La composición de la microbiota duodenal en niños con enfermedad celíaca activa está influenciada por el grado de enteropatía / The duodenal microbiota composition in children with active coeliac disease is influenced by the degree of enteropathy

OBJETIVOS: Comprobar diferencias en la microbiota duodenal al diagnóstico de la enfermedad celíaca (EC) en relación con un grupo control. MATERIAL Y MÉTODOS: Se obtuvieron muestras de biopsias duodenales en 11 pacientes con EC al diagnóstico y en 6 controles. Se analizó la microbiota duodenal total así como la perteneciente al género Lactobacillus mediante la técnica molecular PCR-electroforesis en gel con gradiente desnaturalizante (DGGE). Los patrones de bandas obtenidos en los geles resultantes fueron analizados para determinar las diferencias presentes entre la microbiota de pacientes con EC y de los controles (FPQuest 4.5), mientras que los índices ecológicos (riqueza, diversidad y habitabilidad) fueron calculados con el programa Past versión 2.17. RESULTADOS: La microbiota intestinal de los individuos con histología Marsh 3c presentó similitud del 98% y fue diferente del resto de pacientes celíacos. Las principales diferencias se obtuvieron en los índices ecológicos pertenecientes al género Lactobacillus, con importante reducción de especies en los celíacos respecto al grupo control (riqueza, diversidad y habitabilidad). En los pacientes con EC las bandas principalmente fueron catalogadas con las especies Streptococcus, Bacteroides y E.coli. En los controles las bandas predominantes fueron Bifidobacterium, Acinetobacter y Lactobacillus; sin embargo, los Streptococcus y Bacteroides fueron más bajos. CONCLUSIONES: Los índices ecológicos aplicados al género Lactobacillus fueron significativamente reducidos en los pacientes celíacos. Los casos con mayor afectación histológica presentaron una microbiota duodenal similar

OBJECTIVES: To establish whether the duodenal mucosa microbiota of children with active coeliac disease (CD) and healthy controls (HC) differ in composition and biodiversity. MATERIAL AND METHODS: Samples of duodenal biopsies in 11 CD patients were obtained at diagnosis, and in 6 HC who were investigated for functional intestinal disorders of non-CD origin. Total duodenal microbiota and the belonging to the genus Lactobacillus using PCR-denaturing gradient gel electrophoresis (DGGE) were analysed. The banding patterns obtained in the resulting gels were analysed to determine the differences between the microbiota of CD patients and HC (FPQuest 4.5) while environmental indexes (richness, diversity and habitability) were calculated with the Past version 2.17 program. RESULTS: The intestinal microbiota of patients with Marsh 3c lesion showed similarity of 98% and differs from other CD patients with other type of histologic lesion as Marsh3a, Marsh3b and Marsh2. The main differences were obtained in ecological indexes belonging to the genusLactobacillus, with significant richness, diversity and habitability reduction in CD patients. In CD bands were categorized primarily with Streptococcus, Bacteroides and E.coli species. In HC the predominant bands were Bifidobacterium, Lactobacillus and Acinetobacter, though theStreptococcus and Bacteroides were lower. CONCLUSIONS: The celiac patients with major histological affectation presented a similar microbiota duodenal. The ecological indexes applied to the genus Lactobacillus were significantly reduced in CD

[The duodenal microbiota composition in children with active coeliac disease is influenced by the degree of enteropathy]. / La composición de la microbiota duodenal en niños con enfermedad celíaca activa está influenciada por el grado de enteropatía.

OBJECTIVES: To establish whether the duodenal mucosa microbiota of children with active coeliac disease (CD) and healthy controls (HC) differ in composition and biodiversity. MATERIAL AND METHODS: Samples of duodenal biopsies in 11 CD patients were obtained at diagnosis, and in 6 HC who were investigated for functional intestinal disorders of non-CD origin. Total duodenal microbiota and the belonging to the genus Lactobacillus using PCR-denaturing gradient gel electrophoresis (DGGE) were analysed. The banding patterns obtained in the resulting gels were analysed to determine the differences between the microbiota of CD patients and HC (FPQuest 4.5) while environmental indexes (richness, diversity and habitability) were calculated with the Past version 2.17 program. RESULTS: The intestinal microbiota of patients with Marsh 3c lesion showed similarity of 98% and differs from other CD patients with other type of histologic lesion as Marsh3a, Marsh3b and Marsh2. The main differences were obtained in ecological indexes belonging to the genus Lactobacillus, with significant richness, diversity and habitability reduction in CD patients. In CD bands were categorized primarily with Streptococcus, Bacteroides and E.coli species. In HC the predominant bands were Bifidobacterium, Lactobacillus and Acinetobacter, though the Streptococcus and Bacteroides were lower. CONCLUSIONS: The celiac patients with major histological affectation presented a similar microbiota duodenal. The ecological indexes applied to the genus Lactobacillus were significantly reduced in CD.

La nutrición enteral exclusiva continua siendo el tratamiento de primera línea en la enfermedad de Crohn pediátrica en la era de los biológicos / Exclusive enteral nutrition continues to be first line therapy for pediatric Crohn's disease in the era of biologics

INTRODUCCIÓN: La nutrición enteral exclusiva (NEE) ha demostrado ser más efectiva que los esteroides para alcanzar la curación mucosa sin sus efectos secundarios. OBJETIVOS: Determinar la eficacia de la NEE para inducir la remisión clínica y mejorar el grado de inflamación mucosa en pacientes con EC durante su primer brote. MATERIAL Y MÉTODOS: Revisión de las historias clínicas de pacientes con EC tratados con NEE durante su primer brote. El grado de inflamación mucosa se estimó mediante la calprotectina fecal (CF). Se definió remisión como PCDAI < 10. RESULTADOS: Se incluyó a 40 pacientes (24 varones) con una edad al diagnóstico de 11,6 ± 3,6 años. La duración de la NEE fue de 6,42 semanas (RIC 6,0-8,14). De los 34 pacientes que completaron el período de NEE, 32 (94% en el análisis por protocolo) alcanzaron la remisión clínica. Este porcentaje descendió al 80% en el análisis por intención de tratar. La tasa de cumplimiento fue del 95%. Los valores de CF fueron significativamente más altos en pacientes con brotes moderados y graves. La CF basal fue de 680 μg/g y descendió de forma significativa a 218 μg/g al final del periodo de NEE (p < 0,0001). Hubo correlación estadísticamente significativa entre CF y PCDAI (rho = 0,727; p < 0,0001). La introducción precoz del tratamiento con tiopurinas (antes de las 8 semanas) no se asoció a una mejor evolución durante el seguimiento. CONCLUSIONES: La NEE administrada durante 6-8 semanas es efectiva para inducir la remisión clínica y mejorar el grado de inflamación mucosa. No encontramos diferencias en términos de mantenimiento de la remisión en pacientes tratados precozmente con tiopurinas

INTRODUCTION: Exclusive enteral nutrition (EEN) has been to be more effective than corticosteroids in achieving mucosal healing without their side effects. OBJECTIVES: To determine the efficacy of EEN in terms of inducing clinical remission in newly diagnosed CD children and to study the efficacy of this therapeutic approach in improving the degree of intestinal mucosa inflammation. MATERIALS AND METHODS: The medical records of patients with newly diagnosed Crohn's disease treated with EEN were reviewed retrospectively. The degree of mucosal inflammation was assessed by fecal calprotectin (FC). Remission was defined as a PCDAI < 10. RESULTS: Forty patients (24 males) were included, the age at diagnosis was 11.6 ± 3.6 years. Of the 34 patients who completed the EEN period, 32 (94% per-protocol analysis) achieved clinical remission. This percentage fell to 80% in the intention-to-treat analysis. The compliance rate was 95%. Duration of EEN was 6.42 weeks (IQR 6.0-8.14). FC was significantly higher in patients with moderate and severe disease. Median baseline FC levels (680 μg/g) decreased significantly to 218 μg/g (P < 0.0001) after EEN. We found a statistically significant correlation between FC and PCDAI (rho = 0.727; P < 0.0001). Early use of thiopurines (< 8 weeks) versus subsequent use was not associated with improved outcomes during the follow-up. CONCLUSIONS: EEN administered for 6-8 weeks is effective for inducing clinical remission and decreasing the degree of mucosal inflammation. We did not find differences in terms of maintenance of remission in patients treated early with thiopurines

[Exclusive enteral nutrition continues to be first line therapy for pediatric Crohn's disease in the era of biologics]. / La nutrición enteral exclusiva continua siendo el tratamiento de primera línea en la enfermedad de Crohn pediátrica en la era de los biológicos.

INTRODUCTION: Exclusive enteral nutrition (EEN) has been to be more effective than corticosteroids in achieving mucosal healing without their side effects. OBJECTIVES: To determine the efficacy of EEN in terms of inducing clinical remission in newly diagnosed CD children and to study the efficacy of this therapeutic approach in improving the degree of intestinal mucosa inflammation. MATERIALS AND METHODS: The medical records of patients with newly diagnosed Crohn's disease treated with EEN were reviewed retrospectively. The degree of mucosal inflammation was assessed by fecal calprotectin (FC). Remission was defined as a PCDAI<10. RESULTS: Forty patients (24 males) were included, the age at diagnosis was 11.6 ± 3.6 years. Of the 34 patients who completed the EEN period, 32 (94% per-protocol analysis) achieved clinical remission. This percentage fell to 80% in the intention-to-treat analysis. The compliance rate was 95%. Duration of EEN was 6.42 weeks (IQR 6.0-8.14). FC was significantly higher in patients with moderate and severe disease. Median baseline FC levels (680 µg/g) decreased significantly to 218 µg/g (P<0.0001) after EEN. We found a statistically significant correlation between FC and PCDAI (rho=0.727; P<0.0001). Early use of thiopurines (< 8 weeks) versus subsequent use was not associated with improved outcomes during the follow-up. CONCLUSIONS: EEN administered for 6-8 weeks is effective for inducing clinical remission and decreasing the degree of mucosal inflammation. We did not find differences in terms of maintenance of remission in patients treated early with thiopurines.

Oral tacrolimus for pediatric steroid-resistant ulcerative colitis.

BACKGROUND: Ulcerative colitis (UC) occurring during childhood is generally extensive and is associated with severe flares that may require intravenous steroid treatment. In cases of corticosteroid resistance is necessary to introduce a second-line treatment to avoid or delay surgery. AIMS: To describe the efficacy and safety of oral tacrolimus for the treatment of severe steroid-resistant UC. METHODS: We performed a retrospective study that included all patients under age 18 suffering from severe steroid-resistant UC treated with oral tacrolimus during the period January 1998 to October 2012 and with a follow-up period after treatment of 24 months or more. RESULTS: A total of ten patients were included. The age at baseline was 9.4±4.9 years, and the time from diagnosis was 1.3 months (IQR, 1-5.7). Seven of the patients were in their first flare of disease. All of them received an oral dose of 0.12 mg/kg/day of tacrolimus divided in two doses. Trough plasma levels of tacrolimus were maintained between 4 and 13 ng/ml. Response was seen in 5/10 patients at 12 months, colectomy was eventually performed in 60% of patients during the follow-up period. CONCLUSIONS: Tacrolimus is useful in inducing remission in patients with severe steroid-resistant UC, preventing or delaying colectomy, and allowing the patient and family to prepare for a probable surgery. Tacrolimus may also be used as a treatment bridge for corticosteroid-dependent patients until the new maintenance therapy takes effect.

Estudio clínico-radiológico del síndrome de Sturge-Weber / Clinical-radiological study of Sturge-Weber syndrome

El síndrome de Sturge-Weber es una enfermedadneuroectodérmica congénita rara que se caracterizapor la existencia de malformaciones vasculares enla piel de la cara, las leptomeninges de la cortezacerebral y la coroides ocular.El curso clínico es variable pero la mayoría de losniños tienen epilepsia y deterioro neurológico conatrofia cerebral.Los estudios de imagen (tanto la tomografía computarizadacomo la resonancia magnética) confirmanel diagnóstico y sirven para evaluar la extensiónde la afectación intracraneal

Sturge-Weber syndrome is a rare congenital neuroectodermicdisorder characterized by vascularmalformations involving the face skin, leptomeningesof the cerebral cortex and choroids of the eye.The clinical course is variable but most of childrenhave epilepsy, neurologic deterioration and cerebralatrophy.Imaging studies (both CT and MR) are useful forconfirming the diagnosis of Sturge-Weber syndromeand evaluating the extent of intracranial involvement