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Introduction: Although changes in liver function tests can be non-specific in numerous clinical conditions, they can be the first sign of a potentially serious disease in an asymptomatic patient. Material and methods: Retrospective cohort study, performed by reviewing the records of children of a reference hospital central laboratory with alanine aminotransferase enzyme (ALT) elevation during a 6-month aleatory period. Results: 572 blood tests with serum ALT elevation corresponding to 403 patients have been assessed during the period studied. 98 patients were excluded for presenting abnormal liver test before the study period of comorbidity that could produce ALT elevation. The remaining 305 patients, 22.6% were diagnosed with a medical condition during the first blood test that explained the ALT elevation, although only 33.3% of them were followed up until verifying their normalisation. Final study sample consists of 236 patients with abnormal liver test without apparent liver disease. Adequate follow-up was found only in 29% of them. From this group, 9 patients (13%) were diagnosed with liver disease. The rest of the samples were not properly monitored. In patients with higher serum ALT levels, follow-up was early and more appropriate. Conclusions: In our area, most children without apparent liver disease are no properly monitored. Therefore, an opportunity to diagnosis and treat a potential liver disease was lost in a great number of children. All children with unexplained hypertransaminasaemia must be studied. Resumen: Introducción: Las alteraciones del perfil hepático constituyen un hecho inespecífico propio de numerosas condiciones clínicas. Sin embargo, puede implicar la primera manifestación de una patología potencialmente grave en un paciente asintomático. Material y métodos: Estudio observacional retrospectivo que incluye todas las analíticas sanguíneas con elevación de alanino aminotransferasa (ALT) en pacientes pediátricos solicitadas en un sector sanitario en un período de 6 meses. Resultados: Se registraron 572 analíticas correspondientes a 403 pacientes. Se excluyeron 98 pacientes con hipertransaminasemia ya conocida o comorbilidad. De los 305 restantes, el 22,6% se diagnosticaron de patología asociada a hipertransaminasemia, y de estos, se comprobó normalización en el 33,3%. De los 236 pacientes con hipertransaminasemia sin justificar se realizó un seguimiento en el 29%, encontrando patología hepática en 9 pacientes (13% del grupo). En el resto de la muestra no se comprobó analíticamente la evolución de las transaminasas ni la presencia de posible patología hepática. Los pacientes con cifras más elevadas se controlan mejor y antes que los que presentan cifras más bajas. Conclusiones: En nuestra área, la mayoría de los niños sin enfermedad hepática aparente con hallazgo de ALT elevada no son adecuadamente controlados. Esto hace que se pierda una oportunidad única de diagnosticar y tratar precozmente una enfermedad hepática potencial en un gran número de niños. Todo niño con hipertransaminasemia inexplicada debe ser estudiado.

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 · kg · day 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 · kg · day and 33 kcal · kg · day 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.

Introduction: Cerebral palsy (CP) is the most frequent cause of motor disability in the paediatric age. The aim of this article is the study of the nutritional status of patients with CP followed-up in a reference hospital, as well as the relationship between neurological and nutritional state. Material and methods: A cross-sectional, observational, descriptive and analytical study was conducted on a sample consisting of 4–15 year old patients with CP with Gross Motor Function Classification System (GMFCS) grades III–IV–V, from a specialised paediatric hospital reference area. An interview (collection of general data, medications and nutritional habits), anthropometric study and bioimpedance (BIA) measurements were carried out. Results: The study included 69 patients (recruitment 84.15%), with a mean age of 10.46 ± 0.43 years, and 50.7% females. The distribution according to GMFCS scale was: grade III (36.2%), grade IV (29%), and grade V (34.8%). According to weight for height: moderate malnutrition 21.8% (grade V: 33.3%), severe malnutrition 5.8% (grade V: 12.6%), overweight/obesity 23.2% (grade III: 24%, grade IV: 35%). Adequate level of lean mass for height: grade III (36%), grade IV (55%), and grade V (16.7%). Fat excess: grade III (36%), grade IV (40%), and grade V (29.2%). Fat mass comparison: BIA 6.89 ± 0.64 kg versus anthropometry 5.56 ± 4.43 kg. Conclusions: In CP grade GMFCS V, the weight deficit associated with a decrease in lean body mass is common. Patients with CP grades GMFCS III–IV have a significant prevalence of overweight/obesity. Anthropometry is a useful tool for nutritional assessment in children with CP, although fat levels could be underestimated. Resumen: Introducción: La parálisis cerebral (PC) es la causa más frecuente de discapacidad motora en edad pediátrica. El objetivo es estudiar la situación nutricional de los pacientes con PC controlados en una unidad de referencia, así como la relación entre la afectación neurológica y nutricional. Material y métodos: Estudio transversal, observacional, descriptivo y analítico en el que se han incluido pacientes con PC con grados III–IV–V (GMFCS) procedentes del área de influencia de un hospital pediátrico de referencia, con edades de 4–15 años. Se ha realizado: encuesta (con recogida de datos generales, medicaciones y hábitos nutricionales), estudio antropométrico y bioimpedanciometría (BIA). Resultados: N = 69 pacientes (reclutamiento 84,15%). Edad media 10,46 ± 0.43 años, el 50,7% mujeres. Distribución según GMFCS: grado III (36,2%), grado IV (29%), grado V (34,8%). Según el peso para la talla: desnutrición moderada el 21,8% (grado V: 33,3%), desnutrición grave el 5,8% (grado V: 12.6%), sobrepeso/obesidad el 23,2% (grado III: 24%; grado IV: 35%). Nivel adecuado de masa magra para su talla: grado III (36%), grado IV (55%), grado V (16,7%). Exceso de grasa: grado III (36%), grado IV (40%), grado V (29,2%). Comparación de masa grasa: BIA 6,89 ± 0,64 kg Vs antropometría 5,56 ± 4,43 kg. Conclusiones: En PC grado GMFCS V es frecuente el déficit de peso asociado a disminución de masa magra para su talla. Los pacientes con PC grados GMFCS III-IV presentan una prevalencia llamativa de sobrepeso/obesidad. La antropometría es una herramienta útil para la valoración nutricional en niños con PC, aunque los niveles de grasa tienden a infravalorarse.

Introduction: vitamin D maintains the concentration of calcium and phosphorus within the physiological range, allowing normal metabolism and bone mineralization. Recently, vitamin D deficiency has been related not only with rickets but also with an increased risk of other pathologies. The aim of this descriptive, observational, cross-sectional study was to assess vitamin D concentration levels in a healthy pediatric population, as well as the current situation of prophylaxis. Vitamin D determination was measured by serum 25-hydroxyvitamin D (25(OH)D) concentration. Methods: a total of 258 healthy patients between 3 months and 15 years of age were enrolled (6.77 ± 3.95 years; 73.6 % were male). Results: the mean value of 25-hydroxyvitamin D was 26.60 ng/mL ± 8.02 ng/mL, and up to 20.9 % of the population showed insufficient levels. Statistically significant differences in vitamin D levels were observed between ages (p = 0.002), ethnicity groups (p = 0.038), and skin types (p = 0.000). In addition, a higher prevalence of vitamin D insufficiency in children who had never previously received vitamin D supplementation (41.6 %) was observed compared to those that had taken supplementation in the first year of life (16.7 %). Conclusion: our study shows a high prevalence of vitamin D deficiency among healthy children, and the benefit of prophylaxis with vitamin D supplementation.Introducción: la vitamina D mantiene la concentración de calcio y fósforo dentro del rango fisiológico, permitiendo un metabolismo normal y la correcta mineralización de los huesos. Recientemente, la deficiencia de vitamina D se ha relacionado no solo con el raquitismo sino también con el aumento del riesgo de otras patologías. El objetivo de este estudio descriptivo, observacional y transversal fue conocer los niveles de concentración de vitamina D en una población pediátrica sana y la situación actual en cuanto a la profilaxis. La determinación de la vitamina D se midió mediante la concentración sérica de 25-hidroxivitamina D (25(OH)D). Material y métodos: se inscribieron 258 pacientes sanos de entre 3 meses y 15 años (6,77 ± 3,95 años; 73,6 % de hombres). Resultados: el valor medio de 25(OH)D fue de 26,60 ng/ml ± 8,02 ng/ml; el 20,9 % de la población mostró un nivel insuficiente. Se observaron diferencias estadísticamente significativas entre los niveles de vitamina D de las distintas edades (p = 0,002), grupos étnicos (p = 0,038) y fototipos (p = 0,000). Además, se observó una mayor prevalencia de la insuficiencia de vitamina D en los niños que nunca antes habían recibido suplementos de vitamina D (41,6 %) en comparación con los que habían tomado suplementos en el primer año de vida (16,7 %). Conclusiones: el presente estudio muestra una alta prevalencia del déficit de vitamina D en los niños sanos y el beneficio de una correcta profilaxis en edades tempranas con suplementos de vitamina D.

Introduction: The low FODMAP diet (fermentable oligosaccharides, monosaccharides, disaccharides, and polyols) has shown to be effective in adult patients with irritable bowel syndrome, but there are few studies on paediatric patients. The aim of this study is to assess the implementation and the outcomes of a low FODMAP diet in the treatment of functional abdominal pain in children from a Mediterranean area. Material and methods: A table was designed in which foods were classified according to their FODMAP content, as well as a ‘Symptoms and Stools Diary’. A prospective study was conducted on children with functional abdominal pain in our Paediatric Gastroenterology Unit. Results: A total of 22 patients were enrolled in the trial, and 20 completed it. Data were collected of the abdominal pain features over a period of 3 days, and then patients followed a two-week low FODMAP diet. Afterwards, information about abdominal pain features was collected again. After the diet, they showed fewer daily abdominal pain episodes compared to baseline (1.16 [IQR: 0.41–3.33] versus 2 [IQR: 1.33–6.33] daily episodes, P = .024), less pain severity compared to baseline (1.41 cm [IQR: 0.32–5.23] versus 4.63 cm [IQR: 2.51–6.39] measured by 10-cm Visual Analogue Scale, P = .035), less interference with daily activities, and less gastrointestinal symptoms. Only 15% of patients found it difficult to follow the diet. Conclusions: The implementation of a low FODMAP diet for 2 weeks in a Mediterranean paediatric population diagnosed with functional abdominal pain is possible with adapted diets. It was highly valued by patients, and they showed an improvement in abdominal pain symptoms assessed by objective methods. Resumen: Introducción: La dieta baja en FODMAP (acrónimo en inglés de polioles, monosacáridos, disacáridos y oligosacáridos fermentables) ha demostrado eficacia como tratamiento del síndrome de intestino irritable en adultos, siendo escasos los estudios en niños. Nuestro objetivo es analizar la implantación de esta dieta como tratamiento del dolor abdominal crónico funcional en población pediátrica de un área mediterránea, y su respuesta a esta. Material y métodos: Se elaboró una tabla clasificando los alimentos según su contenido en FODMAP, y se diseñó un «Diario de síntomas y deposiciones» para recoger los datos. Posteriormente se realizó un estudio prospectivo con niños con dolor abdominal crónico funcional de nuestra Unidad de Gastroenterología Pediátrica. Resultados: Se reclutaron 22 pacientes, 20 de los cuales completaron el estudio. Se recogieron durante 3 días datos sobre el dolor abdominal; posteriormente recibieron dieta baja en FODMAP 2 semanas, y al finalizarla recogieron de nuevo dichos datos. Tras la dieta se objetivó disminución en frecuencia diaria de episodios de dolor abdominal (1,16 [RIQ: 0,41-3,33] frente a 2 [RIQ: 1,33-6,33] inicialmente, p = 0,024), menor intensidad del dolor (1,41 cm [RIQ: 0,32-5,23] frente a 4,63 cm [RIQ: 2,51-6,39] inicial, p = 0,035, medido mediante Escala Visual Analógica de 10 cm), menor interferencia con la actividad diaria y menos síntomas acompañantes. Solo un 15% de los pacientes consideraron la dieta difícil. Conclusiones: La implantación de una dieta baja en FODMAP durante 2 semanas en una población pediátrica mediterránea con dolor abdominal crónico funcional es posible utilizando dietas adaptadas, es bien valorada por los pacientes, y su evaluación mediante herramientas objetivas muestra mejoría en los síntomas de dolor abdominal.

Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with mediumchain triglyceride, our patient presents clinical improvement until today. The pathogenesis of chronic diarrhea in patients with mucopolysaccharidosis type IIIB is still unknown. The Linfangiectasia intestinal en un paciente afectado de sindrome de Sanfilippo B Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome presence of intestinal lymphangiectasia in these patients should be investigated, and appropriate dietary treatment should be initiated, if confirmed, to improve their quality of life.

Multicenter study conducted in 15 hospitals including 101 COVID-19 pediatric inpatients aiming to describe associated gastrointestinal (GI) manifestations. GI symptoms were present in 57% and were the first manifestation in 14%. Adjusted by confounding factors, those with GI symptoms had higher risk of pediatric intensive care unit admission. GI symptoms are predictive of severity in COVID-19 children admitted to hospitals.

Bone health problems may be related to the nutritional deficit in pediatric patients with cerebral palsy. It is common to find asymptomatic vertebral fractures when they have low bone mineral density. Fat mass deficit could be related to a lower bone mineral density and a higher risk of vertebral fractures. To study the bone health of patients with CP and its relationship with neurological and nutritional status. Cerebral palsy (CP) is the most common cause of motor disability in pediatric age. Cross-sectional, observational, descriptive, and analytical study in which patients with CP between 4 and 5 years with Gross Motor Function Classification System (GMFCS) grades III–IV–V were included. It was carried out: survey, anthropometric study, bioimpedanciometry (BIA), and bone densitometry. Patients with low bone mineral density (BMD Z score less than − 2.0) underwent lumbar radiography looking for vertebral fractures to be diagnosed with osteoporosis. Total sample: 51 patients (51.0% women). Mean age: 11.0 ± 0.5 years. BMD Z score average: − 2.1 (95% CI − 2.5, − 1.7). BMD Z score according to GMFCS: grade III − 1.6 (− 2.2; − 1.), grade IV − 1.6 (− 2.4; − 0.9), grade V − 3.1 (− 3.9, − 2.2) (p = 0.013). Bone health classification according to the International Society for Clinical Densitometry was: 47.1% normal, 52.9% low BMD. Relationship between low BMD and low fat mass (p = 0.030) and low cell mass (p = 0.040) was found. Prevalence of vertebral fractures in lumbar radiography: 25.9%, increasing as the degree of neurological involvement. Vertebral fractures were found in 5/13 GMFCS grade V, 2/6 GMFCS grade IV, and 0/10 GMFCS grade III. Bone health in the pediatric population with CP is compromised in relation to the degree of neurological involvement and nutritional status. Those patients with moderate-severe cerebral palsy and low BMD seem to present an increased risk of fracture.

Eosinophilic oesophagitis is an emerging and chronic disorder mediated by the immune system, and is characterised by symptoms of oesophageal dysfunction and inflammation with isolated eosinophil infiltration in the oesophagus. It is more common in males and in atopic subjects, and the symptoms vary with age. In younger children, there is vomiting, abdominal pain and dietary problems, with dysphagia and food impaction in older children and adolescents. The diagnosis is based on the presence of symptoms and oesophageal inflammation with ≥15 eosinophils/high power field, and after ruling out other causes of oesophageal eosinophilia. Without treatment, the disease usually persists and can progress to fibrostenotic forms more common in adults. The treatment options included proton pump inhibitors, empirical elimination diets, and swallowed topical corticosteroids. Maintenance therapy is advisable after the induction treatment. Diet is the only treatment that is directed at the cause of the disease, on identifying the triggering food or foods. The response to the treatments requires a histological assessment due to the poor agreement between the symptoms and the oesophageal inflammation.The practical management of Eosinophilic oesophagitis presents with challenges, due to, among other causes, the current lack of availability of specific drugs, and its approach with, occasionally complex, diet treatments. The present document, prepared by the Working Group on Eosinophilic Gastrointestinal Disorders of the Spanish Society of Paediatric Gastroenterology, Hepatology and Nutrition, has as its objective to help in the diagnostic and therapeutic approach to paediatric eosinophilic oesophagitis, based on the recent evidence-based consensus guidelines. Resumen: La esofagitis eosinofílica es una enfermedad emergente, crónica, mediada por el sistema inmune y caracterizada por síntomas de disfunción esofágica e inflamación con infiltración eosinofílica aislada en el esófago. Es más frecuente en varones y en sujetos atópicos y los síntomas varían con la edad: en niños pequeños se manifiesta con vómitos, dolor abdominal y problemas con la alimentación y en niños mayores y adolescentes con disfagia e impactación alimentaria. El diagnóstico se basa en la presencia de síntomas e inflamación esofágica con ≥ 15 eosinófilos/campo de gran aumento, tras descartar otras causas de eosinofilia esofágica. Sin tratamiento, la enfermedad suele persistir y puede evolucionar a formas fibroestenóticas más frecuentes en el adulto. Las opciones terapéuticas incluyen inhibidores de la bomba de protones, dieta de eliminación empírica y corticoides deglutidos. Tras el tratamiento de inducción es aconsejable la terapia de mantenimiento. La dieta es el único tratamiento que se dirige a la causa de la enfermedad, al identificar los alimentos desencadenantes. La respuesta a los tratamientos requiere la evaluación histológica, por la escasa concordancia entre los síntomas y la inflamación esofágica.El manejo práctico de la esofagitis eosinofílica presenta desafíos debido, entre otras causas, a la falta de disponibilidad actual de fármacos específicos y a su abordaje con tratamientos dietéticos, en ocasiones, complejos. El presente documento, elaborado por el Grupo de Trabajo de Trastornos Gastrointestinales Eosinofílicos de la Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátricas, tiene como objetivo facilitar el abordaje diagnóstico y terapéutico de la esofagitis eosinofílica pediátrica, con base en las recientes guías de consenso basadas en la evidencia.

Introduction: Neonatal cystic fibrosis (CF) screening has enabled the disease to be diagnosed early, and is a determining factor in the increase in survival of these patients. Its main disadvantage is its low specificity and elevated number of false positives. The aim of this study is to analyse the differences in immunoreactive trypsin (IRT) between the different groups of newborns (NB) with a positive neonatal screen depending on whether they were healthy, healthy carriers, affected by CF, or CFSPID (Cystic Fibrosis Screen Positive, Inconclusive Diagnosis). Material: Retrospective analytical study of the concentrations of IRT in NB with a positive neonatal screen for CF born in a tertiary hospital during an 8-year period. Results: A total of 790 NB with a positive neonatal screen for CF were analysed. Of these 86.3% were term, 53% girls, and 11.8% were admitted. The mean IRT value was 79.16 ng/mL (range 60–367). Significantly higher concentrations of IRT were found in those affected by CF compared to the other groups (P

Background and Aims: Diagnostic delay (DD) is especially relevant in children with inflammatory bowel disease, leading to potential complications. We examined the intervals and factors for DD in the pediatric population of Spain. Methods: We conducted a multicentric prospective study, including 149 pediatric inflammatory bowel disease patients, obtaining clinical, anthropometric, and biochemical data. Time to diagnosis (TD) was divided into several intervals to identify those where the DD was longer and find the variables that prolonged those intervals. Missed opportunities for diagnosis (MODs) were also identified. Results: Overall TD was 4.4 months (interquartile range [IQR] 2.6-10.4), being significantly higher in Crohn's disease (CD) than in ulcerative colitis (UC) (6.3 [IQR 3.3-12.3] vs. 3 [IQR 1.6-5.6] months, p = 0.0001). Time from the visit to the first physician until referral to a pediatric gastroenterologist was the main contributor to TD (2.4 months [IQR 1.03-7.17] in CD vs. 0.83 months [IQR 0.30-2.50] in UC, p = 0.0001). One hundred and ten patients (78.3%) visited more than one physician (29.9% to 4 or more), and 16.3% visited the same physician more than six times before being assessed by the pediatric gastroenterologist. The number of MODs was significantly higher in CD than that in UC patients: 4 MODs (IQR 2-7) vs. 2 MODs ([IQR 1-5], p = 0.003). Referral by pediatricians from hospital care allowed earlier IBD diagnosis (odds ratio 3.2 [95% confidence interval 1.1-8.9], p = 0.025). Conclusions: TD and DD were significantly higher in CD than those in UC. IBD patients (especially those with CD) undergo a large number of medical visits until the final diagnosis.

Background and objective The objective of the study was to determine the ability of faecal calprotectin to differentiate functional and organic intestinal diseases in paediatric patients, and to evaluate the correlation between inflammatory parameters and levels of faecal calprotectin. Patients and methods This retrospective study involved clinical data from 129 paediatric patients with symptoms of intestinal pathology. Faecal calprotectin was determined by quantitative immunoassay. Patients were classified into three groups: functional (32.8% of patients); organic non-inflammatory bowel disease (IBD, 53.9%); and organic IBD (13.3%). Results Calprotectin levels were significantly different among the three groups; between patients with IBD and the others, and also between patients with non-organic IBD and functional. Positive associations were found between high levels of calprotectin and higher erythrocyte sedimentation rate (rho = 0.497), C-reactive protein (rho = 0.460), and platelet count (rho = 0.232). Nevertheless, an inverse correlation was found between high levels of calprotectin and transferrin saturation (rho = ∧0.310), albumin (rho = ∧0.412), and haemoglobin levels (rho = ∧0.309). Discussion Determination of faecal calprotectin is a complementary tool in clinical practice for discriminating between functional and organic IBD, avoiding, according to the levels of calprotectin, unnecessary invasive procedures in paediatric patients.

Objetivo: Analizar la presencia de rasgos de personalidad comunes y estado de ansiedad en niños y adolescentes con enfermedad inflamatoria intestinal (EII).Pacientes y Método: Estudio longitudinal, prospectivo y analítico mediante la aplicación de los cuestionarios Children’s Personality Questionnaire, High School Personality Questionnaire, State Trait Anxiety Inventory for Children y State Trait Anxiety Inventory a pacientes con EII con edad comprendida entre 9 y 18 años controlados en unidades de referencia en EII de Aragón, España. Se excluyeron aquellos que presentaban enfermedad activa, definido como una puntación ≥ 10 en el Pediatric Crohn Disease Activity Index (PCDAI Score) o ≥ 10 en el Pediatric Ulcerative Colitis Activity Index (PUCAI Score).Resultados: Participaron 26 pacientes (73% varones). El 61,5% presentaba enfermedad de Crohn (EC) y el 38,5% colitis ulcerosa (CU). Ninguno presentaba enfermedad activa. El perfil de personalidad como grupo se caracterizó por ser abierto, emocionalmente estable, calmoso, sobrio, sensato, emprendedor, impresionable, dependiente, sereno, perfeccionista y relajado. El 50% de los pacientes con EC eran emprendedores frente a ningún paciente con CU (p < 0,05). No hubo diferencias estadísticamente significativas al comparar el resto factores de personalidad en función del tipo de EII, edad ni sexo. Los pacientes con EC tienden a ser más calmados (p = 0,0511) y los pacientes con CU más introvertidos (p = 0,0549). La muestra presentaba un nivel de ansiedad estado (A/E) -1,1 ± 0,8 DS respecto a la media poblacional. El nivel de ansiedad como rasgo (A/R) era -0,6 ± 1 DS. Los varones tenían niveles significativamente menores que las mujeres en el caso de la A/E (p < 0,05).Conclusiones: Destaca la presencia de rasgos de personalidad comunes en la población pediátrica con EII pero no se ha constatado mayor ansiedad que en la población de referencia.

Inflammatory bowel disease in children has increased worldwide during the last decades. Clinical presentations are diverse and extraintestinal manifestations are the presenting sign in 6-35 % of patients, the most common of them being peripheral arthritis. An atypical clinical presentation results in diagnosis delay and, added to the greater seriousness of inflammatory bowel disease phenotypes in children, it entails more intestinal complications and sequelae. We describe two cases of inflammatory bowel disease with an initial symptom of lameness due to peripheral arthritis and enthesitis, respectively. © 2019 Sociedad Argentina de Pediatria. All rights reserved. La incidencia de enfermedad in amatoria intestinal en la edad pedia´trica se ha incrementado mundialmente en las u´ltimas de´cadas. La forma de presentacio´n puede ser diversa y, hasta en un 6-35 %, las manifestaciones extraintestinales pueden ser el debut; la artritis perife´rica es la ma´s frecuente de estas. Una presentacio´n ati´pica implica un retraso diagno´stico y, asociado a que el fenotipo de enfermedad in amatoria intestinal es ma´s grave en los nin~os, conlleva un incremento de las complicaciones intestinales y secuelas asociadas. Se presentan dos casos cli´nicos de enfermedad de Crohn cuya cli´nica inicial fue la claudicacio´n de la marcha por una artritis perife´rica y una entesitis, respectivamente.

Dubin-Johnson syndrome is a rare benign inherited disorder, caused by mutations in ABCC2 gen, and it is characterized by predominantly conjugated hyperbilirubinemia that can be increased by intercurrent infectious illnesses or surgical procedures. We report the case of a 10 year-old patient who showed, after a surgical procedure for peritonitis due to appendicitis, jaundice and predominantly conjugated hyperbilirubinemia, and he was diagnosed with Dubin-Johnson syndrome by genetic testing.

Introduction: Dysphagia is very common in children with neurological disabilities. These patients usually suffer from respiratory and nutritional problems. The videofluoroscopic swallowing study (VFSS) is the most recommended test to evaluate dysphagia, as it shows the real situation during swallowing. Objectives: To analyse the results obtained in our centre after one year of the implementation of VFSS, the clinical improvement after confirmation, and the prescription of an individualised treatment for the patients affected. Material and methods: VFSS performed in the previous were collected. The following variables were analysed: age, pathology, degree of neurological damage, oral and pharyngeal and/or oesophageal dysphagia and its severity, aspirations, prescribed treatment, and nutritional and respiratory improvement after diagnosis. A statistical analysis was performed using SPSS v21. Results: A total of 61 VFSS were performed. Dysphagia was detected in more than 70%, being moderate-severe in 58%. Aspirations and/or penetrations were recorded in 59%, of which 50% were silent. Adapted diet was prescribed to 56%, and gastrostomy was performed on 13 (21%) patients. A statistical association was found between neurological disease and severity of dysphagia. The degree of motor impairment is related to the presence of aspirations. After VFSS evaluation and treatment adjustment, nutritional improvement was found in Z-score of weight (+0.3SD) and BMI (+0.4SD). There was respiratory improvement in 71% of patients with dysphagia being controlled in the Chest Diseases Department. Conclusions: After implementation of VFSS, a high percentage of patients were diagnosed and benefited from a correct diagnosis and treatment. VFSS is a fundamental diagnostic test that should be included in paediatric centres as a diagnostic method for children with suspected dysphagia. Resumen: Introducción: La disfagia es muy frecuente en niños con discapacidad neurológica. Estos pacientes suelen presentar problemas respiratorios y nutricionales. El estudio de la deglución por videofluoroscopia (VFC) suele ser el más recomendado, ya que revela la situación real durante la deglución. Objetivos: Estudiar los resultados obtenidos en la evaluación diagnóstica tras un año desde la implantación de la VFC en nuestro centro, y analizar la mejoría clínica tras la confirmación por VFC y la prescripción de un tratamiento individualizado en los niños con disfagia orofaríngea. Material y métodos: Se recogen las VFC realizadas en el último año. Se analizan las siguientes variables: edad, enfermedad, grado de afectación neurológica, tipo de disfagia (oral, faríngea y/o esofágica), gravedad, aspiraciones y/o penetraciones, tratamiento prescrito y mejora nutricional y/o respiratoria tras el diagnóstico. Se realiza análisis estadístico mediante SPSS v21. Resultados: Se realizaron 61 VFC. Se detectó disfagia en más del 70%, siendo moderadas-graves en el 58%. Se visualizaron aspiraciones y/o penetraciones en el 59%, siendo silentes el 50%. Se prescribió dieta adaptada al 56% y gastrostomía en 13 pacientes (21%). Se encontró asociación estadística entre enfermedad neurológica y la gravedad de la disfagia, existiendo relación según el grado de afectación motora y la presencia de aspiraciones. Tras la evaluación por VFC y la adecuación del tratamiento se encontró una mejoría nutricional en Z-score de peso (+0,3 DE) e IMC (+0,4 DE) y una mejoría respiratoria en un 71% de los pacientes disfágicos controlados en Neumología. Conclusiones: Tras la implantación de la VFC se ha diagnosticado a un alto porcentaje de pacientes, que se han beneficiado de un diagnóstico y un tratamiento correctos. La VFC es una prueba diagnóstica fundamental que debería ser incluida en los centros pediátricos, como método diagnóstico de los niños con sospecha disfagia.