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Pediatric Diabetes Insipidus Clinical Presentation

  • Author: Karl S Roth, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
Updated: Nov 03, 2015


Diagnosis of diabetes insipidus (DI) may be difficult in infants and children because of nonspecific presenting features (eg, poor feeding, failure to thrive, irritability). Accordingly, a high index of suspicion is necessary.

The earliest signs of DI include a vigorous suck with vomiting, fever without apparent cause, constipation, and excessively wet diapers from urination. In older infants and young children, irritability is generally due to a borderline state of dehydration coupled with hypernatremia and, sometimes, fever. Nocturia is common and expected because of increased urine production. Central DI tends to develop suddenly.


Physical Examination

The typical examination reveals an irritable infant with a dripping wet diaper, along with detectable signs of dehydration (eg, dry mucous membranes, diminished skin turgor, decreased tearing, tachycardia). Often, skin turgor is not diminished in individuals with hypernatremic dehydration despite significant dehydration. In severely dehydrated patients, the pulse may be thready and rapid. Hypotension may be present because of hypovolemic shock. Mobile fecaliths may be palpable in the abdomen.



Complications include the following:

  • Growth failure
  • Nocturia and enuresis
  • Hypernatremic dehydration
  • Seizures
  • Mental retardation

Dehydration results from an inability to reabsorb free water at a site distal to electrolyte reabsorption. Any patient unable to continuously replace water loss is vulnerable to dehydration, especially in warm weather when insensible water loss through perspiration and respiration substantially increases risk.

Electrolyte abnormalities are caused by the loss of urinary free water, which produces hyperosmolar dehydration, leading to hypernatremia, hyperchloremia, and prerenal azotemia. Diminished blood volume increases blood viscosity and the risk of sludging and thrombosis.

Failure to thrive occurs because of the patient’s constant thirst conferring a sense of fullness that offsets the sense of hunger. The affected individual eats less than necessary for normal growth.

Seizures are a consequence of the electrolyte abnormalities introduced in the central nervous system (CNS) by severe hypernatremia and hyperosmolar dehydration. Mental retardation results from the damage to the CNS caused by severe hyperosmolarity, seizures, and potential hypoxia, all of which are thought to account for the frequent occurrence of mental retardation. Death can occur from a hypovolemic shock or a hypernatremic seizure.

Contributor Information and Disclosures

Karl S Roth, MD Retired Professor and Chair, Department of Pediatrics, Creighton University School of Medicine

Karl S Roth, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Nutrition, American Pediatric Society, American Society for Nutrition, American Society of Nephrology, Association of American Medical Colleges, Medical Society of Virginia, New York Academy of Sciences, Sigma Xi, Society for Pediatric Research, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.


James CM Chan, MD Professor of Pediatrics, Tufts University School of Medicine; Director of Research, The Barbara Bush Children's Hospital, Maine Medical Center

James CM Chan, MD is a member of the following medical societies: American Pediatric Society, Alpha Omega Alpha, American Academy of Pediatrics, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, American College of Endocrinology

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

  1. Knepper MK, Kwon T, Nielsen S. Molecular physiology of water balance. New Engl J Med. 2015 April 2. 372;14:1349-58. [Medline].

  2. Mulders SM, Bichet DG, Rijss JP, et al. An aquaporin-2 water channel mutant which causes autosomal dominant nephrogenic diabetes insipidus is retained in the Golgi complex. J Clin Invest. 1998 Jul 1. 102(1):57-66. [Medline]. [Full Text].

  3. Chandrasekaran K, Karolina DS, Sepramaniam S, Armugam A, Wintour EM, Bertram JF, et al. Role of microRNAs in kidney homeostasis and disease. Kidney Int. 2012 Apr. 81(7):617-27. [Medline].

  4. Agre P, King LS, Yasui M, et al. Aquaporin water channels - from atomic structure to clinical medicine. J Physiol. 2002. 542:3-16. [Medline].

  5. Danziger J, Zeidel ML. Osmotic homeostasis. Clin J Am Soc Nephrol. 2015. 10:852-862. [Medline].

  6. Davies JH, Penney M, Abbes AP, et al. Clinical features, diagnosis and molecular studies of familial central diabetes insipidus. Horm Res. 2005. 64(5):231-7. [Medline].

  7. Garofeanu CG, Weir M, Rosas-Arellano MP, Henson G, Garg AX, Clark WF. Causes of reversible nephrogenic diabetes insipidus: a systematic review. Am J Kidney Dis. 2005 Apr. 45 (4):626-37. [Medline].

  8. Wildin RS, Antush MJ, Bennett RL. Heterogeneous AVPR2 gene mutations in congenital nephrogenic diabetes insipidus. Am J Hum Genet. 1994 Aug. 55(2):266-77. [Medline].

  9. Faerch M, Christensen JH, Corydon TJ, et al. Partial nephrogenic diabetes insipidus caused by a novel mutation in the AVPR2 gene. Clin Endocrinol (Oxf). 2008 Mar. 68(3):395-403. [Medline].

  10. [Guideline] Seidenwurm DJ, Wippold FJ II, Cornelius RS, et al. Expert Panel on Neurologic Imaging. ACR Appropriateness Criteria neuroendocrine imaging. American College of Radiology (ACR). 2008.

  11. Boussemart T, Nsota J, Martin-Coignard D, Champion G. Nephrogenic diabetes insipidus: treat with caution. Pediatr Nephrol. 2009 Sep. 24(9):1761-3. [Medline].

  12. Alon U, Chan JC. Hydrochlorothiazide-amiloride in the treatment of congenital nephrogenic diabetes insipidus. Am J Nephrol. 1985. 5(1):9-13. [Medline].

  13. Saborio P, Tipton GA, Chan JC. Diabetes insipidus. Pediatr Rev. 2000 Apr. 21(4):122-9; quiz 129. [Medline].

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