{"title":"[粘多糖病ⅱ- b型临床特征及x线表现]。","authors":"Q Lan","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Mucopolysaccharidosis-II (MPS-II) and MPS-I are both characterized by gargoylist dwarfism. MPS-II is of X-linked recessive heredity. Five cases of MPS-II-B in our series were followed up for ten years. Corneal opacity and dysmnesia were not found in all of them. Although MPS-II possessed many radiologic similarities to MPS-I, the former presented certain characteristic features such as butterfish-like vertebra, and small triangular malformation of carpal bones and tarsal scaphoid etc.</p>","PeriodicalId":39377,"journal":{"name":"Zhonghua fang she xue za zhi Chinese journal of radiology","volume":"23 5","pages":"287-9"},"PeriodicalIF":0.0000,"publicationDate":"1989-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Clinical features and X-ray manifestations of mucopolysaccharidosis-II-B].\",\"authors\":\"Q Lan\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Mucopolysaccharidosis-II (MPS-II) and MPS-I are both characterized by gargoylist dwarfism. MPS-II is of X-linked recessive heredity. Five cases of MPS-II-B in our series were followed up for ten years. Corneal opacity and dysmnesia were not found in all of them. Although MPS-II possessed many radiologic similarities to MPS-I, the former presented certain characteristic features such as butterfish-like vertebra, and small triangular malformation of carpal bones and tarsal scaphoid etc.</p>\",\"PeriodicalId\":39377,\"journal\":{\"name\":\"Zhonghua fang she xue za zhi Chinese journal of radiology\",\"volume\":\"23 5\",\"pages\":\"287-9\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1989-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Zhonghua fang she xue za zhi Chinese journal of radiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Zhonghua fang she xue za zhi Chinese journal of radiology","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
粘多糖病- ii (MPS-II)和MPS-I都具有石像状侏儒症的特征。MPS-II为x连锁隐性遗传。我们对5例MPS-II-B进行了10年的随访。所有患者均未见角膜混浊和失忆。虽然MPS-II与MPS-I在放射学上有许多相似之处,但前者具有一定的特征性特征,如蝶形椎体,腕骨和跗骨舟状骨的小三角形畸形等。
[Clinical features and X-ray manifestations of mucopolysaccharidosis-II-B].
Mucopolysaccharidosis-II (MPS-II) and MPS-I are both characterized by gargoylist dwarfism. MPS-II is of X-linked recessive heredity. Five cases of MPS-II-B in our series were followed up for ten years. Corneal opacity and dysmnesia were not found in all of them. Although MPS-II possessed many radiologic similarities to MPS-I, the former presented certain characteristic features such as butterfish-like vertebra, and small triangular malformation of carpal bones and tarsal scaphoid etc.