SAS # 29

STRUCTURAL ABNORMALITIES OF THE URINARY TRACT

1. PATENT URACHUS

· A narrow tube(urachus)

· Bladder and umbilicues fails to close

· Common in males

ASSESSMENT:

· Nitrazine paper test for PH; clear fluid as urine

· UTZ

MANAGEMENT:

· Surgical Corrections

· Small Sub-Umbilical Incision

2. EXSTROPHY OF THE BLADDER

· midline closure defect @ 10th week of pregnancy

· both sexes

ASSESSMENT:

· Fetal UTZ (Congenital Anomaly Scan)

· Wide pubic diastasis

· Epispadias

· Kidney Infection

· Waddling gait
MANAGEMENT:

· Surgical closure

· A&D Ointment, Karaya Gum, Maalox

· Sponge Bathe

3. HYPOSPADIAS

· urethral defect

· urethral opening is not the end of the penis

· familial or multifctorial genetic

ASSESSMENT:

· Routine PE

· Minimal (on glans); Maximal (@midshaft)

· Short Chordee - fibrous band

MANAGEMENT:

· Not to be corcumcised

· Chordee repair- straightens

· Meatotomy- extends

· Acetaminophen (Tylenol), Oxybutynin (Ditropan)

INFECTIONS OF THE URINARY SYSTEM AND RELATED DISORDERS

1. HYDRONEPHROSIS

· enlargement of the pelvis of the kidney with urine

 · @ any age; often @ 6 months

ASSESSMENT:

· asymptomatic

· repeated UTIs

· Abdominal mass

· elevated Blood pressure

· Severe back pressure

· Intravenous Pyelogram (IVP) or UTZ

MANAGEMENT:

· Surgical correction before gomerular or tubular destruction occurs

SAS # 30

DISORDERS AFFECTING NORMA URINARY ELIMINATION

1. ENURESIS

· involuntary passage of the urine

· nocturnal or diurnal or both

· bladder training was never achieved

ASSESSMENT:

· >5 years old

· History taking

· Wet @ nights (tired or troubled)

 · Wet @ engrossed activity (need more reminders to void)

· Abdominal pain, burning, frequency: suggests UTI

· Associated with small bladder capacity

DIAGNOSIS:

· UTZ

· Pre-post Bladder Scan or Urodynamic Testing

· Clean-catch urine specimen

· Assess specific gravity, protein and glucose of urine

MANAGEMENT:

· no fluids 2 hours before bed

· Bladder-Stretching Exercises

· Antidiuretic Hormone (Desmopressin [DDAVP]), oral

2. POLYCYSTIC KIDNEY

· large, fluid-filled cysts

· seen as autsomal recessive (frequent) trait or autosomal dominant (rare) trait

· kidneys grow large and feel soft and spongy

· flattened nose or micrognathia (small jaw); Potter Syndrome

MANAGEMENT:

· Surgical removal if only one kidney is diseased

· Renal transplantation if two kidneys are diseased

· Genetic Counseling
3. PRUNE BELLY SYNDROME

· syndrome with a broad spectrum of severity

· associated with Cardiac, Pulmonary, Orthopedic, and Urologic

3 UROLOGIC SYMPTOMS

1. BILATERAL UNDESCENDED TESTES

2. DILATED FAULTY DEVELOPMENT OF THE BLADDER AND UPPER URINARY TRACT

3. RENAL DYSPLASIA -wrinkled abdomen

MANAGEMENT:

· Kidney Transplant

4. ACUTE POSTSTREPTOCOCCAL GLOMERULONEPRITIS

· inflammation of the glomeruli of the kidney

· immune complex diseas

· sybtype of group A beta-hemolytic streptococci

· IgG is detected in the bloodstream

ASSESSMENT:

· 5 and 10 years old

· common in boys

· history of recent respiratory infection (7-14 days), impetigo (within 3 weeks)

· Step throat, tonsillitis, otitis media, or impetigo

· Hematuria
 · Oliguria

· HPN

· increased spicific gravity

· low blood protein level (hypoalbuminemia)

MANAGEMENT:

· course of antibiotics

· diuretics: ethacryb=nic acid or furosemide (Lasix)

· Semi-Fowler's position, digitalization, oxygen administration

· Antihypertensive therapy (Labetalol)

· Phosphate binders: aluminum hydroxide or potassium-removing resin agent (sodium polystyrene

sulfonate [Kayexalate])

· Weigh child every day

· Calculate I & O

5. CHRONIC GLOMERULONEPHRITIS

· result in either diffuse or local nephron damage

ALPORT SYNDROME - hearing loss and ocular changes

- X-linked or recessive disorder

ASSESSMENT:

· Proteinuria

· HPN

· RBC and WBC casts

· Occult blood in urine (below 1.003)

MANAGEMENT:

· Antihypertensive ( Hydralazine [Apresoline])

· Diuretics (Ethacrynic acid [Edecrin])

· Cortocosteroid therapy

· Kidney transplantation

· Peritoneal dialysis or hemodialysis

SYSTEMIC LUPUS ERETHYMATOSUS (SLE)

· autoimmune disease

· deposists of complement in the kidney glomerulus

· glomerulonephritis or heart disease (ultimate cause of death)

6. NEPHROTIC SYNDROME

· altered glomerulus permeability

· due to an immune process or a T-lymphocyte dysfunction

· peak @ 3 years old; often in boys

3 FORMS

1. CONGENITAL - autosomal recessive disorder

- rare

2. SECONDARY - progression of glomerulonephritis

- sickle-cell anemic or SLE

3. IDIOPATHIC - primary

- common
ASSESSMENT:

· Proteinuria, Edema, Hypoalbuminemia, Hyperlipidemia

· Periorbital edema

· Ascites- anorexia, vomiting, or diarrhea

· Marked proteinuria

· Single test: +1 TO +4 protein

· 24 hr total urine test: 15 g of protein

· Erythrocyte Sedimentation Rate (ESR) is elevated

· MRI/Renal Biopsy

MANAGEMENT:

· Corticosteroids: IV methylprednisolone or oral prednisone

· Supplemental Potassium and eat foods rich in potassium (banana and milk)

· IV albumin

· Cyclophosphamide (Cytoxan) or Cyclosporine (Sandimmune)

· Immunosuppressant (mycophenolate mofetil)

7. HEMOLYTIC-UREMIC SYNDROME

· linign of glomerular arterioles becomes inflamed, swollen, and occuded with platelets and fibrin

· RBCs and platelets are damaged

· leads to hemolytic anemia

· E. Coli

ASSESSMENT:

· transient diarrhea
 · elevated fever

· oliguria accompanied by proteinuria, hematuria, and urinary casts

· elevates serum creatinine and BUN

· Pale from anemia

· Thrombocytopenia

MANAGEMENT:

· Renal replacement tehrapy (supportive therapy)

· Peritoneal dialysis - oliguria

· Transfusion of packed RBCs - anemia

SAS #31

NEOPLASIA

· neoplasm (new growth)

· new abnormal growth

· does not respond to normal growth-cpntrol mechanisms

NEOPLASTIC GROWTH

· benign or malignant

THE LEUKEMIAS

1. ACUTE LYMPHOCYTIC LEUKEMIA (LYMPHOBLASTIC) (ALL)

· immature lymphocytes

· RBCs and platelets declines

 · 2 and 6 years old

ASSESSMENT:

· decreased RBC (anemia)

· decreased platelet (thrombocyte) count

· spleen and liver enlarges

· increased leukocyte count

· platelet count and hematocrit value are low

· Bone Marrow Aspiration

MANAGEMENT:

· Vincristine, Prednisone or Dexamethasone, L-asparaginase, Doxorubicin (4 weeks)

· Intrathecal Administration

· Cranial radiation

MAINTENANCE:

· Maintenance chemotherapy

· Daily mercaptopurine, weekly methotrexate, sporadic vincristine and prednisone, Intrathecal

methotrexates (2-3 years)

COMPLICATIONS:

· CNS Disorders

· Renal Disorders

· Reproductive System Disorders

2. ACUTE MYELOID LEUKEMIA (AML)

 · overproliferation of ganulocytes (neutrophils, basophils, and eosinophils)

ASSESSMENT:

· same symptoms as ALL

· local or systemic infection

MANAGEMENT:

· Bone Marrow Aspiration and Biopsy

· Chemotherapy

· Cytarabine (Ara-C) Etoposide (Vefesid), Daunorubicin (Daunoxome)

· Bone Marrow Transplantation

THE LYMPHOMAS

1. HODGKIN'S DISEASE

· lymphocyte proliferate in the lymph glands

· Reed-Stenberg cells develop

· <7 years old

· spreads to lung, liver, adn bone marrow

ASSESSMENT:

· enlargement of lymph node

· fever, night sweats, anorexia

· SR is increased

· Chest CT Scan

· PET-CET
MANAGEMENT:

· Radiation therapy

· Cyclophosphamide, Vincristine, Procarbazine, Prednisone

2. NON-HODGKINS LYMPHOMA

· malignant disorders of lymphocytes (B or T cells)

· 5-15 years old

ASSESSMENT:

· lymph nodes of neck adn chest, comony axillary, abdominal, or inguinal nodes

· Mediastinal lymph glands are swollen

· Abdominal pain

MANAGEMENT:

· Systemic Chemotherapy

· Cyclophosphamide, Doxorubicin (Adriamycin), Vincristine (Oncovin), and Prednisone (CHOP

therapy)

· Allopurinol

SAS #32

NEOPLASMS OF THE BRAIN

· 2nd most common form of cancer

· most common solid tumor

· 1 & 10 years old: peak @ 5 years old

 TYPES OF BRAIN TUMORS

1. ASTROCYTOMAS

· Sslow-growing

· glial or suport tissue

· peak @ 5-8 years old

· 1/4 of the brain

2. MEDULLOBLASTOMAS

· fast-growing (cerebellum)

· peak @ 5-10 years old

· midline

· 5th-7th, 9th-10th cranial nerves

ASSESSMENT:

· increased ICP

· Lethargy

· Diplopial'Ptosis

· Nystagmus

· Bone Scan

· MRI/UZ

· Cerebral Angiography

· Myelography

MANAGEMENT:
 · Radiation therapy

· Carboplatin combined with Thioguanine Procarbazine, Lomutine, Vincristine

BONE TUMORS

· derived from connective tissues: bone and cartilage, muscle, blood vessels, lymphoid tissue
(sarcomas)

· common in girls

· 13 years old

1. OSTEOGENIC SARCOMA

· malignant tumor of log bone

· boys

· distal femur, proximal tibia, proximal humerus

ASSESSMENT:

· painful and swollen

· history of recent trauma

· CBC, UA, Chest X-ray, Chest CT Scan, Bone Scans

MANAGEMENT:

· chemotherapy

· methotrexate, doxorubicin, cisplatin, ifosfamide

· Limb Salvage

· Cadaver or Mand-made Prosthesis

 SAS #33

INTESTINAL DISORDERS

1. VOLVULUS WITH MALROTATION

· twisting of intestine

ASSESSMENT:

· crying and pain

· pulling up the legs

· abdominal distention

· vomiting

· abdominal mass

· UTZ or Lower Barium X-Ray

MANAGEMENT:

· Surgery

2. SHORT BOWEL or SHORT-GUT SYNDROME

· absorptive disorder

ASSESSMENT:

· NEC

· Volvulus

· GIT trauma

MANAGEMENT:
 · adequate hydration, proper intake of essential vitamins and minerals

· Bowel resection surgery, TPN

· Oral or enteral feedings

3. APPENDICITIS

· inflammation of the appendix

· school-age

ASSESSMENT:

· sharp pain

· Symptoms: anorexia, pain or tenderness in RLQ, nausea and vomiting, elevation of temerature
and leukocytosis

· pain of sharpest point (McBurney's point)

· UTZ or CT Scan

MANAGEMENT:

· surgical removal (laparoscopy)

4. RUPTURED APPENDIX

· potential for increased peritonitis

· WBC count: >20,000/mm^3

MANAGEMENT:

· Semi-Folwer's Position

· IV Fluid

· Antibiotics
5. CELIAC DISEASE/ MALABSORPTION SYNDROME/ GLUTEN INDUCED ENTEROPATHY/ CELIAD SPRUE

· immune-mediated abnormal response to gluten

· flattening of villi

· distended abdomen (thin child)

ASSESSMENT:

· poor groth

· bulky stools

· malnutrition

· distended abdomen

· anemia

MANAGEMENT:

· gluten-free diet

· correction of vitamins and mineral defciency

VITAMINS AND MINERALS DEFICIENCIES

1. VITAMIN A

· lack of yellow vegetables

· Xeropthalmia (dryness)

· Keratomalacia (necrosis)

2. VITAMIN B1

· polished rice as diet

 · Beriberi (tingling or numbness)

· Aphonia (crying w/o sound)

· Diarrhea and Vomiting

3. NIACIN

· corn as diet (low in niacin)

· Pellagra (sunburn)

· Diarrhea

· Mental Confusion

4. VITAMIN C

· lack of fresh fruits

· Scurvy (petechiae, muscle tenderness(

5. VITAMIN D

· lack of sunlight

· Rickets (poor bone formation)

· Craniotabes (softening of the skull)

· Tetany (Muscle Spasms)

SAS #34

POISONING

· ingestion, inhalation

· <5 years old

 1. Cosmetic/Personal Care Products

2. Household Cleaning Products

3. Analgesics

4. Foreign bodies (toys)

5. Topical Preparations (

· <6 years old

6. Opioids and Benzodiazepines

7. Acetaminophen

8. Cough and Cold Remedies

9. Ibuprofen

10. Diphenhydramine

ACTIVATED CHARCOAL

· universal antidote

· fine black poweder mixed with water

· excreted (bowel) after 3 days; stools appear black

INCREASED INTRACRANIAL PRESSURE

· increase on CSF Volume

· Hydrocephalus' Guillain-Barre Syndrome

SYMPTOMS:

1. INCREASED HEAD CIRCUMFERENCE

· >2 cm/ moth (first 3 months)

· >1 cm/month (another 3 months)

 · >0.5 cm/months (next 6 months)

2. FONTANELLE CHANGES

· bulging, closing late of anterior fontanelle (>18 months)

3. VOMITING

· absence of nausea

4. EYE CHANGES

· Diplopia

· Papilledema

· White of sclera

5. VITAL SIGNS CHNAGES

· Elevated temperature and BP

· Decreased PR & RR

6. PAIN

· headache

· Valsalva Maneuver

7. MENTATION

· irritability

· sleepiness
ASSESSMENT:

· slow PR &RR

· Decoricate posturing- adducted and flexed

· Decerebrate Posturing - prnation of wrists

INTRACRANIAL PRESSURE MONITORING (ICPM)

· measure pressure in head

· determin high or low CSF

· oscilloscope (analyze waveform)

3 PROCEDURES

1. INTRAVENTRICULAR CATHETERS

· most accurate

2. SUBDURAL SCREW

· dura meter

3. EPIDURAL SENSOR

· sensor

MANAGEMENT:

· Do not put pressure on jugualr veins

· Semi-Fowler's Position

· Corticosteroid; Dexamethasone (DECADRON)

· Osmotic Diuretic (Mannitol, IV)

 · Indwelling Urinary Catheter

· Ventricular Tap

· Monitor Output every hour

SAS #35

NEUROCUTANEOUS SYNDROMES

1. STURGE-WEBER SYNDROME (ENCEPHALOFACIAL ANGIMATOSIS)

· congenital port-wine birthmark

· 5th CN (Trigeminal Nerve); facial expressions are affected

ASSESSMENT:

· blood flow is sluggish

· anoxia (cerebral cortex)

· numbness (hemiparesis)

· intractable seizures

· CT Scan or MRI (railroad track appearance)

· EEG

MANAGEMENT:

· Surgical removal

· Long-term antiseizure therapy

2. NEUROFIBROMATOSIS (VON RECKLINGHAUSEN DISEASE)

 · unexplained deveopment of subcutaneous tumors

· mutation/autosomal dominant trait (long are of chromosome 17)

· pigmented nevi or cafe-au-lait

ASSESSMENT:

· multiple soft cutaneous tumors

· subcutaneous tumors

· acoustic nerve (CN 8), hearing impairment

· Optic nerve, vision loss

3. CEREBRAL PALSY

· group of nonprogressive disorders of upper motor neuron (motor dysfunction)

TYPES

1. SPASTIC TYPE

· excessive tone in voluntary muscles

· (+) Babinski Reflex, Tonic Neck Reflex

· Scissors gait

HEMIPLAGIA- both extremities

QUADRIPLAGIA- all four extremities

DIPLEGIA or PARAPLEGIA- lower extremities

2. DYSKINETIC TYPE/ATHETOID TYPE

· wormlike

· abnormal involuntary movement

 · limp and flaccid

ASSESSMENT:

· drools and speech is difficult to understand

· Choreoid (irregular, jerking)

· Dyskinetic (disordered muscle tone)

3. ATAXIC TYPE

· awkward, wide-based gait

4. MIXED TYPE

· both spasticity and athetoid or both ataxic and athetoid

4. ENCEPHALITIS

· imflammation of brain tissue and meninges as well

· arise from protozoan, bacterial, fungal, or viral invasions

ENTEROVIRUS- most frequent cause followed by Arbovirus

ASSESSMENT:

· headache, high temperature, ataxia,

· Brain biopsy

MANAGEMENT:

· Antipyretic

· Mechanical Ventilation
 · Acyclovir (Zovirax), Antiviral agent , Carbamazepine (Tegretol), Anticonvulsant

· Steroid (Dexamethasone) or Osmotic Diuretic (Mannitol)

5. REYE SYNDROME

· acute encephalopathy

· infiltration of liver, heart, lungs, pancreas, skeletal muscle

· 1-18 years old

ASSESSMENT:

· lethargy, vomiting, confusion, combativeness

MANAGEMENT:

· Avoid using aspirin during viral infections