Exocrine Pancreatic Insufficiency is Undiagnosed in Some Patients with Diarrhea-Predominant Irritable Bowel Syndrome Using the Rome IV Criteria.

BACKGROUND AND AIMS: Irritable bowel syndrome (IBS) is one of the most frequent disorders in clinical practice, with a mean 7.6-10.8% worldwide prevalence. A study showed that 6.1% of patients with diarrhea-predominant IBS (IBS-D) had severe exocrine pancreatic insufficiency (EPI). We aimed to identify the prevalence of EPI based on fecal elastase stool testing (Fel-1) in IBS-D and the clinical characteristics that may predict the diagnosis of EPI. METHODS: Patients aged > 18 years presenting to tertiary hospital outpatient clinics with IBS-D completed validated questionnaires and gave a stool sample where Fel-1 concentration was measured. Patients with Fel-1 < 100 µg/g represented EPI and > 100 to < 200 µg/g underwent testing for pancreatic pathology with laboratory and endoscopic ultrasound (EUS) evaluation. RESULTS: One hundred forty patients (mean age 60 years, females 75.7%) were studied. EPI was found in 5% (95% CI 2.2-10.4), and pancreatic steatosis was the main EUS finding (71%). Dyspepsia was an independent factor associated with EPI (OR 34.7; 95% CI 4.95-366.37, p = 0.0007). After pancreatic enzyme replacement therapy (PERT), patients showed a significant improvement in the Bristol stool scale (p < 0.0001), bowel movements per day (p < 0.005), distension score (0.0009), pain score (0.0277) and IBS severity (0.0034). CONCLUSION: EPI is present in 5% of patients who fulfill Rome IV criteria for D-IBS, and dyspepsia was an independent symptom strongly associated with EPI. Pancreatic steatosis was the main endoscopic ultrasound finding. After PERT therapy, patients had significantly improved stool frequency, stool consistency, abdominal pain, distension and IBS severity score.

Prevalence of celiac disease: multicentric trial among pediatric population from five urban districts in Argentina.

INTRODUCTION: No published material on the prevalence of celiac disease (CD) in the pediatric population of Argentina has been found up to date. Objective. To estimate the prevalence of CD in a pediatric population (hospital-based sample) from 5 urban districts of Argentina. METHODS: In a cross-sectional descriptive study, we analyzed serum samples from 2219 children, aged 3-16 years old, which had been requested for pre-surgical tests and for physical aptitude certificates for sports in the province of Buenos Aires, and cities of Buenos Aires, Córdoba, Santa Fe and Salta. Children with a previous and accurate diagnosis of CD were also included. IgA class tissue transglutaminase antibodies were determined using serum samples, and those samples which turned out positive were also tested for IgA class endomysium antibodies. A small intestine biopsy was proposed for those who had a positive serology. RESULTS: Between May 2008 and August 2009, 29 positive serologies were found. A total of 22 duodenum biopsies were performed, and 21 turned out compatible with CD. Out of 2219 children, 7 had a previous diagnosis. A prevalence of 1.26% (1:79 children), with female gender predominance (p < 0.023) was found. Ninety percent of the celiac children were over 6 years old (p < 0.021). Silent celiac disease predominated but there was a 33% of symptomatic cases. CONCLUSIONS: The results of the trial show a higher prevalence of CD than expected. The finding of symptomatic patients (33%) suggests the undertaking of different activities to spread the knowledge on this disease and promote the indication for serology test, to avoid complications by means of an early diagnosis.

Prevalence of celiac disease: multicentric trial among pediatric population from five urban districts in Argentina / Prevalencia de enfermedad celíaca: estudio multicéntrico en población pediátrica de cinco distritos urbanos de la Argentina

Introduction. No published material on the preva lence of celiac disease (CD) in the pediatric population of Argentina has been found up to date. Objective. To estimate the prevalence of CD in a pediatric population (hospital-based sample) from 5 urban districts of Argentina. Methods. In a cross-sectional descriptive study, we analyzed serum samples from 2219 children, aged 3-16 years old, which had been requested for pre-surgical tests and for physical aptitude certifcates for sports in the province of Buenos Aires, and cities of Buenos Aires, Córdoba, Santa Fe and Salta. Children with a previous and accurate diagnosis of CD were also included. IgA class tissue transglu taminase antibodies were determined using serum samples, and those samples which turned out positive were also tested for IgA class endomysium an tibodies. A small intestine biopsy was proposed for those who had a positive serology. Results. Between May 2008 and August 2009, 29 positive serologies were found. A total of 22 duo denum biopsies were performed, and 21 turned out compatible with CD. Out of 2219 children, 7 had a previous diagnosis. A prevalence of 1.26% (1:79 children), with female gender predominance (p < 0.023) was found. Ninety percent of the celiac children were over 6 years old (p < 0.021). Silent celiac disease predominated but there was a 33% of symptomatic cases. Conclusions. The results of the trial show a higher prevalence of CD than expected. The finding of symptomatic patients (33%) suggests the undertak ing of different activities to spread the knowledge on this disease and promote the indication for serology test, to avoid complications by means of an early diagnosis.

Introducción. Hasta la fecha del estudio no se hallaron estudios poblacionales publicados sobre prevalencia de enfermedad celíaca en la población pediátrica argentina. Objetivo. Estimar la prevalencia de la enfermedad celíaca en población pediátrica a partir de una muestra de base hospitalaria de cinco distritos urbanos. Método. Diseño descriptivo de corte transversal. Bajo consentimiento informado, participaron 2219 niños, de 3 a 16 años, que realizaban estudios de laboratorio para exámenes prequirúrgicos o certificados de aptitud física deportiva del Conurbano bonaerense, y ciudades de Buenos Aires, Santa Fe, Córdoba y Salta. Se incluyeron niños con diagnóstico previo y certero de enfermedad celíaca dentro de esa población. Se determinaron anticuerpos antitransglutaminasa y, en las muestras positivas, anticuerpo antiendomisio. Se propuso biopsia de intestino delgado a quienes presentaron ambas serologías positivas. Resultados: 29 serologías fueron positivas. Se realizaron 22 biopsias de duodeno, 21 fueron compatibles con enfermedad celíaca y 7 presentaron diagnóstico previo. La prevalencia fue de 1,26% (1:79) IC 95% 0,84-1,81, con predominio del sexo femenino (p <0,039). El 90% de los niños celíacos hallados fueron mayores de 6 años. Las formas clínicas silentes predominaron, pero hubo un 33% de casos sintomáticos. Conclusión. Los resultados en la población estudiada muestran una prevalencia mayor que la esperada. El hallazgo de formas sintomáticas (33%) sugiere emprender acciones de difusión del conocimiento de la enfermedad y ampliar la indicación de serología para obtener diagnóstico precoz.

Prevalence of celiac disease: multicentric trial among pediatric population from five urban districts in Argentina / Prevalencia de enfermedad celíaca: estudio multicéntrico en población pediátrica de cinco distritos urbanos de la Argentina

Introduction. No published material on the preva lence of celiac disease (CD) in the pediatric population of Argentina has been found up to date. Objective. To estimate the prevalence of CD in a pediatric population (hospital-based sample) from 5 urban districts of Argentina. Methods. In a cross-sectional descriptive study, we analyzed serum samples from 2219 children, aged 3-16 years old, which had been requested for pre-surgical tests and for physical aptitude certifcates for sports in the province of Buenos Aires, and cities of Buenos Aires, Córdoba, Santa Fe and Salta. Children with a previous and accurate diagnosis of CD were also included. IgA class tissue transglu taminase antibodies were determined using serum samples, and those samples which turned out positive were also tested for IgA class endomysium an tibodies. A small intestine biopsy was proposed for those who had a positive serology. Results. Between May 2008 and August 2009, 29 positive serologies were found. A total of 22 duo denum biopsies were performed, and 21 turned out compatible with CD. Out of 2219 children, 7 had a previous diagnosis. A prevalence of 1.26% (1:79 children), with female gender predominance (p < 0.023) was found. Ninety percent of the celiac children were over 6 years old (p < 0.021). Silent celiac disease predominated but there was a 33% of symptomatic cases. Conclusions. The results of the trial show a higher prevalence of CD than expected. The finding of symptomatic patients (33%) suggests the undertak ing of different activities to spread the knowledge on this disease and promote the indication for serology test, to avoid complications by means of an early diagnosis.(AU)

Introducción. Hasta la fecha del estudio no se hallaron estudios poblacionales publicados sobre prevalencia de enfermedad celíaca en la población pediátrica argentina. Objetivo. Estimar la prevalencia de la enfermedad celíaca en población pediátrica a partir de una muestra de base hospitalaria de cinco distritos urbanos. Método. Diseño descriptivo de corte transversal. Bajo consentimiento informado, participaron 2219 niños, de 3 a 16 años, que realizaban estudios de laboratorio para exámenes prequirúrgicos o certificados de aptitud física deportiva del Conurbano bonaerense, y ciudades de Buenos Aires, Santa Fe, Córdoba y Salta. Se incluyeron niños con diagnóstico previo y certero de enfermedad celíaca dentro de esa población. Se determinaron anticuerpos antitransglutaminasa y, en las muestras positivas, anticuerpo antiendomisio. Se propuso biopsia de intestino delgado a quienes presentaron ambas serologías positivas. Resultados: 29 serologías fueron positivas. Se realizaron 22 biopsias de duodeno, 21 fueron compatibles con enfermedad celíaca y 7 presentaron diagnóstico previo. La prevalencia fue de 1,26% (1:79) IC 95% 0,84-1,81, con predominio del sexo femenino (p <0,039). El 90% de los niños celíacos hallados fueron mayores de 6 años. Las formas clínicas silentes predominaron, pero hubo un 33% de casos sintomáticos. Conclusión. Los resultados en la población estudiada muestran una prevalencia mayor que la esperada. El hallazgo de formas sintomáticas (33%) sugiere emprender acciones de difusión del conocimiento de la enfermedad y ampliar la indicación de serología para obtener diagnóstico precoz.(AU)

Prevalencia de enfermedad celíaca: estudio multicéntrico en población pediátrica en cinco distritos urbanos de Argentina / Prevalence of celiac disease: multicentric trial among pediatric population in five urban districts of Argentina

INTRODUCCIÓN: Hasta la fecha del estudio no se hallaron estudios poblacionales publicados sobre prevalencia de EC en población pediátrica argentina. OBJETIVO: estimar la prevalencia de la EC en población pediátrica de cinco distritos urbanos. MÉTODOS: diseño descriptivo de corte transversal. Se invitó a participar a 2.230 niños, de 3 a 16 años, que realizaban estudios de laboratorio para exámenes prequirúrgicos o certificados de aptitud física deportiva. Se determinaron Anticuerpos Antitransglutaminasa, y en las muestras positivas Anticuerpo Antiendomisio. Se propuso biopsia de intestino delgado a quienes presentaron ambas serologías positivas. Se incluyeron niños con diagnóstico previo de EC que cumplían los criterios de inclusión. La prevalencia se expresó mediante el porcentajey su IC exacto. Las comparaciones entre grupos se efectuaron mediante la prueba exacta de Fisher. RESULTADOS: se testearon los sueros de 2.219 niños, 29 serologías fueron positivas. Se realizaron 22 biopsias de duodeno, 21 fueron compatibles con EC y 7 presentaron diagnóstico previo. La prevalencia fue de 1,26% (1:79)IC 95% 0,84-1.81, con predominio del sexo femenino (p<0,039) e importantes diferencias regionales. El 90% de los niños celíacos hallados fueron > 6 años. Las formas clínicas silentes predominaron pero hubo un 33% de casos sintomáticos. CONCLUSIÓN: los resultados en la población estudiada muestran una prevalencia mayor que estudios previos en adultos. El hallazgo de formas sintomáticas (33%) sugiere emprender acciones de difusión del conocimientode la enfermedad y ampliar la indicación de serología para obtener diagnóstico precoz.

INTRODUCTION: up the date of this study, published material about the prevalence of CD in pediatric population in Argentina has not been found. OBJECTIVE: to estimate CD prevalencein a pediatric population of 5 urban districts. METHODS: descriptive and cross sectional cut design. Were invited 2.230 children, between 3 and 16 years, which had been requested forpre-surgical studies and physical aptitude certificates for sports. IgA class tissue transglutaminase antibodies were determined and to positive samples IgA class endomysium antibodies. A small intestine biopsy was proposed for those who had both positive serology. Children with a previous diagnosis of CD who met the inclusion criteria were included. The prevalence was expressed by means of the percentage and its exact confidence interval and the comparisons between groups were performed using Fisher´s exact test. RESULTS: 2.219 children´s sera were studied. 29 were positive serologies. 22 duodenum biopsies were performed, 21 turned out compatible with CD. 7 children presented a previous diagnosis. A prevalence of 1.26% (1:79) CI 95% (0,84-1,81) was found, with female sex predominance ( p<0.039) and important regional differences as well. Ninety percent of the celiac children found were> 6 years Silent clinical manifestations predominated but there were 33% of symptomatic cases. CONCLUSIONS: the results in the study population showed a higher prevalence than previous studies in adults. The finding of symptomatic manifestations (33%)suggests actions to spread the knowledge of this disease promotingthe indication of serology for early diagnosis.

Low-dose aspirin affects the small bowel mucosa: results of a pilot study with a multidimensional assessment.

BACKGROUND & AIMS: Whether low-dose aspirin (acetylsalicylic acid [ASA]) produces intestinal damage is controversial. Our aim was to determine whether the small bowel is damaged by low-dose ASA on a short-term basis. METHODS: Twenty healthy volunteers (age range, 19-64 years) underwent video capsule endoscopy (VCE), fecal calprotectin, and permeability tests (sucrose and lactulose/mannitol [lac/man] ratio) before and after ingestion of 100 mg of enteric-coated ASA daily for 14 days. Video capsule images were assessed by 2 independent expert endoscopists, fully blinded to the treatment group, by using an endoscopic scale. RESULTS: Post-ASA VCE detected 10 cases (50%) with mucosal damage not apparent in baseline studies (6 cases had petechiae, 3 had erosions, and 1 had bleeding stigmata in 2 ulcers). The median baseline lac/man ratio (0.021; range, 0.011-0.045) increased after ASA use (0.036; range, 0.007-0.258; P = .08), and the post-ASA lac/man ratio was above the upper end of normal (>0.025) in 10 of 20 volunteers (vs baseline, P < .02). The median baseline fecal calprotectin concentration (6.05 microg/g; range, 1.9-79.2) also increased significantly after ASA use (23.9 microg/g; range, 3.1-75.3; P < .0005), with 3 patients having values above the cutoff (>50 microg/g). Five of 10 subjects with abnormal findings at VCE also had lac/man ratios above the cutoff. Median baseline sucrose urinary excretion (70.0 mg; range, 11.8-151.3) increased significantly after ASA administration (107.0 mg; range, 22.9-411.3; P < .05). CONCLUSIONS: The short-term administration of low-dose ASA is associated with mucosal abnormalities of the small bowel mucosa, which might have implications in clinical practice.

Celiac disease screening by immunochromatographic visual assays: results of a multicenter study.

OBJECTIVE: To assay the efficiency for celiac disease (CD) screening of 2 immunochromatographic visual stick assays based on human recombinant tissue transglutaminase (tTG). One was the antitissue transglutaminase antibodies (AtTGA) stick for IgA/G antibodies to tTG detection, the other was the AtTGA/antigliadin antibodies (AGA) stick for IgA antibodies for tTG and/or gliadins. PATIENTS AND METHODS: In a prospective multicenter study, 4 pediatric gastroenterology units from Spain and 2 from Latin America enrolled 72 control children with a normal small bowel mucosa and 113 untreated patients with CD with Marsh type 3 lesions. RESULTS: Evaluation of results by the gastroenterologists and by 2 independent observers at the coordination center showed a remarkably low interobserver variability. For the AtTGA stick, sensitivity was 96.5% and specificity was 98.6%. The AtTGA/AGA stick displayed a sensitivity of 94.5% and a specificity of 98.6% for AtTGA and a sensitivity of 63.1% and a specificity of 95.2% for AGA. The highest efficiency and positive likelihood ratio was obtained for the AtTGA stick, higher than for IgA AtTGA by enzyme-linked immunosorbent assay. One additional advantage was that previous investigation of total serum IgA levels could be eluded. The IgA AtTGA/AGA stick, with an efficiency of 95.1%, compared with 89.2% when the combined results of the 2 enzyme-linked immunosorbent assays were considered, turned out to be an excellent diagnostic tool for infants with no IgA deficiency. CONCLUSION: These 2 assays are extremely efficient for CD screening, by combining a high diagnostic accuracy with the simplicity and rapidity of visual methods.

Clinical characteristics and long-term outcome of patients with refractory sprue diagnosed at a single institution.

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 (78% and 66%). Survivors had a favorable outcome. While 11 patients persists asymptomatic, two other cases still have mild diarrhea and one low body weight. CONCLUSIONS: We confirm that RS is a severe celiac disease-related disorder with very high mortality. Diagnosis of overt lymphoma (12%) in our long-term follow-up was not as frequent as was reported by other groups. A proportion of patients persist in good health for a long time irrespective of the nature of the IEL infiltration or the presence of UJ.

Clinical characteristics and long-term outcome of patients with refractory sprue diagnosed at a single institution / Características clínicas y evolución a largo plazo de una serie de pacientes con sprue refractario diagnosticados en una sola institución

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...

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)...

Clinical characteristics and long-term outcome of patients with refractory sprue diagnosed at a single institution

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)

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)

Clinical characteristics and long-term outcome of patients with refractory sprue diagnosed at a single institution

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)

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)

Clinical characteristics and long-term outcome of patients with refractory sprue diagnosed at a single institution.

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 (78

and 66

). Survivors had a favorable outcome. While 11 patients persists asymptomatic, two other cases still have mild diarrhea and one low body weight. CONCLUSIONS: We confirm that RS is a severe celiac disease-related disorder with very high mortality. Diagnosis of overt lymphoma (12

) in our long-term follow-up was not as frequent as was reported by other groups. A proportion of patients persist in good health for a long time irrespective of the nature of the IEL infiltration or the presence of UJ.

Enfermedad celíaca asociada con diabetes mellitus insulinodependiente / Coeliac disease associated with insulin dependent diabetes mellitus

Con el propósito de establecer la prevalencia de enfermedad celíaca (EC) asintomática u oligosintomática en niños que padecían diabetes mellitus insulinodependiente (DMID) efectuamos un rastreo en una de sus visitas de rutina durante el año 1991. Material y métodos: La detección de EC se realizó mediante la determinación de anticuerpos antigliadina clase IgA (AGA) por inmunoenzimoanálisis (ELISA). A los niños con AGA superior al valor de corte (25 U) se les realizó biopsia peroral de intestino delgado considerándose compatible con EC la presencia de atrofia vellositaria subtotal o total (atrofia grados III-IV). Resultados: Trece pacientes (30,2 por ciento) mostraron AGA elevados y cuatro, biopsias positivas. Considerando la existencia de dos pacientes con diagnóstico previo de EC, la prevalencia obtenida fue de 13,9 por ciento. Conclusiones: Este estudio confirma la asociación frecuente de enfermedad celíaca y diabetes insulinodependiente, obteniéndose una prevalencia de 13,9 por ciento

Enfermedad celíaca asociada con diabetes mellitus insulinodependiente / Coeliac disease associated with insulin dependent diabetes mellitus

Con el propósito de establecer la prevalencia de enfermedad celíaca (EC) asintomática u oligosintomática en niños que padecían diabetes mellitus insulinodependiente (DMID) efectuamos un rastreo en una de sus visitas de rutina durante el año 1991. Material y métodos: La detección de EC se realizó mediante la determinación de anticuerpos antigliadina clase IgA (AGA) por inmunoenzimoanálisis (ELISA). A los niños con AGA superior al valor de corte (25 U) se les realizó biopsia peroral de intestino delgado considerándose compatible con EC la presencia de atrofia vellositaria subtotal o total (atrofia grados III-IV). Resultados: Trece pacientes (30,2 por ciento) mostraron AGA elevados y cuatro, biopsias positivas. Considerando la existencia de dos pacientes con diagnóstico previo de EC, la prevalencia obtenida fue de 13,9 por ciento. Conclusiones: Este estudio confirma la asociación frecuente de enfermedad celíaca y diabetes insulinodependiente, obteniéndose una prevalencia de 13,9 por ciento (AU)

Colitis ulcerosa en niños: niveles de salazosulfapiridina y sulfapiridina durante el tratamiento / Ulcerative colitis in children: levels of salicylazosulfapyridine and sulfapyridine during the treatment

La salazosukfapiridina (SASP) tiene una acción efectiva en la colitis ulcerosa (CU), al desdoblarse en el ciego en sulfapiridina (SP) y 5 aminosalicilato (5 ASA), siendo este último el que actúa por contacto sobre la mucosa colónica. El objetivo fué conocer los niveles séricos efectivos de la droga con eventual presencia o no de efectos colaterales, y también verificar fluctuaciones de los mismos en el intervalo de dosificación. Se estudiaron 10 niños de 6 a 16 años con CU, a quienes se les suministraba SASP de 0.50 a 2 gramos por día y cada 12 horas. Los niveles de SASP y SP en sangre se realizaron a las 6 y 12 horas de administrada la droga, y en materia fecal se cuantificó en recolección de 24 horas. Estos dosajes se realizaron por el método de Hansson y Sandberg. Los niveles lasmáticos de SP fueron a las 6 horas de 6.8 a 36.3 microng/ml (x 17.7-9.0 microng/ml) y a las 12 horas de 5.7 a 25.0 microng/ml (x 14.1 -7.2 microng/ml los de SASP fueron de 2.1 a 53.4 microng/ml (x 15.5-15.4microng/ml) a las 6 horas, y 3.9 a 70.7 microng/ml (x 14.0 -20.4 microng/ml) a las 12 horas. La excreción en materia fecal de SASP durante 24 horas fué de 17.4 a 236 mg., observándo-se una correlación significativa (r: 0.88) con la dosis administrada calculada en gramos por metro cuadrado de superficie corporal en 24 horas. Los niveles de SP y SASP no se correlacionaron con la dosis. Los niveles plasmáticos de SASp y SP no tuvieron diferencia significativa entre las 6 y 12 horas. Los niveles de SP no alcanzaron los mínimos ee toxicidad, no detectándose manifestaciones clíncias ni humorales adversas. Estos hallazgos sugieren que conociendo los niveles en plasma de la droga, la dosis puede ser modificada con mayor amplitud

Colitis ulcerosa en niños: niveles de salazosulfapiridina y sulfapiridina durante el tratamiento / Ulcerative colitis in children: levels of salicylazosulfapyridine and sulfapyridine during the treatment

La salazosukfapiridina (SASP) tiene una acción efectiva en la colitis ulcerosa (CU), al desdoblarse en el ciego en sulfapiridina (SP) y 5 aminosalicilato (5 ASA), siendo este último el que actúa por contacto sobre la mucosa colónica. El objetivo fué conocer los niveles séricos efectivos de la droga con eventual presencia o no de efectos colaterales, y también verificar fluctuaciones de los mismos en el intervalo de dosificación. Se estudiaron 10 niños de 6 a 16 años con CU, a quienes se les suministraba SASP de 0.50 a 2 gramos por día y cada 12 horas. Los niveles de SASP y SP en sangre se realizaron a las 6 y 12 horas de administrada la droga, y en materia fecal se cuantificó en recolección de 24 horas. Estos dosajes se realizaron por el método de Hansson y Sandberg. Los niveles lasmáticos de SP fueron a las 6 horas de 6.8 a 36.3 microng/ml (x 17.7-9.0 microng/ml) y a las 12 horas de 5.7 a 25.0 microng/ml (x 14.1 -7.2 microng/ml los de SASP fueron de 2.1 a 53.4 microng/ml (x 15.5-15.4microng/ml) a las 6 horas, y 3.9 a 70.7 microng/ml (x 14.0 -20.4 microng/ml) a las 12 horas. La excreción en materia fecal de SASP durante 24 horas fué de 17.4 a 236 mg., observándo-se una correlación significativa (r: 0.88) con la dosis administrada calculada en gramos por metro cuadrado de superficie corporal en 24 horas. Los niveles de SP y SASP no se correlacionaron con la dosis. Los niveles plasmáticos de SASp y SP no tuvieron diferencia significativa entre las 6 y 12 horas. Los niveles de SP no alcanzaron los mínimos ee toxicidad, no detectándose manifestaciones clíncias ni humorales adversas. Estos hallazgos sugieren que conociendo los niveles en plasma de la droga, la dosis puede ser modificada con mayor amplitud (AU)