Santos-Machado T M, Cristófani L M, Almeida M T, Maluf P T, Costa P A, Pereira M A, Brito J L, Odone-Filho V
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Braz J Med Biol Res. 1999 Sep;32(9):1095-9. doi: 10.1590/s0100-879x1999000900007.
Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%), protein-losing enteropathy (33.3%) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.
胃肠道（GI）受累作为朗格汉斯细胞组织细胞增多症（LCH）的突出症状并不常见，在所有病例中发生率低于1%至5%，即使疾病处于播散型时也是如此。到目前为止，已有18例LCH伴有胃肠道表现的报道，包括我们的2例，其中腹泻（77.7%）、蛋白丢失性肠病（33.3%）和便血是最常见的表现。作者报告了2例严重腹泻和难治性低蛋白血症患者，并通过铬51标记白蛋白研究证实存在蛋白丢失性肠病。文献综述表明，胃肠道症状的出现通常与全身性疾病以及预后不良相关，主要发生在2岁以下儿童。放射性同位素对于记录多种疾病中的蛋白丢失具有高特异性和敏感性，在此回顾的病例中使用放射性同位素使得6名儿童得以确诊，并改善了治疗管理。
