Observational studyThe Journal of pediatrics2013
Infectious precipitants of acute hyperammonemia are associated with indicators of increased morbidity in patients with urea cycle disorders.
Observational study in The Journal of pediatrics, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00237315 (Longitudinal Study of Urea Cycle Disorders), which is not on this map. Cited by 18 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Longitudinal Study of Urea Cycle Disorders
Who cites it
18 citing papers in PubMed, 39 citations in OpenAlex.
- Clinical and biochemical characteristics of patients with ornithine transcarbamylase deficiency and in silico analysis of OTC gene.Orphanet journal of rare diseases · 2025Article
- Amino Acid Metabolism and Immune Dysfunction in Urea Cycle Disorders: T and B Cell Perspectives.Journal of inherited metabolic disease · 2025Article
- Datamining approaches for examining the low prevalence of N-acetylglutamate synthase deficiency and understanding transcriptional regulation of urea cycle genes.Journal of inherited metabolic disease · 2024Article
- Citrulline in the management of patients with urea cycle disorders.Orphanet journal of rare diseases · 2023Article
- A promoter variant in the OTC gene associated with late and variable age of onset hyperammonemia.Journal of inherited metabolic disease · 2022Article
- Immune Alterations in a Patient With Hyperornithinemia-Hyperammonemia-Homocitrullinuria Syndrome: A Case Report.Frontiers in immunology · 2022Article
- l-Arginine Synthesis from l-Citrulline in Myeloid Cells Drives Host Defense against Mycobacteria In Vivo.Journal of immunology (Baltimore, Md. : 1950) · 2019Article
- Dietary Management of Propionic Acidemia: Parent Caregiver Perspectives and Practices.JPEN. Journal of parenteral and enteral nutrition · 2019Article
- Transatlantic combined and comparative data analysis of 1095 patients with urea cycle disorders-A successful strategy for clinical research of rare diseases.Journal of inherited metabolic disease · 2019Article
- Barriers to drug adherence in the treatment of urea cycle disorders: Assessment of patient, caregiver and provider perspectives.Molecular genetics and metabolism reports · 2016Article
- Computational modeling to predict nitrogen balance during acute metabolic decompensation in patients with urea cycle disorders.Journal of inherited metabolic disease · 2016Article
- Kupffer cells modulate hepatic fatty acid oxidation during infection with PR8 influenza.Biochimica et biophysica acta · 2015Article
- Gene expression in cell lines from propionic acidemia patients, carrier parents, and controls.Molecular genetics and metabolism · 2015Article
- Genotype-Phenotype Correlations in Ornithine Transcarbamylase Deficiency: A Mutation Update.Journal of genetics and genomics = Yi chuan xue bao · 2015Review
- Impaired T cell function in argininosuccinate synthetase deficiency.Journal of leukocyte biology · 2015Article
- Article
- Sudden unexpected fatal encephalopathy in adults with OTC gene mutations-Clues for early diagnosis and timely treatment.Orphanet journal of rare diseases · 2014Article
- A longitudinal study of urea cycle disorders.Molecular genetics and metabolismReview
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 1 country.
Funding
Abstract
objectiveTo prospectively characterize acute hyperammonemic episodes in patients with urea cycle disorders (UCDs) in terms of precipitating factors, treatments, and use of medical resources. STUDY
designThis was a prospective, longitudinal observational study of hyperammonemic episodes in patients with UCD enrolled in the National Institutes of Health-sponsored Urea Cycle Disorders Consortium Longitudinal Study. An acute hyperammonemic event was defined as plasma ammonia level >100 μmol/L. Physician-reported data regarding the precipitating event and laboratory and clinical variables were recorded in a central database.
resultsIn our study population, 128 patients with UCD experienced a total of 413 hyperammonemia events. Most patients experienced between 1 and 3 (65%) or between 4 and 6 (23%) hyperammonemia events since study inception, averaging fewer than 1 event/year. The most common identifiable precipitant was infection (33%), 24% of which were upper/lower respiratory tract infections. Indicators of increased morbidity were seen with infection, including increased hospitalization rates (P = .02), longer hospital stays (+2.0 days; P = .003), and increased use of intravenous ammonia scavengers (+45%-52%; P = .003-.03).
conclusionInfection is the most common precipitant of acute hyperammonemia in patients with UCD and is associated with indicators of increased morbidity (ie, hospitalization rate, length of stay, and use of intravenous ammonia scavengers). These findings suggest that the catabolic and immune effects of infection may be a target for clinical intervention in inborn errors of metabolism.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.