Gambarara M, Diamanti A, Ferretti F, Papadatou B, Knafelz D, Pietrobattista A, Castro M
Gastroenterology Unit, Bambino Gesù Children Hospital, Piazza S. Onofrio 4, 00165 Rome, Italy.
Transplant Proc. 2003 Dec;35(8):3052-3. doi: 10.1016/j.transproceed.2003.10.053.
Microvillous inclusion disease (MID) and epithelial dysplasia (ED) or tufting enteropathy are the most frequent causes of intractable diarrhea with persistent villous atrophy and indefinite dependence on total parenteral nutrition (PN) from early infancy. Since these are intractable diseases, they have been proposed to be elective indication for early bowel transplantation in order to avoid complications, such as PN-related liver disease, that would require a combined small bowel-liver transplant. We describe four cases of intractable diarrhea, two with MID and two with ED, seeking to discover whether these diseases are really elective, early indications for bowel transplant. Among our four patients, only one with ED underwent transplantation. The prognosis of small bowel transplant is still poor and worse than that of prolonged HPN. Further study is necessary to achieve a safe HPN program. Referral for transplant (small bowel only or combined with liver) should be considered when there is a venous access reduction and/or severe and irreversible liver disease.
微绒毛包涵体病（MID）和上皮发育异常（ED）或簇状肠病是婴儿早期持续性绒毛萎缩和长期依赖全胃肠外营养（PN）导致顽固性腹泻的最常见原因。由于这些是难治性疾病，为避免诸如PN相关肝病等并发症（而这些并发症可能需要联合小肠-肝移植），它们被提议作为早期肠移植的选择性指征。我们描述了4例顽固性腹泻病例，其中2例为MID，2例为ED，旨在探究这些疾病是否真的是肠移植的选择性早期指征。在我们的4例患者中，只有1例ED患者接受了移植。小肠移植的预后仍然很差，比长期家庭肠外营养（HPN）的预后更差。有必要进行进一步研究以实现安全的HPN方案。当静脉通路减少和/或出现严重且不可逆的肝病时，应考虑转诊进行移植（仅小肠或联合肝脏）。
