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Combined liver-kidney transplant in polycystic diseases: a case report
Polycystic liver disease, a hereditary pathology, usually manifests as autosomal dominant polycystic kidney disease. The many cysts in the liver cause massive hepatomegaly, majorly affecting the patient’s quality of life. In cases of refractory symptoms, liver transplantation is the only treatment c...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Instituto Israelita de Ensino e Pesquisa Albert Einstein
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10219621/ https://www.ncbi.nlm.nih.gov/pubmed/37255062 http://dx.doi.org/10.31744/einstein_journal/2023RC0282 |
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author | da Fonseca, Olival Cirilo Lucena Martins, Beatriz Costa Nava Jucá, Norma Thomé de Sá, Victor Cruz Rosa Alencar Rabêlo, Priscylla Jennie Monteiro de Melo, Paulo Sérgio Vieira Amorim, Américo Gusmão Lacerda, Cláudio Moura |
author_facet | da Fonseca, Olival Cirilo Lucena Martins, Beatriz Costa Nava Jucá, Norma Thomé de Sá, Victor Cruz Rosa Alencar Rabêlo, Priscylla Jennie Monteiro de Melo, Paulo Sérgio Vieira Amorim, Américo Gusmão Lacerda, Cláudio Moura |
author_sort | da Fonseca, Olival Cirilo Lucena |
collection | PubMed |
description | Polycystic liver disease, a hereditary pathology, usually manifests as autosomal dominant polycystic kidney disease. The many cysts in the liver cause massive hepatomegaly, majorly affecting the patient’s quality of life. In cases of refractory symptoms, liver transplantation is the only treatment choice. A 43-year-old woman was followed up as a hepatology outpatient in August 2020, with a progressive increase in abdominal volume, lower limb edema, and cachexia. The patient was diagnosed with polycystic renal and liver disease with massive hepatomegaly in March 2021, a combined kidney-liver transplant. Liver size represented 13% of the patient’s corporal composition, weighing 8.6kg. The patient was discharged on the 7(th) postoperative day with no complications. Only 10-20% of patients with polycystic liver disease have clinical manifestations, most of which result from hepatomegaly. An increase in liver volume deteriorates liver function until the condition becomes end-stage liver disease, as kidney function is already compromised; liver-kidney transplantation remains the only treatment choice. The case described drew significant attention to the massive hepatomegaly presented in the patient, with the liver representing over 10% of the patient’s body weight, approximately five to six times larger than a normal-sized liver. |
format | Online Article Text |
id | pubmed-10219621 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Instituto Israelita de Ensino e Pesquisa Albert Einstein |
record_format | MEDLINE/PubMed |
spelling | pubmed-102196212023-05-27 Combined liver-kidney transplant in polycystic diseases: a case report da Fonseca, Olival Cirilo Lucena Martins, Beatriz Costa Nava Jucá, Norma Thomé de Sá, Victor Cruz Rosa Alencar Rabêlo, Priscylla Jennie Monteiro de Melo, Paulo Sérgio Vieira Amorim, Américo Gusmão Lacerda, Cláudio Moura Einstein (Sao Paulo) Case Report Polycystic liver disease, a hereditary pathology, usually manifests as autosomal dominant polycystic kidney disease. The many cysts in the liver cause massive hepatomegaly, majorly affecting the patient’s quality of life. In cases of refractory symptoms, liver transplantation is the only treatment choice. A 43-year-old woman was followed up as a hepatology outpatient in August 2020, with a progressive increase in abdominal volume, lower limb edema, and cachexia. The patient was diagnosed with polycystic renal and liver disease with massive hepatomegaly in March 2021, a combined kidney-liver transplant. Liver size represented 13% of the patient’s corporal composition, weighing 8.6kg. The patient was discharged on the 7(th) postoperative day with no complications. Only 10-20% of patients with polycystic liver disease have clinical manifestations, most of which result from hepatomegaly. An increase in liver volume deteriorates liver function until the condition becomes end-stage liver disease, as kidney function is already compromised; liver-kidney transplantation remains the only treatment choice. The case described drew significant attention to the massive hepatomegaly presented in the patient, with the liver representing over 10% of the patient’s body weight, approximately five to six times larger than a normal-sized liver. Instituto Israelita de Ensino e Pesquisa Albert Einstein 2023-05-23 /pmc/articles/PMC10219621/ /pubmed/37255062 http://dx.doi.org/10.31744/einstein_journal/2023RC0282 Text en https://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report da Fonseca, Olival Cirilo Lucena Martins, Beatriz Costa Nava Jucá, Norma Thomé de Sá, Victor Cruz Rosa Alencar Rabêlo, Priscylla Jennie Monteiro de Melo, Paulo Sérgio Vieira Amorim, Américo Gusmão Lacerda, Cláudio Moura Combined liver-kidney transplant in polycystic diseases: a case report |
title | Combined liver-kidney transplant in polycystic diseases: a case report |
title_full | Combined liver-kidney transplant in polycystic diseases: a case report |
title_fullStr | Combined liver-kidney transplant in polycystic diseases: a case report |
title_full_unstemmed | Combined liver-kidney transplant in polycystic diseases: a case report |
title_short | Combined liver-kidney transplant in polycystic diseases: a case report |
title_sort | combined liver-kidney transplant in polycystic diseases: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10219621/ https://www.ncbi.nlm.nih.gov/pubmed/37255062 http://dx.doi.org/10.31744/einstein_journal/2023RC0282 |
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