The Journal of Pediatrics
Volume 150, Issue 5 , Pages 553-555 , May 2007

Sulfonylurea-Responsive Diabetes in Childhood

  • Zohar Landau, MD

      Affiliations

    • Diabetes Unit, E. Wolfson Medical Center, Holon, and Tel Aviv University Sackler Faculty of Medicine, Tel Aviv, Israel
    • Pediatric Endocrinology Unit, E. Wolfson Medical Center, Holon, and Tel Aviv University Sackler Faculty of Medicine, Tel Aviv, Israel
    • ,
  • Julio Wainstein, MD

      Affiliations

    • Diabetes Unit, E. Wolfson Medical Center, Holon, and Tel Aviv University Sackler Faculty of Medicine, Tel Aviv, Israel
    • ,
  • Aaron Hanukoglu, MD

      Affiliations

    • Pediatric Endocrinology Unit, E. Wolfson Medical Center, Holon, and Tel Aviv University Sackler Faculty of Medicine, Tel Aviv, Israel
    • ,
  • Myriam Tuval, PhD

      Affiliations

    • Endocrine Laboratory, E. Wolfson Medical Center, Holon and Tel Aviv University Sackler Faculty of Medicine, Tel Aviv, Israel
    • ,
  • Judith Lavie, MSc

      Affiliations

    • Endocrinology and Metabolism Service, Internal Medicine Department, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
    • ,
  • Benjamin Glaser, MD

      Affiliations

    • Endocrinology and Metabolism Service, Internal Medicine Department, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
    • Corresponding Author InformationReprint requests: Benjamin Glaser, MD, Endocrinology and Metabolism Service, Hadassah-Hebrew University Medical Center, POB 12000, 91120 Jerusalem, Israel.

Received 7 October 2006 ,Revised 16 January 2007 ,Accepted 1 March 2007.

References 

  1. Shield JP, Gardner RJ, Wadsworth EJ, Whiteford ML, James RS, Robinson DO, et al. Aetiopathology and genetic basis of neonatal diabetes. Arch Dis Child Fetal Neonatal Ed. 1997;76:F39–F42
  2. Gloyn AL, Reimann F, Girard C, Edghill EL, Proks P, Pearson ER, et al. Relapsing diabetes can result from moderately activating mutations in KCNJ11. Hum Mol Genet. 2005;14:925–934
  3. Babenko AP, Polak M, Cave H, Busiah K, Czernichow P, Scharfmann R, et al. Activating mutations in the ABCC8 gene in neonatal diabetes mellitus. N Engl J Med. 2006;355:456–466
  4. von Muhlendahl KE, Herkenhoff H. Long-term course of neonatal diabetes. N Engl J Med. 1995;333:704–708
  5. Nestorowicz A, Inagaki N, Gonol T, Schoor KP, Wilson BA, Glaser B, et al. A nonsense mutation in the inward rectifier potassium channel gene, KIR6.2, is associated with familial hyperinsulinism. Diabetes. 1997;46:1743–1748
  6. Hani EH, Boutin P, Durand E, Inoue H, Permutt MA, Velho G, et al. Missense mutations in the pancreatic islet beta cell inwardly rectifying K+ channel gene (KIR6.2/BIR): a meta-analysis suggests a role in the polygenic basis of Type II diabetes mellitus in Caucasians. Diabetologia. 1998;41:1511–1515
  7. Flanagan S, Edghill E, Gloyn A, Mackay D, Temple I, Shield J, et al. Activating KCNJ11 gene mutations are a common cause of remitting diabetes that may be diagnosed as transient neonatal diabetes. Diabetic Medicine. 2006;23:1
  8. Yorifuji T, Nagashima K, Kurokawa K, Kawai M, Oishi M, Akazawa Y, et al. The C42R mutation in the Kir6.2 (KCNJ11) gene as a cause of transient neonatal diabetes, childhood diabetes, or later-onset, apparently type 2 diabetes mellitus. J Clin Endocrinol Metab. 2005;90:3174–3178
  9. Gloyn AL, Pearson ER, Antcliff JF, Proks P, Bruining GJ, Slingerland AS, et al. Activating mutations in the gene encoding the ATP-sensitive potassium-channel subunit Kir6.2 and permanent neonatal diabetes. N Engl J Med. 2004;350:1838–1849
  10. Hattersley AT, Ashcroft FM. Activating mutations in Kir6.2 and neonatal diabetes: new clinical syndromes, new scientific insights, and new therapy. Diabetes. 2005;54:2503–2513
  11. Sagen JV, Raeder H, Hathout E, Shehadeh N, Gudmundsson K, Baevre H, et al. Permanent neonatal diabetes due to mutations in KCNJ11 encoding Kir6.2: patient characteristics and initial response to sulfonylurea therapy. Diabetes. 2004;53:2713–2718
  12. Flanagan SE, Edghill EL, Gloyn AL, Ellard S, Hattersley AT. Mutations in KCNJ11, which encodes Kir6.2, are a common cause of diabetes diagnosed in the first 6 months of life, with the phenotype determined by genotype. Diabetologia. 2006;49:1190–1197

 Supported by a grant from the Russell Berrie Foundation and D Cure, Diabetes Care in Israel.

PII: S0022-3476(07)00239-9

doi: 10.1016/j.jpeds.2007.03.004

The Journal of Pediatrics
Volume 150, Issue 5 , Pages 553-555 , May 2007

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