PEDIATRICS QUESTIONS OF AKU UNIVERSITY

SHORT NOTES ON

A) MEGALOBLASTIC ANEMIA
b) Diagnosis of RF
c) KMC Kangaroo Mother Care
d) Diagnosis and Management of NEONATAL Sepsis
e) Lab findings and treatment of Iron Deficiency Anemia
f) Clinical manifestation and management of congenital hypothyroidism
g) Clinical manifestation and management of Nutritional Rickets
h) Management of Acute Glomerulonephritis
I) Treatment of severe Acute Asthma
j) Management of RDS in neonates
k) Treatment of scorpion sting Envenomation (2x)
l) Management of acute bacterial Meningitiis (2x)
m) Management of severe Malaria
n) Management of neonatal hypothermia
o) Management of hypercyanotic spells
p) Clinical features and Treatment of Scurvy
q) Management of severe acute Dehydration in one year old child
r) SN on Nutrional rickets
s) Management of Status epilepticus
t) Treatment of organophospate poisoning
u) Wilms Tumor
v) Treatment and Management of ALL.



WILMS TUMOR (High yeild)


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Wilm’s Tumor

• Wilm’s tumor: MC primary renal tumor of childhood (2-5 years)Q.

• Wilm’s tumor: 2nd MC malignant abdominal tumor in children (MC is neuroblastoma).

 IMP• Arise from kidney, composed of three elements- blastema, epithelium and stromaQ. (Best)

 • MC presenting feature is asymptomatic abdominal mass or swellingQ. • Mostly unilateral.

 • Characterized by triad of abdominal mass, fever and microscopic hematuriaQ.

• Fever typically resolve after tumor resection

Associated malformations

• WAGR SyndromeQ: It consists of aniridia, genital anomalies and mental retardation. The risk of Wilm’s tumor is increased by 33% in this syndromeQ. Associated with WT-1 gene deletion cocated on chromosQ 11p 13

 • Denys-Drash SyndromeQ: It consists of gonadal dysgenesis (Male pseudohermaphroditism), nephropathy leading to renal failure. Majority of patients with this syndrome have renal failure.

• Beckwith-Wiedmann SyndromeQ: It consists of enlargement of body organs, hemi-hypertrophy, renal medullary cysts and abnormal large cells in adrenal cortex, macroglossia, omphalocele, hepatoblastoma. Associated with WT-2 gene deletion located on chromosome 11p 15.5.

Diagnosis   

• USG (first investigation)Q or CT abdomen for staging.

• MRI is superior to other imaging modalities in delineating nephroblastomatosis elements. • Calcification tends to be more crescent shaped, discrete and peripheralQ in comparison of finely stippled calcification of neuroblastoma.

Treatment

 • Surgical excision (transperitoneal radical nephrectomy) is treatment of choice.

• Routine exploration of contralateral kidney is not necessary if imaging is satisfactory and doesn’t suggest bilateral process.

 • In unfavorable histology, Radiation therapy should be started within 10 daysQ after nephrectomy, Chemotherapy should be started 5 days after surgeryQ.

 • Chemotherapy: VCD (Vincristine + Cyclophosphamide + Doxorubicin or dactinomycin)

 • Whole lung irradiation is recommended for pulmonary metastasis.

Preoperative treatment should be considered

• Solitary kidneyQ

• BilateralQ renal tumors

• Tumor in horse shoe kidneyQ

• Tumor thrombus in IVC above the level of hepatic veinsQ

 • Respiratory distress due to metastaticQ disease

 Prognosis

 • The histologyQ of Wilm’s tumor and tumor stage is identified as most important determinant of prognosisQ (Histology > Stage).

Wilms Tumor (Nephroblastoma)

Wilms’ tumor is the most common primary renal tumor of childhood in USA. This tumor’s

peak age is 2-5years. The risk of Wilms’ tumor is increased in association with at least three

recognizable groups of congenital malformations:

WAGR syndrome

It is characterized by aniridia, genital anomalies, and mental retardation and a 33% chance of

developing Wilms’ tumor. Patients with WAGR syndrome carry constitutional (germline) deletions of

two genes WT1 and PAX6 both located at chromosome 11p13Q.

Denys-Drash syndrome

It is characterized by gonadal dysgenesis (male pseudohermaphroditism) and early-onset nephropathy

leading to renal failure. The characteristic glomerular lesion in these patients is a diffuse mesangial

sclerosis. These patients also have germline abnormalities in WT1. In addition to Wilms’ tumors these

individuals are also at increased risk for developing germ-cell tumors called gonadoblastomas.

Beckwith-Wiedemann syndrome

It characterized by enlargement of body organs (organomegaly), macroglossia, hemihypertrophy,

omphalocele and abnormal large cells in adrenal cortex (adrenal cytomegaly). The genetic locus

involved in these patients is in band p15.5 of chromosome 11 called “WT2”. In addition to Wilms’

tumors patients with Beckwith-Wiedemann syndrome are also at increased risk for developing

hepatoblastoma, adrenocortical tumors, rhabdomyosarcomas and pancreatic tumors.