Peds Final Exam Review Exam Questions And Correct Answers With Rationale

PEDS Final EXAM Review EXAM
QUESTIONS AND CORRECT ANSWERS
WITH RATIONALE

, PEDS FINAL EXAM REVIEW lv lv lv

PEDIATRIC CANCER lv

Wilms Tumor : Unilateral (more common) or bilateral tumor of kidney
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Most common renal neoplasm in children, good prognosis Medi
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an age @ dx: 2-3 yrs
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Clinical Presentation lv

Palpable abd. mass in healthy child, vascularized encapsulated jelly- lv lv lv lv lv lv lv lv

like (RUPTURE), regular borders Some have pain, microscopic or gross hematuria, malaise, fever, HT
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N
G/U abnl’s: ambig genitalia cryptorchidism lv lv lv

hypospadius Congenital hemi lv lv

hypertrophy
Aniridia
Assoc.syndrome: Beckwith- lv

Wiedeman Treatment: 80% good prognosis lv lv lv lv

Unilateral: Nephrectomy lv

Vincristine & Actinomycin-D for 6months- lv lv lv lv

15months Bilateral: Bilateral partial resection, if possible lv lv lv lv lv lv

Same therapy with addition of adriamycin lv lv lv lv lv

Remove kidney with largest tumor, abdominal radiation for remaining Chemo lv lv lv lv lv lv lv lv lv

therapy complications:
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Mucositis, hair loss, nausea vomiting, bone marrow depression
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NO INFECTIONS SHOULD BE TREATED LIGHTLY
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Infections are the leading cause of death. lv lv lv lv lv lv

Should avoid crowds or exposure to communicable diseases.
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Tumor lysis syndrome: metabolic complication caused by breakdown products of dying cells HYPERURICEMI
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A, HYPERPHOSPHATEMIA, HYPERKALEMIA, HYPOCALCEMIA
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Occurs within 24-48 hours of chemotherapy lv lv lv lv lv

Treatment: hydration to increase urine production, correct electrolyte imbalances lv lv lv lv lv lv lv lv lv

Goal: prevent renal failure lv lv lv

Management of Infusion Devices lv lv lv

Most children will have a external central line or implanted venous access device (IVAD or port-a-
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cath) Guidelines for care are institution specific:
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Rapid, easily obtained venous access lv lv lv lv

Monitor lines for infection (must culture if febrile) Main lv lv lv lv lv lv lv lv

tain patency by heparin flushing q 4 weeks lv lv lv lv lv lv lv

Radiation long term effects: cognitive defects, slow bone growth, growth hormone deficiency, dental problems
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Brain Tumors: Most common solid tumor, 2nd only to leukemia among childhood cancers (17% of all childhood CA).
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2200 new dx in US/yr. lv lv lv lv

Classification system is difficult due to lack of a universally accepted grading system.
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5 year survival ranges 55-75%.
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Signs & Symptoms: lv lv

Headaches: Worse in am or nocturnal lv lv lv lv lv lv

Vomiting: Without nausea lv lv

Increased ICP: lv

Personality changes lv l

Drowsiness v

Resistance to being held lv lv lv

Increased FOC: Delayed closure or elevation of anterior fontanel lv lv lv lv lv lv lv lv lv

Loss of developmental milestones lv lv lv

Papilledema H lv

ead tilt lv

Abnormal reflexes and speech C lv lv lv lv

ranial nerve abnormalities lv lv

Presentation varies with site, age, developmental level 60 lv lv lv lv lv lv lv

% infratentorial (cerebellum or brain stem)
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Diploplia
Gait disturbance Gait ataxia, truncal ataxia Nystagm lv lv lv lv lv lv

us
Weakness
40% supratentorial (cerebral hemispheres, hypothalamus, thalamus, optic chiasm, pineal region) Seizur
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es
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, Headaches
Infants: Irritability, Listlessness, Vomiting
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, Diagnosis: Head CT or MRI lv lv lv lv

Treatment: Surgery (complete resection is ideal)
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Radiation: effective, can not be used on child <4 due to effects on brain Chemothe
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rapy: resistant lv

LEUKEMIA (ALL (best), CML (teens), AML (worst prognosis), T-cell ALL)
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ALL - Acute Lymphoblastic Leukemia (lymphocyte)
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Most common childhood cancer (<15 yrs.) 90% cure rate
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Accounts for 75% of all childhood CA lv lv lv lv lv lv

5-
year relative survival with ALL diagnosed in 2000 is near 80% Ma
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les > females (1.2:1)lv lv lv

Peak age 2-3yrs lv lv

Annually: (2,400 cases per year) lv lv lv lv

Downs (trisomy 21) : 1 in 95 (10-20x greater) lv better prognosis and cure rate, require lighter chemo
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??? Etiology ??? lv lv

? Infection lv

? Electromagnetic field exposure lv lv lv

? Exposure during pregnancy lv lv lv

? Radiation lv

? Chemical or drug exposure lv lv lv lv

? Familial predisposition lv lv

? Chromosomal aberrations lv lv

T cell ALL - lv lv lv

thymus, anterior mediastinal mass, deviated trachea Clinical Presentat
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ion of Leukemia
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Leukocytosis/leukopenia
Neutropenia- absolute neutrophil count 3,000-5,000 lv lv lv lv

neutropenic if <1000 lv lv l

Lymphoblasts on peripheral smear v lv lv lv

Anemia: usually present at dx, RBCs lysed by overabundance of WBCs
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Thrombocytopenia (75%) petechiae (does not blanch), purpura, mucosal (nasal/scleral) bleeding, (hem lv lv lv lv lv lv lv lv lv lv

aturia & GI hemorrhage are rare) lv lv lv lv lv

Fever (60%): frequently associated with infection
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Pain in bones/joints (23%): due to bone marrow infiltration by leukemic cells (also called blasts) Anorexi
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a (common) & abdominal pain
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Weight loss (infrequent) lv lv

Pallor and mucosal bleeding (48%) lv lv lv lv

Lymphadenopathy(50%): Clavicular and epitrochlear nodes worrisome lv lv lv lv lv

Splenomegaly (63%) lv

Hepatosplenomegaly (68%) lv l

Diagnostic labs NP/LMD’s office
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CBC: will give a high index of suspicion
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Low hgb, low platelets, high or low WBC count lv lv lv lv lv lv lv lv

Serum chemistry: uric acid elevation due to rapid turnover of malignant cells leads to uric acid nephropathy L
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DH - frequently elevated lv lv lv

Lytes - especially if dehydrated lv lv lv lv

Bone marrow aspirate/biopsy: should be done at tertiary care site, ONLY DIFINITIVE DIAGNOSTIC
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Lumbar puncture: Rule out CNS disease lv lv lv lv lv

Tumor markers, DNA testing, special stains Flolv lv lv lv lv lv

w cytometry lv

Cytogenetics
ALL Leukemia Tmtt: Multi-
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agent chemotherapy, 90% complete remission in 29 days (0.01% leukemia cells) Intrathecal chemo thr
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ough spinal tap (maintain trendelenburg to coat brain, remove CSF prior to)
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3-Phases (approx.. 2 yr. Duration): lv lv lv lv

Induction-
introduce chemo, hospitalized Consolidation-
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CNS chemo, weekly spinal taps lv lv lv lv lv

Maintenance- beginning at 6- lv lv lv

8 months, hair returns, tolerable, monthly visits Will get radiation if relapses to CNS,
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WBCs cross blood brain barrier lv lv lv lv

BMT for first relapse lv lv lv



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