[HTML][HTML] Intravascular hemolysis and the pathophysiology of sickle cell disease

GJ Kato, MH Steinberg… - The Journal of clinical …, 2017 - Am Soc Clin Investig
Hemolysis is a fundamental feature of sickle cell anemia that contributes to its
pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1 …

Sickle cell anemia and its phenotypes

TN Williams, SL Thein - Annual review of genomics and human …, 2018 - annualreviews.org
In the 100 years since sickle cell anemia (SCA) was first described in the medical literature,
studies of its molecular and pathophysiological basis have been at the vanguard of scientific …

[HTML][HTML] Epidemiology of stroke in sickle cell disease

FJ Kirkham, IA Lagunju - Journal of Clinical Medicine, 2021 - mdpi.com
Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and
haemorrhagic, and it also affects adults with the condition. Without any screening or …

[HTML][HTML] Determinants of severity in sickle cell disease

DC Rees, VAM Brousse, JN Brewin - Blood Reviews, 2022 - Elsevier
Sickle cell disease is a very variable condition, with outcomes ranging from death in
childhood to living relatively symptom free into the 8 th decade. Much of this variability is …

Genetic variation and sickle cell disease severity: a systematic review and meta-analysis

JK Kirkham, JH Estepp, MJ Weiss… - JAMA Network …, 2023 - jamanetwork.com
Importance Sickle cell disease (SCD) is a monogenic disorder, yet clinical outcomes are
influenced by additional genetic factors. Despite decades of research, the genetics of SCD …

Minireview: clinical severity in sickle cell disease: the challenges of definition and prognostication

CT Quinn - Experimental biology and medicine, 2016 - journals.sagepub.com
Sickle cell disease (SCD) is a monogenic, yet highly phenotypically variable disease with
multisystem pathology. This manuscript provides an overview of many of the known …

The pleiotropic effects of α‐thalassemia on HbSS and HbSC sickle cell disease: Reduced erythrocyte cation co‐transport activity, serum erythropoietin, and …

JN Brewin, A Nardo‐Marino… - American Journal of …, 2022 - Wiley Online Library
Abstract α‐Thalassemia is one of the most important genetic modulators of sickle cell
disease (SCD). Both beneficial and detrimental effects have been described previously. We …

Sickle cell disease in the older adult

MS Thein, NE Igbineweka, SL Thein - Pathology, 2017 - Elsevier
Sickle cell disease (SCD) is an inherited haemoglobin disorder, associated with recurrent
painful episodes, ongoing haemolytic anaemia and progressive multi-organ damage. Until …

[HTML][HTML] A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania

J Makani, F Tluway, A Makubi, D Soka, S Nkya… - BMC hematology, 2018 - Springer
Background Africa has the highest burden of Sickle cell disease (SCD) but there are few
large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania …

Neurologic complications of sickle cell disease in Africa: a systematic review and meta-analysis

JJ Noubiap, MK Mengnjo, N Nicastro… - Neurology, 2017 - AAN Enterprises
Objective: To summarize prevalence data on the neurologic complications of sickle cell
disease (SCD) in Africa. Methods: We searched EMBASE, PubMed, and African Index …