Effectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease

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Autor(es): dc.contributorUniversidade Estadual Paulista (UNESP)-
Autor(es): dc.creatorNorcia, Luiz Fernando-
Autor(es): dc.creatorWatanabe, Erika Mayumi-
Autor(es): dc.creatorHasimoto, Claudia Nishida-
Autor(es): dc.creatorPelafsky, Leonardo-
Autor(es): dc.creatorOliveira, Walmar Kerche de-
Autor(es): dc.creatorSassaki, Ligia Yukie-
Data de aceite: dc.date.accessioned2025-08-21T20:14:06Z-
Data de disponibilização: dc.date.available2025-08-21T20:14:06Z-
Data de envio: dc.date.issued2023-03-01-
Data de envio: dc.date.issued2023-03-01-
Data de envio: dc.date.issued2022-03-31-
Fonte completa do material: dc.identifierhttp://dx.doi.org/10.1159/000523662-
Fonte completa do material: dc.identifierhttp://hdl.handle.net/11449/240896-
Fonte: dc.identifier.urihttp://educapes.capes.gov.br/handle/11449/240896-
Descrição: dc.descriptionAutosomal 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.-
Descrição: dc.descriptionDepartment of Internal Medicine São Paulo State University (Unesp) Medical School-
Descrição: dc.descriptionDepartment of Radiology São Paulo State University (Unesp) Medical School-
Descrição: dc.descriptionDepartment of Surgery São Paulo State University (Unesp) Medical School-
Descrição: dc.descriptionDepartment of Internal Medicine São Paulo State University (Unesp) Medical School-
Descrição: dc.descriptionDepartment of Radiology São Paulo State University (Unesp) Medical School-
Descrição: dc.descriptionDepartment of Surgery São Paulo State University (Unesp) Medical School-
Formato: dc.format201-208-
Idioma: dc.languageen-
Relação: dc.relationCase Reports in Gastroenterology-
???dc.source???: dc.sourceScopus-
Palavras-chave: dc.subjectCase report-
Palavras-chave: dc.subjectHepatic cysts-
Palavras-chave: dc.subjectHepatomegaly-
Palavras-chave: dc.subjectLaparotomy fenestration-
Palavras-chave: dc.subjectPolycystic liver disease-
Título: dc.titleEffectiveness of Open Fenestration for Autosomal Dominant Polycystic Liver Disease-
Tipo de arquivo: dc.typelivro digital-
Aparece nas coleções:Repositório Institucional - Unesp

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