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Hereditary nephrogenic diabetes insipidus

MedGen UID:
195603
•Concept ID:
C0677501
•
Disease or Syndrome
Synonym: Congenital Nephrogenic Diabetes Insipidus
SNOMED CT: Hereditary nephrogenic diabetes insipidus (61165007); Familial nephrogenic diabetes insipidus (61165007); Vasopressin-unresponsiveness hyposthenuria (61165007); Vasopressin-resistant diabetes insipidus (61165007); ADH-resistant diabetes insipidus (61165007); Adiuretin-resistant diabetes insipidus (61165007); Renal diabetes insipidus (61165007); Vasopressin-resistant hyposthenuria (61165007); Congenital nephrogenic diabetes insipidus (61165007); Primary nephrogenic diabetes insipidus (61165007)

Professional guidelines

PubMed

Hereditary Nephrogenic Diabetes Insipidus: Pathophysiology and Possible Treatment. An Update.
Milano S, Carmosino M, Gerbino A, Svelto M, Procino G
Int J Mol Sci 2017 Nov 10;18(11) doi: 10.3390/ijms18112385. PMID: 29125546Free PMC Article
Hereditary polyuric disorders: new concepts and differential diagnosis.
Bichet DG
Semin Nephrol 2006 May;26(3):224-33. doi: 10.1016/j.semnephrol.2006.02.004. PMID: 16713495

Recent clinical studies

Etiology

Hereditary Nephrogenic Diabetes Insipidus: Pathophysiology and Possible Treatment. An Update.
Milano S, Carmosino M, Gerbino A, Svelto M, Procino G
Int J Mol Sci 2017 Nov 10;18(11) doi: 10.3390/ijms18112385. PMID: 29125546Free PMC Article
Hereditary nephrogenic diabetes insipidus in Japanese patients: analysis of 78 families and report of 22 new mutations in AVPR2 and AQP2.
Sasaki S, Chiga M, Kikuchi E, Rai T, Uchida S
Clin Exp Nephrol 2013 Jun;17(3):338-44. Epub 2012 Nov 14 doi: 10.1007/s10157-012-0726-z. PMID: 23150186
Urinary content of aquaporin 1 and 2 in nephrogenic diabetes insipidus.
Deen PM, van Aubel RA, van Lieburg AF, van Os CH
J Am Soc Nephrol 1996 Jun;7(6):836-41. doi: 10.1681/ASN.V76836. PMID: 8793791

Diagnosis

Hereditary Nephrogenic Diabetes Insipidus: Pathophysiology and Possible Treatment. An Update.
Milano S, Carmosino M, Gerbino A, Svelto M, Procino G
Int J Mol Sci 2017 Nov 10;18(11) doi: 10.3390/ijms18112385. PMID: 29125546Free PMC Article
Differential diagnosis of hereditary nephrogenic diabetes insipidus with desmopressin infusion test.
Bircan Z, Mutlu H, Cheong HI
Indian J Pediatr 2010 Nov;77(11):1329-31. Epub 2010 Sep 3 doi: 10.1007/s12098-010-0181-3. PMID: 20814834
Hereditary polyuric disorders: new concepts and differential diagnosis.
Bichet DG
Semin Nephrol 2006 May;26(3):224-33. doi: 10.1016/j.semnephrol.2006.02.004. PMID: 16713495
Cyclic-3',5'-nucleotide phosphodiesterase isozymes in cell biology and pathophysiology of the kidney.
Dousa TP
Kidney Int 1999 Jan;55(1):29-62. doi: 10.1046/j.1523-1755.1999.00233.x. PMID: 9893113
Hereditary nephrogenic diabetes insipidus and bilateral nonobstructive hydronephrosis.
Uribarri J, Kaskas M
Nephron 1993;65(3):346-9. doi: 10.1159/000187510. PMID: 8289981

Therapy

Hereditary polyuric disorders: new concepts and differential diagnosis.
Bichet DG
Semin Nephrol 2006 May;26(3):224-33. doi: 10.1016/j.semnephrol.2006.02.004. PMID: 16713495
Treatment of nephrogenic diabetes insipidus with prostaglandin synthesis inhibitors.
Libber S, Harrison H, Spector D
J Pediatr 1986 Feb;108(2):305-11. doi: 10.1016/s0022-3476(86)81010-1. PMID: 3080575

Clinical prediction guides

Radioimmunoassay of arginine-vasopressin in human urine and its use in physiological and pathological states.
Khokhar AM, Ramage CM, Slater JD
J Endocrinol 1978 Dec;79(3):375-89. doi: 10.1677/joe.0.0790375. PMID: 744931

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