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About 77 results

ALLMedicine™ Argininosuccinic Aciduria Center

Research & Reviews  37 results

Resting energy expenditure in argininosuccinic aciduria and in other urea cycle disorders.
https://doi.org/10.1002/jimd.12108
Journal of Inherited Metabolic Disease REFERENCES; Brambilla A, Bianchi ML et. al.

May 5th, 2019 - No data are available on the specific energy needs of patients affected with Urea Cycle disorders (UCD) and especially argininosuccinic aciduria (ASA). In our experience, ASA patients tend to develop central adiposity and hypertriglyceridemia when...

Differential Intraoperative Effect of Liver Transplant in Different Inborn Errors of Me...
https://doi.org/10.1097/MPG.0000000000002354
Journal of Pediatric Gastroenterology and Nutrition; Porta F, Romagnoli R et. al.

Apr 9th, 2019 - Liver transplant (LT) is a therapeutic option for a growing number of inborn errors of metabolism (IEM), including some disorders not confined to the liver. Clinical advantages of LT in maple syrup urine disease (MSUD), methylmalonic acidemia (MMA...

Argininosuccinic aciduria: Recent pathophysiological insights and therapeutic prospects.
https://doi.org/10.1002/jimd.12047
Journal of Inherited Metabolic Disease REFERENCES; Baruteau J, Diez-Fernandez C et. al.

Feb 6th, 2019 - The first patients affected by argininosuccinic aciduria (ASA) were reported 60 years ago. The clinical presentation was initially described as similar to other urea cycle defects, but increasing evidence has shown overtime an atypical systemic ph...

Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene tran...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6115417
Nature Communications; Baruteau J, Perocheau DP et. al.

Aug 30th, 2018 - Argininosuccinate lyase (ASL) belongs to the hepatic urea cycle detoxifying ammonia, and the citrulline-nitric oxide (NO) cycle producing NO. ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia, multiorgan dis...

Arginine and Buphenyl in Patients With Argininosuccinic Aciduria (ASA), a Urea Cycle Disorder
https://clinicaltrials.gov/ct2/show/NCT00345605

Jan 30th, 2018 - The cause of liver damage in people with ASA is unknown. However, because ASA is the only urea cycle disorder that is characterized by both liver damage and elevated levels of argininosuccinic acid, researchers believe that the elevated acid level...

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Clinicaltrials.gov  39 results

Resting energy expenditure in argininosuccinic aciduria and in other urea cycle disorders.
https://doi.org/10.1002/jimd.12108
Journal of Inherited Metabolic Disease REFERENCES; Brambilla A, Bianchi ML et. al.

May 5th, 2019 - No data are available on the specific energy needs of patients affected with Urea Cycle disorders (UCD) and especially argininosuccinic aciduria (ASA). In our experience, ASA patients tend to develop central adiposity and hypertriglyceridemia when...

Differential Intraoperative Effect of Liver Transplant in Different Inborn Errors of Me...
https://doi.org/10.1097/MPG.0000000000002354
Journal of Pediatric Gastroenterology and Nutrition; Porta F, Romagnoli R et. al.

Apr 9th, 2019 - Liver transplant (LT) is a therapeutic option for a growing number of inborn errors of metabolism (IEM), including some disorders not confined to the liver. Clinical advantages of LT in maple syrup urine disease (MSUD), methylmalonic acidemia (MMA...

Argininosuccinic aciduria: Recent pathophysiological insights and therapeutic prospects.
https://doi.org/10.1002/jimd.12047
Journal of Inherited Metabolic Disease REFERENCES; Baruteau J, Diez-Fernandez C et. al.

Feb 6th, 2019 - The first patients affected by argininosuccinic aciduria (ASA) were reported 60 years ago. The clinical presentation was initially described as similar to other urea cycle defects, but increasing evidence has shown overtime an atypical systemic ph...

Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene tran...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6115417
Nature Communications; Baruteau J, Perocheau DP et. al.

Aug 30th, 2018 - Argininosuccinate lyase (ASL) belongs to the hepatic urea cycle detoxifying ammonia, and the citrulline-nitric oxide (NO) cycle producing NO. ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia, multiorgan dis...

Arginine and Buphenyl in Patients With Argininosuccinic Aciduria (ASA), a Urea Cycle Disorder
https://clinicaltrials.gov/ct2/show/NCT00345605

Jan 30th, 2018 - The cause of liver damage in people with ASA is unknown. However, because ASA is the only urea cycle disorder that is characterized by both liver damage and elevated levels of argininosuccinic acid, researchers believe that the elevated acid level...

see more →

News  1 results

Nitric Oxide Works When BP Drugs Fail
https://www.medpagetoday.com/cardiology/hypertension/32383

Apr 26th, 2012 - Action Points Nitric oxide supplements were able to rein in treatment-resistant hypertension in a patient with argininosuccinic aciduria (ASA), one of the most common urea-cycle disorders. Note that ASA is a congenital disorder that renders patien...

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