title: Duodenal ulceration in a patient with celiac disease and plasminogen I deficiency: coincidence or cofactors?
abstract: Celiac disease (CD) is a gluten-dependent inflammatory disease of the small bowel that affects up to 1% of the worldwide population. Despite severe mucosal abnormalities including total villous atrophy and autoantibody deposition, duodenal ulcer is not a feature of CD. However, a recent study found an elevated rate of peptic ulcer disease in patients with CD. Plasminogen deficiency (PLD) is an autosomal recessive disease that causes pseudomembranous lesions in different organs, but gastrointestinal involvement is rare. Here we report the case of a 6-year-old girl who had a sudden onset of hematemesis caused by duodenal ulcer. On the basis of mucosal atrophy, elevated celiac antibody levels, decreased plasminogen serum activity, and homozygous missense mutation R216H in the plasminogen gene, CD and PLD were diagnosed. This report is, to our knowledge, the first description of the 2 entities, and results of our double-immunofluorescent studies also suggest that both diseases may have a role in the ulceration process. Excessive amounts of fibrin deposition due to PLD caused the distortion of the vessels and was responsible for the unusual celiac immunoglobulin A and tissue transglutaminase 2 in vivo binding pattern. On the basis of this result, patients with CD and unknown cause of gastrointestinal ulcer may require investigation for PLD.
authors.affiliation: First Department of Pediatrics, Semmelweis University, Budapest, Hungary. vergab@gyer1.sote.hu
authors.forename: Gabor
authors.initials: G
authors.lastname: Veres
authors.forename: Ilma
authors.initials: I
authors.lastname: Korponay-Szabó
authors.forename: Erika
authors.initials: E
authors.lastname: Maka
authors.forename: Tibor
authors.initials: T
authors.lastname: Glasz
authors.forename: Petar
authors.initials: P
authors.lastname: Mamula
authors.forename: Volker
authors.initials: V
authors.lastname: Schuster
authors.forename: Katrin
authors.initials: K
authors.lastname: Tefs
authors.forename: Volker
authors.initials: V
authors.lastname: Schuster
authors.forename: Katrin
authors.initials: K
authors.lastname: Tefs
authors.forename: Maria
authors.initials: M
authors.lastname: Papp
authors.forename: Antal
authors.initials: A
authors.lastname: Dezsöfi
authors.forename: Andras
authors.initials: A
authors.lastname: Arató
mesh_terms: D015746:Abdominal Pain / Q000175:diagnosis / Q000209:etiology; D001707:Biopsy, Needle; D001774:Blood Chemical Analysis; D002446:Celiac Disease / Q000150:complications* / Q000175:diagnosis* / Q000178:diet therapy; D002648:Child; D003677:Deficiency Diseases / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D055050:Diet, Gluten-Free; D004381:Duodenal Ulcer / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D004384:Duodenoscopy / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006396:Hematemesis / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D007150:Immunohistochemistry; D007413:Intestinal Mucosa / Q000473:pathology; D009853:Omeprazole / Q000627:therapeutic use; D010958:Plasminogen / Q000172:deficiency*; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: C000717608:TGM2 protein, human; D010958:Plasminogen; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D009853:Omeprazole