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 …

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 …

The worst things in life are free: the role of free heme in sickle cell disease

OT Gbotosho, MG Kapetanaki, GJ Kato - Frontiers in immunology, 2021 - frontiersin.org
Hemolysis is a pathological feature of several diseases of diverse etiology such as
hereditary anemias, malaria, and sepsis. A major complication of hemolysis involves the …

Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia

O Niss, R Fleck, F Makue, T Alsaied… - Blood, The Journal …, 2017 - ashpublications.org
Sickle cell anemia (SCA)–related cardiomyopathy is characterized by diastolic dysfunction
and hyperdynamic features. Diastolic dysfunction portends early mortality in SCA. Diastolic …

Chronic organ failure in adult sickle cell disease

E Vichinsky - Hematology 2014, the American Society of …, 2017 - ashpublications.org
Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan
failure, particularly involving the brain and kidney. The etiology is multifactorial; it includes …

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 nephropathy: an update on pathophysiology, diagnosis, and treatment

E Hariri, A Mansour, A El Alam, Y Daaboul… - … Urology and Nephrology, 2018 - Springer
Sickle cell nephropathy is a major complication of sickle cell disease. It manifests in different
forms, including glomerulopathy, proteinuria, hematuria, and tubular defects, and frequently …

Long-term endothelin-A receptor antagonism provides robust renal protection in humanized sickle cell disease mice

M Kasztan, BM Fox, JS Speed… - Journal of the …, 2017 - journals.lww.com
Sickle cell disease (SCD)–associated nephropathy is a major source of morbidity and
mortality in patients because of the lack of efficacious treatments targeting renal …

Pathophysiological characterization of the Townes mouse model for sickle cell disease

J Alvarez-Argote, TA Dlugi, T Sundararajan… - Translational …, 2023 - Elsevier
A deeper pathophysiologic understanding of available mouse models of sickle cell disease
(SCD), such as the Townes model, will help improve preclinical studies. We evaluated …

[HTML][HTML] Sickle cell disease and CKD: an update

RS Zahr, SL Saraf - American Journal of Nephrology, 2024 - karger.com
Background: Sickle cell disease is an inherited red blood cell disorder that affects
approximately 100,000 people in the USA and 25 million people worldwide. Vaso-occlusion …