ReviewNature reviews. Nephrology2025
International expert consensus statement on the diagnosis and management of congenital nephrogenic diabetes insipidus (arginine vasopressin resistance).
Review in Nature reviews. Nephrology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed.
- Novel Roles of GPCRs in the Renal Collecting Duct.Physiology (Bethesda, Md.) · 2026Review
- From mechanisms to clinical practice: a review of diuretic therapy in pediatric nephrology.Pediatric nephrology (Berlin, Germany) · 2026Review
- Failure to thrive in children with tubulopathies.Pediatric nephrology (Berlin, Germany) · 2026Review
- Genetic and biological mechanisms of pediatric enuresis: integrating pathophysiology, GWAS associations, and multi-omics research.European journal of pediatrics · 2026Review
- X-Linked Nephrogenic Diabetes Insipidus Associated with the AVPR2 c.964C>T (p.Pro322Ser) Variant: A Family Case Series.Journal of clinical medicine · 2026Article
- Clean intermittent catheterization reverses hydronephrosis in a child with congenital nephrogenic diabetes insipidus: a case report.The Canadian journal of urology · 2026Article
- Use, efficacy, and safety of desmopressin for congenital nephrogenic diabetes insipidus in children: a nationwide survey.Endocrine journal · 2026Observational
- Copeptin in the diagnosis and management of renal tubular disorders.Pediatric nephrology (Berlin, Germany) · 2026Review
- European Reference Networks - a flagship activity of the EU in the field of rare and complex diseases: from 2017 to 2025.Orphanet journal of rare diseases · 2026Review
- Beyond expensive innovations: affordable and effective strategies for managing tubulopathies in adults.Clinical kidney journal · 2026Review
- Congenital AVP-Resistance: CANDID Insights for Earlier, Safer, Gene-Anchored Care.Indian journal of pediatrics · 2026Article
- Medication-Induced Severe Hyponatremia After Elective Surgery in a Patient With Known Arginine Vasopressin Deficiency.JCEM case reports · 2026Article
- Acute Kidney Injury in Children with Polyuria: A Systematic Review.Journal of clinical medicine · 2026Review
- Drug spectrum and disproportionality signals for diabetes insipidus in FAERS: multi-method analysis, JADER cross-database assessment, and PubMed-based case-level evidence.Frontiers in pharmacology · 2026Article
- Infant Nephrogenic Diabetes Insipidus: Challenges Leading to Delayed Management.Case reports in medicine · 2026Article
- HomozygousRenal failure · 2025Article
- Late Diagnosis of X-Linked Arginine Vasopressin Resistance Presenting With Flow Uropathy in Adulthood.JCEM case reports · 2025Article
- Clinical outcomes among children with primary nephrogenic diabetes insipidus.Clinical kidney journal · 2025Article
- Beyond Biomarkers: Blending Copeptin and Clinical Cues to Distinguish Central Diabetes Insipidus from Primary Polydipsia in Children.Biomedicines · 2025Article
- A Novel Pathogenic Variant of theGenes · 2025Article
Corrections and comments
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Authors and funding
13 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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. Here, we present 36 recommendations for diagnosis, treatment and follow-up in both children and adults, as well as emergency management, genetic counselling and family planning, for patients with NDI. These recommendations were 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 were validated by a voting panel in a Delphi process. The goal of these recommendations is to provide guidance to health care professionals who care for patients with NDI and to patients and their families. In addition, we emphasize 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.
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