7. Investigations:
- CT or MRI of chest, abdomen and pelvis
Treatment/management:
- Surgical treatment: partial or radical nephrectomy
- Immunotherapy with tyrosine kinase inhibitors
9. Wilms tumour
● Also called nephroblastoma
● “A malignant tumour of the kidneys, usually
occurring in children younger than 5 years of
age.”
● named after the German doctor, Dr. Max Wilms, who
first described it in 1899
● Most common primary renal tumour of childhood
● Highly malignant primary embryonal tumour
10. Etiology
● Age group: children between 2 and 5 years of age
● Defect in genes
● Pathogenesis: changes in WT1, WTX genes
17. Autosomal-dominant (adult) polycystic kidney disease
(ADPKD)
- Multiple thin-walled spherical cysts in cortex and
medulla of both kidneys, which cause destruction
of kidney tissue and lead to renal failure
- More common
- Mutation in PKD1 gene on chromosome 16 or
PKD2 on chromosome 4
18. Clinical features:
- Any age from second decade
- Acute flank or loin pain
- Hematuria
- Subarachnoid hemorrhage due to rupture of berry
aneurysm
- Hypertension
- Renal failure
- polycythemia
Investigations:
- USG abdomen
- MRI
- IVU drooping water lily sign
19. Treatment:
- Control hypertension
- Control pain
- Treat UTI
- Renal dialysis or transplantation
- Vaptans: vasopressin receptor antagonists