Horm Metab Res 2009; 41(7): 580-582
DOI: 10.1055/s-0029-1192020
Short Communication

© Georg Thieme Verlag KG Stuttgart · New York

Transfer from Insulin to Sulfonylurea Treatment in a Chinese Patient with Permanent Neonatal Diabetes Mellitus due to a KCNJ11 R201H Mutation

X. Xiao 1 , T. Wang 1 , W. Li 1 , H. Song 2 , C. Gong 3 , C. Diao 1 , M. Yu 1 , T. Yuan 1 , Y. Zhang 1 , X. Sun 1 , Q. Zhang 1 , K. Lu 2 , H. Wang 1 , O. Schmitz 4 , T. Hansen 5
  • 1Key Laboratory of Endocrinology, Ministry of Health, Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
  • 2Department of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
  • 3Department of Endocrinology, Beijing Children's Hospital, Beijing, China
  • 4Department of Endocrinology and Diabetes, Aarhus University Hospital, Aarhus C, Denmark
  • 5Steno Diabetes Center Niels Steensens Vej 1, Gentofte, Denmark
Further Information

Publication History

received 20.10.2008

accepted 06.01.2009

Publication Date:
26 February 2009 (online)


Neonatal diabetes mellitus (NDM) is a rare and specific type of diabetes which is characterized by onset of hyperglycemia within the first 6 months of life. The reported incidence of NDM is 1:300 000–400 000 in newborns [1] [2]. It is clinically classified into two groups: transient neonatal diabetes mellitus (TNDM) and permanent neonatal diabetes mellitus (PNDM). TNDM usually spontaneously resolves within the first months of life, with possible recurrence in childhood or adolescent stage. It represents 50% to 60% of cases of NDM, and is often associated with an abnormality of the imprinted region of 6q24 [1] [3]. In contrast, PNDM needs a lifelong medication. It has been recognized that heterozygous activating mutation in the KCNJ11 gene which encodes the Kir6.2 subunits of the ATP sensitive K+ channel (KATP channel) is a major cause of PNDM [1] [3]. And cases have been reported in which a better glycemic control was obtained after initiation of oral sulfonylurea treatment compared to insulin therapy [4] [5]. Here we report the first Chinese case of PNDM with a KCNJ11 R201H mutation and the successful transition from insulin injections to oral sulfonylurea therapy.


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X. Xiao

Department of Endocrinology

Peking Union Medical College Hospital

Chinese Academy of Medical Sciences & Peking Union Medical College

100730 Beijing

P. R. China

Phone: +86/10/6529 50 73

Fax: +86/10/6529 40 70

Email: [email protected]