title: Classification of intestinal lymphangiectasia with protein-losing enteropathy: white villi type and non-white villi type.
abstract: OBJECTIVE
  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).
  
  
  CONCLUSIONS
  Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response.
authors.affiliation: Department of Gastroenterology, Fujita Health University School of Medicine, Toyoake, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Ohmiya
authors.forename: Masanao
authors.initials: M
authors.lastname: Nakamura
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yamamura
authors.forename: Koji
authors.initials: K
authors.lastname: Yamada
authors.forename: Asuka
authors.initials: A
authors.lastname: Nagura
authors.forename: Toru
authors.initials: T
authors.lastname: Yoshimura
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Hirooka
authors.forename: Ichiro
authors.initials: I
authors.lastname: Hirata
authors.forename: Hidemi
authors.initials: H
authors.lastname: Goto
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D058582:Double-Balloon Enteroscopy; D004378:Duodenal Diseases / Q000097:blood / Q000145:classification / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D005243:Feces / Q000737:chemistry; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007579:Jejunal Diseases / Q000097:blood / Q000145:classification / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D008201:Lymphangiectasis, Intestinal / Q000097:blood / Q000145:classification / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D011504:Protein-Losing Enteropathies / Q000097:blood / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D000515:alpha 1-Antitrypsin / Q000032:analysis
chemical_list: D005938:Glucocorticoids; D000515:alpha 1-Antitrypsin; D011239:Prednisolone