A Rare Association of Autosomal Recessive Polycystic Kidney (ARPKD) Disease with AV Canal Defect


Case Report

Author Details : Devendra Nema, Rahul Sinha, K. Venkatnarayan, Shamsher Dalal

Volume : 2, Issue : 1, Year : 2016

Article Page : 42-45


Suggest article by email

Get Permission

Abstract

ARPKD is an autosomal recessive condition characterized by cystic dilations of the renal collecting tubules. The incidence of ARPKD is estimated to be between 1 in 10,000 - 40,000. It is the most common childhood-onset ciliopathy. The majority of patients present in infancy, although presentation can occasionally be as late as early adulthood. Extrarenal manifestations of this disorder include congenital hepatic fibrosis or Caroli disease, which involves non-obstructive dilation of intrahepatic bile ducts. We report a rare association of autosomal recessive polycystic kidney disease (ARPKD) with AV canal defect in a 3month old infant who presented with failure to thrive, abdominal distension and respiratory distress.

Key Words: Autosomal recessive, AV canal, Cyst, Polycystic


How to cite : Nema D, Sinha R, Venkatnarayan K, Dalal S, A Rare Association of Autosomal Recessive Polycystic Kidney (ARPKD) Disease with AV Canal Defect. IP Int J Med Paediatr Oncol 2016;2(1):42-45


This is an Open Access (OA) journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.







View Article

PDF File  


Copyright permission

Get article permission for commercial use

Downlaod

PDF File    






Article Access statistics

Viewed: 1598

PDF Downloaded: 567