Mahjoub Fatemeh, Sani Mehri Najafi, Tabari Ahmad Khaleghnejad, Monajemzadeh Maryam, Zandieh Saeed
Department of Pathology, Valiasr Hospital, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran.
Department of Gastroenterology, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran.
European J Pediatr Surg Rep. 2014 Jun;2(1):29-31. doi: 10.1055/s-0033-1348040. Epub 2013 May 31.
Introduction Protein losing enteropathy is a symptom characterized by loss of protein in intestines resulting in low protein levels in serum and generalized edema. Several causes are reported for this condition. Hereby we report an as yet unreported cause of protein losing enteropathy that we named meso-intestinal fibrosis. Case Report A 2.5-year-old girl referred with features of partial intestinal obstruction and underwent laparotomy. She had history of protein losing enteropathy since 16 months of age with generalized edema and received albumin every other week. Workup of protein losing enteropathy was inconclusive and only a histology report denoted increase in eosinophils in lamina propria of small intestine and hypoallergenic diet was started for her, but no significant response was noted. Laparotomy revealed lace-like white areas in meso of small intestine and intestinal wall was firm in palpation in some areas. Biopsy was taken from these sites and histology revealed severe fibrosis of meso overlying muscularis propria and also patchy fibrosis of intestinal meso led to severe lymphangiectasis in submucosa of small intestine. Discussion Secondary lymphangiectasis due to obstruction of lymphatic flow is mentioned as cause of protein losing enteropathy. Meso-intestinal fibrosis seen in this case that led to secondary lymphangiectasis and also motility disorder has not been reported as yet.
引言 蛋白丢失性肠病是一种以肠道蛋白质丢失导致血清蛋白水平降低和全身性水肿为特征的症状。已报道了该病症的多种病因。在此，我们报告一种尚未报道的蛋白丢失性肠病病因，我们将其命名为中肠纤维化。病例报告 一名2.5岁女孩因部分肠梗阻症状前来就诊并接受了剖腹手术。她自16个月大起就患有蛋白丢失性肠病，伴有全身性水肿，每隔一周接受一次白蛋白治疗。对蛋白丢失性肠病的检查结果不明确，仅有一份组织学报告显示小肠固有层嗜酸性粒细胞增多，于是开始为她采用低敏饮食，但未观察到明显效果。剖腹手术显示小肠系膜有花边状白色区域，部分区域肠壁触诊质地坚硬。从这些部位取了活检，组织学显示固有肌层上方的系膜严重纤维化，小肠系膜的片状纤维化导致小肠黏膜下层严重淋巴管扩张。讨论 淋巴管阻塞导致的继发性淋巴管扩张被认为是蛋白丢失性肠病的病因。本病例中所见的导致继发性淋巴管扩张及动力障碍的中肠纤维化尚未见报道。
