Phenotypic definition of cystic fibrosis-related liver disease^[1]

Cystic fibrosis-related liver disease with cirrhosis, with or without portal hypertension

Based on any of the following: Clinical examination, imaging, histology, laparoscopy

Noncirrhotic portal hypertension

Based on histology and imaging or clinical examination

Cystic fibrosis-related liver involvement without cirrhosis or portal hypertension

Patients can have more than 1 of the following features:

Persistent (>6 months) elevation of AST, ALT, or GGT >2 times upper limit of normal

Intermittent elevations of the above laboratory values

Steatosis (histologic determination)

Fibrosis (histologic or elastography determination)

Cholangiopathy (based on ultrasound, MRI, CT, or ERCP)

Ultrasound abnormalities not consistent with cirrhosis

Preclinical – No evidence of liver involvement

Based on clinical examination, laboratory values, and imaging if available

AST: aspartate aminotransferase; ALT: alanine aminotransferase; GGT: gamma-glutamyl transferase; MRI: magnetic resonance imaging; CT: computed tomography; ERCP: endoscopic retrograde cholangiopancreatography.

Reference:

van de Peppel IP, Bertolini A, Jonker JW, et al. Diagnosis, follow-up and treatment of cystic fibrosis-related liver disease. Curr Opin Pulm Med 2017.

Original figure modified for this publication. From: Flass T, Narkewicz MR. Cirrhosis and other liver disease in cystic fibrosis. J Cyst Fibros 2013; 12:116. Table used with the permission of Elsevier Inc. All rights reserved.

Graphic 114904 Version 2.0

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Phenotypic definition of cystic fibrosis-related liver disease[1]

Phenotypic definition of cystic fibrosis-related liver disease^[1]