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 …

Sickle cell disease: a neglected chronic disease of increasing global health importance

S Chakravorty, TN Williams - Archives of disease in childhood, 2015 - adc.bmj.com
Sickle cell disease (SCD) is a single gene disorder causing a debilitating systemic
syndrome characterised by chronic anaemia, acute painful episodes, organ infarction and …

Pediatric pulmonary hypertension: guidelines from the American heart association and American thoracic society

SH Abman, G Hansmann, SL Archer, DD Ivy, I Adatia… - Circulation, 2015 - Am Heart Assoc
Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic
diseases in neonates, infants, and older children and contributes to significant morbidity and …

An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease

ES Klings, RF Machado, RJ Barst… - American journal of …, 2014 - atsjournals.org
Background: In adults with sickle cell disease (SCD), an increased tricuspid regurgitant
velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro …

Pathophysiology and treatment of pulmonary hypertension in sickle cell disease

VR Gordeuk, OL Castro… - Blood, The Journal of the …, 2016 - ashpublications.org
Pulmonary hypertension affects∼ 10% of adult patients with sickle cell disease (SCD),
particularly those with the homozygous genotype. An increase in pulmonary artery systolic …

Differences in the clinical and genotypic presentation of sickle cell disease around the world

SL Saraf, RE Molokie, M Nouraie, CA Sable… - Paediatric respiratory …, 2014 - Elsevier
Sickle cell disease (SCD), caused by a mutation in the β-globin gene HBB, is widely
distributed in malaria endemic regions. Cardiopulmonary complications are major causes of …

[HTML][HTML] The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe

M Nouraie, JS Lee, Y Zhang, T Kanias, X Zhao… - …, 2013 - ncbi.nlm.nih.gov
The intensity of hemolytic anemia has been proposed as an independent risk factor for the
development of certain clinical complications of sickle cell disease, such as pulmonary …

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 …

Vasculopathy and pulmonary hypertension in sickle cell disease

KP Potoka, MT Gladwin - American Journal of Physiology …, 2015 - journals.physiology.org
Sickle cell disease (SCD) is an autosomal recessive disorder in the gene encoding the β-
chain of hemoglobin. Deoxygenation causes the mutant hemoglobin S to polymerize …

Reconstructing sickle cell disease: a data‐based analysis of the “hyperhemolysis paradigm” for pulmonary hypertension from the perspective of evidence‐based …

RP Hebbel - American journal of hematology, 2011 - Wiley Online Library
Abstract The “hyperhemolytic paradigm”(HHP) posits that hemolysis in sickle disease
sequentially and causally establishes increased cell‐free plasma Hb, consumption of NO, a …