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Case Reports
. 2019 Apr-Jun;41(2):300-303.
doi: 10.1590/2175-8239-jbn-2018-0081. Epub 2018 Sep 6.

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

[Article in English, Portuguese]
Vinicius Danieli Scarioti  1 , Lucia Tabim de Oliveira  2 , Anye Caroline Mattiello  1 , Nayara Dos Santos Gomes  1
Affiliations
Case Reports

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

[Article in English, Portuguese]
Vinicius Danieli Scarioti et al. J Bras Nefrol. 2019 Apr-Jun.

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.

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Figures

Figure 1
Figure 1. Upper abdomen MRA showing signs of chronic liver disease, polycystic kidneys, and a shunt between the left renal vein and a collateral vessel originating in the splenic vein (*).
Figure 2
Figure 2. Hematoxylin and eosin (H&E) staining - 100x - Ducts with cysts (arrows) covered by cuboidal epithelial cells.
Figure 3
Figure 3. H&E - 40x - Liver cirrhosis defined by large fibrous septa delimiting residual parenchymal nodules (arrows).
See this image and copyright information in PMC

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References

    1. Guay-Woodford LM, Bissler JJ, Braun MC, Bockenhauer D, Cadnapaphornchai MA, Dell KM, et al. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr 2014;165:611-7. - PMC - PubMed
    2. Guay-Woodford LM, Bissler JJ, Braun MC, Bockenhauer D, Cadnapaphornchai MA, Dell KM, et al. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr. 2014;165:611–617. - PMC - PubMed
    1. Büscher R, Büscher A, Weber S, Mohr J, Hegen B, Vester U, et al. Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes. Pediatr Nephrol 2013;29:1915-25. - PubMed
    2. Büscher R, Büscher A, Weber S, Mohr J, Hegen B, Vester U, et al. Clinical manifestations of autosomal recessive polycystic kidney disease (ARPKD): kidney-related and non-kidney-related phenotypes. Pediatr Nephrol. 2013;29:1915–1925. - PubMed
    1. Hartung EA, Guay-Woodford LM. Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics 2014;134:e833-45. - PMC - PubMed
    2. Hartung EA, Guay-Woodford LM. Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 2014;134:e833–e845. - PMC - PubMed
    1. Turkbey B, Ocak I, Daryanani K, Font-Montgomery E, Lukose L, Bryant J, et al. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol 2008;39:100-11. - PMC - PubMed
    2. Turkbey B, Ocak I, Daryanani K, Font-Montgomery E, Lukose L, Bryant J, et al. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF) Pediatr Radiol. 2008;39:100–111. - PMC - PubMed
    1. Telega G, Cronin D, Avner ED. New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications. Pediatr Transplant 2013;17:328-35. - PMC - PubMed
    2. Telega G, Cronin D, Avner ED. New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications. Pediatr Transplant. 2013;17:328–335. - PMC - PubMed

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