Osteonecrosis of the humeral head in sickle cell disease

• Published in: Clinical orthopaedics and related research no. 289; p. 136
• Main Authors: Milner, P F, Kraus, A P, Sebes, J I, Sleeper, L A, Dukes, K A, Embury, S H, Bellevue, R, Koshy, M, Moohr, J W, Smith, J
• Format: Journal Article
• Language: English
• Published: United States 01.04.1993
• Subjects: Adolescent; Adult; Age Factors; alpha-Thalassemia - complications; alpha-Thalassemia - genetics; Anemia, Sickle Cell - complications; Anemia, Sickle Cell - genetics; beta-Thalassemia - complications; beta-Thalassemia - genetics; Child; Child, Preschool; Genotype; Hemoglobin SC Disease - complications; Hemoglobin SC Disease - genetics; Humans; Humerus; Incidence; Middle Aged; Osteonecrosis - diagnostic imaging; Osteonecrosis - epidemiology; Osteonecrosis - etiology; Prevalence; Prospective Studies; Radiography; Range of Motion, Articular; Time Factors; United States - epidemiology
• ISSN: 0009-921X
• DOI: 10.1097/00003086-199304000-00018

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.