The Pathophysiology of Biliary Epithelia 2020
DOI: 10.1201/9780367813888-31
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Liver Disease in Cystic Fibrosis

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Cited by 12 publications
(16 citation statements)
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“…Moreover, a distinct consideration accorded to this complication as a result of a previous study [24] which reported an alarming prevalence of 31%. Considering the negative influence of liver failure on the pulmonary outcome in terms of susceptibility to infection [17], lung function deterioration [25] and antibiotics liver toxicity, a special concern should be addressed to early detection of the liver disease [23] and appropriate therapy.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Moreover, a distinct consideration accorded to this complication as a result of a previous study [24] which reported an alarming prevalence of 31%. Considering the negative influence of liver failure on the pulmonary outcome in terms of susceptibility to infection [17], lung function deterioration [25] and antibiotics liver toxicity, a special concern should be addressed to early detection of the liver disease [23] and appropriate therapy.…”
Section: Discussionmentioning
confidence: 99%
“…Cystic fibrosis associated liver disease (CFLD) was diagnosed by the presence of at least two of the following features: hepatomeg-aly+/-splenomegaly, clinically detected and confirmed by ultrasound, persistent elevation of liver function test (more than 2 determinations) and liver parenchyma alteration, detected on ultrasound examination, transient elastography or magnetic resonance imaging, according Colombo criteria [17], for the diagnosis of cystic fibrosis related diabetes (CFRD) the European criteria were used [18].…”
Section: Methodsmentioning
confidence: 99%
“…Liver disease is the third leading cause of death for individuals with CF, accounting for 2.5% of overall mortality. 147,149 Paramount in the surveillance for CFLD is a careful physical examination for hepatomegaly and/or splenomegaly, as serum biomarkers for liver disease can remain normal despite advanced disease. It is recommended that AST, ALT, gammaglutamyltransferase (GGT), alkaline phosphatase (ALP), and platelet count be assessed annually.…”
Section: Cf-related Liver Diseasementioning
confidence: 99%
“…Hepatic steatosis is a common hepatic finding in CF at any age, with reported prevalence of 23 to 70%, although no specific neonatal data are available. 149,159 It is often associated with malnutrition or deficiency of a trace element or mineral (choline, carnitine, essential fatty acids); however, this is not always the case, so could also be secondary to CFTR dysfunction. 149,160,161 It typically presents as hepatomegaly with soft and smooth liver edge to palpation, without signs of chronic liver disease or portal hypertension, possibly in association with elevated transaminases.…”
Section: Hepatic Steatosismentioning
confidence: 99%
“…O fígado e as vias biliares também são afetados na FC e alterações se apresentam de forma variável, incluindo colestase neonatal, elevação de aminotransferases, esteatose hepática, fibrose hepática, cirrose biliar focal e cirrose multilobular, com ou sem hipertensão portal (FLASS; NARKEWICZ, 2013). A doença hepática relacionada à FC ocorre em 5 a 15% dos pacientes (COLOMBO et al, 2006), geralmente na primeira década de vida.…”
Section: Introductionunclassified