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[镰状细胞贫血中的血管阻塞性危机]

[Vaso-occlusive crises in sickle-cell anemia].

作者信息

Lang S M, Adler S, Karbach U, Emmerich B

机构信息

Medizinische Klinik Innenstadt, Universität München.

出版信息

Dtsch Med Wochenschr. 1995 Jul 28;120(30):1040-4. doi: 10.1055/s-2008-1055442.

Abstract

HISTORY AND FINDINGS

Severe pain suddenly occurred in the shaft of the right thigh in a 21-year-old patient of Turkish descent with known homozygotic sickle cell anaemia. He also had marked scleral jaundice and anaemia (haemoglobin 9.1 g/dl). An X-ray film of the lower leg pointed to a bone infarct. Type of pain, partial pressure of oxygen in blood (70 mm Hg), concentration of lactate dehydrogenase and the bilirubin level (4,5 mg/dl) as signs of haemolysis, as well as the fall in haemoglobin, indicated a vascular occlusive crisis in sickle cell anaemia.

TREATMENT AND COURSE

The symptoms regressed within 8 days of the patient receiving fluids, analgesics and oxygenation by nasal tube. A few weeks later he developed a fever, productive cough and severe pain in joints and abdomen. Blood p02 was 54 mm Hg. Pneumonia in the right lung base was the cause of this renewed life-threatening crisis, which was again associated with haemolysis. Klebsiella having been found in sputum he was treated with cefotaxim (1 g twice daily intravenously) and oxygen insufflation via nasal tube (7 l/min). His condition quickly improved.

CONCLUSIONS

In patients with sickle cell anaemia it is important (1) to regard crises as potentially life-threatening conditions, (2) to detect and treat infections as possible causes early and (3) to provide sufficient oxygen insufflation quickly, especially in pulmonary infections.

摘要

病史及检查结果

一名21岁具有土耳其血统的患者,已知患有纯合子镰状细胞贫血,右大腿骨干突然出现剧痛。他还伴有明显的巩膜黄疸和贫血(血红蛋白9.1g/dl)。小腿的X线片显示有骨梗死。疼痛类型、血液中氧分压(70mmHg)、乳酸脱氢酶浓度以及作为溶血迹象的胆红素水平(4.5mg/dl),还有血红蛋白下降,均表明为镰状细胞贫血的血管闭塞性危机。

治疗及病程

患者接受补液、止痛药物治疗并通过鼻管吸氧,症状在8天内消退。几周后,他出现发热、咳痰、关节和腹部剧痛。血氧分压为54mmHg。右肺底部肺炎是此次再次危及生命的危机的原因,且再次伴有溶血。痰中发现克雷伯菌后,给予头孢噻肟(每日两次,每次1g静脉注射)治疗,并通过鼻管吸氧(7l/min)。他的病情迅速好转。

结论

对于镰状细胞贫血患者,重要的是:(1)将危机视为潜在的危及生命的状况;(2)尽早发现并治疗可能的感染病因;(3)迅速提供充足的吸氧,尤其是在肺部感染时。

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