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
Medical College of Georgia, Augusta 30912-3600.
Clin Orthop Relat Res. 1993 Apr(289):136-43.
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.
通过对2524名患者进行前瞻性研究并平均随访5.6年，确定了镰状细胞病患者肱骨头骨坏死（ON）的患病率和发病率。入组时，5.6%的患者双肩或单肩有X线片显示的骨坏死证据。各基因型之间经年龄调整后的患病率差异不大，但年龄别发病率存在显著差异。在5至24岁年龄段观察到，镰状细胞贫血（S/S）患者中有3.25%发生骨坏死，而镰状细胞病（S/C）患者中只有1.1%发生骨坏死。年龄小于25岁的S/β+地中海贫血患者入组时均无骨坏死。经年龄调整后的发病率最高的是伴有α地中海贫血的S/S患者（每100患者年4.85例），其次是S/β0地中海贫血（每100患者年4.84例）、S/β+地中海贫血（每100患者年2.61例）、无α地中海贫血的S/S患者（每100患者年2.54例）和S/C患者（每100患者年1.66例）。只有20.9%的患者在诊断时报告有疼痛或活动范围受限。镰状细胞病是肱骨头骨坏死的常见原因，尤其是在儿童和年轻人中。
