title: Interim assessment of liver damage in patients with sickle cell disease using new non-invasive techniques.
abstract: We explored transient elastography (TE) and enhanced liver fibrosis (ELF™) score with standard markers of liver function to assess liver damage in 193 well patients with sickle cell disease (SCD). Patients with HbSS or HbSβ0thalassaemia (sickle cell anaemia, SCA; N = 134), had significantly higher TE results and ELF scores than those with HbSC (N = 49) disease (TE, 6·8 vs. 5·3, P < 0·0001 and ELF, 9·2 vs. 8·6 P < 0·0001). In SCA patients, TE and ELF correlated significantly with age and all serum liver function tests (LFTs). Additionally, (weak) positive correlation was found with lactate dehydrogenase (TE: r = 0·24, P = 0·004; ELF: r = 0·26 P = 0·002), and (weak) negative correlation with haemoglobin (TE: r = -0·25, P = 0·002; ELF: r = -0·25 P = 0·004). In HbSC patients, correlations were weaker or not significant between TE or ELF, and serum LFTs. All markers of iron loading correlated with TE values when corrected for sickle genotype (serum ferritin, β = 0·25, P < 0·0001, total blood transfusion units, β = 0·25, P < 0·0001 and LIC β = 0·32, P = 0·046). The exploratory study suggests that, while TE could have a role, the utility of ELF score in monitoring liver damage in SCD, needs further longitudinal studies.
authors.affiliation: Faculty of Life Sciences & Medicine, Molecular Haematology, King's College London, London, UK.|Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK.
authors.forename: Emma
authors.initials: E
authors.lastname: Drasar
authors.affiliation: Department of Paediatric Hepatology, King's College Hospital, London, UK.
authors.forename: Emer
authors.initials: E
authors.lastname: Fitzpatrick
authors.affiliation: Faculty of Life Sciences & Medicine, Molecular Haematology, King's College London, London, UK.|Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK.
authors.forename: Kate
authors.initials: K
authors.lastname: Gardner
authors.affiliation: Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK.
authors.forename: Moji
authors.initials: M
authors.lastname: Awogbade
authors.affiliation: Department of Paediatric Hepatology, King's College Hospital, London, UK.
authors.forename: Anil
authors.initials: A
authors.lastname: Dhawan
authors.affiliation: Institute of Hepatology, King's College Hospital, London, UK.
authors.forename: Adrian
authors.initials: A
authors.lastname: Bomford
authors.affiliation: Institute of Hepatology, King's College Hospital, London, UK.
authors.forename: Abid
authors.initials: A
authors.lastname: Suddle
authors.affiliation: Faculty of Life Sciences & Medicine, Molecular Haematology, King's College London, London, UK.|Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, UK.
authors.forename: Swee L
authors.initials: SL
authors.lastname: Thein
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000473:pathology; D054459:Elasticity Imaging Techniques; D005260:Female; D006450:Hemoglobin SC Disease; D006451:Hemoglobin, Sickle; D006801:Humans; D019190:Iron Overload / Q000175:diagnosis; D008103:Liver Cirrhosis / Q000175:diagnosis; D008107:Liver Diseases / Q000209:etiology* / Q000473:pathology; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D055815:Young Adult
chemical_list: D006451:Hemoglobin, Sickle
keywords: cirrhosis; fibrosis; haemoglobinopathy; liver disease; sickle cell anaemia