口服磺脲类药物治疗短暂性新生儿糖尿病。

An oral sulfonylurea in the treatment of transient neonatal diabetes mellitus.

作者信息

Loomba-Albrecht Lindsey A, Glaser Nicole S, Styne Dennis M, Bremer Andrew A

机构信息

Department of Pediatrics, Division of Endocrinology, University of California Davis Medical Center, Sacramento, California 95817-2208, USA.

出版信息

Clin Ther. 2009 Apr;31(4):816-20. doi: 10.1016/j.clinthera.2009.04.003.

DOI:10.1016/j.clinthera.2009.04.003

PMID:19446154

Abstract

BACKGROUND

Neonatal diabetes mellitus (NDM) is rare, with a prevalence of approximately 1 in 500,000 infants worldwide. NDM may be caused by several different genetic abnormalities, and might either be transient (TNDM) or permanent. Until recently, clinical management of most permanent types of NDM required lifelong subcutaneous insulin treatment. However, due to activating mutations in the genes that encode the adenosine triphosphate-sensitive K(+) channel, some permanent types of NDM have been found to be amenable to oral sulfonylurea therapy. TNDM can last for a median of 12 weeks and completely resolve by 18 months. Although TNDM is typically treated with subcutaneous insulin, this mode of therapy might be difficult for some caregivers.

CASE SUMMARY

A small for gestational age male infant born at term developed NDM on day of life (DOL) 3. No other factors, such as sepsis, infection, or dextrose-containing intravenous fluids, that could have accounted for the hyperglycemia were present. In addition, there was no family history of DM or hyper-glycemic disorders. The patient was initially treated with subcutaneous regular insulin (0.25 U at a concentration of 10 U/L) q4h PRN for blood glucose concentrations >200 mg/dL. However, due to persistent blood glucose concentration fluctuations, a continuous insulin infusion (0.05 U/kg/h) was started on DOL 7. Because subcutaneous insulin injections could not be administered by the parents outside of the hospital, oral sulfonylurea therapy was attempted. A glyburide oral suspension, prepared by dissolving half of a 1.25-mg tablet in 1 mL of preservative-free, sterile water, was started at 0.2 mg/kg/d in 2 divided doses. The suspension was prepared immediately prior to each dose and was administered via syringe during feedings. On DOL 21, the patient's NDM was managed solely with an oral sulfonylurea, target blood glucose concentrations of 150 to 250 mg/dL were achieved with glyburide 0.7 mg/kg/d in 2 divided doses, and insulin administration was no longer required. On DOL 25, the glyburide dosage was decreased to 0.5 mg/kg/d in 2 divided doses. On DOL 27, the patient was discharged on the same dosage. The patient's NDM subsequently resolved by DOL 49.

CONCLUSION

An oral sulfonylurea was a useful treatment option in the management of TNDM in this patient.

摘要

背景

新生儿糖尿病（NDM）较为罕见，全球范围内每50万例婴儿中约有1例患病。NDM可能由多种不同的基因异常引起，可分为短暂性（TNDM）或永久性。直到最近，大多数永久性NDM的临床管理都需要终身皮下胰岛素治疗。然而，由于编码三磷酸腺苷敏感性钾（K+）通道的基因发生激活突变，已发现某些类型的永久性NDM适合口服磺脲类药物治疗。TNDM的持续时间中位数为12周，18个月时可完全缓解。虽然TNDM通常采用皮下胰岛素治疗，但这种治疗方式对一些护理人员来说可能有困难。

病例摘要

一名足月出生的小于胎龄男婴婴在333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333333婴儿在出生第3天患NDM。不存在其他可能导致高血糖的因素，如败血症、感染或含葡萄糖的静脉输液。此外，没有糖尿病或高血糖疾病的家族史。患者最初采用皮下注射正规胰岛素（浓度为10 U/L，0.25 U），根据需要每4小时注射一次，用于血糖浓度>200 mg/dL的情况。然而，由于血糖浓度持续波动，在出生第7天开始持续胰岛素输注（0.05 U/kg/h）。由于父母无法在院外进行皮下胰岛素注射，尝试了口服磺脲类药物治疗。将半片1.25 mg片剂溶解在1 mL无防腐剂的无菌水中制备的格列本脲口服混悬液，以0.2 mg/kg/d的剂量分2次给药。每次给药前立即制备混悬液，并在喂奶期间通过注射器给药。在出生第21天，患者的NDM仅通过口服磺脲类药物进行管理，格列本脲剂量为0.7 mg/kg/d，分2次给药，血糖目标浓度达到150至250 mg/dL，不再需要胰岛素给药。在出生第25天，格列本脲剂量降至0.5 mg/kg/d，分2次给药。在出生第27天，患者以相同剂量出院。患者的NDM随后在出生第49天缓解。