Literature DB >> 2278013

Hutchinson-Gilford progeria syndrome in siblings. Report of three new cases.

J U Monu1, L B Benka-Coker, Y Fatunde.   

Abstract

The Hutchinson-Gilford progeria syndrome is a rare, inherited, pediatric condition with features of premature and accelerated aging. The pattern of inheritance is uncertain though both autosomal dominant and autosomal recessive modes have been proposed. The patients usually present after the 1st year of life with progressive skin and skeletal changes that give rise to a characteristic physical appearance. Three siblings seen at the University of Benin Teaching Hospital are described in this report, the third documenting the occurrence of progeria in African black patients. The two older siblings show the classic physical and radiologic changes described in progeria whereas the third, a 2-year-old boy, manifests only the early physical and radiologic changes of the disease. We compare the radiologic features of progeria with those of other progeroid conditions: acrogeria, Werner's and Cockayne's syndromes.

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Year:  1990        PMID: 2278013     DOI: 10.1007/bf00241281

Source DB:  PubMed          Journal:  Skeletal Radiol        ISSN: 0364-2348            Impact factor:   2.199


  35 in total

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Journal:  J Bone Joint Surg Am       Date:  1982-04       Impact factor: 5.284

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Journal:  Eur J Pediatr       Date:  1977-01-26       Impact factor: 3.183

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Authors:  P P Franklyn
Journal:  Clin Radiol       Date:  1976-07       Impact factor: 2.350

Review 10.  Cockayne syndrome: unusual neuropathological findings and review of the literature.

Authors:  D Soffer; H W Grotsky; I Rapin; K Suzuki
Journal:  Ann Neurol       Date:  1979-10       Impact factor: 10.422

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  2 in total

1.  Generation of a Hutchinson-Gilford progeria syndrome monkey model by base editing.

Authors:  Fang Wang; Weiqi Zhang; Qiaoyan Yang; Yu Kang; Yanling Fan; Jingkuan Wei; Zunpeng Liu; Shaoxing Dai; Hao Li; Zifan Li; Lizhu Xu; Chu Chu; Jing Qu; Chenyang Si; Weizhi Ji; Guang-Hui Liu; Chengzu Long; Yuyu Niu
Journal:  Protein Cell       Date:  2020-07-29       Impact factor: 14.870

2.  Hutchinson-Gilford progeria syndrome accompanied by severe skeletal abnormalities in two Chinese siblings: two case reports.

Authors:  Zhimin Xiong; Yanmei Lu; Jinjie Xue; Sanchuan Luo; Xiaojuan Xu; Lusi Zhang; Hao Peng; Wei Li; Dengming Chen; Zhengmao Hu; Kun Xia
Journal:  J Med Case Rep       Date:  2013-03-08
  2 in total

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