Osteonecrosis of the humeral head in sickle cell disease.
The prevalence and incidence of osteonecrosis (ON) of the humeral head in sickle cell disease was determined by a study of 2524 patients who were entered into a prospective study and followed for an average of 5.6 years. At entry, 5.6% had roentgenographic evidence of ON in one or both shoulders. There was little difference in age-adjusted prevalence among genotypes, but there were striking differences in age-specific rates. Observed at ages ranging from five to 24 years, 3.25% of sickle cell anemia (S/S) patients, but only 1.1% of sickle cell disease (S/C) patients, had ON. No S/beta+ thalassemia patients younger than 25 years of age had ON on entry. The highest age-adjusted incidence rate was found in S/S patients with concomitant alpha-thalassemia (4.85 per hundred patient-years), followed by S/beta zero-thalassemia (4.84 per hundred patient-years), S/beta+ thalassemia (2.61 per hundred patient-years), S/S without alpha-thalassemia (2.54 per hundred patient-years), and S/C (1.66 per hundred patient-years). Only 20.9% of patients reported pain or had limited range of movement at the time of diagnosis. Sickle cell disease is a frequent cause of ON of the humeral head, especially in children and young adults.
Milner
P F
PF
Medical College of Georgia, Augusta 30912-3600.
Kraus
A P
AP
Sebes
J I
JI
Sleeper
L A
LA
Dukes
K A
KA
Embury
S H
SH
Bellevue
R
R
Koshy
M
M
Moohr
J W
JW
Smith
J
J
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005838:Genotype; D006450:Hemoglobin SC Disease / Q000150:complications* / Q000235:genetics; D006801:Humans; D006811:Humerus; D015994:Incidence; D008875:Middle Aged; D010020:Osteonecrosis / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology; D015995:Prevalence; D011446:Prospective Studies; D011859:Radiography; D016059:Range of Motion, Articular; D013997:Time Factors; D014481:United States / Q000453:epidemiology; D017085:alpha-Thalassemia / Q000150:complications* / Q000235:genetics; D017086:beta-Thalassemia / Q000150:complications* / Q000235:genetics