Keeley K, Buchanan G R
J Pediatr. 1982 Aug;101(2):170-5. doi: 10.1016/s0022-3476(82)80111-x.
The clinical features of long bone infarction in patients with sickle cell disease have not been well defined, and differentiation of bone infarct from osteomyelitis has accordingly been difficult. We reviewed records from 192 children with sickle hemoglobinopathies and identified 41 episodes of acute long bone infarction in 21 patients. The most commonly affected bones were the humerus (38%), tibia (23%), and femur (19%). The distal segment was more commonly involved. Tenderness and prominent swelling occurred in all cases; other findings included impaired joint motion (68%), local heat (65%), and erythema (145). Fever was usually absent or low grade, and patients did not appear ill. Laboratory studies included negative bacterial cultures in all cases, absence of left shift in WBC count in most, and variable erythrocyte sedimentation rate. Plain roentgenographs were unremarkable. Contrary to previous reports, radionuclide bone and bone marrow scans were not helpful in differentiation of bone infarction from osteomyelitis. Patients received supportive therapy and improved within several days. Long-term sequelae were not evident. The rarity of osteomyelitis in our sickle cell population (five cases in 22 years) precluded direct comparison of most of its clinical features with those of bone infarction. Acute long bone infarction is at least 50 times more common than bacterial osteomyelitis in sickle cell disease.
镰状细胞病患者长骨梗死的临床特征尚未明确界定，因此骨梗死与骨髓炎的鉴别诊断存在困难。我们回顾了192例镰状血红蛋白病患儿的病历，确定21例患者出现41次急性长骨梗死发作。最常受累的骨骼是肱骨（38%）、胫骨（23%）和股骨（19%）。远端节段更常受累。所有病例均有压痛和明显肿胀；其他表现包括关节活动受限（68%）、局部发热（65%）和红斑（145）。通常无发热或低热，患者看起来病情不重。实验室检查包括所有病例细菌培养均为阴性，大多数患者白细胞计数无左移，红细胞沉降率不一。X线平片无明显异常。与先前报道相反，放射性核素骨扫描和骨髓扫描对骨梗死与骨髓炎的鉴别诊断并无帮助。患者接受支持性治疗，数天内病情好转。无明显长期后遗症。我们镰状细胞病患者中骨髓炎罕见（22年中有5例），无法将其大多数临床特征与骨梗死的特征进行直接比较。在镰状细胞病中，急性长骨梗死的发生率至少是细菌性骨髓炎的50倍。
