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Congenital Nephrogenic Diabetes Insipidus Presenting after Acute Pyelonephritis
並列摘要
Diabetes insipidus (DI) is characterized by the inability to concentrate urine. While central DI is caused by failure to release enough functional vasopressin, nephrogenic DI (NDI) is due to the insensitivity of the distal nephron to the effect of antidiuretic hormone (ADH). Case Report: A s-day-old newborn male was admitted for isolated fever and a questionable early right upper lobe infiltrate. He gradually developed hypernatremia and increased osmolality. As part of his work up for fever, he had a urine culture of 30K colonies of Enterococcus faecalis. His vasopressin test was negative. Conclusion: The polyuria and polydipsia associated with genetic NDI usually presents within the first several weeks of life but may only become apparent after weaning or with longer periods of nighttime fasting. The acute pyelonephritis of this newborn may have been the initial trigger for the congenital NDI. Accurate diagnosis of this patient helped to also diagnose his maternal uncle and provide clues to the current condition of his maternal grandmother. Early diagnosis and management can prevent the development of neurological and developmental complications associated with NDI.