Al-Saedi Zainulabdeen, Miqdad Mohammed A, Alatta Lina, Spinowitz Bruce, Kuo Sheng
Medicine/Nephrology, NewYork-Presbyterian Queens, New York, USA.
Research, Michigan State University, East Lansing, USA.
Cureus. 2024 Dec 7;16(12):e75253. doi: 10.7759/cureus.75253. eCollection 2024 Dec.
High anion gap metabolic acidosis (HAGMA) is a common biochemical abnormality in hospitalized patients, often linked to conditions such as lactic acidosis, renal failure, or drug toxicity. A rare etiology, 5-oxoprolinuria, resulting from acetaminophen use, malnutrition, and sepsis, is increasingly recognized in critically ill patients. We report a 29-year-old male with a history of intellectual disability and normal baseline kidney function who was admitted with acute necrotizing pancreatitis and developed severe metabolic acidosis and acute kidney injury (AKI). Despite extensive management, including continuous renal replacement therapy (CRRT) and hemodialysis, he exhibited persistent HAGMA resistant to standard treatments. Following a prolonged hospital course complicated by various interventions, elevated urine 5-oxoproline levels were identified, leading to the discontinuation of acetaminophen and the initiation of N-acetylcysteine therapy. This case highlights the challenges in diagnosing 5-oxoproline acidosis, particularly in the context of multi-factorial illness involving sepsis and malnutrition. The significant accumulation of 5-oxoproline underscores the metabolic stress associated with reactive oxygen species (ROS) depletion in critically ill patients. The recognition of this condition is crucial, as it indicates underlying metabolic derangements and necessitates prompt therapeutic intervention. Continued awareness and understanding of 5-oxoproline acidosis may improve outcomes in similar patients by guiding appropriate management strategies.
高阴离子间隙代谢性酸中毒(HAGMA)是住院患者常见的生化异常,常与乳酸酸中毒、肾衰竭或药物毒性等情况相关。一种由对乙酰氨基酚使用、营养不良和脓毒症引起的罕见病因——5-氧脯氨酸尿症,在重症患者中越来越受到认可。我们报告了一名29岁男性,有智力残疾病史且基线肾功能正常,因急性坏死性胰腺炎入院,并发严重代谢性酸中毒和急性肾损伤(AKI)。尽管进行了广泛的治疗,包括持续肾脏替代治疗(CRRT)和血液透析,但他仍表现出对标准治疗有抵抗的持续性HAGMA。在经历了因各种干预而延长的住院病程后,发现尿中5-氧脯氨酸水平升高,导致停用对乙酰氨基酚并开始N-乙酰半胱氨酸治疗。该病例突出了诊断5-氧脯氨酸酸中毒的挑战,特别是在涉及脓毒症和营养不良的多因素疾病背景下。5-氧脯氨酸的显著积累强调了重症患者中与活性氧(ROS)消耗相关的代谢应激。认识到这种情况至关重要,因为它表明潜在的代谢紊乱,需要及时进行治疗干预。持续认识和理解5-氧脯氨酸酸中毒可能通过指导适当的管理策略来改善类似患者的预后。