不要放过任何蛛丝马迹：减肥手术后严重营养不良与高氨血症性脑病：病例报告与文献复习

　　减肥手术后神经系统并发症并不少见。由于获得性或先天性尿素循环障碍引起的高氨血症性脑（HAE）也包括在内。儿科营养支持医师熟识尿素循环障碍，而成人医师未必。在此，我们报道一例最初被误诊为肝硬化的Roux-en-Y胃旁路手术4年后反复发生HAE患者。她被诊断为尿素循环起始端障碍（鸟氨酸氨甲酰转移酶缺乏症）和严重蛋白质热量营养不良。该患者在针对性专业营养和药物支持后恢复。文献复习表明该情况多发死亡，提示早期诊断和适当营养支持的重要性。

JPEN J Parenter Enteral Nutr. 2015 Nov;39(8):977-85.

Treating Every Needle in the Haystack: Hyperammonemic Encephalopathy and Severe Malnutrition After Bariatric Surgery-A Case Report and Review of the Literature.

Singh S, Suresh S, McClave SA, Cave M.

University of Louisville School of Medicine, Louisville, Kentucky.

University of Louisville, Louisville, Kentucky.

Robley Rex Louisville Veterans Affairs Medical Center, Louisville, Kentucky.

Neurologic complications are not uncommon following bariatric surgery. Hyperammonemic encephalopathy (HAE) due to an acquired or unmasked urea cycle deficit is among the rarest of these. Pediatric nutrition support specialists are familiar with recognizing urea cycle deficits, but adult specialists may not be. Here we present a case of a patient initially misdiagnosed with cirrhosis who presented with recurrent HAE 4 years after Roux-en-Y gastric bypass. She was diagnosed with a proximal urea cycle deficit and severe protein calorie malnutrition. The patient recovered with specialized nutrition and medical support targeting this condition. A literature review indicates multiple fatalities from this condition, indicating the importance of early diagnosis and appropriate nutrition support.

KEYWORDS: OTC deficiency; bariatric surgery complications; hyperammonemic; noncirrhotic encephalopathy

PMID: 25185153

DOI: 10.1177/0148607114546900