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纯合子镰状细胞病中的体位性血管收缩与腿部溃疡

Postural vasoconstriction and leg ulceration in homozygous sickle cell disease.

作者信息

Mohan J S, Marshall J M, Reid H L, Thomas P W, Serjeant G R

机构信息

Department of Physiology, University of the West Indies, Kingston, Jamaica.

出版信息

Clin Sci (Lond). 1997 Feb;92(2):153-8. doi: 10.1042/cs0920153.

DOI:10.1042/cs0920153
PMID:9059316
Abstract
  1. Chronic leg ulceration is a major cause of morbidity in patients with homozygous sickle cell disease; the ulcers commonly resolve on bed rest. We have therefore compared the cutaneous vascular response to dependency in three groups of eight patients with sickle cell disease (those with an active ulcer, with an ulcer scar and with no history of ulceration) and in eight subjects with normal haemoglobin and no history of leg ulceration. 2. We monitored, with a laser Doppler flowmeter, the change in red cell (erythrocyte) flux induced in the skin of the leg, at two sites proximal to the malleoli, with the leg horizontal and 5 and 10 min after moving the leg to the dependent position. 3. With the leg horizontal, mean cutaneous red cell flux was substantially higher in normal skin of patients with sickle cell disease than in normal subjects and was higher still at the site of the ulcer or scar. On dependency, red cell flux fell not only in normal subjects but also in the patients with sickle cell disease, both in the normal skin and at the site of the ulcer or scar; there was no difference in any group between the 5- and 10-min values. The fall in red cell flux in normal skin of patients with sickle cell disease was smaller than in normal subjects when considered as a percentage of the control values (32%, 36%, 30% and 61% respectively in sickle cell patients with an active ulcer, with an ulcer scar and with no history of ulceration and in normal subjects), but in absolute terms the falls in red cell flux were similar in sickle cell patients and normal subjects. By contrast, the fall in red cell flux at the ulcer or scar site was greater than in normal skin from sickle cell patients whether considered as a percentage of the control value (48% and 49% respectively in those with an active ulcer or ulcer scar) or in absolute terms. 4. We propose that high resting perfusion is important in patients with sickle cell disease to maintain normal integrity of cutaneous tissue and that pronounced vasoconstriction on dependency hinders the healing and encourages recurrence of leg ulcers.
摘要
  1. 慢性腿部溃疡是纯合子镰状细胞病患者发病的主要原因;溃疡通常在卧床休息时愈合。因此,我们比较了三组各8名镰状细胞病患者(有活动性溃疡的患者、有溃疡瘢痕的患者和无溃疡病史的患者)以及8名血红蛋白正常且无腿部溃疡病史的受试者在腿部下垂时皮肤血管的反应。2. 我们用激光多普勒血流仪监测了在踝关节近端两个部位,腿部水平时以及将腿部置于下垂位置5分钟和10分钟后,腿部皮肤中红细胞(红细胞)通量的变化。3. 腿部水平时,镰状细胞病患者正常皮肤中的平均皮肤红细胞通量显著高于正常受试者,在溃疡或瘢痕部位更高。腿部下垂时,正常受试者以及镰状细胞病患者的正常皮肤和溃疡或瘢痕部位的红细胞通量均下降;任何一组在5分钟和10分钟时的值均无差异。将镰状细胞病患者正常皮肤中红细胞通量的下降幅度与对照值相比(有活动性溃疡、有溃疡瘢痕和无溃疡病史的镰状细胞病患者以及正常受试者分别为32%、36%、30%和61%),其下降幅度小于正常受试者,但绝对下降量在镰状细胞病患者和正常受试者中相似。相比之下,溃疡或瘢痕部位红细胞通量的下降幅度无论是与对照值相比(有活动性溃疡或溃疡瘢痕的患者分别为48%和49%)还是绝对下降量,都大于镰状细胞病患者的正常皮肤。4. 我们认为,高静息灌注对镰状细胞病患者维持皮肤组织的正常完整性很重要,而腿部下垂时明显的血管收缩会阻碍愈合并促使腿部溃疡复发。

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