Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease
Autor(a) principal: | |
---|---|
Data de Publicação: | 2022 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UNESP |
Texto Completo: | http://dx.doi.org/10.1159/000523662 http://hdl.handle.net/11449/240896 |
Resumo: | Autosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient's age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future. |
id |
UNSP_b1fc6a0868ba5924dd2c3c66455d719a |
---|---|
oai_identifier_str |
oai:repositorio.unesp.br:11449/240896 |
network_acronym_str |
UNSP |
network_name_str |
Repositório Institucional da UNESP |
repository_id_str |
2946 |
spelling |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver DiseaseCase reportHepatic cystsHepatomegalyLaparotomy fenestrationPolycystic liver diseaseAutosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient's age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future.Department of Internal Medicine São Paulo State University (Unesp) Medical SchoolDepartment of Radiology São Paulo State University (Unesp) Medical SchoolDepartment of Surgery São Paulo State University (Unesp) Medical SchoolDepartment of Internal Medicine São Paulo State University (Unesp) Medical SchoolDepartment of Radiology São Paulo State University (Unesp) Medical SchoolDepartment of Surgery São Paulo State University (Unesp) Medical SchoolUniversidade Estadual Paulista (UNESP)Norcia, Luiz Fernando [UNESP]Watanabe, Erika Mayumi [UNESP]Hasimoto, Claudia Nishida [UNESP]Pelafsky, Leonardo [UNESP]Oliveira, Walmar Kerche de [UNESP]Sassaki, Ligia Yukie [UNESP]2023-03-01T20:37:31Z2023-03-01T20:37:31Z2022-03-31info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article201-208http://dx.doi.org/10.1159/000523662Case Reports in Gastroenterology, v. 16, n. 1, p. 201-208, 2022.1662-0631http://hdl.handle.net/11449/24089610.1159/0005236622-s2.0-85129059661Scopusreponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPengCase Reports in Gastroenterologyinfo:eu-repo/semantics/openAccess2024-08-14T17:23:33Zoai:repositorio.unesp.br:11449/240896Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462024-08-14T17:23:33Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false |
dc.title.none.fl_str_mv |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease |
title |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease |
spellingShingle |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease Norcia, Luiz Fernando [UNESP] Case report Hepatic cysts Hepatomegaly Laparotomy fenestration Polycystic liver disease |
title_short |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease |
title_full |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease |
title_fullStr |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease |
title_full_unstemmed |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease |
title_sort |
Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease |
author |
Norcia, Luiz Fernando [UNESP] |
author_facet |
Norcia, Luiz Fernando [UNESP] Watanabe, Erika Mayumi [UNESP] Hasimoto, Claudia Nishida [UNESP] Pelafsky, Leonardo [UNESP] Oliveira, Walmar Kerche de [UNESP] Sassaki, Ligia Yukie [UNESP] |
author_role |
author |
author2 |
Watanabe, Erika Mayumi [UNESP] Hasimoto, Claudia Nishida [UNESP] Pelafsky, Leonardo [UNESP] Oliveira, Walmar Kerche de [UNESP] Sassaki, Ligia Yukie [UNESP] |
author2_role |
author author author author author |
dc.contributor.none.fl_str_mv |
Universidade Estadual Paulista (UNESP) |
dc.contributor.author.fl_str_mv |
Norcia, Luiz Fernando [UNESP] Watanabe, Erika Mayumi [UNESP] Hasimoto, Claudia Nishida [UNESP] Pelafsky, Leonardo [UNESP] Oliveira, Walmar Kerche de [UNESP] Sassaki, Ligia Yukie [UNESP] |
dc.subject.por.fl_str_mv |
Case report Hepatic cysts Hepatomegaly Laparotomy fenestration Polycystic liver disease |
topic |
Case report Hepatic cysts Hepatomegaly Laparotomy fenestration Polycystic liver disease |
description |
Autosomal dominant polycystic liver disease (ADPLD) is a rare disease with variable clinical presentations, characterized by cystic enlargement of the liver. The diagnosis is made based on family history, patient's age, and liver phenotype and is confirmed by imaging tests. The treatment aims to reduce symptoms caused by the increased liver volume and can be performed by aspiration with sclerotherapy, fenestration, and liver resection. Although ADPLD is a rare disease, it is an important differential diagnosis of cystic diseases such as polycystic kidney disease; therefore, the aim of this article was to present the diagnostic and therapeutic approach of a case of ADPLD and conducting a literature review. This is the case of a 32-year-old male patient, who was hospitalized due to abdominal pain, hepatomegaly, lack of appetite, and weight loss. Imaging propaedeutics showed a significant increase in the liver volume due to hepatic cysts. After a multidisciplinary evaluation, given the clinical changes and the location of the hepatic cysts, fenestration was performed by laparotomy. The postoperative period was uneventful. The treatment was efficient in promoting symptomatic relief and improving the quality of life in this patient. Case reports on this disease are quite limited in the currently available literature, and there are gaps in knowledge with regard to the diagnosis and management of ADPLD. The importance of this article is that it will highlight the limitations in treatment options and allow physicians to make a more informed decision when diagnosing and treating a patient with ADPLD in the future. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-03-31 2023-03-01T20:37:31Z 2023-03-01T20:37:31Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://dx.doi.org/10.1159/000523662 Case Reports in Gastroenterology, v. 16, n. 1, p. 201-208, 2022. 1662-0631 http://hdl.handle.net/11449/240896 10.1159/000523662 2-s2.0-85129059661 |
url |
http://dx.doi.org/10.1159/000523662 http://hdl.handle.net/11449/240896 |
identifier_str_mv |
Case Reports in Gastroenterology, v. 16, n. 1, p. 201-208, 2022. 1662-0631 10.1159/000523662 2-s2.0-85129059661 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Case Reports in Gastroenterology |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
201-208 |
dc.source.none.fl_str_mv |
Scopus reponame:Repositório Institucional da UNESP instname:Universidade Estadual Paulista (UNESP) instacron:UNESP |
instname_str |
Universidade Estadual Paulista (UNESP) |
instacron_str |
UNESP |
institution |
UNESP |
reponame_str |
Repositório Institucional da UNESP |
collection |
Repositório Institucional da UNESP |
repository.name.fl_str_mv |
Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP) |
repository.mail.fl_str_mv |
|
_version_ |
1808128170586537984 |