Sickle cell disease

RE Ware, M de Montalembert, L Tshilolo, MR Abboud - The Lancet, 2017 - thelancet.com
Sickle cell disease is a common and life-threatening haematological disorder that affects
millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in …

The nephropathy of sickle cell trait and sickle cell disease

KI Ataga, SL Saraf, VK Derebail - Nature Reviews Nephrology, 2022 - nature.com
Sickle cell syndromes, including sickle cell disease (SCD) and sickle cell trait, are
associated with multiple kidney abnormalities. Young patients with SCD have elevated …

Mechanisms of haemolysis-induced kidney injury

K Van Avondt, E Nur, S Zeerleder - Nature Reviews Nephrology, 2019 - nature.com
Intravascular haemolysis is a fundamental feature of chronic hereditary and acquired
haemolytic anaemias, including those associated with haemoglobinopathies, complement …

Hemolysis derived products toxicity and endothelium: model of the second hit

M Frimat, I Boudhabhay, LT Roumenina - Toxins, 2019 - mdpi.com
Vascular diseases are multifactorial, often requiring multiple challenges, or 'hits', for their
initiation. Intra-vascular hemolysis illustrates well the multiple-hit theory where a first event …

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 …

Complement activation in sickle cell disease: Dependence on cell density, hemolysis and modulation by hydroxyurea therapy

LT Roumenina, P Chadebech, G Bodivit… - American journal of …, 2020 - Wiley Online Library
The complement system is an innate immune defense cascade that can cause tissue
damage when inappropriately activated. Evidence for complement over activation has been …

Sickle cell trait and the risk of ESRD in blacks

RP Naik, MR Irvin, S Judd, OM Gutiérrez… - Journal of the …, 2017 - journals.lww.com
Blacks, compared with whites, have an increased risk of progression to end-stage renal
disease (ESRD). Emerging evidence suggests that, in addition to APOL1 high-risk …

[HTML][HTML] Drug therapies for the management of sickle cell disease

P Rai, KI Ataga - F1000Research, 2020 - ncbi.nlm.nih.gov
Sickle cell disease (SCD) afflicts millions of people worldwide but is referred to as an orphan
disease in the United States. Over the past several decades, there has been an increasing …

The spectrum of sickle hemoglobin-related nephropathy: from sickle cell disease to sickle trait

RP Naik, VK Derebail - Expert review of hematology, 2017 - Taylor & Francis
Introduction: Renal dysfunction is among the most common complication of sickle cell
disease (SCD), from hyposthenuria in children to progression to overt chronic kidney …

Sickle cell dehydration: Pathophysiology and therapeutic applications

C Brugnara - Clinical hemorheology and microcirculation, 2018 - content.iospress.com
Cell dehydration is a distinguishing characteristic of sickle cell disease and an important
contributor to disease pathophysiology. Due to the unique dependence of Hb S …