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Indices of Severity and Prognosis for Sickle Cell Disease

2014-08-27 03:57:50 | BioPortfolio

Summary

To develop a clinical severity index that could prospectively identify sickle cell disease patients who were at high risk for a turbulent clinical course and a poor prognosis.

Description

DESIGN NARRATIVE:

Using the database, univariate analysis showed that factors associated with the occurrence of cerebrovascular accident (51 patients) included hematocrit, rate of change of pocked red cell count, and polymer fraction at 40 percent oxygen saturation (PF40). Only low hematocrit was predictive of death in the pediatric cohort. There were 45 disease-related deaths.

Study Design

Observational Model: Natural History

Conditions

Anemia, Sickle Cell

Status

Completed

Source

National Heart, Lung, and Blood Institute (NHLBI)

Results (where available)

View Results

Links

Published on BioPortfolio: 2014-08-27T03:57:50-0400

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Medical and Biotech [MESH] Definitions

An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia.

One of the sickle cell disorders characterized by the presence of both hemoglobin S and hemoglobin C. It is similar to, but less severe than sickle cell anemia.

A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.

An acute purulent infection of the meninges and subarachnoid space caused by Streptococcus pneumoniae, most prevalent in children and adults over the age of 60. This illness may be associated with OTITIS MEDIA; MASTOIDITIS; SINUSITIS; RESPIRATORY TRACT INFECTIONS; sickle cell disease (ANEMIA, SICKLE CELL); skull fractures; and other disorders. Clinical manifestations include FEVER; HEADACHE; neck stiffness; and somnolence followed by SEIZURES; focal neurologic deficits (notably DEAFNESS); and COMA. (From Miller et al., Merritt's Textbook of Neurology, 9th ed, p111)

K-Cl cotransporter ubiquitously expressed with higher expression levels in ERYTHROCYTES of ANEMIA, SICKLE CELL. It mediates active potassium and chloride cotransport across the plasma membrane and contributes to cell volume homeostasis

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