[Infantile hepatic cholestatis: An unusual case of Langerhans cells histiocytosis associated with advanced fibrosis]. / Colestasis hepática infantil: presentación de un caso inusual de histiocitosis de células de Langerhans asociado a fibrosis avanzada.

Neonatal cholestasis is a clinical metabolic alteration requiring investigation of its eitiology. It is characterized by elevation of liver enzymes with cholestasis pattern and, in some cases, with acute liver failure. Its etiology is variable although the most frequent cause is atresia of extrahepatic bile ducts. We present a case of a 23-month-old boy who presented with cholestasis and was finally diagnosed with systemic Langerhans cell histiocytosis.

Colestasis hepática infantil: presentación de un caso inusual de histiocitosis de células de Langerhans asociado a fibrosis avanzada / Infantile hepatic cholestatis: An unusual case of Langerhans cells histiocytosis associated with advanced fibrosis

La colestasis neonatal es una situación clinicoanalítica que requiere una determinación urgente de su etiología. Se caracteriza por elevación de enzimas hepáticas con patrón de colestasis y en algunos casos con situación de fallo hepático agudo. Su etiología es variable aunque la causa más frecuente es la atresia de vías biliares extrahepáticas. En el presente caso describimos el caso de un niño de 23 meses de vida que presentó comienzo colestásico y que finalmente fue diagnosticado de histiocitosis de células de Langerhans sistémica.(AU)

Neonatal cholestasis is a clinical metabolic alteration requiring investigation of its eitiology. It is characterized by elevation of liver enzymes with cholestasis pattern and, in some cases, with acute liver failure. Its etiology is variable although the most frequent cause is atresia of extrahepatic bile ducts. We present a case of a 23-month-old boy who presented with cholestasis and was finally diagnosed with systemic Langerhans cell histiocytosis.(AU)

Bone mineral density in spanish children at the diagnosis of inflammatory bowel disease.

The association between low bone mineral density (BMD) and inflammatory bowel disease (IBD) is already known. Our study, performed in Spanish pediatric IBD patients at diagnosis onset, shows that low BMD already existed at the beginning of the disease. Low weight and height are also associated with low BMD and have to be considered as risk factors. INTRODUCTION: Inflammatory bowel disease (IBD) has been reported to be associated, even at disease onset, with low bone mass. The aim of this study was to know the bone mineral density (BMD) status in the IBD pediatric population of group of Spanish children, at the time of diagnosis. MATERIAL AND METHODS: Retrospective review of patients' records from pediatric IBD patients diagnosed in our unit in the last 10 years. BMD was measured at the time of diagnosis and was expressed by Z-score. RESULTS: Fifty-seven patients were included. Sixty-one percent were male and 47.4% had Crohn's disease (CD). Average age was 11.18 (SD 2.24) years old. Median BMD Z-score was - 0.30 (interquartile range: - 1.10 to + 0.10). Low BMD, defined as Z-score ≤ - 2SD, was present in 5% of patients, but there was no single patient with osteoporosis. There were no differences in BMD between Ulcerative Colitis (UC) and CD. Statistical differences appeared between healthy Spanish pediatric population and our IBD cohort, these having lower BMD for the same age and gender. A linear regression analysis showed a significant association between BMD Z-score and patient´s weight and height Z-score with a p values of 0.001 and 0.048, respectively. CONCLUSIONS: Suboptimal bone density is present at diagnosis in Spanish pediatric patients with IBD. There is no difference in BMD between patients with CD and UC. Lower weight and height are associated with a lower BMD; thus these data at IBD diagnosis should be considered as a risk factor for bone disease in the pediatric population.

Experience With Teduglutide in Pediatric Short Bowel Syndrome: First Real-life Data.

OBJECTIVES: The aim of the study was to describe the experience with teduglutide of several Spanish hospitals in pediatric patients with SBS (SBS). METHODS: Seventeen pediatric patients with intestinal failure associated with SBS were treated with teduglutide. Patients received 0.05 mg ·â€Škg ·â€Šday of subcutaneous teduglutide. Patients' demographics and changes in parenteral nutrition (PN) needs, fecal losses, and citrulline level initially and at 3, 6, and 12 months were collected, as well as any adverse events. RESULTS: Patients were receiving 55 ml ·â€Škg ·â€Šday and 33 kcal ·â€Škg ·â€Šday of parenteral supplementation on average at baseline (2 patients received only hydroelectrolytic solution). A total of 12/17 patients achieved parenteral independence: 3 patients after 3 months of treatment, 4 patients at 6 months, and 5 after 12 months. One patient discontinued treatment 1 year after the beginning as no changes in parenteral support or fecal losses were obtained. All others decreased their intravenous requirements by 50%. One patient suffered an episode of cholecystitis, and another one with a pre-existing cardiac disease, developed a cardiac decompensation. CONCLUSIONS: Teduglutide seems to be a safe and effective treatment in the pediatric SBS population with better results than in the pivotal study as well as in the adult population.

Anticuerpos antitransglutaminasa no relacionados con la ingesta de gluten / Anti-tissue transglutaminase antibodies not related to gluten intake

INTRODUCCIÓN: Los anticuerpos antitransglutaminasa (ATG) poseen alta especificidad para el diagnóstico de enfermedad celíaca (EC). Sin embargo, se han descrito anticuerpos ATG positivos en pacientes no celíacos. OBJETIVO: Valorar la presencia de anticuerpos ATG positivos no relacionados con la ingesta de gluten. PACIENTES Y MÉTODOS: Revisión retrospectiva de historias clínicas y seguimiento de pacientes con sospecha de EC y con un comportamiento serológico atípico, es decir, anticuerpos ATG positivos a pesar de una dieta sin gluten y disminución de anticuerpos ATG tomando gluten. RESULTADOS: Se incluyeron 9 casos. De ellos, 5 casos tenían afectación histológica Marsh 3 en la biopsia inicial y diagnóstico de EC (grupo A). Se retiró el gluten de la dieta y se retiraron las proteínas de leche de vaca (PLV) por la afectación nutricional. Al reintroducir las PLV aumentaron los ATG y al retirarlas se volvieron a normalizar. Los otros 4 pacientes presentaban una biopsia inicial normal (grupo B): en estos no se retiró el gluten, pero sí las PLV por sospecha de alergia no IgE mediada. Los síntomas desaparecieron y se normalizaron los ATG al retirar las PLV manteniendo dieta con gluten. Todos presentan el haplotipo de susceptibilidad para EC. CONCLUSIONES: En algunos celíacos, la reintroducción de PLV en la dieta tras un período de exclusión induce un aumento de los anticuerpos ATG IgA. Si se han descartado transgresiones con gluten, las PLV pueden causar esta respuesta inmune. Hemos observado también esta respuestaen pacientes con alergia no IgE, mediada por las PLV, portadores del haplotipo de susceptibilidad HLA DQ2/DQ8

INTRODUCTION: Anti-tissue transglutaminase antibodies (tTG) have high specificity for coeliac disease (CD). However, positive anti-tTG antibodies have been described in non-coeliac patients. Aim To assess positive anti-tTG antibodies not related to gluten intake. PATIENTS AND METHODS: Retrospective review and follow up conducted on patients with suspected CD (increase anti-tTG levels and gastrointestinal symptoms) but with atypical serology results, positive anti-tTG with gluten free diet and a decrease in anti-tTG levels despite gluten intake. RESULTS: A total of 9 cases were reviewed in which 5 cases had Marsh 3 involvement in the initial biopsy, and were diagnosed with CD (Group A). They began a gluten free diet and also a cow's milk protein (CMP) free diet because of their nutritional status. When CMP was re-introduced, anti-tTG increased, and returned to normal after the CMP was withdrawn again. The other 4 patients had a normal initial biopsy (Group B). Gluten was not removed from their diet, but they started a CMP free diet because a non IgE mediated CMP allergy was suspected. Symptoms disappeared, and anti-tTG was normal after CMP free diet with gluten intake. All the patients had susceptibility haplotype HLA DQ2/DQ8. CONCLUSIONS: CMP ingestion after an exclusion diet can induce an increase in anti-tTG in some coeliac subjects. CMP can produce this immune response if there were no gluten transgressions. This response has also been observed in non-IgE mediated CMP allergy patients with the susceptibility haplotype HLA DQ2/DQ8

[Anti-tissue transglutaminase antibodies not related to gluten intake]. / Anticuerpos antitransglutaminasa no relacionados con la ingesta de gluten.

INTRODUCTION: Anti-tissue transglutaminase antibodies (tTG) have high specificity for coeliac disease (CD). However, positive anti-tTG antibodies have been described in non-coeliac patients. Aim To assess positive anti-tTG antibodies not related to gluten intake. PATIENTS AND METHODS: Retrospective review and follow up conducted on patients with suspected CD (increase anti-tTG levels and gastrointestinal symptoms) but with atypical serology results, positive anti-tTG with gluten free diet and a decrease in anti-tTG levels despite gluten intake. RESULTS: A total of 9 cases were reviewed in which 5 cases had Marsh 3 involvement in the initial biopsy, and were diagnosed with CD (Group A). They began a gluten free diet and also a cow's milk protein (CMP) free diet because of their nutritional status. When CMP was re-introduced, anti-tTG increased, and returned to normal after the CMP was withdrawn again. The other 4 patients had a normal initial biopsy (Group B). Gluten was not removed from their diet, but they started a CMP free diet because a non IgE mediated CMP allergy was suspected. Symptoms disappeared, and anti-tTG was normal after CMP free diet with gluten intake. All the patients had susceptibility haplotype HLA DQ2/DQ8. CONCLUSIONS: CMP ingestion after an exclusion diet can induce an increase in anti-tTG in some coeliac subjects. CMP can produce this immune response if there were no gluten transgressions. This response has also been observed in non-IgE mediated CMP allergy patients with the susceptibility haplotype HLA DQ2/DQ8.

DEVELOPMENT AND VALIDATION OF TWO FOOD FREQUENCY QUESTIONNAIRES TO ASSESS GLUTEN INTAKE IN CHILDREN UP TO 36 MONTHS OF AGE.

BACKGROUND AND OBJECTIVE: precise information on gluten consumption is crucial for specifically studying the impact of gluten introduction and gluten intake in celiac disease development. Our aim was to develop and validate tools (food frequency questionnaires, FFQs) for the assessment of gluten consumption in Spanish children aged 7-36 months. METHODS: a total of 342 children, who attended primary healthcare centers for routine health surveys or La Fe Hospital for minor health problems as well as healthy children (recruited in nurseries and primary schools) participated in this survey. We have developed two different FFQs (one for 7-12 months and other for 13-36 months). For validation, results from two FFQs were compared with results of 2-day food records and also with the gold standard 7-day records. The mean gluten intake obtained by the 2DR vs. FFQ and the 7DR vs. FFQ, were compared using the Bland Altman plot method and also Lin's concordance correlation coefficient. RESULTS: we found a good agreement between our FFQs and the 2DR and 7DR according to the results of both the Bland-Altman plots and Lin's concordance correlation coefficient. CONCLUSIONS: our two new FFQs are therefore the only validated questionnaires available to determine gluten consumption in Spanish children. They are user-friendly and offer excellent instruments to assess gluten intake in children up to 36 months of age.

Antecedentes y objetivos: una información precisa sobre el consumo de gluten es muy importante para estudiar el verdadero impacto de la introducción y la ingesta de gluten en el desarrollo de la enfermedad celiaca. El objetivo del estudio fue desarrollar y validar herramientas (cuestionarios de frecuencia de consumo, CFC) para evaluar el consumo de gluten en niños con edades comprendidas entre los 7 y 36 meses. Métodos: se incluyeron un total de 342 niños reclutados en el Hospital Universitario y Politécnico de La Fe, así como en guarderías y escuelas de primaria con problemas menores de salud. Se desarrollaron dos CFC diferentes (uno para niños de 7 a 12 meses y otro para niños de 13 a 36 meses). Para su validación, los resultados obtenidos con los CFC se compararon con un registro alimentario de dos días (2RA) y también con un registro alimentario de soete días (7RA), considerado el "estándar de oro". La ingesta media de gluten obtenida de la comparación del 2RA vs. CFC y de 7RA vs. CFC fueron comparadas usando el método de Bland Altman plot y también el coeficiente de correlación de concordancia de Lin's. Resultados: de acuerdo a los resultados de los dos métodos estadísticos usados para la validación, se encontró una buena correlación entre los CFC y los registros alimentarios de dos y siete días, lo que indica que los CFC son fiables para la evaluación de la ingesta de gluten. Conclusiones: estos dos nuevos CFC son los únicos validados y disponibles en España para la evaluación de la ingesta de gluten. Además, son herramientas útiles y fáciles de usar para el cálculo del consumo de gluten en niños de hasta tres años de edad.

Development and validation of two food frequency questionnaires to assess gluten intake in children up to 36 months of age / Desarrollo y validación de dos cuestionarios de frecuencia de consumo para evaluar la ingesta de gluten en niños hasta los 3 años

Background and objective: precise information on gluten consumption is crucial for specifically studying the impact of gluten introduction and gluten intake in celiac disease development. Our aim was to develop and validate tools (food frequency questionnaires, FFQs) for the assessment of gluten consumption in Spanish children aged 7-36 months. Methods: a total of 342 children, who attended primary healthcare centers for routine health surveys or La Fe Hospital for minor health problems as well as healthy children (recruited in nurseries and primary schools) participated in this survey. We have developed two different FFQs (one for 7-12 months and other for 13-36 months). For validation, results from two FFQs were compared with results of 2-day food records and also with the gold standard 7-day records. The mean gluten intake obtained by the 2DR vs. FFQ and the 7DR vs. FFQ, were compared using the Bland Altman plot method and also Lin’s concordance correlation coefficient. Results: we found a good agreement between our FFQs and the 2DR and 7DR according to the results of both the Bland-Altman plots and Lin’s concordance correlation coefficient. Conclusions: our two new FFQs are therefore the only validated questionnaires available to determine gluten consumption in Spanish children. They are user-friendly and offer excellent instruments to assess gluten intake in children up to 36 months of age (AU)

Antecedentes y objetivos: una información precisa sobre el consumo de gluten es muy importante para estudiar el verdadero impacto de la introducción y la ingesta de gluten en el desarrollo de la enfermedad celiaca. El objetivo del estudio fue desarrollar y validar herramientas (cuestionarios de frecuencia de consumo, CFC) para evaluar el consumo de gluten en niños con edades comprendidas entre los 7 y 36 meses. Métodos: se incluyeron un total de 342 niños reclutados en el Hospital Universitario y Politécnico de La Fe, así como en guarderías y escuelas de primaria con problemas menores de salud. Se desarrollaron dos CFC diferentes (uno para niños de 7 a 12 meses y otro para niños de 13 a 36 meses). Para su validación, los resultados obtenidos con los CFC se compararon con un registro alimentario de dos días (2RA) y también con un registro alimentario de siete días (7RA), considerado el “estándar de oro”. La ingesta media de gluten obtenida de la comparación del 2RA vs. CFC y de 7RA vs. CFC fueron comparadas usando el método de Bland Altman plot y también el coeficiente de correlación de concordancia de Lin’s. Resultados: de acuerdo a los resultados de los dos métodos estadísticos usados para la validación, se encontró una buena correlación entre los CFC y los registros alimentarios de dos y siete días, lo que indica que los CFC son fiables para la evaluación de la ingesta de gluten. Conclusiones: estos dos nuevos CFC son los únicos validados y disponibles en España para la evaluación de la ingesta de gluten. Además, son herramientas útiles y fáciles de usar para el cálculo del consumo de gluten en niños de hasta tres años de edad (AU)