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Congenital nephrogenic diabetes insipidus (NDI; also known as arginine vasopressin resistance) is a rare inherited disorder of water homeostasis, caused by insensitivity of the distal nephron to arginine vasopressin. Consequently, the kidney loses its ability to concentrate urine, which leads to polyuria, polydipsia, and the risk of hypertonic dehydration. The diagnosis and management of NDI are very challenging and require an integrated, multidisciplinary approach.
In this article, 36 recommendations for diagnosis, treatment, and follow-up in both children and adults are presented, as well as emergency management, genetic counselling, and family planning for patients with NDI. These recommendations are formulated and graded by an international group of experts in NDI from paediatric and adult nephrology, urology, and clinical genetics from the European Rare Kidney Disease Reference Network and the European Society of Paediatric Nephrology, as well as patient advocates, and are validated by a voting panel in a Delphi process.
The goal of these recommendations is to provide guidance to healthcare professionals who care for patients with NDI and to patients and their families. In addition, the need for further research on different aspects of this potentially life-threatening disorder to support the development of evidence-based guidelines in the future is emphasized. Read the full article here.