title: Hemoglobin S-C disease revisited: clinical study of 106 adults.
abstract: We describe the clinical features of S-C hemoglobin disease in 106 adults seen during the years 1972-2000 and followed for a mean period of 6.8 years (range 1-27 years). The median age of the patients was 50 years. Common clinical features were pain crisis (65%), avascular necrosis of the hip (23%), proliferative sickle retinopathy (34%), and splenic infarction/splenic sequestration syndrome (19%). Acute splenic sequestration crisis occurred in 10 patients and was the presenting feature in two. Obesity (19.8%), essential hypertension (20.7%), and type-2 diabetes mellitus (10.3%) were common. The frequent occurrence of these co-morbidities among patients with hemoglobin S-C disease has not been reported previously.
authors.affiliation: The Department of Medicine, Division of Hematology-Oncology, Cook County Hospital, Chicago, Illinois, USA.
authors.forename: P R
authors.initials: PR
authors.lastname: Koduri
authors.forename: B
authors.initials: B
authors.lastname: Agbemadzo
authors.forename: S
authors.initials: S
authors.lastname: Nathan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000453:epidemiology; D015897:Comorbidity; D005500:Follow-Up Studies; D006801:Humans; D008875:Middle Aged; D012189:Retrospective Studies