[镰状细胞贫血患者异常的自主心血管反应]

[Abnormal autonomic cardiovascular responses in patients with sickle cell anemia].

作者信息

Romero-Vecchione E, Pérez O, Wessolosky M, Rosa F, Liberatore S, Vásquez J

机构信息

Cátedra de Farmacología, Escuela de Medicina J.M. Vargas, Universidad Central de Venezuela.

出版信息

Sangre (Barc). 1995 Oct;40(5):393-9.

PMID:8553174

Abstract

PURPOSE

To evaluate the presence of anomalies of the autonomic reflex cardiovascular response in patients with chronic sickle-cell anaemia.

PATIENTS AND METHODS

The study was extended to 30 patients with sickle-cell anaemia, 10 patients with iron-lack anaemia and 30 healthy subjects. Age and sex distribution was similar in each group. To be included in the study, patients should have had no painful crisis or blood transfusion in the 6 months previous to the assay. Clinico-laboratory survey, chest x-ray and EKG were performed in every case. Blood cell count and abnormal haemoglobin study on cellulose acetate were carried out as well. The evaluation of reflex autonomic responses was performed by means of active orthostatism, cold pressor test, Valsalva maneuver and urine catecholamine output. The statistical analysis was performed with the variance analysis (ANOVA) for multiple groups.

RESULTS

The following abnormalities were found: 12 patients had haemoglobin SS, 8 had haemoglobin SS and F, 3 had haemoglobin SC, 2 had haemoglobin S and beta-thalassaemia, and 5 had combined haemoglobin SS,F and A2. Systolic pressure and heart frequency in the supine position were similar in all groups. Diastolic pressure was lower in the sickle-cell anaemia group with respect to the normals. Patients with sickle-cell disease had lower heart frequency in the active orthostatism test with regard to the other groups, along with paradoxal changes in systolic pressure and lesser increase of the diastolic pressure. Significantly lower response to the cold pressor test was seen in the sickle-cell patients as compared with the iron-lack cases and the normal controls. Reduced sympathetic tachycardia was seen with the Valsalva maneuver, whereas the bradycardia was similar to the other groups. The urine noradrenaline in output was significantly lower in the sickle-cell patients, it was normal in the other groups (p < 0.01).

CONCLUSION

These results suggest a defective sympathetic activity of heart and arteries in patients with sickle-cell anaemia.

摘要

目的

评估慢性镰状细胞贫血患者自主反射性心血管反应异常的情况。

患者与方法

该研究纳入了30例镰状细胞贫血患者、10例缺铁性贫血患者和30名健康受试者。每组的年龄和性别分布相似。要纳入该研究，患者在检测前6个月内不应有疼痛性危机或输血情况。对每例患者均进行临床实验室检查、胸部X线检查和心电图检查。还进行了血细胞计数以及醋酸纤维素异常血红蛋白研究。通过主动直立试验、冷加压试验、瓦尔萨尔瓦动作和尿儿茶酚胺排出量评估自主反射反应。采用多组方差分析（ANOVA）进行统计分析。

结果

发现以下异常情况：12例患者为血红蛋白SS型，8例为血红蛋白SS和F型，3例为血红蛋白SC型，2例为血红蛋白S和β地中海贫血型，5例为血红蛋白SS、F和A2型组合。所有组仰卧位时的收缩压和心率相似。镰状细胞贫血组的舒张压低于正常组。与其他组相比，镰状细胞病患者在主动直立试验中的心率较低，同时收缩压出现反常变化，舒张压升高幅度较小。与缺铁性贫血患者和正常对照组相比，镰状细胞病患者对冷加压试验的反应明显降低。瓦尔萨尔瓦动作时交感神经心动过速减弱，而心动过缓与其他组相似。镰状细胞病患者的尿去甲肾上腺素排出量明显较低，其他组则正常（p<0.01）。