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 …
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 …
syndrome characterised by chronic anaemia, acute painful episodes, organ infarction and …
Pediatric pulmonary hypertension: guidelines from the American heart association and American thoracic society
Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic
diseases in neonates, infants, and older children and contributes to significant morbidity and …
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 …
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 …
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
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 …
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
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 …
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 …
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 …
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 …
sequentially and causally establishes increased cell‐free plasma Hb, consumption of NO, a …