Pediatric Diabetes Insipidus Treatment & Management

  • Author: James CM Chan, MD; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Jul 25, 2011
 

Approach Considerations

Treat patients with diabetes insipidus (DI) in an inpatient setting because of the risk of severe dehydration. Destructive or compressive intracranial lesions mandate inpatient stay. Demonstration of an intracranial mass necessitates surgical care.

Distinguishing between central and nephrogenic etiologies is essential to treatment.[9] Transfer to an academic center is highly advised for initial diagnosis and treatment, especially because central DI may require involved diagnostic studies and neurosurgical or oncologic treatment. Surgical procedures of any kind require replacement of fluids at a much higher rate than normal maintenance; inattention to this may result in serious consequences.

Subsequent admissions are determined by the need for intravenous (IV) rehydration, especially during intercurrent gastrointestinal (GI) illnesses.

Next

Pharmacologic Therapy

For central DI, the treatment of choice is desmopressin (a synthetic vasopressin analogue). It is available in parenteral, intranasal, and oral dosage forms. The doses widely vary depending on the preparation used, so take care to correctly calculate the dose. Other useful medications include chlorpropamide and thiazide diuretics. The latter 2 can result in a 25-75% reduction in urine volume and can be used in combination with each other.

Nephrogenic DI cannot be effectively treated with desmopressin, because the receptor sites are defective and the kidney is prevented from responding. Thiazide diuretics, amiloride,[10, 11] and indomethacin or aspirin are useful when coupled with a low-solute diet.

Aqueous vasopressin (Pitressin) and desmopressin (DDAVP) preparations are available for intravenous (IV) use in emergency circumstances. Overtreatment with desmopressin can result in hyponatremia and seizures.

Previous
Next

Diet and Activity

Provide affected infants with a breast milk diet to decrease solute load. Protein should account for 6% of caloric intake, and sodium should be reduced to 0.7 mEq/kg/day.

Provide young children with 8% of their caloric intake in the form of protein to enable normal growth. Sodium intake must be maintained at 0.7 mEq/kg/day. (See the video below.)

Carbs for Kids-Count Them In: The Constant Carbohydrates Diet.

Activities resulting in increased insensible water loss should be moderated in the presence of massive urinary water loss to prevent dehydration. Heat exposure should be minimized, especially when participating in sports. Avoid creating barriers to drinking water.

Previous
Next

Consultations and Long-Term Monitoring

Consultation with the following specialists may be appropriate:

  • Nephrologist
  • Endocrinologist - The presence of central DI should prompt an evaluation of anterior pituitary function.
  • Diagnostic radiologist

Regular follow-up visits with an endocrinologist (for central DI) or a nephrologist (for nephrogenic DI) are necessary for dosage adjustment. When indomethacin is used in long-term therapy, carefully observe renal function for any signs of toxicity.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

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: Alpha Omega Alpha, American Academy of Pediatrics, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, and International Society of Nephrology

Disclosure: Nothing to disclose.

Coauthor(s)

Karl S Roth, MD  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 Clinical 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, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and Phi Beta Kappa

Disclosure: Nothing to disclose.

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 Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  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, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Satoh M, Ogikubo S, Yoshizawa-Ogasawara A. Correlation between clinical phenotypes and X-inactivation patterns in six female carriers with heterozygote vasopressin type 2 receptor gene mutations. Endocr J. 2008;55:277-284. [Full Text].

  2. Friedman E, Bale AE, Carson E, et al. Nephrogenic diabetes insipidus: an X chromosome-linked dominant inheritance pattern with a vasopressin type 2 receptor gene that is structurally normal. Proc Natl Acad Sci U S A. Aug 30 1994;91(18):8457-61. [Medline]. [Full Text].

  3. 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. Jul 1 1998;102(1):57-66. [Medline]. [Full Text].

  4. 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].

  5. Garofeanu CG, Weir M, Rosas-Arellano MP, et al. Causes of reversible nephrogenic diabetes insipidus: a systematic review. Am J Kidney Dis. Apr 2005;45(4):626-37.

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

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

  8. [Guideline] Seidenwurm DJ, Wippold FJ II, Cornelius RS, et al. Expert Panel on Neurologic Imaging. ACR Appropriateness Criteria neuroendocrine imaging. [online publication]. Reston (VA): American College of Radiology (ACR); 2008. 11 p.

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

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

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

 
Previous
Next
 
Carbs for Kids-Count Them In: The Constant Carbohydrates Diet.
Diabetes Sick Day Rules.
Taking Diabetes Back to School.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.