Jun Woo Young, Cho Min Jeng, Han Hye Seung, Bae Sun Hwan
Department of Pediatrics, Konkuk University Medical Center, Seoul, Korea.
Department of Pediatric Surgery, Konkuk University Medical Center, Seoul, Korea.
Pediatr Gastroenterol Hepatol Nutr. 2016 Dec;19(4):286-290. doi: 10.5223/pghn.2016.19.4.286. Epub 2016 Dec 28.
Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.
浓缩胆汁综合征(IBS)是一种罕见病症,即浓稠的管腔内胆汁(包括胆栓、胆泥或结石)阻塞婴儿的肝外胆管。一名5周大的女婴因黄疸和无胆汁粪便入院评估。包括超声检查、磁共振胰胆管造影和肝胆扫描在内的诊断测试均无定论。尽管诊断尚不明确,但在给予熊去氧胆酸和含ω-3多不饱和脂肪酸(PUFAs)的脂质乳剂3周后,临床和实验室检查结果逐渐改善。然而,肝脏活检提示为胆道闭锁。这一发现促使我们进行术中胆管造影,结果显示胆总管通畅但有浓稠胆汁堵塞。我们进行了生理盐水冲洗,症状得到改善,最终诊断为IBS。因此,我们在此报告,ω-3 PUFAs可作为手术干预的替代方法用于治疗IBS。