[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"fatty-acid-oxidation-disorder\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:fatty-acid-oxidation-disorder":28},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,2,0,[8,43],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":4,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":18,"targetDuration":4,"studyType":21,"phases":22,"briefSummary":24,"conditions":25,"keywords":4,"overallStatus":30,"whyStopped":4,"lastUpdateSubmitDate":31,"lastUpdatePostDateStruct":32,"startDateStruct":35,"completionDateStruct":37,"leadSponsor":39,"locationsCount":42},"100589368","at-home-ammonia-monitoring-of-inborn-errors-of-ammonia-metabolism-100589368",false,"NCT06953505","At Home Ammonia Monitoring of Inborn Errors of Ammonia Metabolism","Pilot Study of At Home Ammonia Monitoring in Patients With an Inborn Error of Ammonia Metabolism","Inclusion Criteria:\n\n* Confirmed ammonia disorder such as\n\n  * Ornithine transcarbamylase deficiency\n  * Systemic primary carnitine deficiency\n  * Type I citrullinemia\n  * Argininosuccinic aciduria\n  * Isolated methylmalonic acidemia\n  * Type II citrullinemia\n  * Propionic acidemia\n  * Isovaleric acidemia\n  * Multiple acyl-CoA dehydrogenase deficiency\n  * Pyruvate carboxylase deficiency\n  * Argininemia (arginase deficiency)\n  * Carbamoyl phosphate synthase I deficiency\n  * Dihydrolipoamide dehydrogenase deficiency\n  * Lysinuric protein intolerance\n  * Hyperornithinemia-hyperammonemia-homocitrullinuria\n  * Carnitine-acylcarnitine translocase deficiency\n  * Carbonic anhydrase VA deficiency\n  * N-acetyl glutamate synthetase deficiency\n* English Literacy\n\nExclusion Criteria:\n\n* Incarcerated\n* Presence of a non-inborn error of metabolism medical condition associated with abnormal ammonia metabolism (e.g., end stage liver disease).\n* Unable to read in English","ALL","12 Years",{"count":19,"type":20},30,"ESTIMATED","INTERVENTIONAL",[23],"NA","The goal of this observational study is to learn if people with certain ammonia metabolism disorders will measure their ammonia levels at home.\n\nThe main question it aims to answer is:\n\n• Will participants measure their ammonia every day?\n\nParticipants will be asked to:\n\n* Attend two in-person study visits at the clinic.\n* Measure temperature, heart rate, and blood oxygen every day.\n* Complete a short survey every day.\n* Measure ammonia every day.",[26,27,28,29],"Urea Cycle Disorders","Organic Acidemias","Fatty Acid Oxidation Disorder","Ammonia; Metabolic Disorder","RECRUITING","2026-05-21",{"date":33,"type":34},"2026-05-27","ACTUAL",{"date":36,"type":34},"2025-09-23",{"date":38,"type":20},"2027-08",{"name":40,"class":41},"Sequitur Health Corp.","INDUSTRY",1,{"id":44,"slug":45,"hasResults":11,"nctId":46,"briefTitle":47,"officialTitle":47,"acronym":4,"eligibilityCriteria":48,"healthyVolunteers":11,"sex":16,"minAge":49,"maxAge":50,"enrollmentInfo":51,"targetDuration":4,"studyType":53,"phases":4,"briefSummary":54,"conditions":55,"keywords":61,"overallStatus":30,"whyStopped":4,"lastUpdateSubmitDate":76,"lastUpdatePostDateStruct":77,"startDateStruct":79,"completionDateStruct":81,"leadSponsor":83,"locationsCount":42},"100408734","systemic-biomarkers-of-brain-injury-from-hyperammonemia-100408734","NCT04602325","Systemic Biomarkers of Brain Injury From Hyperammonemia","Inclusion Criteria:\n\n1. Inherited Hyperammonemias:\n\n   1. A clinical diagnosis of 1 of 7 diagnosed urea cycle disorders:\n\n      * N-acetylglutamate Synthetase Deficiency (NAGS)\n      * Carbamyl Phosphate Synthetase Deficiency (CPSD)\n      * Ornithine Transcarbamylase Deficiency (OTCD)\n      * Argininosuccinate Synthetase Deficiency (ASD)\n      * Argininosuccinate Lyase Deficiency (ALD)\n      * Arginase Deficiency (AD)\n      * Hyperammonemia-Hyperornithinemia-Homocitrullinuria (HHH)\n   2. A clinical diagnosis of 1 of 2 organic acidemias:\n\n      * Propionic Acidemia (PA)\n      * Methylmalonic Acidemia (MMA)\n2. Acute metabolic disorder without hyperammonemia, with neurological sequelae\n\n   1. Maple Syrup Urine Disease (MSUD)\n   2. Glutaric Acidemia (GA1)\n3. Acute metabolic disorder without hyperammonemia and without neurological sequelae\n\n   * Fatty Acid Oxidation Disorders:\n   * Medium Chain-Acyl CoA Dehydrogenase Deficiency\n   * Very Long Chain-Acyl CoA Dehydrogenase Deficiency\n   * Trifunctional Protein Deficiency\n   * Long Chain Hydroxyacyl-CoA Dehydrogenase Deficiency\n   * Carnitine Palmitoyltransferase I or II Deficiency\n   * Carnitine\u002FAcylcarnitine Translocase Deficiency\n   * Primary Carnitine Transport Deficiency\n4. Hypoxic-Ischemic Encephalopathy\n\nExclusion Criteria:\n\n* Prior Solid-Organ Transplant\n* Use of any other investigational drug, biologic, or therapy or any clinical or laboratory abnormality or medical condition that, as determined by the investigator, may interfere with or obscure the biomarker measurements","7 Years","18 Years",{"count":52,"type":20},24,"OBSERVATIONAL","Ammonia is a waste product of protein and amino acid catabolism and is also a potent neurotoxin. High blood ammonia levels on the brain can manifest as cytotoxic brain edema and vascular compromise leading to intellectual and developmental disabilities. The following aims are proposed:\n\nAim 1 of this study will be to determine the chronology of biomarkers of brain injury in response to a hyperammonemic (HA) brain insult in patients with an inherited hyperammonemic disorder.\n\nAim 2 will be to determine if S100B, NSE, and UCHL1 are altered in patients with two other inborn errors of metabolism, Maple Syrup Urine Disease (MSUD) and Glutaric Acidemia (GA1).",[56,57,58,59,28,60],"Urea Cycle Disorder","Organic Acidemia","Maple Syrup Urine Disease","Glutaric Acidemia I","Hypoxic-Ischemic Encephalopathy",[62,63,64,65,66,67,68,69,70,71,72,73,74,75],"N-acetylglutamate Synthetase Deficiency","Carbamyl Phosphate Synthetase Deficiency","Ornithine Transcarbamylase Deficiency","Argininosuccinate Synthetase Deficiency","Argininosuccinate Lyase Deficiency","Arginase Deficiency","Hyperammonemia-Hyperornithinemia-Homocitrullinuria","Medium Chain-Acyl CoA Dehydrogenase Deficiency","Very Long Chain-Acyl CoA Dehydrogenase Deficiency","Trifunctional Protein Deficiency","Long Chain Hydroxyacyl-CoA Dehydrogenase Deficiency","Carnitine Palmitoyltransferase I or II Deficiency","Carnitine\u002FAcylcarnitine Translocase Deficiency","Primary Carnitine Transport Deficiency","2024-02-06",{"date":78,"type":34},"2024-02-07",{"date":80,"type":34},"2020-07-09",{"date":82,"type":20},"2027-05",{"name":84,"class":85},"Children's National Research Institute","OTHER"]