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Clinical characteristics and long-term outcome of patients with refractory sprue diagnosed at a single institution
Mauriño, Eduardo; Niveloni, Sonia; Cherñavsky, Alejandra C; Sugai, Emilia; Vázquez, Horacio; Pedreira, Silvia; Periolo, Natalia; Mazure, Roberto; Smecuol, Edgardo; Moreno, María L; Litwin, Néstor; Nachman, Fabio; Kogan, Zulema; Bai, Julio César.
Affiliation
  • Mauriño, Eduardo; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Niveloni, Sonia; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Cherñavsky, Alejandra C; Universidad de Buenos Aires. Hospital de Clínicas José de San Martín. Department of Immunogenetics. Buenos Aires. Argentina
  • Sugai, Emilia; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Vázquez, Horacio; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Pedreira, Silvia; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Periolo, Natalia; Universidad de Buenos Aires. Hospital de Clínicas José de San Martín. Department of Immunogenetics. Buenos Aires. Argentina
  • Mazure, Roberto; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Smecuol, Edgardo; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Moreno, María L; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Litwin, Néstor; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Nachman, Fabio; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Kogan, Zulema; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
  • Bai, Julio César; Carlos Bonorino Udaondo Gastroenterology Hospital. Service of Pathology. Department of Medicine. Small Bowel Section. s.p
Acta gastroenterol. latinoam ; 36(1): 10-22, mar. 2006. tab, graf
Article in English | BINACIS | ID: bin-123355
Responsible library: BR1.1
ABSTRACT

BACKGROUND:

Refractory sprue (RS) is a rare and severe celiac-like enteropathy not responding to a strict gluten-free diet. Although prognosis is generally poor, little is known about the long-term outcome of patients.

AIM:

to report baseline characteristics and long-term outcome of a series of patients diagnosed and treated in a single institution. MATERIALS We report a retrospective cohort of 25 consecutive patients (15 females; mean age 46 yr; range 28-71) diagnosed with RS based on the presence of a non-responsive celiac-like enteropathy. All patients were intensively treated with a gluten-free diet, steroids, nutritional support and immunosupression.

RESULTS:

Clinical and biological characteristics of patients suggest that, at least, 24 patients had clear evidences of celiac disease. HLA DQ2/DQ8 genes were present in all the 24 patients typed and autoimmune enteropathy was excluded in all. According to the genotyping, 12 patients had a polyclonal lymphocyte population (RS type I) and 13 exhibited monoclonal TCR-gamma gene rearrangements (RS type II). Sixteen patients had evidence of ulcerative jejunitis (UJ) (7 in RS type I and 9 in type II). Overall median follow-up time after diagnosis of RS was 29 mo/patient (range 7 to 204) (45 mo for type I and 24 mo for type II). Overall mortality was 48% (12 patients), 6 in each type. Eight patients with UJ (50%), 3 with lymphoma (two T-cell and one B-cell type) and 4 (44%) without ulcers died during follow-up. The causes of death were sepsis in the context of a progressive deterioration but without overt malignancies (n=5), vascular causes (n=3) and severe malnutrition (n=1). Three- and 5-yr survival rate after diagnosis of RS for the overall population was 60% and 56%. There was no differences between type I (67%, 58%) and type II RS patients (54% for both periods). Patients with UJ had lower but non-significant 3- and 5-yr survival rates (56% and 50%, respectively) compared with patients without ulcers... (AU)
Subject(s)
Full text: Available Collection: National databases / Argentina Database: BINACIS Main subject: Celiac Disease Type of study: Diagnostic study / Prognostic study Limits: Adult / Female / Humans / Male Country/Region as subject: South America / Argentina Language: English Journal: Acta gastroenterol. latinoam Year: 2006 Document type: Article Institution/Affiliation country: Carlos Bonorino Udaondo Gastroenterology Hospital/s.p / Universidad de Buenos Aires/Argentina
Full text: Available Collection: National databases / Argentina Database: BINACIS Main subject: Celiac Disease Type of study: Diagnostic study / Prognostic study Limits: Adult / Female / Humans / Male Country/Region as subject: South America / Argentina Language: English Journal: Acta gastroenterol. latinoam Year: 2006 Document type: Article Institution/Affiliation country: Carlos Bonorino Udaondo Gastroenterology Hospital/s.p / Universidad de Buenos Aires/Argentina
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