PROTEUS SYNDROME

被引：15

作者：

BARMAKIAN, JT ^{[1
]}

POSNER, MA ^{[1
]}

SILVER, L ^{[1
]}

LEHMAN, W ^{[1
]}

VINE, DT ^{[1
]}

机构：

[1] HOSP JOINT DIS & MED CTR,INST ORTHOPAED,HAND & PEDIAT ORTHOPAED SERV,NEW YORK,NY 10003

来源：

JOURNAL OF HAND SURGERY-AMERICAN VOLUME | 1992年 / 17A卷 / 01期

关键词：

D O I：

10.1016/0363-5023(92)90109-3

中图分类号：

R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学（修复外科学）];

学科分类号：

摘要：

Proteus syndrome is a rare congenital disorder that is characterized by a wide variety of deformities including macrodactyly. Skin and soft tissue lesions are common; they may increase in size as the child develops and may assume tremendous proportions. The syndrome is often mistaken for other more commonly recognized conditions such as neurofibromatosis. Unlike neurofibromatosis, the soft tissue masses in Proteus syndrome are not nerve tumors but, rather, are hamartomas composed primarily of lipomatous tissue. The hand surgeon should be aware of this condition when evaluating a child with macrodactyly.

引用

页码：32 / 34

页数：3

共 8 条

[1]

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[2]

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[3]

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[4]

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[5]

Costa, Fitch, Azouz, Proteus syndrome: report of two cases with pelvic lipomatosis, Pediatrics, 70, pp. 984-989, (1985)

[6]

Viljoen, Nelson, de Jong, Beighton, Proteus syndrome in Southern Africa: natural history and clinical manifestations in six individuals, Am J Med Genet, 27, pp. 87-97, (1987)

[7]

Clark, Donnai, Rogers, Cooper, Baraitser, Proteus syndrome: an expanded phenotype, Am J Med Genet, 27, pp. 99-117, (1987)

[8]

Malamitsi-Puchner, Kitsiou, Bartsocas, Brief clinical report: severe Proteus syndrome in an 18-month-old boy, Am J Med Genet, 27, pp. 119-125, (1987)

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