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Supplementary material-CARE_ADPKD.pdf (2.15 MB)

Supplementary Material for: Unveiling Neglected Concerns: possible severe hepatic complications after nephrectomy in autosomal dominant polycystic kidney disease. A case report.

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posted on 2024-06-06, 07:15 authored by DeRosa L.I., Catania M., Tunesi F., Vespa M., Bucci R., Kola K., Vezzoli G., SciarroneAlibrandi M.T.
Introduction Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease and the 4th leading cause of renal replacement therapy in the world. ADPKD is a systemic disorder as cysts may develop in several organs. Liver cysts are the most common extrarenal manifestations and are often incidentally detected. Even though cysts do not influence liver's function, they can grow to a very great size and can significantly enlarge liver volume, causing structural distortion of the biliary tree and cause patient discomfort due to the mass effect. Nephrectomy is frequently considered in preparation of renal transplantation in patients with remarkable kidneys’ enlargement. There are currently no globally recognized clinical guidelines for nephrectomy. Although cysts do not normally affect liver function in ADPKD, after nephrectomy cases of liver fibrosis and Budd Chiari have been reported. These are uncommon disorders due to the obstruction of the blood flow in the hepatic venous causing spleen and liver volume enlargement, portal hypertension and hepatic cirrhosis. Case presentation We present a case of hepatic fibrosis with splenomegaly and severe pancytopenia as a tardive complication after bilateral nephrectomy in 47 years old ADPKD patient. Conclusion This finding underscores the critical significance of meticulously examining the anatomical relationship between polycystic kidneys and the liver before performing nephrectomy. Additionally, it highlights the importance of assessing liver involvement and associated complications. By integrating liver assessment into the criteria, we can significantly enhance patient care and improve the overall management of ADPKD before kidney transplantation.

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    Case Reports in Nephrology and Dialysis

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