LONG-TERM COURSE OF NEONATAL DIABETES
LONG-TERM COURSE OF NEONATAL DIABETES
复制标题
DOI：
10.1056/nejm199509143331105
复制
发表时间：
1995-09-14
影响因子：
158.5
通讯作者：
HERKENHOFF, H
中科院分区：
文献类型：
--
作者：
VONMUHLENDAHL, E;HERKENHOFF, H
背景新生儿糖尿病在这里定义为在生命的第一个月内发生的高血糖症，持续至少两周，需要胰岛素治疗-是一种非常罕见的疾病。人们对它知之甚少，特别是关于它的长期进程。我们研究了患有新生儿糖尿病的两兄弟，并获得了文献中描述的34例患者的随访信息以及另外21例患者的信息。47例患者患有上述定义的新生儿糖尿病，另外10例患者在出生后第1个月至第3个月发病。57名婴儿中有26名患有永久性糖尿病，18名患有短暂性糖尿病，13名患有7至20岁时复发的短暂性糖尿病。新生儿糖尿病与Wolcott-Rallison综合征有关的6名婴儿，高尿酸血症由于磷酸核糖ATP焦磷酸酶活性亢进的2名，和腹腔疾病的2名。45名新生儿中有41名的妊娠期和出生体重是已知的，他们的胎龄较小。有两对受影响的双胞胎和其他四个家庭有两个或更多的新生儿糖尿病婴儿，但只有三个父母患有糖尿病。德国新生儿糖尿病的发病率估计为1/500，000新生儿。新生儿糖尿病与胰岛素依赖型糖尿病的不同之处在于其病程高度可变。有些患者有永久性糖尿病，但其他人有短暂或持久的缓解。
Background. Neonatal diabetes mellitus defined here as hyperglycemia occurring within the first month of life that lasts for at least two weeks and requires insulin therapy - is a very rare form of the disease. Little is known about it, particularly with respect to its long-term course.Methods. We studied two brothers who had neonatal diabetes and obtained follow-up information on 34 patients described in the literature as well as information on 21 additional patients. Forty-seven of the patients had neonatal diabetes, as defined above, and in 10 others the onset was between the first and third month of life.Results. Twenty-six of the 57 infants had permanent diabetes, 18 had transient diabetes, and 13 had transient diabetes that recurred when they were 7 to 20 years old. Neonatal diabetes was associated with the Wolcott-Rallison syndrome in six infants, hyperuricemia due to phosphoribosyl-ATP pyrophosphatase hyperactivity in two, and celiac disease in two. Forty-one of 45 neonates in whom the duration of gestation and birth weight were known were small for their gestational ages. There were two pairs of affected twins and four other families with two or more infants with neonatal diabetes, but only three parents had diabetes. The incidence of neonatal diabetes mellitus in Germany has been estimated to be 1 in 500,000 neonates.Conclusions. Neonatal diabetes differs from insulin-dependent diabetes in that its course is highly variable. Some patients have permanent diabetes, but others have transient or lasting remissions.