PERIODIC, self-limited episodes of excruciating musculoskeletal pain punctuate the lives of patients with reap hook cell disease. Often referred to as crises, these episodes atomic number 18 the principal let of morbidity among these patients. Although Western observers named reap hook cell anemia for its prying microscopical morphologic features, African cultures have named it for its painful episodes. In the Ga language of Ghana, for example, the disease is effn as chwechweechwe â"relentless, crying chewing. 1 Despite the obvious importance of this clinical syndrome, we know little about its epidemiologic characteristics. Here we report the findings of the necessitate Heart, Lung and Blood Institutes Cooperative Study of Sickle Cell Disease, in which we sought to answer questions frequently asked by patients, clinicians, and investigators: What is the incidence of the episodes of pain, what are the associated risk factors, and are patients with high rates of pain at risk for early death?
METHODS
Study Population
The design of the study has been described elsewhere.2 , 3 The present analysis includes 3578 patients (Table 1TABLE 1Summary of Inclusions and Exclusions from the Study analysis among 4082 Patients.*) ranging at enrollment from newborns to persons up to 66 years old.
Patients were enrolled by 23 clinical centers across the continental United States, with a broad geographic and urbanâ"rural distribution of subjects. They were followed among 1979 and 1988 (patients entering after May 31, 1986, were excluded from this analysis); the retention rate was 74.3 pct at the end of follow-up (67.7 percent of the patients were under bustling study, and 6.6 percent had died). Diagnoses were assigned at the Centers for Disease Control on the basis of hemoglobin electrophoresis â" i.e., a diagnosis of sickle cell anemia, sickle ?0-thalassemia, sickle ?+-thalassemia, or hemoglobin SC disease. Patients without diagnoses were excluded from analysis. Alpha-gene mapping4 , 5was...If you want to get a full essay, order it on our website: Orderessay
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