Sickle Retinopathy in a Person with Hemoglobin S/New York Disease
A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease.
Donovan Calder +4 more
doaj +1 more source
Objective: The aim of the present study was to characterize sickle cell disease retinopathy in children and teenagers from Bahia, the state in northeastern Brazil with the highest incidence and prevalence of sickle cell disease.
Dayse Cury de Almeida Oliveira +5 more
doaj +1 more source
Global Burden of Sickle Cell Anaemia in Children under Five, 2010-2050: Modelling Based on Demographics, Excess Mortality, and Interventions [PDF]
The global burden of sickle cell anaemia (SCA) is set to rise as a consequence of improved survival in high-prevalence low- and middle-income countries and population migration to higher-income countries.
Gupta, Sunetra +23 more
core +1 more source
Introduction: The benefits of manual versus automated red blood cell exchange have rarely been documented and studies in young sickle cell disease patients are scarce. We aim to describe and compare our experience in these two procedures.
Carlos Escobar +9 more
doaj +1 more source
The post-mortem diagnosis of vasocclusive crisis in sickle cell disease
Sickle cell disease (SCD) comprises a group of genetic blood disorders that affect the hemoglobin molecular structure, and in some cases, the association with hemoglobin synthesis.
Varsha Bhatia +2 more
doaj +1 more source
A clinico-hematological study of sickle cell disease among adult patients in Makkah, Saudi Arabia. [PDF]
Background: Sickle cell disease is an autosomal recessive disease characterized by aberrant production of hemoglobin S (HbS). The clinical picture includes hemolytic anemia, vascular occlusion events, acute and persistent pain, and varied organ damage ...
Ahmad Arbaeen
doaj +1 more source
Structure of Sickled Erythrocytes and of Sickle-Cell Hemoglobin Fibers [PDF]
Deoxyhemoglobin from patients homozygous for sickle-cell anemia (deoxyhb S) aggregates into long straight fibers. These may extend through most of the length of the sickled cell, forming either square or hexagonally packed bundles with lattice constants of 170-180 Å. Each fiber is a tube made up of six thin filaments, which are wound around the tubular
J T, Finch +3 more
openaire +2 more sources
Prevalence of hemoglobin abnormalities in an apparently healthy population in Benin
Background: Sickle cell disease is the most common monogenic disorder in humans and is a major public health concern in sub-Saharan Africa. In Benin, the prevalence of sickle cell disease is estimated to be 4.8%.
Alban Zohoun +3 more
doaj +1 more source
Structural and functional characterization of CSDA protein complexes involved in the modulation of fetal globin gene expression [PDF]
Impaired switching from fetal hemoglobin (HbF) to adult globin gene expression leads to hereditary persistence of fetal hemoglobin (HPFH) in adult life.
Gaudino, Sara
core +1 more source
Haptoglobin and sickle cell polymorphisms and risk of active trachoma in Gambian children. [PDF]
BACKGROUND: Susceptibility and resistance to trachoma, the leading infectious cause of blindness, have been associated with a range of host genetic factors.
Doherty, CP +52 more
core +1 more source

