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Brazilian Journal of Nephrology

Print version ISSN 0101-2800On-line version ISSN 2175-8239

Abstract

SCARIOTI, Vinicius Danieli; OLIVEIRA, Lucia Tabim de; MATTIELLO, Anye Caroline  and  GOMES, Nayara dos Santos. A teenage patient with autosomal recessive polycystic kidney disease, a splenorenal shunt, and congenital hepatic fibrosis: a case report. J. Bras. Nefrol. [online]. In press. , pp.-.  Epub Sep 06, 2018. ISSN 0101-2800.  http://dx.doi.org/10.1590/2175-8239-jbn-2018-0081.

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.

Keywords : Polycystic Kidney, Autosomal Recessive; Liver Cirrhosis; Adolescent.

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