TY - JOUR
T1 - Classification of intestinal lymphangiectasia with protein-losing enteropathy
T2 - White villi type and non-white villi type
AU - Ohmiya, Naoki
AU - Nakamura, Masanao
AU - Yamamura, Takeshi
AU - Yamada, Koji
AU - Nagura, Asuka
AU - Yoshimura, Toru
AU - Hirooka, Yoshiki
AU - Hirata, Ichiro
AU - Goto, Hidemi
N1 - Publisher Copyright:
© 2014 S. Karger AG, Basel.
PY - 2014/12/25
Y1 - 2014/12/25
N2 - Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: 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. Results: 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). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response.
AB - Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: 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. Results: 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). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response.
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U2 - 10.1159/000365987
DO - 10.1159/000365987
M3 - Article
C2 - 25278259
AN - SCOPUS:84919828421
SN - 0012-2823
VL - 90
SP - 155
EP - 166
JO - Digestion
JF - Digestion
IS - 3
ER -