[Macroamylasaemia in paediatrics]. / Macroamilasemia en pediatría.

Macroamylasaemia should be considered in any patient with high plasma amylase, no clinical signs and negative additional investigations for pancreatic or parotid diseases. It is characterised by an increase in serum amylase due to circulating high molecular mass macrocomplexes, most often formed due the binding of the amylase to an immunoglobulin. With a normal renal function, a hyperamylasaemia without an increase in urine amylase suggests the diagnosis, and is confirmed by identifying the macromolecular components. It is an uncommon entity in paediatrics. It has been described as a casual finding associated to abdominal pain and to celiac disease. We report two paediatric cases of macroamylasaemia, and a review of the tests needed for its diagnosis. The better understanding of this biochemical anomaly allows us to differentiate it from other situations associated to hyperamylasaemia, in order to avoid additional invasive explorations and unnecessary treatments.

[Exogenous lipoid pneumonia]. / Neumonía lipoidea exógena.

The aspiration of lipoid material following the accidental ingestion of lipid formulations is the most frequent cause of exogenous lipoid pneumonia in paediatrics. The presence of cough, increasing dyspnea and chest pain, together with alveolar infiltrates in the chest radiography and the previous accidental intake of a lipid substance and vomiting should make us suspect this diagnosis. We present two cases of aspiration lipoid pneumonia in paediatric patients, with a different clinical presentation and radiological outcome, pointing out in one of them the appearance of pneumatoceles as a consequence of aspiration.

[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.

[Guidelines for the follow up of patients with bronchopulmonary dysplasia]. / Protocolo de seguimiento de los pacientes con displasia broncopulmonar.

Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth, and remains a major problem in pediatric pulmonology units. The decision of discharging from the Neonatal Unit should be based on a thorough assessment of the condition of the patient and compliance with certain requirements, including respiratory and nutritional stability, and caregiver education on disease management. For proper control of the disease, a schedule of visits and complementary tests should be established prior to discharge, and guidelines for prevention of exacerbations and appropriate treatment should be applied. In this paper, the Working Group in Perinatal Respiratory Diseases of the Spanish Society of Pediatric Pulmonology proposes a protocol to serve as a reference for the follow up of patients with BPD among different centers and health care settings. Key factors to consider when planning discharge from the Neonatal Unit and during follow up are reviewed. Recommendations on treatment and prevention of complications are then discussed. The final section of this guide aims to provide a specific schedule for follow-up and diagnostic interventions to be performed in patients with BPD.

[Respiratory treatments in neuromuscular disease]. / Tratamientos respiratorios en la enfermedad neuromuscular.

In a previous article, a review was presented of the respiratory pathophysiology of the patient with neuromuscular disease, as well as their clinical evaluation and the major complications causing pulmonary deterioration. This article presents the respiratory treatments required to preserve lung function in neuromuscular disease as long as possible, as well as in special situations (respiratory infections, spinal curvature surgery, etc.). Special emphasis is made on the use of non-invasive ventilation, which is changing the natural history of many of these diseases. The increase in survival and life expectancy of these children means that they can continue their clinical care in adult units. The transition from pediatric care must be an active, timely and progressive process. It may be slightly stressful for the patient before the adaptation to this new environment, with multidisciplinary care always being maintained.

Protocolo de seguimiento de los pacientes con displasia broncopulmonar / Guidelines for the follow up of patients with bronchopulmonary dysplasia

La displasia broncopulmonar (DBP) es la secuela más prevalente del recién nacido pretérmino, y sigue suponiendo un motivo frecuente de consulta en las unidades de Neumología Pediátrica. La decisión del alta de la unidad neonatal debe apoyarse en una valoración exhaustiva de la situación clínica del paciente y en el cumplimiento de unos requisitos, que incluyen la estabilidad respiratoria y nutricional, y la instrucción a los cuidadores en el manejo domiciliario. Para un control adecuado de la enfermedad, es necesario que quede establecido, previamente al alta, un calendario de visitas y de exploraciones complementarias, y deben aplicarse las pautas de prevención de exacerbaciones y el tratamiento apropiados. El concepto de DBP como enfermedad multisistémica es fundamental en el seguimiento de los pacientes y debe ser tenido en cuenta para un buen control de la enfermedad. En este documento, el Grupo de Trabajo de Patología Respiratoria Perinatal de la Sociedad Española de Neumología Pediátrica propone un protocolo que sirva como referencia para unificar el seguimiento de los pacientes con DBP entre los diferentes centros y ámbitos asistenciales. Se revisan los aspectos a tener en cuenta en la evaluación previa al alta de la Unidad Neonatal y las principales complicaciones durante el seguimiento. Seguidamente, se detallan las recomendaciones en materia de tratamiento de la enfermedad y prevención de complicaciones, los controles tras el alta y su cronología

Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth, and remains a major problem in pediatric pulmonology units. The decision of discharging from the Neonatal Unit should be based on a thorough assessment of the condition of the patient and compliance with certain requirements, including respiratory and nutritional stability, and caregiver education on disease management. For proper control of the disease, a schedule of visits and complementary tests should be established prior to discharge, and guidelines for prevention of exacerbations and appropriate treatment should be applied. In this paper, the Working Group in Perinatal Respiratory Diseases of the Spanish Society of Pediatric Pulmonology proposes a protocol to serve as a reference for the follow up of patients with BPD among different centers and health care settings. Key factors to consider when planning discharge from the Neonatal Unit and during follow up are reviewed. Recommendations on treatment and prevention of complications are then discussed. The final section of this guide aims to provide a specific schedule for follow-up and diagnostic interventions to be performed in patients with BPD

Tratamientos respiratorios en la enfermedad neuromuscular / Respiratory treatments in neuromuscular disease

Revisadas en un artículo anterior la fisiopatología respiratoria del enfermo neuromuscular (ENM), así como su evaluación clínica y las principales complicaciones causantes de su deterioro pulmonar, en el presente artículo se describen los tratamientos respiratorios necesarios para preservar la función pulmonar del ENM durante el mayor tiempo posible, así como en situaciones especiales (infecciones respiratorias, cirugía de escoliosis, etc.). Se hace especial hincapié en la utilidad de la ventilación no invasiva cuyo uso está cambiando la historia natural de muchas de estas enfermedades. La prolongación de la vida en estos niños permite que lleguen a las unidades de neumología de adultos para proseguir su atención. La transición desde la pediatría debe ser un proceso activo, progresivo en el tiempo y poco estresante para el paciente ante la adaptación a ese nuevo entorno, manteniendo siempre una atención multidisciplinar

In a previous article, a review was presented of the respiratory pathophysiology of the patient with neuromuscular disease, as well as their clinical evaluation and the major complications causing pulmonary deterioration. This article presents the respiratory treatments required to preserve lung function in neuromuscular disease as long as possible, as well as in special situations (respiratory infections, spinal curvature surgery, etc.). Special emphasis is made on the use of non-invasive ventilation, which is changing the natural history of many of these diseases. The increase in survival and life expectancy of these children means that they can continue their clinical care in adult units. The transition from pediatric care must be an active, timely and progressive process. It may be slightly stressful for the patient before the adaptation to this new environment, with multidisciplinary care always being maintained

Trasplante pediátrico pulmonar / Pediatric lung transplantation

El trasplante pulmonar constituye la última alternativa para los niños con una enfermedad pulmonar terminal cuando se han agotado todo los recursos terapéuticos. En las dos últimas décadas se ha experimentado una vance consideable en la supervivencia de lso niños trasplantados de pulmón, por las mejoras en la técnica del trasplante, la preervación de los órganos, el manejo perioperatorio y la inmunosupresión. El programa de trasplatne pulmonar pediátrico en el Hospital Universitari Vall d´Hebron se inició en el año 1996. En nuestro hospital se han realizado 672 trasplantes entre los años 1990 y 2012, de los que 51 (7,6%) corresponde a trasplantes pediátricos (menores de 18 años). La indicación más frecuente para el trasplante en los iños es la fibrosis quística. En nuestro centro, la fibrosis quística ha representado solo el 8,7% de los traplantes realizados enmenores de 11 años y el 86% de los realizados entre 12 y 17 años. Los otros grupos importantes de indicaciones los constituyen las enfermedades vasculares pulmonares (hipertensión pulmonar) y las neumopatías intersticiales crónicas. La superviviencia de los niños trasplantados en nuestro entro es similar a la reflejada en el registro internacional: 55% a los 5 años y 47% a los 7 años (AU)

Lung trasplantation has become an accepted therapeutic option for children with end-stage lung disease. Advances have been made over the last 2 decades in lung transplantation concerning the procurement and presevation of donor lung, surgical techniques, early post-operative care and immunosuppression. The pediatric lung transplant program at Hospital Universitari Vall d´Heborn was started in 1996. A total of 672 lung tansplants have been performed in our hospital since 1990, with 51 (7.6%) corresponding to children (younger than 18 years). Cystic fibrosis is the leading cause for pediatric patients to requere lung transplantation. In our hospital cystic fibrosis represent only 8.7% of lung transplants in children less tha 11 years-old, but 86% in children 12-17 years old. Other important indications for lung trnasplantation in children are pediatric forms of interstitial lung dieases and pulmonary vascular disorders (mainly pulmonary hypertension). Actuarial survival is our center (55% at 5 years and 47% at 7 years) is similar to survival published in the International Registry (AU)

Macroamilasemia en pediatría / Macroamylasaemia in paediatrics

La macroamilasemia es una entidad que debe sospecharse ante cualquier paciente que presente concentraciones elevadas de amilasa plasmática sin datos clínicos ni de investigaciones complementarias que demuestren la existencia de una afección pancreática o parotídea. Se caracteriza por la elevación de amilasa plasmática debido a macrocomplejos circulantes de alto peso molecular, formados por una molécula de amilasa unida generalmente a una inmunoglobulina. En ausencia de enfermedad renal, una hiperamilasemia sin aumento de amilasuria orienta hacia este diagnóstico, que se confirma al identificar a los componentes de la macromolécula. Es una entidad infrecuente en pediatría. Se ha descrito como un hallazgo casual asociado a dolor abdominal y a enfermedad celíaca. Se presentan 2 casos pediátricos de macroamilasemia, así como las pruebas necesarias para su diagnóstico. El conocimiento de esta anomalía bioquímica permite distinguirla de otras situaciones que cursan con elevación de amilasa, con el fin de evitar exploraciones complementarias y tratamientos invasivos innecesarios (AU)

Macroamylasaemia should be considered in any patient with high plasma amylase, no clinical signs and negative additional investigations for pancreatic or parotid diseases. It is characterised by an increase in serum amylase due to circulating high molecular mass macrocomplexes, most often formed due the binding of the amylase to an immunoglobulin. With a normal renal function, a hyper-amylasaemia without an increase in urine amylase suggests the diagnosis, and is confirmed by identifying the macromolecular components. It is an uncommon entity in paediatrics. It has been described as a casual finding associated to abdominal pain and to celiac disease. We report two paediatric cases of macroamylasaemia, and a review of the tests needed for its diagnosis. The better understanding of this biochemical anomaly allows us to differentiate it from other situations associated to hiperamylasaemia, in order to avoid additional invasive explorations and unnecessary treatments (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)

Neumonía lipoidea exógena / Exogenous lipoid pneumonia

La broncoaspiración de sustancias lipídicas tras la ingesta accidental de productos con un elevado contenido en lípidos es la causa más frecuente de neumonía lipoidea exógena en pediatría. La presencia de tos, dificultad respiratoria creciente y dolor torácico, junto con infiltrados alveolares en la radiografía de tórax y el antecedente claro de intoxicación y vómitos deben hacer sospechar este diagnóstico. Presentamos dos casos clínicos de neumonía lipoidea por aspiración en pacientes pediátricos, que difieren en su evolución clínica y radiológica. En uno de ellos destaca la aparición de neumatoceles como secuela de la aspiración (AU)

The aspiration of lipoid material following the accidental ingestion of lipid formulations is the most frequent cause of exogenous lipoid pneumonia in paediatrics. The presence of cough, increasing dyspnea and chest pain, together with alveolar infiltrates in the chest radiography and the previous accidental intake of a lipid substance and vomiting should make us suspect this diagnosis. We present two cases of aspiration lipoid pneumonia in paediatric patients, with a different clinical presentation and radiological outcome, pointing out in one of them the appearance of pneumatoceles as a consequence of aspiration (AU)