Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Liver injury is associated with mortality in sickle cell disease
source: Alimentary Pharmacology & Therapeutics
year: 2015
authors: Feld JJ, Kato GJ, Koh C, Shields T, Hildesheim M, Kleiner DE, Taylor JG 6th, Sandler NG, Douek D, Haynes-Williams V, Nichols JS, Hoofnagle JH, Jake Liang T, Gladwin MT, Heller T
summary/abstract:BACKGROUND:
Increased life expectancy in sickle cell disease (SCD) has resulted in greater recognition of the consequences of repeated intravascular vaso-occlusion and chronic haemolysis to multiple organ systems.
AIM:
To report the long-term consequences of liver dysfunction in SCD.
METHODS:
A cohort of SCD patients was prospectively evaluated at the National Institutes of Health (NIH) Clinical Center. The association of mortality with liver enzymes, parameters of liver synthetic function and iron overload was evaluated using Cox regression.
RESULTS:
Exactly, 247 SCD patients were followed up for 30 months of whom 22 (9%) died. After controlling for predictors, increased direct bilirubin (DB), ferritin, alkaline phosphatase and decreased albumin were independently associated with mortality. In a multivariable model, only high DB and ferritin remained significant. Ferritin correlated with hepatic iron content and total blood transfusions but not haemolysis markers. Forty patients underwent liver biopsies and 11 (28%) had fibrosis. Twelve of 26 patients (48%) had portal hypertension by hepatic venous pressure gradient (HVPG) measurements. All patients with advanced liver fibrosis had iron overload; however, most patients (69%) with iron overload were without significant hepatic fibrosis. Ferritin did not correlate with left ventricular dysfunction by echocardiography. DB correlated with bile acid levels suggesting liver pathology. Platelet count and soluble CD14 correlated with HVPG indicating portal hypertension.
CONCLUSIONS:
Ferritin and direct bilirubin are independently associated with mortality in sickle cell disease. Ferritin likely relates to transfusional iron overload, while direct bilirubin suggests impairment of hepatic function, possibly impairing patients’ ability to tolerate systemic insults.
DOI: 10.1111/apt.13347
read more full text
Related Content
-
Sylvia Benjamin, MB, BS, FRCP, FRCPathSylvia Benjamin is Consultant in Haemato...
-
Priapism (Painful Erections) Associated With Sickle Cell DiseaseThis leaflet explains about priapism ass...
-
Sudam L. Kate, MSc, PhDA true pioneer for sickle cell advocacy,...
-
Sickle cell patients suffer as disparities in care and research persistWhen 9-year-old Jeremy Brown is in pai...
-
Blood Disorders That the Newborn Screen DetectsToday in the United States all newborns ...
-
Citizens for Quality Sickle Cell CareThe New England Sickle Cell Institute at...
-
Rare Diseases and Orphan Products Breakthrough SummitEach year, the NORD Summit, one of the l...
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.