Sickle cell disease: clinical presentation and management of a global health challenge

ME Houwing, PJ De Pagter, EJ Van Beers, BJ Biemond… - Blood reviews, 2019 - Elsevier
Sickle cell disease is an autosomal recessive, multisystem disorder, characterised by
chronic haemolytic anaemia, painful episodes of vaso-occlusion, progressive organ failure …

Management of sickle cell disease complications beyond acute chest syndrome

UO Ogu, NU Badamosi, PE Camacho… - Journal of Blood …, 2021 - Taylor & Francis
Sickle cell disease results in numerous complications that can lead to significant morbidity
and mortality. Amongst them, acute chest syndrome is the leading cause of mortality. As a …

P-selectin drives complement attack on endothelium during intravascular hemolysis in TLR-4/heme-dependent manner

NS Merle, R Paule, J Leon, M Daugan… - Proceedings of the …, 2019 - National Acad Sciences
Hemolytic diseases are frequently linked to multiorgan failure subsequent to vascular
damage. Deciphering the mechanisms leading to organ injury upon hemolytic event could …

Hepatobiliary complications in children with sickle cell disease: a retrospective review of medical records from 616 patients

S Allali, M de Montalembert, V Brousse… - Journal of clinical …, 2019 - mdpi.com
Hepatobiliary complications in children with sickle cell disease (SCD) are rarely reported but
can be life-threatening. We retrospectively assessed their prevalence in a cohort of 616 …

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 …

Mouse models of sickle cell disease: imperfect and yet very informative

S Kamimura, M Smith, S Vogel, LEF Almeida… - Blood Cells, Molecules …, 2024 - Elsevier
The root cause of sickle cell disease (SCD) has been known for nearly a century, however,
few therapies to treat the disease are available. Over several decades of work, with …

NLRP3 inflammasome and bruton tyrosine kinase inhibition interferes with upregulated platelet aggregation and in vitro thrombus formation in sickle cell mice

S Vogel, S Kamimura, T Arora, ML Smith… - Biochemical and …, 2021 - Elsevier
The nucleotide-binding domain leucine-rich repeat containing protein 3 (NLRP3)
inflammasome is a critical inflammatory mechanism identified in platelets, which controls …

Hyperbilirubinemia-induced pro-angiogenic activity of infantile endothelial progenitor cells

M Jabarpour, V Siavashi, S Asadian, H Babaei… - Microvascular …, 2018 - Elsevier
Objectives Bilirubin, a by-product of heme degradation, is suggested to have a role for
vascular protection. There is increasing evidence that bilirubin may directly affect the …

Manifestations of sickle cell disorder at abdominal and pelvic imaging

N Solomon, N Segaran, M Badawy, KM Elsayes… - Radiographics, 2022 - pubs.rsna.org
Sickle cell disorder (SCD) refers to a spectrum of hematologic disorders that cause a
characteristic clinical syndrome affecting the entire body. It is the most prevalent …

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 …