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Successful subcutaneous glucagon use for persistent hypoglycaemia in congenital hyperinsulinism

  • Orla M. Neylon EMAIL logo , Margaret M. Moran , Anastasia Pellicano , Michael Nightingale and Michele A. O’Connell
Published/Copyright: June 29, 2013
Journal of Pediatric Endocrinology and Metabolism
From the journal Journal of Pediatric Endocrinology and Metabolism Volume 26 Issue 11-12

Abstract

Congenital hyperinsulinism (CHI) results from inappropriate excessive insulin secretion by the beta cells in the pancreas. A wide clinical spectrum of disease exists and a genetic diagnosis is now possible for approximately 50% of affected children. We describe a patient with atypical diffuse CHI caused by mosaic ABCC8 mutation inheritance, unmasked by paternal uniparental disomy. Hypoglycaemia persisted despite two subtotal pancreatectomies and trials of diazoxide and nifedipine were unsuccessful. Octreotide resulted in anaphylaxis, precluding its use. Continuous subcutaneous glucagon infusion was successful in restoring normoglycaemia and attenuating weight gain, with concomitant improvement of developmental milestones. No adverse effects have been encountered after >12 months of therapy. Administration problems (e.g., line crystallisation) may complicate continuous glucagon therapy; hence a practical description of infusion constitution is included. We recommend consideration of continuous subcutaneous glucagon infusion as a therapeutic option for persistent refractory hypoglycaemia in CHI.

Keywords: glucagon; hyperinsulinism; hypoglycaemia; subcutaneous
Corresponding author: Dr. Orla M. Neylon, Murdoch Childrens Research Institute, Department of Endocrinology and Diabetes, The Royal Children’s Hospital, 50 Flemington Road, Parkville, Victoria 3052, Australia, Phone: +61 3 9345 5951, Fax: +61 3 9345 6240, E-mail:

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Received: 2013-3-21
Accepted: 2013-6-3
Published Online: 2013-06-29
Published in Print: 2013-11-01

©2013 by Walter de Gruyter Berlin Boston

Articles in the same Issue

  1. Masthead
  2. Masthead
  3. Review article
  4. Application of imaging modalities for evaluating neuroblastoma
  5. Images in pediatric endocrinology
  6. Lingual thyroid
  7. Original articles
  8. A levothyroxine dose recommendation for the treatment of children and adolescents with autoimmune thyroiditis induced hypothyroidism
  9. Fasting glucagon-like peptide-1 in patients with overt hyperthyroidism and euthyroid congenital hypothyroidism
  10. Perinatal complications and higher risks of offspring thyroid dysfunction in early childhood of Graves’ disease mothers with euthyroidism
  11. Clinical characteristics and management of cranial diabetes insipidus in infants
  12. Desmopressin administration in children with central diabetes insipidus: a retrospective review
  13. An electronic surveillance system for monitoring the hospital presentation of nutritional vitamin D deficiency in children in Scotland
  14. Frequency and severity of ketoacidosis at onset of autoimmune type 1 diabetes over the past decade in children referred to a tertiary paediatric care centre: potential impact of a national programme highlighted
  15. Vitamin D status is associated with early markers of cardiovascular disease in prepubertal children
  16. Evaluation of bone mineral density in children with type 1 diabetes mellitus
  17. Observational study of diabetes management in type 1 diabetic school-age children during holiday versus school days
  18. Relationship of body mass index and GAD65 antibody status on β-cell secretion at diabetes onset in African-American children
  19. Serum dipeptidyl peptidase 4 activity in children with type 1 diabetes mellitus
  20. Concomitant autoantibodies in newly diagnosed diabetic children with transient celiac serology or proven celiac disease
  21. Assessment of user-friendliness of the Norditropin FlexPro for pediatric patients treated with recombinant human growth hormone: results of an open-label user survey
  22. Cognitive impairment and gray/white matter volume abnormalities in pediatric patients with Turner syndrome presenting with various karyotypes
  23. Prevalence of metabolic syndrome among urban Indian adolescents and its relation with insulin resistance (HOMA-IR)
  24. Prevalence of metabolic syndrome in obese Chilean children and association with gene variants of the leptin-melanocortin system
  25. Evaluation of serum neopterin levels and its relationship with adipokines in pediatric obesity-related nonalcoholic fatty liver disease and healthy adolescents
  26. Could GSD type I expand the spectrum of disorders with elevated plasma chitotriosidase activity?
  27. Patient reports
  28. Treatment of hyperinsulinemic hypoglycemia because of diffuse nesidioblastosis with nifedipine after surgical therapies in a newborn
  29. Successful subcutaneous glucagon use for persistent hypoglycaemia in congenital hyperinsulinism
  30. A novel atypical presentation of insulin autoimmune syndrome (Hirata’s disease) in a child
  31. Diagnosis of septo-optic dysplasia in a neonate with hypernatremia, hypoglycemia, and persistent hypothermia
  32. Speech and language delay in two children: an unusual presentation of hyperthyroidism
  33. Lissencephaly presenting with congenital hypothyroidism
  34. PHEX gene mutation in a Chinese family with six cases of X-linked hypophosphatemic rickets
  35. Primary hyperparathyroidism as an extremely rare cause of secondary myelofibrosis in childhood
  36. A family with Camurati-Engelman disease: the role of the missense p.R218C mutation in TGFbeta1 in bones and endocrine glands
  37. A case of SCNN1A splicing mutation presenting as mild systemic pseudohypoaldosteronism type 1
  38. Central precocious puberty in a girl with Prader-Willi syndrome
  39. Type 1 diabetes mellitus in a patient with homozygous sickle cell anemia
  40. Short communication
  41. No impact of obesity susceptibility loci on weight regain after a lifestyle intervention in overweight children
  42. Letter to the Editor
  43. Reliability and validity of homeostasis model assessment for insulin resistance and β-cell dysfunction in critically ill children with hyperglycemia
https://doi.org/10.1515/jpem-2013-0115
Keywords for this article
glucagon; hyperinsulinism; hypoglycaemia; subcutaneous

Articles in the same Issue

  1. Masthead
  2. Masthead
  3. Review article
  4. Application of imaging modalities for evaluating neuroblastoma
  5. Images in pediatric endocrinology
  6. Lingual thyroid
  7. Original articles
  8. A levothyroxine dose recommendation for the treatment of children and adolescents with autoimmune thyroiditis induced hypothyroidism
  9. Fasting glucagon-like peptide-1 in patients with overt hyperthyroidism and euthyroid congenital hypothyroidism
  10. Perinatal complications and higher risks of offspring thyroid dysfunction in early childhood of Graves’ disease mothers with euthyroidism
  11. Clinical characteristics and management of cranial diabetes insipidus in infants
  12. Desmopressin administration in children with central diabetes insipidus: a retrospective review
  13. An electronic surveillance system for monitoring the hospital presentation of nutritional vitamin D deficiency in children in Scotland
  14. Frequency and severity of ketoacidosis at onset of autoimmune type 1 diabetes over the past decade in children referred to a tertiary paediatric care centre: potential impact of a national programme highlighted
  15. Vitamin D status is associated with early markers of cardiovascular disease in prepubertal children
  16. Evaluation of bone mineral density in children with type 1 diabetes mellitus
  17. Observational study of diabetes management in type 1 diabetic school-age children during holiday versus school days
  18. Relationship of body mass index and GAD65 antibody status on β-cell secretion at diabetes onset in African-American children
  19. Serum dipeptidyl peptidase 4 activity in children with type 1 diabetes mellitus
  20. Concomitant autoantibodies in newly diagnosed diabetic children with transient celiac serology or proven celiac disease
  21. Assessment of user-friendliness of the Norditropin FlexPro for pediatric patients treated with recombinant human growth hormone: results of an open-label user survey
  22. Cognitive impairment and gray/white matter volume abnormalities in pediatric patients with Turner syndrome presenting with various karyotypes
  23. Prevalence of metabolic syndrome among urban Indian adolescents and its relation with insulin resistance (HOMA-IR)
  24. Prevalence of metabolic syndrome in obese Chilean children and association with gene variants of the leptin-melanocortin system
  25. Evaluation of serum neopterin levels and its relationship with adipokines in pediatric obesity-related nonalcoholic fatty liver disease and healthy adolescents
  26. Could GSD type I expand the spectrum of disorders with elevated plasma chitotriosidase activity?
  27. Patient reports
  28. Treatment of hyperinsulinemic hypoglycemia because of diffuse nesidioblastosis with nifedipine after surgical therapies in a newborn
  29. Successful subcutaneous glucagon use for persistent hypoglycaemia in congenital hyperinsulinism
  30. A novel atypical presentation of insulin autoimmune syndrome (Hirata’s disease) in a child
  31. Diagnosis of septo-optic dysplasia in a neonate with hypernatremia, hypoglycemia, and persistent hypothermia
  32. Speech and language delay in two children: an unusual presentation of hyperthyroidism
  33. Lissencephaly presenting with congenital hypothyroidism
  34. PHEX gene mutation in a Chinese family with six cases of X-linked hypophosphatemic rickets
  35. Primary hyperparathyroidism as an extremely rare cause of secondary myelofibrosis in childhood
  36. A family with Camurati-Engelman disease: the role of the missense p.R218C mutation in TGFbeta1 in bones and endocrine glands
  37. A case of SCNN1A splicing mutation presenting as mild systemic pseudohypoaldosteronism type 1
  38. Central precocious puberty in a girl with Prader-Willi syndrome
  39. Type 1 diabetes mellitus in a patient with homozygous sickle cell anemia
  40. Short communication
  41. No impact of obesity susceptibility loci on weight regain after a lifestyle intervention in overweight children
  42. Letter to the Editor
  43. Reliability and validity of homeostasis model assessment for insulin resistance and β-cell dysfunction in critically ill children with hyperglycemia
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