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单卵双生男性双胞胎中仅一人患进行性单侧面部萎缩:一种可影响面部以外器官的复杂疾病1例
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作者 Hulzebos C. V. de vries t. w. +1 位作者 Armbrust w. 李开 《世界核心医学期刊文摘(儿科学分册)》 2005年第5期14-15,共2页
Progressive facial hemiatrophy (PFH) is a ubiquitous disease, characterized by hyperpigmentation of the skin followed by unilateral craniofacial atrophy of su bcutaneous tissues, including fat, muscle and bone. Heredi... Progressive facial hemiatrophy (PFH) is a ubiquitous disease, characterized by hyperpigmentation of the skin followed by unilateral craniofacial atrophy of su bcutaneous tissues, including fat, muscle and bone. Hereditary factors have been postulated to be involved in the aetiology of PFH. Yet, the occurrence of PFH i n one of two identicalmale twins reported heremakes this possibility unlikely. P FH usually occurs in the first two decades of life, and the clinical presentatio n resembles linear scleroderma. PFH may be complicated by autoimmune, neurologic al, ocular and dental disorders. Management of PFH comprises a long term follow -up of somatic disorders, and prevention of psychological problems. Treatment o f PFH is symptomatic and consists of plastic surgery after the disease activity has stopped. Conclusion: The occurrence of PFH in one of a monozygotic twin pair suggests that genetic factors are not involved in its aetiology. Early diagnosi s of PFH and accurate follow-up is essential to disclose the occurrence of comp lications. 展开更多
关键词 单卵双生 单侧面 硬皮症 躯体障碍 皮肤色素沉着 遗传因素 早期诊断 心理障碍 发病机制 临床症状
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