Etiologies of central diabetes insipidus in children

doi:10.1016/0887-8994(94)90001-9

Pediatric Neurology

Volume 11, Issue 4, November 1994, Pages 273-277

https://doi.org/10.1016/0887-8994(94)90001-9 Get rights and content

Abstract

The last major review of the etiologies of central diabetes insipidus in children was performed a quarter century ago, prior to the development of modern neuroimaging techniques. We retrospectively reviewed the records of children with central diabetes insipidus identified at Children's Hospital of Buffalo from 1979 to 1992. Of the 35 patients identified, 27 were males and 8 were females. Their ages ranged from 3 weeks to 20 years. Nineteen children had brain tumors, 7 had cerebral malformations, 3 had central nervous system infections, 1 had traumatic brain injury, and 5 were considered idiopathic. Patients with brain death were excluded from the review. Thirty-one of 35 patients developed diabetes insipidus in conjunction with other endocrinopathies. Brain tumor and its treatment account for the most common cause. Cranial magnetic resonance imaging has improved the identification of structural lesions and the understanding of the pathophysiology of central diabetes insipidus.

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Cited by (71)

Central diabetes insipidus from a patient's perspective: management, psychological co-morbidities, and renaming of the condition: results from an international web-based survey
2022, The Lancet Diabetes and Endocrinology
Citation Excerpt :
Most commonly, central diabetes insipidus results from acquired disruptions of the hypothalamic–pituitary axis, and less than 10% of the cases are hereditary.7,8 The spectrum of causes of central diabetes insipidus indicated by our data are consistent with available literature, with hypothalamo–pituitary tumours or cysts the most common cause of central diabetes insipidus (46%); however, there was a large proportion of idiopathic cases (30–50%), especially in isolated central diabetes insipidus.7–9 In anterior pituitary dysfunction, gonadotropins and growth hormone are usually more likely to be affected than adrenocorticotropic hormone and thyroid-stimulating hormone.
Central diabetes insipidus is a rare neuroendocrine condition. Data on treatment-associated side-effects, psychological comorbidities, and incorrect management are scarce. The aim of this study was to investigate patients’ perspectives on their disease.
This study used a cross-sectional, web-based, anonymous survey, developed by endocrinologists and patient representatives, to collect the opinions of patients with central diabetes insipidus on management and complications of their disease, psychological comorbidities, degree of knowledge and awareness of the condition among health-care professionals, and renaming the disease to avoid confusion with diabetes mellitus (diabetes).
Between Aug 23, 2021, and Feb 7, 2022, 1034 patients with central diabetes insipidus participated in the survey. 91 (9%) participants were children and adolescents (37 [41%] girls and 54 [59%] boys; median age 10 years [IQR 6–15]) and 943 (91%) were adults (757 [80%] women and 186 [20%] men]; median age 44 years [34–54]). 488 (47%) participants had isolated posterior pituitary dysfunction and 546 (53%) had combined anterior and posterior pituitary dysfunction. Main aetiologies were idiopathic (315 [30%] of 1034 participants) and tumours and cysts (pre-surgical 217 [21%]; post-surgical 254 [25%]). 260 (26%; 95% CI [0·23–0·29]) of 994 patients on desmopressin therapy had hyponatraemia leading to hospitalisation. Patients who routinely omitted or delayed desmopressin to allow intermittent aquaresis had a significantly lower prevalence of hyponatraemia compared with those not aware of this approach (odds ratio 0·55 [95% CI 0·39–0·77]; p=0·0006). Of patients who had to be hospitalised for any medical reason, 71 (13%; 95% CI 0·10–0·16) of 535 patients did not receive desmopressin while in a fasting state (nil by mouth) without intravenous fluid replacement and reported symptoms of dehydration. 660 (64%; 0·61–0·67) participants reported lower quality of life, and 369 (36%; 0·33–0·39) had psychological changes subjectively associated with their central diabetes insipidus. 823 (80%; 0·77–0·82) participants encountered a situation where central diabetes insipidus was confused with diabetes mellitus (diabetes) by health-care professionals. 884 (85%; 0·83–0·88) participants supported renaming the disease; the most favoured alternative names were vasopressin deficiency and arginine vasopressin deficiency.
This is the largest survey of patients with central diabetes insipidus, reporting a high prevalence of treatment-associated side-effects, mismanagement during hospitalisation, psychological comorbidities, and a clear support for renaming the disease. Our data are the first to indicate the value of routinely omitting or delaying desmopressin.
Swiss National Science Foundation, Swiss Academy of Medical Sciences, and G&J Bangerter-Rhyner-Foundation.
Disorders of the Posterior Pituitary
2020, Sperling Pediatric Endocrinology: Expert Consult - Online and Print
Maintenance of the tonicity of extracellular fluids within a very narrow range is crucial for proper cell function. Extracellular osmolality regulates cell shape, as well as intracellular concentrations of ions and other osmolytes. Furthermore, proper extracellular ionic concentrations are necessary for the correct function of ion channels, action potentials, and other modes of intercellular communication. Extracellular fluid tonicity is regulated almost exclusively by the amount of water intake and excretion, whereas extracellular volume is regulated by the level of sodium chloride intake and excretion. In children and adults, normal blood tonicity is maintained over a 10-fold variation in water intake by a coordinated interaction among thirst, vasopressin, and renal systems. Dysfunction in any of these systems can result in abnormal regulation of blood osmolality, which if not properly recognized and treated may cause life-threatening dysfunction in neuronal and other cellular activities.
Disorders of the posterior pituitary
2014, Pediatric Endocrinology: Fourth Edition
Clinical characteristics of central diabetes insipidus in taiwanese children
2013, Journal of the Formosan Medical Association
Citation Excerpt :
With increasing sensitivity of diagnostic imaging techniques, a specific etiology can now be established in some patients with CDI. In the present study, approximately 8% of patients had idiopathic CDI, which is similar to previous reports.5,21,22 Two patients were initially diagnosed as having idiopathic CDI, but later proven to have treatable intracranial lesions nine months (one patient) and two years (one patient) later.
Data on the clinical features of children with central diabetes insipidus (CDI) are lacking in Taiwan. This study investigated the clinical manifestations and etiology of CDI in Taiwanese children.
From 1983 to 2012, 62 children with permanent diabetes insipidus were enrolled in the study. They were diagnosed at the Department of Pediatrics of National Taiwan University Hospital. Their medical records were thoroughly reviewed and their clinical symptoms and signs, laboratory data, and etiologies were analyzed.
The patients’ median age at diagnosis was 10 years and the median interval between initial manifestations and diagnosis was 0.5 years. The most common symptoms and signs were polyuria, polydipsia, nocturia, and growth retardation. Most patients had low urine osmolality and elevated plasma osmolality on diagnosis. Absence of a posterior pituitary hyperintense signal and thickening of the pituitary stalk were common findings on magnetic resonance imaging. Approximately 80% of the patients had anterior pituitary hormone deficiency and all patients had growth hormone deficiency. Approximately 60% of patients had intracranial lesions, the most common causes of which were germ cell tumor and Langerhans cell histiocytosis. Two patients were initially believed to have idiopathic CDI but intracranial lesions were detected during the follow-up period.
Because a delayed diagnosis of CDI is common in Taiwanese children, a high index of suspicion is important. The underlying etiology of CDI in children may not initially be obvious. Long-term surveillance is therefore necessary, especially for the early detection of evolving treatable intracranial lesions.
Natural history of idiopathic diabetes insipidus
2011, Journal of Pediatrics
To determine what percentage of diabetes insipidus (DI) in childhood is idiopathic and to assess the natural history of idiopathic DI.
We conducted a retrospective chart review of 105 patients with DI who were born or had DI diagnosed between 1980-1989 at 3 medical centers. A second cohort of 30 patients from 6 medical centers in whom idiopathic DI was diagnosed after 1990 was evaluated retrospectively for subsequent etiologic diagnoses and additional hypothalamic/pituitary deficiencies and prospectively for quality of life.
In the first cohort, 11% of patients had idiopathic DI. In the second cohort, additional hypothalamic/pituitary hormone deficiencies developed in 33%, and 37% received an etiologic diagnosis for DI. Health-related quality of life for all the patients with idiopathic DI was comparable with the healthy reference population.
Only a small percentage of patients with DI will remain idiopathic after first examination. Other hormone deficiencies will develop later in one-third of those patients, and slightly more than one-third of those patients will have an etiology for the DI diagnosed. Long-term surveillance is important because tumors have been diagnosed as long as 21 years after the onset of DI. Quality of life for these patients is as good as the reference population.
Disorders of the Posterior Pituitary
2008, Pediatric Endocrinology

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Review articleEtiologies of central diabetes insipidus in children

Abstract

J Pediatr

Lancet

J Pediatr

Disorders of posterior pituitary in children

Simplifying the diagnosis of diabetes insipidus in children

Am J Dis Child

Hypodipsic hypernatremia with normal osmoregulation of vasopressin

N Engl J Med

Endocrine function, morbidity, and mortality after surgery for craniopharyngioma

Arch Dis Child

Surgical management of craniopharyngiomas

J Neurosurg

Treatment of the young child with postoperative central diabetes insipidus

Am J Dis Child

Review article
Etiologies of central diabetes insipidus in children