Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females - Report of five cases
Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females - Report of five cases
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DOI：
10.1056/nejm193704292161701
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发表时间：
1937-01-01
影响因子：
158.5
通讯作者：
Smith, P
中科院分区：
文献类型：
--
作者：
Albright, F;Butler, AM;Smith, P
似乎是完全不相关的，却发现在足够多的患者中同时发生，它们之间的某种关系是明显的。这就是本文的主题——奇异综合症的情况。这里报告了5例这种综合征。第六例是在纽约的长老会医院发现的，麦考恩在另一份通讯中报告了这一病例。第七例是最近在奥尔巴尼发现的，将由戈汉姆、坎贝尔和霍华德报告。5此外，在文献检索中，麦考恩和我们自己发现了9例惊人相似的病例和5例疑似相似的病例。该综合征的特点是：(a)骨病变有明显的单侧倾向，组织学检查表现为纤维性骨炎。(b)皮肤的褐色、未升高的色素区，往往与骨骼病变在同一侧。(c)女性男性与性早熟有关的内分泌功能障碍。病例报告病例1。西北（总汇编号330635）。患者，23岁，1933年7月9日入院，主诉右髋关节疼痛15年。她的问题始于8岁，当时她在玩耍时奔跑时右股骨骨折。随后，她的右股骨在不同部位发生了七次骨折。有一处骨折
seem to be totally disconnected, are found to occur together in a sufficient series of patients, some relation between them is apparent. Such is the case in thebizarre syndrome which is the subject of this paper. Five cases of this syn-drome are reported here. A sixth case was seen at the Presbyterian Hospital in New York and is being reported in a separate communication by McCune^ t A seventh case was seen more recently in Albany and will be reported by Gorham, Campbell and Howard. 5t In ad-dition, in a search of the literature McCune and we ourselves have found 9 strikingly similar cases and 5 suggestively similar ones. The characteristics of the syndrome are:(a) Bone lesions which have a marked tend-ency to be unilateral and which show osteitis fibrosa on histologie examination.(b) Brown nonelevated pigmented areas of the skin which tend to be on the same side as the bone lesions.(c) An endocrine dysfunction which in fe-males isassociated with precocious puberty.CASE REPORTS Case 1. NW (M. GH No. 330635). The pa-tient, aged23, entered the hospital, July 9, 1933, complaining of pain in the right hip of 15 years' duration. Her trouble first began at the age of 8 when she sustained a fracture of the right femur while running about at play. She had had seven subsequent fractures of the right femur at different sites. There had been one fracture of the