You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information.
What is All of the following are true about childhood polycystic kidney disease, except and explain in detail?
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