Hourigan S K, Anders R A, Mitchell S E, Schwarz K B, Lau H, Karnsakul W
Division of Pediatric Gastroenterology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Pediatr Transplant. 2012 Nov;16(7):E328-31. doi: 10.1111/j.1399-3046.2012.01686.x. Epub 2012 Apr 10.
An 18-month-old female status post-orthotopic liver transplant for biliary atresia presented nine months after transplant with severe diarrhea and intolerance of feeds. She was found to have a PLE as evidenced by a low serum albumin and a persistent elevation of fecal A1AT. Investigation eventually revealed that the cause of the PLE was a stricture at the anastomosis site between the hepatic vein and inferior cava, supported by resolution of the PLE after venoplasty of the stricture. The patient has subsequently required several repeat venoplasties for recurrence of her symptoms correlating with recurrence of the stricture. This is a very rare presentation of hepatic venous outflow obstruction. Moreover, normal duplex ultrasound imaging of liver vasculature and her unusual presentation led to a delay in her diagnosis highlighting the need for an increased index of suspicion.
一名18个月大的女性，因胆道闭锁接受原位肝移植，移植后9个月出现严重腹泻和不耐受喂养。她被发现患有蛋白丢失性肠病，表现为血清白蛋白降低和粪便α1抗胰蛋白酶持续升高。最终调查发现，蛋白丢失性肠病的原因是肝静脉与下腔静脉吻合部位狭窄，狭窄部位经血管成形术后蛋白丢失性肠病症状缓解，证实了这一点。该患者随后因症状复发（与狭窄复发相关）需要多次重复血管成形术。这是肝静脉流出道梗阻的一种非常罕见的表现。此外，肝脏血管的双功超声成像正常以及她不寻常的表现导致诊断延迟，凸显了提高怀疑指数的必要性。
