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[骨硬化症(阿尔伯斯-尚伯格病)。III. 当前的疾病分类学方面以及与其他致密性骨病的关系]

[Osteopetrosis (Albers-Schoenberg disease). III. Current nosographic aspects and relations to other condensing osteopathies].

作者信息

Lovisetto P, Donetto L, Biarese V, Marchi L

出版信息

Minerva Med. 1977 Apr 7;68(17):1115-25.

PMID:193069
Abstract

A malignant paediatric variety and an adult variety of Albers-Schönberg disease are normally distinguished. On the basis of recent findings and personal observation it would appear advisable to accept two different courses of Albers-Schönberg disease in adults: one resembling the malignant infant form and the other with slow, practically asymptomatic (apart, obviously, from the skeletal lesions) course allowing for prolonged survival. This classification is of considerable practical importance for prognosis and therapeutic purposes. Other hereditary-familial and constitutional condensing osteopathy pictures exist that present radiological stigmata similar to those seen in Albers-Schönberg disease. The interest of the relations between A-S disease and certain of these condensing osteopathic conditions is obvious.

摘要

通常可区分出恶性小儿型和成人型阿尔伯斯-尚伯格病。基于近期的研究发现和个人观察,似乎有必要承认成人阿尔伯斯-尚伯格病存在两种不同病程:一种类似于恶性婴儿型,另一种病程缓慢,几乎没有症状(显然,除了骨骼病变外),可长期存活。这种分类对于预后和治疗目的具有相当重要的实际意义。还存在其他遗传性-家族性和体质性骨硬化症影像,其呈现出与阿尔伯斯-尚伯格病相似的放射学特征。阿尔伯斯-尚伯格病与某些此类骨硬化症之间关系的重要性显而易见。

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