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[埃勒斯-当洛综合征和马方综合征患儿尿中吡啶啉的排泄及β受体阻滞剂和复合维生素疗法对其的影响]

[Excretion of pyridinoline with urine in children with Ehlers-Danlos syndrome and Marfan's syndrome and the effect of beta blocker and complex vitamin therapy on it].

作者信息

Prozorovskaia N N, Kozlov E A, Luk'ianov S V, Rudakov S S, Prozorovskiĭ V N, Del'vig A A

出版信息

Vopr Med Khim. 1991 Mar-Apr;37(2):66-9.

PMID:1680259
Abstract

Excretion of pyridinoline and polypeptide-bound hydroxyproline with urine was studied in 27 children with hereditary impairment of connective tissue. At the same time, effects of beta-adrenoblocking agents and vitamin complex, prescribed during preoperation period before thoracoplasty in hereditary chest deformation, were investigated. Clinical efficiency of the treatment depended distinctly on the initial value of ratios pyridinoline/creatinine and polypeptide-bound hydroxyproline/creatinine. Total positive effect of the therapeutic course, considering also echocardiographic examination and postoperational complications, was observed in 44% of patients with Ehlers-Dunlop syndrome and in 75% of patients with Marfan syndrome.

摘要

对27名患有遗传性结缔组织损伤的儿童进行了尿中吡啶啉和多肽结合羟脯氨酸排泄情况的研究。同时,对遗传性胸廓畸形患者在胸廓成形术前的围手术期使用β-肾上腺素能阻滞剂和复合维生素的效果进行了调查。治疗的临床效果明显取决于吡啶啉/肌酐和多肽结合羟脯氨酸/肌酐比值的初始值。综合超声心动图检查和术后并发症来看,治疗过程的总体积极效果在44%的埃勒斯-当洛综合征患者和75%的马凡综合征患者中观察到。

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