Hepatobiliary complications in autosomal recessive polycystic kidney disease

Česká verzia

Authors: M. Brndiarová ¹ ; N. Haláčová ¹; M. Kozár ² ; Michal Demeter ³ ; M. Schnierer ³ ; Z. Havlíčeková ¹
Authors place of work: Klinika detí a dorastu JLF UK a UN Martin ¹; Neonatologická klinika JLF UK a UN Martin ²; Interná klinika – gastroenterologická JLF UK a UN Martin ³
Published in the journal: Gastroent Hepatol 2023; 77(6): 495-501
Category:
doi: https://doi.org/10.48095/ccgh2023495

Summary

Introduction: Autosomal recessive polycystic kidney disease (ARPKD) is a rare, phenotypically variable primary ciliopathy. Kidney and liver damage are typical manifestations, and the prognosis of the disease depends on their progression over time. The main extrarenal manifestation is fibrocystic rebuilding of the liver, which is manifested by intrahepatic portal hypertension and cholangitis. Methods: The aim of the retrospective study was to evaluate the incidence and development hepatobiliary manifestations. Results: Eight children with polycystic autosomal recessive kidney disease were included in the group. Renal damage and hypertension were present in all children. Hepatobiliary manifestations were described in five children (62.5%). Thrombocytopenia preceded splenomegaly by one year. The results also include three case reports. Conclusion: Patients with ARPKD should be examined with a focus on the presence of hepatic manifestations of the disease.

Keywords:

osomal recessive polycystic kidney disease – esophageal varices – fibrocystic disorder – thrombocytopenia

Zdroje

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