Pathophysiology of sickle cell disease

P Sundd, MT Gladwin, EM Novelli - Annual review of pathology …, 2019 - annualreviews.org
Since the discovery of sickle cell disease (SCD) in 1910, enormous strides have been made
in the elucidation of the pathogenesis of its protean complications, which has inspired recent …

Cardiovascular complications of sickle cell disease

V Sachdev, DR Rosing, SL Thein - Trends in cardiovascular medicine, 2021 - Elsevier
Sickle cell disease (SCD) is the most common inherited blood disorder in the United States,
and a global health problem. Pathological features of the abnormal hemoglobin (HbS) result …

[HTML][HTML] CXCR4-dependent macrophage-to-fibroblast signaling contributes to cardiac diastolic dysfunction in heart failure with preserved ejection fraction

N Zhang, Q Ma, Y You, X Xia, C Xie… - … journal of biological …, 2022 - ncbi.nlm.nih.gov
Rationale: Heart failure with preserved ejection fraction (HFpEF) can arise from
hypertension‐induced cardiac remodeling. Monocyte/macrophage accumulation and …

Robust clinical and laboratory response to hydroxyurea using pharmacokinetically guided dosing for young children with sickle cell anemia

PT McGann, O Niss, M Dong… - American journal of …, 2019 - Wiley Online Library
Hydroxyurea is FDA‐approved and now increasingly used for children with sickle cell
anemia (SCA), but dosing strategies, pharmacokinetic (PK) profiles, and treatment …

Atrial arrhythmia in adults with sickle cell anemia: a missing link toward understanding and preventing strokes

T d'Humières, Z Sadraoui, L Savale, L Boyer… - Blood …, 2024 - ashpublications.org
Although patients with homozygous sickle cell anemia (SCA) carry both significant left atrial
(LA) remodeling and an increased risk of stroke, the prevalence of atrial arrhythmia (AA) has …

Hydroxycarbamide exposure and ovarian reserve in women with sickle cell disease in the Multicenter Study of Hydroxycarbamide

LH Pecker, S Hussain, MS Christianson… - British journal of …, 2020 - Wiley Online Library
The application of modern ovarian reserve measures to women with sickle cell disease
(SCD) may help answer longstanding questions about whether SCD or hydroxycarbamide …

Cardiovascular disease and its management in children and adults undergoing hematopoietic stem cell transplantation

SJ Rotz, TD Ryan, SS Hayek - Journal of thrombosis and thrombolysis, 2021 - Springer
Hematopoietic stem cell transplantation (HSCT) is a potentially curative treatment for many
malignancies, hemoglobinopathies, metabolic diseases, bone marrow failure syndromes …

IL-18 mediates sickle cell cardiomyopathy and ventricular arrhythmias

A Gupta, YD Fei, TY Kim, A Xie, K Batai… - Blood, The Journal …, 2021 - ashpublications.org
Previous reports indicate that IL18 is a novel candidate gene for diastolic dysfunction in
sickle cell disease (SCD)–related cardiomyopathy. We hypothesize that interleukin-18 (IL …

Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure

KC Wood, MT Gladwin, AC Straub - Heart, 2020 - heart.bmj.com
Sickle cell disease (SCD) is caused by a single point mutation in the gene that codes for
beta globin synthesis, causing haemoglobin polymerisation, red blood cell stiffening and …

Early initiation of disease-modifying therapy can impede or prevent diffuse myocardial fibrosis in sickle cell anemia

O Niss, J Detterich, JC Wood, TD Coates… - Blood, The Journal …, 2022 - ashpublications.org
Cardiovascular disease is a major cause of mortality in patients with sickle cell disease
(SCD). Niss et al previously reported that cardiac magnetic resonance in 25 patients showed …