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Clinical characteristics and management of cranial diabetes insipidus in infants

  • Ambika Karthikeyan , Noina Abid , Prem C. B. Sundaram , Nicholas J. Shaw , Tim G. Barrett , Wolfgang Högler and Jeremy M. W. Kirk EMAIL logo
Published/Copyright: June 8, 2013
Journal of Pediatric Endocrinology and Metabolism
From the journal Journal of Pediatric Endocrinology and Metabolism Volume 26 Issue 11-12

Abstract

Aim: Cranial diabetes insipidus (CDI) is rare in infants with no guidelines on its management. We describe the first case series, characterizing the clinical features and treatment challenges.

Method: Retrospective case note review of infants diagnosed with CDI between April 1992 and February 2011.

Results: Nineteen infants (52% male) were identified. Eight were born preterm. Median (range) age at diagnosis was 24 days (5–300); preterm babies were younger at diagnosis (21 vs. 46 days). In 58% (11/19) of infants, hypernatraemia was discovered incidentally. In 37% of cases there was associated midline anomalies, however, only four patients (21%) had absent posterior pituitary signal on a magnetic resonance imaging brain scan. The most frequent (5/19) underlying diagnosis was septo-optic dysplasia. Eight patients had isolated CDI and 11 had multiple pituitary hormone deficiencies. Isolated CDI tended to be more common in preterm, compared to term babies (p=0.11). Des-amino arginine vasopressin (DDAVP) was administered intranasally in eight and orally in 11 infants. Plasma sodium nadir following DDAVP administration was lower following intranasal compared to an oral route of administration (median: 128 vs. 133 mmol/L, p=0.022). No cases resolved on follow-up.

Conclusions: CDI in infants is often diagnosed incidentally. Aetiology, clinical, and imaging features are very variable, with some differences between preterm and term infants. Oral DDAVP appears to be superior to intranasal with less pronounced serum sodium fluctuations.

Keywords: diabetes insipidus; hyponatraemia; hypopituitarism; infancy; neonates; vasopressin
Corresponding author: Jeremy M. W. Kirk, Department of Endocrinology and Diabetes, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK, Phone: +44 121 333 8188, Fax: +44 121 333 8191, E-mail:

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Received: 2013-1-19
Accepted: 2013-5-14
Published Online: 2013-06-08
Published in Print: 2013-11-01

©2013 by Walter de Gruyter Berlin Boston

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https://doi.org/10.1515/jpem-2013-0026
Keywords for this article
diabetes insipidus; hyponatraemia; hypopituitarism; infancy; neonates; vasopressin

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
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  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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