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 nx nx nx

PEDIATRIC CANCER nx

Wilms Tumor : Unilateral (more common) or bilateral tumor of kidne
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y Most common renal neoplasm in children, good prognosis
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Median age @ dx: 2-3 yrs nx nx nx nx nx

Clinical Presentation nx

Palpable abd. mass in healthy child, vascularized encapsulated jelly-
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like (RUPTURE), regular borders Some have pain, microscopic or gross hematuria, malaise, fever,
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HTN x

G/U abnl’s: ambig genitalia cryptorchidism
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hypospadius Congenital hem nx nx

ihypertrophy
Aniridia
Assoc.syndrome: Beckwith- nx

Wiedeman Treatment: 80% good prognosi nx nx nx nx

s
Unilateral: Nephrectomy nx

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

15months Bilateral: Bilateral partial resection, if possibl nx nx nx nx nx nx

e
Same therapy with addition of adriamycin nx nx nx nx nx

Remove kidney with largest tumor, abdominal radiation for remaining Che nx nx nx nx nx nx nx nx nx

motherapy complications:nx

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. nx nx nx nx nx nx

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

Treatment: hydration to increase urine production, correct electrolyte imbalance
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s Goal: prevent renal failure
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Management of Infusion Devices nx nx nx

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 nx nx nx nx

Monitor lines for infection (must culture if febrile) M
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aintain patency by heparin flushing q 4 weeks nx nx nx nx nx nx nx

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

Headaches: Worse in am or nocturnal nx nx nx nx nx

Vomiting: Without nausea
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Increased ICP: nx

Personality changes nx

Drowsiness nx

Resistance to being held nx nx nx

Increased FOC: Delayed closure or elevation of anterior fontan
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el Loss of developmental milestones
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Papilledema nx

Head tilt nx

Abnormal reflexes and speech nx nx nx nx

Cranial nerve abnormalities nx nx

Presentation varies with site, age, developmental level 6 nx nx nx nx nx nx nx

0% infratentorial (cerebellum or brain stem)
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Diploplia
Gait disturbance Gait ataxia, truncal ataxia Nystag nx nx nx nx nx nx

mus
Weakness
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,40% supratentorial (cerebral hemispheres, hypothalamus, thalamus, optic chiasm, pineal region) Sei
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zures
Headaches
Infants: Irritability, Listlessness, Vomiting
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, Diagnosis: Head CT or MRI nx nx nx nx

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

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 rat
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e Accounts for 75% of all childhood CA
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5-
year relative survival with ALL diagnosed in 2000 is near 80%
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Males > females (1.2:1)
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Peak age 2-3yrs nx nx

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

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

? Infection nx

? Electromagnetic field exposure
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? Exposure during pregnancy nx nx nx

? Radiation nx

? Chemical or drug exposure nx nx nx nx

? Familial predisposition nx nx

? Chromosomal aberrations nx nx

T cell ALL -
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thymus, anterior mediastinal mass, deviated trachea Clinical Presen
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tation of Leukemia
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Leukocytosis/leukopenia
Neutropenia- absolute neutrophil count 3,000-5,000 nx nx nx nx

neutropenic if <100 nx nx

0 Lymphoblasts on peripheral smear
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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, (h
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ematuria & GI hemorrhage are rare) nx nx nx nx nx

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) An
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orexia (common) & abdominal pain
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Weight loss (infrequent) nx nx

Pallor and mucosal bleeding (48%)
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Lymphadenopathy(50%): Clavicular and epitrochlear nodes worrisome nx nx nx nx nx

Splenomegaly (63%) nx

Hepatosplenomegaly (68%) nx

Diagnostic labs NP/LMD’s offic
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e
CBC: will give a high index of suspicion
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Low hgb, low platelets, high or low WBC count
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Serum chemistry: uric acid elevation due to rapid turnover of malignant cells leads to uric acid nephropat
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hy LDH - frequently elevated
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Lytes - especially if dehydrated
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Bone marrow aspirate/biopsy: should be done at tertiary care site, ONLY DIFINITIVE DIAGNOST
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IC Lumbar puncture: Rule out CNS disease
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Tumor markers, DNA testing, special stains Fl
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ow cytometry nx

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

Induction-
introduce chemo, hospitalized Consolidation
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- CNS chemo, weekly spinal taps
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Maintenance- beginning at 6- nx nx nx

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