Dysphagia in patients undergoing esophageal atresia surgery: Assessment using a functional scale. / Disfagia en pacientes intervenidos de atresia de esófago: valoración con una escala funcional.

INTRODUCTION: Dysphagia is defined as difficulty swallowing. Up to 84% of patients undergoing esophageal atresia surgery have dysphagia beyond the neonatal period. MATERIALS AND METHODS: A retrospective study of patients undergoing esophageal atresia surgery from 2005 to 2021 was carried out. The Functional Oral Intake Scale (FOIS) was used to assess dysphagia in 4 age groups (< 1 year old, 1-4 years old, 5-11 years old, and > 11 years old). FOIS scores < 7 or symptoms of choking, impaction, or food aversion were regarded as dysphagia. RESULTS: 63 patients were analyzed. 74% (47/63) had dysphagia during follow-up. Prevalence was 50% in patients < 1 year old (FOIS mean 4.32), 77% in patients aged 1-4 (FOIS mean 5.61), 45% in patients aged 5-11 (FOIS mean 5.87), and 38% in patients > 11 years old (FOIS mean 6.8). The most frequent causes of dysphagia were stenosis, which occurred in 38% of the patients (n=24), and gastroesophageal reflux (n=18), which was present in 28% of the patients. Both conditions were associated with significantly lower mean FOIS scores (p< 0.05) in the patients under 11 years of age. Differences (p< 0.05) were found in the dysphagia-associated perinatal factors in the various age groups, with longer ventilation assistance times, parenteral nutrition, and hospital stays. CONCLUSIONS: Dysphagia is an extremely frequent symptom at any given age in patients undergoing esophageal atresia surgery. A standardized, cross-disciplinary follow-up is key to improve quality of life.

INTRODUCCION: La disfagia se define como dificultad en el proceso de alimentación. Hasta un 84% de pacientes intervenidos de atresia de esófago tienen disfagia más allá del periodo neonatal. MATERIAL Y METODOS: Estudio retrospectivo de serie de casos intervenidos por atresia de esófago 2005-2021. Se utilizó la escala FOIS (Functional Oral Intake Scale) para cuantificar la disfagia en 4 grupos de edad (menores de 1 año, 1-4 años, 5-11 años y mayores de 11 años). Se consideró disfagia cualquier valor de FOIS < 7 o síntomas de atragantamiento, impactación o aversión alimentaria. RESULTADOS: Se obtuvieron datos de 63 pacientes. El 74% (47/63) presentó disfagia durante el seguimiento. La prevalencia fue del 50% < 1 año (media FOIS 4.32), 77% 1-4 años (media FOIS 5.61), 45% 5-11 años (media FOIS 5.87) y 38% > 11 años (media FOIS 6.8). Las causas más frecuentes de disfagia fueron la estenosis, que presentó un 38% de los pacientes (n= 24) y el reflujo gastroesofágico (n= 18), que presentó a su vez un 28% de los pacientes. Ambas condiciones se asociaron con unos valores medios de FOIS significativamente menores (p< 0,05) en los pacientes menores de 11 años. Se encontraron diferencias (p< 0,05) en factores perinatales asociados a disfagia en los distintos periodos de edad, a destacar mayor tiempo medio de: asistencia ventilatoria, nutrición parenteral e ingreso hospitalario. CONCLUSIONES: La disfagia es un síntoma extremadamente frecuente a cualquier edad en los pacientes intervenidos de atresia de esófago. Un seguimiento estandarizado y multidisciplinar es esencial para mejorar la calidad de vida de estos pacientes.

Disfagia en pacientes intervenidos de atresia de esófago: valoración con una escala funcional / Dysphagia in patients undergoing esophageal atresia surgery: Assessment using a functional scale

Introducción: La disfagia se define como dificultad en el procesode alimentación. Hasta un 84% de pacientes intervenidos de atresia deesófago tienen disfagia más allá del periodo neonatal.Material y métodos: Estudio retrospectivo de serie de casos intervenidos por atresia de esófago 2005-2021. Se utilizó la escala FOIS(Functional Oral Intake Scale) para cuantificar la disfagia en 4 gruposde edad (menores de 1 año, 1-4 años, 5-11 años y mayores de 11 años).Se consideró disfagia cualquier valor de FOIS < 7 o síntomas de atragantamiento, impactación o aversión alimentaria.Resultados: Se obtuvieron datos de 63 pacientes. El 74% (47/63)presentó disfagia durante el seguimiento. La prevalencia fue del 50%< 1 año (media FOIS 4.32), 77% 1-4 años (media FOIS 5.61), 45% 5-11años (media FOIS 5.87) y 38% > 11 años (media FOIS 6.8). Las causasmás frecuentes de disfagia fueron la estenosis, que presentó un 38% delos pacientes (n= 24) y el reflujo gastroesofágico (n= 18), que presentóa su vez un 28% de los pacientes. Ambas condiciones se asociaron conunos valores medios de FOIS significativamente menores (p< 0,05) enlos pacientes menores de 11 años. Se encontraron diferencias (p< 0,05)en factores perinatales asociados a disfagia en los distintos periodosde edad, a destacar mayor tiempo medio de: asistencia ventilatoria,nutrición parenteral e ingreso hospitalario. Conclusiones: La disfagia es un síntoma extremadamente frecuentea cualquier edad en los pacientes intervenidos de atresia de esófago. Unseguimiento estandarizado y multidisciplinar es esencial para mejorarla calidad de vida de estos pacientes(AU)

Introduction: Dysphagia is defined as difficulty swallowing. Up to84% of patients undergoing esophageal atresia surgery have dysphagiabeyond the neonatal period. Materials and methods: A retrospective study of patients undergoing esophageal atresia surgery from 2005 to 2021 was carried out. TheFunctional Oral Intake Scale (FOIS) was used to assess dysphagia in 4age groups (< 1 year old, 1-4 years old, 5-11 years old, and 11 years old). FOIS scores < 7 or symptoms of choking, impaction, or food aversionwere regarded as dysphagia. Results: 63 patients were analyzed. 74% (47/63) had dysphagiaduring follow-up. Prevalence was 50% in patients < 1 year old (FOISmean 4.32), 77% in patients aged 1-4 (FOIS mean 5.61), 45% in patientsaged 5-11 (FOIS mean 5.87), and 38% in patients > 11 years old (FOISmean 6.8). The most frequent causes of dysphagia were stenosis, whichoccurred in 38% of the patients (n=24), and gastroesophageal reflux(n=18), which was present in 28% of the patients. Both conditions wereassociated with significantly lower mean FOIS scores (p< 0.05) in thepatients under 11 years of age. Differences (p< 0.05) were found in thedysphagia-associated perinatal factors in the various age groups, withlonger ventilation assistance times, parenteral nutrition, and hospital stays. Conclusions: Dysphagia is an extremely frequent symptom at anygiven age in patients undergoing esophageal atresia surgery. A standardized, cross-disciplinary follow-up is key to improve quality of life.(AU)

Protocolo de actuación en atención primaria en la enfermedad inflamatoria intestinal en la edad pediátrica / Protocol of action in primary care in the pediatric inflammatory bowel disease

Dadas las características de la enfermedad inflamatoria intestinal de los pacientes pediátricos y las diferencias clínicas, es muy importante realizar un diagnóstico y tratamiento lo más precoz posible. El retraso en el inicio del tratamiento implica una menor respuesta al mismo y una evolución hacia una mayor gravedad. El trabajo conjunto desde los centros de salud y los niveles hospitalarios siempre resulta beneficioso para el paciente, a la vez que ayuda y acerca a los profesionales. En la literatura médica no se han documentado muchos protocolos dirigidos al diagnóstico precoz y el manejo de esta patología en pacientes pediátricos en atención primaria en coordinación con la atención hospitalaria

An early diagnosis and treatment in the pediatric inflammatory bowel disease is essential. Pediatric patients have special age-related features that make them different to de adults. Delayed beginning treatment implies a lower response and evolution towards a higher severity. A delayed treatment implies lower response and a worse illness outcome. Coordination between primary health care and tertiary care centers is mandatory to benefit the patients and to get better outcomes. This approach in the pediatric patients is not described in scientific papers

Análisis de la calidad de vida en los pacientes afectos de escoliosis vertebral / Analysis of the quality of life in patients affected by scoliosis

Objetivos: Analizar la calidad de vida mediante el test SRS 22 en los pacientes con escoliosis de 20 o más grados Cobb. Material y método: Estudio descriptivo prospectivo realizado a pacientes con escoliosis de al menos 20 grados Cobb y con edad comprendida entre 10 y 20 años. Fue realizado entre abril y mayo de 2016.Se les registró peso, talla, índice de masa corporal y cuestionario SR 22, que es un cuestionario específico de calidad de vida para pacientes con escoliosis. Los pacientes se dividieron en 2 grupos para su análisis: a) escoliosis entre 20 y 29 grados Cobb (n=44); y b) escoliosis de 30 grados Cobb o superior (n=32). Resultados: Existen diferencias significativas en las dimensiones que evalúan el dolor, la autopercepción de la imagen y la satisfacción del tratamiento, siendo peor valoradas cuando el grado de escoliosis es de 30 grados Cobb o superior. No hay diferencias significativas en la función/actividad ni en la salud mental. La puntuación global del cuestionario también fue peor en el grupo con mayor grado de escoliosis. El peso, talla e índice de masa corporal no han mostrado diferencias significativas según el mayor o menor grado de escoliosis. Conclusiones: La escoliosis afecta de forma importante a la calidad de vida de las personas que la padecen, existiendo una correlación negativa entre la gravedad de la escoliosis medida mediante grados Cobb y la calidad de vida

Objectives: To assess the quality of life using the SRS 22 test in patients with scoliosis of 20 or more degrees Cobb. Material and methods: A prospective descriptive study was conducted between April and May 2016 on patients with scoliosis of at least 20 degrees Cobb and aged between 10 and 20 years. A record was made of weight, height, body mass index, and the SR 22 specific quality of life questionnaire for patients with scoliosis was completed. Patients were divided into two groups for analysis: a) scoliosis between 20 and 29 degrees Cobb (n=44); and b) scoliosis with a Cobb of 30 degrees or greater (n=32). Results: There were significant differences in the dimensions that assess pain, image self-perception, and satisfaction with treatment, being valued worse when the degree of scoliosis Cobb is 30 degrees or higher. There were no significant differences in function/activity or mental health. The overall score of the questionnaire was also worse in the group with the highest degree of scoliosis. The weight, height, and BMI showed no significant differences due to the varying degrees of scoliosis. Conclusions: Scoliosis significantly affects the quality of life of people who suffer it, and there is a negative correlation between the severity of scoliosis measured by degrees Cobb and quality of life

[Analysis of the quality of life in patients affected by scoliosis]. / Análisis de la calidad de vida en los pacientes afectos de escoliosis vertebral.

OBJECTIVES: To assess the quality of life using the SRS 22 test in patients with scoliosis of 20 or more degrees Cobb. MATERIAL AND METHODS: A prospective descriptive study was conducted between April and May 2016 on patients with scoliosis of at least 20 degrees Cobb and aged between 10 and 20 years. A record was made of weight, height, body mass index, and the SR 22 specific quality of life questionnaire for patients with scoliosis was completed. Patients were divided into two groups for analysis: a) scoliosis between 20 and 29 degrees Cobb (n=44); and b) scoliosis with a Cobb of 30 degrees or greater (n=32). RESULTS: There were significant differences in the dimensions that assess pain, image self-perception, and satisfaction with treatment, being valued worse when the degree of scoliosis Cobb is 30 degrees or higher. There were no significant differences in function/activity or mental health. The overall score of the questionnaire was also worse in the group with the highest degree of scoliosis. The weight, height, and BMI showed no significant differences due to the varying degrees of scoliosis. CONCLUSIONS: Scoliosis significantly affects the quality of life of people who suffer it, and there is a negative correlation between the severity of scoliosis measured by degrees Cobb and quality of life.

Impacto de la instauración de un protocolo de actuación clínica ante el dolor abdominal en urgencias pediátricas / Impact of establishment of a clinical protocol for abdominal pain in pediatric emergencies

Objetivos: En enero de 2013 se implantó un protocolo de actuación para el manejo del dolor abdominal en el servicio de urgencias de pediatría, que pretendía reducir la realización de radiografías de abdomen no indicadas, disminuir la administración de enemas no indicados y aplicar una pauta de analgesia mayor. Se ha revisado el impacto del protocolo sobre la modificación de la práctica clínica y si estas variaciones se mantienen. Material y métodos: Estudio observacional, descriptivo, analítico y retrospectivo, realizado en 684 pacientes que acudieron al servicio de urgencias por presentar dolor abdominal de causa aparentemente no orgánica, distribuidos en cuatro periodos: diciembre de 2012 (P1), febrero de 2013 (P2), noviembre de 2013 (P3) y mayo de 2015 (P4). Se han recogido los datos sobre las pruebas diagnósticas realizadas y los tratamientos empleados en estos pacientes. Resultados: Radiografía abdominal: P1= 14,7%, P2= 6,9%, P3= 1,8%, P4= 0% (p <0,01); pacientes con estreñimiento: P1= 23,4%, P2= 13,5%, P3= 0%, P4= 0% (p= 0,001). Ecografía abdominal: P1= 11%, P2= 12,5%, P3= 9,4%, P4= 10,1% (p >0,05). Administración de enema en el servicio de urgencias: P1= 21,5%, P2= 8,3%, P3= 17,1%, P4= 11,7% (p= 0,005); pacientes con estreñimiento: P1= 51,1%, P2= 21,6%, P3= 31,3%, P4= 32,5% (p= 0,036). Tratamiento con polietilenglicol: P1= 4,3%, P2= 6,3%, P3= 9,8%, P4= 4,7% (p >0,05); pacientes con estreñimiento: P1= 12,8%, P2= 21,6%, P3= 40,6%, P4= 20,5% (p= 0,034). Pauta de analgesia: P1= 42,9%, P2= 53,5%, P3= 53,7%, P4= 62,7% (p= 0,02). Reconsultas al servicio de urgencias: P1= 10,4%, P2= 2,1%, P3= 1,8%, P4= 13% (p <0,01). Conclusiones: El protocolo ha logrado reducir de forma significativa la realización de radiografías abdominales y el empleo de enemas rectales. Ha aumentado la prescripción de analgesia en pacientes con dolor abdominal. El protocolo ha logrado homogeneizar la actuación de los profesionales, disminuyendo el riesgo de yatrogenia y aumentando el confort de los pacientes y sus familias (AU)

Objectives: In January 2013 a protocol for the management of abdominal pain at pediatric emergency was implanted, with the following objectives: reducing abdominal radiographs not indicated, reduce treatment with enemas not indicated and more prescription of analgesia. It has been reviewed the impact of the protocol on changing clinical practice and whether these variations are maintained. Material and methods: Retrospective, descriptive and analytical observational study with 684 patients attending emergency department for abdominal pain apparently no organic cause, divided into four periods: December 2012 (P1), February 2013 (P2), November 2013 (P3) and May 2015 (P4). We collected data about diagnostic tests performed and treatments used in these patients. Results: Abdominal radiography: P1= 14.7%, P2= 6.9%, P3= 1.8%, P4= 0% (p <0.01); constipated patients: P1= 23.4%, P2= 13.5%, P3= 0%, P4= 0% (p= 0.001). Abdominal ultrasound: P1= 11%, P2= 12.5%, P3= 9.4%, P4= 10.1% (p >0.05). Patients treated with rectal enema: P1= 21.5%, P2= 8.3%, P3= 17.1%, P4= 11.7% (p= 0.005); constipated patients: P1= 51.1%, P2= 21.6%, P3= 31.3%, P4= 32.5% (p= 0.036). Patients treated with polyethylenglycol: P1= 4.3%, P2= 6.3%, P3= 9.8%, P4= 4.7% (p >0.05); constipated patients: P1= 12.8%, P2= 21.6%, P3= 40.6%, P4= 20.5% (p= 0.034). Patients treated with analgesia: P1= 42.9%, P2= 53.5%, P3= 53.7%, P4= 62.7% (p= 0.02). Reconsultations the emergency department: P1= 10.4%, P2= 2.1%, P3= 1.8%, P4= 13% (p <0.01). Conclusions: The protocol has reduced significantly the performance of abdominal radiographs and use of rectal enemas. It has increased the prescription of analgesia in patients with abdominal pain. Protocol has managed to standardize the medical intervention, reducing the risk of iatrogenic and increasing comfort for patients and their families (AU)

La Unidad de Gastroenterología, Hepatología y Nutrición Pediátrica del Hospital Infantil Universitario Miguel Servet / Pediatric Gastroenterology, Hepatology and Nutrition Unit of the Hospital Infantil Universitario Miguel Servet

La gastroenterologia pediátrica es una especialidad amplia que incluye las patologías del tubo digestivo, pancreas e hígado, ademas de la nutrición infantil. En los ultimos años, ha adquirido relevancia la realización de técnicas complementarias como la endoscopia digestiva, la videofluoroscopia y el analisis corporal por impedanciometría. La Unidad de Gastroenterologia, Hepatologia y Nutrición Pediátrica del Hospital Infantil Miguel Servet es Unidad de referencia de la Comunidad de Aragón. En este artículo se refleja la estructura, pruebas complementarias y la actividad asistencial, docente e investigadora (AU)

Pediatric Gastroenterology and Nutrition is an intricate speciality that involves the gastrointestinal tract, liver and pancreas pathologies, but also, the children’s nutrition. In our speciality, every year it’s becoming more important to perform examination procedures, as endoscopy, videofluoroscopy or bioelectrical impedance analysis. The Miguel Servet Children’s Hospital Pediatric Gastroenterology, Hepatology and Nutrition Unit is a reference unit in the Aragon Community. This paper shows the Unit structure, the examination procedures and our teaching and research activity (AU)

La Unidad de Neumología Pediátrica y Fibrosis Quística del Hospital Infantil Universitario Miguel Servet / Pediatric pneumology and cystic fibrosis of the Hospital Infantil Universitario Miguel Servet

Se describe en este artículo la estructura, actividad asistencial y docente de la Unidad de Neumología Pediátrica y Fibrosis Quística del Hospital Infantil Miguel Servet, dedicada integramente al cuidado de los niños con enfermedades respiratorias. Es la Unidad de referencia para niños con problemas respiratorios de mayor complejidad en toda la Comunidad de Aragón, en especial para pacientes con asma de difícil control o fibrosis quística, asi como para la realización de diferentes técnicas específicas, como la fibrobroncoscopia (AU)

This article describes the structure, clinical and teaching activity of Pediatric Pulmonology and Cystic Fibrosis Unit in the Children’s Hospital Miguel Servet, entirely dedicated to the care of children with respiratory diseases. It is the reference Unit for children with severe respiratory problems in the community of Aragon, especially for patients with difficult asthma or cystic fibrosis, and also to perform different specific techniques, such as flexible bronchoscopy (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.

Assessment of plasma ammonia and glutamine concentrations in urea cycle disorders.

OBJECTIVES: To analyze the association between ammonia and glutamine used for metabolic control in inherited urea cycle disorders (UCD) in a large series of patients. DESIGN AND METHODS: Paired plasma amino acid-ammonia data from 26 UCD patients were analyzed (n=921). RESULTS: Increased plasma glutamine values were consistently observed in UCD patients, despite normal plasma ammonia concentrations, especially for mitochondrial UCD. CONCLUSIONS: Further therapeutic efforts are probably needed to control increased glutamine values, considering their potentially neurotoxic effect.

[Autoimmune hepatitis in pediatric patients]. / Hepatitis autoinmune en la edad pediátrica.

BACKGROUND: Autoimmune hepatitis (AIH) is an inflammatory disease of unknown origin that is responsible for progressive liver necrosis and ultimately cirrhosis. OBJECTIVE: Our aim was to evaluate the characteristics of autoimmune hepatitis presenting in the pediatric age. MATERIAL AND METHODS: We conducted a retrospective study of all patients diagnosed with AIH in our hospital department during the last 10 years. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology, treatment, need for transplant, and clinical evolution. According to the positive level of auto-antibodies, AIH patients were classified as type I AIH (ANA and/or smooth-muscle antibodies) and type II (anti-LKM-1). RESULTS: Seven patients were diagnosed in this period -5 girls (71.5%) and 2 boys (28.5%). Five patients presented with type-I serological markers, and two with type-II markers. Age range at diagnosis was from 21 months to 12 years. In the type-I group, 3 patients presented with acute hepatitis while 2 other patients were diagnosed from laboratory findings while asymptomatic. Elevated aminotransferase (10 times the normal level) was observed in 71.5%, and 85% had elevated immunoglobulins. Treatment with azathioprine and prednisone was started after diagnosis with an average time to remission of 14 months. Two patients relapsed following steroid withdrawal. CONCLUSION: AIH can have different forms of clinical presentation, and is sometimes indistinguishable from viral hepatitis. AIH must be ruled out in patients presenting with concomitant elevation of aminotransferases and immunoglobulins. The commonly accepted treatment is a combination of azathioprine and corticosteroids. A high percentage of patients experience a relapse of disease after steroids are withdrawn. Therefore, some patients will need to stay on combined therapy with minimal doses of steroids.

Hepatitis autoinmune en la edad pediátrica / Autoimmune hepatitis in pediatric patients

Introducción: la hepatitis autoinmune es una enfermedad inflamatoria de origen desconocido responsable de una destrucción progresiva del hígado y evolución hacia la cirrosis. Objetivo: el objetivo es evaluar las características de las hepatitis autoinmunes en la población infantil. Material y métodos: estudio retrospectivo de pacientes diagnosticados en nuestro servicio en los últimos 10 años. Las variables analizadas son: edad, sexo, forma de presentación, función hepática, inmunoglobulinas, autoinmunidad, histología, tratamiento, necesidad de trasplante y evolución clínica. Según la positividad de los auto-anticuerpos se clasifican en tipo I (ANA y/o ASMA) y tipo II (LKM-1). Resultados: se diagnostica a siete pacientes, 5 mujeres (71,5%) y 2 varones (28,5%); tipo I 5 y tipo II dos pacientes. La edad al diagnóstico es 21 meses a 12 años. En el tipo I la presentación clínica es como hepatitis aguda en 3 casos y 2 pacientes con insuficiencia hepática progresiva. Las tipo II se diagnostican tras un hallazgo analítico siendo asintomáticas. La elevación de transaminasas (x10 su valor) se observa en el 71,5% e hipergammaglobulinemia en el 85%. El tratamiento instaurado es azatioprina y corticosteroides con un tiempo medio de remisión de 14 meses. Dos pacientes recaen al retirar corticosteroides. Conclusión: las formas de presentación son variadas y puede ser indistinguible a una hepatitis viral. Se debe sospechar ante una elevación de las aminotransferasas y la presencia de hipergammaglobulinemia. Con buenos resultados el tratamiento recomendado sería azatioprina y corticosteroides. Existen altos porcentajes de recaídas al retirar la corticoterapia por lo que algunos pacientes precisarían de dosis mínimas para mantener la remisión

Background: autoimmune hepatitis (AIH) is an inflammatory disease of unknown origin that is responsible for progressive liver necrosis and ultimately cirrhosis. Objective: our aim was to evaluate the characteristics of autoimmune hepatitis presenting in the pediatric age. Material and methods: we conducted a retrospective study of all patients diagnosed with AIH in our hospital department during the last 10 years. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology, treatment, need for transplant, and clinical evolution. According to the positive level of auto-antibodies, AIH patients were classified as type I AIH (ANA and/or smooth-muscle antibodis) and type II (anti-LKM-1). Results: seven patients were diagnosed in this period –5 girls (71.5%) and 2 boys (28.5%). Five patients presented with type-I serological markers, and two with type-II markers. Age range at diagnosis was from 21 months to 12 years. In the type-I group, 3 patients presented with acute hepatitis while 2 other patients were diagnosed from laboratory findings while asymptomatic. Elevated aminotransferase (10 times the normal level) was observed in 71.5%, and 85% had elevated immunoglobulins. Treatment with azathioprine and prednisone was started after diagnosis with an average time to remission of 14 months. Two patients relapsed following steroid withdrawal. Conclusion: AIH can have different forms of clinical presentation, and is sometimes indistinguishable from viral hepatitis. AIH must be ruled out in patients presenting with concomitant elevation of aminotransferases and immunoglobulins. The commonly accepted treatment is a combination of azathioprine and corticosteroids. A high percentage of patients experience a relapse of disease after steroids are withdrawn. Therefore, some patients will need to stay on combined therapy with minimal doses of steroids

Colitis seudomembranosa secundaria a infección por Salmonella / Pseudomembranous colitis due to Salmonella infection

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Tirosinemia tipo I: dos formas atípicas de presentación clínica / Tyrosinemia type I: two atypical forms of clinical presentation

Se exponen dos casos de tirosinemia tipo I con presentación atípica. El primero se inicia con crisis convulsivas secundarias a hipoglucemias graves. En el estudio metabólico para el diagnóstico de las hipoglucemias se detecta un perfil típico de tirosinemia tipo I en sangre y orina. El segundo caso fue un hallazgo casual en el contexto de un ingreso por bronquitis. Ambos casos siguen tratamiento con 2-(2-nitro-4-trifluorometilbenzoil}-1-3-ciclohexanediona( NTBC) en combinación con dieta con bajo contenido en proteínas naturales suplementas con fórmula especial sin tirosina ni fenilalanina. En la actua.lidad no presentan alteraciones clínico-analíticas ni complicaciones

The authors describe two cases of tyrosinemia type 1 with atypical clinical presentation. The first patient presented with refractory seizure secondary to hypoglycemic episodes. The metabolic study for the differential diagnosis of hypoglycemi, revealed a pattern suggestive of tyrosinemia type I. In the second patient the presence of tyrosinemia was detected during a hospital stayowing to bronchitis. In both cases, treatment with 2-(2-nitro-4, trifluoromethylbenzoyl)-1,3-cyclohexane(NTBC) combine (with a low protein diet supplemented with a tyrosine-and phenylalanine-free formula was prescribed. There have beenn no complications during the follow-up period in either case and, at the present time, the clinical and analytical findings are normal

[Benign paroxysmal vertigo. Our 14 years' experience with this entity]. / Vértigo paroxístico benigno. Nuestra experiencia de 14 años.

INTRODUCTION: Benign paroxysmal vertigo (BPV) is characterised by the sudden onset of brief recurring episodes of loss of balance, sometimes accompanied by vegetative symptoms, which tend to disappear spontaneously in a matter of months or years and have no intercritical alterations. PATIENTS AND METHODS: We analyse the clinical and developmental characteristics of the patients who were listed as possible cases of benign paroxysmal vertigo in the database of the Neuropaediatric Unit of the Hospital Miguel Servet in Zaragoza over a 14 year period. Clinical records were reviewed and cases no longer controlled were contacted by telephone. RESULTS: 18 patients were considered to be cases of BPV, with the typical criteria, and 13 were found to be possible BPV (p-BPV). The atypical data of the p-BPV included late age of onset, prolonged persistence of the episodes and long duration of each episode. Neuroimaging was performed in 8 (44%) of the BPV and 10 (77%) of the p-BPV, and electroencephalogram recordings were carried out in 15 (83%) of the BPV and 11 (85%) of the p-BPV, with normal results in all cases. CONCLUSIONS: Establishing a diagnosis of BPV with strict criteria can mean that some atypical cases are excluded. As no biological marker is available, the diagnosis is clinical-developmental and sometimes complementary tests need to be conducted in order to rule out other pathological conditions. A broader clinical spectrum, cases in which the episodes last longer and a greater range of ages of onset and complete remission are all possible. A diagnosis of BPV should reassure the patients' families as it is a benign process that tends to disappear spontaneously.

Vértigo paroxístico benigno. Nuestra experiencia de 14 años / Benign paroxysmal vertigo. Our 14 years’experience with this entity

Introducción. El vértigo paroxístico benigno (VPB) se caracteriza por episodios breves y recurrentes de pérdida de equilibrio, de inicio brusco, acompañados en ocasiones de síntomas vegetativos, con tendencia a la desaparición espontánea en meses o años y sin alteraciones intercríticas. Pacientes y métodos. Se han analizado las características clínicas y evolutivas de los pacientes que figuran como posibles casos de vértigo paroxístico benigno en la base de datos de la Sección de Neuropediatría del Hospital Miguel Servet de Zaragoza durante un período de 14 años. Se revisaron las historias y se contactó telefónicamente con los casos que no seguían control. Resultados. Se han considerado 18 pacientes VPB, con los criterios típicos, y 13 como posible VPB (p-VPB). Los p- VPB tuvieron como datos atípicos edad tardía de presentación, persistencia prolongada de los episodios y duración larga de los episodios. Se realizó, con resultados normales, neuroimagen a ocho (44%) de los VPB y a diez (77%) de los p-VPB, y electroencefalograma a 15 (83%) de los VPB y a 11 (85%) de los p-VPB. Conclusiones. Establecer el diagnóstico de VPB con criterios estrictos puede excluir algunos casos atípicos. Al no disponer de un marcador biológico, el diagnóstico es clinicoevolutivo y, en ocasiones, deben realizarse exámenes complementarios para descartar otras patologías. Puede haber un espectro clínico más amplio, casos de mayor duración de los episodios y mayor rango en las edades de presentación y desaparición. El diagnóstico de VPB debe tranquilizar a las familias, al ser un proceso benigno con tendencia a la desaparición espontánea

Introduction. Benign paroxysmal vertigo (BPV) is characterised by the sudden onset of brief recurring episodes of loss of balance, sometimes accompanied by vegetative symptoms, which tend to disappear spontaneously in a matter of months or years and have no intercritical alterations. Patients and methods. We analyse the clinical and developmental characteristics of the patients who were listed as possible cases of benign paroxysmal vertigo in the database of the Neuropaediatric Unit of the Hospital Miguel Servet in Zaragoza over a 14 year period. Clinical records were reviewed and cases no longer controlled were contacted by telephone. Results. 18 patients were considered to be cases of BPV, with the typical criteria, and 13 were found to be possible BPV (p-BPV). The atypical data of the p-BPV included late age of onset, prolonged persistence of the episodes and long duration of each episode. Neuroimaging was performed in 8 (44%) of the BPV and 10 (77%) of the p-BPV, and electroencephalogram recordings were carried out in 15 (83%) of the BPV and 11 (85%) of the p-BPV, with normal results in all cases. Conclusions. Establishing a diagnosis of BPV with strict criteria can mean that some atypical cases are excluded. As no biological marker is available, the diagnosis is clinical-developmental and sometimes complementary tests need to be conducted in order to rule out other pathological conditions. A broader clinical spectrum, cases in which the episodes last longer and a greater range of ages of onset and complete remission are all possible. A diagnosis of BPV should reassure the patients’ families as it is a benign process that tends to disappear spontaneously

[Cleft palate and cleft lip. Clinical review]. / Fisura palatina y labio leporino. Revisión clínica.

UNLABELLED: The aim of this study is to do an analytical study of cleft palate and cleft lip in our hospital. PATIENTS AND METHODS: 85 clinical charts of patients attended in our hospital born between 1976 and 2001 in Aragon and Rioja were reviewed. We studied the incidence of oral cleft, associated malformations and morbidity, familial antecedents and perinatal data, phonatory disfunctions, serose otitis, growth failure and psychiatry problems. RESULTS: The mean incidence was 0.5/1000 newborns. 41.5% presented associated malformations and 19.3% were associated with a specific syndrome, being more frequent in patients affected of cleft palate and cleft lip (50%) than patients with only cleft palate (41.2%) or only cleft lip (8.8%). The most frequent malformations were: facial defects (50%), skeletal (33%), congenital cardiopathies (33%). 19% were born prematurely. The percentage of serose otitis that required control at hospital was 37.3%. 34.2% presented phonatory problems. There was a high incidence of growth failure and psychiatry problems. CONCLUSION: Oral clefts represent a complex clinical condition with a high percentage of medical complications that require a multidisciplinary treatment. The high incidence of congenital defects associated with this condition demand an exhaustive screening in the newborns affected.

Psychomotor and intellectual development of children born with intrauterine growth retardation (IUGR).

The possible impact of IUGR on the intellectual outcome of children born with IUGR gives special relevance to this condition. In order to determine the psychomotor and intellectual development of such children, we analyzed the evolution of 60 children through appropriate tests, along the years, and the possible influence of two factors, the socio-economic status of the family, and whether or not there was catch-up growth. Our results show a negative impact of IUGR on the intellectual outcome of these children, independent of catch-up growth, although those with catch-up growth showed better evolution. The socio-economic status plays a limited role only at older age. Those children followed longitudinally for 1 year did not show any amelioration of their IQ.

Estudio del desarrollo psicomotor e intelectual de niños nacidos con crecimiento intrauterino retardado (CIR) / No disponible

No disponible