Ensenauer R, Hentschel R, Rückauer K, Rädecke J, Brandis M, Berner R
Universitäts-Kinderklinik Freiburg, Germany.
Eur J Pediatr Surg. 1999 Aug;9(4):244-7. doi: 10.1055/s-2008-1072254.
In children with short-bowel syndrome and the need for long-term parenteral nutrition, hepatic dysfunction is a multifactorial phenomenon that has not been completely understood. Alterations in gut motility lead to intraluminal stasis which is thought to be a major etiologic factor for bacterial overgrowth and subsequent cholestasis, especially when the ileocecal valve is absent. We report on two infants with short-bowel syndrome caused by gastroschisis and intestinal atresia. The intestinal lengths after resection were 18 and 55 cm. Long-term parenteral nutrition (PN) was obligatory due to intestinal shortness in the first patient and dilatation of the preatretic bowel segment with ineffective peristalsis in the second patient. Despite multiple trials of enteral nutrition and medical therapy for gut decontamination and stimulation of bowel motility, hepatopathy developed in both patients in a similar period of time and to about the same degree. At the age of 4 and 6 weeks, respectively, increasing bilirubin values were measured. Deterioration of liver function and thrombocytopenia at the age of 3 to 4 months led to the diagnosis of acute cytomegalovirus (CMV) infection. Treatment with ganciclovir followed. Both patients died of acute liver failure at the age of 7 and 9 months, respectively. Additional hepatic injury secondary to CMV infection might have contributed to the rapid deterioration of liver disease. Screening for further hepatotoxic factors, especially infectious etiologies, is therefore recommended in children with short-bowel syndrome. Liver transplantation should be considered early in cases of progressive hepatic dysfunction.
在患有短肠综合征且需要长期肠外营养的儿童中，肝功能障碍是一种尚未完全被理解的多因素现象。肠道动力改变导致肠腔内淤滞，这被认为是细菌过度生长及随后胆汁淤积的主要病因，尤其是在回盲瓣缺失的情况下。我们报告了两名因腹裂和肠闭锁导致短肠综合征的婴儿。切除术后的肠长分别为18厘米和55厘米。由于第一名患者肠道过短，第二名患者闭锁前肠段扩张且蠕动无效，因此长期肠外营养（PN）是必要的。尽管多次尝试肠内营养以及进行肠道去污和刺激肠道动力的药物治疗，但两名患者均在相似的时间段内且以大致相同的程度出现了肝病。分别在4周和6周龄时，测得胆红素值升高。在3至4月龄时肝功能恶化和血小板减少导致了急性巨细胞病毒（CMV）感染的诊断。随后采用更昔洛韦治疗。两名患者分别在7个月和9个月龄时死于急性肝衰竭。CMV感染继发的额外肝损伤可能导致了肝病的迅速恶化。因此，建议对短肠综合征患儿筛查其他肝毒性因素，尤其是感染性病因。对于进行性肝功能障碍的病例，应尽早考虑肝移植。
