FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4387250049> ?p ?o ?g. }

• W4387250049 endingPage "A1921" @default.
• W4387250049 startingPage "A1920" @default.
• W4387250049 abstract "SESSION TITLE: Thinking Fast: Early Recognition and Innovative Solutions in the ICU SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/09/2023 09:40 am - 10:25 am INTRODUCTION: Hyperammonemia due to an unrecognized urea cycle disorder (UCD) can cause encephalopathy, cerebral edema, and death. These uncommon disorders are typically diagnosed in neonates. However, they can rarely present in adults following a precipitating metabolic stressor with associated increased protein catabolism, such as major surgery, significant trauma, certain medications (e.g., corticosteroids), prolonged fasting, or serious infection. Ornithine transcarbamylase deficiency (OTCD) is the most common UCD diagnosed in adulthood and is X-linked. Recently, idiopathic UCDs have been described with normal genetic testing. Herein, we report a case of near-fatal severe hyperammonemia in an otherwise healthy adult male following an uncomplicated, minimally invasive gastrectomy. CASE PRESENTATION: A 55-year-old Taiwanese male with a history of benign prostatic hyperplasia and tobacco abuse underwent an uneventful robotic-assisted gastrectomy for localized gastric adenocarcinoma. On postoperative day two, the patient developed hyper- and hypoactive delirium prompting admission to the intensive care unit. Admission workup revealed an ammonia level of 186. Other diagnostic components of an exhaustive encephalopathy investigation were unremarkable including EEG (revealing diffuse background slowing only), initial CT head, and renal and liver function. His ammonia level peaked on postoperative day three at 464. He developed cerebral edema and multisystem organ dysfunction necessitating intubation, mechanical ventilation, vasopressors, sedation, paralysis, and multimodal therapy for a suspected UCD. For his hyperammonemia, the patient received lipid and amino acid infused TPN, arginine, lactulose, rifaximin, an insulin infusion, and he underwent hemodialysis. Elevated urine glutamine and orotic acid, decreased serum citrulline, and elevated serum glutamine were later identified. These findings are consistent with a proximal UCD such as OTCD. The patient's family denied any prior dietary restrictions or preferences, preexisting behavioral changes, liver disease, nor use of any medications known to precipitate hyperammonemia. His serum ammonia levels eventually normalized, though his neurological exam remained poor, and he required tracheostomy and PEG tube placement. The patient was gradually weaned from the ventilator and his mental status improved slowly over the coming months. He was discharged home, decannulated from his tracheostomy, his PEG tube was removed, and he is currently independent in his ADLs. DNA testing for genetic mutations associated with UCDs was negative, indicating a likely idiopathic urea cycle enzymatic defect. DISCUSSION: This case highlights the catastrophic consequences that may occur with unmasked UCDs and the need for vigilance and prompt intervention. Our case is unusual considering the patient's age, lack of preexisting behavioral changes, negative genetic testing, minimally invasive surgery, absence of dietary modifications or more common precipitants of hyperammonemia, and his excellent outcome. Although hyperammonemia owing to such disorders is rare, it is commonly fatal as described in multiple case reports. His survival may be due to an early and multifaceted treatment approach which was implemented prior to a definitive diagnosis. CONCLUSIONS: While mild hyperammonemia can be common in critically ill patients and does not necessarily require treatment nor workup, ammonia levels should be obtained in cases of otherwise unspecified marked encephalopathy, with consideration of an unmasked UCD precipitated by a metabolic stressor (e.g., surgery) in those patients with unexplained severe hyperammonemia. REFERENCE #1: Clay AS, Hainline BE. Hyperammonemia in the ICU. Chest. 2007 Oct;132(4):1368-78. doi: 10.1378/chest.06-2940. PMID: 17934124. REFERENCE #2: Sakusic A, Sabov M, McCambridge AJ, Rabinstein AA, Singh TD, Mukesh K, Kashani KB, Cook D, Gajic O. Features of Adult Hyperammonemia Not Due to Liver Failure in the ICU. Crit Care Med. 2018 Sep;46(9):e897-e903. doi: 10.1097/CCM.0000000000003278. PMID: 29985210; PMCID: PMC6095817. REFERENCE #3: Walker V. Severe hyperammonaemia in adults not explained by liver disease. Annals of Clinical Biochemistry. 2012;49(3):214-228. DISCLOSURES: Advisory Committee Member relationship with Atavistik Bio Please note: 2021-present Added 03/31/2023 by Ralph DeBerardinis, source=Web Response, value=Consulting fee No relevant relationships by Alina Dulu No relevant relationships by Nina Raoof No relevant relationships by Ryan Sieli" @default.
• W4387250049 created "2023-10-03" @default.
• W4387250049 creator A5013924501 @default.
• W4387250049 creator A5060825131 @default.
• W4387250049 creator A5091057018 @default.
• W4387250049 creator A5092984845 @default.
• W4387250049 date "2023-10-01" @default.
• W4387250049 modified "2023-10-03" @default.
• W4387250049 title "UREA CYCLE DISORDER UNMASKED: CAN EARLY INTERVENTION IMPROVE SURVIVAL?" @default.
• W4387250049 doi "https://doi.org/10.1016/j.chest.2023.07.1321" @default.
• W4387250049 hasPublicationYear "2023" @default.
• W4387250049 type Work @default.
• W4387250049 citedByCount "0" @default.
• W4387250049 crossrefType "journal-article" @default.
• W4387250049 hasAuthorship W4387250049A5013924501 @default.
• W4387250049 hasAuthorship W4387250049A5060825131 @default.
• W4387250049 hasAuthorship W4387250049A5091057018 @default.
• W4387250049 hasAuthorship W4387250049A5092984845 @default.
• W4387250049 hasBestOaLocation W43872500491 @default.
• W4387250049 hasConcept C126322002 @default.
• W4387250049 hasConcept C177713679 @default.
• W4387250049 hasConcept C185592680 @default.
• W4387250049 hasConcept C187212893 @default.
• W4387250049 hasConcept C2777468819 @default.
• W4387250049 hasConcept C2778377652 @default.
• W4387250049 hasConcept C2778621155 @default.
• W4387250049 hasConcept C2779493303 @default.
• W4387250049 hasConcept C2779753318 @default.
• W4387250049 hasConcept C2780469793 @default.
• W4387250049 hasConcept C2781099255 @default.
• W4387250049 hasConcept C515207424 @default.
• W4387250049 hasConcept C55493867 @default.
• W4387250049 hasConcept C71924100 @default.
• W4387250049 hasConcept C77061444 @default.
• W4387250049 hasConceptScore W4387250049C126322002 @default.
• W4387250049 hasConceptScore W4387250049C177713679 @default.
• W4387250049 hasConceptScore W4387250049C185592680 @default.
• W4387250049 hasConceptScore W4387250049C187212893 @default.
• W4387250049 hasConceptScore W4387250049C2777468819 @default.
• W4387250049 hasConceptScore W4387250049C2778377652 @default.
• W4387250049 hasConceptScore W4387250049C2778621155 @default.
• W4387250049 hasConceptScore W4387250049C2779493303 @default.
• W4387250049 hasConceptScore W4387250049C2779753318 @default.
• W4387250049 hasConceptScore W4387250049C2780469793 @default.
• W4387250049 hasConceptScore W4387250049C2781099255 @default.
• W4387250049 hasConceptScore W4387250049C515207424 @default.
• W4387250049 hasConceptScore W4387250049C55493867 @default.
• W4387250049 hasConceptScore W4387250049C71924100 @default.
• W4387250049 hasConceptScore W4387250049C77061444 @default.
• W4387250049 hasIssue "4" @default.
• W4387250049 hasLocation W43872500491 @default.
• W4387250049 hasOpenAccess W4387250049 @default.
• W4387250049 hasPrimaryLocation W43872500491 @default.
• W4387250049 hasRelatedWork W1760466333 @default.
• W4387250049 hasRelatedWork W2012976763 @default.
• W4387250049 hasRelatedWork W2031971621 @default.
• W4387250049 hasRelatedWork W2063920409 @default.
• W4387250049 hasRelatedWork W2107833912 @default.
• W4387250049 hasRelatedWork W2113926559 @default.
• W4387250049 hasRelatedWork W2282465991 @default.
• W4387250049 hasRelatedWork W3019902980 @default.
• W4387250049 hasRelatedWork W3105558991 @default.
• W4387250049 hasRelatedWork W3120956988 @default.
• W4387250049 hasVolume "164" @default.
• W4387250049 isParatext "false" @default.
• W4387250049 isRetracted "false" @default.
• W4387250049 workType "article" @default.