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Nephrogenic diabetes insipidus (NDI) is a rare kidney disorder in which the kidneys fail to respond to the antidiuretic hormone (ADH), also known as vasopressin, leading to excessive excretion of dilute urine and constant thirst. Unlike diabetes mellitus, which involves high blood sugar levels, NDI is caused by the kidneys' inability to retain water, resulting in dehydration and electrolyte imbalance. The condition may be hereditary, due to genetic mutations in the AVPR2 gene (X-linked) or AQP2 gene (autosomal), or acquired, resulting from kidney disease, chronic lithium use, hypercalcemia, or low potassium levels.

Symptoms commonly include polyuria (frequent urination), polydipsia (excessive thirst), dehydration, and fatigue. In infants, poor weight gain, irritability, and fever may be observed, while adults may experience nocturia and weakness. Diagnosis involves urine osmolality tests, water deprivation tests, and genetic analysis to differentiate it from central diabetes insipidus. Treatment focuses on controlling symptoms and preventing dehydration through adequate water intake, low-salt diet, and drugs such as thiazide diuretics or NSAIDs that help reduce urine output. With proper management, patients can maintain a stable quality of life.