Hemolysis is a fundamental feature of sickle cell anemia that contributes to its
pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1 …

This review presents evidence for two overlapping yet distinctive clinical types of sickle cell
disease. The basis of one is the vaso-occlusive crisis; the other is the consequence of …

Sickle cell disease is characterized by recurrent episodes of ischemia-reperfusion injury to
multiple vital organ systems and a chronic hemolytic anemia, both contributing to …

Sickle cell disease has been very well characterized as a single amino acid molecular
disorder of hemoglobin leading to its pathological polymerization, with resulting red cell …

Background: Home oxygen therapy is often required in children with chronic respiratory
conditions. This document provides an evidence-based clinical practice guideline on the …

Sickle cell disease (SCD) is one of the most common genetic causes of illness and death in
the world. This is a review of SCD in Africa, which bears the highest burden of disease. The …

During the past decade a large body of experimental and clinical studies has focused on the
hypothesis that nitric oxide (NO) depletion by plasma hemoglobin in the microcirculation …

Many mechanisms contribute to the complex pathophysiology of sickle cell disease (SCD),
with dysfunction of the vascular endothelium as a unifying theme. Specifically, hemolysis …

Abstract The “hyperhemolytic paradigm”(HHP) posits that hemolysis in sickle disease
sequentially and causally establishes increased cell‐free plasma Hb, consumption of NO, a …

Sickle cell anemia (SCA or SS homozygous sickle cell disease) is an inherited blood
disorder caused by single nucleotide substitution in the ß-globin gene that renders their …