Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type
2014; Karger Publishers; Volume: 90; Issue: 3 Linguagem: Inglês
10.1159/000365987
ISSN1421-9867
AutoresNaoki Ohmiya, Masanao Nakamura, Takeshi Yamamura, Kôji Yamada, Asuka Nagura, Toru Yoshimura, Yoshiki Hirooka, Ichiro Hirata, Hidemi Goto,
Tópico(s)Gastrointestinal disorders and treatments
ResumoWe classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses.Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy.Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal α1-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015).Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response.
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