Article ; Online: Liver complications of haemoglobin H disease in adults.
British journal of haematology
2020 Volume 192, Issue 1, Page(s) 171–178
Abstract: Haemoglobin H (HbH) disease is a type of non-transfusion-dependent thalassaemia. This cross-sectional study aimed at determining the prevalence and severity of liver iron overload and liver fibrosis in patients with HbH disease. Risk factors for advanced ...
| Abstract | Haemoglobin H (HbH) disease is a type of non-transfusion-dependent thalassaemia. This cross-sectional study aimed at determining the prevalence and severity of liver iron overload and liver fibrosis in patients with HbH disease. Risk factors for advanced liver fibrosis were also identified. A total of 80 patients were evaluated [median (range) age 53 (24-79) years, male 34%, non-deletional HbH disease 24%]. Patients underwent 'observed' T2-weighted magnetic resonance imaging examination for liver iron concentration (LIC) quantification, and transient elastography for liver stiffness measurement (LSM) and fibrosis staging. In all, 25 patients (31%) had moderate-to-severe liver iron overload (LIC ≥7 mg/g dry weight). The median LIC was higher in non-deletional than in deletional HbH disease (7·8 vs. 2.9 mg/g dry weight, P = 0·002). In all, 16 patients (20%) had advanced liver fibrosis (LSM >7.9 kPa) and seven (9%) out of them had probable cirrhosis (LSM >11.9 kPa). LSM positively correlated with age (R = 0·24, P = 0·03), serum ferritin (R = 0·36, P = 0·001) and LIC (R = 0·28, P = 0·01). In multivariable regression, age ≥65 years [odds ratio (OR) 4·97, 95% confidence interval (CI) 1·52-17·50; P = 0·047] and moderate-to-severe liver iron overload (OR 3·47, 95% CI 1·01-12·14; P = 0·01) were independently associated with advanced liver fibrosis. The findings suggest that regular screening for liver complications should be considered in the management of HbH disease. |
|||||
|---|---|---|---|---|---|---|
| MeSH term(s) | Adult ; Aged ; Cross-Sectional Studies ; Female ; Humans ; Iron/analysis ; Iron Overload/etiology ; Iron Overload/pathology ; Liver Cirrhosis/etiology ; Liver Cirrhosis/pathology ; Liver Diseases/etiology ; Liver Diseases/pathology ; Male ; Middle Aged ; Young Adult ; alpha-Thalassemia/complications ; alpha-Thalassemia/pathology | |||||
| Chemical Substances | Iron (E1UOL152H7) | |||||
| Language | English | |||||
| Publishing date | 2020-10-23 | |||||
| Publishing country | England | |||||
| Document type | Journal Article ; Research Support, Non-U.S. Gov't | |||||
| ZDB-ID | 80077-6 | |||||
| ISSN | 1365-2141 ; 0007-1048 | |||||
| ISSN (online) | 1365-2141 | |||||
| ISSN | 0007-1048 | |||||
| DOI | 10.1111/bjh.17115 | |||||
| Shelf mark |
|
|||||
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
Full text online
More links
Kategorien
In stock of ZB MED Cologne/Königswinter
| Uh III Zs.182: Show issues | Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG) |
Order via subito
This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.