[Gastric tube esophagoplasty using the retroesternal route in children]. / Sustitución esofágica con estómago tubulizado retroesternal en la infancia.

INTRODUCTION: Esophageal replacement is a surgical alternative once native esophagus can't be preserved. Different organs and routes for the replacement have been described, being the retroesternal route the least used. The aim is to present our results using gastric tube esophagoplasty with a retroesternal approach. PATIENTS AND METHODS: We performed a retrospective and descriptive study of 11 patients operated from 2000 to 2015. Median age at surgery was 2.2 years (5 months-9 years) and median weight was 11.2 kg (7.8-21). A gastric tube esophagoplasty using the retroesternal route, forced pyloric dilatation and end-to-side esophago-gastric cervical anastomosis were performed. RESULTS: Ten esophagus replacements had long-gap esophageal atresia and one, severe esophagus caustication secondary to button battery ingestion. No intraoperatory complications were observed. Three patients developed anastomosis leak. Two cases developed anastomotic stenosis managed with endoscopic dilatation in 2 and 4 occasions, respectively. Four patients showed occasional dumping syndrome and are asymptomatic after medical treatment. With a median follow up of 6.3 years (0.2-14.8), all our patients are alive and complete oral diet has been established in all of them. CONCLUSIONS: Gastric tube esophagoplasty using the retroesternal route is a suitable technique in order to reestablish gastrointestinal continuity once native esophagus can't be preserved. In our experience is a safe option, related to few complications.

INTRODUCCION: La sustitución esofágica es una de las opciones quirúrgicas en pacientes en los que no es posible la preservación del esófago. Existen diferentes técnicas según el órgano ascendido y la vía de ascenso, siendo la vía retroesternal la menos empleada. Se describen los resultados con el uso de estómago tubulizado retroesternal. PACIENTES Y METODOS: Estudio descriptivo retrospectivo de una serie de 11 pacientes intervenidos entre los años 2000 y 2015, con una edad media en el momento de la intervención de 2,2 años (5 meses-9 años) y un peso de 11,2 kg (7,8-21 kg). Se realizó gastroplastia tubulizada con dilatación forzada de píloro, ascenso gástrico por vía retroesternal y anastomosis esófago-gástrica cervical término-lateral. RESULTADOS: Diez sustituciones se realizaron en pacientes con atresia de esófago long-gap y una, tras una causticación esofágica por pila de botón. No hubo ninguna complicación intraoperatoria. En tres pacientes hubo fuga anastomótica. En dos pacientes se produjo estenosis que precisó dilataciones en 2 y en 4 ocasiones, respectivamente. Cuatro pacientes presentaron síndrome dumping ocasional que se resolvió con tratamiento médico. Con un seguimiento medio de 6,3 años (0,2-14,8), ningún paciente ha fallecido y en todos se ha logrado la nutrición oral completa. CONCLUSIONES: La gastroplastia tubulizada retroesternal es una técnica eficaz para restablecer la continuidad gastrointestinal en aquellos pacientes en los que no es posible preservar el esófago. Puede ser una opción segura y con escasas complicaciones.

Sustitución esofágica con estómago tubulizado retroesternal en la infancia / Gastric tube esophagoplasty using the retroesternal route in children

Introducción. La sustitución esofágica es una de las opciones quirúrgicas en pacientes en los que no es posible la preservación del esófago. Existen diferentes técnicas según el órgano ascendido y la vía de ascenso, siendo la vía retroesternal la menos empleada. Se describen los resultados con el uso de estómago tubulizado retroesternal. Pacientes y métodos. Estudio descriptivo retrospectivo de una serie de 11 pacientes intervenidos entre los años 2000 y 2015, con una edad media en el momento de la intervención de 2,2 años (5 meses-9 años) y un peso de 11,2 kg (7,8-21 kg). Se realizó gastroplastia tubulizada con dilatación forzada de píloro, ascenso gástrico por vía retroesternal y anastomosis esófago-gástrica cervical término-lateral. Resultados. Diez sustituciones se realizaron en pacientes con atresia de esófago long-gap y una, tras una causticación esofágica por pila de botón. No hubo ninguna complicación intraoperatoria. En tres pacientes hubo fuga anastomótica. En dos pacientes se produjo estenosis que precisó dilataciones en 2 y en 4 ocasiones, respectivamente. Cuatro pacientes presentaron síndrome dumping ocasional que se resolvió con tratamiento médico. Con un seguimiento medio de 6,3 años (0,2-14,8), ningún paciente ha fallecido y en todos se ha logrado la nutrición oral completa. Conclusiones. La gastroplastia tubulizada retroesternal es una técnica eficaz para restablecer la continuidad gastrointestinal en aquellos pacientes en los que no es posible preservar el esófago. Puede ser una opción segura y con escasas complicaciones

Introduction. Esophageal replacement is a surgical alternative once native esophagus can’t be preserved. Different organs and routes for the replacement have been described, being the retroesternal route the least used. The aim is to present our results using gastric tube esophagoplasty with a retroesternal approach. Patients and methods. We performed a retrospective and descriptive study of 11 patients operated from 2000 to 2015. Median age at surgery was 2.2 years (5 months-9 years) and median weight was 11.2 kg (7.8-21). A gastric tube esophagoplasty using the retroesternal route, forced pyloric dilatation and end-to-side esophago-gastric cervical anastomosis were performed. Results. Ten esophagus replacements had long-gap esophageal atresia and one, severe esophagus caustication secondary to button battery ingestion. No intraoperatory complications were observed. Three patients developed anastomosis leak. Two cases developed anastomotic stenosis managed with endoscopic dilatation in 2 and 4 occasions, respectively. Four patients showed occasional dumping syndrome and are asymptomatic after medical treatment. With a median follow up of 6.3 years (0.2-14.8), all our patients are alive and complete oral diet has been established in all of them. Conclusions. Gastric tube esophagoplasty using the retroesternal route is a suitable technique in order to reestablish gastrointestinal continuity once native esophagus can’t be preserved. In our experience is a safe option, related to few complications

Utilidad de la entero-resonancia magnética en el estudio de la enfermedad de Crohn pediátrica / MRI enterography in the assessment of paediatric Crohn’s disease

Objetivo: Evaluar la utilidad de la resonancia magnética intestinal con contraste oral (RMI) para el estudio de extensión y actividad de la enfermedad de Crohn (EC) pediátrica, comparando los hallazgos con índices clínicos, tests biológicos, endoscopia y otras técnicas de imagen. Pacientes y métodos: Fueron valoradas las RMI efectuadas en pacientes menores de 18 años diagnosticados de EC. Para la preparación se administró 500-1000ml de polietilenglicol una hora antes de las imágenes (1,5-TMR). Se realizaron secuencias T2 HASTE con o sin Fat SAT, T2 true-FISP, T1 Fat-SAT VIBE pre/posgadolinio, HASTE dinámico y difusión. Se valoraron el engrosamiento de pared intestinal, la hipercaptación mucosa y las complicaciones extraintestinales. Se establecieron 5 patrones de RMI: normal, fibrosis, actividad leve, moderada y severa-transmural. Los hallazgos se compararon con PCDAI, parámetros inflamatorios, resultados endoscópicos e histológicos. Resultados: Incluimos para la evaluación 22 estudios. El 82% presentaba una distensión intestinal óptima. Observamos efectos secundarios leves en el 12% de los pacientes. Encontramos una relación significativa entre los patrones de RMI versus PCDAI (p=0,002), VSG (p=0,006) y PCR (p=0,047); no hallamos relación estadísticamente significativa (p=0,571) con la histología. La RMI valoró correctamente la extensión de la enfermedad a nivel ileal (80%) y a nivel cólico (66%). Un 86,4% de los estudios mostraron complicaciones extraintestinales, sin presentar relación estadística con la clasificación de RMI (p=0,274). Conclusiones: Existe una relación estadísticamente significativa entre nuestros patrones de RMI y PCR, VSG y PCDAI. La RMI presenta excelente concordancia con las ileoscopias. La RMI valora zonas no accesibles mediante endoscopia y permite el diagnóstico de complicaciones extraintestinales sin irradiación (AU)

Objective: To determine the usefulness of MRI enterography for assessing the extension and activity of paediatric Crohn's disease. MRI findings were compared with clinical, biological, endoscopic and other imaging data. Patients and methods: Studies of MRI enterography use in patients younger than 18 years of age were reviewed. Patients received 500-1000mL of polyethylene glycol one hour before examination (1.5-TMR). T2 HASTE sequences with or without fat saturation, T2 true-FISP, T1 with fat saturation, pre- and post gadolinium-enhanced VIBE sequences, and dynamic and diffusion HASTE were acquired. Thickening of the bowel wall, mucosal enhancement, and extra-luminal complications were evaluated. Five MRI patterns (normal, fibrosis, mild, moderate, and severe transmural activity) were defined. Findings were compared with PCDAI scores, inflammatory parameters, and endoscopic and histological results. Results: Twenty-two studies were reviewed. Optimal intestinal distension was present in 82% of the cases. Mild side effects were observed in 12% of patients. There was a significant relationship between MRI patterns and PCDAI scores (P=0.002), sedimentation rate (P=0.006) and serum PCR levels (P=0.047) and a non-significant relationship with the histology (P=0.571). MRI enterography correctly assessed the ileal (80%) and colonic (66%) extension. Extra-luminal complications unrelated to MRI classification (P=0.274) were reported in 86.4% of studies. Conclusions: There was a significant relationship between MRI patterns and PCR, sedimentation rate, and PCDAI scores. MRI enterography showed excellent agreement with ileoscopies, and allowed endoscopically non-accessible areas to be assessed, as well as the diagnosis of extra-luminal complications without irradiation (AU)

Analysis of the Spanish national registry for pediatric home enteral nutrition (NEPAD): implementation rates and observed trends during the past 8 years.

BACKGROUND/OBJECTIVES: The home enteral nutrition (HEN) provides nutritional support to children with chronic diseases who are nutritionally compromised and allows them to be discharged more quickly from hospitals. In 2003, a web-based registry (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition -NEPAD-) was created with the objective of gathering information about pediatric HEN practices in Spain. AIM: The aim of this study was to report the implementation of the NEPAD (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition) registry of pediatric HEN in Spain and to analyze data evolution trends from 2003 to 2010. SUBJECTS/METHODS: The data from the Spanish NEPAD registry were analyzed according to the following variables: demographic data, diagnosis, indication for HEN, nutritional support regime and administration route. RESULTS: Over the study period, 952 patients (1048 episodes) from 20 Spanish hospitals were included in the NEPAD registry. The most frequent indication for HEN was decreased oral intake (64%), and neurological disease was the most prevalent illness. HEN was delivered via a nasogastric tube in 573 episodes (54.7%), by gastrostomy in 375 episodes (35.8%), oral feeding in 77 episodes (7.3%) and by jejunal access in 23 episodes (2.2%). Significant differences in the mode of administration were observed based on the pathology of the child (χ(2), P<0.0001). The cyclic feeding was the most widely used technique for the administration of HEN. Most of the patients used a pump and a polymeric formula. Transition to oral feeding was the primary reason for discontinuation of this type of support. CONCLUSIONS: Since the NEPAD registry was established in Spain, the number of documented patients has increased more than 25-fold. Many children with chronic illness benefit from HEN, mainly those suffering from neurological diseases.

[MRI enterography in the assessment of paediatric Crohn's disease]. / Utilidad de la entero-resonancia magnética en el estudio de la enfermedad de Crohn pediátrica.

OBJECTIVE: To determine the usefulness of MRI enterography for assessing the extension and activity of paediatric Crohn's disease. MRI findings were compared with clinical, biological, endoscopic and other imaging data. PATIENTS AND METHODS: Studies of MRI enterography use in patients younger than 18 years of age were reviewed. Patients received 500-1000mL of polyethylene glycol one hour before examination (1.5-TMR). T2 HASTE sequences with or without fat saturation, T2 true-FISP, T1 with fat saturation, pre- and post gadolinium-enhanced VIBE sequences, and dynamic and diffusion HASTE were acquired. Thickening of the bowel wall, mucosal enhancement, and extra-luminal complications were evaluated. Five MRI patterns (normal, fibrosis, mild, moderate, and severe transmural activity) were defined. Findings were compared with PCDAI scores, inflammatory parameters, and endoscopic and histological results. RESULTS: Twenty-two studies were reviewed. Optimal intestinal distension was present in 82% of the cases. Mild side effects were observed in 12% of patients. There was a significant relationship between MRI patterns and PCDAI scores (P=.002), sedimentation rate (P=.006) and serum PCR levels (P=.047) and a non-significant relationship with the histology (P=.571). MRI enterography correctly assessed the ileal (80%) and colonic (66%) extension. Extra-luminal complications unrelated to MRI classification (P=.274) were reported in 86.4% of studies. CONCLUSIONS: There was a significant relationship between MRI patterns and PCR, sedimentation rate, and PCDAI scores. MRI enterography showed excellent agreement with ileoscopies, and allowed endoscopically non-accessible areas to be assessed, as well as the diagnosis of extra-luminal complications without irradiation.

Nutrición en los errores innatos del metabolismo en adultos / Nutritional support in adult patients with inborn errors of metabolism

Los Errores Innatos del Metabolismo (EIM) son trastornos genéticos caracterizados por disfunción de una proteína involucrada en el metabolismo celular, lo que provoca una alteración en el funcionamiento fisiológico de la célula. Dependiendo de la función de la proteína y de cuál sea la vía metabólica afectada, puede producirse una toxicidad por acúmulo del sustrato no metabolizado, o por la aparición de sustancias producidas por el metabolismo de dicho sustrato a través de vías alternativas, o bien fenómenos derivados del déficit del producto final de la vía metabólica. Las EIM son un grupo de enfermedades genéticas muy numeroso y complejo, con un elevado grado de heterogeneidad genética y, en consecuencia, clínica y bioquímica. En los pacientes adultos, podemos encontrar dos situaciones clínicas: pacientes con EIM diagnosticados en la edad pediátrica que alcanzan la edad adulta, o formas de inicio tardío diagnosticadas ya en la edad adulta, a menudo formas atípicas. El tratamiento nutricional es uno de los pilares fundamentales del tratamiento de muchos EIM, en ocasiones el único eficaz. En el paciente adulto, una vez alcanzada la talla final, el tratamiento tendrá como objetivo cubrir los requerimientos nutricionales del paciente y evitar descompensaciones. Dentro del tratamiento nutricional de los EIM tiene especial relevancia el uso de suplementos nutricionales específicos que ayudarán a cubrir los requerimientos nutricionales de los pacientes afectos de EIM, en muchas ocasiones difíciles de conseguir con alimentos convencionales. El uso de algunas vitaminas que actúan como cofactores enzimáticos también adquiere especial relevancia en algunos de los EIM del adulto (AU)

The Inborn Errors of Metabolism (IEM) are genetic disorders characterized by dysfunction of a protein involved in cell metabolism, causing an alteration in the physiological functioning of the cell. Depending on the protein function and the metabolic pathway which is affected, toxicity may occur because of accumulation of unmetabolized substrate, or the development of substances produced by the metabolism of the substrate through alternative pathways, or deficit of the final product of the metabolic pathway. EIM are a group of genetic diseases too numerous and complex, with a high degree of genetic heterogeneity and, consequently, clinical and biochemical. In adult patients, we can find two clinical situations: patients with IEM diagnosed in children reaching adulthood, or late-onset forms diagnosed in adulthood and often atypical. Nutritional therapy is one of the mainstays of treatment of many IEM, sometimes the only effective. In the adult patient, after reaching final height, the treatment will aim to meet the nutritional requirements of the patient and avoid worsening. Within the nutritional management of EIM is especially relevant the use of specific nutritional supplements that will help meet the nutritional requirements of patients with IEM, often difficult to achieve with conventional foods. The use of some vitamins that acts as enzyme cofactors is also especially relevant in some of the adult EIM (AU)

Seguridad y eficacia del polietilenglicol 3350 con electrolitos en el tratamiento del estreñimiento funcional en niños / Safety and efficacy of polyethylene glycol 3350 plus electrolytes for the treatment of functional constipation in children

Introducción: El objetivo del trabajo ha sido evaluar la seguridad del PEG 3350 con electrolitos (PEG+E) a nivel renal y digestivo. Objetivo secundario: valorar su eficacia y dosis de efectividad. Pacientes y métodos: Quince pacientes con estreñimiento funcional (criterios de Roma III) y función renal normal fueron evaluados. La mediana de edad fue de 6,2 años (r=2-9). Sobres pediátricos de PEG+E fueron administrados durante 4 semanas (4ST) La dosis media administrada fue de 0,44g/kg/día. La natruria y osmolaridad urinaria se midieron al inicio y a las 4ST. La determinación de principios inmediatos en heces mediante FENIR (análisis de reflexión de infrarrojos) y una prueba de hidrógeno espirado fueron efectuadas a las 4ST. La eficacia del tratamiento fue evaluada mediante el cambio en el número de deposiciones por semana y la consistencia de las heces (escala de Bristol). Resultados: A las 4ST el número de deposiciones por semana fue de 5,29±1,68 vs 2,46±0,71 al inicio (p<0,001). La puntuación de la escala de Bristol fue de 4,5±0,91 tras 4TS vs 2,47±1,24 al inicio (p<0,001). No se encontraron diferencias estadísticas entre los valores de sodio y osmolalidad en orina al inicio vs 4ST. Los valores de FENIR fueron normales en todos los pacientes. La prueba del aliento de hidrógeno fue normal con una mediana de 7ppm. Conclusiones: No se observaron efectos adversos renales ni alteraciones digestivas. El PEG+E puede ser recomendado para el tratamiento del estreñimiento funcional en los niños (AU)

Introduction: Polyethylene glycol 3350 plus electrolytes (PEG+E) efficacy has been validated in some studies, but not many have evaluated its safety in children. The aim of our study was to evaluate the safety; renal, malabsorption or excessive production of gas and efficacy of PEG+E treatment in our paediatric population. Patients and methods: Fifteen patients who suffered functional constipation (Rome III criteria) were evaluated. Median age was 6.2 years (r 2-9). All patients had normal renal function. PEG+E were administered for 4weeks (4WP). The mean dose was 0.44g/kg/day, titrated according to age, weight and response. Urine screens (sodium and osmolality) were performed at the beginning and 4WP. Stool sample NIRA (near-infrared reflectance analysis) and hydrogen breath test analysis samples were performed at 4WP. To analyse the efficacy of the treatment, the number of stools per week and stool form type (Bristol stool scale) were recorded. Results: The number of stools per week was higher after 4weeks (2.46±0.71 vs 5.29±1.68, P<0.001), as well as the stool form score (2.47±1.24 vs 4.5±0.91, P<0.001). No statistical differences were obtained between urine sodium and urine osmolality values at the beginning and 4WP. After 4WP the NIRA median values were normal in all patients [fat 4.45% (range (r) 3.6-7.09); nitrogen 0.78% (r 0.4-1); sugars 1.4% (r 0.47-2.35) and water 68% (r 59-74)]. Median breath hydrogen test was 7ppm (r 2-18). Conclusions: No adverse effects on biochemistry values or gastrointestinal disturbances were observed. PEG+E can be recommended for the treatment of functional constipation in children (AU)

[Safety and efficacy of polyethylene glycol 3350 plus electrolytes for the treatment of functional constipation in children]. / Seguridad y eficacia del polietilenglicol 3350 con electrolitos en el tratamiento del estreñimiento funcional en niños.

INTRODUCTION: Polyethylene glycol 3350 plus electrolytes (PEG+E) efficacy has been validated in some studies, but not many have evaluated its safety in children. The aim of our study was to evaluate the safety; renal, malabsorption or excessive production of gas and efficacy of PEG+E treatment in our paediatric population. PATIENTS AND METHODS: Fifteen patients who suffered functional constipation (Rome III criteria) were evaluated. Median age was 6.2 years (r 2-9). All patients had normal renal function. PEG+E were administered for 4 weeks (4WP). The mean dose was 0.44 g/kg/day, titrated according to age, weight and response. Urine screens (sodium and osmolality) were performed at the beginning and 4WP. Stool sample NIRA (near-infrared reflectance analysis) and hydrogen breath test analysis samples were performed at 4WP. To analyse the efficacy of the treatment, the number of stools per week and stool form type (Bristol stool scale) were recorded. RESULTS: The number of stools per week was higher after 4 weeks (2.46 ± 0.71 vs 5.29 ± 1.68, P<.001), as well as the stool form score (2.47 ± 1.24 vs 4.5 ± 0.91, P<.001). No statistical differences were obtained between urine sodium and urine osmolality values at the beginning and 4WP. After 4WP the NIRA median values were normal in all patients [fat 4.45% (range (r) 3.6-7.09); nitrogen 0.78% (r 0.4-1); sugars 1.4% (r 0.47-2.35) and water 68% (r 59-74)]. Median breath hydrogen test was 7 ppm (r 2-18). CONCLUSIONS: No adverse effects on biochemistry values or gastrointestinal disturbances were observed. PEG+E can be recommended for the treatment of functional constipation in children.

Administración de la nutrición enteral / No disponible

No disponible

Home parenteral nutritrion in children: procedures, experiences and reflections / La nutrición parenteral pediátrica domiciliaria: procedimientos, experiencias y reflexiones

This document summarizes the issues raised in a think tank meeting held by professionals with expertise in pediatric Home Parenteral Nutrition. This nutritional technology enables patients to return home to their family and social environment, improves their quality of life and decreases health-care costs; however, it is complex and requires an experienced nutritional support team. Patient selection is normally made according to their underlying disease, the estimated duration of support and family and social characteristics. The patient’s family must agree to take on caregiver’s responsibilities and should be able to perform treatment safely and effectively after receiving proper training from the nutritional support team. Close monitoring must be carried out to ensure tolerance and effectiveness of nutritional support, there by avoiding complications. This nutritional treatment achieves, in most cases, recovery and intestinal adaptation in varying periods of time. In certain diseases, and when home parenteral nutrition becomes complicated, intestinal transplant may be recommendable, so referral to rehabilitation units and Intestinal Transplantation should be made early on (AU)

El presente documento resume los aspectos abordados en una Jornada de puesta en común con la participación de profesionales con experiencia en nutrición parenteral domiciliaria pediátrica. Este tratamiento permite el retorno de los pacientes a su medio familiar y social, mejora su calidad de vida y disminuye los costes sanitarios pero es complejo y requiere un equipo de soporte nutricional experimentado. La selección del paciente se realizará en función de su enfermedad de base, la duración estimada del soporte y las características familiares y sociales. La familia del paciente ha de querer hacerse cargo de su cuidado y debe ser capaz de realizar el tratamiento de forma segura y eficaz tras recibir la formación adecuada por el equipo de soporte nutricional. El seguimiento ha de efectuarse de forma estrecha para asegurar la tolerancia y eficacia del soporte, evitando las complicaciones. Este tratamiento nutricional consigue, en la mayoría de los casos, la recuperación y adaptación intestinal en periodos variables de tiempo. En ciertas patologías y cuando la nutrición parenteral domiciliaria se complica puede estar indicado el trasplante intestinal, por lo que la remisión a las Unidades de Rehabilitación Intestinal y Trasplante debe hacerse de forma precoz (AU)

Home parenteral nutrition in children: procedures, experiences and reflections.

This document summarizes the issues raised in a think-tank meeting held by professionals with expertise in pediatric Home Parenteral Nutrition. This nutritional technology enables patients to return home to their family and social environment, improves their quality of life and decreases health-care costs; however, it is complex and requires an experienced nutritional support team. Patient selection is normally made according to their underlying disease, the estimated duration of support and family and social characteristics. The patient''s family must agree to take on caregiver's responsibilities and should be able to perform treatment safely and effectively after receiving proper training from the nutritional support team. Close monitoring must be carried out to ensure tolerance and effectiveness of nutritional support, thereby avoiding complications. This nutritional treatment achieves, in most cases, recovery and intestinal adaptation in varying periods of time. In certain diseases, and when home parenteral nutrition becomes complicated, intestinal transplant may be recommendable, so referral to rehabilitation units and Intestinal Transplantation should be made early on.

Guía para la elaboración de fórmulas infantiles en polvo en el medio hospitalario. Sistema de análisis de peligros y puntos de control crítico / Powdered infant formulae preparation guide for hospitals based on Hazard Analysis and Critical Control Points (HACCP) principles

Esta guía para la elaboración de fórmulas infantiles en polvo es el resultado del trabajo conjunto de varios servicios hospitalarios; adapta la reglamentación nacional y europea vigente, así como las recomendaciones de sociedades científicas y reuniones de expertos internacionales. Está basada en el sistema de evaluación de peligros y puntos de control críticos propuesto por el Codex Alimentarius y pone énfasis en las medidas de comprobación, en los controles microbiológicos del proceso y especifica las medidas correctoras en caso de que no se cumplan los límites propuestos. Es una guía dinámica que cuenta con procedimientos de evaluación que le permiten una adaptación constante (AU)

This guide for the preparation of powdered infant formulae in hospital environments is a collaborative work between several hospital services and is based on national and European regulations, international experts meetings and the recommendations of scientific societies. This guide also uses the Hazard Analysis and Critical Control Point principles proposed by Codex Alimentarius and emphasises effective verifying measures, microbiological controls of the process and the corrective actions when monitoring indicates that a critical control point is not under control. It is a dynamic guide and specifies the evaluation procedures that allow it to be constantly adapte (AU)

[Powdered infant formulae preparation guide for hospitals based on Hazard Analysis and Critical Control Points (HACCP) principles]. / Guía para la elaboración de fórmulas infantiles en polvo en el medio hospitalario. Sistema de análisis de peligros y puntos de control crítico.

This guide for the preparation of powdered infant formulae in hospital environments is a collaborative work between several hospital services and is based on national and European regulations, international experts meetings and the recommendations of scientific societies. This guide also uses the Hazard Analysis and Critical Control Point principles proposed by Codex Alimentarius and emphasises effective verifying measures, microbiological controls of the process and the corrective actions when monitoring indicates that a critical control point is not under control. It is a dynamic guide and specifies the evaluation procedures that allow it to be constantly adapted.

[Optimising azathioprine treatment: determination of thiopurine methyltransferase activity and thiopurine metabolites]. / Optimización del tratamiento con azatioprina: determinación de la actividad de tiopurina metiltransferasa y las concentraciones de metabolitos tiopurínicos.

INTRODUCTION: Individualised doses of azathioprine (AZA) may be prescribed by monitoring the levels of the enzyme thiopurine methyltransferase (TPMT). The measurements of thiopurine metabolites of AZA, 6-thioguanine (6-TGN) and 6-methylmercaptopurine (6-MMP), have also been reported as new markers of AZA activity. OBJECTIVES: To describe TPMT phenotype in our population and to establish a relationship between thiopurine metabolites,and therapeutic activity and adverse effects. MATERIAL AND METHODS: Data on TPMT were retrospectively collected from 107 patients, and 6-TGN and 6-MMP levels in 18 patients currently on treatment with AZA (Crohn's disease 5, ulcerative colitis 5, autoimmune hepatitis 5). RESULTS: Mean value of TPMT was 20.19U/ml. None of the patients had a TPMT activity<5U/ml. Of the 18 patients on treatment, 13 showed sub-therapeutic levels of 6-TGN (<235pmol/8x10(8) red blood cells). Clinical remission was maintained in 45% of patients. Mean levels of 6-TGN in patients with clinical remission were 259pmol/8x10(8) red blood cells versus 209pmol/8x10(8) red blood cells in non-responders (p=0.37). There was an inverse relationship (r=-0.28) between TPMT and 6-TGN levels. Toxic effects occurred in 6 of 18 patients, with leukopenia in 5 and hyperamylasemia in 1. CONCLUSIONS: Determination of TPMT and monitoring of thiopurine metabolites allows AZA treatment to be optimised, although further studies are necessary to establish therapeutic effectiveness and toxicity ranges.

Optimización del tratamiento con azatioprina: determinación de la actividad de tiopurina metiltransferasa y las concentraciones de metabolitos tiopurínicos / Optimising azathioprine treatment: determination of thiopurine methyltransferase activity and thiopurine metabolites

Introducción: la determinación de la enzima tiopurina metiltransferasa (TPMT) nos permite pautar la dosis inicial individualizada de azatioprina (AZA). Las determinaciones de los metabolitos tiopurínicos de la AZA, la 6-tioguanina (6-TGN) y la 6-metilmercaptopurina (6-MMP) se han descrito como nuevos marcadores de la actividad del fármaco. Objetivos: describir el fenotipo de TPMT en nuestra población y relacionar los valores de los metabolitos tiopurínicos con la actividad terapéutica y los efectos adversos. Material y métodos: se recogieron retrospectivamente los valores de TPMT de 107 pacientes y de 6-TGN y 6-MMP de 18 pacientes en tratamiento con AZA (8 con enfermedad de Crohn, 5 con colitis ulcerosa y 5 con hepatitis autoinmune). Resultados: la media de determinación de TPMT fue 20,19U/ml. Ninguno presentó actividad de TPMT menor que 5U/ml. De los 18 pacientes, 13 mostraron concentraciones subterapéuticas de 6-TGN (<235pmol/8×108 hematíes). El 45% de los pacientes mantuvieron remisión clínica. La media de concentración de 6-TGN en los pacientes en remisión fue 259pmol/8×108 hematíes frente a 209pmol/8×108 hematíes en los no respondedores (p=0,37). Hay una relación inversa (r=−0,28) entre los valores de TPMT y los de 6-TGN. En 6/18 pacientes encontramos toxicidad: 5 con leucocitopenia y uno con hiperamilasemia. Conclusiones: la determinación de TPMT y la monitorización de los metabolitos tiopurínicos nos permite optimizar tratamiento con AZA, aunque son necesarios nuevos estudios que permitan el correcto conocimiento de los intervalos de efectividad terapéutica y toxicidad (AU)

Introduction: Individualised doses of azathioprine (AZA) may be prescribed by monitoring the levels of the enzyme thiopurine methyltransferase (TPMT). The measurements of thiopurine metabolites of AZA, 6-thioguanine (6-TGN) and 6-methylmercaptopurine (6-MMP), have also been reported as new markers of AZA activity. Objectives: To describe TPMT phenotype in our population and to establish a relationship between thiopurine metabolites,and therapeutic activity and adverse effects. Material and methods: Data on TPMT were retrospectively collected from 107 patients, and 6-TGN and 6-MMP levels in 18 patients currently on treatment with AZA (Crohn’s disease 5, ulcerative colitis 5, autoimmune hepatitis 5). Results: Mean value of TPMT was 20.19U/ml. None of the patients had a TPMT activity<5U/ml. Of the 18 patients on treatment, 13 showed sub-therapeutic levels of 6-TGN (<235pmol/8×108 red blood cells). Clinical remission was maintained in 45% of patients. Mean levels of 6-TGN in patients with clinical remission were 259pmol/8×108 red blood cells versus 209pmol/8×108 red blood cells in non-responders (p=0.37). There was an inverse relationship (r=−0.28) between TPMT and 6-TGN levels. Toxic effects occurred in 6 of 18 patients, with leukopenia in 5 and hyperamylasemia in 1. Conclusions: Determination of TPMT and monitoring of thiopurine metabolites allows AZA treatment to be optimised, although further studies are necessary to establish therapeutic effectiveness and toxicity ranges (AU)

Optimización de la función intestinal en pacientes con fibrosis quística mediante la administración de probióticos / Improvement of intestinal function in cystic fibrosis patients using probiotics

Introducción: La fibrosis quística puede cursar con inflamación de la mucosa intestinal y síndrome de hipercrecimiento bacteriano (SHB). Se ha argumentado que los probióticos actúan como inmunomoduladores, antiinflamatorios y reguladores de la microbiota. El objetivo del presente estudio es conocer la prevalencia de SHB en pacientes con fibrosis quística y tratar de optimizar la función intestinal mediante la administración de probióticos. Pacientes y método: Fueron valorados 20 pacientes afectados de fibrosis quística, con una edad media de 10,33 años (rango, 5-17 años). El estudio del SHB se efectuó en 10 pacientes mediante el test de hidrógeno espirado tras una sobrecarga de dextrosa al 20 %, a dosis de 2 g/kg. Tras la prueba se administró Lactobacillus rhamnosus LGG a dosis de1011 ufc dos veces al día durante 4 semanas. La determinación de grasa, nitrógeno, agua y azúcares en las heces se efectuó antes y después del tratamiento mediante análisis de reflexión de infrarrojos (FENIR). Resultados: Cinco pacientes (50%) presentaron SHB. En los valores de H2 se detectó una correlación positiva con respecto a las cifras de esteatorrea (R = 0,57) y de azúcares (R = 0,52). Los valores del FENIR pretratamiento frente a postratamiento, expresados en gramos, fueron: grasa 6,2 +/- 3,3 frente a 4,9 +/- 2,1 (p < 0,5), azúcares 6,7 +/- 3,6 frente a 5 +/- 2,6(p < 0,05) y nitrógeno 0,87 +/- 0,27 frente a 0,91 +/- 0,14 (NS). En 13 pacientes (81,25 %) se evidenció una mejoría de la comodidad intestinal y del aspecto de las deposiciones, yen 9 pacientes (56,25 %) disminuyó el número de deposiciones. Conclusiones: El tratamiento con probióticos mejora la función intestinal en los pacientes afectados de fibrosis quística desde el punto de vista clínico y bioquímico. Su administración podría ser pautada de una manera regular sobre todo en casos de SHB (AU)

Introduction: In some cases, cystic fibrosis may include intestinal inflammation and bacterial overgrowth. Probiotics are considered as immunomodulatory, anti-inflammatory and microbiotic regulator substances. The aim of our study is to determine the prevalence of bacterial overgrowth in cystic fibrosis patients and try to improve the intestinal function with the administration of probiotics. Patients and method: We examined 20 patients with cystic fibrosis (mean age10.33, range 5 to 17 years). The expired hydrogen test with a 2 g/kg of 20 % dextrose overload was performed on 10 patients. After the test, Lactobacillus rhamnosus LGG1011 CFU was administered twice daily for four weeks. Faecal near infrared spectroscopy (FENIR) of water, fat, nitrogen and sugar content in faeces was performed before and after probiotics administration. Results: Five patients (50 %) showed bacterial overgrowth. Weobtained a positive correlation between the hydrogen test and steator rhea (R = 0.57) and sugar in faeces (R = 0.52).The FENIR results pre-treatment vs post-treatment were: fat 6.2 g +/- 3.3 g vs. 4.9 g +/- 2.1 g (p < 0.05), sugar6.7 g +/- 3.6 g vs. 5 g +/- 2.6 g (p < 0.05) and nitrogen 0.87 g +/- 0.27 g vs. 0.91 g +/- 0.14 g (NS) respectively. Thirteen patients (81.25 %) had improved stool appearance and intestinal comfort and nine (56.25 %) decreased the number of daily stools. Conclusions: Probiotics improved not only clinical but also biochemical intestinal function in cystic fibrosis patients. These could be given as a regular treatment in this type of patients and in those with bacterial overgrowth (AU)

[Improvement of intestinal function in cystic fibrosis patients using probiotics]. / Optimización de la función intestinal en pacientes con fibrosis quística mediante la administración de probióticos.

INTRODUCTION: In some cases, cystic fibrosis may include intestinal inflammation and bacterial overgrowth. Probiotics are considered as immunomodulatory, anti-inflammatory and microbiotic regulator substances. The aim of our study is to determine the prevalence of bacterial overgrowth in cystic fibrosis patients and try to improve the intestinal function with the administration of probiotics. PATIENTS AND METHOD: We examined 20 patients with cystic fibrosis (mean age 10.33, range 5 to 17 years). The expired hydrogen test with a 2 g/kg of 20% dextrose overload was performed on 10 patients. After the test, Lactobacillus rhamnosus LGG 10(11) CFU was administered twice daily for four weeks. Faecal near infrared spectroscopy (FENIR) of water, fat, nitrogen and sugar content in faeces was performed before and after probiotics administration. RESULTS: Five patients (50%) showed bacterial overgrowth. We obtained a positive correlation between the hydrogen test and steatorrhea (R = 0.57) and sugar in faeces (R = 0.52). The FENIR results pre-treatment vs post-treatment were: fat 6.2 g +/- 3.3 g vs. 4.9 g +/- 2.1 g (p < 0.05), sugar 6.7 +/- g 3.6 g vs. 5 g +/- 2.6 g (p < 0.05) and nitrogen 0.87 g +/- 0.27 g vs. 0.91 g +/- 0.14 g (NS) respectively. Thirteen patients (81.25%) had improved stool appearance and intestinal comfort and nine (56.25%) decreased the number of daily stools. CONCLUSIONS: Probiotics improved not only clinical but also biochemical intestinal function in cystic fibrosis patients. These could be given as a regular treatment in this type of patients and in those with bacterial overgrowth.