A teenage patient with autosomal recessive polycystic kidney disease, a splenorenal shunt, and congenital hepatic fibrosis: a case report

ABSTRACT A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an end...

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Bibliographic Details
Main Authors: Vinicius Danieli Scarioti, Lucia Tabim de Oliveira, Anye Caroline Mattiello, Nayara dos Santos Gomes
Format: Article
Language:English
Published: Sociedade Brasileira de Nefrologia 2018-09-01
Series:Brazilian Journal of Nephrology
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0101-28002018005031102&lng=en&tlng=en
Description
Summary:ABSTRACT A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.
ISSN:2175-8239