Lee Jyh-Hong, Tsai Wen-Yu, Chou Hung-Chieh, Tung Yi-Ching, Hsieh Wu-Shiun
Department of Pediatrics, College of Medicine, National Taiwan University, Taipei, Taiwan.
J Formos Med Assoc. 2003 Dec;102(12):883-6.
Neonatal diabetes mellitus (NDM) is a very rare disease defined as hyperglycemia that occurs during the first month of life, requires insulin treatment, and lasts more than 2 weeks. There are 2 types of NDM: permanent neonatal diabetes mellitus (PNDM) and transient neonatal diabetes mellitus (TNDM). We report a case of PNDM in a 3-day-old female infant. This full-term neonate was born small for gestational age. Respiratory distress, poor activity, hypothermia, poor feeding, dehydration, and ketoacidosis were noted at the age of 3 days. After insulin therapy and fluid replacement, her condition became stable. Glucagon test done at the age of 26 days showed serum C-peptide level to be low for her age. During the first year of life she had catch-up growth, but insulin therapy was still required. Serum C-peptide level was undetectable at the age of 15 months. The course of this case indicates the importance of a high index of suspicion for patients with PNDM in order to correct metabolic derangement as early as possible and facilitate normal growth and development under insulin therapy.
新生儿糖尿病（NDM）是一种非常罕见的疾病，定义为在出生后第一个月内发生的高血糖症，需要胰岛素治疗，且持续超过2周。NDM有两种类型：永久性新生儿糖尿病（PNDM）和暂时性新生儿糖尿病（TNDM）。我们报告一例3日龄女婴的PNDM病例。这名足月儿出生时小于胎龄。出生3天时出现呼吸窘迫、活动差、体温过低、喂养困难、脱水和酮症酸中毒。经过胰岛素治疗和补液后，她的病情稳定。出生26天时进行的胰高血糖素试验显示，其血清C肽水平低于同龄人。在生命的第一年，她实现了追赶生长，但仍需要胰岛素治疗。15个月大时血清C肽水平检测不到。该病例的病程表明，对于PNDM患者高度怀疑很重要，以便尽早纠正代谢紊乱，并在胰岛素治疗下促进正常生长发育。
