Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Instituto Israelita de Ensino e Pesquisa Albert Einstein 2023
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
_version_ 1785049052342124544
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
work_keys_str_mv AT dafonsecaolivalcirilolucena combinedliverkidneytransplantinpolycysticdiseasesacasereport
AT martinsbeatrizcostanava combinedliverkidneytransplantinpolycysticdiseasesacasereport
AT jucanormathome combinedliverkidneytransplantinpolycysticdiseasesacasereport
AT desavictorcruzrosaalencar combinedliverkidneytransplantinpolycysticdiseasesacasereport
AT rabelopriscyllajenniemonteiro combinedliverkidneytransplantinpolycysticdiseasesacasereport
AT demelopaulosergiovieira combinedliverkidneytransplantinpolycysticdiseasesacasereport
AT amorimamericogusmao combinedliverkidneytransplantinpolycysticdiseasesacasereport
AT lacerdaclaudiomoura combinedliverkidneytransplantinpolycysticdiseasesacasereport