LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT
Autor(a) principal: | |
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Data de Publicação: | 2021 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) |
Texto Completo: | http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/764 |
Resumo: | Introduction: Low-phospholipid-associated cholelithiasis syndrome (LPAC) is a rare form of intrahepatic lithiasis linked to a defect of phospholipid canalicular secretion into bile. LPAC is associated with a phenotype of ABCB4/MDR3 gene mutation and it’s defined by the recurrence of biliary pain, acute cholangitis, or pancreatitis after cholecystectomy. Objectives: Report a case of LPAC to expand the knowledge of this pathology to physicians and health professionals to benefit patients. Case report: Patient, feminine, 39 years old, sought medical attention with pain on the right hypochondrium associated with nausea and vomit that initiated two days ago. The patient reported the first episode of acute pancreatitis in 2019 and six more episodes afterward. In the last episode, on April 2020, cholecystectomy was performed during hospitalization. Three more episodes of acute pancreatitis occurred since the surgery. Elevated lipase levels appeared on laboratory evaluation. Therefore, the diagnosis of acute pancreatitis and LPAC were made based on clinical and laboratory signs and clinical signs respectively. Conclusion: Althought there is few articles about LPAC syndrome, it must be reminded as a differential diagnosis in cases of recurrence of biliary pain, acute pancreatitis after cholecystectomy, and when these events occur in young patients with no risk factors. The understanding of this disease will happen with the awareness and wide dissemination of diagnostic criteria, which will facilitate the screening and treatment of patients. Key-words: Cholestasis; Ursodeoxycholic Acid, Cholelithiasis. |
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LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORTLow-Phospholipid-Associated Cholelithiasis Syndrome (LPAC): quando suspeitar? Relato de caso / Low-Phospholipid-Associated Cholelithiasis Syndrome (LPAC): when to suspect? Case reportIntroduction: Low-phospholipid-associated cholelithiasis syndrome (LPAC) is a rare form of intrahepatic lithiasis linked to a defect of phospholipid canalicular secretion into bile. LPAC is associated with a phenotype of ABCB4/MDR3 gene mutation and it’s defined by the recurrence of biliary pain, acute cholangitis, or pancreatitis after cholecystectomy. Objectives: Report a case of LPAC to expand the knowledge of this pathology to physicians and health professionals to benefit patients. Case report: Patient, feminine, 39 years old, sought medical attention with pain on the right hypochondrium associated with nausea and vomit that initiated two days ago. The patient reported the first episode of acute pancreatitis in 2019 and six more episodes afterward. In the last episode, on April 2020, cholecystectomy was performed during hospitalization. Three more episodes of acute pancreatitis occurred since the surgery. Elevated lipase levels appeared on laboratory evaluation. Therefore, the diagnosis of acute pancreatitis and LPAC were made based on clinical and laboratory signs and clinical signs respectively. Conclusion: Althought there is few articles about LPAC syndrome, it must be reminded as a differential diagnosis in cases of recurrence of biliary pain, acute pancreatitis after cholecystectomy, and when these events occur in young patients with no risk factors. The understanding of this disease will happen with the awareness and wide dissemination of diagnostic criteria, which will facilitate the screening and treatment of patients. Key-words: Cholestasis; Ursodeoxycholic Acid, Cholelithiasis.Introdução: A Síndrome LPAC (Low-phospholipid-associated cholelithiasis syndrome) é uma forma rara de colelitíase intra-hepática, associada ao defeito na secreção canalicular de fosfolipídios para a bile. Faz parte do espectro de doenças hepáticas associadas a deficiências do ABCB4/ MDR3 e é caracterizada por eventos recorrentes de cólica biliar, colangite aguda ou pancreatite em pacientes após a colecistectomia. Objetivo: Relatar um caso suspeito de LPAC de forma a expandir o conhecimento da doença. Relato de Caso: Paciente feminino, 39 anos, procurou atendimento por quadro de dor em região de hipocôndrio direito comirradiação para dorso, associada a náuseas e vômitos há dois dias. Relatou primeiro episódio de pancreatite aguda em 2019, com seis episódios subsequentes, sendo o últimoem abril de 2020 quando foi submetida à colecistectomia videolaparoscópica durante a internação. Desde então, referiu outros três episódios. Alterações laboratoriais incluíamaumento significativo de lipase. Baseado nos critérios clínicos e laboratoriais, estabeleceu-se diagnóstico de pancreatite aguda e por critérios clínicos suspeita-se do diagnóstico deLPAC. Conclusão: A síndrome LPAC, apesar de pouco documentada na literatura, deve ser lembrada como hipótese diagnóstica em casos de cólica biliar e pancreatite de origem biliar, que recorrem mesmo após a colecistectomia ou se manifestam em pacientes jovens sem fatores de risco clássicos para tais doenças.Palavras chave: Colestase, Ácido ursodesoxicólico, Colelitíase ABSTRACTIntroduction: Low-phospholipid-associated cholelithiasis syndrome (LPAC) is a rare form of intrahepatic lithiasis linked to a defect of phospholipid canalicular secretion intobile. It is part of the spectrum of liver diseases associated with ABCB4/MDR3 deficiencies and is characterized by recurrent events of biliary colic, acute cholangitis, or pancreatitis in patients after cholecystectomy. Objectives: To report a suspected case of LPAC to expand the knowledge of this pathology. Case report: Patient, female, 39 years old, sought medical attention with pain on the right hypochondrium associated with nausea and vomit that initiated two days ago. The patient reported the first episode of acute pancreatitis in 2019 and six more episodes afterward. In the last episode, on April 2020, cholecystectomy was performed during hospitalization. Three more episodes of acute pancreatitis occurred since then. Elevated lipase levels appeared on laboratory evaluation. Therefore, the diagnosis of acute pancreatitis was made based on clinical and laboratory signs and the diagnosis of LPAC was suspected based on clinical signs. Conclusion: Althought there is few articles about LPAC syndrome it must be reminded as a differential diagnosis in cases of recurrence of biliary pain, acute pancreatitis after cholecystectomy, and when these events occur in young patients with no classic risk factors for such diseases.Keywords: Cholestasis, Ursodeoxycholic acid, CholelithiasisFaculdade de Ciências Médicas da Santa Casa de São Paulo2021-10-29info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfapplication/pdfhttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/76410.26432/1809-3019.2021.66.035Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo; v. 66 (2021): Jan/Dez; 1 of 51809-30190101-6067reponame:Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online)instname:Faculdade de Ciências Médicas da Santa Casa de São Pauloinstacron:FCMSCSPporhttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/764/1092http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/764/1090Copyright (c) 2021 Leticia Scopel Miossi, Lara Pin Venturini , Julia Dal Bem Assad , Jessica Martins Torres, Lívia Zardo Trindade , Mariana Poltronieri Pachecohttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessMiossi, Leticia ScopelVenturini , Lara PinAssad , Julia Dal Bem Torres, Jessica MartinsTrindade , Lívia Zardo Pacheco, Mariana Poltronieri 2021-11-26T12:27:57Zoai:ojs2.arquivosmedicos.fcmsantacasasp.edu.br:article/764Revistahttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSPONGhttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/oaiarquivosmedicos@fcmsantacasasp.edu.br||1809-30190101-6067opendoar:2021-11-26T12:27:57Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) - Faculdade de Ciências Médicas da Santa Casa de São Paulofalse |
dc.title.none.fl_str_mv |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT Low-Phospholipid-Associated Cholelithiasis Syndrome (LPAC): quando suspeitar? Relato de caso / Low-Phospholipid-Associated Cholelithiasis Syndrome (LPAC): when to suspect? Case report |
title |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT |
spellingShingle |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT Miossi, Leticia Scopel |
title_short |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT |
title_full |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT |
title_fullStr |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT |
title_full_unstemmed |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT |
title_sort |
LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT |
author |
Miossi, Leticia Scopel |
author_facet |
Miossi, Leticia Scopel Venturini , Lara Pin Assad , Julia Dal Bem Torres, Jessica Martins Trindade , Lívia Zardo Pacheco, Mariana Poltronieri |
author_role |
author |
author2 |
Venturini , Lara Pin Assad , Julia Dal Bem Torres, Jessica Martins Trindade , Lívia Zardo Pacheco, Mariana Poltronieri |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Miossi, Leticia Scopel Venturini , Lara Pin Assad , Julia Dal Bem Torres, Jessica Martins Trindade , Lívia Zardo Pacheco, Mariana Poltronieri |
description |
Introduction: Low-phospholipid-associated cholelithiasis syndrome (LPAC) is a rare form of intrahepatic lithiasis linked to a defect of phospholipid canalicular secretion into bile. LPAC is associated with a phenotype of ABCB4/MDR3 gene mutation and it’s defined by the recurrence of biliary pain, acute cholangitis, or pancreatitis after cholecystectomy. Objectives: Report a case of LPAC to expand the knowledge of this pathology to physicians and health professionals to benefit patients. Case report: Patient, feminine, 39 years old, sought medical attention with pain on the right hypochondrium associated with nausea and vomit that initiated two days ago. The patient reported the first episode of acute pancreatitis in 2019 and six more episodes afterward. In the last episode, on April 2020, cholecystectomy was performed during hospitalization. Three more episodes of acute pancreatitis occurred since the surgery. Elevated lipase levels appeared on laboratory evaluation. Therefore, the diagnosis of acute pancreatitis and LPAC were made based on clinical and laboratory signs and clinical signs respectively. Conclusion: Althought there is few articles about LPAC syndrome, it must be reminded as a differential diagnosis in cases of recurrence of biliary pain, acute pancreatitis after cholecystectomy, and when these events occur in young patients with no risk factors. The understanding of this disease will happen with the awareness and wide dissemination of diagnostic criteria, which will facilitate the screening and treatment of patients. Key-words: Cholestasis; Ursodeoxycholic Acid, Cholelithiasis. |
publishDate |
2021 |
dc.date.none.fl_str_mv |
2021-10-29 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/764 10.26432/1809-3019.2021.66.035 |
url |
http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/764 |
identifier_str_mv |
10.26432/1809-3019.2021.66.035 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/764/1092 http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/764/1090 |
dc.rights.driver.fl_str_mv |
https://creativecommons.org/licenses/by/4.0 info:eu-repo/semantics/openAccess |
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https://creativecommons.org/licenses/by/4.0 |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf application/pdf |
dc.publisher.none.fl_str_mv |
Faculdade de Ciências Médicas da Santa Casa de São Paulo |
publisher.none.fl_str_mv |
Faculdade de Ciências Médicas da Santa Casa de São Paulo |
dc.source.none.fl_str_mv |
Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo; v. 66 (2021): Jan/Dez; 1 of 5 1809-3019 0101-6067 reponame:Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) instname:Faculdade de Ciências Médicas da Santa Casa de São Paulo instacron:FCMSCSP |
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Faculdade de Ciências Médicas da Santa Casa de São Paulo |
instacron_str |
FCMSCSP |
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FCMSCSP |
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Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) |
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Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) |
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Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) - Faculdade de Ciências Médicas da Santa Casa de São Paulo |
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arquivosmedicos@fcmsantacasasp.edu.br|| |
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