Clinical features of 13 cases with neonatal diabetes mellitus

Abstract

Objectives To analyze the clinical features among 13 cases of neonatal diabetes mellitus (NDM). Methods Thirteen cases with NDM received treatment in our department between July 2001 and June 2012.The clinical features were reviewed retrospectively. Results The age at diagnosis was 0.4-5.0 months, the median age of diagnosis was 3 months. Seven cases were small for gestational age(SGA) infants. Infection presented in 8 cases, 2 cases presented with viscose urine, 2 cases presented with poor reaction, 2 cases presented with poor appetite, 1 case presented with seizure. Seizure was seen in 4 cases. Six cases had diabetic ketoacidosis(DKA). The mean glycated hemoglobin A1c(HbA1c) at onset was 10.0%(7.4%-14.2%). Insulin treatment was started in all 13 patients, the initial dose was 1.0-1.2 U·kg-1·d-1, and their blood glucose was well under control. Among the 13 cases followed up, 8 cases were verified as permanent NDM(PNDM), 4 were transient NDM(TNDM), 1 case missed after discharge. One child had skeletal dysplasia and was diagnosed as Wolcott-Rallison syndrome. Six cases were treated with glyburide, only 1 boy who diagnosed as developmental delay, epilepsy and neonatal diabetes syndrome(DEND) got good sugar control, 2 cases stopped glyburide treatment for gastrointestinal side effects. Unfortunately, the patient diagnosed with DEND died at the age of 2.5 years because of DKA, another case died of hepatic and renal failure at the age of 1.5 years. The blood glucose of most patients was well controlled at the follow-up. Conclusions The clinical expressions of NDM were atypical and easily miss-diagnosed. Of all the NDM cases, up to 31% was TNDM and relieved automatically. Insulin was the best choice before genetic identification. Key words: Diabetes mellitus, permanent neonatal; Diabetes mellitus, transient neonatal; Insulin; Glyburide