Lacaille F, Vass N, Sauvat F, Canioni D, Colomb V, Talbotec C, De Serre N Patey-Mariaud, Salomon J, Hugot J-P, Cézard J-P, Révillon Y, Ruemmele F M, Goulet O
Pediatric Gastroenterology-Hepatology-Nutrition Unit, Necker-Enfants malades Hospital, 149 rue de Sèvres, 75015 Paris, France.
Gut. 2008 Apr;57(4):455-61. doi: 10.1136/gut.2007.133389. Epub 2007 Dec 13.
Small bowel (SB) transplantation (Tx), long considered a rescue therapy for patients with intestinal failure, is now a well recognised alternative treatment strategy to parental nutrition (PN). In this retrospective study, we analysed graft functions in 31 children after SBTx with a follow-up of 2-18 years (median 7 years).
Twelve children had isolated SBTx, 19 had combined liver-SBTx and 17 received an additional colon graft. Growth, nutritional markers, stool balance studies, endoscopy and graft histology were recorded every 2-3 years post-Tx.
All children were weaned from PN after Tx and 26 children remained PN-free. Enteral nutrition was required for 14/31 (45%) patients at 2 years post-Tx. All children had high dietary energy intakes. The degree of steatorrhoea was fairly constant, with fat and energy absorption rates of 84-89%. Growth parameters revealed at transplantation a mean height Z-score of -1.17. After Tx, two-thirds of children had normal growth, whereas in one-third, Z-scores remained lower than -2, concomitant to a delayed puberty. Adult height was normal in 5/6. Endoscopy and histology analyses were normal in asymptomatic patients. Chronic rejection occurred only in non-compliant patients. Five intestinal grafts were removed 2.5-8 years post-Tx for acute or chronic rejection.
This series indicates that long-term intestinal autonomy for up to 18 years is possible in the majority of patients after SBTx. Subnormal energy absorption and moderate steatorrhoea were often compensated for by hyperphagia, allowing normal growth and attainment of adult height. Long-term compliance is an important pre-requisite for long-term graft function.
小肠移植长期以来一直被视为治疗肠衰竭患者的一种挽救性疗法，如今已成为一种公认的替代肠外营养（PN）的治疗策略。在这项回顾性研究中，我们分析了31例小肠移植患儿的移植物功能，随访时间为2至18年（中位时间7年）。
12例患儿接受了单纯小肠移植，19例接受了肝小肠联合移植，17例还接受了结肠移植物。移植后每2至3年记录生长情况、营养指标、粪便平衡研究、内镜检查及移植物组织学检查结果。
所有患儿移植后均停用了肠外营养，26例患儿不再需要肠外营养。移植后2年时，14/31（45%）的患者需要肠内营养。所有患儿的膳食能量摄入量都很高。脂肪泻程度相当稳定，脂肪和能量吸收率为84%至89%。移植时生长参数显示平均身高Z值为-1.17。移植后，三分之二的患儿生长正常，而三分之一患儿的Z值仍低于-2，同时伴有青春期延迟。6例中有5例成年身高正常。无症状患者的内镜检查和组织学分析均正常。慢性排斥反应仅发生在依从性差的患者中。5例肠移植物在移植后2.5至8年因急性或慢性排斥反应被切除。
该系列研究表明，大多数小肠移植术后患者可实现长达18年的长期肠道自主功能。能量吸收不足和中度脂肪泻通常可通过多食得到补偿，从而实现正常生长和达到成年身高。长期依从性是移植物长期功能的重要前提条件。
