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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.
Afiliação
  • 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
Artigo em Inglês | BINACIS | ID: bin-119487
Biblioteca responsável: BR1.1
Localização: 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)
RESUMEN

Introducción:

El sprue refractario (SR) es una rara y severa entidad consistente en una enteropatía tipo celíaca que no responde a una estricta dieta libre degluten. Aún cuando el pronóstico es generalmente pobre, poco es conocido acerca de la evolución de lospacientes a largo plazo.

Objetivo:

reportar las característicasclínicas y la evolución a largo plazo de una serie de pacientes diagnosticados y tratados en una solainstitución. Materiales Reportamos una cohorteretrospectiva de 25 pacientes consecutivos (15 mujeres; edad media 46 años; rango 28-71) diagnosticadoscomo SR sobre la base de una enteropatía tipo celíaca que no respondió a la dieta libre de gluten. Todos los pacientes recibieron un tratamiento intensivo consistenteen dieta libre de gluten, alimentación enteral o parenteral, corticosteroides e inmunosupresión.

Resultados:

Los elementos clínicos y biológicos sugierenque 24 pacientes exhibían claras evidencias de enfermedadcelíaca. Los genes HLA DQ2/DQ8 estuvieron presentes en los 24 pacientes estudiados y se excluyó laenteropatía autoinmune en todos los casos. De acuerdo al genotipo, 12 pacientes presentaron una poblaciónlinfocitaria intraepitelial policlonal (SR tipo I) y 13 exhibieron un rearreglo genético monoclonal del TCR-γ (SR tipo II). Dieciséis pacientes presentaron evidencias de yeyunitis ulcerativa (YU) (7 en SR tipo I y 9 enel tipo II). El tiempo promedio de seguimiento luego del diagnóstico de SR fue 29 meses/paciente (rango 7 -204) (45 y 24 meses para tipo I y tipo II, respectivamente). La mortalidad global fue del 48% (12 pacientes),6 en cada tipo de SR. Ocho pacientes con YU (50%) murieron durante el seguimiento, 3 con linfoma(dos de células T y uno de células B) y cuatro(44%) individuos sin úlceras también fallecieron. Lascausas de muerte fueron vasculares (n=3), sepsis en elmarco de deterioro progresivo sin desarrollo de malignidad(n=5) y desnutrición progresiva (n=1)...(AU)
Assuntos
Texto completo: Disponível Coleções: Bases de dados nacionais / Argentina Base de dados: BINACIS Assunto principal: Doença Celíaca Tipo de estudo: Estudo diagnóstico / Estudo prognóstico Limite: Adulto / Feminino / Humanos / Masculino País/Região como assunto: América do Sul / Argentina Idioma: Inglês Revista: Acta gastroenterol. latinoam Ano de publicação: 2006 Tipo de documento: Artigo Instituição/País de afiliação: Carlos Bonorino Udaondo Gastroenterology Hospital/s.p / Universidad de Buenos Aires/Argentina
Texto completo: Disponível Coleções: Bases de dados nacionais / Argentina Base de dados: BINACIS Assunto principal: Doença Celíaca Tipo de estudo: Estudo diagnóstico / Estudo prognóstico Limite: Adulto / Feminino / Humanos / Masculino País/Região como assunto: América do Sul / Argentina Idioma: Inglês Revista: Acta gastroenterol. latinoam Ano de publicação: 2006 Tipo de documento: Artigo Instituição/País de afiliação: Carlos Bonorino Udaondo Gastroenterology Hospital/s.p / Universidad de Buenos Aires/Argentina
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