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中主动脉综合征系由Takayasu氏病引起降主动脉获得性缩窄,导致年轻成人上肢高血压。然而它可发生于童年,过去已报道过2例。本文再报导2例。例1,女,11岁,因重度活动时不能耐受、心悸和咳嗽有痰4个月入院,无风湿热史。体检。发育,营养不良,体重仅16kg,颈静脉怒张且有搏动。无紫绀和杵状指。右上肢及两下肢脉搏均未触及,左臂动脉压160/90mmHg,血沉50mmH_2O,结素试验阳性。胸片示心影增大而无肋骨切迹。心电图示双心室肥厚,V_1、V_2、V_3导程T波倒置。主动脉造影显示胸部降主动脉和上腹主动脉弥漫性狭窄。经用适量毛地黄、利尿剂、类固醇、利血平及补充营养等治疗后恢复满意,体重增加,血压仅略减低。住院治疗3个月后因病情改善未行手术出院,但4个月后可能因心力衰竭而突然死亡。未作尸检。
Middle aorta syndrome causes acquired descending aorta by Takayasu’s disease, leading to upper extremity hypertension in young adults. However, it can occur in childhood, two cases have been reported in the past. This article reports 2 cases again. Case 1, female, 11 years old, can not tolerate severe activity, palpitations and cough with sputum admitted for 4 months, no history of rheumatic fever. Physical examination. Development, malnutrition, weight only 16kg, jugular vein engorgement and pulsation. No cyanosis and clubbing. Right upper limb and lower extremity pulse were not touched, left arm arterial pressure 160 / 90mmHg, erythrocyte sedimentation rate 50mmH_2O, tuberculin test positive. Chest X-ray showed increased without rib notch. ECG double ventricular hypertrophy, V_1, V_2, V_3 lead T wave inversion. Aortic angiography shows the descending thoracic aorta and the aortic diffuse stenosis. After using the appropriate amount of foxglove, diuretics, steroids, reserpine and nutritional supplements such as recovery satisfaction, weight gain, blood pressure was only slightly reduced. Three months after hospitalization, the patients were discharged without surgery because of their condition improvement. However, they may suddenly die of heart failure after 4 months. No autopsy.