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| Hepatic, pancreatic and renal manifestations of a ciliopathy |
| Sreelakshmi Kotha ∗ , Philip Berry |
Department of Gastroenterology, Guy’s and St Thomas’ Foundation Trust, London, UK
∗ Corresponding author.
E-mail address: sreelakshmi_kotha@yahoo.com (S. Kotha). |
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Abstract Hepatorenal fibrocystic diseases are inherited disorders, characterized by developmental abnormalities and fibrocystic degeneration of the portobiliary system and kidneys [1]. They are part of a larger group of disorders referred to as “ciliopathies” which affect 1 in 1000 people. Polycystic kidney disease is the most common kidney manifestation, while others include nephronophthisis, glomerulocystic disease and cystic dysplasia. Hepatic manifestations include Caroli’s disease, congenital hepatic fibrosis and polycystic liver disease.
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2021, Vol. 20 
