floor). .5mg/kg/dose IV q8hours (max 50mg/dose)  Consider an arterial line (1unit heparin per cc NS @3cc/hour) if repeat blood draws needed. Detemir (Levemir). Transition to Maintenance Therapy  The time to transition.  Mindful to try to limit Chloride infusion. Subtract the rate of the insulin drip and arterial line fluids.  PICU admission required for altered level of consciousness. reassess after first bolus  Do NOT give insulin bolus. Watch for emesis. hinges on clinical presentation.  Fluid requirement should be replaced over 36 to 48 hours. and cumulative I&Os  Q2hour: BMP. VS.g. This is usually 1 ½ MIVFs. Use Regular insulin. Fluid Management  Calculate fluids by adding maintenance fluids.  Do not give more than 4L/m2/day. patient’s home insulin regimen (if available) and time of day. give 15 grams of carbs and reassess in 15 minutes. Insulin Infusion  Began insulin infusion at 0.1units/kg/hour. etc. fluid deficit. Monitoring  Q1hour: Bedside glucose. Often used with basal-bolus regimen. +/. and the type of insulin to switch to.Blood gas  Q6-12hours: urine glucose and ketones  Consider following urine ketones until clear.  Sodium Bicarbonate should be avoided unless hemodynamic instability. E. neuro checks. and ongoing losses. Phosph. If not. HYPOGLYCEMIA IN THE DIABETIC   If awake and alert. Nursing Orders  Cumulative I&Os  Vital signs with neuro checks q1hour  Ranitidine 1. Anticipate starting insulin after fluid bolus. serum osmolality.

If Lantus is not given immediately. Depending on the time of day. If transitioning at other times. Start po fluids (sugar-free) when HCO3 >15-18. Reassess patient’s need for persistent IVFs.e. give Regular SubQ insulin 30minutes before 1st meal and then feed. give the ―correction‖ insulin (see formula above) until it is time for the basal insulin. the basal insulin (i. If using a basal bolus insulin regime. turn off the insulin infusion and dextrose fluids at the same time the Rapid acting insulin is given. When doing this it is important to follow glucoses q2hours so corrections can be given. Turn off insulin infusion and dextrose fluids 30min after SubQ Regular insulin given. NOTES  . you can use the past (or projected) home regimen. Lantus) may be given. Consult Endocrinology if not already done. you may wish to first use a temporary sliding scale of Regular insulin. If using a spit fixed insulin regime.95      If transition occurs at ―usual‖ meal time.

Cosyntropin stimulation testing can aid in diagnosis. fever. Dexamethasone does not interfere with testing. or shock Non-acute: weakness. In infants (esp. bolus NS 20cc/kg. mental status changes. palms) DIAGNOSIS        BMP. check aldosterone and renin. repeat if necessary If patient is hypoglycemic. abdominal pain. A level 18mcg/dl or lower is abnormal. cortisol If electrolyte abnormalities. ACTH. check 17-hydroxyprogesterone Interpret random cortisol levels in the context in which they were drawn. elbows.5-2 times maintenance Correct life-threatening electrolyte abnormalities (hyperkalemia) Other electrolyte abnormalities may correct with the institution of therapy Identify precipitating condition (i. with ambiguous genitalia).05-0. hyperkalemia. ACTH and cortisol should be drawn at baseline. the dose should be tapered back to maintenance and changed to po Stress mineralocorticoid replacement is generally not necessary if giving hydrocortisone Mild hyponatremia may exist as cortisol is required to excrete free water If patient is a ―salt loser‖ use Florinef 0. vomiting. glucose. This is 2-3 times the normal physiologic replacement dose Physiologic dosing: Hydrocortisone NaSuccinate (Solu-Cortef) 7-15mg/m2 BSA/day po Stress Dosing: Solu-Cortef load 25-50mg/m2 BSA IV bolus then 100mg/m2 BSA/day IV continuous infusion or divided q6hours See Quick calculations for BSA calculation When stable. 1mcg Cosyntropin is given and cortisol is checked at 10 minutes and repeated at 30 minutes. 250mcg is given and cortisol is checked in 1hour. and acidosis Physical exam: hyperpigmentation (fingernails. knees. hypoglycemia.e. 2cc/kg of D25 (centrally) or 5cc/kg D10 Fluids should be given at 1. To diagnose a primary adrenal source.96 Adrenal Crisis     Adrenal crisis: hypotension (may be postural).2mg/day (only available po) for mineralocorticoid replacement     . gums. infection) and treat appropriately Need to give stress dose of glucocorticoid (hydrocortisone) in cases of acute adrenal crisis. TREATMENT AND MANAGEMENT         ABCs If patient is hypotensive. dehydration Metabolic disturbance: hyponatremia. To diagnose a central source.

97 BIOEQUIVALENCY OF GLUCOCORTICOIDS      1mg of prednisone equivalent to 4mg hydrocortisone 1mg of prednisolone equivalent to 5mg hydrocortisone 1mg dexamethasone equivalent to 50-100 mg hydrocortisone Dexamethasone and Methylprednisolone have no mineralcorticoid effect Prednisone and prednisolone have less mineralcorticoid effect than hydrocortisone NOTES .

Aspirin.       . Maple Syrup Urine Disease) Sepsis or other hypermetabolic states Dumping syndrome (children with Gtube/Nissen) EVALUATION     Historical Facts: diet. Amino Acidopathy (e. insulinomas (rare) Adrenal (cortisol) or Growth Hormone Deficiency. Carbohydrate Metabolic Disorders (e. or seizure. Symptoms with prolonged fasting or illness.g. Seen in thin children age 18mo5years. small optic nerves.g. hypopituitarism)). social history (exposure to alcohol) Plot on growth curve: Short stature concerning for endocrine/IEM etiology Physical Exam: Organomegaly (IEM). and look for midline defects (i. new foods. medications. Symptoms may include altered mental status.e. Hereditary Fructose Intolerance).e. Iron. hyperpigmentation (primary adrenal). congenital hyperinsulinism (focal vs. Infants of diabetic mothers. MCAD deficiency). Male predominance. FH. May need to teach families to monitor for ketones during illness. Hypopituitarism Drugs: Alcohol. Prevention with frequent complex carbs and protein. oral hypoglycemics. Hyperinsulinism: Insulin overdose. central incisor (i. diffuse). recent steroid use (iatrogenic adrenal insufficiency).von Gierke Disease). Galactosemia. microphallus. It is important to collect ―critical sample‖ while the patient is hypoglycemic. nausea. unripe Ackee fruit Inborn errors of metabolism (IEM): Fatty Acid Oxidation Disorders (e. Organic acidurias. cleft lip/palate. Glycogen Storage Disorders (eg. tremor. Unknown etiology.g. ETIOLOGY  Ketotic Hypoglycemia: most common etiology. pallor.98 Hypoglycemia The definition of pathologic hypoglycemia is defined as serum glucose (not ―fingerstick‖) less than 40mg/dl. developmental delay.

pro-insulin.99 CRITICAL SAMPLE Serum Glucose ( confirmation of fingerstick) Red Top (3-5cc. Insulin and/or C-peptide Red Top (3-5cc) refrigerate for possible later use Urinalysis (ketones/reducing substances) Other lab tests: CMP. If >10-15mg glucose/kg/min required.8cc/kg/hr). or glucagon. workup should include: insulin. or Glycogen Storage Diseases Nonglucose reducing substances are seen with Galactosemia and Hereditary Fructose Intolerance Acidosis and elevated ammonia suggest IEM Insulin level over 2-3 micro-IU/ml or insulin to glucose ratio > 0. somatostatin. fatty acid oxidation defects. Ketones are not seen with hyperinsulinism. free/total carnitine profile (to Greenwood Genetics). plasma acylglycines. Ammonia need not be done at time of hypoglycemia If symptoms recurrent or suggestive of IEM. ideally during acute episode but can be done later. Consult Endocrine and consider starting oral diazoxide (3-8mg/kg/day given TID.25 suggests hyperinsulin state Low C-peptide with elevated insulin suggests exogenous administration TREATMENT     Bolus with 5cc/kg of D10 peripherally or 2cc/kg of D25 centrally Begin continuous infusion of D10 at a rate to provide 6-8mg of glucose/kg/min (3. Formula for Glucose Infusion Rate (see Quick Calculations). urine organic acids. NOTES . Further treatment is guided by suspected underlying pathology.6-4. 15mg/kg/day given TID for infants). and Cpeptide. If suspect insulin overdose. Growth Hormone. consider hyperinsulinemic state. iced) for Cortisol. workup should include: serum amino acids.

hirsuitism. Early rapid growth is abnormal. total T4. and DHEA-S  2nd tier includes MRI of brain with contrast and hypothalamic-pituitary cuts (1st tier if concerning history or physical findings) False Puberty (gonadotropin independent. Pulsatile secretion of LH and FSH (gonadotropins) heralds the onset of puberty leading to gonadal maturation and production of the sex steroids. There is controversial data suggesting that Caucasian girls in North America may have normal pubertal onset after age 7 and African-American girls after the age of 6. They are helpful when evaluating delayed or late puberty. They have a higher peak growth velocity and longer duration of growth (occurs at Tanner IV). menses. recalcitrant acne.adrenarche  Etiologies include: idiopathic. CNS lesions. thelarche is typically the first sign of true puberty. FSH. pink vaginal mucosa. this is most likely idiopathic while in boys it is most likely pathologic  Rapid progression through stages is abnormal  Complete neurologic exam needed (including visual fields)  Workup includes: Gender-specific sex steroid (sensitive estradiol in girls and total testosterone in boys). thickened. however. Clinically. male patterned (temporal) baldness. Biochemically. non-progressive CNS disorders (CP)  In girls. PRECOCIOUS PUBERTY Defined as Girls < 8 and Boys < 9 True Puberty (gonadotropin dependent)  Normal cascade of events with activation of HPG axis. Under age 2. Obtaining a bone age is essential for the workup of abnormal puberty. Menarche occurs at Tanner IV (2 years after thelarche). earlier onset does not negate the need for some evaluation.       . and consider LH. Peak linear growth rate in height occurs at Tanner II-III. It is considered abnormal if a difference of 2 standard deviations exists (typically >2years). This is a film of the left hand/wrist. Random LH and FSH may not be helpful in evaluation of precocious puberty. adrenal DHEA-sulfate (DHEA-S) may be measurable before gonadal steroids. It is important to follow the rate of growth. testicular enlargement is the first sign of true puberty. In Girls. deepening of the voice  Signs of estrogen: breast tissue. +/.  Signs of virilization: clitoromegaly. TSH. acne). maturation of the hypothalamic-pituitary-adrenal (HPA) axis parallels the activation of the hypothalamic-pituitary-gonadal (HPG) axis leading to adrenarche (pubic/axillary hair. and increased height velocity In Boys.100 Puberty  Normal ages of onset: Males 9-14 and Females 8-13. peripheral precocious puberty)  Does not proceed through normal order of pubertal events. a left hemi-skeleton should be requested.

and bone age Hypergonadotropic Hypogonadism (increased LH/FSH): Turner syndrome. Family history Physical Exam: Measurement of growth. McCune-Albright syndrome or severe hypothyroidism can do this as can. ―Delayed‖ puberty is when the signs of puberty occur after the above noted ages. or local neoplasm. Initial w/u includes 17-hydroxyprogesterone. Bilateral gonadal failure (traumatic. iatrogenic (post-surgical). and virlizing drugs/tumors (includes anabolic steroids. rapid progression. and TSH. Rule out foreign body. T4. Request 50-100 cells to exclude ―mosaic‖ Turner. advanced bone age. assess pubertal status (Tanner staging). DELAYED PUBERTY Defined as Girls > 13 and Boys >14 Absence of menarche by 16 ―Arrested‖ puberty when menarche occurs 5years after normal thelarche    Confusing nomenclature: ―late‖ puberty is normal puberty but along the latter end of the normal timing of adolescence. and a bone age.     . If labs abnormal proceed with pelvic ultrasound. and especially those with pubertal delay warrant a karyotype to rule this out. thyroid functions. galactosemia (poorly controlled esp. complete neurologic exam (includes visual fields. may need to assess olfactory sense or color perception (can be done in the Endocrinology Office)) Initial evaluation includes LH. infection. topical) source. or other signs of estrogen exposure.101   Etiology secondary to abnormal sex steroids from gonads/adrenal gland or exogenous (ingested. TSH. βHCG secreting tumors (in boys). in girls). autoimmune. total T4. DHEA-S. No evidence of growth acceleration. Premature menarche: Rare presentation of precocity. vanishing testes syndrome)  Turner Syndrome: Most common cause of ovarian failure in females. Adrenarche typically normal. sensitive estradiol. postinfectious (mumps). total testosterone. Benign elevation of adrenal androgens (DHEA-S). abuse/trauma. stigmata of Turner Syndrome or other genetic disorders. FSH. Premature Thelarche: Appearance of breasts prior to age 8. No growth acceleration. colored-blindness. pelvic ultrasound and bone age. Premature Pubarche (only pubic hair) and/or Adrenarche (pubic and axillary hair): Etiologies include benign premature adrenarche. signs of hypothyroid. If present consider bone age. simple virilizing/nonclassic congenital adrenal hyperplasia. ovarian disease (dominant cyst or malignancy) must be excluded. If no other evidence of sexual maturation exists. creams/lotions). All significantly short females.  Premature Adrenarche: Most common cause of early pubarche. anosmia/hyposmia. 10% evolve to true precocious puberty. rapid progression. or virilization.  Actually not an uncommon finding in girls ages 18-24 months. menses rare. Historical facts: chronic illness. May have minimal breast development. Klinefelter syndrome. Evaluation includes sensitive estradiol. rarely.

102  Normal to low Gonadotropins: Constitutional Delay, Hypothalamic dysfunction, Chronic Illness, Hypopituitarism, Hypothyroid, Hyperprolactinemia, Kallman syndrome (isolated Gonadotropin deficiency)  Constitutional Delay: Pubertal increase in gonadotropins is slow to develop. More common in boys. Usually + family history. Evaluation normal except delayed bone age consistent with pubertal stage and height age. Amenorrhea with normal pubertal development: Rule out pregnancy and Rokitansky syndrome. Consider thyroid functions, prolactin, pelvic ultrasound. Gyn referral to assess anatomy. Polycystic Ovarian Syndrome/Hirsutism: Associated with obesity, glucose intolerance, and anovulatory cycles. Differential diagnosis includes late-onset CAH. Work up includes free testosterone, 17-hydroxyprogesterone, and fasting insulin with glucose, T4 (free T4 may be superior here). Treatment may include Metformin and/or OCPs. NOTES

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ARF occasionally presents with high urine output (high output failure). renal vs. Oliguria is defined as urine output less than 0. postrenal). Urinary Indices to Classify ARF Urine OSM Urine NA FENa% >500 <20 <1% >350 <2. One such parameter is the fractional excretion of sodium (Una/Pna)(Pcr/Ucr) x 100.5 ml/kg/hr in infants or less than 500cc/m2 per day in older children.5% >350 >60 ETIOLOGY Pre-Renal Disease Hypoperfusion of the kidneys is secondary to a decreased effective circulating blood volume.5%(Newborn) (newborn) <350 >40 >2.106 Acute Renal Failure When the diagnosis of ARF is entertained. There are several laboratory parameters that will help in identifying the location of pathology (prerenal vs. It can occur in these clinical settings: Hypovolemia Hyper/normovolemia Dehydration Congestive heart failure Hemorrhage Hepatorenal syndrome DI Cardiac tamponade ↑ Insensible losses (burns) 3rd space losses (sepsis. The FENa is typically less than 1% in prerenal failure and over 2% in renal failure. one must rule out severe dehydration. NS) Lab findings in Pre-Renal Disease  ↑ BUN>>Cr  Uosm > 400-500  Una < 10-20  FENa < 1% Intrinsic Renal Disease  Acute Tubular Necrosis (ATN)  Ischemic/hypoxic injury Classification Prerenal Renal Postrenal . cap leak. Oliguria/anuria and azotemia are often seen in the setting of ARF. Anuria is defined as total cessation of urinary output.

IgA Nephropathy.107  Evolution of pre-renal failure as vasoconstriction leads to tubular injury and necrosis  Labs: ↑ BUN. granular casts Nephrotoxic ARF  Drugs: aminoglycosides. heavy metals  Labs: large anion gap metabolic acidosis. Una > 30-40. acyclovir. ↑ CPK. renal vein  Cortical necrosis  Labs: hemolytic anemia. cimetidine  Post-infectious  Idiopathic  Labs: U/A: eosinophils. WBCs. HSP. methanol. ANCA. Low C3. RBC and/or WBC casts. U/A: hematuria. Type IV RTA        . thrombocytopenia. MPGN. hypocalcemia. IV contrast. amphotericin B. +ANA. proteinuria. hypocalcemia Acute Interstitial nephritis  Drugs: penicillins. SLE. lasix. WBC casts Eosinophilia Acute glomerulonephritis  Post infectious. hyperuricemia. NSAIDS. NSAIDS. FENa> 2 U/A: protein. heme + urine Nephropathy of malignancy  ALL  B-cell lymphoma  Labs: hyperkalemia. anti-GBM Ab Vascular Disease  HUS  Thrombosis. rifampin. hyperkalemia. hyperphosphatemia. sulfonamides. hematuria/proteinuria Post-Renal (Obstruction)    Bladder outlet obstruction (PUV) Ureteropelvic junction obstruction (UPJ) Ureterovesicular junction obstruction (UVJ)  Labs: hydronephrosis.renal artery. Wegener’s granulomatosis. Goodpasture’s disease  Labs: gross hematuria. Chemotherapy  Labs: same as ATN Exogenous toxins  Ethylene glycol. C4. Ca oxalate crystals Endogenous toxins  Hemoglobinuria  Myoglobinuria  Labs: metabolic acidosis. Uosm < 350.

Treatment of choice for toxin removal. Monitor strict I’s and O’s. C4. For the oliguric euvolemic patient. urine myoglobin. C3. difficult if hemodynamically unstable. Initial fluid administration of isotonic saline (10-20 cc/kg/dose) should be used to restore intravascular volume. hyperphosphatemia. hypocalcemia. Hypervolemia Therapy for prerenal failure involves volume replacement and treatment of the underlying condition that resulted in prerenal failure. Cause of ARF.108 EVALUATION         Urinalysis and culture Urine osmolality. Watch for polyuric recovery phase Initiate therapy for complications such as hyponatremia. metabolic acidosis. Euvolemia vs. Ultrafiltration is driven by osmotic pressure gradient generated by dextrose. Advantages include rapid correction of metabolic disturbances. skin turgor. Advantages include relatively easy to     . ANA MANAGEMENT    Remove any offending agent Fluid balance: Hypovolemia vs. vital signs. Peritoneal Dialysis: solute and water exchange from peritoneal capillaries into dialysate instilled into the peritoneal cavity. and sodium CBC CMP Magnesium and phosphorous Renal ultrasound or other imaging study Renal biopsy in certain cases Other tests as indicated: CPK. Disadvantages include large venous access required. start fluids to replace insensible loss (≈ 1/3 maintenance) + urinary losses cc for cc. capillary refill. and hypertension should they arise RENAL REPLACEMENT THERAPY Indications     Child’s age. Rapidity of onset Electrolyte abnormalities Nutritional issues Fluid overload Modalities  Hemodialysis: aggressive solute and water removal over limited period of time by passing blood through artificial filter with countercurrent dialysate flow. hyperkalemia. ASO. creatinine. weights (BID or more often if needed).

CRRT: continuous more gentle removal of solute and water by passing blood through artificial filter +/. safe for hemodynamically unstable patients.countercurrent dialysate flow.109 perform. less control. risk of peritonitis. vascular access not required. does not require hemodynamic stability. Disadvantage requires sedated patient and vascular access. NOTES  . Advantages include tighter control. Disadvantages include slower correction of metabolic disturbances.

or hypertonic). Serum sodium is necessary in order to determine the type of dehydration (hypotonic. TBW equals  Birth: wt(kg) x 80%  6 mo: wt(kg) x 75%  1-15 yr: wt(kg) x 65%  Adult: wt(kg) x 50-60% MAINTENANCE REQUIREMENTS  Surface Area  Water: 1500-1800 ml/m²/day  NA+: 30-60 mEq/m²/day  K+: 20-40 mEq/m²/day Weight  Water: 0-10 kg = 100 ml/kg/day 11-20 kg = 1000ml + 50 ml/kg over 10 kg >20 kg = 1500 ml + 20 ml/kg over 20 kg  Na+: 3-4 mEq/kg/day  K+: 1-2 mEq/kg/day ASSESSMENT OF DEHYDRATION The degree of dehydration can be determined clinically from history and exam. Sign and Symptoms ≤ 5% Decreased fluid intake ↓ Postural pulse change No change Postural DBP change No change Fontanel/skin turgor Mucous membranes Tears Urine output Urine SG BUN Urine Na/FENa Hct/Albumin Normal Normal Present Normal/slight decrease Normal Normal Normal Normal 10% ↓↓ ↑ ≥ 10 beats/min ↓ ≥ 10 mmHg ↓ Dry Reduced Oliguria ↑ ↑ ↓ ↑ 15% ↓↓↓↓ ↑↑↑↑ ↓↓↓ or frank hypotension ↓↓ Very dry Severe oliguria or anuria ↑↑ ↑↑ ↓↓↑ ↑↑  . isotonic. TBW is comprised of the intracellular (2/3) and extracellular (1/3) compartments.110 Fluids and Electrolytes GENERAL PRINCIPLES   Total body water as a % of body water decreases with age.

7 kg .111 HYPOTONIC AND ISOTONIC DEHYDRATION For hypotonic (sodium less than 130) or isotonic (sodium 130-150) dehydration.0 give none initially If patient is acidotic  K < 4. admission sodium is 125. Potassium Replacement If not acidotic (HCO3 > 18):  K < 3. rehydrate over 24 hours by giving 50% over the first 8 hours.  Maintenance water: (1500)(0.7 kg  Water deficit: 8.0-5.6 x Wt (kg) Convert total sodium over total water to amount/liter to write fluid orders. Calculate fluid and electrolyte replacement as follows:          Water Replacement Maintenance + Deficit = Total water needed. Corrected Sodium: (135-actual Na) x 0.4m²) = 16 mEq  Corrected sodium = (135-125)(0. then the remainder over 16 hours. Wt1 = present wt in Kg. 140 mEq/L x water loss in liters equals deficit sodium in mEq.6)(8kg) = 48 mEq  Deficit sodium = (140 mEq) (0. Therefore. Wt2 = calculated rehydrated weight Deficit: Pt’s Wt2 = Wt2/(100 . Maintenance Sodium: 3-4 mEq/kg/day or 30-60mEq/m2/day Deficit Sodium: Assume water loss is isotonic.4 m² is clinically 8% dehydrated.0-6.0 kg = 0.0 give 20 mEq/L  K > 5.4) = 600cc  Rehydrated weight: 8/100-8% = 8.% dehydration) Rehydrated weight (Wt2) – present weight (Wt1) = deficit water (1gm=1ml) Sodium Replacement Maintenance Sodium + Deficit Sodium + Corrected Sodium (hypotonic only) = total sodium needed in mEq for the next 24 hours.0 give 40 mEq/L  K 4.75 NS 1300cc 1000cc .0 give none initially Example: 8 kg child who at 0.0 give 20 mEq/L  K > 6.8.7 or 700cc  Total water needed = 600cc + 700cc= 1300cc  Maintenance sodium = (40mEq)(0.7) = 98 mEq  Total sodium = 16 + 48 + 98 = 162 mEq  Fluid concentration = 162mEq = 124 meEq = approximately 0.5 give 40 mEq/L  K 3.0 give 30 mEq/L  K 5.5-5.

12cc of 3% saline per kg should raise the patient’s serum sodium by 10 HYPOMAGNESEMIA    For serum magnesium level less than 1 mg/dl (<0.6 x Wt(kg) = mEq of NaHCO3 needed. then 40 cc/hr x 16 hours HYPERTONIC DEHYDRATION Hypertonic dehydration is corrected slowly over 48 hours with D5 0. a mEq of NA is given and this should be considered when calculating sodium needs.112 Order written as follows: D5 0. a repeat level should be obtained by venous stick. there is a risk of cerebral edema. Thrombocytosis will also falsely elevate the potassium level.75 NS at 81 cc/hr x 8 hours. ECG findings. If it is felt that the potassium level is falsely elevated. and urine output. replacing potassium by the PO route is preferred. acid/base status. treat with magnesium sulfate(50% solution) Typical dose is 50 mg/kg up to max of 2 grams per dose Should be given IV slowly INTERPRETING AND CORRECTING POTASSIUM LEVELS Serum potassium levels should be interpreted in the context of the patient’s clinical status. Monitor serum sodium closely. Administer the volume necessary in cc’s to correct the sodium over 10-15 minutes  In an emergent situation.2 to D5 0. Remember that for every mEq HCO3. Administer over 8 hours. .6) = number of mEq of sodium needed  3 % saline contains 0. Symptomatic hyponatremia should be corrected with 3% saline. The patient should have continuous ECG monitoring during replacement.5 mEq/kg/hour for symptomatic hypokalemia.513 mEq Na/mL.4mmol/l). METABOLIC ACIDOSIS If initial HCO3 < 12 then replace as follows: (12 – serum bicarb) x 0. Samples obtained by heel stick often are hemolyzed which will falsely elevate the potassium level. Hypokalemia Treat with 1 mEq/kg/dose of KCL given at rate of 0. If pt is asymptomatic. If re-hydrated too quickly.  (120 – serum NA) x (weight in kg) x (0.45 NS. SYMPTOMATIC HYPONATREMIA Severe hyponatremia can lead to mental status changes and seizures.

may give glucose 1-2 grams/kg and regular insulin 0.  If necessary.  Then if necessary.5 mg/dl (0. hypocalcemia should be corrected with oral supplements. Infuse over 2 hours. In an asymptomatic patient. Use IV calcium cautiously due to possibility of extravasation and subsequent necrosis of surrounding tissue.113 Hyperkalemia The emergent treatment of hyperkalemia is designed to stabilize the cardiac membrane and drive potassium into the cells.  Calcium Chloride (10%) 20mg/kg to max of 1 gram per dose. May be repeated twice.  Calcium Gluconate (10%) 50 mg/kg to max of 2 grams per dose  Exclude hypomagnesemia NOTES . Depending on the degree of ECG changes present the treatment varies. The patient should have cardio-respiratory monitoring during administration. such as albuterol aerosols.0 mg/dl or an ionized calcium < 3. give 10% calcium chloride 10-20 mg/kg over 5 minutes. Kayexalate 1gm/kg PO in 70% sorbitol or PR in 30% sorbitol.  Beta agonists. If the patient is symptomatic or hemodynamically compromised.  1-2 mEq/kg NaHCO3 over 20 minutes for T wave changes  For widened QRS complex or rhythm changes.87 mmol/l). Give slowly. the calcium should be corrected IV preferably through a central line. also drive the potassium intracellularly HYPOCALCEMIA Hypocalcemia is defined as a total serum calcium of < 7.3 units/gram of glucose.

PATHOGENESIS      There are 2 sources of Vitamin D. PRIMARY PRESENTATION        Incidental finding on radiograph or physical exam.25-dihydroxyvitamin D As 25-hydroxyvitamin D levels decrease. scoliosis Failure to thrive/short stature Nonspecific musculoskeletal complaints Delayed development Seizures Tetany Weakness RADIOGRAPHIC FINDINGS IN VITAMIN D DEFICIENCRY RICKETS    Widening of metaphysis Cup shaped metaphysis Osteopenia .25-dihydroxyvitamin D levels. Thus. produced endogenously in the skin following exposure to ultraviolet radiation Vitamin D is hydroxylated in the liver to 25-hydroxyvitamin D and subsequently in the kidney to 1. have a minimum intake of 200 IU of vitamin D per day beginning during the first two months of life.craniotabes. Vitamin D deficiency is more common among breastfed than formula fed infants. coxa vara. Decreased sunlight exposure during the winter months also increases the risk for developing rickets. PTH increases the activity of 1-alpha hydroxylase enzyme in the kidney to increase 1. if an infant is ingesting at least 500cc (approximately 16 oz) per day of formula.vitamin D2.114 Rickets Vitamin deficiency rickets remains a significant cause of nutritional disease in infants. In addition. PREVENTION OF RICKETS AND VITAMIN D DEFICIENCY  It is recommended that all infants. African American infants are at increased risk because of decreased production of vitamin D in the skin. enlargement at the wrist and ankles or costochondral junctions. The end result is increased mobilization of calcium from the bone matrix leading to the development of rickets. he or she will receive the recommended vitamin D intake. obtained exogenously from the diet Cholecalciferol.vitamin D3.cholecalciferol and ergocalciferol Ergocalciferol. including those who are exclusively breastfed. All infant formulas are supplemented with Vitamin D to provide 400 IU/L.

**= decrease or normal or slight increase (relatively low for degree of hypocalcemia). *= decrease or normal or increase. PTH= parathyroid hormone. Mg= magnesium.25-hydroxyvitamin D Long bone radiographs CAUSES OF HYPOCALCEMIA AND THEIR BIOCHEMICAL PROFILE CLUES Serum Causes of Hypocalcemia Vitamin D-deficient Rickets Vitamin D-dependent Rickets: type 1 Vitamin D-dependent rickets type II Ca ↓↔ P ↔↓ PTH ↑ Alk Phos ↑ 250HD3 ↓ 1. ?= difficult to interpret owing to different degree of renal immaturity in preterm infants.115   Rib flaring. ↔= normal.―rachitic rosary‖ Multiple fractures in various stages of healing LABORATORY EVALUATION       CMP Mg and Phos PTH 25-hydroxyvitamin D 1. 25(OH)2 * Mg ↔ Urine Ca ↓ ↓ ↓ ↓↔ ↓↔ ↓ ↑ ↑ ↔↓ ↑ ↑↔ ↔ ↑ ↑ ↔ ↓ ↑ ↑ ↑ ↑ ↑ ↑ ↔ ↔ ↑ ↑ ↔ ↔ ↔ ↔ ↔ ↔ ↔ ↔ ↔ ↔ ↔ ↓ ↑ ↔↓ ↔↓ ↔↓ ↔↑ ↓ ↔↓ ↔ ↓ ↔ ↔ ↔ ↔ ↔ ↔ ↔ ↑ ↔ ↓ ↓ ↓ ↓ ↓ ? ↓ Vitamin D Resistant rickets ↔ (hypophosphatemic rickets) Hypoparathyroidsim ↓ Pseudohypoparathyroidism ↓ Rickets of prematurity Renal osteodystrophy Calcium-sensing receptor defect Mg deficiency ↔↓ ↓ ↓ ↓ ** ** ↑ Ca= calcium. ↓= decrease. P=phosphorus. alk phos= alkaline phosphatase. ↑= increase. .

116 TREATMENT AND MANAGEMENT  Vitamin D supplementation may be initiated once laboratory and radiologic tests confirm the diagnosis. Continue supplements until serum alkaline phosphatase levels and skeletal deformities return to normal. Most experts recommend administration of oral vitamin D (ergocalciferol.2000-5000 IU/day). NOTES .

5)  Variable potassium abnormalities.Hypokalemia. or s/p GI surgery Increased anion gap  MUDPILES CLINICAL MANIFESTATIONS       FTT Polyuria Constipation Vomiting Nephrocalcinosis/nephrolithiasis Rickets TYPE I DISTAL RTA Distal tubule is impaired and cannot excrete acid due to defect in H+ secretion.diminished Na+ reabsorption causes volume contraction and secondary hyperaldosteronism  Hypercalciuria  Hypocitriuria  Nephrocalcinosis/nephrolithiasis  Bone dissolution  Sensorineural deafness MAJOR CAUSES OF TYPE I DISTAL RTA   Primary  Idiopathic/sporadic Familial .117 Renal Tubular Acidosis Renal Tubular Acidosis is a clinical syndrome characterized by impaired renal acidification due to either impaired reabsorption of bicarbonate or impaired excretion of hydrogen ions. Common features and associated findings include:  Nonanion gap hyperchloremic metabolic acidosis  Urine pH inappropriately high ( > 5. This results in a hyperchloremic metabolic acidosis with a normal anion gap. fistula. normal 12 mEq/l +/.2)  RTA  GI loss of bicarbonate from diarrhea. METABOLIC ACIDOSIS   Normal anion gap (AG = Na – (Cl+CO2).

due to secondary hyperaldosterism.Amphotericin B.more common and severe than in Distal RTA  Loss of glucose. Sjogren’s. Hyperparathyroid. Type IV is the most common form of RTA. Sickle cell  Autoimmune-SLE. and uric acid in the urine secondary to Fanconi’s syndrome  Rickets  Urine pH may be either high or low depending on serum bicarbonate level MAJOR CAUSES OF TYPE II PROXIMAL RTA WITH/WITHOUT FANCONI SYNDROME   Primary  Idiopathic/sporadic Familial  Cystinosis  Tyrosinemia  Galactosemia  Hereditary fructose intolerance  Glycogen storage disease  Wilson’s disease  Lowe’s syndrome Acquired  Malignancy  Multiple myeloma  Renal transplant  Heavy metals-lead.Ehlers-Danlos.118  Autosomal dominant  Autosomal recessive Secondary  Genetic diseases. Features include:  Hyperkalemia   . transplant rejection  Drugs. bicarbonate. Lithium. chronic pyelonephritis. Features include:  Hypokalemia. Thyroiditis. amino acids. phosphate. mercury. Marfan. Associated with decreased renal bicarbonate threshold.Idiopathic hypercalciuria. Fabry’s. copper  Carbonic anhydrase inhibitors TYPE IV RTA Due to either aldosterone deficiency or aldosterone resistance (obstructive uropathy). Wilson’s. Ifosfamide TYPE II PROXIMAL RTA Characterized by impaired capacity of the proximal tubule to reabsorb bicarbonate. Rheumatoid arthritis  Nephrocalcinosis. Vit D intoxication  Obstructive uropathy.

K Citrate supplementation as needed.5 Conditions associated with Type IV RTA  Hypoaldosteronism  DM. Addison’s.replacement bicarbonate usually requires more alkali therapy than Type I.Replacement bicarbonate (average dose 3.5 mEq/kg/day). . Phosphate supplements if rickets present. NSAIDs.5-2 mEq/kg/day. Urine culture Urine Ca/Cr ratio Renal ultrasound Serum uric acid Urine AA Tubular reabsorption of phosphate Fractional excretion of bicarbonate Bone films Aldosterone/renin levels (if suspect Type IV) TREATMENT OF RTA    Type I. Amyloidosis  Aldosterone-resistance states  Obstructive uropathy  Tubulointerstitial diseases LABORATORY EVALUATION Tier I       Tier II       Urine electrolytes (Urine Na + K.dose can be as high as 14 mEq/kg/day. SLE. interstitial nephritis.bicarbonate replacement.1. Type IV. Surgical repair of obstruction if present. Type II. Nephrosclerosis. K+ supplementation. K+ restriction. Determine urinary anion gap BMP (venous) Phosphorous U/A.Cl).119  Urine pH usually < 5.

5 High Positive Obstructive uropathy NOTES . <10 mEq Greater than 5.waste bicarb.glu/phos/aa/uric acid Usually 12-20 mEq Variable Low/normal Negative Rickets Fanconi Type IV Decreased aldosterone secretion or resistance Defect Serum Bicarbonate Urine pH Plasma K+ Urine anion gap Comments Variable.120 OVERVIEW OF RTA Type I Impaired distal acidification Type II Impaired proximal bicarb reabsorption Fanconi.5 Low or high Positive Nephrocalcinosis Hypercalciuria Mildly acidotic Usually < 5.

easy bruising History of HSP Trauma Chronic illnesses. Strep. abdominal mass Presence of arthralgias.strenuous exercise.microscopic. edema.121 Hematuria DEFINITION Hematuria is defined as 5 or more red blood cells per high power field on a spun urine sample on at least 2 weekly urines (Dipstick only helpful if negative). or intermittent gross with persistent micro Non-Isolated/Symptomatic. gross. Factitious Hematuria: A positive dipstick without the presence of RBCs on microscopic exam may suggest hemoglobinuria secondary to hemolysis or myoglobinuria secondary to rhabdomyolysis. May be microscopic or gross. presence of gross hematuria. symptoms. Check urine myoglobin. PREVALANCE 4-6% of asymptomatic school age children on a single specimen. CLASSIFICATION    Functional. rash. Impetigo. Most likely a benign process when microscopic hematuria only.URI. umbilical vessel catherization Preceding infection. frequency. or other urine abnormalities.microscopic.dysuria. fever. physical exam findings (nl growth and BP). There is also a high false positive rate with the urine dipstick for heme so keep that in mind with the well asymptomatic child. gross. dehydration Isolated/Asymptomatic. PERTINENT HISTORICAL FACTS         Birth history: asphyxia. Diarrhea Urinary symptoms. medications FAMILY HISTORY   Hematuria Hearing loss . intermittent gross with persistent micro Isolated Hematuria. blood clots Abdominal pain or Flank pain.lack of associated history.

masses. petechiae. Phase II (Add to phase 1 if gross hematuria present) . If HTN. pleural rub. follow BP and U/A yearly.122         Renal failure Stones Cystic kidney disease Recurrent UTI Hypertension Lupus Sickle cell disease Bleeding disorders PHYSCIAL EXAM        Growth parameters (short stature or failure to thrive) Blood pressure Fundoscopic exam Signs of fluid overload.rashes. ascites. rales. or decreased renal function.edema. then proceed to phase II workup. proteinuria. Family hx  U/A with micro. gallop Presence of abdominal bruits Abdominal exam. Urine culture (if symptomatic)  Spot urine Ca:Cr ratio  UA of first degree relatives  Sickle screen if indicated  Hearing screen  If ALL above is normal. HSM.MCNS Nonglomerular renal Hypercalciuria Pyelonephritis Sickle cell disease Polycystic kidney Reflux nephropathy Renal tumor Renal Vein thrombosis Renovascular HTN Nonrenal Stones Cystitis/prostatitis Foreign body Bladder tumors AV malformation Hydronephrosis HUS UPJ LABORATORY EVALUATION Phase I  Complete H&P.FSGS. purpura ETIOLOGIES OF HEMATURIA Glomerular Benign familial Exercise induced Post-infectious GN IgA nephropathy HSP Alport syndrome SLE Membranoproliferative GN. pain Skin.

6 (7 to 18 mos)  0. duration for > 6 -12 months. C4. IDIOPATHIC HYPERCALCIURIA Ca to Cr ratio:  >0. C3. BMP. gross hematuria.8 (under 7 mos) PROGNOSIS    Isolated/asymptomatic microscopic hematuria.4 (19 mos to 6 yrs)  0.123  CBC. ANA. hypertension. persistently low complement levels. and as suggested by phase II workup.variable and often guarded prognosis NOTES . dsDNA  Renal biopsy Indications for phase II workup include non-isolated hematuria.excellent prognosis with very low incidence of pathology Asymptomatic gross hematuria.very good prognosis but identifiable causes more likely Non-isolated hematuria. ASO or streptozyme. significant family history. Indications for renal biopsy include persistent renal insufficiency. coexistent gross hematuria. Hep B & C  Renal ultrasound  VCUG if indicated by RUS Phase III  Further imaging as indicated  Further serology: ANCA. proteinuria.2 ( 7 yrs and older)  >0.

NOTES Dosing varies with phosphorous level.5-1 gram/kg/dose. Calcium Carbonate 1-2 tablets PO with meals OTHERS Renagel Phos level Dose 6-7. When hematocrit ≥ 36% then decrease epogen dose by 25% A test dose of ¼ of total dose should be given prior to starting therapy.124 Nephrology Cocktails CALCIUM AND VITAMIN D Product Calcitriol Calcium Acetate Dose 0.5 mg/dl 2 caps TID 7. Erythropoietin Iron dextran Albumin/Lasix .5-9 mg/dl 3 caps TID >9 mg/dl 4 caps TID 50-100 units/kg three times a week <10 kg = 25 mg 10-20 kg = 50 mg >20 kg = 100 mg Lasix up to 1mg/kg/hr. Albumin (5%) . Used for treating fluid overload and occasionally nephrotic syndrome.25-2 mcg/day PO or IV 1-2 tablets PO with meals Comments Vitamin D analog. Give with meals to bind dietary phosphorous.0. Goal phosphorous level < 6. but usually is 1-2 capsules TID with meals. Used to treat renal osteodystrophy Goal phosphorous level < 6. Max dose 6 grams/kg/day. Give with meals to bind dietary phosphorous.

False positives can be obtained on the urine dipstick by concentrated or alkaline urine or with certain antiseptics.125 Proteinuria DEFINITION Proteinuria is a fairly common finding in the pediatric patient and may be benign. The first step in evaluation is determining whether the proteinuria is significant or a laboratory phenomenon. renal insufficiency. False negatives can be obtained if the urine is very dilute.25 for age > 2yrs > 100 mg/m2/day CLASSIFICATION AND CAUSES OF PROTEINURIA Functional Proteinuria  Fever  Pregnancy  Seizures  CHF  Emotional stress  Exercise Isolated Proteinuria.5 for age 6 mos to 2yrs > 0. HTN. . Negative history.015 > 0.015 or 2+ if urine SG > 1. MEASUREMENT OF PROTEINURIA           Dipstick Random urine protein:creatinine ratio 24 hour urine collection Fractionated 24 hour urine collection (recumbent/upright) Serum albumin DIPSTICKS Trace (10-20mg/dl) +1 (30 mg/dl) +2 (100 mg/dl) +3 (300 mg/dl) +4 (1000 mg/dl) DETERMINING IF PROTEINURIA IS SIGNIFICANT    Dipstick Pro/Cr 24 hr urine ≥ 1+ if urine SG ≤ 1. or growth failure.lack of associated signs or symptoms such as casts hematuria.

 Renal biopsy ORTHOSTATIC PROTEINURIA      Benign condition in which the patient spills protein when in the erect position. the first morning void is flushed and then all other subsequent voids during the day are collected in the AM jug. IgA nephropathy. Pathologic Proteinuria  Glomerular. Membranoproliferative. follow with yearly first AM urine for U/A and Pro/Cr ratio Phase II  CMP  CBC  Lipid profile  C3.VCUG if indicated by the RUS or prior UTI Phase III  Further serology. reflux nephropathy. Orthostatic.label 2 jugs. cystic disease. Urine culture  24 hour split urine collection (recumbent/upright). On day 1 of collection. etc. guarded prognosis. fixed. Fanconi syndrome LABORATORY EVALUATION Phase I  Complete history and physical  First AM U/A with micro. Postinfectious.FSGS. ANA. SLE. Hepatitis B and C  AM and one PM.ANCA. all voids are collected in the PM jug including the first morning void of Day 2.proteinuria in 80% of specimens. pyelo. HSP. ASO or streptozyme. Sickle Cell  Tubulointerstitial – obstructive uropathy.  AM and PM random Pro/Cr ratio (if not potty trained)  BMP If above consistent with orthostatic proteinuria. benign clinical course.60% of children with proteinuria. Membranous.C4. MCNS. toxic or ischemic injury. +/. transient vs. fixed. but not when recumbent 50-60% of otherwise healthy kids with proteinuria (primarily adolescents) Usually < 1000 mg total protein in 24 hrs Transient vs. During the night. Persistent asymptomatic.126 Transient asymptomatic-no long term sequelae. interstitial nephritis. ds-DNA.both have benign clinical course Follow yearly with first AM urine for U/A and protein:Creatinine ratio    . should be < 1 gm/24hr.

2 or more relapses in 6 months Steroid dependent. and hyperaggregable platelets Edema.need Biopsy first COMPLICATIONS    Infection. Antibiotics are given if patient is febrile or there is suspicion for infectionpatients at risk for serious pneumococcal infections such as peritonitis. however. consider nephritis as the underlying kidney pathology. HTN. when renal biopsy should be considered in patients with nephrotic syndrome:  Children less than 1 year of age  Atypical least 2 consecutive relapses on therapy or within 14 days of treatment Steroid resistant. loss of immunoglobulins and opsonins.increased fibrinogen. May repeat every 8 hours. Do not restrict fluids  Cytoxan for frequent relapser or steroid dependent.Prednisone works(on avg 2-3 weeks. hypoalbuminemia. This is a clinical diagnosis and does not require a renal biopsy prior to initiation of treatment. renal insufficiency  Non-responsive to steroids after 8 week course  Frequently relapsing or steroid dependent  If you are considering cytoxan or cyclosporine treatment CLINICAL COURSE OF MINIMAL CHANGE NS      Steroid responsive. hematuria. After initial work up. decreased antithrombin III. If nephrotic syndrome is associated with hypertension. Nephrosis is a constellation of findings. these patients are treated with steroids and tend to improve quickly.leading cause of mortality.25% salt poor albumin (0. MINIMAL CHANGE DISEASE The most common cause of nephrotic syndrome in pediatrics is minimal change disease.lack of response after 8 weeks Treatment includes:  Prednisone 1mg/kg/dose (max 40mg) BID. There are certain incidences. can be given in cases of severe edema. allow up to 8 weeks) Frequent relapser. The underlying disease causing the nephrosis should be determined. NOTES . or red cell casts. Thrombosis.5-1 gm/kg over 2-3 hours) followed by lasix (1mg/kg).127 NEPHROTIC SYNDROME Nephrotic syndrome is defined by massive proteinuria.nephritis. and edema and is often associated with hypercholesterolemia and hypertriglyceridemia. and not a diagnosis.Initial course 12 weeks (6 weeks daily and 6 weeks QOD with taper)  Low sodium (2000-2500mg/day).

128 NOTES .

PREVALENCE    Most common prenatally detected congenital anomaly. require a repeat study between 2 weeks to 3 months of age. urine culture  BMP  VCUG  If the VCUG is positive then start prophylactic antibiotics.Amoxicillin10 mg/kg/day  If the VCUG is negative and pt with moderate to severe hydronephrosis. In general.up to 1% live births Majority of cases of antenatal hydronephrosis are not clinically significant Hydronephrosis occurs twice as often in males than females WORKUP  Infants with hydronephrosis identified antenatally should have an ultrasound in the postnatal period. infants should have a postnatal renal ultrasound in the first 2-7 days of life depending on the severity of prenatal findings.  Infants who had persistent antenatal hydronephrosis and then a normal ultrasound in the first few days of life. start antibiotic prophylaxis and proceed with Mag 3 lasix renogram after 6 weeks of age to detect possible obstruction CAUSES    Transient/Physiologic Vesicoureteral reflux UPJ obstruction  UPJ obstruction(Ureteropelvic junction)  UVJ (Ureterovesicular junction). Infants with persistent hydronephrosis documented postnatally should have the following workup:  Cath UA. Postnatal ultrasound should be avoided in the first two days of life due to the fact that hydronephrosis may not be detected because of physiologic volume depletion and relative oliguria.if ureter dilated  . Fetuses with severe bilateral hydroureteronephrosis prenatally from bladder outlet obstruction can be associated with severe impaired renal function and require urgent evaluation on the first postnatal day. sometimes referred to as pelviectasis or caliectasis if the renal calices are dilated.129 Hydronephrosis DEFINITION Distention of the intrarenal collecting system.

if bilateral with dilated ureters and thickened bladder NOTES .130  PUV (Posterior urethral valves).

131 .

132 Infectious Disease .

133 .

Obtain serum glucose before LP CRP If Clinically Indicated CXR PT/PTT. etc) PHYSICAL EXAM  A complete physical exam should be performed paying attention to the child’s appearance and activity (lethargic. Sickle cell. purpura). consolable). grunting. After 1 year of age. . Laboratory Data Routine Blood and urine cultures CBC with diff BMP to monitor for SIADH. and then q8-12hours initially. including headache. cyanosis. FSP ABG Aspirates of purpuric lesions. abscesses. signs of meningeal irritation may occur. especially pupillary signs or cardiovascular instability. irritable. and joints. or positive Kernig’s and Brudzinski’s sign. fibrinogen. effusions Throat Culture and sensitivities Stool culture Head imaging should be considered prior to lumbar puncture when focal neurologic signs are present. or a high pitched cry. skin (petechiae.134 Meningitis CLINICAL MANIFESTATIONS    Infants have nonspecific findings: fever. stiff neck. Seizures and a bulging fontanel may occur and are highly suggestive of meningitis. irritability. perfusion. Determine whether the patient has any underlying chronic medical conditions (prematurity. occasional photophobia. HIV. trauma. joints. poor feeding. lethargy. vital signs.

. or if there has been no improvement. CSF findings may resemble viral meningitis. Coli H.000 PMN <40 or <½ serum glucose Elevated Viral 10-3. Salmonella sp. pneumoniae Neisseria meningitides E. if a resistant pneumococcus is isolated.135 BACTERIAL CAUSES OF MENINGITIS Age Group Newborn                Pathogens Group B Streptococcus E. Coli Listeria monocytogenes Klebsiella pneumonia Enterococcus sp. PMNs predominate initially. Group B Streptococcus Listeria monocytogenes Strep. A repeat lumbar puncture should be done 24-48hours after admission in most cases of neonatal meningitis. then lymphocytes appear. Bactogens may be helpful in these cases ²Mean WBCs in a term neonate is 8 (range of 0-22) ³ In viral meningitis. pneumoniae N. influenzae 4-12 weeks Over 12 weeks CEREBROSPINAL FLUID ANALYSIS Indices Cell Count Cell Type Glucose (mg/dl) Protein (mg/dl) Normal 0-5² Lymphs >40 or >½ serum glucose Preterm: 65-150 Term: 20-170 Child: 5-40 Negative Bacterial¹ 100-20. Influenzae Strep.000 Lymphs³ Normal Fungal TB Lymphs Decreased Lymphs/Monos Decreased Elevated but less than 200 Elevated Markedly Elevated Positive Negative Varies Negative Gram Stain ¹In partially treated bacterial meningitis. meningitides H.

consider acyclovir for possible HSV infection. acidosis. add doxycycline. COMPLICATIONS         SIADH Septic Shock and DIC/Coagulopathy Cerebral Edema Neurologic findings.e. children. abnormal CSF with positive gram stain. age <2 years. or antibiotics within the previous month). ANTIBIOTIC THERAPY FOR MENINGITIS Regimens¹ Ampicillin and Gentamicin Or Ampicillin and Cefotaxime (Claforan) 1 to 3 months Ampicillin and Ceftriaxone (Rocephin) Or Ampicillin and Cefotaxime Infants over 3 months. smoke exposure. Antibiotic therapy should be guided by the patient’s age. renal or liver disease). and any underlying medical conditions (i. and pneumonia Seizures: evaluation (EEG and CT scan) should be considered for any seizures after 48-72 hours or focal seizures. and Ceftriaxone adolescents Or Cefotaxime ¹ Add Vancomycin if risk factors for resistant-pneumococcus present (daycare attendance. CSF analysis. indwelling catheters. or shunt in place. such as deficits in cranial nerves (especially VI) and hearing. NOTES Age Newborn Infant . Subdural effusions and empyema Hydrocephalus: communicating versus obstructive Bacteremia with resultant septic arthritis. both transient and persistent. For risk factors for HSV infection see section on Infant with fever of unknown source. shunts.136 MANAGEMENT The goal should be to stabilize the patient with concomitant evaluation for hypoxia. dehydration. pericarditis. In infants. Initial antibiotic regimens should be broad spectrum and based on most likely organism. DIC and electrolyte abnormalities. If considering Rickettsial disease. ill appearing. increased intracranial pressure.

137 NOTES .

Most infections occur within one month of the placement of the shunt but can occur later. if parent feels there is something wrong with the shunt then it should be evaluated. irritability. which is a predisposing cause. have reported incidence of infection between 2. TREATMENT Vancomycin (60mg/kg/day divided Q6 hours) and ceftazadime (150 mg/kg/day divided Q8 hours) are generally recommended until the organism is identified. However. placed for hydrocephalus. CT scan may reveal a shunt malfunction. CLINICAL MANIFESTATIONS     Fever. NOTES . gram negative organisms are also possible pathogens.4-9. Neurosurgery consultation is the general rule prior to tapping the shunt. but a normal CT does not rule out infection. Thus a head CT and ―shunt series‖ are generally obtained when evaluating a child for an infected shunt. CNS changes Shunt malfunction resulting in increased intracranial pressure and vomiting Peritoneal signs can develop Never underestimate the importance of the parent’s history. A lumbar puncture can be performed as long as there is no obstruction. Vancomycin peak levels of 30-45 mcg/mL and trough levels of 15-20 mcg/mL are often needed for adequate treatment. The diagnosis is made by obtaining a sample of CSF. Skin flora account for the majority of infections with coagulase negative staph leading the list. Removal of the shunt and/or intrashunt aminoglycosides are other treatment options in resistant cases.4 percent. The ability to ―pump‖ the shunt reservoir is not a reliable indication of shunt infection or malfunction.138 Shunt Infections Ventriculoperitoneal shunts.

blood culture x 2. cath U/A. Any toxic or ill appearing infant should have appropriate lab evaluation. Any infant with fever should have a careful exam to locate a source of infection. Vital signs should be measured and recorded. and respiratory rate. then the patient should be managed accordingly. and viral cultures in selected patients and as appropriate to season. If no source is located. should have a complete sepsis work-up which includes CBC. Group B Streptococcus or antibiotic treatment RISK FACTORS FOR POSSIBLE HSV INFECTIONS IN INFANTS        Primary maternal HSV infection at delivery Known exposure to HSV infected persons Fetal scalp electrodes Maternal history of STDs or unexplained fever at delivery CSF pleocytosis with a negative gram stain Failure of fever to abate within 24-48 hrs after starting antibiotics Unexplained CNS signs – such as a seizure INFANTS LESS THAN 60 DAYS Generally. heart rate. and empiric antibiotics.if risk factors are present. meningitis or other serious bacterial illnesses (SBI) that may not be immediately evident on physical exam. and a lumbar puncture.139 Infant with fever of uncertain source Management of the infant with fever is largely dependent on the infant’ s age and clinical exam.4 and no source. lab evaluation is necessary to evaluate for UTI. chest x-ray if respiratory signs are present. hospital admission. If a source of infection is identified. occult bacteremia. RISK FACTORS FOR SBI       History of prematurity Perinatal antibiotics Treated for unexplained jaundice History of previous rehospitalization Chronic illness Intrapartum history of mother with fever. Many consider pulse ox to be the fifth vital sign. blood pressure. Well appearing infants may have an SBI. Consider laboratory evaluation for neonatal HSV infections in infants less than 30 days. urine Gram stain and culture. Also to be considered are stool studies if diarrhea is present. . CRP. The following vital signs should be measured and recorded: temperature. infants less than 60 days with a rectal temperature over 100.

CRP.140 ADMISSION CRITERIA     All infants 0-30 days of age with fever of uncertain source (FUS) should be hospitalized Infants 31-60 days of age with FUS should be evaluated for low risk vs. If pt meets high risk. If decision is made to treat with antibiotics. Viral studies in selected patients and as appropriate to season. Consider adding IV ampicillin for severely ill infants or with findings suggestive of urinary tract infection. high risk criteria for SBI Any infant 31-60 days of age with FUS identified as high risk clinically or by laboratory data should be hospitalized Infants 31-60 days of age with FUS identified as low risk may be managed as outpatients or inpatients HIGH RISK CRITERIA FOR SBI       WBC < 5.000 Bands > 1500 Urinalysis > 10 WBCs/hpf or positive Gram stain Abnormal CSF indices When diarrhea present. and CSF) are obtained and follow up arranged. > 5 WBCs/hpf on stool specimen CRP > 10mg/dl if less than 4 weeks of age MEDICATIONS   All infants 0-30 days with FUS should be treated with IV ampicillin plus a third generation cephalosporin or gentamicin Recommendations for treatment of infants 31-60 with FUS vary depending on laboratory and clinical findings  Inpatient and outpatient low risk infants may be managed without antibiotics pending culture results and or change in clinical status.2 – evaluate pt for high risk verses low risk with diagnostic test or antibiotics needed unless clinically indicated. IM rocephin may be given after all cultures (blood.  Any infant 31 – 60 days of age with FUS identified as high risk should be hospitalized and receive an IV third generation cephalosporin. INFANTS BETWEEN 60 DAYS AND 36 MONTHS    If child appears toxic. Return if fever persists for >48 hours or rise in temp curve.Close follow-up. Tylenol for fever.000 or > 15.admit to hospital and perform full sepsis workup with IV antibiotics If temp < 102. If temperature > 102. Chest x-ray if respiratory symptoms present. urine.2 and child appears well. U/A Gram stain and culture. give .

141 Rocephin and send blood culture. Follow up in the clinic the next day. In addition. The infant should be seen by an attending that will be responsible for insuring proper follow up. The infant should be monitored for several hours and reexamined prior to being released home. NOTES .management of the outpatient must include a reliable family. LP should be performed first if there is any concern for meningitis on physical exam.  These are only guidelines that must be individualized based on clinical experiences and circumstances.

colony count. Diagnosis of UTI requires a urine culture. multiple organisms and proper handling of sample A suprapubic specimen with any gram negative or > 1000 gram positive colony forming units has > 99% probability for a UTI A catheter specimen with greater than 1000 colony forming units is suspicious for a UTI.5º C RISK FACTORS FOR UTI           Uncircumcised Male Fever > 102.000 is likely a UTI.2 Bacterial colonization Urinary stasis Obstruction Urinary reflux Foreign body Dysfunctional voiding Previous UTI Sexual practice/abuse . single vs. A gram stain will improve the sensitivity and specificity of the urinalysis as well as aid in empiric antibiotic selection. and a specimen with >100.000 cfu in a boy is likely a UTI.000 has a 95% probability for a UTI Clean catch specimens with greater than 100. a specimen with >10. A urinalysis should not be used to exclude or diagnose a UTI. Pyelonephritis Significant fever Abdominal /flank pain Vomiting Ill appearing Dehydration Cystitis Dysuria Frequency Urgency Enuresis Abdominal pain Temp < 38. A clean catch specimen with > 10.142 Urinary Tract Infections Urinary tract infections are a frequent source of fever in young children and the most common serious bacterial infection in infants and young children.000 cfu in a girl has 90% probability for a UTI. DEFINTION     Definitive diagnosis is by urine culture.significance of positive culture influenced by collection method.

infants. abdominal exam.  Proteus. or toxic patients Any patient with renal complications such as hypertension. dehydrated.newborns  Staph saprophyticus. obtain electrolytes. anuria. Seen more in very young infants. Pay close attention to blood pressure. CRP and start patient on IVFs – maintenance at a minimum. and those with suspected pyelonephritis. Causative agent in >80% of first UTIs. or known underlying uropathy Patients with unreliable or non-compliant families All infants < 30 days of age Infants 31-60 days of age identified as high risk by laboratory data .most common in teenage girls-sexually active  Coagulase negative staph  Staph aureus CLINICAL SIGNS AND SYMPTOMS OF UTI BY AGE Newborn Vomiting Temp instability Failure to Thrive Jaundice Infant Vomiting Fever Failure to Thrive Diarrhea Strong urine odor Irritability School Age Vomiting Fever Abdominal pain Dysuria Frequency Urgency Enuresis MANAGEMENT A detailed and thorough history and physical exam is important in the management of urinary tract infections.more common in males  Enterobacter species  Pseudomonas species Gram positive  Enterococci . Cr.most common in infants < 30 days of age  Group B Strep. In the hospitalized pt.  Klebsiella species-second most common organism. It is not recommended that routine follow up urine cultures be conducted during the initial course of inpatient or outpatient therapy. INDICATIONS FOR HOSPITALIZATION      Ill appearing. Blood cultures should be obtained in all toxic patients. BUN. elevated creatinine. sacrum. and GU exam.most common organism.143 ORGANISMS ASSOCIATED WITH UTI Gram negative  Escherichia coli. growth curve.

hypertension. family history of vesicoureteral reflux  Consider in any patient with pyelonephritis regardless of age  All children with recurrent UTI without known risk factors  The VCUG may be done within the therapeutic course or during hospitalization once the fever has resolved. The classic fluoroscopy VCUG allows for grading of reflux and delineation of anatomy. and labs trending down. Voiding Cystourethrogram (VCUG)  All children with first UTI at any age with any of the following: abnormal ultrasound. Pyelonephritis Cystitis Prophylaxis PO antibiotics based on sensitivities. Outpatient IM Rocephin followed by PO antibiotics based on sensitivities. Treat for 7-10 days.144  Consider in all children < 5 yrs of age with pyelonephritis (not absolute indication) TREATMENT OPTIONS Inpatient Parenteral Antibiotics: CephalosporinsCeftriaxone. Change to PO once patient afebrile > 48hrs. Renal ultrasound is not reliable as a diagnostic test for reflux. prophylactic antibiotics should be started at the end of the treatment course until the study can be done. consider adding ampicillin. Treat for total of 2-3 weeks. If the VCUG is delayed. Amoxicillin (10mg/kg qhs) Septra (2mg/kg TMP qhs) Nitrofurantoin (2mg/kg qhs) INDICATIONS FOR IMAGING Renal Ultrasound  All patients with first time pyelonephritis should receive a renal ultrasound regardless of age or sex. cefotaxime. combine with ampicillin. taking po well. pyelonephritis or renal scarring.If 0-30 days of age. Radionucleotide Cystogram (RNC) . Treat for total duration of 2-3 weeks. If 31-60 days of age. cefuroxime. poor growth.

GRADING SYSTEM OF VESICOURTERAL REFLUX Grade I. and ureter  Grade IV.Up ureter into pelvis and calyces.Mild dilatation of pelvis. calyces. and calyces with significant calyceal blunting The role of prophylaxis is evolving and may vary with age of the child.Gross dilatation and tortuosity of ureter. No dilatation. Sedation usually required. and the calyces are blunted  Grade V. Requires IV injection of radioisotope with imaging 2 hours later. pelvis. and the results of the imaging studies. NOTES    . Recent studies suggest prophylaxis is no longer required for grades I-III. severity of the initial illness.uses DMSA or glucoheptonate. normal calyceal fornices.Ureter and pelvis are moderately dilated.Into distal ureter Grade II.(DMSA or Glucoheptonate)  Primary role is identifying renal scars.  Grade III.145 A radionucleotide cystogram also called a nuclear cystogram is recommended for follow up studies because it involves less radiation than the standard VCUG but offers little anatomic detail Renal Cortical Scan.

   . Tunnel Infection or Port Abscess  Tunnel infection is defined as erythema. are also common. Positive culture from cellulitic area or any drainage from local site Positive culture of the catheter tip if removed. Order cultures for fungus and anaerobes as indicated. aureus.146 Catheter Related Sepsis A major complication of indwelling catheters is infection with bacteria or fungi. S. Failure to respond as manifested by persistent fever or progressing cellulitis should prompt further evaluation for unusual organisms. Two sets of cultures should be obtained with at least one set drawn percutaneously. and others Fungi-Candida species. and whether it is necessary or desirable to salvage the central line. hospitalization and empiric IV antibiotics. Skin biopsy/aspiration should be strongly considered if patients do no respond to IV therapy. S. Streptococcus species are also found. MANAGEMENT Management depends on the type of infection. the clinical status of the patient. Close follow-up is indicated and hospitalization should be considered if blood culture is positive and/or cellulitis is progressing. Acinetobacter. tenderness and induration overlying the subcutaneous tunnel tract which extends for more than 2 cm from the exit site.e. Greater than 15 colonies of organism reflect infection rather than contamination. Infections can range from exit site cellulitis to bacteremia with or without shock. MAJOR PATHOGENS    Gram positive bacteria-coagulase negative Staph (i. Exit Site Cellulitis  In the immunocompetent host who appears nontoxic and has localized cellulitis without evidence of rapid progression. Pseudomonas. If there is greater quantitative growth from the central culture than the peripheral this suggests that the CVC is the etiology. Gram negative bacilli-E. Klebsiella. coli. it may be appropriate to obtain local cultures and begin oral antibiotics. Enterobacter.  Neutropenic or immunocompromised hosts require a sepsis work-up. others are rare DIAGNOSIS  Blood cultures should be obtained when CVC infection is suspected. both MSSA and MRSA. epidermidis) is the most common cause.

Empiric therapy should include Vancomycin and an aminoglycoside or Fortaz pending appropriate culture results. Please ask for these at the nurse’s station or the pharmacy. aureus. it can be managed with antibiotics through the CVC. Pseudomonas. endocarditis.e. skin. fungi) or multiple organisms  Failure to respond clinically and clear bacteremia in 48-72 hours  Insertion site infection  Neutropenia  Complications arise (septic thrombophlebitis. valvular heart disease.147    Do appropriate cultures (blood.  CVC should be removed  Severe clinical presentation (i. drainage) and place on empiric IV antibiotics Catheter removal is usually necessary as these infections rarely respond to antibiotics After catheter removal antibiotics are generally continued for 7-10 days Catheter Related Sepsis  In many cases. NOTES . metastatic abscesses) Antibiotic Lock Therapy: involves instilling a high concentration of an antibiotic into the catheter lumen then ―locking‖ them in for a period of time when the catheter is not in use  This can be used when it is important to salvage the tunneled central venous catheter  Response varies with site of infection and organism (coagulase negative Staph shows best response)  Best when done in conjunction with systemic antibiotics  Recurrence can be as high as 20%  Therapy is generally continued for 2 weeks  Protocols for antibiotic/ethanol locks for catheter-related infections as well as protocols for the management of CVL occlusion exist. shock)  Certain organisms (S.

osteomyelitis. and sepsis. The rise of community based methicillin resistant Staph Aureus infections in healthy children poses a significant challenge in managing these infections. ‖boils‖ Folliculitis/pustular lesions Furuncle/carbuncle ―Insect/spider bite‖. TYPICAL COMMUNITY ACQUIRED MRSA INFECTIONS     Skin and soft tissue infections. Erythro susceptible.mostly skin abscesses. endocarditis. Obtain specimen for culture and susceptibility testing prior to starting antibiotic therapy..148 MRSA Infections in Children Staphylococcus aureus causes a wide variety of infections in children. Treatment failures with clindamycin have occurred with MRSA isolates that possess clindamycin –inducible resistance. RISK FACTORS FOR HEALTH CARE ASSOCIATED MRSA      Hospitalization in the past year Surgery in the past year Permanent indwelling catheter or percutaneous medical device Dialysis Long term care ANTIBIOTIC SUSCEPTIBILITY PATTERNS Most CA-MRSA isolates are resistant to macrolides but remain susceptible to clindamycin and trimethoprim-sulfamethoxazole. Clindamycin inducible resistance can be detected by the D-Test. cellulitis  Drainage of purulent collections is usually indicated. In vitro resistance to erythromycin but susceptibility to clindamycin by routine testing may not predict clinical effectiveness of clindamycin because of a property associated with erythromycin resistant CA-MRSA called inducible resistance to clindamycin. Clinda susceptible Negative D test Positive D test Then interpret as…. Clinda resistant Erythro resistant. request D test Clinda susceptible Clinda resistant . TESTING STAPH AUREUS FOR SUSCEPTIBILITY TO CLINDAMYCIN If lab reports…. arthritis. from skin infections such as impetigo and furuncles to more serious conditions such as pneumonia. Clinda susceptible Erythro resistant.. Clinda susceptible Clinda resistant Unknown.

Skin and soft tissue infections without severe illness  Empiric-Clindamycin if the prevalent community MRSA is clindamycin susceptible or the patient is penicillin allergic otherwise may use empiric augmentin.Lather and sit for 3 minutes three times per week for 3 weeks Chlorox rinse.1 tsp per gallon of water.clindamycin CONSIDERATIONS IN DECOLONIZATION TO PREVENT RECURRENCE   Efficacy is unproven Suggested criteria for decolonization  Multiple (>2) infections in the patient or within the household. renal ultrasound  If admitted but without serious illness. underwear. Betadine skin cleanser (7. cephalexin  Culture proven MRSA  Clindamycin.Povidine-iodine. CT chest/abd/pelvis.5%). change towels. and sleepwear daily. Will need to check for metastatic spread.echo. Bactroban to anterior nares BID for 2 weeks.Be aware that resistance to mupirocin is increasing worldwide and there are no studies that find topical antibiotics to be useful for eradicating nasal MRSA. washcloths.Ensure D-test negative if erythromycin resistant  Trimethoprim-sulfamethoxazole  Linezolid (very expensive) Inpatient-Suspected invasive illness and/or severe illness  Empiric-Vancomycin. Wear cotton undergarments. infections caused by culture-proven S Aureus with similar susceptibilities COMMON PROTOCOL     Keep fingernails clean and cut short. Linezolid (if intolerant of vancomycin) plus gentamicin.149 ANTIBIOTIC THERAPY FOR COMMUNITY STAPH AUREUS INFECTIONS Outpatient.2 times per week NOTES .

150 Hematology/Oncology .

151 .

DIC. thrombocytopenia. Should expect some hemolysis and drop in hemoglobin of 1-2 gm/dL. or abnormal lateral neck films  Wet purpura or gross mucous membrane hemorrhage  Deep muscle (e. Von Willebrand’s Disease. or any disorder in the coagulation cascade.g. quadriceps) hemorrhage presents with an enlarging. or who has paresthesia of the anterior thigh  Retropharyngeal hemorrhage should be considered in any known hemophiliac with pharyngitis. A lymphocytosis may be present from a recent viral infection. severe hip pain. and usually presents with bruising and petechiae. consider leukemic process as etiology. Christmas Disease. typically is self-limited. . the inability to perform a straight-leg raise. the following treatments may be employed:  WinRho-Rh immunoglobulin for patients who are Rh positive. tender muscle IMMUNE THROMBOCYTOPENIC PURPURA (ITP) General Information     Peak incidence around 2-7 years. but may have gross bleeding Platelet count typically is less than 20. vomiting. ITP. Less than 1% of patients will have serious/life threatening bleeding with ITP.  IVIG at 1 g/kg/day x 2.000/cc3. If necessary. For the vast majority of patients with ITP. or meningeal signs  Major organ hemorrhage presents with signs specific to that organ  Retroperitoneal hemorrhage should be considered in any patient with known coagulopathy who complains of abdominal pain. Treatment of hemorrhage demands knowledge of why a person is bleeding.  Steroids-Discuss with hematologist before initiating. Do not have hepatosplenomegaly or lymphadenopathy. In the presence of anemia and/or neutropenia. and what therapeutic options are available. focal neurologic findings. The following are types of hemorrhage which require immediate care:  Central nervous system bleeds can present with headache.152 Emergency Treatment of Bleeding Disorders Abnormal bleeding can be caused by platelet dysfunction. respiratory compromise. dysphagia. The most common defects encountered are caused by Hemophilia. See IVIG Administration Protocol in the Appendices. site of bleeding. Discuss dosing with hematologist. and malignancy. retinal hemorrhages.. no treatment is necessary.

). each with specific administration rates. This dose may be repeated 24 hours later if necessary. cryoprecipitate. Recombinant factor VIII concentrate is the treatment of choice in persons with Hemophilia A due to low risk of infection. use conservative measures first. give 125-200 IU/kg/dose). and commercially prepared factor VIII concentrate. give 250 IU/kg/dose.153 Treatment of CNS Hemorrhage  IV Immunoglobulin or Rh immune globulin: There are many preparations of IVIG commercially available (Gammagard. 1 gm/kg/day should be given. Rh immune globulin can only be used if the patient is Rh positive (if HgB ≥ 10. the dosage is the same for all. if HgB <10. Factor VIII activity is reduced. you should give 50 units/kg of factor VIII. Platelet Transfusion: In general. In the emergency situation. In treating major hemorrhage or providing hemostasis for surgery. Steroids: May be useful for intracranial midline shifts with CNS hemorrhage Plasmapheresis: Transient benefit Emergency splenectomy: Will increase platelet count in 80% of ITP patients. However. the initial dose should be followed by continuous replacement either as bolus doses of 25 units/kg every 6-12 hours or by continuous infusion. If patient is stable. in the emergency situation. Gamimune N. transfusion of platelets in acute ITP is not indicated. 500 mg/kg/day can be given Q day for 4 to 5 days (see chapter on IVIG administration). they do play an important role. However. Always consult neurosurgeons in patients with a CNS hemorrhage FACTOR VIII DEFICIENCY (HEMOPHILIA A) Patients with Hemophilia A will have prolonged PTT but normal PT and bleeding time. Alternatively. Desired Factor Activity Levels for Specific Situations Type of Hemorhage Desired Factor Level Life Threatening Bleeds  CNS 100%  Major Surgery/Trauma  Retropharyngeal  Retroperitoneal Spontaneous Joint Bleed 40-60% Spontaneous Muscle Bleed 40-60% Severe Abdominal Pain 40-80% Simple Dental Extraction 40%      . Sandoglobulin. Factor VIII may be administered by using fresh frozen plasma. etc. Management of Bleeding One unit of factor VIII/kg will raise the plasma activity level 2%. So to raise the plasma level from 0 to 100% activity.

but FFP has one unit of Factor IX per milliliter.  Pure Factor IX concentrates such as Alphanine. VII. Benefix raises the activity by 0. This is the treatment of choice.5% for each unit/kg administered. give the whole vial. Management of Bleeding Goals for desired Factor IX activity levels are the same as in Factor VIII deficiency (see table above). Amikar is contraindicated with hepatic disease or hematuria. It can be given intravenously or orally. An alternative is tranexamic acid or Cyklokapron. VII. you may want to use aminocaproic (Amikar). Cryoprecipitate has no Factor IX. Oral Bleeding If the child has oral bleeding from trauma or dental extraction. Maximum dose is 30 grams/24 hours. FACTOR IX DEFICIENCY (CHRISTMAS DISEASE) Patients with factor IX deficiency will have a prolonged PTT and low Factor IX activity. Konyne. Alphanine contains only trace amounts of Factors II. thus 20 units/kg will raise the activity level to 40%  20 units/kg x 18 kg= 360 units of Factor VIII should be administered  Always round up the dose to the vial size available. which is also used for oral bleeding. has a bleed into his knee.154 Example: A child with Hemophilia A. If your calculated dose is 360 units and the vial size is 510. Doses should be adjusted accordingly in the presence of impaired renal function. PCC’s contain not only Factor IX. and X. The dosage is 25 mg/kg given QID. but also Factors II.8% for each unit/kg. who weighs 18 kg. Factor IX concentrates are commercially available and are generally placed in one of three categories:  Recombinant Factor IX: Benefix. Because of the presence of the other factors. Potential sites of bleeding are similar to Factor VIII deficiency. Alphanine will raise activity by 1% for each unit/kg. They should be used only when a limited number of administrations is expected. X. there is a significant thrombogenic potential with repeated doses. and can be safely used in a situation where repeated administration is necessary. a fibrinolytic blocking agent. PCCs will raise the activity 1.  Prothrombin Complex Concentrates (PCCs) including Profilnine. . The initial dose is 100-200 mg/kg followed by 100 mg/kg Q4-6 hours. What amount of factor VIII should be administered?  Current factor VIII activity (always assume 0% in a severe bleed): 0%  Desired factor VIII activity (from chart above): 40%  1 unit/kg of Factor will increase serum activity by 2%.

renal. petechiae.) heparin therapy may play a role. Steroids should not be used unless the underlying condition may be benefited or if shock is present  . LOOK AT THE SMEAR!!! Platelet count less than 100. such as brain or kidney. consumptive coagulopathy. where there is a cycle of thrombus formation and lysis that consumes clotting factors. It should not be considered a disease. Platelet transfusion may be necessary Exchange transfusion can be used to quickly correct the physiologic deficiencies Heparin therapy is controversial. Some common causes of DIC include:  Shock or sepsis  Malignancy. collagen vascular diseases Laboratory Evaluation        Anemia with red cell fragmentation (schistocytes. such as fluid replacement. or thrombus formation.000/mm3 (commonly even less than 30. Factors VIII and V are reduced in DIC. However. but the underlying diagnosis prompts a laboratory evaluation. Often there is no obvious clinical evidence. such as antithrombin III. and protein S. There may be evidence of ischemia in a major organ. protein C. The dose is 10 cc/kg and may be repeated as needed. treatment of shock. but its primary role is to replace anticoagulants. and these assays can be helpful in difficult cases where the diagnosis of DIC is unclear Management      Successful treatment of DIC is primarily dependent on appropriate treatment of the underlying disease. correction of acid-base imbalances Fresh frozen plasma provides replacement of clotting proteins. especially acute promyelocytic leukemia  Severe trauma. crush injury. etc. or burns  TTP. a bolus dose of 100 U/kg is given followed by a continuous infusion of 100 U/kg given over 6 hours. in patients with serious thrombotic complications (brain. and is not typically necessary in most cases of DIC. The fibrinogen can be normal in diseases that are associated with ―chronic‖ DIC. such as acute promyelocytic leukemia. which confirms DIC.000/m3) D-dimers are elevated PT/PTT prolonged Prolonged thrombin time Decreased fibrinogen (<200) with acute DIC. bite cells). gangrene. Clinical presentations can include bleeding. If it to be used. but rather a process that can be initiated by a variety of stimuli.155 DISSEMINATED INTRAVASCULAR COAGULATION (DIC) DIC can be described as a paradoxical.

156 NOTES .

Hodgkins disease. chest CT. Finally. throat. EBV Nasopharyngeal swab for RSV. abscess. many hematology-oncology patients (e. and lungs  GI tract with particular attention to the RLQ (typhlitis) and the perianal area (NO RECTAL TEMPS OR SUPPOSITORIES)  Hepatomegaly and splenomegaly as evidence of fungal or viral infections  Integument looking for cellulitis.g. pleural tap. The following are guidelines for the evaluation and treatment of patients who are immunocompromised and febrile. previous infection of catheter PHYSICAL EXAM It must be remembered in the neutropenic patient that the signs of inflammation may be muted or absent. as indicated by H&P Consider viral titers/PCR for CMV. sinuses. hemolytic anemias) will be functionally or anatomically asplenic. Consider sinus CT. radiation or chemotherapy—meds and dates received) Exposures.g.. sickle cell disease. influenza if indicated . CRX can interrupt skin and mucous membrane integrity allowing an entry site for microorganisms to invade systemically. U/A and C&S. septic emboli (small erythematous or hemorrhagic papules) or varicella-zoster and examination of indwelling catheter site  Pain alone may be a sign/location of an infection LABORATORY EVALUATION        CBC with differential Blood culture from all central lines. Also. chronic ITP. etc. teeth. CRX most commonly causes neutropenia but also affects immunoglobulin production and cell mediated immunity. adenovirus. urine gram stain if indicated Chest X-ray in any patient with respiratory symptoms or young age. abdominal CT.157 Fever in Immunocompromised Children Children may be immunocompromised for a variety of reasons. Obtain PA and lateral in radiology department if at all possible. HISTORY      History of present illness and comprehensive ROS to help define the source of infection Underlying disease Recent therapy (e.. rendering them more susceptible to encapsulated organisms. The most common form of immunodeficiency is secondary to chemotherapy (CRX) in the patient with a malignancy. Proceed with care!  Thorough sinopulmonary exam to include ears. Dull TMs may be the only sign of a severe otitis media. especially to varicella Presence of an indwelling catheter.

Gram positive. utilize empiric therapy as above plus therapeutic TMP-SMX and possibly macrolide When fungus is suspected use caspofungin All patients should be under neutropenic precautions NOTES . do not wait for screening labs prior to starting antibiotics. Organisms Requiring Coverage      In all patients consider gram negatives (including Pseudomonas) and gram positives (e.. oral or GI pathology. no source is evident) must include Gram negative coverage Due to increased frequency of Gram positive line infections. anaerobic coverage: meropenem Metronidazole or clindamycin should be used if there is the possibility of anaerobic infection (abscess. or appear toxic need initial Gram positive coverage along with Gram negative coverage AML patients with fever/neutropenia need initial Gram positive/Gram negative coverage Example single agent Gram negative coverage: ceftazidime Example single agent Gram negative. steroids. previous Gram positive line infection. ANC is calculated by multiplying the percent neutrophils plus percent bands times the total white blood cell count {i. initial Gram positive coverage may be indicated Patients who have mucositis. ANC=(WBC) (%bands + %neutrophils)}.e. In an ill appearing patient.e. prolonged broad spectrum antibiotics.g. fluid bolus and monitor vital signs and I/O carefully Empiric antibiotics should be administered within an hour of presentation. consider fungus. After evaluation is completed and cultures have been obtained. administer O2. begin empiric antibiotics covering the most likely organisms.158 TREATMENT OF THE FEBRILE. Empiric therapy (i.. NEUTROPENIC PATIENT Neutropenia is defined as an absolute neutrophil count (ANC) less than 1000. Staph and Strep) If indwelling catheter: Staph epi and MRSA If GI symptoms or chronic sinusitis: anaerobes If interstitial pneumonia: pneumocystis If prolonged neutropenia.. Treatment             If the patient shows signs of early septic shock.5°F x 2 in 24 hours or ≥ 101°F x 1 is defined as a fever in the immunocompromised patient. sinus) If pneumonitis is suspected. A temperature over 100.

Elevations of BNP may be associated with adverse outcomes. unexplained tachypnea or syncope. cough. Anticardiolipin Ab) Inherited Antithrombin III Deficiency Protein C Deficiency Protein S Deficiency Factor V Leiden Mutation (Activated Protein C Resistance) Prothrombin Gene Defect Increased Factor VIII Increased Fibrinogen.159 Thrombotic Disorders Questions about management regarding deep vein thrombosis (DVT) and pulmonary embolus (PE) can often be answered by calling 1-800-NO-CLOTS. If the Doppler ultrasound is negative. yet high clinical suspicion remains. hemoptysis. effusion. Risk Factors for Thrombosis Acquired Central Venous Line Congenital Heart Disease Trauma TPN Infection Surgery and Immobilization Oral Contraceptive Use Obesity Nephrotic Syndrome Inflammatory Bowel Disease Diabetes Mellitus Systemic Lupus Erythematosus Antiphospholipid Syndrome (Lupus Anticoagulant. and/or discoloration of affected limb Inability to draw blood from a central line Swelling of face and neck in the superior vena-cava syndrome Pulmonary embolism may present as dyspnea.   . or chest pain (pleuritic) Fever occurs in up to 20% of PE. pain. although sensitivity is decreased with calf vein thrombosis. Dysfibrinogenemia Plasminogen Deficiency Homocysteinuria MTHFR mutation CLINICAL PRESENTATION      Swelling. hypoxia. a venogram or MRI may be diagnostic. or infiltrate) BNP (Brain natriuretic peptide) has a sensitivity and specificity of only 60-62%. tachypnea. A patient can be predisposed to developing thrombosis by both inherited disorders as well as acquired risk factors. mimicking pneumonia EVALUATION OF DVT/PE  Compression Doppler Ultrasound: Is the best and least invasive test for DVT. Sensitivity is approximately 90%. Chest X-Ray: Findings are generally non-specific. although most patients with a PE will have abnormalities present (atelectasis.

Values provided are starting suggestions. Relative contraindications to lytic therapy include history of stroke. Expensive product.6 PT. although it is invasive and 5% of patients will have complications due to catheter insertion or dye reactions. If D-dimer is greater than 500 ng/dL then the sensitivity for PE is over 95%. Lovenox) or heparin is also used for patients with a DVT/PE. however. TCT. most patients with a PE will have a low or intermediate probability scan. . It is recommended. APTT. 1 Start heparin therapy immediately after completion of thrombolytic therapy. The length of time for optimal maintenance is uncertain. but only 40% sensitivity. A loading dose of heparin may be omitted. regardless of whether thrombolytic therapy is utilized. thus if there is high clinical suspicion for PE further testing is warranted. Activator (tPA) Maintenance: 0. however specificity is low with the D-dimer test. that heparin therapy not be initiated until after thrombolytic therapy is complete. Pulmonary Angiography: This is the gold standard test for PE. transient ischemic attack. neurologic disease. Anticoagulation therapy with low molecular weight heparin (LMWH. Echocardiography: Only useful in massive PE where rapid diagnosis is necessary to justify use of thrombolytic therapy THERAPY FOR THROMBOEMBOLISM Thrombolytic therapy is indicated in patients with hemodynamic instability (hypotension). Agent of choice mg/kg/hr for 6 hours in children due to fibrin Max dose: 100mg given specificity and low over 6 hours. or right ventricle dysfunction associated with massive PE. and hypertension. immunogenicity.1-0. A high probability scan has 95% specificity. Systemic Thrombolytic Therapy1 Medication Dose and Route Comments Tissue Plasminogen Loading: None Monitor fibrinogen.160      D-dimer: If low probability of PE and D-dimer less than 500 ng/dL then PE is very unlikely. Ventilation-Perfusion (V/Q) Scan: A normal scan essentially rules out a PE. some patients may respond to longer or shorter courses. However. A negative spiral CT has good negative predictive value. severe hypoxia. Helical or Spiral CT: A spiral CT scan has a sensitivity of 70-90%.

5-1. then 4 hrs after next dose >2. Older children and adults: Once therapeutic obtain Load: 80 units/kg daily CBC and PTT.000 units Monitoring Anti-Factor Xa Levels in Pediatric Patients Anti-Factor Xa Hold Next Dose? Dose Change? Repeat Anti-Factor Level Xa level? <0.0 below).20.5 Decrease by 40% Before next dose.5 4 hrs after 3rd dose and 4 hrs (Lovenox) mg/kg/dose SQ Q12 hrs after dose adjustments (see >2 months or >5 kg: 1.0 units/mL Until anti-Xa 0. Obtain >1 yr: 20 units/kg/hr IV PTT 4 hrs after bolus or any change in infusion rate.35 units/mL No Increase by 25% 4 hrs after next dose 0. Max dose: 150 mg prophylactic Xa level is 0.0 units/mL No No Next day.1-1.0 u/mL.5 units/mL repeat Q12 hours .4 u/mL.75 less monitoring. and possibly less >2 months: 0.5-1. and monthly thereafter (all 4 hrs after dose) 1.3-0. Heparin Load: 75 units/kg IV over Adjust heparin to maintain 10 min PTT between 60-85 seconds Maintenance: (approx. less HIT mg/kg/dose SQ Q12 hrs syndrome.6-2. then 1 wk later.5 units/mL No Decrease by 20% Before next dose 1. Therapeutic Xa mg/kg/dose SQ Q12 hrs level is 0. if units/mL not <0. Potential Prophylaxis: advantages over heparin are <2 months: 0.5 mg/kg/dose osteoporosis with long term SQ Q12 hrs use. Advantages over warfarin are lack of drug/food interactions.49 units/mL No Increase by 10% 4 hrs after next dose 0.161 Anticoagulation Therapy for DVT or PE Medication Dose and Route Comments Enoxaparin/Low Molecular Treatment: Monitor anti-factor Xa level Weight Heparin <2 month or <5 kg: 1. Maintenance: 18units/kg/hr Max dose: 10.0 units/mL 3 hours Decrease by 30% Before next dose.35-0. equal to anti-factor <1 yr: 28 units/kg/hr IV Xa level of 0.7).

The timing of this work-up is controversial. Infusion rate of 10 mg/mL solution should not exceed 5 mg/min.75 mg/units of heparin 60-120 minutes 0. at least two doses of LMWH should be withheld and.375 mg/units of heparin Maximum dose of 50 mg. The same protocol for protamine administration for the reversal of heparin is used for LMWH. all children deserve a work-up for the etiology of their thrombo-embolic event. Protein S Activity Protein C and S Antigen Activate Protein C Resistance (surrogate marker for Factor V Leiden Mutation) Antithrombin III Prothrombin Gene Defect ANA Fasting Homocysteine Factor VIII Activity Level D-dimer MTHFR gene Lipoprotein (A) Anti-cardiolipin Dilute Russell Viper Venom TREATMENT OF ANTICOAGULANT INDUCED BLEEDING Protamine sulfate is used to reverse heparin and LMWH therapy when an immediate effect is required. Prior to lumbar punctures or epidural procedures. Hypersensitivity reactions may occur in patients with known reactions to fish or protamine containing insulin. 1 . if possible. It is often helpful to evaluate the child’s parents as well.0 mg/units of heparin 30-60 minutes 0.25-0.5 mg/units of heparin >120 minutes 0. However.375-0.              Laboratory Evaluation of a Thromboembolic Event Protein C Activity. determine anti-factor Xa levels prior to the procedure.5-0. Reversal of Heparin Therapy1 Time Since Last Heparin Dose Protamine Dose <30 minutes 1. but tests that may be helpful are presented below. Thus. A hematology consult is warranted in all children with a thromboembolic event. it is probably best to initiate the work-up at the time of the event and repeat the abnormal tests once anticoagulation therapy is terminated. as some tests may be abnormal because of the recent event.162 EVALUATION Children are more likely to have abnormalities within the coagulation cascade than adults.

163 TREATMENT OF BLOCKED CATHETERS Thrombolytics are used in low doses to restore catheter patency. Attempt to aspirate the volume of tPA instilled into the line PLUS 2 mL of blood (0.2 mL in nursery patients). Streptokinase should not be used to reestablish patency due to potential allergic reactions with repeated doses. NOTES . This will assure the drug was removed from the line and not pushed into the systemic circulation.5 mL NS NS. Fill the IV line with the dose of tPA as indicated in the table below.5 mg in 0.5 mg in 0. tPA is currently the treatment of choice.5 mg in 0. treat one lumen NS at a time >10 KG 2 mg in 2 mL NS 2 mg in 2 mL NS. Allow tPA to dwell for 1-2 hours.5 mL 0. 2 mg in 2 mL NS treat one lumen at a time 1 Also see pharmacy preprinted order sets for catheter occlusions which should be located in each nursing station.5 mL 0. Guidelines for Local Instillation of tPA in Central Venous Lines1 Weight Single-Lumen Double-Lumen SC Port CVL CVL ≤ 10 KG 0. Procedure may be repeated one time if necessary.

and administration of parenteral antibiotics. Always consider that a patient with sickle cell disease can have pain that is not related to sickle cell disease (e. pneumonia. Clinical Diagnosis  Children less than five years may present with dactylitis. BUN. However. pain may change to any locality. a swelling of the hands and feet. and long bones.5 maintenance) using D5 ½ NS with appropriate amount of KCl added.164 Sickle Cell Disease PAIN CRISIS Pain associated with a vaso-occlusive crisis is due to ischemia secondary to occlusion in the microvasculature. appropriate cultures.. as renal salt wasting occurs commonly in children with sickle cell disease.g.) Laboratory Evaluation     Laboratory evaluation should include CBC with differential and reticulocyte count If the patient requires admission.g. Common sites are the abdomen. and type and cross should be obtained if clinically indicated Check oxygen saturation Treatment  Fluid hydration at 1800-2000cc/m2 /day (or approximately 1. Monitor fluids and electrolytes closely. any temperature over 101 requires a complete history and physical in search of a source. back.). and liver enzymes should be checked Blood cultures (if febrile). pancreatitis. As the child gets older. Patients with vaso-occlusive crisis may have a low-grade temperature. appendicitis. creatinine. osteomyelitis. CXR (if pain is located to the thorax). etc..25 to 1. infection. electrolytes. dehydration. Avoid the use of meperidine Do not give supplemental oxygen unless the patient is hypoxic Do not use Ketorolac for greater than 5 days       . A thorough history and physical should be performed to try to discover a possible triggering event for the painful crisis (e. and fluid overload predisposes to ACS. This may be a patient’s initial presentation with sickle cell disease. etc.

codeine 1 mg/kg) PO Q4-6 hours PRN  Encourage 1 ½ maintenance fluid intake MODERATE PAIN:  Usually managed in the clinic or emergency department.5 mg/kg Q6 hours (Max: 15 mg if <50 kg. 30 mg if >50 kg) plus morphine PCA (see table below). OR  Ketorolac 1 mg/kg IV load (Max: 15 mg if <50 kg. the rescue dose should be 25% of initial dose.165 MILD PAIN:  Usually can be managed at home with Ibuprofen 10 mg/kg PO Q6-8 hours plus acetaminophen 10-15 mg/kg (+/. Start PCA after pain controlled with boluses as above. If mildly sedated but still in pain.  Bolus 10-20 cc/kg of isotonic fluids. admission will be required  If able to discharge. Give rescue dose of morphine every 30 minutes for breakthrough pain. then give 1 ½ maintenance fluids either PO or IV  If the patient has not improved after 6 to 8 hours. send home on ibuprofen and acetaminophen with codeine at above doses around the clock for 24 to 48 hours SEVERE PAIN:  Ketorolac 0.05-0.5 mg/kg Q6 hours (Max: 15 mg if <50 kg.  Ketorolac 1 mg/kg IV load (Max: 15 mg if <50 kg. rescue dose should be 50% of initial dose.1 mg/kg (Max: 10 mg) IV Q3-4 hours.1 mg/kg/dose (Max: 10 mg) IV Q3-4 hours  Reassess in 30-60 minutes and give rescue dose of morphine every 30 minutes for breakthrough pain. If no pain relief or sedation. 30 mg if >50 kg) plus morphine 0. the rescue dose should be 25% of initial dose. 30 mg if >50 kg) plus morphine 0. rescue dose should be 50% of initial dose. If no pain relief or sedation. . OR  Ketorolac 0.05-0. 30 mg if >50 kg) plus acetaminophen with codeine 1 mg/kg of codeine PO. Any patient receiving IV narcotics should have a continuous pulse oximeter. If three or more rescue doses are required within a 24 hour period. If mildly sedated but still in pain. increase the maintenance morphine dose by 2550%.

166 Morphine PCA1 Adjustments   Increase 4 hour Limit by 10-20% if patient reaching the limit and pain  not controlled   Increase PCA bolus dose and/or  basal rate by 10-20% if patient requiring frequent PCA boluses or frequent pushing of PCA button 1 Any patient receiving IV narcotics should have a continuous pulse oximeter. slurred speech. then give 1 ½ maintenance fluids either IV or PO. colace) Opiates tend to cause itching due to histamine release and can cause nausea/vomiting.e. Be aware that excess fluids may precipitate or exacerbate acute chest syndrome. especially in the young adult. Promethazine should be used for nausea/vomiting.18-0. EEG if indicated Lumbar puncture if indicated Type and cross for exchange transfusion RBC phenotype Quantitative hemoglobin electrophoresis Starting Dose Basal Rate: 0. Diagnostic clues to a thrombotic cerebrovascular accident (CVA) include hemiparesis. nalbuphine (10-20 mcg/kg/dose IV Q4-6 hrs PRN). Most events are caused by thrombotic phenomena. but strokes can also be due to hemorrhage. a head CT without contrast should be ordered.    Bolus 10-20 cc/kg of isotonic fluids. and seizure. CEREBROVASCULAR ACCIDENTS Strokes and other cerebrovascular events are major causes of mortality and morbidity in HgbSS patients. Diphenhydramine. alteration in mental status.04 mg/kg/hr PCA Bolus: 0.04 mg/kg/dose Lock Out: 6-15 minutes 4 Hour Lock Limit: 0. All patients should have a form of incentive spirometry and a stool softener (i.5 mg/kg .24-0. or naloxone (5-10 mcg/kg/dose IV Q6 hrs PRN) should be used PRN for itching..01-0. If unable to obtain stat MRI/MRA. Laboratory Evaluation          CBC with differential CMP Reticulocyte count MRI/MRA without contrast.

If not resolved within 2 hrs.  Consider pseudoephedrine 30 mg PO QHS (<10 years of age) or 60 mg PO (>10 years of age) for priapism prophylaxis APLASTIC CRISIS An aplastic crisis is a decreasing hemoglobin. Give sickle negative. then surgical intervention with Winter shunt should be considered. Without adequate treatment. leukodepleted. PRIAPISM Priapism is a persistent. with a simultaneous fall in reticulocytosis (retic count ≤ 2%). and analgesics. painful erection due to vascular engorgement of the corpora cavernosa by sickled blood. The patient may also have coexisting splenic sequestration. This is usually managed at home with frequent emptying of bladder.167 Treatment Consult hematology-oncology attending. antigen matched PRBC. and Kell negative as well as sickle negative and leukodepleted. warm baths. PRBC should be C. Fatigue. or heart failure may all be signs of an aplastic crisis. The Red Cross can perform an automated exchange transfusion on patients over 18kg. priapism leads to impotence in 80% of patients. consult hematology-oncology attending  Intravenous fluid hydration at 2500 cc/m2/day  Analgesics as used in a pain crisis  Consider foley catheter if patient not adequately emptying bladder on his own  Consider simple transfusion if the above measures are not successful  Some centers recommend aspiration and irrigation with epinephrine within 12 hours  Surgical management is controversial. and if available. E. but if detumescence has not occurred within 24 hours. pallor. Management Acute Reversible Priapism: Typically resolves in less than 2-4 hours (stuttering priapism). It may follow a viral prodrome. approximately 1 gm per day. increased oral intake of fluids. tachycardia. . commonly associated with an infection with parvovirus B19. Acute Prolonged Priapism:  Requires admission. patient should alert their physician. Treatment consists of exchange transfusion with the goal of reducing HgS to less than 30% and total hemoglobin to 10 gm/dL. If antigen matched blood is not available. dizziness.

   . and reticulocyte count. e. SPLENIC SEQUESTRATION CRISIS A sequestration crisis is a relatively sudden entrapment of RBCs in an enlarged spleen associated with hemoglobin 2 gm/dL below baseline. Physical exam reveals an enlarged spleen. may also be present. Assess the patient and then give another 5 cc/kg if needed. Do not over transfuse as the hemoglobin will rise 1-2 gm/dL once sequestered RBCs are released from the spleen. HbSC). If anemia is severe (Hgb <5). reticulocyte count. give 5 cc PRBCs/kg over 3-4 hrs. Management in Clinic or ED   Patient should be rapidly triaged and evaluated Laboratory evaluation should include CBC with differential.g. urinalysis. it is necessary to rapidly stabilize the patient with intravascular fluids/PRBCs and emergently consult a surgeon for splenectomy Splenectomy is the treatment of choice for recurrent sequestrations or a single severe episode SICKLE CELL DISEASE AND FEVER (TEMP ≥ 101° F) The child with sickle cell disease and fever should never be taken lightly. total bilirubin. Signs of intravascular depletion. The goal is to obtain a hemoglobin of 8-9 gm/dL. There is a 15-35% mortality rate for pneumococcal sepsis in sickle cell patients. If signs of fluid overload.. Laboratory evaluation should include a CBC. and blood culture. Other labs. such as tachycardia or shock. larger aliquots (10 cc/kg) may be used if necessary.168 Management Transfusions are the mainstay of therapy if the patient is symptomatic and/or the hemoglobin is less than 5 gm/dL without evidence of erythroid recovery. If the patient is hemodynamically unstable. Remember not to transfuse to a hemoglobin over 12 gm/dL. stool studies. Management  Transfuse if the patient is symptomatic or hemoglobin is <5 gm/dL (or <7-8 gm/dL in patients with a higher baseline. a dose of Lasix 1 mg/kg can be given. There is usually an increased reticulocyte count and an increased indirect bilirubin. and CXR should be obtained when clinically indicated. influenzae. liver enzymes. CSF studies. The principle is to increase their hemoglobin without putting them into fluid overload. Give 5 cc/kg over 3-4 hours and repeat if needed. Once the patient’s hemoglobin is above 7. including CMP. such as pneumococcus and H. It must be remembered that children with sickle cell disease are at increased risk for bacterial infections due to encapsulated organisms.

Give ceftriaxone 75 mg/kg/day IV Q24 hours. use ampicillin/sulbactam 50 mg/kg/dose IV Q8 hours. or aplastic crisis) Poor compliance or follow-up Inpatient Management    CBC and reticulocyte count should be obtained daily. stroke.5 mg/kg/dose IV Q8 hours. use clindamycin 10 mg/kg/dose (600 mg Max) Q6 hours plus gentamicin 2. unstable vitals. or CNS infection add vancomycin 10-15 mg/kg/dose (1 gram Max) IV Q 8 hours Patient should be admitted or at a minimum observed for 6 hours in the clinic/ED setting (see below for admission criteria) If discharged home. PO intake plus IV rate should be 1 to 1.000 Hemoglobin <5 gm/dL History of pneumococcal sepsis or bacteremia or other serious bacterial infection Dehydration Infiltrate on CXR or O2 saturations below baseline Allergic to penicillins or cephalosporins Other acute complications (severe pain. the patient should be seen the following day and given a second dose of ceftriaxone Admission Criteria              Age less than 1 years Temperature over 102. Patient should be assessed frequently with attention to vital signs.000 or less than 5.2°F Hypotension {SBP <70 mmHg + (2)(age in years)} Poor perfusion WBC >30. Other labs should be checked as clinically indicated. Use ceftriaxone 75 mg/kg (2gm max) IV. If in shock. If allergic to cephalosporins and penicillins. splenic sequestration.000 Platelet <100.169  Antibiotics should be administered promptly. Acetaminophen 15 mg/kg/dose PO Q4-6 hours. acute chest syndrome. or cefotaxime 50 mg/kg/dose IV Q8 hours if less than 50 kg or cefotaxime 1-2 grams/dose IV Q8 hours if over 50 kg (Max dose 12 grams/24 hours).5 times maintenance Antibiotics should be continued until cultures are negative. and/or ibuprofen 10 mg/kg/dose PO Q8 hours PRN fever control Add vancomycin 10-15 mg/kg/dose IV Q8 hours if critically ill (1 gram max single dose)      . If allergic to cephalosporins but not penicillins. priapism.

cough. with pulmonary symptoms (chest pain. etc. Also give azithromycin 10 mg/kg/day IV/PO on day one and 5 mg/kg/day IV/PO on days 2 through 5 (other macrolides may be used instead) If critically ill. Monitor electrolytes if on IV fluids Repeat CXR if acutely worsens PO intake plus IV fluids should equal 1 times maintenance (fluid overload can make acute chest syndrome worse) Give ceftriaxone 75 mg/kg/day Q24 hours (2 grams max dose). hypoventilation and atelectasis. tachypnea. sickle negative. and tolerating PO fluids and medications ACUTE CHEST SYNDROME Acute chest syndrome is defined as a new infiltrate on chest x-ray. fat embolization. wheezing. Evaluation     CXR CBC with differential. hypoxia. antigen matched PRBCs Pulse oximetry Management             CBC and reticulocyte count should be obtained daily. There is a multifactorial etiology and pathophysiology. and sputum cultures (if obtainable) Type and cross leukodepleted. or deteriorating (remember to taper steroids over 7-10 days to prevent a rebound in pain) . infarction. afebrile for 24 hours. add vancomycin 10-15 mg/kg/dose IV Q8 hours (1 gram max single dose) Use supplemental oxygen if needed for sats <92% or below patient’s baseline Give bronchodilator therapy with albuterol with PEP Q4-6 hours Simple transfusion with 10 cc/kg of PRBCs over 3-4 hours if severely ill. blood cultures. deteriorates during hospitalization or does not improve within 12-24 of hospitalization (do not transfuse above a hemoglobin of 12 gm/dL) Exchange transfusion will be necessary if critically ill or starting hemoglobin is too high Incentive spirometry 10 inhalations Q2 hours while awake or bubble therapy if too young for incentive spirometry Consider steroids if history of asthma.) and/or fever. blood and other cultures are negative at 48 hours. May give cefotaxime 50 mg/kg/dose IV Q8 hours if less than 50 kg or cefotaxime 1-2 grams/dose IV Q8 hours if over 50 kg (Max dose 12 grams/24 hours).170  Patient may be discharged when they are clinically stable. retractions. prolonged expiratory phase. including infection. reticulocyte count. pulmonary edema.

Likewise. try to avoid undertreating pain which can lead to splinting and worsening of acute chest syndrome. NOTES . Try to avoid over sedation which can lead to hypoventilation.171  Use pain medications as described in above section on pain management. atelectasis. and worsening acute chest syndrome.

000 and in AML when WBC >100. Maintain plts > 75. sludging and intracranial hemorrhage or pulmonary stasis. Uric Acid. Management        Keep patient upright Notify anesthesia and radiotherapy Do not paralyze for intubation Begin tumor lysis therapy Try to obtain a diagnosis via BM aspirate. dyspnea. Phos.172 Oncologic Emergencies HYPERLEUKOCYTOSIS Elevated WBC in a patient with acute leukemia in which there is risk of increased viscosity. SUPERIOR VENA CAVA SYNDROME A Mediastinal mass which causes compression or occlusion of the SVC and/or trachea. etc RX with Rad Rx (2 Gray/d) until symptoms resolve May need prednisone 40-60 mg/m2/day . pleural tap.000 to avoid CNS bleed Do not transfuse RBCs unless Hgb <7 gm% so viscosity is not increased further. papilledema. There is no increased risk when leukocytosis is due to mature cells as in infection or CML. Will need double lumen renal dialysis catheter placed by surgeons or PICU attending in femoral vein. transfuse slowly in 5 cc/kg aliquots. decreased sensorium. headaches. plethora or cyanosis of the face/neck. distended veins of the neck and collaterals on the chest. Usually associated with non Hodgkin’s lymphoma or leukemia/lymphoma. Symptoms may include: stridor. U/A) Treatment     Immediate leukoreduction: Arrange an emergency leukophoresis to be done by the American Red Cross (251-6000). If Hgb < 7.0. Risk increases significantly in ALL when WBC >500. Ca. Evaluation    CBC Type and Cross match Tumor lysis labs (BMP. Hydrate at 1 ½ maintenance provided patient does not have signs or symptoms of elevated intracranial pressure.000.

may need to transfuse 1st to avoid CHF. Can also use Amphogel.173 TUMOR LYSIS SYNDROME TLS occurs primarily in patients with rapidly dividing cells who present with large tumor burdens (high WBC. PICU transfer is warranted. If severely anemic. Lo-phos. kayexalate 1-2 mg/kg/day divided Q4-6 hours PO (See also Fluids and Electrolytes in handbook) Dialysis if develops fluid overload or recalcitrant hyperkalemia For severe hyperuricemia. DIC is the most common tumor lysis consequence. Uric acid. Volume status may require CVP. massive organomegaly. may use Rasburicase (Elitek®) 0. Frequency of monitoring labs depends upon the severity of lab abnormalities. hyperphosphatemia with associated hypocalcemia. especially when anti-tumor therapy is necessary quickly. Allopurinol 10 mg/kg/day divided Q8 hours Aluminum hydroxide (Basogel) for prevention and treatment of hyperphosphatemia. It may occur at diagnosis or upon initiating therapy.5 and 7. Treatment   Strict I & O’s and daily weights Hydration at 2400-4000 cc/m2/day with fluids containing sodium bicarbonate 50 mEq/liter to maintain a urine pH between 6.3 mg/kg by inhalation. Ca. furosemide 1 mg/kg. stop alkalinization NOTES        . In severe TLS. U/A If there is abnormal involvement or elevated creatinine. hyperkalemia. In AML. The most common abnormalities include: hyperuricemia.1-0. Phos. Many others may occur. obtain ultrasound of abdomen to rule out renal leukemic infiltration or ureteral obstruction. Renal irradiation if obstruction or infiltration is present and renal dysfunction occurs For hyperkalemia: albuterol 0. Evaluation   STAT BMP. especially APML. large mediastinal or other tumor mass).15 mg/kg daily for one to five days If hyperphosphatemia or hypocalcemia are more severe than hyperuricemia.

000 Children >15 kg=1 pheresis pack Wait one hour to check platelet count. . Calculations Simple transfusion  Vol cells (mL)= Est. blood volume x desired change in hct Hct of pRBC Or 10 mL/kg of pRBC will raise hgb approx 3gm% Wait four hours to repeat hgb/hct Exchange transfusion for severe anemia and CHF  Exchange vol= Est. blood volume x desired hgb rise 22gm/dL-HgbR Where HgbR=hgb(initial) +hgb(desired) 2 PLATELETS A platelet pheresis is a single donor product. Host Disease (GVHD): All oncology patients and newborns are at risk for transfusion induced GVHD. Cytomegalovirus: All oncology patients and newborns are at risk for transfusionrelated CMV. All cellular blood products should be irradiated before transfusing. SPECIAL CONSIDERATIONS   Prophylaxis against Graft vs. 55 gm of hgb.174 Transfusions RED BLOOD CELLS RBCs are usually transfused in the form of packed RBCs (pRBC). The hct of pRBCs is 66% and the hgb is 22gm%. There are about 250 mL in each unit. you MUST use CMV specific products. The total volume of a platelet pheresis averages 250-300 mL. thus each unit contains approx. Dose: Children <15 kg=10-15 mL/kg will raise platelet count by 50-100. All immunosuppressed patients should receive only leukodepleted products. If leukodepleted blood products are not available.

and antigen matched blood. NOTES .175   Prophylaxis for transfusion reactions Sickle cell disease: Always use sickle-dex negative. send blood for RBC phenotype. If patient has not received pRBCs previously. leukodepleted.

176 Appendices .

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4)(3.178 Quick Calculations Blood Pressure Norms Percentile for age (over 2 years old) <5th 50th 95th Systolic (mmHg) 70 + (2 x age in years) 90 + (2 x age in years) 100 + (2 x age in years) Diastolic (mmHg) 70 + (1.6 (glucose/100 -1) Body Mass Index 13 + age abnormal Body surface area (actual) = square root of [wt (kg) x ht (cm)]/3600 Body surface estimated = [(wt(kg) x 4) +7] / (wt + 90) Fractional Excretion of Sodium FENa = (Urine Na x Serum Cr) x 100 (Urine Cr x Serum Na) Transfusing to a Desired Hemoglobin *Equation assumes 70cc/kg of total blood volume and pRBC hemoglobin concentration of 22 grams/dL pRBC volume (cc) = (Desired HgB – Actual HgB)(wt in kg)(3.5)(3.5 x age in years) 70 + (2 x age in years) Glucose Infusion Rate Rate of infusion mg/kg/min = (%dextrose in soln x 10 x rate of infusion (ml/hr))/ 60 x wt in kg Serum Osmolality Serum osm= 2(Na) + (BUN/2.5.18) For example: A 34 kg Sickle Cell patient with acute chest syndrome has a HgB of 5.5? pRBC volume (cc) = (10.18) = 540cc .8) + (Glucose/18) Adjusted Sodium for Hyperglycemia Adjusted Na = Na x 1. What volume of pRBC should be transfused to increase the patient’s HgB to 10.5 – 5.

If infusion at this rate and concentration causes the patient no distress. It is recommended that antecubital veins be used. then 4ml/kg/hr end of infusion Example: 70kg patient 0. Slowing or stopping the infusion usually allows the symptoms to disappear promptly. fatigue. then 2ml/kg/hr x 30 minutes. and Steven’s Johnson Syndrome (2gram/kg) It is recommended that the infusion of IVIG be initiated at a rate of 0. chills. backache. then Every hour until complete . then 1ml x 70 = 70ml/hr for 30 minutes.5ml/kg/hr. This may reduce the likelihood of the patient experiencing discomfort at the infusion site.5ml/kg/hr x 30 minutes. leg cramps. and vomiting may occur. Kawasaki Disease (2grams/kg). nausea. fever. hemolytic anemia.179 IVIG Administration Protocol         Common inpatient uses include ITP (1gram/kg). the administration rate may be gradually doubled to a maximum rate of 4ml/kg/hr. aseptic meningitis. Less common reactions include anaphylaxis. Premedication with Benadryl and Tylenol may be useful Initiate Infusion: Patient weight = _____ 0. Various minor reactions such as headache. then 1ml/kg/hr x 30 minutes. urticaria. then Every 30 minutes x 2 hours. then 2ml x 70 = 140ml/hr for 30 minutes.5ml x 70 = 35ml/hr for 30 minutes. lightheadness. then 4ml x 70 = 280ml/hr until end of infusion Vital signs Every 15 minutes x 1 hour. and noninfectious hepatitis IVIG should be used with caution if patients with known IgA deficiencies A rate of administration which is too rapid may cause flushing and changes in blood pressure. if possible.

MD Lyanna Yuchongtian. MD Adnan Qadeer. MD 252-7001 252-3886 252-5219 Brookland Community Peds 500 North 12th Street West Columbia. Suite E Leesville. MD Lynn Wilson.. Suite 210 Columbia. Andrews Rd. MD Patrice Chishom. MD Mary Parrish. MD Stuart Hamilton. MD Luke Pruitt. MD 647-1265 647-1266 John Carroll. MD Ragin Monteith. MD . MD Stuart Hamilton. Box 1806 Orangeburg. SC 29212 Sterling Sharpe Pediatrics 4605 Monticello Rd.O. MD Lillie Bates. SC 29203 Phone 932-2200 Fax 932-2225 Physicians Luke Bonnett. MD Philip Mubarak. SC 29003 Carolina Pediatrics 7033 St. MD 376-2838 407-1386 Heather Kruger. SC 29203 Carolina Pediatrics 114 Gateway Corporate Blvd. MD 256-0531 256-0536 765-9052 376-4447 419-2647 Tanya Russo. SC 29115 Lake Murray Pediatrics 448 Old Cherokee Road Lexington. MD 359-8855 359-3384 359-1257 Dwight Reynolds. MD Tom Brady. Suite 100 Columbia. MD Stephanie Waters. SC 29209 Carolina Pediatrics 2113 Adams Grove Rd. MD Ruby Albert. SC 29006 Family Health Center P. MD Heather Kruger.180 Phone Directory Practice Chapin Pediatrics 119 Amicks Ferry Road Chapin. Suite 103 Columbia. MD Pediatric & Adult Care of Batesburg-Leesville 120 West Church St. SC 29169 739-6982 939-1650 Dan Bodison. MD Kimberly Hightower.. SC 29203 Lexington Pediatrics 346 West Butler Street 532-2208 604-0207 531-6900 531-6963 520-5800 531-9629 Charles Kilgore. Columbia. MD Joseph Delaney. SC 29036 Children’s Choice Pediatrics 6108 Garners Ferry Road Columbia. MD Kyle Guyton. MD Katherine Atkinson.

MD Michael Barker. SC 29204 Orangeburg Pediatric Clinic 940 Holly Street. SC 29072 Medically Fragile Children’s Program 5 Medical Park Columbia. MD Randy Colby. Suite 101 Columbia. NE Orangeburg. MD 788-0577 788-4016 788-5760 Susan Claytor. SC 29072 Luberoff Pediatrics 109 Vista Oaks Drive Lexington. MD 536-2725 534-1441 799-9044 799-9241 432-1931 256-8119 432-1176 Pediatric Associates 1165 Hwy. SC 29115 Pediatric Associates 14 Medical Park. MD Douglas Luberoff. Suite100 Columbia. Extension. MD Teresa Baggett.MD Jennifer Soroos. MD Kay Moore. MD 808-0523 358-0615 434-2300 254-2611 Julie Anderson. MD Karen Connelly. MD Christine Sellers. SC 29203 Midlands Pediatrics 2712 Middleburg Drive. MD Ted Kalutz. MD Melissa Mills. SC 29020 Caroline Webber. MD Jerri Lynn Shealy. MD . SC 29203 Palmetto Pediatrics 16 Woodcross Drive Columbia. MD Trey Castles. MD Becky Riley. Suite 100 Lugoff. MD Barry Cabiness. Suite 100 Columbia. MD Constance Yearling. MD Brad Painter. SC 29203 Pediatric Associates 1346 Haile Street Camden. SC 29203 Medical Park Pediatrics and Adolescences 120 Highland Center Drive. MD Sarah Grooms. MD Tracey MacPherson. MD Catherine Miller. SC 29223 438-3311 438-4020 788-4886 788-5020 Palmetto Pediatrics 3250 Harden St. MD Tom Joseph. 1 South.181 Lexington. MD 254-4257 525-7334 Clarence Dollison. MD Sylvia Brook. SC 29212 779-4001 779-4039 252-9458 732-0140 732-0142 732-4848 Nada Hamzy. SC 29078 Palmetto Pediatrics 74 Polo Road Columbia. MD Marguerite Charlton. MD Charlotte Lindler. MD Lloyd Kapp. MD Cara O’Neill. MD Leigh Bartlett. MD Rick Shrouds. MD Thomas Gue. Suite 410 Columbia. MD Debbie Greenhouse. MD Guy Castles.

MD Michael Finch. SC 29070 Pediatrics of Newberry P. Suite 320 Columbia. MD Grant Willard. MD John Rowe. SC 29072 Pediatric Care Center 6614 Augusta Highway Leesville. MD Teresa Buschor. Suite 1B West Columbia. MD 749-0181 749-3229 . MD Ted Dubose. Suite E Batesburg-Leesville. MD Deanne Geurkink. SC 29070 Sandhills Pediatrics 9 Medical Park. MD Patricia Risinger. SC 29229 Sandhills Pediatrics 4568 Sunset Blvd Lexington. SC 29150 407-0704 407-2529 744-9000 744-9004 520-5147 944-9008 520-5150 Laura Lidlie. MD 604-8400 604-8404 405-0220 405-0222 Karl Holtzer. MD 775-6311 775-5620 778-5131 The Kids Group 206 Medical Circle. SC 29072 Sumter Pediatrics 237 Church Street Sumter. MD Sonya Head. SC 29203 358-2370 358-2376 Jim Dewar. SC 29203 William Westerkam. MD 114 Gateway Corp. MD Christie Thomas. SC 29063 Sandhills Pediatrics 110 Summit Centre Drive Columbia. MD Melinda O’Leary. MD Elizabeth Haile. MD Carol Heebner. MD 796-9200 796-9226 212-7130 212-7160 Melissa Arscott.O Box 355 Newberry. MD Tye Whitaker. MD 252-1801 252-1816 252-1825 540-2877 Sandhills Pediatrics 7936 Broad River Road Irmo. Blvd. MD Doug Dodds.182 Palmetto Pediatrics 1404-A West Main Street Lexington. MD Carl Whetsell. Suite 110 Columbia. MD Mark Mitchiner. MD Kristine McCorquodale. MD Bruce Cope. MD Bill Taylor. SC 29169 University Primary Care 2 Medical Park. SC 29108 Tanya Reid. MD James Durant. MD 699-7555 622-3588 532-2877 462-3704 532-5430 Linda Crout Wingard. MD Marc Todd. MD Kevin Wessinger. MD Eileen Walsh. Suite 203 Columbia. MD Earl Bryant. MD Laura Rickenmann. MD Timothy Key. SC 29201 Ridge Pediatrics and Adolescent Center 338 East Columbia Avenue.

SC 29212 ..183 320 Harbison Blvd. Suite 290 Columbia.

SC CHOC CHOC. MD CENTA Medical Group Children’s First Medical Center. MD Hematology/Oncology Infectious Disease James Glasser. MD Dental Clinic Developmental Pediatrics Edward Cheeseman Jr. MD Greta Harper. MD Elijah Adkins. Frederick Piehl.184 Hospital & Other Useful Numbers Physicians 9 East 9 West Abdullah Sakarcan. MD Admissions Amy Richburg Annette Lynn Ashley Lynn Baptist Hospital Bernarda Strauss Black River Health Care Blood Bank Cardiac Diagnostics Cardiology Carla Roberts. Dr Keith PHR Blood Bank Amy Arnold Pediatric Hem/Onc Pediatric Administration ENT Dr Umeh 654-9764 540-1050 434-3855 433-4230 5829 352-1408 2189 434-5952 434-2262 434-3094 434-8606 799-4624 581-5015 434-6979 Clarendon Hospital 748-1040 799-5860 935-5380 USC Dept. MD Genetics George Kotchmar. MD Caughman Taylor.D. M. MD Phone 434-7161 434-7326 434-2394 434-6881 434-7553 540-1157 434-7606 865-4500 799-5390 433-4321 434-7611 434-7401 434-7940 434-3510 434-7387 256-2483 581-5000 434-6155 434-7055 435-5243 434-4603 748-7555 799-5022 434-6567 935-5604 434-6836 434-4555 434-7990 635-5548 434-4649 254-2495 256-4107 799-5390 434-7980 434-4603 434-3533 434-7995 434-4555 434-4666 Pager/Cell Fax 434-6034 434-6041 434-8607 Practice Pediatric Inpatient Unit Pediatric Inpatient Unit Pediatric Nephrology Admissions Dermatology Resident Coordinator Baptist Hospital Genetics Dr Garma. of Ophthalmology Pediatric Surgery Fairfield County Hospital Teacher Otorhinolaryngology Orthopaedic Surgery Infectious Diseases Pediatric Critical Care Pediatric Pulmonology Allergy/Immunology Dental Clinic 434-4599 434-4669 1281 253-6676 3228 352-0582 434-8607 434-3866 434-3094 434-7983 434-4599 434-3855 1547 Pediatric Surgery General Pediatrics . Chester. MD Endocrine Fairfield County Hospital Flossie Harvey Frederick Garner.Resident room Clarendon Hospital Critical Care Dan Brown David Amrol. MD James Stallworth..

M. MD Joey Delaney. MD Kate Clarkson. MD L. B.D. MD Juan Camps. Lab (Chem) Lab (ER) Lab (Histo) Lab (Micro) Lab (Molecular) Lab (Send Out) Lab (STAT) Laura Pirich. MD Julia Ballance.D. MD Lexington Hospital Lib Jennings Lita Middleton Lowrys Pediatrics. M. MD Medical Records Medically Fragile Michael Bykowsy.D. Katie Stephenson. MD Neonatal ICU Neonatology Nephrology 779-4928 434-8251 434-2833 434-5027 434-4555 434-7025 799-5390 434-4629 434-3745 434-3508 254-6391 434-2327 434-2222 434-6710 434-7623 434-4977 434-7609 434-2323 434-3625 935-7543 736-4560 434-8323 791-2000 434-7638 434-4320 581-2400 434-7974 434-7098 227-8161 434-4603 935-5347 434-4652 434-7972 434-7111 434-2300 765-9233 765-1919 254-2495 751-2210 434-7929 434-7151 434-6392 434-3572 654-0159 977-1062 352-0584 7226 654-5885 434-4699 434-3855 434-2834 434-3855 434-4599 434-3855 799-5391 434-3855 434-3094 799-0682 Obstetrics & Gynecology General Pediatrics Urology General Pediatrics Pediatric Surgery General Pediatrics Genetics (Pediatrics) General Pediatrics Clinical Dietitian Pediatric Hem/Onc Neurology 2333 7227 2359 931-9044 2771 2856 352-0784 352-1405 312-0237 434-3094 935-5380 434-8326 1007 434-3855 581-2401 434-2262 434-4669 434-3866 935-5380 434-2262 Pediatric Hem/Onc Developmental Peds Dermatology Neurological Surgery Lexington Hospital Social Worker Pediatric Residency Coordinator Dr Mello Pediatric Cardiology Pediatric Endocrinology Orthopaedic Surgery Pediatric Critical Care Developmental Peds General Pediatrics Pediatric Cardiology Medical Records Allergy/Immunology Midlands Audiology Moncrief Pediatrics Pediatric Rheumatology Neonatal ICU 799-0344 654-5798 751-2014 434-2262 434-6401 434-8607 . MD Lawrence Siegel. MD Kelly Lewis. MD Lee Carson Lenwood Smith. MD Mark McDonald. MD Malaka Jackson. MD Midlands Audiology Midlands ENT Moncrief Natasha Ruth. MD Mark Locke. M. SC Luther Williams. Matt Wienecke. PhD Matt Garber. MD Mark Posey. MD Jason Hawn. Chester. MD Jeffery Ehreth. Mauldin.185 Janice Bacon. RD Kevin McRedmond.

NICU Pharmacist.186 Neurology Neurosurgery NP on call Newberry County Memorial Hospital Newborn Nursery Olga Rosa. Floor Pharmacist. MD Patty Leopard Pediatric Clinic Pediatric ER Pharmacy Pharmacist. MD Sara Lindsey. Assistant Peds Outpatient Clinic Pediatric ER Pharmacy 434-7981 355-9296 654-0158 434-2262 434-3855 434-3855 434-4582 3408 4208 2269 1832 434-7475 777-1117 434-4555 865-4500 434-4300 748-7555 254-1006 434-5989 434-6490 434-4625 434-4603 434-7528 434-3511 434-8971 254-2495 434-1096 895-2273 296-5513 434-7453 642-9204 434-3522 434-4569 774-4500 434-4555 434-7901 434-4599 PICU Poison Control Pediatric Surgery Dr Heath. PICU Pharmacist. MD Robert Holleman. MD Providence NE Psychiatry Pulmonology Rathna Amanarth. MD Shuri Coleman Southside Pediatrics Staci Dupre Stephanie Cox Sumter Family Health Surgery 434-7961 352-1415 276-7570 434-6455 434-3823 395-2200 434-7973 4347945/7020 434-6155 434-6684 434-6421 434-6068 434-3821 Newberry County Memorial Hospital Newborn Nursery Forensic Pediatrics Orangeburg Regional Medical Center Pediatric Cardiology General Pediatrics Admin. MD Robin Stanfield.D. MD Ronnie Neuberg. CBD PICU Poison Control Privthi Reddy. Dr Luther. M. Sharon Kaminer. MD Shaw Air Force Base Sheila Jones. MD Rheumatology Richard Cartie. MD ScottThompson. MD Robert Hubbird. MD Orangeburg Regional Medical Center Ozzie Shuler. Dr Tiffany Social Work Pediatric Residency Admin. Assistant Sumter Family Health 0529 648-3633 3961 434-3855 . Dr Stuck 748-1040 254-2090 434-2262 352-1523 352-0583 1836 954-3925 7225 654-1074 434-8607 434-3866 434-3855 434-3094 434-3855 254-2148 434-2262 895-6080 Pediatric Gastroenterology Pediatric Intensive Care Pediatric Nephrology Pediatric Critical Care General Pediatrics Pediatric Hem/Onc General Pediatrics Otorhinolaryngology Pediatric Cardiology Shaw Air Force Base Pediatrics Pitts Radiology Caseworker Dr Collins.

MD Teen Clinic Tim Livingston. MD Trey Brown.187 Susan Luberoff. Wilson Building Pediatric Neurology Pediatric Pulmonology Library SOM Neonatal-Perinatal Medicine ENT 241-0639 434-4309 . MD William Giles 898-1470 434-4118 434-3796 748-7555 733-3344 434-6392 256-2483 255-3435 434-4123 434-7981 748-1040 ARC. MD USC SOM Library Victor Iskersky.

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