【medical-news】AJRCCM：PiZZ纯合子相关肺疾病患者常伴有慢性肝脏疾病
发布于 2013-07-04 · 浏览 912 · IP 山东山东
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Rationale:α1-Antitrypsin deficiency is one of the most common heritable human diseases,predisposing to liver and lung injury. Significant heterogeneity in phenotypicexpression is well documented, but less is known of the prevalence, severity,and correlates of chronic liver disease among individuals presenting with lungdisease.
bjectives: To determine the frequency of and riskfactors for severe liver fibrosis and cirrhosis among individuals withPiZZ-related lung disease.
Methods: A well-characterized cohort of 57 PiZZ adultsattending a tertiary referral respiratory clinic was screened prospectively forclinical, laboratory, radiologic, and (when appropriate) histologic evidence ofchronic liver disease.
Measurements and Main Results: Thirty-six (63.2%) of 57had a history or clinical findings suggestive of liver disease; or had one ormore abnormalities of liver function, or liver ultrasound, and 24 of theseunderwent liver biopsy. Ten (17.5%) had evidence of severe fibrosis orcirrhosis and were more likely to have higher body mass index (P = 0.04),alanine transaminase (P = 0.0001), alkaline phosphatase (P = 0.0009),prothrombin time (P = 0.0005), and maximal vital capacity (VCmax) (P = 0.04);lower platelet count (P = 0.007); abnormal liver echogenicity (P < 0.001);and splenomegaly (P = 0.001) at ultrasound. Screening with liver ultrasoundprovided a sensitivity and negative predictive value for severe fibrosis orcirrhosis of 100%, as were the specificity and positive predictive value forplatelet count less than or equal to 174,000 per mm3 and splenomegaly. Amongindividuals undergoing liver biopsy, fibrosis stage correlated with increasingVCmax (P = 0.02) and % predicted VCmax (P = 0.05), and decreasing residualvolume/total lung capacity (TLC) (P = 0.02) and % predicted residual volume/TLC(P = 0.05).
Conclusions: Significant chronic liver disease iscommon in PiZZ individuals with lung disease and can be screened effectively bya combination of conventional tests of liver function, platelet count, andliver ultrasound.
bjectives: To determine the frequency of and riskfactors for severe liver fibrosis and cirrhosis among individuals withPiZZ-related lung disease.
Methods: A well-characterized cohort of 57 PiZZ adultsattending a tertiary referral respiratory clinic was screened prospectively forclinical, laboratory, radiologic, and (when appropriate) histologic evidence ofchronic liver disease.
Measurements and Main Results: Thirty-six (63.2%) of 57had a history or clinical findings suggestive of liver disease; or had one ormore abnormalities of liver function, or liver ultrasound, and 24 of theseunderwent liver biopsy. Ten (17.5%) had evidence of severe fibrosis orcirrhosis and were more likely to have higher body mass index (P = 0.04),alanine transaminase (P = 0.0001), alkaline phosphatase (P = 0.0009),prothrombin time (P = 0.0005), and maximal vital capacity (VCmax) (P = 0.04);lower platelet count (P = 0.007); abnormal liver echogenicity (P < 0.001);and splenomegaly (P = 0.001) at ultrasound. Screening with liver ultrasoundprovided a sensitivity and negative predictive value for severe fibrosis orcirrhosis of 100%, as were the specificity and positive predictive value forplatelet count less than or equal to 174,000 per mm3 and splenomegaly. Amongindividuals undergoing liver biopsy, fibrosis stage correlated with increasingVCmax (P = 0.02) and % predicted VCmax (P = 0.05), and decreasing residualvolume/total lung capacity (TLC) (P = 0.02) and % predicted residual volume/TLC(P = 0.05).
Conclusions: Significant chronic liver disease iscommon in PiZZ individuals with lung disease and can be screened effectively bya combination of conventional tests of liver function, platelet count, andliver ultrasound.
最后编辑于 2022-10-09 · 浏览 912
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