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Juvenile gout

An autosomal dominant disorder characterized by hyperuricemia due to a low fractional excretion of URIC ACID, defective urinary concentrating ability, interstitial nephropathy, and progression to CHRONIC KIDNEY FAILURE. Both HNFJ1 and medullary cystic kidney disease (MCKD2) (OMIM: 603860) are caused by mutations in the UMOD gene. OMIM: 162000
Also Known As:
Familial Gout-Kidney Disease; Familial Gouty Nephropathy; Familial Juvenile Hyperuricemic Nephropathy; Gouty nephropathy, familial juvenile; HNFJ; HNFJ1; Hyperuricemic Nephropathy, Familial Juvenile; Hyperuricemic Nephropathy, Familial Juvenile 1; Mckd2; Medullary Cystic Kidney Disease Type 2; Nephropathy, familial, with gout; Umod-Related Kidney Disease; Uromodulin Storage Disease; Uromodulin-Associated Kidney Disease
Networked: 82 relevant articles (0 outcomes, 3 trials/studies)

Disease Context: Research Results

Related Diseases

1. Juvenile gout
2. Hyperuricemia
3. Gout
4. Renal Insufficiency (Renal Failure)
5. Chronic Kidney Failure (Chronic Renal Failure)

Experts

1. Lhotta, Karl: 6 articles (11/2020 - 01/2003)
2. Thakker, Rajesh V: 5 articles (11/2020 - 01/2003)
3. Aigner, Bernhard: 5 articles (04/2014 - 11/2009)
4. Kemter, Elisabeth: 5 articles (04/2014 - 11/2009)
5. Rathkolb, Birgit: 5 articles (04/2014 - 11/2009)
6. Wolf, Eckhard: 5 articles (04/2014 - 11/2009)
7. Hosoyamada, Makoto: 4 articles (08/2015 - 03/2008)
8. Rampoldi, Luca: 4 articles (01/2015 - 12/2004)
9. Fuchs, Helmut: 4 articles (01/2014 - 11/2009)
10. Gailus-Durner, Valérie: 4 articles (01/2014 - 11/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Juvenile gout:
1. UromodulinIBA
2. Hypoxanthine Phosphoribosyltransferase (Hypoxanthine Guanine Phosphoribosyltransferase)IBA
3. 4-phenylbutyric acid (Buphenyl)FDA Link
4. Uric Acid (Urate)IBA
5. Proteins (Proteins, Gene)FDA Link
6. HypoxanthineIBA
7. TransferasesIBA
8. SaltsIBA
9. GuanineIBA
10. Hepatocyte Nuclear Factor 1-betaIBA

Therapies and Procedures

1. Caloric Restriction
2. Renal Replacement Therapy (Therapies, Renal Replacement)
3. Kidney Transplantation
4. Renal Dialysis (Hemodialysis)
5. Drug Therapy (Chemotherapy)