=Q_ Q Study sets, textbooks, questions Science / Medicine / Pediatries Pediatrics - Topnotch Find free textbook solutions Solve tough problems on your own with expert-written solutions for your textbook. Search on Quizlet > Terms in this set (912) BA9286 | NBs, repubtie act? Ashours | NBS shoutd be done after how many hours? IE NBS was done <24 hours it should be repeated 2 weeks old when? can | m1 hycroxytase detcleney Galactose 1 phosphate Enzymes deficient in galactosemia deficiency Galacrokinase deficiency Galactose 4 epimerase deficiency Print Most common manifestatio ‘ Developmental delay Combine PKU? Export L| Flat, bi th wellder EMPEE Monglian spots | at bive gray with Welle! Report seen in sacral area. Pediatrics - Topnotch ips com 259587apedtes onsen ashes! werPeis Tore esha [Ot Cutis marmorata Lacy pattern on the skin similar to cobblestones. ‘Vasomotor response to cold stress, Cephathematoma ‘Subperiosteal bleed. Does not cross the suture tine, Resolves in few weeks. Caput succedaneum Swelling of scalp due to birth trauma, Involves presenting part. Crosses the suture lines. Resolves in few days. Ipsitateral diaphragmatic paralysis Erb duchenne palsy with Cé involvement Horner syndrome Klumpke palsy with TI sympathetic fibers inyolvelement, Hydrocoete ‘Accumulation of fluid in tunica vaginalis. Not later than 9 to 15 months Surgical treatment of undescended testis should be done when? Due to hyperinsulinism, Pectus excavatum Funnel shaped chest Pectus carinatum Pigeon shape chest >42 weeks Post term, AOG? Hypoglycemic LGA, NB is hypoglycemic or hyperglycemic? RDS Ground glass pattern in CXR, Meconium aspiration syndrome MCC of persistent pulmonary HPN? NSAID use during 3rd trimester Contributory factor that leads persistent pulmonary HPN of the NB. Pediatrics - Topnotch ips com 259587apedtes onsen ashes! TR.< 5 maldlidav neaks at 15Pathologic jaundice > 5 mg/dl/day peaks at 15 DB>10% of TB Mother blood type 0, baby type Aor B In ABO incompatibility, what is the blood type mother and baby? Breastfeeding jaundice ‘Type of jaundice, decreased milk intake with dehydration, reduced caloric intake, Breast milk jaundice Type of jaundice, presence of glucoronidase in breast milk. “Ampicillin + 3rd gen cephalosphorin or aminoglycosides, Emperic treatment for neonatal sepsis. CMV AND RUBELLA blueberry muffin rash cMy Most common congenital infection Ganciclovir DOC for cmv Cataract PDA Congerital rubella syndrome MR Deafness Blueberry muffin cMy Chorioretinits Microcephaly. Periventricular calcification, Blueberry muffin ras. Toxoplasmosis, Chorioretinitis. Intracranial calcification, Hydrocephalus Hepatitis, lucR Syphilis Pediatrics - Topnotch ips com 259587apedtes onsen ashes! snuffles. Periostitis of long bone, Hutchinson teethPeis Tore esha [Ot NEC Preumatosis intestinalis Nec MC life threatening emergency of the GITin neonates, Prematurity Greatest RF for NEC. Triad Intestinal ischemi Nec testinal ischemia. Enteral nutrition, Pathogenic organism, 3kg ‘Average weight of the new born 50cm Average birth length of the newborn, 33to35em Average head circumference of the newborn, 6 years old Approximate age where adult HC is attained Mandibular central incisor First tooth to erupt Tre True or false, theres a 10% decrease in birth weight ‘on the first week of life of anew born? 2nd year of life Physiologic anorexia occurs when? 2nd year of lite ‘Complete myelination occurs when? 4 years old VA of 20/20 3 years old All primary teeth errupted at? 3 years old Best time to read poetry. Yellow fever and cholera Vaccines that cannot be given simultaneously Pediatrics - Topnotch ips com 259587apedtes onsen ashes!I dose of BCG at birth, within 12 months. 3 doses of BCG and OPV every ‘4 weeks, (Starting 2 months) 3 doses of Hepa B after birth. (018) dose of meastes vaccing at 9 months. Fully immunized child means? Plus MMR (6 months after measles vaccine) and Hib (9 moths) HLA DRG Polyarticular disease type ofjuvinile RA. HLA TYPE? HLADR8 & DRS Pavciarticular disease type of RA, HLA TYPE? Reiters syndrome Reactive AS would present as? AS Sacroilits in xray. re specific for Lu “antes DA More specific for lupus and reflects the degree of disease activity, Dermatomyositis MC of the pediatric inflammatory myopathies. Reynauds phenomenon Earliest manifestation of scleroderma Kawasaki disease <5yo, Bliateral bulbar conjunctival injection, non purulent, Dry fissured lips, Strawberry tongue, Edema and erythema of the hands and feet in acute phase and periungual desquamation in the subacute phase. Truncal rash, polymorphus, non vesicular, Cervical LAD. Medium sized artery (coronary artery) BY most affect by kawasaki Perineal erythema Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Important PE finding in patient with kawasaki less than Wyo,Fibrosis affecting the dermis and arteries of the Scleroderma lung, kidney and GIT. Kawasaki ‘Vasculitis of all blood vessel esp medium sized arteries Hsp IgA mediated vasculitis of small vessel Hsp MCC of nonthrombocytopenic purpura in children. 2t0 8 yo. Male, Hsp Palpable, raised purpura, red to brown, lasts for 5 to 10 days. Intermittent abdominal pain. Palpable petichae or purpura thatevolve from red to brown that last from 3 to 10 days. Hallmark of HSP. Leukocytoclastic angitis ‘Skin biopsy finding of HSP. IgA mesangial deposition Renal biopsy finding of HSP. Dermatomyolysis 6y0 Female Heliotrophic rash (periorbital violaceous erythema may cross the nasal bridge) Gottron papules (alligator skin like appearance) (ANA with speckled pattern in >60% Tightness, thickening, non-pitting induration involving proximal to the MCP or MTP joints. Scleroderma Sclerodactyly Digttal pitting scars resulting from digital ischemia. Bibasilar pulmonary fioros's not attributable to primary lung disease. Most common esophageal disorder in children of GERD pn all ages Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Coins MC foreign object swallowed by 6 months to 3 years old? Pyloric stenosis MCC of nonbillous vomiting, Hypochioremic metabolic acidosis ‘Acid base balance abnoramality in patient with pyloric stenosis, Pyloric stenosis Firm, olive-shaped mass, post-prandial vomiting. Pyloric stenosis Barium: shoulder sign, double tract sign Ramstedt pyloromyotomy Surgical procedure for pyloric stenosis. Volvulus Bird's beak sign. Inverted U sign, Coffee bean sign, Doudenal atresia Double bubble sign. Billous vomiting, Polyhydramnios, Jaundice, Fallure to recanalize the lumen during 4th and Sth week AOG, Fallure of the cecum to move into RL@ MC type involve in malrotation. Meckels diverticulum Remnant of omphalomesenteric duct whick connects the yolk sac to the gut in the embryo. Meckel diverticulum, Intermittent painless rectal bleeding and brick colored stool. MC congenital Gl anomaly Hirschprung disease Delayed passage of meconivm Chronic constipation, Dec blood flow and deterioration of mucosal barrier leading to enterocolitis. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Hirschprung disease in older patient. (Currarino triad ‘Anorectal malformation Sacral bonevanomalies Presacral masses, Rectal manometry and rectal suction biopsy Diagnostic test for hirschprung disease. Currant jelly stool, Doubles up (legs and knee are flexed with loud crying) Severe paroxysmal colicky pain that recurs at (Osm: 245mmolfL Intussusception frequent intervals with straining efforts. Sausage shape mass in RUG. BARIUM: coiled spring sign. UTZ: doughnut or target sign, Glucose: 75 Standard ORS composition NaS ce 6s 20 Citrate: 10 Acute pancreatitis Sentinel loop. Colon cut off sign. Heus. Blurring in left psoas margin. Perpamereatic extraluminal gas bubbles, Ransons criteria and APACHE Prognostic system for acute pancreatitis in adult Not in children. AUT First clinical evidence of HBV infection. IgM anti HBC Window phase of HBV infection. Marker for active viral replication, HBeAg Inc risk of transmitting HBV. Mi ingle serologic marker of acute HBV Anti H8cag lost valuable single serologic marker of acute Infection, Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Presence of this will confer lower risk of transmitting Anti HBe HBY. hus Microangiopathic hemolytic anemia, thrombocytopenia, uremia Helmet cell Hus Burr celt Fragmented RBCs Most common chronic glomerular disease IgA nephropathy worldwide. 3 ‘Complement decreases in APSGN. Anaphylotoxin and cSa ‘What major noxious products of complement activation are produced after c3 adtivation in APSGN? APSGN Tea colored urine, hematuria Periorbital edema Hypertension, Oliguria. DNAse antigen Best single Ab titer to document skin infection in patient with APSGN, Ten day course of penicilline. Furosemide Img/kg/dose IV. Sodium restriction, ACE inhibitor or CCB, Mangement for APSGN. PCKD Bilateral flank mass. Poter facies. Hus Most common cause of acute renal failure in young children, Docosahexaenoic acid DHA Essential for newborn's functioning of the visual pigment rhodopsin, vsD Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Most commom acyanotic heart disease.Peis Tore esha [Ot Fat Major energy source in breastmilk Perimembranous MC type of VSD. Nyctalopia Initial manifestation of vitamin A deficiency, Left side and PA, What chamber of heart enlarges in VSD? General softening of the bones. Rachitic rosary. Rickets Harrison groove. Winswept groove. Sustolic regurgitant murmur at the lett lower sternal vsb se border, loud and single s2 Rickets Rachitic rosary Left side and PA, ‘Chamber of the heart enlarger in PDA. Niacin Kasal’s necklace tea Mor common ct ofan congeraea Vitamin © Scorbulic rosary Toa Egg shaped cardiac sihouelte. Niacin Pellagra tor RV tap along left sternal border. Systolic ejection murmur radiates atthe back Vitamin 66 Microeytic anemia Truncus arteriosus Failure of development of the spiral septum. Vitamin C deficiency Corckserew hair Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Peis Tore esha [Ot Extreme exogenous obesity in which patient have Pickwickian syndrome severe cardiorespiratory distress with alveolar hypoventlition, TAPVR Snowman sign. Not weight loss but to siow or halt weight gain - weight ‘Aim for the treatment of obesity. maintenance vs In truncus arteriosus what septal defect is always present? 100,0001U Vitamin A dosage for ages 6 to 12 months Descending aorta MC location of COA 200,0001U Vitamin A dosage for age one year and older. Turner's syndrome Congenital anomaly related to coarctation of aorta, 3rd and th week NTD isa failure of spontaneous closure of neural tube during? Hypertension in upper extremities. COA Yo pp Rio notching in children 7yo. Folic acid will prevent the occurance of NTO from 12th week AOS : ‘prevel preconception until? Pulmonic stenosis Associated with congenital rubella, noonan and willam syndrome. (4g OD Folic acid dosage Aortic stenosis Harsh systolic ejection murmur at the Right upper sternal border. Systolic thrill - Suprasternal notch, Anencephaly Failure to closure of the rostral neuropore. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Peis Tore esha [Ot Pulmonary stenosis ‘systolic ejection murmur at the left upper sternal border with radiation to the back Meningocoele Severe form of NID. Brocks procedure Surgery done in pulmonary stenosis. Ussencephaly ‘Absent cerebral convolution and a poorly formed sylvian fissure. Ross procedure Valve translocation from pulmonary valve. Surgery done in aortic stenosis, Uni or bilateral clefts within the cerebral Schizencephal ehaly hemispheres, RHD Aschotf bodies Porencehaly Presence of cyst or cavities within the brain Mitral stenosis Most common valvular involvement in adults. Holoprosencephaly Due to defective cleavage of the prosencephalon and inadequate induction of the forebrain structure, Mitral regurgitation Most common valvular involvement in children with RHD. Pinocytosis CSF is reabsorbed through what process? Mitral stenosis Fish mouth buttonhole deformity, ‘Weak peripheral pulses with narrow pulse pressure Hemontysis. Arachnoid villus cells CSF is reabsorbed by what celi? Mitral regurgitation Systolic regurgitant murmur at the apex with transmission to the left axilla. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!enna. r14AM Peis Tore esha [Ot Setting sun sign Long tract sign Cracked pot sensation or macewen sign Hydrocephalus Chiari malformation (foreshortened occiput) Dandy walker malformation (prominent occiput) LAand RV ‘Chamber enlarge in MS. 'S. Epidermidis infection Major complication of shunts? Wide pulse pressure and bounding water hammer Aortic reguraitation, pulse, Diastolic murmur heard best at 3rd and 4th LCIS, Vigabatrin Treatment for infantile spasm. MP Most common valvular heart disease. Febrile seizure Most common seizure disorders in childhood? Palpitation Most common complaint, MVP. Phenythoin DOC for status epilepticus. ASD Murmur at 2nd LICS with widely split $2. Migraine without aura Most prevalent type of migraine Ps Murmur at 2nd LICS with radiation to the upper back. ‘Tuberous sclerosis Brain tesion consist of tubers located in the covolutions of the cerebral hemispheres at subependymal region that calcified and may produce a candle dripping appearance. COA Murmur at 2nd LICS with radiation to the Interscapular area, Lisch nodules Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Hamartomas located within irisPeis Tore esha [Ot Murmur at 2nd RICS. Tuberous scleros's ‘Ash leaf skin Shagreen path Subungval or periungual fibromas L. monocytogenes vsp Murmur at LLSB, cas Most common cause of bacterial meningitis in Ist 2 E.cotle months of life? cel Murmur at LLSB with radiation to the left anterior wr t ith radiation to the left ant axillary tine. S. Pneumonia MCC of bacterial meningitis in 2mos. To 12 years H. influenzae N. mengitiais PIN Lipman sacks endocarditis, Sterile endocarditis, SLE Echovirus MCC of viral meninaitis. Infective endocarditis Osler nodes. Janeway lesion, Splinter hge. Roth spots. 4to8yo Brain abscess if common in age? Serum somatomedin ¢ (IGF-I) Use to diagnose hyperpituitarism. Cerebrum MC site of brain abscess. Emotional disturbance with motor hyperactivity. Earliest sign of graves disease Pediatrics - Topnotch ips com 259587apedtes onsen ashes! ct and mast canctant sien nf muactheniaProlonged physiologic Jaundice Earliest sign of hypotnyroidism. Myasthenia gravis Proximal muscle weakness, symptomatic late in the day. MC cause of thyroid disease in childrem and Thyroiditis ae adolescent. Thymoma ‘Thought to be the site of Ach receptor antibodies. om MC endocrine metabolic disorder of childhood and adolescent. Edrophonium chloride IV Or Tensilon test A short acting choinestearse inhibitor use for clinical test of patient with guillain barret syndrome. ‘Slight improvement in ptosis and ophthalmoplegia. . Aplastic anemia with microcephaly, microphthalia, hearing oss, imb abnormalities. Not associated wth other cranial neuropathies or raiser dustuneton, Bell's palsy Praise " Occurs after sytem viral infection Paltor Most impt sign of IDA fav Vius associated with bells palsy Hsv Mumps virus Hb hematopoiesis Difrtve clagnostis test for alpha thalasemia Guillain barre syndrome ‘Acute demyelinating polyradiculoneuropathy / symmetric ascending muscle weakness or paralysis ‘Alpha thalassemia Target cell, heinz bodies Gullain Barre Syndrome Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Landry ascending paralysis.Sickle cell disease * inc erythropoiesis cusing marrow expansion and new bone formation. Crew cut or hair on end appearance in skull radiograph. Tendon reflex and lower extremities Last to resolve in gullain barre syndrome. Sickle cell disease Howell jolly bodies. Erythromycin 40-S0mg/ka/day xladays Used to eradicates nasopharyngeal carriage of diphtheria. Idipathic thrombocytopenic purpura MCC of thrombocytopenic purpura in childhood. purpura Direct contact via Airborne MOT of diphtheria respiratory droplets Fever Anemia Thrombocytopenia Thrombotic thrombocytopenic Renal dysfunction Nervous system changes Inc bun and crea. Deficiency in metalloproteinase (ADAMTS-13). DHA Essential for new borns functioning of the visval pigment, Arachidonic acid Linotenic acid Omega 3 DHA Linoleic acia Omega é Pinocytosis Process of CSF reabsorption Close contact via aerosol ‘drops Pediatrics - Topnotch ips com 259587apedtes onsen ashes! MOT pertusis,Peis Tore esha [Ot Desmopressin and factor 8 concentrate Treatment for von willebrand disease. Pertusis Explosive outburst in seres of Sto 10 rapid coughs in one expiration and ending in ahigh pitched whoop (forceful inspiratory gase) often associated with suffusion of face or popping out of eyes and vomiting Well in between paroxysm Have petechial hge on the upper body. Astrocytoma MC posterior fosaa tumor in chiloren, Leukomoid reaction Blood picture with pertusis. Craniopharyngioma MC supratentorial tumor in ehitaren, Erythromycin 40-50mg/ka/day 6 xlddays DOC pertusis Wims tumor ‘Abdominal mass that does not midline. Person to person through infected droplets MOT meningococcemia. Neuroblastoma ‘Alsdominal mass that crosses the midline. Meningococcemia Fever. Morbliform rash > petechial rash > purpurie rash >echymotic rash Osteosarcoma Sunburst pattern Pen G 250k-400k IV divided ‘doses for atleast Sto7 days DOC meningococcemia Ewing sarcoma ‘Onion skin, moth eaten appearance, Rifampicin Prophylaxis for meningococcemia, Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Peis Tore esha [Ot mg/kg/day x14-Zidays Salmonella Rose spots Diaphysis Ewing tumor, what part of long bone? Blood culture Mainstay of diagnosis for salmonella. ue Barking cough Chloramphenicol 50-75 DOC thypoid fever suo sace Location of 18 eto tnpeponnens aero dart ah Acute patos Thumper tat sl orl ay ot meades us steese son Measis bray desquanaton Supragiottic Location of acute epiglottitis. Vitamin A. Vitamins given on complicated measles. Acute epigtotitis Muffled voice, drooling. German measles/rubella Bday fever Rsv Most common cause of brochiltis. German meastes/rubella Forschelmer spot Chlamydia Staccato cough Congenital rubella syndrome Blueberry muffin skin lesion Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Peis Tore esha [Ot svt Most common significant pediatric cardiac arrhythmia, Congenital syphilis Mulberry molars Adenosine DOC for SVT. Congenital syphilis ‘Olympian brow. Higoumenakis sign, Saber shin Hutchinson teeth Saddle nose. Rhagade. Clutton joint Crigater naljar syndrome Gilberts syndrome Familial nonhemolytic unconjugated hyperbilirubinemia Meningoencephalits Most frequent complication of mumps. Dubin johnson syndrome Rotor syndrome Inherited conjugated hyperbilirubineria Roseola suppresses what cellular lineage within al bone marrow? Spiramycin Maternal treatment for toxoplasmosis. Roseola Nagayama spots - uvulopalatogtossal junction omy Paraventrieular caeifieation. Congenital VZV. Interruption of limb development al 6 to 12 weeks AOG. ‘eye and brain involvement in 16-20 weeks AOG. Congenital syphillia Snuffles. Within 24 hours the onset of rash Pediatrics - Topnotch ips com 259587apedtes onsen ashes! When is the best time to give acyclovir in VZV.Peis Tore esha [Ot Cox Ale HFMD causative agent, Radiograph of left hand and wrist Use to measure the bone age-reference standards. CoxA, Herpangina causative agent Sexval maturity ‘Skeletal maturity is linked to? Sexual maturity or chronological age? Erythema infectiosum Sth disease Whey Dominant protein in human milk Erythema infectiosum Slapped cheek appearance Casein Dominant milk in cows milk, Erythroid cell line Primary cell line affected by erythema infectiosum. Wiskott-aldrich syndrome Eczema Thrombocytopenia Recurrent infection Decreased IgM Tand B cell deficiency Eryhthema infectiosum Lacy, reticulated appearance rash, sparing the palm. and soles without desquamation. Chronic granutomAtous disease Prolonged detachment of the umbilical cord. Roseola bth disease Gianotti crosti syndrome ‘Associated with IM. ‘symmetric rash on the cheek. Erythematous papules. omy Most common congenital infection Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Peis Tore esha [Ot Cettuitis Tender, warm, erythematous plaques with ill defined borders. Nikolsky sign Mild rubbing of the skin results in epidermal ‘separation leaving a shiny, moist, red surface. Stratum corneum Malassrzia furfur invades what layer of epidermis? Sarcoptes scabiei Causative agent of scabies. Scabies Brown crusted nodules on the trunk; s-shaped burrows Molluscum contagoisum White or yellow I-4mm discrete papules with a central umbilication, Psoriasis Thick silvery scales, nail involvement and isomorphic phenomenon, Erythema multiforme ‘Oval or round, fixed, red skin lesionwith dusky central zone. Target lesion, Iris lesion, No systemic signs and symptoms. Due to HSV specific host response. HR Fetal CO depends on? 10-15 hours Functional closure of DA. 2-3 weeks ‘Anatomical closure of DA. Inc in oxygen saturation ‘Strongest stimulus for constriction of the ductal smooth muscle. COstium secundum Most common ASD, Right side and PA ‘Chamber enlarge in ASD. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!‘The significance of the lead cord clamping is to? Decrease the incidence of anemia and prevents brain hemorrhage Essential newborn care (ENBC) }. Immediate drying- prevents hypothermia, 2, Interrupted skin to skin contact (60-90 mins)- prevents hypothermia, increases colonization with protective family bacteria and improves breast- feeding initiation and exclusively 3. Delayed cord clamping after 1 to 3 minutes- decreases anemia and prevents brain hemorrhage. 4, Breast-feeding- with in first hour of life prevents, 191% of all neonatal deaths. Hypothermic in the newborn may lead to the following, Acidosis, hypoglycemia, hypoxemia, increased renal ‘excretion of water and solutes The target temperature for newborn is? 36510 375 The following are the most Convection, conduction, heat radiation, Classification of prematurity based on birth weight and gestational age ‘common mechanisms of heat evaporation. loss in the immediate newborn: Birthweight: Low «2500 Very tow 1500 g Extremely low < 1000 g Gestational age (preterm) Late =/> 34 and <37 weeks Very <32 weeks Extremely «28 weeks Small for gestational age aka intrauterine growth retardation (IUGR) BWis < 10 percentile for calculated gestational age, Growth of the fetus is affected by fetal, uterine, placental and maternal factors. (See pg. for IUGR table) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Large for gestational age (LGA) BW of 90th percentile for gestational age. Maternal diabetes and obesity are predisposing factors, at risk for hypoglycemia, polycythemia, RDS, congenital cardiac defects, lumbosacral agenda is, hyperbllirubinemia, birth injuries. The most important ‘component of the Apgar score Respiration ‘The best description of the Assesses neonates in need of resuscitation. Apgar score Rapidly assess the need to resuscitate. It may reflect how will the infant is responding to resuscitation, A low one minute score does not correlate with ‘Apgar score outcome. A five minute score is a valid predictor of neonatal mortality Newborn screening test Done at 48 hours old. If blood is collected « 24 hours old repeat at 2 weeks. For preterms, ideal time should be at 5 to 7 days old RA #9288 Expanded newborn screening Covers 28 diseases Newborn screening tests Congenital hypothyroidism, congenital adrenal hyperplasia, galactosemia, phenylketonuria, G6PD deficiency, MSUD Most common cause of congenital hypothyroidism Thyroid dysgenesis (aplasia, hypoplasia, ectopic gland) Earliest physical sign of congenital hypothyroidism |. prolonged jaundice 2. delayed tooth itruption Treatment of congenital hunnthurniticen Pediatrics - Topnotch ips com 259587apedtes onsen ashes! LevothyroxineCongenital adrenal hyperplasia Autosomal recessive disorder of cortisol biosynthesis Deficiency in cortisol results to increase corticotrophin ACTH resulting to adrenal cortical hyperplasia and overproduction of intermediate metabolites Most commonly deficient enzyme in cortisol deficiency (CAH) 2 hydroxylase enzyme deficiency (90%) Hyperplasia (CAH) treatment Congenital Adrenal Normal at birth but signs of sexual and somatic Hyperplasia (CAH) precocity appear within the Ist 6 months of life. Lab findings: Hyponatremia, hyperkalemia, hypoglycemia Congenital Adrenal Hyperplasia (CAH) Elevated 17 hydroxyprogesterone (normally ‘elevated in the first 2 to 5 days of life and in sick preterm infants) 1. Glucocorticoid replacement - Hydrocortisone 15 to 20 mg/m?/24 hours dally administered orally in 3 divided doses 2, Mineralocorticold replacement for salt wasting Congenital Adrenal - Fludrocortisone 3. Surgical management 4, Prenatal treatment - Dexamethasone suppresses androgens and prevents the virlization if given by 6 weeks AOG Galactosemia Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Autosomal recessive disorder; caused by lack of ‘enzyme needed to convert milk sugar galactose Into glucose. ‘An absolute contraindication to breast-feeding ‘Causes congenital cataract. Increased risk for €, coll Neonatal sepsis. Treatment: lactose free milk Lona-lerm seauelae even with treatment: Ovarian3 distinct enzyme deficiencies in Galactosemia 1. Galactose I phosphate uridyltransferase deficiency (GALT)- classic form 2, Galactokinase deficiency (GALK)- cataracts 3 Galactose 4 epimerase deficiency (GALE)- Iron deficiency anemia Most common cause of anemia in children Cotrimoxazole Sulfonamide ‘Trimethoprim-suifamethoxazole combo Prophylaxis for pneumocystis pneumonia Cannot be given for G6PD deficiency G6PD deficiency Xdinked recessive Episode or chronic hemolytic anemia, Immune to malaria because G6PD deficiency has problem in pentose phosphate pathway. Low NADPH leads to hemolytic anemia (free radicals) with bite cells and heinz bodies, Fava beans, sulfonamides, dapsone, nalidixic acid, nitrofurantoin, vit k analogs, aspirin, benzene, naphthalene, chloramphenicol, antimalarials Phenyiketonuria (PKU) What enzyme is deficient? Phenylalanine hydroxylase— byperphenylalaninemia Most common manifestation of PKU without treatment is? Developmental delay (gradval) PKU Unpleasant musty odor Maple Syrup Urine Disease (sup) A genetic disorder characterized by the inability to metabolize branched-chain amino acids [Deficiency In branched amino acids (Leu, Lys, Val)]: symptoms Include a maple syrup smell in the urine, MSUD (Maple Syrup Urine Disease) Genetic metabolic disorder resulting from the detective activity of the enzyme branched-chain alpha-keto acid dehydrogenase complex. MsuD Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Leucine- causes brian damage Isoleucine- accumulates & causes maple syrup odorVernix Caseosa Waxy or cheesy" white substance found coating the skin of newborn, Usually absent in post term infants. ‘Cutis Marmorata (mottling) ‘The purplish reticulated pattern noted on the skin of neonate with exposed to cold. This is transient & physiologic. Mottling of the skin w/ venous prominence. Cobblestone, Lacy appearance Mongolian spots SLATE-BLUE well-demarcated areas of pigmentation over the BUTTOCKS and BACK. Erythema Toxicum ‘Small, white locationally vesiculopustular or papules: ‘on an erythematous base develop after Io 3 days contains EOSINOPHILS. Postular melanosis Vesiculopustular eruption over a dark macular base ‘around the chin, neck, back, and soles. contains NEUTROPHILS. Epstien pearls ‘Cysts on the hard palate which is composed of accumulations of epithelial celts. Milia Pearly white papules seen mostly on the chin and. around the cheeks Neonatal acne ‘Open and closed comedones or inflammatory pustules and papules on the cheeks of the baby usually after a week from delivery. Nevus simplex Salmon patch/stork bite Pink macular lesions on the nape, glabella, upper ‘eyelids, or nasolabial region. Lanugo Used for Ballard scoring Hair that covers the skin of preterm infants. Seen on. term infants around the shoulders. Papal benediction sign, ape hand Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Median nerve injuryWrist Drop (loss of extension) Radial nerve injury after mid shaft humeral fracture or use of crutches, accompanied by deep brachial artery damage Loss of posterior sensation (arm/forearm/hand) Claw hand Ulnar nerve injury (C8-T) Foot drop, Peroneal nerve injury Esophageal atresia Congenital absence of part of the esophagus. Food ‘cannot pass from the baby's mouth to the stomach, Feeding exacerbates the symptoms, causes regurgitation & precipitates aspiration, Manif: Frothing & bubbling at mouth & nose, cough, cyanosis, respiratory distress Dx: Inability to pass an NGT or OGT EA with TEF Most common EA (87%) EA with double TEF and EA with proximal TEF Least common (both 1%) Esophageal atresia types AL EA with TEF 87% B- Isolated EA 8% C- Isolated TEF 4% D- EA with proximal TEF 1% E- EA with double TEF 1% Congenital Diaphragmatic Hernia (CDH) opening in the diaphragm through which abdominal contents herniate into the thoracic cavity during prenatal development -results in some degree of pulmonary hypoplasia, determined by the timing and size of the herniation -degree of pulmonary hypoplasia determines ‘outcome Bochdalek hernia Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Most common CDH SCAPHOID ABDOMEN Herniation through embryonic plevroperitoneat canal. Neonates present with large hernias withGastrischisis More common on the RIGHT side of umbilicus Necrosis of intestinal mucosa and possible Necrotizing enterocolitis perforation, Risk factor Triad: J. Intestinal ischemia or injury 2. Enteral nutrition 3. Pathologic organism - E.coli, clostridium, Klebsiella, Necrotizing enterocolitis Triad 1. Thrombocytopenia 2. Persistent metabolic acidosis 3 Severe refractory hyponatremia CRP. Diagnostic and prognostic Primaturity- most important risk factor Necrotizing Enterocolttis ‘Ampicillin + gentamicin + metronidazole treatment Surgery if with pneumoperitoneum What is the name for fated recanalization of the Duodenal atresia ‘duodenum resulting in polyhydramnios, bile- containing vomitus, and a distended stomach? Double bubble sign Duodenal atresia A preterm baby won't stop Necrotizing enterocolitis ‘crying. Developed abdominal distention with abdominal ‘erythema. Diagnosis? Coagulation necrosis Histologic findings in necrotizing enterocolitis Pneumatos's intestinalis Thickened bowel walls and air in the bowel wall. Accepted interventions for Broad-spectrum antibiotic therapy, IV volume necrotizing enterocolitis replacement, surgery. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!A day old male neonate being referred for vomiting, ‘The vomitus is greenish yellow- Ish in color according to the mother. PE revealed a nondistended, nontender abdomen. What is the most likely finding on work up? DOUBLE BUBBLE sign- Duodenal atresia Pyloric stenosis narrowing of the opening of the stomach to the ‘duodenum. Increased pyloric thickness. Intussusception telescoping of the intestines DONUTRING sign Lung surfactant Decreases surface tension in alveoli Present by 20 weeks AOG. ‘Appears in amniotic fluid between 28 and 32 weeks AOG. Mature levels of pulmonary surfactant are present usually after 35 weeks AOG. Phosphatidyicholine (lecithin) Most abundant tung surfactant. Accounts for 65% of glycerophospholipids in surfactant. Cessation of breathing for > 20 seconds or for any Apnea ‘duration if accompanied by cyanosis and bradycardia. More common in preterm infants. Apnea Most common cause is idiopathic apnea of prematurity ‘Apnea management Stimulation, PPY, CPR, Methylxanthines (caffeine or theophylline) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!A 23-year-old primigravid gave birth at 32 weeks AOG to a male newborn via SVD. Several minutes after birth, the newborn developed to tachypnea, prominent grunting, Intercoastal and subcostal retractions, nasal flaring which progressed to cyanosis. Upon auscultation, breath sounds were diminished with the harsh to tubular quality and on deep Inspiration, fine crackles were heard, Which of the following Is. ‘your initial diagnosis? Respiratory distress syndrome (RDS) - needs positive pressure ventilation - caused by surfactant deficlency RDS chest x-ray findings Find reticular granularity of the parenchyma, and ait bronchograms (ground glass appearance) 23-year-old primigravid gave birth at 38 weeks AOG via CS. toamale newborn, Several minutes after birth, the newborn develop tachypnea, retractions, expitatory grunting, Upon auscuitation, the lungs are clear without crackles or wheezes, Which of the following is the initial iagnosis? ‘Transient tachypnea of the newborn Primary cause of transient tachypnea of the newborn Slow absorption of fetal ung fluid Transient tachypnea of the newborn chest x-ray findings Prominent pulmonary vascular markings, fluid in the intralobar fissures, overaeration, flat diaphragms. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!‘A 32-year-old GAPS gave birth toa 42 week male neonate via SVD after 18 hours of labor. The neonate was noted to have aspirated meconium. 12 hours after birth, the neonate was noted to have grunting, nasal flaring and intercostal retractions. He was also tachycardic and was hypoxemic at 60% O2 sat. After drawing blood from the right radial artery and umbilical artery, a PaO2 gradient was noted. Which of the following is the intial diagnosis? Bronchopulmonary dysplasia Primary cause of bronchopulmonary dysplasia Persistence of the fetal circulatory pattern of right to left shunt thing through the PDA and foramen ovale after birth. Bronchopulmonary dysplasia chest xray findings. Normal chest x-ray Diagnostic test for bronchopulmonary dysplasia Real time echocardiography with Doppler flow Hyaline membrane disease CXR: Finely granular lungs (ground glass appearance) UNDERaeration ‘Transient tachypnea of the newborn (CXR: Prominent pulmonary vascular markings, fluid Lines in fissure OVERaeration Bronchopulmonary dysplasia CXR: Bubbly lungs (cystic Lucencies) OVERaeration Free radical damage to lung early n life before lungs are mature enough to handle pure oxygen Meconium aspiration syndrome Pediatrics - Topnotch ips com 259587apedtes onsen ashes! CXR: Coarse streaking granular pattern on both lung fieldsNeonatal pneumonia (CXR: PERIHILAR streaking OVERaeration Grunting A sign of impending respiratory distress or failure. Forced expiration against a partially closed glottis. Persistent Pulmonary Hypertension of the Newborn (PPHN) Respiratory disease resulting from persistence of the fetal circulatory pattern of right-to-left shunting of blood away from the lungs and through the ductus arteriosus and patent foramen ovale Bhutani chart For neonatal jaundice T2mg/dl and above - Pathological Ismg/dl and above - High risk (tx: phototherapy) 25mg/dL and above - Needs direct volume ‘exchange transfusion Elevated direct bilirubin °2) ‘Always pathological (think of TORCH) CT scan (computed tomography) Diagnostic modality that can confirm hyperbilirubinemia Intracranial calcifications Caused by: Toxoplasmosis CT scan: Most likely seen in a patient with hyperbilirubinemia of 15 mg/dl. and above Bilirubin encephalopathy Keemicterus) ‘The most serious complication of hyperbilirubinemia in the newborn Physiologic jaundice ‘The most common cause of jaundice in neonates. Normal newborn physiology, no other ‘manifestations, appears after 24 hours. Jaundice visible only on the 2 to 3 day. Pathologic jaundice Result of an underlying disease appearing before 24 hours (or persistent after day 7) Appears on the first 24 to 36 hours of fe. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Jaundice risk factor in neonatal hyperbilirubinemia J+ Jaundice visible on the Ist day of Ufe ‘A. Assibling with new needle jaundice or anemia U- Unrecognized hemolysis/UDP-glucuronyl transferase deficiency N- Non-optimal feeding D- Deficiency of G6PD I+ Infection, immaturity, infant of diabetic mother C- Cephalohematoma or bruising, central hematocrit » 65% (polycythemia) E- East Asian, Mediterranean, Native American heritage Kernicterus (bilirubin encephalopathy) Levels >25 mg/dl. Neurological syndrome caused by UNCONJUGATED bilirubin depositing in basal ganglia and Brainstem, 3 phases or clinical manifestation Kernicterus: Phase I- Poor sucking, stupor, HYPOtonia, seizure Phase 2- HYPERtonia, opisthotonos, fever Phase 3- HYPERtonia ‘ABO incompatibility Most common cause of hemolytic disease of the newborn, Hemolytic disease that occurs when the mother’s blood type is O and the newborns A, B, or AB ‘ABO Incompatibility lab test results: Positive direct Coomb’s test Spherocytes in blood smear Normal hemoglobin or low as 10 to 12 g/dL Increased reticulocyte count in 10 to 18% Increased B1 (20 mg/dL in 10 to 20%) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Rn incompatibility, D antigen ‘A complication of pregnancy in which a MOTHER who is Rh NEGATIVE carries an Rh POSITIVE FETUS; under certain conditions, antibodies in the mother's blood can attack the fetus's red blood cells. Rare in frst pregnancy. Injection of anti-D gamma globulin (RhoGAM) into the mother immediately after the delivery of each RH positive infant reduces Rh hemolytic disease. RH Incompatibility lab test results before treatment Positive Direct Coomb’s test Anemia Increased reticulocyte count Bi rises rapialy in the Ist 6 hours of life B2 may also be elevated Blueberry muffin rash/syndrome Congenital rubella syndrome Congenital Rubella Syndrome Classic triad: congenital cataracts (white pupils), sensory-neural deafness, and patent ductus arteriosus (could be pulmonary artery stenosis as well) Could potentially also result in microcephaly and mental retardation. ‘non-pregnant females should be vaccinated with the live MMR vaccine prior to pregnancy. Prematurity Birth before 37 weeks gestation, ‘The most important risk factor that predisposes a neonate to sepsis. Congenital infections (TORCH) Hsv Mc Hsv2 Vesicular lesions on the face and mouth ‘Congenital infections (TORCH) Rubella Purpuric hemorrhagic lesions all over the body Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Congenital infections (TORCH) cmy Chorioretintis, Imaging; Periventricviar calcifications Congenital infections (TORCH) Toxoplasmosis Chorioretinitis, microcephaly, hepatosplenomegaly Imaging: Intracerebral calcifications ‘Congenital infections (TORCH) Varicella ‘Cutaneous scars Imaging: cortical atrophy Genitourinary anomalies |. Hydrocoete- accumulation of fluid in the tunica vaginalis, majority or non-communicating, resolves. by 12 months ol 2. Hernias- usually inairect inguinal hernias, presents as a reducible scrotal swelling, Undescended testes Cryptorchialsm Majority descend simultaneously during the first 3 months of Ute. If the testes does not descended by & months, it will remain undescended. ‘Treated surgically not later than 9-15 months old. Regular or periodic follow-up of weight and height ‘The most practical and pertinent guide in evaluating nutritional status in children, IgA Colostrum is produced over the first 2 days of lactation. The secretion is higher protein and lower fat and lactose then mature human milk. Likewise, it Is especially rich in_ Whey to casein ratio in mature human milk ratio 32 6 months You can feed breastmilk solely to a child unti this ‘month “Breast-feeding is best for 2 years and beyond. Sett-regulation by infant Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Feeding in the first six months of life = Initiated as soon as after birth (within I to 4 hrs) * there is no need to feed infants every time theyBreast milk 1. Foremilk- watery- high lactose, high protein 2, Hindmilk- creamy- high fat (Sx the fat of foremilk) Stage of lactation 1.Colostrum- 0 to 7 days, Low fat and low carb 2. Transitional mit 3. Mature milk- 10-14 DOL, Colostrum, high fat & lactose 4 Involutionary milk GERD definitive de: Esophageal pH probe Esophageal pH monitoring of DISTAL esophagus - document acidic refiux episodes Infant with galactosemia Absolute contraindication to breastfeeding Fat soluble vitamins in breast mi Vitamin A, DE, K Vitamin A. In breast milk 90% Components of retail pigments for bone & tooth devt Vitamin A deficiency Nyctalopoa, photophobia, xerophthalmia, keratomalacia, 8ITOT spot Vitamin D Depends on maternal vitamin D status (maternal sunlight exposure) Vitamin D deficiency Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Children only before fusion of the epiphyses. RICKETS in children and OSTEOMALACIA in adults CRANIOTABES- softening of the cranial bones. Hypocalcemic tetany Widening of costochondral junctions leads to RACHITIC ROSARY “HARRISON GROOVE" horizontal depression along the lower anterior chest Windswept deformity (VALGUS DEFORMITY of I leg. vw/ virus deformity of the other leg) ‘Te STOSS THERAPY (vit D 300,000-600,000 1U orally or IM as 2-4 doses over 1 day) followed by 400 1U/day as a multivitamin Adequate dietary calcium & phosphorousVitamin € Antioxidant Protects PUFA from oxidative damage Vitamin E deficiency 1. RBC hemolysis in premature infants 2. Loss of neural integrity 3. Posterior column and cerebellar dysfunction 4, Pigmentary retinopathy Vitamin kK Prothrombin formation, coag factors 2,7, 9,10 (1972) In breast milk: 01-04 if/100m| & is higher in colostrum than mature mk Vitamin K deficiency Hemorrhagic disease of the newborn or Vitamin K deficiency bleeding (VKDB) VKDB w/ breastfeeding Is due to low overall intakes ‘of vitamin K in the frst week of life than low vit k content of breast milk. VKDB Early VKDB: "CLASSIC HEMORRHAGIC DISEASE" Occur at 114 days of age Late VKOB: Occur at 2-16 weeks of life (some up to 6 mos) Third form: Maternal medication (warfarin, phenobarbital, phenytoin) Micronutrient deficiency Zinc, essential fatty acids, Niacin (pellagra vit 83), biotin (it B7), vit A, vit C Page 13, Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Water soluble vitamins in breast milk B1 (Thiamine): ketoacid decarboxylation, BERIBERI B2 (Riboflavin): FAD coenzyme, ANGULAR, CHEILOSIS (perteche), GLOSSITIS (magenta tongue) BS (Niacin): NAD coenzyme, PELLAGRA, DERMATITIS, DIARRHEA, DEMENTIA (3 D's) B6 (Pyridoxine): mother’s tong term high estrogen (OCP use, MICROCYTIC ANEMIA, chellosis, glossitis, dermatitis B7/BH (Biotin): ALOPECIA, NEURO symptoms, dermatitis, HYPOtonia 89 (Folate) & B12 (Cobalamin): coenzyme for 5- ‘methyl tetrahydrofolate formation. Vitamin B 12 deficiency leading to severe and persistent your logical damage. MEGALOBLASTIC ANEMIA, PERIPHERAL NEUROPATHY Scurvy Vitamin C deficiency Generalized tenderness especially in the legs, pseudoparalysis, low-grade fever, irritability, tachypnea, anorexia. SCORBUTIC ROSARY at the costochondral junction and depression of the sternum, ‘CORKSCREW HAIR, bluish spongy welling of mucus membranes ‘Angulation of the scorbutic beads is sharper than the rachitic rosary, X-ray: distal ends of long bones with a ground glass appearance Te 3-407 orange/tomato juice Vit ¢ 100-200mg orally or parenterally months Iron and Zine supplement should be started at what month? Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Vitamin D deficiency Bowleg/knock knee, caput quadratum, craniotabes, RACHITIC ROSARY, pigeon chest, HARRISON GROOVE Vitamin C deficiency SCORBUTIC ROSARY / BEADS Vitamin B3 (niacin) deficiency ‘CORN madalas kinakain will cause this deficiency PELLAGRA: 4 D's- Diarrhea, Dermatitis, dementia, and death, CASAL NECKLACE, glove & boots lesion Vitamin B2 (riboflavin) deficiency PERIECHE Vitamin A deficiency BITOT SPOTS, xerosis conjunctivae, xersosis cornea Vitamin A TOXICITY Hyperostosis, absence of metaphyseal changes Zine A deficiency of this trace element is associated with skin concerns, reduced immune response and hypogonadal dwarfism Hypervitaminosis A Irritability, pruritus, painful extremities, with Brawny swelling, coarse hair, dry skin, seborrhea and Increased intracranial pressure pseudotumor cerebri Antibody production In children with malnutrition, the most seriously ‘compromised immunologic function is? Undernutrition The greatest risk occurs in the first 1000 days (0 to 24 months) Malnutrition Covers under nutrition to overweight PROTEIN DEFICIENCY A child prone to diarrhea, underweight, has EDEMA Kwashiorkor face or lower ext, ABDOMEN PROTUBERANT, ‘sparse hair with the reddish streaks, dermatosis, poor appetite, FATTY INFILTRATION OF LIVER A. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Severe acute malnutrition (SAM) Defined as severe wasting and or bilateral Edema Severe wasting is extreme thinness diagnosed by weight or length or height below -3 standard deviation of the WHO child growth standards. Refeeding syndrome Excessive carbohydrates will ead to surge of insulin Leaving to hypokalemia, hypo. Athena, hypomagnesemia, Hallmark: SEVERE HYPOPHOSPHATEMIA, Sox Serum phosphate levels of = or < 0.5 mmol / liter Dysregulation of caloric intake and energy Obesity pathogeness 'Y pathos expenditure a measure of body weight relative to height BMI BMIF wt (kg) / nt (m2) Obesity Excessive accumulation of body fat Obesity in childhood The leading cause of pediatric hypertension Associated with type 2 DM, coronary Heart disease and low self-esteem Total cholesterol level »200 ma/a. Pickwickian syndrome ‘Obesity Hypoventialtion Sydrome, often accompanies OSA Rare complication of extreme exogenous obesity in which patient some Cardio respiratory distress with, alveolar hypoventilation and a decrease in pulmonary, tidal and expiratory reserve volume Medication for childhood obesity Orlistat an intestinal lipase inhibitor decreases fat absorption for *12 years old Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Preventive pediatrics Immunization Ultimate goat: eradication of cisease. Immediate goat prevention of disease. 2 types: 1) active immunization 2) passive immunization Active immunization administration of antigens so patient actively mounts a protective immune response Types of antigen for active immunization: 1) tive attenuated virus or bacteria (weaken) 2) Inactivated vaccine (killed microorganisms) Inactivated vaccines contain killed pathogens, which induce a weaker Immune response Hepatitis 8, OPT, H influenza B, pneumococcal, hepatitis A, meningococcal, influenza trivalent, HPV, typhoid fever IM, rabies, inactivated polio vaccine Live vaccines ‘Antigenic preparations containing weakened attenuated) microbes; typically such vaccines are more antigenic than killed preparations, Weakened (attenuated) organisms used to stimulate cell- mediated immunity and create long term protection BCC, measles, MMR, varicella, rotavirus, influenza attenuated intranasal, typhoid fever oral, oral polio, Japanese encephalitis, dengue vaccine Exceptions to vaccines To live vaccines simultaneously. But if not giving in ‘one day, wait for 4 weeks to give the other one. Contrainaications to vaccine ‘Anaphylactic reaction & encephalopathy due to another identifiable cause occurring within 7 days aller pertussis (decelluiar) vaccination 2 temporary, indications to live vaccines but not with inactivated box means Pregnancy & immunosuppression Transplacental transfer of maternal antibodies Example of naturally acauited passive immunity Pediatrics - Topnotch ig qu con2095907 aed ences)Meningococcemia Rifampicin, Ceftriaxone, Ciprofloxacin (adults) Diptheria All close contact regardless of immune system Give booster if none within last 5 years Erythromycin 40-50ma/ka/d or Benzathine Pen Measies Post Exposure Prophylaxis IM: choice of site is based on the volume of the Injected material and the size of the muscle. Children younger than 1 y/o: anterolateral aspect of, thigh (largest muscle & preferred site) ‘Older children: deltoid muscle for IM injection Upper, outer butt Not for active immunization, can damage sciatic nerve. Measles MC complication: 1) Otitis media 2) Diarrhea Measles MCC of death: Pneumonia lron supplementation avoid antacid, tetracyclines and dairy, absorbed better on empty stomach but not tolerable (Grapefruit juice aids in absorption) Supramental zine therapy Zine 10 to 20 mg per day for 10 to 14 days ‘Adeyear-old child is currently being treated with Cefuroxime for pneumonia but is not improving, If you suspect that he has mycoplasma infection, you expect him to improve with? ‘Azithromycin Growth and development Newborn: 0-28 days Infant & toddler: °28 days to 23 months Children: 2-11 y/o Adolescents: 12-18 y/o Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Teeth w of teeth: Age in months - 6 Begins at 6 months Suspect thyroid disorder is no teeth by 15 mos. All20 primary teeth have erupted by 3 years old, Central mandibular incisors Erupts first Objective assessment: weight, length, head Growth circumference Ave, Weight of newborn: 3 kilos (6 tbs) 2-5 months When does the posterior fontanel close? Lifts head when prone, tracks past midline, alert at months ts head when prone, tracks past midline, alert, coos, social smile es month Rolls front to back, grasp rattle, laughs and squeals, -5 montis looks around and laughs Sits with pelvic LESS support, transfer objects, 4 months babbies without association, stranger anxiety oo month Cras, cruising, immature pincher grasp, mama dada, waves bye-bye, object permanence ‘Walks alone, two finger pincher, 1-3 words, follows 12 months ner * fone step commands, separation anxiety Handedness 3 years old Up to dy/o- Gis Bed wetting Pte tu Up to 5 y/o- Boys Language explosion 2yjo Parachute reflex ‘Among the following reflexes, which normally persistent about tife? Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Moro reflex (startle reflex) Sudden loud noise will cause symmetric abduction land extension of the arms followed by adduction ‘and fl exion of the arms over the body. Disappears. by 3to4 months, Elicited by supporting the infant in a semi-erect correct position and then allowing the Infants head to fall backwards onto the examiners hand. 1 Normal: symmetric extension and abduction of the fingers and upper extremities, followed by flexion of the upper extremities and an audible cry. = Absence on one side (Asymmetric response) Rule ‘out brachial plexus injury, fractured clavicle, shoulder dystocia or hemipares's 1 Absence on both sides (No response): Rule out spinal cord or brain lesion. 1 Older infant: Persistence of Moro reflex abnormal Rule out brain pathology. Grasp response A reflexive action that occurs when the fingers or Interior surface of the hand (the palm) are stimulated and they immediately begin to close around the stimulus, Elicited by placing a finger in the open palm of each hand. 37 weeks of gestation, strong enough that they can lift the infant from the bed with gentle traction, Tonic neck reflex (fencer position) elicited by turning an infant's head to one side infant extends arm and leg on that side and flexes arm and leg on the opposite side birth to 3 to 4 months Parachute reflex Slightly older infants, evoked by holding the infants, trunk and then suddenly lowering the infant as if she were falling, The arms will spontaneously extend to break the infants fall This reflex is a prerequisite to walking. Landau and parachute reflex ‘Age of disappearance is covered by voluntary action Pediatrics - Topnotch ips com 259587apedtes onsen ashes!‘Autism Spectrum Disorder (asp) Encompasses: autistic disorder, asperger disorder, childhood disintegrative disorder, rett disorder, pervasive developmental disorder NOS Autism Spectrum Disorder (ASD) ‘Characterized by deficits in 2 core domains: ). Deficits in social communication and social Interaction 2. Restricted repetitive patterns of behavior, Interest, and activities Attention-deticit/nyperactivity disorder (ADHD) Dsmv: ‘Age of onset: Several inattentive or hyperactive Impulsive symptoms were present prior to age 12. ‘A comorbid diagnosis with ASD is now allowed. Neurodevelopmental disorder defined by Impalring Levels of inattention, disorganization, and or hyperactivity impulsivity. Cholelothiasis in children Usually due to hemolytic dyscrasia Direct Cholangiography Bilary atresia GOLD standard for dx? Determines presence & the site of obstruction, dx for cholestatis jaundice? Neonatal hepatitis, biliary atresia, Alagille syndrome Neonatal cholestasis Neonatal cholestasis: Elevated CONJUGATED bilirubin, markers and magnitude vary, Prolong elevation of the serum levels of CONJUGATED bilirubin beyond the first 14 days of life. “Physiologic jaundice - usually resoves in two ‘weeks and is classically unconjugated bilirubin Biliary atresia (* Noneystic ‘obliterate cholangiopathy’) MC form (85%)- obliteration of the entire ‘extrahepatic biliary tree at or above the porta hepatis, Liver transplant Definitive tx of Bilary atresia Tlanovlar cord sion I Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Biliary atresiaKasai procedure Procedure done for treatment of biliary atresia in which a segment of the small intestine is used to form a ble duct. Rotavirus The most important global cause of infantile gastroenteritis viral gastroenteritis in infants. Acute diarrhea Does NOT ast longer than 14 days Acute diarrhea Excessive loss of fluid and electrolytes in the stool. Sudden onset of excessively loose stools of > JOmL/kg/day in infants and > 200 g / 24 hours in ‘older children, which lasts < 14 days. Cholera Te fluids & electrolytes DOC: Tetracycline ALT: Doxycycline, Tmp-Smx Erythromycin, Furazolidone Invasion of intestinal epithelium Pathophysiology of BLOODY aiarrhea in SHIGELLA, Bone marrow Best specimen/highest yield for sample collection In typhoid fever Enteric Fever (Typhoid Fever) Salmonella typhi MC MOT: Ingestion of food or water contaminated. with S. Typhi from human feces Enteric Fever (Typhoid Fever) High grade fever, hepatosplenomegaly, maculopapular rashes (ROSE SPOTS) Enteric fever complications Intestinal hemorrhage & perforation is infrequent in children, If with perforations: RLQ pain, tenderness, vomiting, features of peritonitis Chronic carrier of enteric fever ‘Those who excrete S. Typhi for > 3 months Enteric fever bx Ceftriaxone: safest to give pag wala pang culture. Ciprofloxacin: 2nd safest to give. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Shigeliosis (Dysentery) Virutence trait: Ability to invade intestinal epithelial cells. Patho changes occur in colon M cells overiying the peyer patches, S. Dysenteriae serotype I produces SHIGA TOXIN (@ protein synthesis-inhibiting exotixin causes HUS). Shigellosis symptoms Severe abdominal pain, high fever, emesis, anorexia, generalized toxicity, painful defecation, urgency Shigellosis PE findings Abdominal distention and tenderness, hyper active bowel sounds, tender rectum on digital exam What to expect in Shigeliosis Significant dehydration Watery voluminous diarrhea intially evolving into frequent small volume, bloody mucoid stools, Shigellosis DDx (See CC ¥) Salmonella, Entero invasive E. coli (EIEC), Entamoeba histolytica Campylobacter jejuni Clostridium difficile Yersinia enterocolitica Shigellosis Dx Presumptive diagnosis: fecal leukocytes 50 to 100, PMN/HPF & fecal blood, increased WBC In CBC Definitive diagnosis: Culture of stool and rectal swab specimen (MacConkey agar, xylose-lysine deoxycholate, $$ agar) Use ctinical judgment in the management of clinical ‘symptoms consistent with shigellosis Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Shigellosis Te Cirpoftoxacin 3 days ang isagot Nelsons: Cefixime 8 ma/ka/day PO every 12 hours for 5 days. Ceftrixone 50 mg/kg/ day IV or IM once aday for $ days or ‘Azithromycin 12 mg/kg/day PO Ist day followed by ‘6 mg/ka/ day for the next 4 days. All px with BLOODY diarrhea Ciprofloxacin 30 MG/KG/day in2 divided doses Zine 20 MG/day for Vs days improves immune response 11/2 male baby was brought into the elinie due to crying spells accompanied by knee flexion, There are intermittent ‘episodes that he feels well. The patient reportedly has been vornited once during an ‘episode of abdominal pain, voritus consists of previously ingested food and sometimes clear watery fluid, There is poor appetite. Which among the following should be asked? Diagnosis: INTUSSUSCEPTION (intestinal obstruction) ‘Ask for any blood in the stool Initia diagnostic test of choice: UTZ of the abdomen (ook for DONUT RING SIGN Intussusception Common in kids with CF. Obstruction may cause fecal emesis, CURRANT JELLY/STRAWBERRY JAM stools. enema---resolution*bowel movements TELESCOPING of a segment of intestines Intussusception ‘Occurs when a portion of the alimentary tract is telescoped into an agjacent segment. Intussusception Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Most common cause of intestinal obstruction between 3 months to 6 years old. More common in male Correlated with adenovirus.Intussusception clinical manifestations Severe paroxysmal colicky pain that recurs at frequent intervals with straining efforts, legs and knees are flexed with loud crying, 60% of infants pass currant jelly stool (strawberry jam) Child looks well in between the paroxysms of pain, Intussusception diagnostic tests: Plain abdominal x-ray Barium enema: COILED-SPRING sign Abdominal UTZ: DONUT RING sign or TARGET sign Intussusception treatment Diagnosed by xray, ultrasound, air or barium contrast enema, which sometimes reduces the ‘obstruction without need for surgery Surgery is indicated when barium or air contrast ‘enema does not reduce the intussusception (OPEN LAPAROSCOPIC CHOLECYSTECTOMY) Pyloric stenosis ‘A two month old boy was noted to have vomiting every feeding for the past week There were no ‘other symptoms noted. On physical exam: irritable, slightly tachycardic, no retractions, clear breath sounds, (+) firm, movable. 2.x 2.¢m mass on the abdomen, What is your impression? Pyloric stenosis narrowing of the opening of the stomach to the due Pyloric stenosis diagnosis Contirmatory: Ultrasound (pyloric thickness >4 mm ‘or length >14 mm) Barium: Elongated pyloric channel, a buldge of the pyloric muscle into the antrum (SHOULDER SIGN) and streaks of barium in the narrowed channel (DOUBLE TRACT SIGN). Duodenal atresia ‘What is the name for fated recanalization of the ‘duodenum resuiting in polyhydramnios, bile- containing vomitus, and a distended stomach? Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Duodenal atresia Due to failure to recanalize the lumen during the 4ith-Sth week of gestation ‘Obstruction usually DISTAL to the ampulla of vater. May have other congenital anomalies (Trisomy 2). Duodenal atresia clinical manifestations Hallmark: BILIOUS vomiting WITHOUT abdominal distention on the Ist DOL, Polyhydramnios in 50%- due to failure of absorption ‘of amniotic fluid in DISTAL intestine, JAUNDICE in 1/3 if px. Duodenal atresia Plain abd xray: DOUBLE BUBBLE sign due to distended & gas-filled stomach & proximal ‘duodenum ‘Tx NGT/OGT decompression w/ IV fluid replacement; surgery (Duodenostomy with gastrostomy tube) Duodenal atresia ‘8.48 hour old boy born 34 weeks AOG was noted to vomit GREENISH materia after every feeding, Physical examination: awake, alert, no respiratory distress, clear breath sounds, distinct heart sounds, non-distended abdomen with hyper active bowel ‘sounds what is your impression? Hirschsprung disease absent ganglion cells in submucosal/myenteric plexus rectosigmoid ‘A S-year-old boy presents with chronic constipation since he ‘was an infant You find a boy ‘who looks active and playful His abdomen was globular, nontender and rectal exam revealed a normal sphincteric tone and no feces on the ‘examining finger. What is your most likely impression? Hirschsprung disease Most affected in this case: Rectosigmoid colon (0%) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Hirschsprung disease (congenital aganglionic megacolon) ‘The most common cause of lower intestinal ‘obstruction in neonates. M>F Absence of meissner & averbach plexus Anganlionic segment limited to the rectosigmoid (e0%), Arrest of neuroblast migration from the proximal to distal bowel- absence of ganglion cells in the bowel wall beginning in the internal anal sphincter. Hirschsprung disease genes RET genes on chia! & EDNRB gene on chl5q22 Hirschsprung disease GOLD standard diagnostic tool: RECTAL SUCTION, BIOPSY Hirschsprung disease MC symptoms: at birth- Delayed passage of meconium Some- chornic constipation Hirschsprung disease treatment Temporary colostomy and wait until the infantis 6 to 12 months old to perform definitive repair Definitive procedures: Swenson, Duhamel, Boley endorect pull-through via Laparoscopy Meckat’s diverticulum Outpouching of distal ileum Mecke'’s diverticulum Rule of 25:2 inches long, 2 feet from the leocecum, 2 years old on presentation, in 2% ofthe population, 2 times more common in females, 2 types of common ectopic tissue (gastric, pancreatic ete) ‘embryonic duct origin Functional constipation ROME CRITERIA: Infants to 4 y/o: 1 month of at least 2 criteria must be met = 4-18 y/o: 2 or more criteria (w/ insufficient criteria for irritable bowel syndrome) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Constipation Delay or difficulty in defecation present for 2 weeks. or longer and causes distress to the patient. Usually starts after the neonatal period, Encopresis is ‘common. Encopresis a childhood disorder characterized by repeated defecating in inaporopriate places, such as one's clothing (at least once a month for 3 consecutive months once) at least 4 y/o. lactose intolerance Osmotic diarthea Cholera Secretory diarrhea Foreign body aspiration (airway) emergency order, CXR neck Xray (differentiate croup, epiglottts), Bronchoscopy after CXR, cefazolin, prednisone (reduce edema and infection), Foreign body ingestion (esophagus) can be a cause of stridor in children Caustic ingestion Ingestion results in esophagitis, perforation & stricture formation Liquid alkali = Liquefactive necrosis Acidic agent = Coagulation necrosis (bitter taste so less ingested) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!‘Acute pancreatitis Most common pancreatic disorder in children MCC: blunt abdominal injury then mumps/viral int Auto-digestion Lecithin-> phospholipase A2 —> toxic Lysolecithin Pain increases in intensity for 24-48 hrs w/ vomiting CULLEN SIGN GREY TURNER SIGN Serum LIPASE SENTINEL LOOP (dilated prox jejunal (oop in LUG) CUT-OFF SIGN (lated transverse colon) (CT scan better than abd UTZ ‘TX: NPO, TPN, antibiotics, analgesics Ranson's criteria & APACHE score: inappropriate for children (for adults only) Inatin Best measure of determining GFR ASPHN Decreased C3 Immune complex mediated disease Hematuria Blood in the urine (> § rbes/hpf centrifuged) RBC casts formed when they enmesh in protein matrix False positive: urine pH >8 (aikaline) & H202 False negative: Formalin & High urinary ascorbic acid Acidic urine Uric acid crystals Alkaline urine Phosphate crystals, Normal specific gravity of urine 1015 to 1.025 Falsely high specific gravity Glucose, abundant protein and with iodine containing contrast materials Grossly red urine (with or Pediatrics - Topnotch ips com 259587apedtes onsen ashes! From the lower urinary tractBrown colored or tea colored urine Glomeruiar in origin Proteinuria >! 00 MG/DL on dipstick, RBC casts, ‘dysmorphic RBCs (ACANTHOSIS) Cloudy urine Maybe normal, due to Crystal formation at room temp Urinary tract infection (UTI) ‘The most common cause of gross hematuria in pediatric population 5 to 12 years old Prior strep infection: “Throat- ASO *Skin- anticdeoxyribonuclease B APSGEN oe NEPHRITIC syndrome | to 2 weeks after throat infection 3 to 6 weeks after skin infection Most common postinfectious renal disease in APSGN Pe childhood Resolves and normalize by: “Acute phase: 6 10 8 weeks “Urinary protein excretion and hypertension: 46 APSGN yer ve weeks *Microscopic hematuria: Ito 2 years *CBlevel: 6 to 8 weeks Type ill hypersensitivity Antibody is produced and combines with a Pathogenesis of PSGN Circulating antigen unrelated to the kidney then IC accumulate in glomeruli and activate the ‘complement system which causes immune injury Lowcs-c9 APSGN Normal Cl, C& HIGH ASO Pediatrics - Topnotch ips com 259587apedtes onsen ashes!What major noxious products ‘of complement activation are produced after C3 activation? 1) Anaphylatoxin-increases vascular permeability 2) CSa- release substances that damage cells and basement membranes End effect: inflammation DNAse B antigen Best single antibody tighter to document SKIN (cutaneous) infection (APSGN) ‘The only causes of renal insufficiency and that cause decreased C3: 1. PSGN 2.MPGN, 3. Lupus nephritis, Urine dipstick test Negative Trace = 10 to 29 MG/DL Je = 50 MG/DL 2+= 100 MG/DL 3+ = 300 MG/DL de => 26/01 Urine dipstick postive if *> trace in a sample with $G <1. 01 if 1018 To know if proteinuriais evident 24 hour urine protein: more accuratel! Nephrotic range >4Omg/m2/hr Urine proteinscreatinine ratio. >2is nephrotic range proteinuria Causes of transient proteinuria Fever, exercise, dehydration, cold exposure, congestive heart failure, seizure, stress Fixed proteinuria Indicates potential glomerular or tubular disorder Infection ‘The major complication of nephrotic syndrome Bacterial peritonitis ‘The most frequent type of infection (in nephrotic syndrome) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Pneumococcus (Streptococcus pneumoniae) The most common organism causing peritonitis. + Alpha-Hemolytic Streptococcus; lysed by bile; sensitive to Optochin Minimal change disease (lipoid nephrosis) (anephrotic syndrome) LM = normal glomeruli EM - Foot process effacement Often postinfectious Most common in children. Responds to corticosteroids. MCC of childhood nephrotic syndrome, features podocyte fusion and loss of negative charge in glomerular BM -> selective loss of albumin, Minimal change disease Treatment, prednisone, cyclophosphamide for resistant ccases/frequent relapse Subepithetial humps on EM Immune complex humps in acute post- streptococcal glomerulonephritis ‘The site of pathology as seen in the electron ‘glomeruionephritis Subepithetiat microscope on membranoproliferative ‘lomervionephritis (MPGN) Lt defuse thickening of glomerular capilary wall EM: sub epithelial deposits of electron dense Membranous material I granvlar IgG and C3 SPIKE AND DOME APPEARANCE Minimal change disease LM- normal glomeruti EM. defuse effacement of epithelial foot process (foot process effacement) Selective loss of albumin, not globulins, due to GBM polyanion loss. Membranoproliterative Glomerulonephritis (MPGN) Pediatrics - Topnotch ips com 259587apedtes onsen ashes! LM: large, hypercellular glomeruli Increased ‘mesangial matrix, OOUBLE CONTOUR or TRAM TRACK APPEARANCEFocal Segmental Glomerulosclerosis (FSGS) LM: focal segmental SCLEROSIS and HYALINOSIS EM: loss of foot process (podocyte foot process effacement), epithelial denudation Associated with HIV, heroin use, sickle cell disease, african americans E.Coli Most common cause of UTI in children Bacteria in UTI E.coli, Klebsiella, Proteus Three basic forms of UTI Pyelonephritis, cystitis, asymptomatic bacteuria Pyelonephritis ‘The most common and serious bacterial infection in younger than 24 months, ‘Abdominal pain, back pain flank pain, fever, malaise, nausea, vomiting, diarrhea Fever may be the only manif ‘Acute hemorrhagic cystitis E.Colo Interstitial cystitis (painful bladder syndrome) Irritated voiding symptoms relieved by voiding. Negative urine culture, Adolescent girls, Idiopathic, Cystitis Inflammation of the urinary bladder. Limited to lower urinary tract. Dysuria, urgency, frequency, suprapubic pain, incontinence, malodorous urine, Does not cause fever and does not result in renal injury ‘A symptomatic bacteriuria Positive urine culture without any manifestation of Infection, Most common in girls. Does not cause renal injury except in pregnant women. Bacteriuria accompanied by physical signs of UT! (dysuria, suprapubic discomfort, hematuria, fever) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!True regarding the diagnosis of UTIin chitaren “Urine culture is necessary. “In tollet trained children, midstream urine sample is vusvally satisfactory, “In children who are not toilet trained, a catheterized or suprapubic aspirate should be ‘obtained, > 50,000 colonies of single pathogen from uml suprapubic tap or catheterized sample OR 10,000 colonies + symptoms Bag sample, positive urinalysis, symptomatic and Presumptive UTI Seem’ Pi me single organism with culture colony count » 100,000 Hemolytic Uremic Syndrome (Hus) Triad: RAT Renal failure (renal insufficiency), anemia (microangiopathic hemolytic), thrombocytopenia Thrombocytopenic Purpera cre) Pentad: FAT RN Fever, anemia (microangiopathic), thrombocytopenia, renal failure, neurologic E. coli O1S7H7 (VTEC) The most common ideologic agent for HUS A nine-year-old boy presents with a five day history ‘of abdominal pain and diarrhea. One day PTC, he had bloody stools. He is admitted for IVF replacement and further work up. After four days, his abdominal pain subsided but the urine is grossly bloody and he looked pale. His blood spear showed fragmented RBCs. Microangiopathic changes Schistocytes, burr cells, helmet cells (on peripheral blood smear) Hus MC form of HUS is due to toxin-producing E. Coli ‘Asia & Southern Afrixa: The shiga toxin of Shigella dysenteriae type 1 Western countries: Veratoxin-producing €. Coli (VTEC) 01577 Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Hallmark of HUS Microvascular injury with the endothelial cell damage Hus Onset is preceded by gastroenterts Sudden OLIGURIA after 5-10 days after the initial -gastroentertis/respiratory illness Peripheral smear: helmet cells, burr cells, fragmented RBCs Do NOT give platelets and antibiotics. Microanglopathic anemia RECs are damaged when passing through obstructed or narrow vessel lumina curs: Chronic dse, Lead, IDA, Thalassemia, Sideroblastic Hemoglobin F Predominant Hg found in the fetus/newborn until 6 months old Breastfeeding jaundice Expected in the first 8-12 weeks. Explained by the down regulation of EPO prod, Nadir Hab levels rarely falls between 100 mg/l Physiologic anemia of INFANCY ‘A harmless condition in which the hemoglobin level ‘drops in the first 6 to 8 weeks after birth (lasts 8 ‘2months), then reverts to normal levels, NV hgb: 1 g/t Physiologic anemia of PREMATURITY More extreme and rapid decline in Hb Minimal levels up to 7-9 g/dl Iron deficiency anemia MCC of anemia in children MICROCYTIC HYPOCHROMIC rhe Pallor, pica (paint chips), pagophagia (ice), neurocognitive defects (ireversiole) IDA Pediatrics - Topnotch ips com 259587apedtes onsen ashes! High: ROW &TIBC Low: Retic count, FerritinAnemia Micracytic: MCV <80 Normocytic: MCV 80-100 Macrocytic: MCV >100, Microcytic anemia ‘Thalassemia, IDA, lead poisoning, sideroblastic J serum iron, { total ron-binding capacity (TIBC), and normal or 1 ferritin Notmocytic anemia ‘Anemia of chronic disease, uremia, HYPOthyroidism, bone marrow failure (aplastic anemia) Macrocytic anemia Folate and Vit BI2 deficiency, drug & alcohot Induced anemia ‘due to folate or vitamin 812(cobalamin) deficiency (megaloblastic anemia), THF-M —-> VitBI2-M —> Methionine, (transfer of methyl groups) Iron deficiency anemia (IDA) The most common hematologic disease of infancy and childhood. Most common in 9 to 24 months of age. Most common causes poor dietary intake. Pallor (blanching or pale color) ‘The most important SIGN (hgb 7-8g/d) in ron deficiency anemia (IDA) Max dose: 150-200 ma/day Expect a rise in Hb= 01 to 0.4 g/dl /day Response to Iron Therapy 12-24 hrs: Subjective improvement 26-28 hrs Initial bone marrow response 48-72 hrs: Reticulocytosis 4-30 days: Increasing hgb levels J-3 months: Repletion of iron stores Thalassemias Inherited microcytic hemolytic anemia due to abnormal due to hemoglobin synthesis Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Alpha Thalassemia Decrease in alpha globulin synthesis common in ‘Asians and African-Americans Silent: asymptomatic Thalassemia trait: mild anemia Hemoglobin H disease: splenomegaly, Increases BA Hydrops fetal: incompatible with lite Alpha Thalassemia Decreased retic count, microcytic hypochromic rbc, TARGET CELLS & HEINZ BODIES on PBS Definitive dx: HBG ELECTROPHORESIS ‘Tx Deferoxamine (chelation tx) or splenectomy Beta Thalassemia, Due to decrease in betaglobin chain synthesis or absence of beta chain, Common in people of Megiterranean origin and SEA descent. Definitive Dx: hemoglobin electrophoresis (increase HOF, HbA2) ‘Te aggressive transfusions, chelation therapy as needed, hydroxyurea, splenectomy “Beware of Yersinia Enteroltica infection which eats up the free iron! Beta thalassemia major (COOLEY ANEMIA) No beta globin production. Anemia of 6 months {dectine in normal hemoglobin F and rise in hemoglobin A), Spleno megaly, extra medullary hematopoiesis Beta thalasseia minor Asymptomatic carriers, mild or no anemia Hemolytic anemia Results from a decrease in RBC survival Common findings or pallor, jaundice due to Increased indirect bilirubin, increased LDH. NORMOCYTIC, NORMOCHROMIC RBC, increased reticulocyte count on PBS Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Intrinsic RBC defects, autoimmune destruction,Hemolytic anemia due to intrinsic RBC defects Spherocytosis - defect in membrane spectrin or ankyrin Causes {oss of biconcave shape then cells will be more fragile than cells will be trapped in the spleen and destroyed. ‘Autosomal dominant Hemolytic anemia ‘Osmotic fragility test = Confirms the presence of fragile sphere-shaped RECs MCHC increased, MCV normal, high reticulocyte count, increased indirect Bilirubin ‘1% splenectomy and folate supplementation Sickie Cell Disease (SCD) Episodes of painful crisis due to hypoxic tissue Injury from microvascular occlusions “inherited, autosomal recessive disorder characterized by abnormal hemoglobin S (HS) in the erythorocyte: stiffen & elongate sickle shape ‘due to low oxygen level -identiied during infancy or early childhood “incurable disease -predominant in African Americans “lin 400 births -never free from pain but learn to tolerate lin 400 births Sickie Cell Disease (SCD) ‘CREW CUT (sites of extra medullary hematopoiesis) or HAIR ON END (also seen on any anemia or Thalassemia) appearance on radiograph Sickie Cell Disease (SCD) Sickle cells and HOWELL JOLLY BODIES on PBS Definitive Dx: hemoglobin electrophoresis (HbS) Ankle Common site of bleeding in hemophilia Idiopathic Thrombocytopenic Purpura (ITP) Pediatrics - Topnotch ips com 259587apedtes onsen ashes! disorder marked by platelet destruction by macrophages resulting in bruising and bleeding from mucous membranesHemophilia, ‘An X-linked recessive disorder in which blood falls to clot properly, leading to excessive bleeding if Injured, Normal platelet count, 87 & PT Prolonged PPT Ex. Swelling of bilateral knees and hematoma of the thighs with eechymos’s Von Willebrand Disease The most common hereditary bleeding disorder ‘The most common and most serious congenital Hemophilia A coagulation factor deficiencies 5 ‘The most common hereditary hypercoagulable disorder Prolonged bleeding The hallmark of hemophilia Ankles The earliest joint hemorrhages in children Management of choice for von Willebrand disease Desmopressin ADH agonist Von Willebrand disease Result of a quantitative (type 1 or 5) or qualitative (type 2) detect in VWF YW Isa large proteins made by endothelial cells and megakaryocyte, a carrier for factor 8 and is a cofactor for platelet achesion. Von Willebrand disease Bleeds profusely when teeth are brushed Von Willebrand disease diagnosis Low: VWF and factor 8 High: PTT Prolonged 8T ‘Abnormal platelet adhesion Ristocetin cofactor assay Von Willebrand disease Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Mild bleeding in type |: desmopressin,Most children with Leukernia present with: Unexplained fever, pallor, hemorrhage ALL acute lymphocytic leukemia The type of leukemia in childhood that has the best response to chemotherapy ‘Acute Lymphocytic Leukemia (aL The most common malignancy in children over all. Result from leukemic expansion and crowding out of the normal marrow, The single most important prognostic factor in ALL TREATMENT Three of the most important protective factors in ALL 1. Age of the patient atthe time of diagnosis 2. Initial leukocyte count 3. Speed of response to treatment (most important) ALL Sites of relapse: bone marrow, CNS (Increased ICP isolated CN palsies), testes (painless swelling) Spread to: Liver, spleen, lymph nodes Poor prognostic factors: <10 years old, Male, WBC >100,000 u/L, Presence of CNS leukemia and mediastinal mass ‘Wilma Tumor (nephroblastoma) Renal tumor of embryonal origin ‘The second most common malignant abdominal tumor in childhood (2 to 5 years old) Associated with: Nevrofibromatosis, Beckwith-Widemann syndrome, WAGR syndrome Wilms tumor (nephroblastoma) Painless abdominal enlargement with blank mass that does NOT cross the midline, hematuria, hypertension. Most common site of Mets: LUNGS Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Cotrimoxazole ‘A9-year-old gitl is recelving chemotherapy for ALL. The drug of choice for PCP prophylaxis is? Embryo tumor of neural crest cell origin, 3rd most common pediatric cancer CROSSES midline. Differential diagnosis of small, round, bive cell tumors: (WARMER PN) Neuroblastoma Mainstay be Surgical resection Most common site of Mets: BONES Diagnosis: Bone marrow aspirate * urine BMA and HVA (NEUROBLASTOMA) ‘Wilms tumor, acute leukemia, rhabdomyosarcoma, ‘mesothelioma/medulloblastoma, ewing sarcoma, retinoblastoma, primitive neuroectodermal tumor (PTEN), neuroblastoma Neuroblastoma associated with: N-mye oncogene, neurofibromatosis,hirschsrpung, disease, tuberous closes, pheochromocytoma Neuroblastoma Ssx: Reflect the tumor site and extent of disease, Crosses midline, nontender abd mass, Horner ‘syndrome, hypertension, cord compression Prognostic factors: age at diagnosis, stage of disease, SHIMADA histology. Younger age more favorable prognosis, Most supractavicuiar lymphadenopathy are? Lymphoma Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Lymphomas Neoplasms of lymphoid cells (lymphocytes & histiocytes)- 6% of childhood cancers. ANN ARBOR STAGING CLASSIFICATION HODGKIN vs NON-HODGKIN Painless, nontender, firm, rubbery cervical or supraclavicular adenopathy B symptoms: impt for staging (weightloss over 3 ‘mos, unexplained high fever, drenching night sweats) Reed-Sternberg cells, Hodgkin's Lymphoma Aplastic anemia anormocytic-normochromic type of anemia characterized by the failure of bone marrow to produce red blood cells Results from failure or autoimmune destruction of myeloid stem cells causing pancytopenia, Aplastic anemia PBS: hypocellularity & pancytopenia Confirmatory dx: bone marrow biopsy (hypocellular bone marrow with a fatty infiltrate) Fanconi anemia ‘Autosomal Recessive: Pancytopenia (aplastic anemia), Microcephaly, microphthalmia, hearing oss, hypoplastic thumb and radius, hyperpigmentation, abnormal facial features Leukemia (ALL) Most common malignancy Brain tumor (kids) Most common solid tumor Infratentorial: medulloblastoma (cerebellum), pilocytoma (GFAP) Supratentorial: craniopharyngioma (cerebrum) ~ rathke's pouch Mauwahlasioma I Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Most common soli timer outside CNSRhabdomyosarcoma Most common soft tissue tumor Brain (PNET) Malignancy with highest mortality Seizures/convulsions Paroxysmal, time-limited changes in motor activity, and/or behavior that result from abnormal electrical activity in the brain. Seizure disorder ‘A general term to include any one of the following: epilepsy, febrile seizures, seizures secondary to metabolic, infectious, or other etiologies Epilepsy This order of the brain characterized by an enduring predisposition to generate seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition, Two or more unprovoked seizures occurring in the timeframe of > 24 hours. Epileptic syndrome This order that manifests one or more specific. seizure types and has a specific age of onset and prognosis, Epileptic seizures The end point of hyper or hypo natremia Focal seizures (partial seizures) Limited to part of one cerebral hemisphere Simple partial seizures Focal seizures WITHOUT Impairment of consciousness Complex partial seizures Focal seizures WITH impairment of consciousness Generalized seizures Clinical and £26 changes indicate synchronous Involvement of all or both hemispheres Febrile seizures Six months to six years old, expect normal neural EEG, normal neurological PE Genetic epilepsy "Idiopathic epilepsy" Structural/matahalic anitaney | Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Unknown epilepsy “Cryptogenic epilepsy" Benign epilepsy syndromes with focal seizures “"Rolandic seizures* Most common type is benign childhood epllepsy with Centro temporal spike Benign epilepsy with occipital spikes Panayiotopoulus type “early Childhood “complex partial seizures with ictal vomiting Gastaut type *Late childhood “Complex partial seizure, Visual auras, migraine headaches Early infantile epileptic encephalopathy “OHTAHARA SYNDROME” Manifest tonic seizures and is usually caused by brain malformations or syntaxin Binding protein } mutations Severe myoclonic epilepsy of infancy “DRAVET SYNDROME" Starts as vocal febrile status epilepticus or focal febrile seizures and later manifest myoclonic and other seizure types West Syndrome (Infantile Spasms, Infantile Myocionic Seizures, Jackknife Epilepsy) Usually between 2 to 12 months Triad Infantile epileptic spasms, developmental regression, EEG: hypsarthythmia Lennox-Gastaut syndrome Manifests between ages 2 to 10 years, triad of: 1 Developmental delay 2. BEG: 1fo 2 HD spike in slow waves, polyspike ‘burst in sleep and snow background and waketuiness (Diffuse siow spe and wave pattern on) 3. Multiole types of generalized seizures -Pts. commoniy have status epilepticus Absence seizures (petit mal) Usually preceded by aura Went to do a lumbar tap on Pediatrics - Topnotch All infants younger than six months of age who present with fever and seizure (or ifthe child is stil ig qu con2095907 aed ences)Simple febrile seizure ‘The most common seizure disorder during childhood Lasts 15 mins or LESS. ‘Occurs only once in 24 hours. Rare befor 9 months and after 5 years of age, peak age of onset is 14 to 18 months of age. Complex or complicated febrile seizures Duration is »15 minutes Repeated convulsions occur within 24 hours Status epilepticus Continues seizure activity or recurrent seizure activity without regaining of consciousness lasting for °5 minutes as part of an operational definition put forth with in the past few years. In the past the cut off time is 30 minutes, Reflectory status epilepticus Status epilepticus that has failed to respond to. therapy, usually with at least two medications such ‘as benzodiazepine and another medication. Status epilepticus treatment The strongest evidence for intial and emergent therapy is for diazepam or lorazepam, followed by Phenytoin/fosphenytoin and phenobarbital, then valproate and levitiracetam. HHV6 and HHY 7, Inpatients developing febrile status epilepticus, these viral infections are most commonly associated In V3 of the cases, -Roseola: high fevers for several days that can cause seizures, followed by a diffuse macular rash (see picture) “Transmitted by saliva Dravet Syndrome (Severe Myaclonic Epilepsy of Infancy) SCNIA gene West Syndrome (Infantile Spasms, Infantile Myoctonic Seizures, Jackknife Epilepsy) Pediatrics - Topnotch ips com 259587apedtes onsen ashes! ‘ARX Gene; polymerase GOhtanara syndrome ‘syntaxin binding protein 8 meningit’s Triad: hydrocephalus, basal enhancements, infarcts, ‘Obstructive or Non- ‘Communicating Hydrocephalus ‘Obstruction with in the ventricular system ‘Caused by: abnormality of the aqueduct or a lesion in the Ath ventricle (Aqueductal stenosis) Non-obstructive or ‘Communicating bliteration of the subarachnoid cistern. Malfunction of the arachnoid vill Caused by: follows a subarachnoid hemorrhage or Hydrocephalus ‘yarocep' levkeric infiltrates Produced inthe choroid plexus epithelium within the cerebral ventricles, Cerebrospinal fui (CSF) Reabsorbed: arachnoid villus cells, which are Located in the superior sagittal sinus, return CSF to the bloodstream within vacuoles by a process called PINOCYTOSIS. Absolute contraindication of lumbar tap Unstable, cardiovascular compromised Partial contraindication of lumbar tap Skin infection on site Naegleria fowieri (brain eating amoeba) Pre-frontal headache, high fever, disturbance in ‘smell, swimming in warm water Borrelia burgdorferi Bells palsy, systemic disease, cutaneous lesions, carditis Campylobacter jejuni Ascending paralysis, history of GIT infection Hemophilus influenzae B (Hi8) Ascending paralysis, history of RESPIRATORY Infection Reye syndrome Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Lethargy and irritabilty, history of viral infection, chickenpox, aspirin use. Encephalopathy and liver damage in children7B meningit’s Ocular nerve palsy, previous history of TB or active 3 Brain abscess MC in 4-8 y/o Cranial CT scan & MRI: most reliable Do NOTdo CSF test to avoid herniation Prophylaxis for neisseria meningitidis (meningococcus) Rifampin, Ceftriaxone, Ciprofloxacin Meningococcemia Pink macules and papules, petechiae, hemorrhagic petechiae, hemorrhagic bullae, purpura fulminans MOT by aerosol droplets or through contact with respiratory secretions Defuse adrenal hemorrhage without vasculitis, DIC, ‘coma and death —» Waterhouse Friderichsen syndrome (fulminant cases) Meningococcemia treatment Pen G, Ceftriaxone, Cefotaxime Meningococcemia prophylaxis {for exposed person: Children: Rifampicin or Ceftriaxone 218 y: Ciprofloxacin Meningocele The most severe form of neutral tube defect (dysraphism) Due to failure of the neural tube to close spontaneously between the 3rd and 4th week of in utero development, the congenital herniation of the meninges through a defect in the skull or spinal column Sepsis, seizure, irritability, lethargy, bulging of ces fontanelles,rigiity, pix <2mos old Headache, fever, confusion, lethargy, nuchal rigilty, KiB vorniting, no mention of proper vaccines, <5 years old Pediatrics - Topnotch ips com 259587apedtes onsen ashes!N. meningitis Headache, fever, confusion, lethargy, nuchal rigidity, vomiting, properly vaccinated child, abrupt in onset, toxic looking with rashes all over Strep pneumoniae (oneumococcus) Headache, fever, confusion, lethargy, nuchal rigidity, vvorniting, young adult Usteria Headache, fever, confusion, lethargy, nuchal rigidity, vomiting, renal transplant patient Enterovirus “Poliovirus and coxsackievirus Headache, fever, confusion, lethargy, nuchal rigidity, ‘vomiting, Gradual onset of signs and symptoms, not toxic looking Hsv Headache, fever, confusion, lethargy, nuchal rigility, ‘vomiting, RBCs in the CSF exam Myelomeningocele most severe form of spina bifida in which the spinal ‘cord and meninges protrude through the spine Prevented by Folie acid 0.4mg OD (ang on the 2nd preg) Located in lumbosacral (75%) ‘Assoc with hydrocephalus (type Il chiari) Amnold-Chiari malformation ‘Type I: herniation of cerebellar tonsils, asymptomatic/headache ‘Type Il: herniation of cerebellum and medulla, often ‘causes hydrocephalus. Type Ill: extremely rare, bony defect at occipito cervical level with herniation of cerebellum into encephalocele Migraine Recurrent headaches with symptom-free intervals and at least 3 of the following: Family history, relief following sleep, unilateral Location, associated aura, abdominal pain, nausea ‘and vorniting, throbbing in nature Pediatrics - Topnotch ips com 259587apedtes onsen ashes!‘Aura in migraine “Cortical spreading depression’- A phenomenon associated with high CNS hydrogen and potassium ‘ons with the release of glutamate and nitrous oxide Status migrainosus headache that lasts at least 72 hours in duration (3 ays) Tx Prochlorperazine IV 015mg/kg max 10 mg Migraine prophylaxis Propranolol 10-20mg tid for >7 yo Flunarizine 5mg at bedtime Neurocutaneous syndromes |. Tuberous sclerosis, 2. Neurofibromatosis (Von Reckling-Hausen) Arise from a defect in differentiation of the primitive ectoderm Tuberous Sclerosis Calcified tubers in periventricular area—> CANDLE DRIPPING APPEARANCE Tuberous sclerosis complex genes TSCI- HAMARTIN protein TSC2- TUBERIN protein Both genes act as tumor suppressor genes. ASH LEAF (HYPOpigmented skin lesions) SHAGREEN PATCH- roughened, raised lesion with ‘an orange peel consistency located in the lumbosacral region ‘Adolescence: subungual or periungual fibromas from the fingers & toes Neurofibromatosis (NF) von Recklinghausen disease Chromosome 17 Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Neurofibromatosis 1 (NF) Most prevalent type 2out of 7: "6 or more CAFE AU LAIT macules »5mm diameter (prepubertal) & >15mm (postpubertal) in trunk & extremities (FACE SPARING) “aaillary or inguinal FRECKLING (hyperpigmentation) 23mm diameter "2 or more Iris LISCH NODULES (HAMARTOMAS. w/in iris) "2 or more neurofibromas or | plexiform neurofibroma “osseous lesions “optic glioma “st degree relative w/ NFI (paternal germline) Neurotibromatosis 2 (NF2) lof the ff “bilateral 8th nerve masses (ACOUSTIC NEUROMA) “Parent, Sibling, or child with an NF2 and either unilateral 8th nerve masses or any 2 of the following neurofibromas, meningoma, glioma, schwannoma Guillain-Barre syndrome (G88) “ACUTE DEMYELINATING POLYRADICULONEUROPATHY™ Symmetric ascending muscle weakness or paralysis Post infectious polyneuropathy involving mainly motor. Auto immune reaction that develops in response to a previous infection (GIR or RT) LANDRY ASCENDING PARALYSIS. Weakness begins in the lower extremities and progressively involves the trunk, upper limbs and bulbar muscles GBS associated Physical findings CRANIAL NERVE DEFICITS leading to dysphagia, ‘dysarthria, facial weakness, papilledema, autonomic dysfunction, respiratory muscle paralysis, MILLER FISCHER SYNDROME: acute: ‘ophthalmoplegia, ataxia, arefiexia Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Tendon reflexes usually the last function to recover and lower extremity weakness last to resolve. CSF analysis: Increased protein, normal glucose, normal cells, no pleocytosis oes Dissociation between high CSF protein and a lack of cellular response is diagnostic (albuminocytologic dissociation) IVIG 0.4 g/KG/day for 5 days GBS treatment Supportive High dose pulse methyiprednisolone IV for relapses. Medulloblastoma Heterogenous enhancements in the cerebellum, often in the 4th ventricle, and can cause obstructive hydrocephalus. HOMER-WRIGHT ROSETTES: Circular patterns of tumor cells surrounding a center of neutrophils Infratentorial tumors 1) Cerebellar astrocytoma- most common, best prognosis, causes hydrocephalus 2) Medulloblastoma- spread to extracranial sites 3) Brain stem glioma 4) Ependymoma Supratentorial tumors 1) Craniopharyngioma- short stature, pressure to ‘optioc chiasm (bitemporal visual field defects) 2) Optic nerve glioma- decreased visual acuity, pallor of discs, 25% neurofibromatosis, invasion of ypothalamis causing obesity or DI 3) Astrocytoma 4) Choroid plexus papilloma Arterlopathy ‘The most common cause of arterial ischemic stroke In pediatric population. Unilateral irregular stenosis of the MCA. Epidural This type of intracranial hemorrhage is almost always associated with trauma Pediatrics - Topnotch ips com 259587apedtes onsen ashes!‘The acute onset of the focal neurologic deficit ina child is Until proven otherwise, Diagnostic of choice is__ STROKE ; MRI Arterial ischemic stroke (AIS) Strokes most often involve the MIDDLE CEREBRAL, ARTERY territory but can occur in any cerebral artery of any size AIS 3 etiologies: 1) Arteriopathy- leading cause, 2) Cardiac 3) Hematologic Als mngt: Heparin, Aspirin Cerebral Sinovenous ‘Thrombosis (CSVT) Greatest risk in the neonatal. Diagnostic of choice: CT venography or MR venography Hemorrhagic stoke CT highly sensitive Instantaneous or thunderclap headache, (oss of consciousness, nuchal rigilty, focal neurologic deficits, seizures, maybe rapidly fatal 1) Abusive head trauma- primarity subdural or intraparenchymal 2) Epidural hemorrhage- nearly all are caused by trauma '3) Subdural hemorrhage- May occur spontaneously in children with brain atrophy (stretching of the bridging veins) (Hemorrhagic stroke) AV malformation The most common cause of childhood subarachnoid and intraparenchymal hemorrhagic stroke, CHARGE syndrome Pediatrics - Topnotch ips com 259587apedtes onsen ashes! ‘Coloboma of the eye Heart defects Atresia of the choanae Renal or CNS anomalies, retarded growth Genital anomalies or hypogonadismRespiratory problems rank high ‘among Filipino children, The nature of this problem is frequently? Infectious Rhinovirus (common cold) The principal agent that causes the common colds Acute Epigtotttis Inflammation of the epiglottis; H infivenzae type b Is the most common cause, especially in nonimmunized children. Common symptoms are high fever and sore throat Dysphagia, dysphonia, drooling, distress SECURE AIRWAY WITH INTUBATION: spot CHERRY RED EPIGLOTTIS via direct laryngoscopy THUMB SIGN or LEAF SIGN. Viral Croup (aryngotracheobronchitis) Parain‘luenza virus Prodome onset (1-7 days) Low fever Barking cough, hoarseness Stridor improves STEEPLE SIGN Epigtottits H. Influenza B (or staph aureus if vaccinated) Rapid onset (4-12 hrs) High fever Muffled voice, drooling THUMBPRINT SIGN / LEAF SIGN Stridor vs Wheeze in foreign body aspiration Stridor- Upper airway lodge Wheeze- Lower airway lodge Nuts Foreign body most commonly obtained from respiratory tract of children Allergic rhinitis (hay fever) Prominent itching and sneezing, nasal eosinophilia Foreign body Unilateral foul smelling discharge, bloody nasal secretions Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Headache, facial pain, periorbital edema, Sinusitis shinorrhea for »2 weeks Persistent rhinorrhea with onset in the first three months of life (snuffles) Congenital Syphilis, -deformes teeth hutchinsois tee (Treponema Palidum) " -mental retardation hydrocephalus, -deatness, blindness History of prolonged use of topical or oral decongestant Rhinitis Medicamentosa rebound nasal congestion commonly associated with overuse of over-the-counter nasal decongestants Bordetella pertussis Pertussis (whooping cough) Paroxysms of cough, leaving the baby breathless land subconjunctival hemorrhages Streptococcus pneumoniae, Non-typable H. Influenza, Moraxella catarthalis Sinusitis Co-amoxiclay 14 days. Sinuses present at birth Maxillary & Ethmoid sinuses y Parletoacanthial projection (Waters) shows what sinuses? Sphenoid sinus Sinus that is pneumatized at 4 years old Frontal sinus Sinus begins to develop at 7-8 years old Macrolide (Azithrimycin, Best treatment for Pertussis (whooping cough) Erythromycin, Clarithromycin) Given to close/household contact too. Pediatrics - Topnotch ips com 259587apedtes onsen ashes! esrPertussis (whooping cough) Intermittent episodes of continuous coughing until the child turns purple followed by a deep loud Ingpiration, One week prior child had episodes of sneezing and rhinorrhea Gram negative coccobacilli BORDETELLA PERTUSSIS Stages of pertussis (lasting 2 weeks each) Catarrhal, Paroxysmal, Convalent Pertussis catarrhal stage Most infectious during this time, Resemble a common cold, Pertussis: Period of ‘communicability Greatest during catarrhal stage before onset of paroxysms From seven days after exposure to four weeks after onset of typical paroxysms 1:32 days. ‘The most common complication of acute Otitis meia nasopharyngitis ‘To prevent occurrence of the glomerulonephritis or Penicilin theumatic fever in children, cases of acute tonsillopharynalts is best treated with? Recurrent strep pharyngitis, Culture + strep pharynaits that has been severe and, frequent: >7 episodes in the previous year or >5 in each of the preceding 2 years so consider tonsillectomy!! Ssx of Upper airway ‘obstruction plus: Bacterial tracheltis Patchy infiltrates and ragged ‘Tx antibiotics trachea column Ssx of upper airway Epiglottits ‘obstruction plus: THUMB SIGN ‘Tx antibiotics Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Ssx of upper airway Croup obstruction plus: SUBGLOTTIC NARROWING ‘Tx aerosolized racemic epinephrine and STEEPLE SIGN Ssx of upper airway Foreign body aspiration obstruction plus: Air trapping on the right lung Te Rigid bronchoscopy with mediastinal shift towards the right tung Child coughing and wheezing Bronchial asthma usually worse at night. ‘Wheezing on bilateral (ung fields, occasional rhoneti, prolonged expiratory phase Bronchial muscle spasm, bronchial mucosal edema, ‘mucus hypersecretion Respiratory difficulty in vs bronchial asthma is due to: (Exprratory wheezing Is due to the alrway constriction) Infection of the bronchioles (smaller air-tubes in the lungs) sronchiolot Via RESPIRATORY SYNCYTIAL VIRUS (RSV) Xray: flattened diaphragm, hyperinflated lungs with patchy atelectasis, Pneumonia triad Fever, cough, tachypnea Wheezing + Broncheottis History of viral infection among the family members. Two years old Wheezing + Asthma History of atopy in the family Recurrent wheezing using ‘especially after mild viral Pediatrics - Topnotch ips com 259587apedtes onsen ashes! ‘oorWheezing + Wheezes are heard loudest ‘over the trachea, Persistent wheezing never seems to 90 away. Chondromatacia Wheezing + ‘Absent breath sounds on the right lung, Three years old Foreign body Present with Pneumonia Fever, cough, tachycardia + poorly nourished, unvaccinated, with the onset of rashes all over the body Measles Present with Pneumonia Fever, cough, tachycardia + (CF, CGD, burn patient, neutropenic Pseudomonas Present with Pneumonia Fever, cough, tachycardia + Teens or young aduit lives in a dormitory, intial cough is nonproductive Mycoplasma pneumoniae Present with Pneumonia Fever, cough, tachycardia + Patient has his own aviarium in his home Psittacosis Present with Pneumonia Fever, cough, tachycardia + History of eye discharge ‘during the first 5 to 14 days of, neonatal period Chiamygia ‘Cough, colds, fever, wheezing, stridor + Lobar consolidation Pneumococcus Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Cough, colds, fever, wheezing, stridor + Hyper inflation with bilateral interstitial infiltrates in Periorbronchial cuffing RSV Cough, colds, fever, wheezing, stridor + Prominent areas of cavitations and multiple pneumatoceles Staphylococcus Cough, colds, fever, wheezing, stridor + Right sided hilar adenopathy Tuberculosis Croup (laryngotracheobronctitis) BARKING cough, STRIDOR, erythematous posterior pharynx, Bacterial tracheltis (Gtaphylococcus) BRASSY cough, RHONCHI, erythematous posterior pharynx, RAGGED AIR COLUMN SIGN on lateral xray of the neck (eseudomembrane detachment in the trachea) Can have STEEPLE SIGN Recent conversion of PPD to positive In most children, the only evidence of primary TB is this Tuberculin sensitivity develops _ days after after its 72 novrs (3 days Gadays) administration on the forearm Infected LN of the neck Seorfula The most common extrapulmonary form of tuberculosis in ehisren In. Postve tuberculin skin test px, Thisis Hepstosplenomegaly suggestive of MILIARYTB Pediatrics - Topnotch ips com 259587apedtes onsen ashes!(Pulmonary Tuberculosis) Primary complex (Ghon complex) 1. Primary pulmonary focus 2. Regional LN 3 Peritracheal LN 4, Localized pleurisy between the middle & lower lobes PCAP-C CXR findings Bilateral interstitial infiltrates Tachypnea ‘The most consistent clinical manifestation of pneumonia in children Mycoplasma pneumoniae “Walking pneumoniat Chlamycia “STACCATO COUGH' Retropharyngeal abscess HOT POTATO VOICE (muffled voice) Bulging of the posterior pharyngeal wall AIRWAY OBSTRUCTION EMERGENCY tonsils are HUGE ‘Abscess is in the back of the throat behing the tonsils Sox stiff neck, difficulty swallowing, FVR ‘can go into paravertebral spac must admit meninaitis Get CT to confirm abscess Te tonsilectomy and emergency drainage Bronchitis HACKING COUGH Peritonsillar abscess Bacterial invasion thru the capsule of the tonsils. Group A strep & anaerobes. Fever, sore throat, DYSPHAGIA, TRISMUS PE: A symmetric tonsillar bulge with displaced uvula CT scan 1&D or needle aspiration & antibiotics Retropharyngeal abscess Pediatrics - Topnotch ips com 259587apedtes onsen ashes! PE: drooling, neck held in hyper extension, bulging of the posterior pharyngeal wall, neck pain, muffled voice, respiratory distress. Group A streptococcus, anaerobes, staphylococcus aureus, IV antibiotics with or without surgical drainageJoint pain, fever, fatigue & rash Pain syndrome (fibromyalgia) Symptoms suggestive of Growing pains rheumatic disease Patellofemoral syndrome Benign hypermobility syndrome Juvenile Dermatomyositis ‘The most common of pediatric inflammatory GDM) myopathies Medium sized vessel vasculitis Fever at least 5 days plus 4/5: C conjunctivitis without exudate Re rash (scarlantiniform) ‘A. adenopathy (cervical >.Scm, unilateral) S- strawberry tongue/oropharyngeal changes H- hand changes (induration, brawny edema, peeling around nail beds/desquamation) Kawasaki Te IMIG 26/KG over 10 to 12 hours GOTTRON PAPULES HELIOTROPE RASH/eyelids GOWER SIGN SHAWL SIGN Juvenile Dermatomyositis DM) Dysphagia- a severe prognostic factor Classic rash + 3/4 Symmetrical weakness, elevated muscle enzyme, electromyographic changes, muscie biopsy Alanine aminotransferase: most commonly elevated ‘on initial presentation JDM CANA in >80% “De corticosteroids (methyloredrisolone), methotrexate, folic acid, IVIG Pediatrics - Topnotch ips com 259587apedtes onsen ashes! srIGA mediated vasculitis of small vessels. Most common cause of non-thrombocytopenic Purpura in children, Henoch-Schonlein Purpura Linked to HLA B34 & HLA DRBI (HsP) IgA type Ill hypersensitivity —> vasculltis/nephropathy, palpable purpura + arthralgias + GI symptoms, often follows URTI (immediately, not delayed like PSGN) 2h ). Palpable purpura 2. Age of onset less than 20 years old Hsp 3. Bowel angina (post prandial abdominal pain, bloody diarrhea) 4, Biopsy demonstrating intramural granulocytes in small arterioles and or venules Functional closure: 10-15 hours Ductus Arteriosus Anatomic closure: 2-3 weeks The strongest stimulus for ‘A post-natal increase in 02 saturation of the anatomic closure of Ductus systemic circulation Arteriosus The MC cyanotic heart disease in an Xe F fetralogy of Fallot (TOF) INFANT/TODDLER ‘The MC cyanotic heart disease in NEWBORN Transposition of the Great A defect in which the great vessels are reversed; the arteries (16) en) aorta is connected to the right ventricle, and the. pulmonary artery is conectes to the left ventricle Pulmonary stenosis ‘The cause of dyspnea nTOF Pediatrics - Topnotch ips com 259587apedtes onsen ashes! 07Hypercyanotic/tet spelis DUE TO DECREASED PULMONARY BLOOD FLOW Unoxygenated blood enters the systemic circulation and causes cyanosis with crying, feeding, or defecation - Often seen in infants with Tetrology of Fallot - May occur in any child whose heart defect Includes obstruction to pulmonary blood flow & ‘communication between ventricles - Sudden infundibular spasm decreases pulmonary blood flow & increases right-to-left shunting = Occur more often in the morning - May be preceded by feeding, crying, defecation, or stressful procedures Managment of Hypoxic spells Morphine Sulfate 02mg/kg SC knee-chest position NaHCO31 mEq/kg slow IV 02 Phenylephrine (vasoconstrictors) Propanolot Ketamine Hyperoxia test screening to distinguish cyanotic congenitah heart disease vs pulmonary disease Oc test where you put baby in 100% oxygen. If their Low pulse ox was due to pulmonary cause, thelr 02 ssat would go up; if it was caused by their heart, their pulse ox would not go up much ‘done when the patient is cyanotic -room air 2 level from ABG then again after 100% o2 improves = pulmonary pathology ~"failed" test = neonatal emergency Down syncrome ‘Assoc w/ presence if an endocardial cushion defect (sp) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Cyanosis manifesting with in few hours at birth or within few days of tie TGA Single and loud 82, no heart murmur is heard in infants with an intact ventricular septum Hypoxemia unresponsive to oxygen inhalation Cyanosis manifesting after the first year of life, TOF usually in an infant or a toddler Pathophy: the aorta arises from the RV carrying the saturated blood to the body; the PE arises posteriorly from the LV carrying oxygenated blood to the lungs. Result: complete separation of pulmonary and systemic circulations —> Hypoxemic blood circulating throughout the body and hyperoxemic Transposition of the great given yereye (00d circulating in the pulmonary circuit vowels (1G) blood circulating in the pulmonary circuit Signs of CHF with dyspnea and feeding difficulties, developed during the newborn period, Xray: EGG SHAPED cardiac silhouette w/ anarrow, superior mediastinum EGG ON A STRING SIGN Femoral pulses are weak and delayed bilaterally (CoA) Turner syndrome ‘A chromosomal disorder in females in which either an X chromosome is missing, making the person XO Instead of XX, or part of one X chromosome is, deleted. Pediatrics - Topnotch ips com 259587apedtes onsen ashes! warcOA Mor Lesion is in the descending aorta, distal to the origin of the left subclavian artery, Pathyphy: pressure buildup in the proximal aorta land LV causing hypertension in the upper extremity, WEAK, DELAYED, or ABSENT FEMORAL PULSES: BP higher in arms CXR or ECG: LVH and RIB NOTCHING at the Inferior part Best bx: primary re-anastomosis or a patch aortoplasty Obstructive lesions COA, aortic stenosis, pulmonic stenosis Pulmonie stenosis narrowing of the opening and valvular area between the pulmonary artery and right ventricle SYSTOLIC EJECTION MURMUR at LUSS (radiates to upper back) ‘Tx Balloon valvuloplasty or valvulotomy (BROCK PROCEDURE) Aortic stenosis calcification of aortic valve cusps that restricts forward flow of blood during systole SYSTOLIC EJECTION MURMUR at RUSB Tx: Balloon valvuloplasty or ROSS PROCEDURE (valve translocation) PDA Continuous murmur, cyanosis on lower ext, Left to right shunt Exercise intolerance, easy fatiguabitity + Late systolic murmur with an ‘opening click Mvp Pediatrics - Topnotch ips com 259587apedtes onsen ashes!exerci intolerance, easy fatiguabitty + Disparity in pulsation and 8P in the arms and legs. Weak popliteal. posterior tibial and dorsalis pedis pulses COA Exercise intolerance, easy fatiquability + S2 widely split and fixed in all phases of respiration ASD Exercise intolerance, easy fatiguability + Loud harsh blowing holosystolic murmur sD Exercise intolerance, easy fatiguabilty + Wide pulse pressure, bounding peripheral artery, pulses ‘continuous murmur PDA ‘TOF surgical procedure Blalock-taussig shunt w/ GoreTex conduit Aortopulmonary window shunt Waterson cooley Pott shunt TGA surgical procedure Rashkind arterial septostomy Jantene arterial switch Senning and mustard Tricuspid atresia surgical procedure Fontan procedure hypoplastic left heart syncrome Norwood procedure Glenn anastomosis Rubella (German Measles) PDA DM TGA Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Aspirin ‘Causes persistent pulmonary HPN Alcohol VSD, PS Lithium Ebstein anomaly Streptococcal infection : Ab ‘Throat : Anti-streptolysin © titer ‘Skin : Anti-deoxyribonuclease B St. Vidus dance Sydenhams chorea/chorea minor Tachycardia in rheumatic fever is significant i this is Sleeping noted during Prolonged PR interval First degree AV block According to the Jones criteria this is considered a minor manifestation of acute rheumatle fever. Signs of CH Gallop rhythm, distant heart sounds, cardiomegaly ‘The most consistent feature of acute rheumatic Valvulits, fever (ARF) ‘Subclinical carditis (kaw to) The circumstance in which classis auscultatory findings of valvular dysfunction are not present or are not recognized by the clinician but 2D-echo reveal mitral or aortic valvulitis. Erythema marginatum in rheumatic fever Non-pruritic or serpiginous or annular ‘erythematous evanescent rashes most prominent on the trunk and inner proximal portions of the extremities. Never on the face, disappear on ‘exposure to cold in reappear atter a hot shower or if covered with a blanket Subcutaneous nodules Hard, painless, non-pururitc, freely movable ‘swelling 02 to 2.¢m in diameter, Found symmetrically, singly or in clusters on the extensor surfaces of large and small joints. Lasts for weeks. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Sydenham Chorea (St. Vitus dance) Occurs more often in prepubertal gis. Spontaneous purposeless movements followed by motor weakness. Secondary prophylaxis to prevent recurrence of ‘ARF /theumatic fever with carditis and persistent valvular heart disease 10 years after the last attacked or until 40 years old Murmur? The most common presentation of PDA in children Grading murmurs 1-6 1 audible by expert in optimal setting 2isquiet 3. moderately loud 4, markedly loud and has thrill 5. very Loud with thrit 6, audible without stethoscope Murmur grading ‘Swooshing sound-turbulent blood flow Posture- supine, left side, and sitting, leaning forward Grade |- barely audible (soft, difficult) Grade 2- audible, but faint (heard easily) Grade 3- loud, easy to hear Grade 4- (oud w/ palpable thrill, Grade 5- thrill, loud, heard w/ edge of steth Grade 6- thrill, (oud, heard w/o stethoscope After 3, you have a THRILL Infective Endocarditis (IE) Infection of endocardial surface of the heart, usually heart valves, OSLER NODES JANEWAY LESIONS: SPLINTER HEMORRHAGES Ritter Disease (SSSS) Staph. Aureus NIKOLSKY POSITIVE Exotoxin: Exfollatin A or 8 Adenovirus Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Pharyngoconjunetival fever in children is most ‘commonly caused by this:Reiter Syndrome Triad “Cant see, can't pee, cantt climb a tree’ Blindness Urethrtis Arthritis Rubella (German measles; 5- Posterior cervical, cervical or auricular day meastes) lymphadenopathy, fever, rash 3C’s (cough, coryza, conjunctivitis), fever disappears Rubeola (Measles) ‘once rash has moved down to feet, RASHES APPEAR AT PEAK OF FEVER Roseola (6th disease; ‘exanthem subitum) Homan herpes virus 6 (HHV-6) Fever disappears then rash will appear Erythema infectiosum (Fifth Disease) Slapped cheek looking rashes then spread to trunk with central clearing Vitamin A supplements Decreases mortality in rubeola/measles infection Measies/rubeola post exposure prophylaxis |. Measles IG for prevention and attenuation with in 6 days of exposure (0.25 ML/KG max of 15 ML IM) 2. Measles active vaccine for susceptible children » year old within 72 hours Infants <6 months old, pregnant and immunocompromise persons should receive IG but not the active vaccine, Rubeola (Measles) Warthin-Finkeldey cells Rabies Negri bodies Herpes Cowdry Type A inclusions Yellow fever & hepatitis Counciman bodies Forschelmer spots, Rubella (german measles/3 day measles) Petechiae on soft palate Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Koplik spots Rubeola (measles) [APPEARS 2-3 DAYS BEFORE RASHES Clustered white lesions of buccal mucosa, adjacent 2nd-Brd lower molar Nagayama spots Roseola (6th disease; exanthem subitum) Red papules/uicers on soft palate. Strawberry tongue ‘Scarlet fever, Kawasaki disease, toxic shock syndrome, Anti-rubella IgM in the infant Indicates congerital rubella Hand-foot-mouth disease RNA Virus: Coxsackievirus AI6 Erythema Infectiosum (Fifth Disease) Parvovirus 819 A six-year-old male patient presents to the ER with a fever 40°C and sore throat. Upon inspection there are multiple vesicles and also ulcers over the tonsillar pillars and posterior pharyngeal wail, no rashes on the body or extremities. What is the most likely diagnosis HERPANGINA, by COXSACKIE A virus ‘Tx Supportive Meningoencephalitis Most frequent complication of mumps Leptospirosis, Primary damage to the endothelial lining of small blood vessels Anicteric leptospirosis Initial/septicemic phase: conjunctival suffusion with photophobia & orbital pain, hepatosplenomegaly, truncal red macutopapular rash, BIPHASIC fever Icteric leptospirosis (Weil syndrome) Most severe form Azotemia, oliguria, anuria Microscopie slide agglutination Pediatrics - Topnotch ips com 259587apedtes onsen ashes! Most useful screening testLeptospirosis, Clinical evidence and serologic test establishes the diagnosis Leptospirosis treatment Penicillin or Tetracycline Treatment before the 7th day shortens the clinical course and decrease the severity of infection Double bling placebo controlled food challenge The gold standard in the diagnosis of food allergy Wiskott-Aldrich Syndrome (WAS) = X-Linked disorder xterized by the triad of thrombocytopenia, eczema, and recurrent bacterial Infections, - The thrombocytopenia is caused by decreased platelet production, and the few platelets that typically quite small. Often have prolonged bleeding from the circumcision site or bloody diatthea during infancy ‘Tx of choice: Bone marrow or cord blood transplantation Bruton’s X-linked, agammaglobulinemia Defect in BTK, a tyrosine kinase gene leads to no B- cell maturation. Xclinked recessive (INC in Boys) Recurrent Bacterial and enterovirus infections after loss of protective maternal IgG (6 mo) Labs: Absent B cells in peripheral blood, DEC Ig of all classes. Pediatrics - Topnotch ips com 259587apedtes onsen ashes!SCID (severe combined immunodeficiency) ‘The mast severe immunodeficiency. + Defect in early stem cell differentiation - 7 different gene defects: adenosine deaminase deficiency - No B or Tl only NK cells Left! 1. Severe recurrent infections + chronic mucocutcandidiasis - fatal / recurrent RSV, VZV, HSV, measles, flu, parainfluenza - PCP pneumonia 2. Chronic diarrhea 3 Failure to thrive - No thymic shadow on newborn CXR (also seen in diGeorge) DiGeorge Syndrome failure 3rd/4th pharyngeal pouches to develop; T cell deficiency; absent thymus Increased susceptibility to viral, fungal and protozoal infections. CATCH 22 C- cardiac defect (TOF) ‘A- abnormal fascies 1: thymic aplasia/T cell abnormality © Cleft palate H- Chromosome 22 deletion Duncan disease + Xelinked lymphoproliferation syndrome - patients w/Duncan disease who get EBV will have severe illness w/ chronic infectious mononucleosis, agammaglobulinemia, B-cell lymphoma, death (1/3) Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Job syndrome (hyper-IgE syndrome) Job is A-DOPE AD or sporadic STATS gene AR-HIES Clinical features: 1) Abscesses and skin infections 2) atopic Dermatitis 3) Osteopenia 4) Pneumonia and other sinopulmonary infections (eneumocete) '5) hyper IgE, hyper Eos ~~ coarse faces: broad nasal bridge, prominent forehead > dental abnormalities + retention of primary teeth - double-row of teeth Hyper-IgE syndrome (Job's syndrome): FATED F = coarse Facies ‘A= staphylococcal Abscesses T= retained primary Teeth increased IgE D = dermatologic problems (eczema) Disorders of neutrophil function 1. Degranulation abnormality: Chediak-Higashi syndrome, 2, Adhesion abnormality: Leukocyte adhesion deficiency (1-3) 3. Microbicidal activity: Chronic granulomatous disease Chronic Granulomatous - Lack of NADPH oxidase activity —> impotent phagocytes Disease (CGD) Failure to kil catalase postive microbes. Hallmark is granuloma for mission and inflammatory process. TO to 14 days Umbilical cord falls off during this time Pediatrics - Topnotch ips com 259587apedtes onsen ashes!Peis Tore esha [Ot Leukocyte Adhesion Deficiency (an) immune deficiency caused by defects in leukocyte ‘function: failure in adhesion Recurrent bacterial infection with lack of pass for ‘mission, bleeding tendencies, DELAYED umbilical cord separation. After 6 hours of parenteral nutrition, the nurse checks 8S to be 120, what to do? Washing of the newborn should be delayed after? 1to 3 minutes Cord clamping should be delayed at? 60-90 minutes Non-separation of mother and newborn should for breast feeding should last for? 05 % erythromycin V% tetracycline Newborn eye prophylaxis «15 kg - 05mg >1Skg -15 mg Vitamin K dosage for newborn, cH CAH GePD PKU Galactosemia MsuD NBS. ips com 259587apedtes onsen ashes!