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Cystic fibrosis related liver disease and endocrine considerations.
Sherwood, Jordan S; Ullal, Jagdeesh; Kutney, Katherine; Hughan, Kara S.
Afiliação
  • Sherwood JS; Department of Pediatrics, Diabetes Research Center, Division of Pediatric Endocrinology, Massachusetts General Hospital, 55 Fruit St, Boston, MA 02114, United States.
  • Ullal J; Department of Medicine, UPMC Center for Diabetes and Endocrinology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, United States.
  • Kutney K; Department of Pediatrics, Case Western Reserve University, Cleveland, OH 44106, United States.
  • Hughan KS; Department of Pediatrics, Division of Pediatric Endocrinology and Diabetes, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA 15224, United States.
J Clin Transl Endocrinol ; 27: 100283, 2022 Mar.
Article em En | MEDLINE | ID: mdl-35024343
ABSTRACT
Cystic fibrosis-liver disease (CFLD) is one of the most common non-pulmonary complications in the CF population, is associated with significant morbidity and represents the third leading cause of mortality in those with CF. CFLD encompasses a broad spectrum of hepatobiliary manifestations ranging from mild transaminitis, biliary disease, hepatic steatosis, focal biliary cirrhosis and multilobular biliary cirrhosis. The diagnosis of CFLD and prediction of disease progression remains a clinical challenge. The identification of novel CFLD biomarkers as well as the role of newer imaging techniques such as elastography to allow for early detection and intervention are active areas of research focus. Biliary cirrhosis with portal hypertension represents the most severe spectrum of CFLD, almost exclusively develops in the pediatric population, and is associated with a decline in pulmonary function, poor nutritional status, and greater risk of hospitalization. Furthermore, those with CFLD are at increased risk for vitamin deficiencies and endocrinopathies including CF-related diabetes, CF-related bone disease and hypogonadism, which can have further implications on disease outcomes and management. Effective treatment for CFLD remains limited and current interventions focus on optimization of nutritional status, identification and treatment of comorbid conditions, as well as early detection and management of CFLD specific sequelae such as portal hypertension or variceal bleeding. The extent to which highly effective modulator therapies may prevent the development or modify the progression of CFLD remains an active area of research. In this review, we discuss the challenges with defining and evaluating CFLD and the endocrine considerations and current management of CFLD.
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Texto completo: 1 Base de dados: MEDLINE Tipo de estudo: Prognostic_studies / Screening_studies Idioma: En Ano de publicação: 2022 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Tipo de estudo: Prognostic_studies / Screening_studies Idioma: En Ano de publicação: 2022 Tipo de documento: Article