The liver in sickle cell disease

E Theocharidou, AR Suddle - Clinics in liver disease, 2019 - liver.theclinics.com
Sickle cell disease (SCD) or homozygous sickle cell anemia is the most common inherited
disorder of erythrocytes, characterized by the presence of pathologic hemoglobin S (HbS). A …

Sickle hepatopathy

DL Praharaj, AC Anand - Journal of Clinical and Experimental Hepatology, 2021 - Elsevier
Sickle hepatopathy is an umbrella term describing various pattern of liver injury seen in
patients with sickle cell disease. The disease is not uncommon in India; in terms of …

Regulation of iron homeostasis through the erythroferrone‐hepcidin axis in sickle cell disease

AA Mangaonkar, F Thawer, J Son… - British journal of …, 2020 - Wiley Online Library
Sickle cell disease (SCD) has a distinct pattern of transfusional iron overload (IO) when
compared to transfusion‐dependent β‐thalassaemia major (TDT). We conducted a single …

Clinical management of sickle cell liver disease in children and young adults

E Kyrana, D Rees, F Lacaille, E Fitzpatrick… - Archives of disease in …, 2021 - adc.bmj.com
Liver involvement in sickle cell disease (SCD) is often referred to as sickle cell hepatopathy
(SCH) and is a complication of SCD which may be associated with significant mortality. This …

Molecular mechanisms of hepatic dysfunction in sickle cell disease: Lessons from Townes mouse model

T Pradhan-Sundd, GJ Kato… - American Journal of …, 2022 - journals.physiology.org
Sickle cell disease (SCD) is an autosomal recessive genetic disorder that affects∼ 100,000
Americans and millions of people worldwide. Erythrocyte sickling, vaso-occlusion, sterile …

Management of liver complications in sickle cell disease

AR Suddle - Hematology 2014, the American Society of …, 2019 - ashpublications.org
Liver disease is an important cause of morbidity and mortality in patients with sickle cell
disease (SCD). Despite this, the natural history of liver disease is not well characterized and …

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series

LH Pecker, N Patel, S Creary, A Darbari… - Pediatric blood & …, 2018 - Wiley Online Library
The hepatic complications of sickle cell disease (SCD) are associated with increased
morbidity and mortality in adults; children usually survive but may suffer significant sequelae …

Liver damage and sickle cell disease: genotype relationship

M Bortolotti, R D'Ambrosio, M Fraquelli, P Pedrotti… - Annals of …, 2020 - Springer
Sickle hepatopathy is a severe and not rare complication of sickle cell disease (SCD),
showing mainly a cholestatic pattern. So far, no effective approaches to prevent or treat this …

Association between Transfusion-Related Iron Overload and Liver Fibrosis in Survivors of Pediatric Leukemia: A Cross-Sectional Study

M Sobhani, N Honar, M Fattahi… - Pediatric …, 2024 - pmc.ncbi.nlm.nih.gov
Purpose Patients who receive frequent blood transfusions are at an elevated risk of
developing hepatic fibrosis due to iron overload in the liver. In this study, we evaluated the …

Vibration Controlled Transient Elastography (Fibroscan®) in sickle cell liver disease‐could we strike while the liver is hard?

G Ben Yakov, D Sharma, H Alao… - British journal of …, 2019 - Wiley Online Library
Vibration controlled transient elastography (VCTE) is validated for the evaluation of hepatic
fibrosis in different liver diseases. Sickle cell liver disease (SCLD) results from a cumulative …