Current treatment of hereditary nephrogenic diabetes insipidus in children

Nephrogenic diabetes insipidus (NDI) is a rare disorder characterized by the inability of the kidneys to respond to arginine vasopressin, leading to polyuria, polydipsia, and chronic dehydration. This condition often results in severe complications, including growth failure, behavioral problems, and irreversible brain damage, particularly in young children with hereditary forms. Early diagnosis and effective management are critical for minimizing these complications and improving patients' quality of life. Current treatments focus primarily on symptom management through dietary interventions, fluid therapy, and pharmacological strategies. Medications, such as thiazide diuretics, potassium-sparing diuretics, and nonsteroidal anti-inflammatory drugs, play pivotal roles in reducing urine output and improving fluid balance. However, their use requires careful monitoring to mitigate the risks of side effects, including electrolyte imbalances and renal dysfunction. This review provides an overview of the pharmacological and non-pharmacological management of hereditary NDI, emphasizing the mechanisms of action, efficacy, and safety profiles of commonly used therapies. It also highlights the importance of a multidisciplinary approach to optimize care, particularly for pediatric patients who require tailored interventions for growth and development. Further research is required to explore novel therapeutic avenues that target the underlying molecular pathways of NDI.