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很久以前已报道过胸腺不发育伴特发性甲状旁腺功能低下有典型的面部表现及严重的先天性心脏病。DiGeorge将这些情况与免疫学联系起来。本文介绍了一种简单的指数,将完全型(cDGS)与部分型(pDGS)DiGeorge综合征区别开来。作者自1980~1984年在联邦德国汉诺威城从小儿心脏科和内分泌科住院病人中诊断出16例DiGeorge综合征患儿,其中13例为pDGS,当时年龄为1岁(1天~15.5岁),女8男5;cDGS年龄为2天(1~7天),皆为女婴。
It has been reported long ago that hypothymic hypoplasia with idiopathic hypoparathyroidism has typical facial manifestations and severe congenital heart disease. DiGeorge relates these conditions to immunology. This article describes a simple index that distinguishes between complete (cDGS) and partial (pDGS) DiGeorge syndromes. The authors diagnosed 16 children with DiGeorge syndrome from pediatric cardiology and endocrinology inpatients from Hanover, Germany, from 1980 to 1984, of whom 13 were pDGS and were 1 year old (1 day to 15.5 years old) Female 8 male 5; cDGS age 2 days (1 ~ 7 days), all for the baby girl.