The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

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The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability.

To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, ...

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Munchausen sickle cell painful crisis.

A 22-year-old female with factitious sickle cell anemia and recurrent painful crises is described. Because she had sickle cell trait and iron deficiency anemia, she could successfully feign the symptoms of homozygous sickle cell anemia. The identification of this syndrome in patients with genetic disorders is presented.

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In a 10-year retrospective study a close correlation was found between low temperatures and hospital admissions for severe painful crises of sickle-cell disease.

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Aplastic crises in homozygous sickle cell disease in Jamaica predominantly affect children and occur in epidemics. Of 67 cases in a cohort study of 314 children with homozygous sickle cell disease, 62 were attributable to human parvovirus infection. Affected children were aged 0.5-12.5 years, and the incidence rose to 28% by 10 years. No recurrences were seen. Symptoms and signs on presentation...

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Nucleosomes and neutrophil activation in sickle cell disease painful crisis.

Activated polymorphonuclear neutrophils play an important role in the pathogenesis of vaso-occlusive painful sickle cell crisis. Upon activation, polymorphonuclear neutrophils can form neutrophil extracellular traps. Neutrophil extracellular traps consist of a meshwork of extracellular DNA, nucleosomes, histones and neutrophil proteases. Neutrophil extracellular traps have been demonstrated to ...

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ژورنال

عنوان ژورنال: Blood

سال: 1988

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v72.6.2056.2056