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Volume 14
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Review

Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients

by
Amaya Bélanger-Quintana
1,*,
Francisco Arrieta Blanco
2,
Delia Barrio-Carreras
3,
Ana Bergua Martínez
4,
Elvira Cañedo Villarroya
5,
María Teresa García-Silva
3,
Rosa Lama More
6,
Elena Martín-Hernández
3,
Ana Moráis López
4,
Montserrat Morales-Conejo
7,
Consuelo Pedrón-Giner
5,
Pilar Quijada-Fraile
3,
Sinziana Stanescu
1 and
Mercedes Martínez-Pardo Casanova
1
1
Department of Paediatric Inborn Errors of Metabolism, Hospital Universitario Ramón y Cajal, Carretera de Colmenar Viejo, Km. 9, 100, 28034 Madrid, Spain
2
Department of Adult Inborn Errors of Metabolism, Hospital Universitario Ramón y Cajal, Carretera de Colmenar Viejo, Km. 9, 100, 28034 Madrid, Spain
3
Department of Inborn Errors of Metabolism, Hospital Universitario 12 de Octubre, Avenida de Córdoba, s/n, 28041 Madrid, Spain
4
Department of Paediatric Nutrition and Metabolic Diseases, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain
5
Department of Gastroenterology and Nutrition, Hospital Universitario Niño Jesús, Avenida de Menéndez Pelayo, 65, 28009 Madrid, Spain
6
Department of Paediatric Nutrition, Centro Médico D-Medical, Calle del Príncipe de Vergara, 44, 28001 Madrid, Spain
7
Department of Internal Medicine, Hospital Universitario 12 de Octubre, Avenida de Córdoba, s/n, 28041 Madrid, Spain
*
Author to whom correspondence should be addressed.
Nutrients 2022, 14(13), 2755; https://doi.org/10.3390/nu14132755
Submission received: 24 May 2022 / Revised: 28 June 2022 / Accepted: 30 June 2022 / Published: 2 July 2022
(This article belongs to the Special Issue Amino Acid Metabolism in Human Health and Disease)
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Abstract

Hyperammonaemia is a metabolic derangement that may cause severe neurological damage and even death due to cerebral oedema, further complicating the prognosis of its triggering disease. In small children it is a rare condition usually associated to inborn errors of the metabolism. As age rises, and especially in adults, it may be precipitated by heterogeneous causes such as liver disease, drugs, urinary infections, shock, or dehydration. In older patients, it is often overlooked, or its danger minimized. This protocol was drafted to provide an outline of the clinical measures required to normalise ammonia levels in patients of all ages, aiming to assist clinicians with no previous experience in its treatment. It is an updated protocol developed by a panel of experts after a review of recent publications. We point out the importance of frequent monitoring to assess the response to treatment, the nutritional measures that ensure not only protein restriction but adequate caloric intake and the need to avoid delays in the use of specific pharmacological therapies and, especially, extrarenal clearance measures. In this regard, we propose initiating haemodialysis when ammonia levels are >200–350 µmol/L in children up to 18 months of age and >150–200 µmol/L after that age.
Keywords: ammonia; hyperammonaemia; diagnosis; therapeutics; urea cycle disorders; haemodialysis ammonia; hyperammonaemia; diagnosis; therapeutics; urea cycle disorders; haemodialysis

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MDPI and ACS Style

Bélanger-Quintana, A.; Arrieta Blanco, F.; Barrio-Carreras, D.; Bergua Martínez, A.; Cañedo Villarroya, E.; García-Silva, M.T.; Lama More, R.; Martín-Hernández, E.; López, A.M.; Morales-Conejo, M.; et al. Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients. Nutrients 2022, 14, 2755. https://doi.org/10.3390/nu14132755

AMA Style

Bélanger-Quintana A, Arrieta Blanco F, Barrio-Carreras D, Bergua Martínez A, Cañedo Villarroya E, García-Silva MT, Lama More R, Martín-Hernández E, López AM, Morales-Conejo M, et al. Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients. Nutrients. 2022; 14(13):2755. https://doi.org/10.3390/nu14132755

Chicago/Turabian Style

Bélanger-Quintana, Amaya, Francisco Arrieta Blanco, Delia Barrio-Carreras, Ana Bergua Martínez, Elvira Cañedo Villarroya, María Teresa García-Silva, Rosa Lama More, Elena Martín-Hernández, Ana Moráis López, Montserrat Morales-Conejo, and et al. 2022. "Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients" Nutrients 14, no. 13: 2755. https://doi.org/10.3390/nu14132755

APA Style

Bélanger-Quintana, A., Arrieta Blanco, F., Barrio-Carreras, D., Bergua Martínez, A., Cañedo Villarroya, E., García-Silva, M. T., Lama More, R., Martín-Hernández, E., López, A. M., Morales-Conejo, M., Pedrón-Giner, C., Quijada-Fraile, P., Stanescu, S., & Casanova, M. M.-P. (2022). Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients. Nutrients, 14(13), 2755. https://doi.org/10.3390/nu14132755

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