Adipsic Diabetes Insipidus in Children: A Case Report and Practical Guide

Area of Science:

• Pediatric Endocrinology
• Nephrology
• Neurology

Background:

• Diabetes insipidus (DI) is a condition of polyuria and polydipsia due to antidiuretic hormone (ADH) issues.
• Central DI involves ADH deficiency, while nephrogenic DI is characterized by resistance to ADH.
• Adipsic DI, especially with cognitive impairment or limited water access, can lead to severe hypernatremia, morbidity, and mortality.

Observation:

• A 2-year-old boy with global developmental delay and post-surgical hypothalamic dysfunction developed central DI and thirst center dysfunction.
• The patient presented with symptoms indicative of adipsic DI, necessitating a novel management strategy.

Findings:

• A practical management approach was implemented, featuring fixed desmopressin dosing, timed water replacement based on diaper weight, and twice-daily bodyweight-based fluid correction.
• Nutritional and fluid requirements were met at fixed intervals, mimicking a healthy child's intake.
• This regimen effectively managed plasma sodium levels and fluid balance without impeding the child's growth.

Implications:

• This management plan offers a practical and effective home-based treatment for adipsic DI in children.
• Successful implementation relies heavily on consistent family adherence to the prescribed regimen.
• The approach demonstrates the feasibility of maintaining stable physiological parameters in children with complex DI and thirst dysfunction.