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在基因诊断前,通过持续皮下胰岛素输注成功治疗患有新生儿糖尿病的幼儿。

Successful treatment of young infants presenting neonatal diabetes mellitus with continuous subcutaneous insulin infusion before genetic diagnosis.

作者信息

Rabbone Ivana, Barbetti Fabrizio, Marigliano Marco, Bonfanti Riccardo, Piccinno Elvira, Ortolani Federica, Ignaccolo Giovanna, Maffeis Claudio, Confetto Santino, Cerutti Franco, Zanfardino Angela, Iafusco Dario

机构信息

Department of Pediatrics, Regina Margherita Hospital, University of Turin, Piazza Polonia 94, Turin, Italy.

Department of Experimental Medicine and Surgery, University of Tor Vergata, Rome, Italy.

出版信息

Acta Diabetol. 2016 Aug;53(4):559-65. doi: 10.1007/s00592-015-0828-7. Epub 2016 Feb 1.

Abstract

AIMS

Neonatal diabetes mellitus (NDM) is defined as hyperglycemia and impaired insulin secretion with onset within 6 months of birth. While rare, NDM presents complex challenges regarding the management of glycemic control. The availability of continuous subcutaneous insulin infusion pumps (CSII) in combination with continuous glucose monitoring systems (CGM) provides an opportunity to monitor glucose levels more closely and deliver insulin more safely.

METHODS

We report four cases of young infants with NDM successfully treated with CSII and CGM. Moreover, in two cases with Kir 6.2 mutation, we describe the use of CSII in switching therapy from insulin to sulfonylurea treatment.

RESULTS

Insulin pump requirement for the 4 neonatal diabetes cases was the same regardless of disease pathogenesis and c-peptide levels. No dilution of insulin was needed. The use of an integrated CGM system helped in a more precise control of BG levels with the possibility of several modifications of insulin basal rates. Moreover, as showed in the first two case-reports, when the treatment was switched from insulin to glibenclamide, according to identification of Kir 6.2 mutation and diagnosis of NPDM, the CSII therapy demonstrated to be helpful in allowing gradual insulin suspension and progressive introduction of sulfonylurea.

CONCLUSIONS

During the neonatal period, the use of CSII therapy is safe, more physiological, accurate and easier for the insulin administration management. Furthermore, CSII therapy is safe during the switch of therapy from insulin to glibenclamide for infants with permanent neonatal diabetes mellitus.

摘要

目的

新生儿糖尿病(NDM)定义为出生后6个月内出现的高血糖和胰岛素分泌受损。虽然罕见,但NDM在血糖控制管理方面带来了复杂的挑战。持续皮下胰岛素输注泵(CSII)与持续葡萄糖监测系统(CGM)的联合应用为更密切地监测血糖水平和更安全地注射胰岛素提供了机会。

方法

我们报告了4例使用CSII和CGM成功治疗的NDM幼儿病例。此外,在2例伴有Kir 6.2突变的病例中,我们描述了在从胰岛素治疗转换为磺脲类药物治疗时CSII的使用情况。

结果

4例新生儿糖尿病病例对胰岛素泵的需求与疾病发病机制和C肽水平无关。无需稀释胰岛素。集成CGM系统的使用有助于更精确地控制血糖水平,并有可能对胰岛素基础率进行多次调整。此外,如前两个病例报告所示,当根据Kir 6.2突变的鉴定和永久性新生儿糖尿病的诊断将治疗从胰岛素转换为格列本脲时,CSII治疗有助于逐渐停用胰岛素并逐步引入磺脲类药物。

结论

在新生儿期,CSII治疗安全、更符合生理、准确且便于胰岛素给药管理。此外,对于永久性新生儿糖尿病患儿,在从胰岛素治疗转换为格列本脲治疗期间,CSII治疗是安全的。

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