Acute chest syndrome in children with sickle cell disease

S Jain, N Bakshi, L Krishnamurti - Pediatric allergy, immunology …, 2017 - liebertpub.com
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle
cell disease (SCD). Patients may present with ACS or may develop this complication during …

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 …

Extracellular hemin crisis triggers acute chest syndrome in sickle mice

S Ghosh, OA Adisa, P Chappa, F Tan… - The Journal of …, 2013 - Am Soc Clin Investig
The prevention and treatment of acute chest syndrome (ACS) is a major clinical concern in
sickle cell disease (SCD). However, the mechanism underlying the pathogenesis of ACS …

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: Genetic basis of heterogeneity and severity in sickle cell disease

A Habara, MH Steinberg - Experimental Biology and …, 2016 - journals.sagepub.com
Sickle cell disease, a common single gene disorder, has a complex pathophysiology that at
its root is initiated by the polymerization of deoxy sickle hemoglobin. Sickle vasoocclusion …

Association of Variants at BCL11A and HBS1L-MYB with Hemoglobin F and Hospitalization Rates among Sickle Cell Patients in Cameroon

A Wonkam, VJ Ngo Bitoungui, AA Vorster, R Ramesar… - PloS one, 2014 - journals.plos.org
Background Genetic variation at loci influencing adult levels of HbF have been shown to
modify the clinical course of sickle cell disease (SCD). Data on this important aspect of SCD …

Acute chest syndrome of sickle cell disease: genetics, risk factors, prognosis, and management

ES Klings, MH Steinberg - Expert Review of Hematology, 2022 - Taylor & Francis
Introduction Sickle cell disease, one of the world's most prevalent Mendelian disorders, is a
chronic hemolytic anemia punctuated by acute vasoocclusive events. Both hemolysis and …

The intersection between asthma and acute chest syndrome in children with sickle-cell anaemia

MR DeBaun, RC Strunk - The Lancet, 2016 - thelancet.com
Acute chest syndrome is a frequent cause of acute lung disease in children with sickle-cell
disease. Asthma is common in children with sickle-cell disease and is associated with …

Clinical and genetic factors are associated with pain and hospitalisation rates in sickle cell anaemia in Cameroon

A Wonkam, K Mnika, VJ Ngo Bitoungui… - British journal of …, 2018 - Wiley Online Library
We aimed to investigate the clinical and genetic predictors of painful vaso‐occlusive crises
(VOC) in sickle cell disease (SCD) in Cameroon. Socio‐demographics, clinical …

Inflammatory mediators of endothelial injury in sickle cell disease

CC Hoppe - Hematology/Oncology Clinics, 2014 - hemonc.theclinics.com
SCD is now recognized as a complex disease characterized by acute and chronic
inflammation. The incidence of nearly every clinical manifestation of SCD correlates with …