A baby of weight 2 kg born at 30 weeks, for a 19 year old primigravida died after 48 hours. Apgar scores were 5 and 8 at 1 and 5 minutes. On autopsy bilateral enlarged kidney with multiple radially arranged cysts were found. Which of the following finding is expected to be associated with it?
Correct Answer: Hepatic cyst and fibrosis
Description: Autosomal recessive polycystic kidney disease is increasingly diagnosed by prenatal ultrasound. In its most severe form, the cystic kidneys are nonfunctional in utero, and, therefore, newborns can have Potter facies and other complications of oligohydramnios. In infancy and childhood, kidney enlargement by cysts may initially be recognized by abdominal palpation of renal masses. Hypeension is an early problem. The rate of the progression of renal insufficiency varies, as does growth failure and other complications of chronic renal failure. Two genes (ADPKD1 and ADPKD2) account for 80% and 10% of cases of autosomal dominant polycystic kidney disease, respectively. The other most notable feature of ARPKD is liver involvement disease in the liver is typically diffuse presenting as poal and interlobular fibrosis. Liver involvement is characterized by bile duct proliferation and ectasia as well as by hepatic fibrosis. Ref: Lum G.M. (2012). Chapter 24. Kidney & Urinary Tract. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.
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