Nephrogenic diabetes insipidus (NDI) is a condition where the kidneys cannot concentrate urine, leading to excessive urination and thirst, despite normal or high levels of arginine vasopressin (AVP). This overview examines the clinical features, diagnosis, causes, and treatment options for NDI. The condition can be hereditary, often due to genetic mutations affecting the AVP signaling pathway, or acquired, usually linked to drug exposure (particularly lithium) or electrolyte imbalances. The management and symptoms of NDI vary depending on its cause, and the article also explores potential future therapeutic developments.
Nephrogenic diabetes insipidus (NDI) is a condition where the kidneys cannot concentrate urine, leading to excessive urination and thirst, despite normal or high levels of arginine vasopressin (AVP). This overview examines the clinical features, diagnosis, causes, and treatment options for NDI. The condition can be hereditary, often due to genetic mutations affecting the AVP signaling pathway, or acquired, usually linked to drug exposure (particularly lithium) or electrolyte imbalances. The management and symptoms of NDI vary depending on its cause, and the article also explores potential future therapeutic developments.
Clinical manifestations of the disease vary according to the degree of dehydration and hyperosmolality, being worse when renal water losses cannot be properly compensated by fluid intake. Regarding the diagnosis of NDI, it is important to consider the symptoms of the patient and the diagnostic tests, including the water deprivation test and the baseline plasma copeptin measurement, a stable surrogate biomarker of AVP release. Without proper treatment, patients may developcomplications leading to high morbidity and mortality, such as severe dehydration and hypernatremia. In that sense, the treatment of NDI consists in decreasing the urine output, while allowing appropriate fluid balance, normonatremia, and ensuring an acceptable quality of life. Therefore, therapeutic options include nonpharmacological interventions, including sufficient water intake and a low-sodium diet, and pharmacological treatment. The main medications used for NDI are thiazide diuretics, nonsteroidal anti-inflammatory drugs (NSAIDs), and amiloride, used isolated or in combination.
Clinical manifestations of the disease vary according to the degree of dehydration and hyperosmolality, being worse when renal water losses cannot be properly compensated by fluid intake. Regarding the diagnosis of NDI, it is important to consider the symptoms of the patient and the diagnostic tests, including the water deprivation test and the baseline plasma copeptin measurement, a stable surrogate biomarker of AVP release. Without proper treatment, patients may developcomplications leading to high morbidity and mortality, such as severe dehydration and hypernatremia. In that sense, the treatment of NDI consists in decreasing the urine output, while allowing appropriate fluid balance, normonatremia, and ensuring an acceptable quality of life. Therefore, therapeutic options include nonpharmacological interventions, including sufficient water intake and a low-sodium diet, and pharmacological treatment. The main medications used for NDI are thiazide diuretics, nonsteroidal anti-inflammatory drugs (NSAIDs), and amiloride, used isolated or in combination.