All of the following are true about childhood polycystic kidney disease, except
Childhood polycystic kidney disease. Autosomal recessive Caused by mutation in PKHD1 gene encoding fibrocystin or polyductin. Characterised by fusiform dilatation of collecting tubules which are arranged radially from coex to medulla. Affected children usually present in neonatal period with oliguria, respiratory insufficiency and palpable kidneys. It is sometimes diagnosed in young children presenting with hypeension, renal insufficiency and enlarged kidneys or with poal hypeension due to associated congenital hepatic fibrosis. USG shows enlarged bright kidneys . Children born with autosomal recessive PKD may develop kidney failure within a few years and often experience the following: High blood pressure Urinary tract infections Frequent urination The disease also usually affects the liver, spleen, and pancreas, resulting in low blood cell counts, varicose veins, and hemorrhoids. Reference: GHAI essential Paediatrics
Autosomal dominant
Pulmonary hypoplasia
Renal cyst present at bih
Hepatic fibrosis
Urinary tract