首页    期刊浏览 2024年10月06日 星期日
登录注册

文章基本信息

  • 标题:Recommendations for the Diagnosis and Therapeutic Management of Hyperammonaemia in Paediatric and Adult Patients
  • 本地全文:下载
  • 作者:Amaya Bélanger-Quintana ; Francisco Arrieta Blanco ; Delia Barrio-Carreras
  • 期刊名称:Nutrients
  • 电子版ISSN:2072-6643
  • 出版年度:2022
  • 卷号:14
  • 期号:13
  • DOI:10.3390/nu14132755
  • 语种:English
  • 出版社:MDPI Publishing
  • 摘要: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.
  • 关键词:enammoniahyperammonaemiadiagnosistherapeuticsurea cycle disordershaemodialysis
国家哲学社会科学文献中心版权所有