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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

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.


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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