Hyperammonemia

Common Name(s)

Hyperammonemia

Description for this condition is not yet available.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hyperammonemia" for support, advocacy or research.

National Urea Cycle Disorders Foundation

The National Urea Cycle Disorders Foundation is the only organization solely dedicated to saving the lives of children and adults with urea cycle disorders and leads the search for a cure. Internationally recognized, NUCDF serves as a lifeline to families and medical professionals around the world seeking life saving information. NUCDF is the driving force stimulating and supporting research for new treatments, improved outcomes, and a Cure. NUCDF efforts include educating UCD patients, families and medical professionals on diagnosis, treatment and management, and providing guidance, resources, information and support to all those affected by urea cycle disorders. The UCD International Patient Registry (www.ucdregistry.org), empowers UCD patients and caregivers to self-report their own unique experiences with UCD and compare them to others with UCD. Participants in the UCD Registry can help improve the understanding of UCDs, improve care, and accelerate research.

Last Updated: 11 Jun 2015

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hyperammonemia" for support, advocacy or research.

National Urea Cycle Disorders Foundation

The National Urea Cycle Disorders Foundation is the only organization solely dedicated to saving the lives of children and adults with urea cycle disorders and leads the search for a cure. Internationally recognized, NUCDF serves as a lifeline to families and medical professionals around the world seeking life saving information. NUCDF is the driving force stimulating and supporting research for new treatments, improved outcomes, and a Cure. NUCDF efforts include educating UCD patients, families and medical professionals on diagnosis, treatment and management, and providing guidance, resources, information and support to all those affected by urea cycle disorders. The UCD International Patient Registry (www.ucdregistry.org), empowers UCD patients and caregivers to self-report their own unique experiences with UCD and compare them to others with UCD. Participants in the UCD Registry can help improve the understanding of UCDs, improve care, and accelerate research.

http://www.nucdf.org

Last Updated: 11 Jun 2015

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General Support Organizations

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General Resources

UCD International Patient Registry

Join other UCD families in the only registry in existence designed to accelerate new research specifically for UCDs. Patients & caregivers self-report their own unique experiences with UCD to help improve care and find new treatments.

Updated 18 Mar 2013

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Hyperammonemia" returned 77 free, full-text research articles on human participants. First 3 results:

A novel mutation in ornithine transcarbamylase gene causing mild intermittent hyperammonemia.
 

Author(s): Sarar Mohamed, Muddathir H Hamad, Altaf A Kondkar, Khaled K Abu-Amero

Journal: Saudi Med J. 2015 Oct;36(10):1229-32.

 

We report a 3-year-old Saudi boy with recurrent episodes of vomiting, poor feeding, and altered mental status accompanied by an intermittent mild hyperammonemia, and a large elevation of urinary orotic acid. Sanger sequencing of the ornithine transcarbamylase (OTC) gene revealed a ...

Last Updated: 8 Oct 2015

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Hyperammonemia in Urinary Tract Infections.
 

Author(s): Tsuneaki Kenzaka, Ken Kato, Akihito Kitao, Koki Kosami, Kensuke Minami, Shinsuke Yahata, Miho Fukui, Masanobu Okayama

Journal:

 

The present study investigated the incidence of hyperammonemia in urinary tract infections and explored the utility of urinary obstruction relief and antimicrobial administration to improve hyperammonemia.

Last Updated: 21 Aug 2015

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GR3027 antagonizes GABAA receptor-potentiating neurosteroids and restores spatial learning and motor coordination in rats with chronic hyperammonemia and hepatic encephalopathy.
 

Author(s): Maja Johansson, Ana Agusti, Marta Llansola, Carmina Montoliu, Jessica Strömberg, Evgenya Malinina, Gianna Ragagnin, Magnus Doverskog, Torbjörn Bäckström, Vicente Felipo

Journal: Am. J. Physiol. Gastrointest. Liver Physiol.. 2015 Sep;309(5):G400-9.

 

Hepatic encephalopathy (HE) is one of the primary complications of liver cirrhosis. Current treatments for HE, mainly directed to reduction of ammonia levels, are not effective enough because they cannot completely eliminate hyperammonemia and inflammation, which induce the neurological ...

Last Updated: 2 Sep 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Hyperammonemia" returned 3 free, full-text review articles on human participants. First 3 results:

The hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
 

Author(s): Diego Martinelli, Daria Diodato, Emanuela Ponzi, Magnus Monné, Sara Boenzi, Enrico Bertini, Giuseppe Fiermonte, Carlo Dionisi-Vici

Journal:

 

Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive disorder of the urea cycle. HHH has a panethnic distribution, with a major prevalence in Canada, Italy and Japan. Acute clinical signs include intermittent episodes of vomiting, confusion ...

Last Updated: 16 Apr 2015

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Alterations of blood brain barrier function in hyperammonemia: an overview.
 

Author(s): Marta Skowrońska, Jan Albrecht

Journal: Neurotox Res. 2012 Feb;21(2):236-44.

 

Ammonia is a neurotoxin involved in the pathogenesis of neurological conditions associated with hyperammonemia, including hepatic encephalopathy, a condition associated with acute--(ALF) or chronic liver failure. This article reviews evidence that apart from directly affecting the ...

Last Updated: 28 Dec 2011

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Hyperammonemia and neonatal cardiac failure.
 

Author(s): T Adamovic, P Jouvet, S Vobecky, L Garel, A L Rougemont, F Alvarez

Journal: Minerva Anestesiol. 2011 May;77(5):554-7.

 

Severe hyperammonemia (hyperNH3) in neonatal cardiac failure after cardiac surgery is rare. We report a case of a 2470-g female infant born at the week 37 of gestation with complex congenital heart disease (truncus arteriosus type III, interrupted aortic arch and tricuspid valve insufficiency) ...

Last Updated: 4 May 2011

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Blood Sugars in Children With Idiopathic Seizures.
 

Status: Recruiting

Condition Summary: Seizures; Hypoglycemia; Hyperammonemia

 

Last Updated: 6 Sep 2006

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N-Carbamylglutamate (Carbaglu) In The Treatment Of Hyperammonemia
 

Status: Recruiting

Condition Summary: Inborn Errors of Metabolism

 

Last Updated: 11 Dec 2015

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Short-term Outcome of N-Carbamylglutamate in the Treatment of Acute Hyperammonemia
 

Status: Recruiting

Condition Summary: Propionic Acidemia (PA); Methylmalonic Acidemia (MMA); Late-onset CPS1 Deficiency (CPSD); Late-onset Ornithine Transcarbamylase Deficiency (OTCD)

 

Last Updated: 26 Jan 2016

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