Bronquiolitis obliterante. Evolución a medio plazo / Bronchiolitis obliterans: Outcome in the medium term

Introducción: La bronquiolitis obliterante es una enfermedad respiratoria obstructiva crónica de la vía respiratoria inferior que se presenta principalmente después de infecciones víricas. La evolución a medio plazo difiere según el área geográfica. El objetivo ha sido comunicar los resultados en nuestro medio. Material y métodos: Estudio retrospectivo de casos diagnosticados de bronquiolitis obliterante (BO) entre diciembre-1994 y febrero-2011. El diagnóstico se estableció mediante TCAR pulmonar y función pulmonar; un caso precisó biopsia pulmonar. La exploración funcional se realizó con espirometría forzada (≥3 años de edad), pletismografía (≥5 años), pruebas de broncodilatación (PBD), difusión de monóxido de carbono (DLCO) (≥6 años) y óxido nítrico exhalado (FENO).Variables cuantitativas: edad al diagnóstico, morbilidad respiratoria antes del diagnóstico, tiempo de evolución hasta diagnóstico y de seguimiento, morbilidad respiratoria tras diagnóstico, resistencia (sReff) y conductancia específica de la vía aérea (sGeff), volumen residual, capacidad pulmonar total, volumen de gas intratorácico, FEV1, FVC, FEV1/FVC, FEF25-75, DLCO y FENO. Variables cualitativas: sexo, motivo de consulta, infecciones virales, atopia, tabaquismo, TCAR pulmonar y tratamiento. Análisis descriptivo y estudio de patrón de función pulmonar antes y después de PBD mediante t de Student y test de Wilcoxon para muestras apareadas. Resultados: Cohorte de 22 casos, 10 (45,4%) niños y 12 (54,5%) niñas. Edad media al diagnóstico 4,87±3,27 años. El tiempo de seguimiento fue 5 años (rango 1-8 años). Virus relacionados con BO: influenza 2 (9,09%), parainfluenza 2 (9,09%), adenovirus 3 (13,69%), virus respiratorio sincitial 1 (4,5%) y rinovirus 1 (4,54%). TCAR pulmonar: patrón en mosaico en 22 (100%) y bronquiectasias en 8 (36,36%). Función pulmonar al diagnóstico: patrón obstructivo fijo e incremento de volúmenes pulmonares. El análisis global evolutivo constató disminución progresiva de FEV1, FVC, FVC/FEV1 y FEF25-75 e incremento de sReff y volumen residual con disminución de sGeff. Individualmente, hay tendencia a la estabilidad de la función pulmonar respecto a valores al diagnóstico. Morbilidad respiratoria baja tras diagnóstico. Conclusiones: En la mayoría de los casos se objetiva patrón funcional obstructivo fijo y estabilidad evolutiva de la función pulmonar con morbilidad respiratoria baja. La TCAR pulmonar y la función pulmonar son las pruebas diagnósticas que apoyan el diagnóstico(AU)

Background: Bronchiolitis obliterans is a chronic obstructive lung disease that follows a severe insult to the lower respiratory tract. Severity and outcome are different depending on the geographic area. The aim of this study was to communicate the results of our sample. Methods: A retrospective study was performed in a sample of boys and girls with a medical diagnosis of bronchiolitis obliterans (BO) between December 1994 and February 2011. The diagnosis was made by high-resolution computed tomography (HRCT) scan and lung function. Lung biopsy was performed in one case. Pulmonary function testing included; forced spirometry (≥ 3 years old), plethysmography (≥ 5 years), bronchodilator tests (BDT), single-breath determination of carbon monoxide uptake in the lung (DLCO) (≥ 6 years) and measurement of exhaled lower respiratory nitric oxide (FENO). Quantitative variables were; age at diagnosis, respiratory morbidity before the diagnosis, time from the start of the symptomatology until the diagnosis, follow-up time, respiratory morbidity after the diagnosis, airway specific resistance (sReff), airway specific conductance (sGeff), residual volume, total lung capacity, thoracic gas volume, FEV1, FVC, FEV1/FVC, FEF25-75, DLCO and FENO. Qualitative variables were; sex, reason for consulting, viral infections, atopy, smoke exposure, HRCT scan and current treatment. Descriptive analysis was performed and pulmonary function before and after BDT was studied using paired-sample Wilcoxon and Student's t-test. Results: We studied a cohort of 22 cases, 10 (45.4%) boys and 12 (54.5%) girls. The mean age at the diagnosis was 4.87±3, 27 years old. The follow-up period was 5 years (range 1 to 8 years). Related virus were; influenza 2 (9.09%), parainfluenza 2 (9.09%), adenovirus 3 (13.69%), syncytial respiratory virus 1 (4.5%) and rhinovirus 1 (4.54%). HRCT scan: mosaic pattern of lung attenuation 22 (100%) and bronchiectasis 8 (36.36%). Lung function at diagnosis showed fixed airflow obstruction and increased lung volumes. The global analysis showed a progressive decrease in FEV1, FVC, FVC/FEV1, FEF25-75 and increase in sReff and residual volume with decrease in sGeff. Individually, there was a tendency to stabilise the lung function comparing with initial values at diagnosis. Respiratory morbidity was low after the diagnosis. Conclusions: In most cases, fixed air-flow obstruction and lung function stability with low respiratory morbidity were observed. HRCT scan and lung function testing supported the diagnosis(AU)

[Bronchiolitis obliterans: outcome in the medium term]. / Bronquiolitis obliterante. Evolución a medio plazo.

BACKGROUND: Bronchiolitis obliterans is a chronic obstructive lung disease that follows a severe insult to the lower respiratory tract. Severity and outcome are different depending on the geographic area. The aim of this study was to communicate the results of our sample. METHODS: A retrospective study was performed in a sample of boys and girls with a medical diagnosis of bronchiolitis obliterans (BO) between December 1994 and February 2011. The diagnosis was made by high-resolution computed tomography (HRCT) scan and lung function. Lung biopsy was performed in one case. Pulmonary function testing included; forced spirometry (≥ 3 years old), plethysmography (≥ 5 years), bronchodilator tests (BDT), single-breath determination of carbon monoxide uptake in the lung (D(LCO)) (≥ 6 years) and measurement of exhaled lower respiratory nitric oxide (FE(NO)). Quantitative variables were; age at diagnosis, respiratory morbidity before the diagnosis, time from the start of the symptomatology until the diagnosis, follow-up time, respiratory morbidity after the diagnosis, airway specific resistance (sR(eff)), airway specific conductance (sG(eff)), residual volume, total lung capacity, thoracic gas volume, FEV(1), FVC, FEV(1)/FVC, FEF(25-75), D(LCO) and FE(NO). Qualitative variables were; sex, reason for consulting, viral infections, atopy, smoke exposure, HRCT scan and current treatment. Descriptive analysis was performed and pulmonary function before and after BDT was studied using paired-sample Wilcoxon and Student́s t-test. RESULTS: We studied a cohort of 22 cases, 10 (45.4%) boys and 12 (54.5%) girls. The mean age at the diagnosis was 4.87 ± 3, 27 years old. The follow-up period was 5 years (range 1 to 8 years). Related virus were; influenza 2 (9.09%), parainfluenza 2 (9.09%), adenovirus 3 (13.69%), syncytial respiratory virus 1 (4.5%) and rhinovirus 1 (4.54%). HRCT scan: mosaic pattern of lung attenuation 22 (100%) and bronchiectasis 8 (36.36%). Lung function at diagnosis showed fixed airflow obstruction and increased lung volumes. The global analysis showed a progressive decrease in FEV(1), FVC, FVC/FEV(1,) FEF(25-75) and increase in sR(eff) and residual volume with decrease in sG(eff). Individually, there was a tendency to stabilise the lung function comparing with initial values at diagnosis. Respiratory morbidity was low after the diagnosis. CONCLUSIONS: In most cases, fixed air-flow obstruction and lung function stability with low respiratory morbidity were observed. HRCT scan and lung function testing supported the diagnosis.

Hamartoma condromesenquimal endobronquial / Endobronchial chondromesenchymal hamartoma

Los tumores pulmonares primarios son poco frecuentes en la infancia. Dentro de estos, los endobronquiales son todavía menos frecuentes y entre los benignos, los pseudotumores inflamatorios y los hamartomas son los de mayor incidencia. Se presenta el caso de una niña de 2 años y medio de edad afecta de un hamartoma condromesenquimal endobronquial izquierdo con obstrucción del 90% de la luz bronquial. Se realizó resección completa de la masa endobronquial mediante broncoscopio rígido y aplicación de mitomicina C tópica. Desde la resección del tumor y tras un periodo de seguimiento de 12 meses, la paciente ha presentado una evolución favorable con desaparición inmediata de la clínica respiratoria y con fibrobroncoscopias sucesivas sin objetivar tumor residual (AU)

Primary lung tumours are uncommon in childhood. Among these tumours, endobronchial masses are even less common and, among benign tumours, inflammatory pseudotumours and hamartomas have the highest incidence in children. We present the case of a 2.5-year-old girl with a left endobronchial chondromesenchymal hamartoma with obstruction of 90% of the bronchial lumen. Complete resection of the endobronchial mass was performed by rigid bronchoscopy and application of topical mitomycin C. After tumour resection and a 12-month follow-up, the patient has shown a favourable outcome with immediate disappearance of respiratory symptoms. Successive fibreoptic bronchoscopies have shown no residual tumour (AU)

[Endobronchial chondromesenchymal hamartoma]. / Hamartoma condromesenquimal endobronquial.

Primary lung tumours are uncommon in childhood. Among these tumours, endobronchial masses are even less common and, among benign tumours, inflammatory pseudotumours and hamartomas have the highest incidence in children. We present the case of a 2.5-year-old girl with a left endobronchial chondromesenchymal hamartoma with obstruction of 90% of the bronchial lumen. Complete resection of the endobronchial mass was performed by rigid bronchoscopy and application of topical mitomycin C. After tumour resection and a 12-month follow-up, the patient has shown a favourable outcome with immediate disappearance of respiratory symptoms. Successive fibreoptic bronchoscopies have shown no residual tumour.

[Forced spirometry in healthy preschool children]. / Espirometría forzada en preescolares sanos bajo las recomendaciones de la ATS/ERS: estudio CANDELA.

INTRODUCTION: Reference values for spirometry in healthy preschool children have not yet been obtained in accordance with American Thoracic Society (ATS) and the European Respiratory Society (ERS) guidelines. The objective was to establish reference values for spirometry in healthy preschoolers under the ATS/ERS 2007 statement. MATERIAL AND METHOD: Children of at least 2 and under 7 years of age were tested in 9 pediatric pulmonary function laboratories. The technicians were trained to apply a standardized protocol to perform spirometry. RESULTS: Valid spirometry results were obtained in 455 (81.54%) out of 558 children: 242 boys (53.2%) and 213 girls (46.8%). Ages were as follows: 31 at least 2 and under 3 years old; 96, at least 3 and under 4; 108, at least 4 and under 5; 122, at least 5 and under 6 years, and 98, at least 6 and under 7 years. Formulas were used to calculate the reference values for all the spirometry variables in preschoolers. CONCLUSIONS: Spirometry is feasible in the majority of preschool children under the new guidelines. The availability of the reference values presented is an important step, both for the care of preschoolers and for further research on pulmonary function.

[Outcome of two patients with Hurler's syndrome under enzyme replacement therapy with human recombinant alpha-L-iduronidase]. / Evolución de dos pacientes con síndrome de Hurler en tratamiento con enzima recombinante humana alpha-L-iduronidasa.

We performed a prospective study of two patients with Hurler's syndrome (aged 4.8 years and 17 months at the beginning of the intervention) under enzyme replacement therapy with human recombinant alpha-L-iduronidase for 452 and 28 weeks respectively. The aim of this study was to analyze the safety and efficacy of the intervention during the treatment periods. Several diagnostic imaging tests, clinical examinations, and serial laboratory determinations were performed to demonstrate the effectiveness of the therapy in both patients. In patient 1 (a boy aged 4.8 years, homozygote W402X), the treatment was always intended to be palliative because of the advanced stage of the disease. In patient 2 (a 17-month-old girl, heterozygote W402X) the treatment was initiated early with subsequent clinical stabilization without acquisition of regressive factors. Bone marrow transplantation from an unrelated donor was successful. Currently, because of the lack of histocompatible bone marrow donors, transplantation of hematopoietic stem cells from umbilical cord blood or peripheral blood are being performed with satisfactory results. In the future, gene therapy may be able to prevent the diseases associated with Hurler's syndrome and halt the neurocognitive deterioration characteristic of these patients.

Evolución de dos pacientes con síndrome de Hurler en tratamiento con enzima recombinante humana α -L-iduronidasa / Outcome replacement of two patients with Hurler´s syndrome under enzyme replacement therapy with human recombinant α -L-iduronidase

Estudio prospectivo de 2 pacientes con enfermedad de Hurler (de 4,8 años y 17 meses de edad al inicio de la intervención) en tratamiento enzimático sustitutivo con enzima recombinante humana α -L-iduronidasa durante 52 y 28 semanas, respectivamente, con el objetivo de analizar la eficacia y seguridad de la intervención durante dicho período de tiempo. Se realizaron para ello diversas pruebas diagnósticas por imagen, exámenes clínicos y analíticos seriados que demostraron en ambos casos ser una terapia efectiva. En el caso 1 (varón de 4,8 años, homocigoto W402X) la intervención se planteó con fines paliativos desde el comienzo debido a su estado clínico muy evolucionado. En el caso 2 (mujer de 17 meses, heterocigota para W402X) la intervención se realizó de forma precoz y se observó una estabilización clínica posterior sin la adquisición de factores regresivos. Posteriormente se realizó con éxito un trasplante de médula ósea de donante no emparentado. Actualmente, ante la carencia de donantes de médula ósea histocompatibles, se está realizando trasplante de células madre hematopoyéticas procedentes de cordón o de sangre periférica con resultados satisfactorios. La terapia génica se considera el tratamiento futuro capaz de prevenir la enfermedad asociada al síndrome de Hurler y detener el deterioro neurocognitivo característico de estos pacientes.

We performed a prospective study of two patients with Hurler's syndrome (aged 4.8 years and 17 months at the beginning of the intervention) under enzyme replacement therapy with human recombinant α -L-iduronidase for 452 and 28 weeks respectively. The aim of this study was to analyze the safety and efficacy of the intervention during the treatment periods. Several diagnostic imaging tests, clinical examinations, and serial laboratory determinations were performed to demonstrate the effectiveness of the therapy in both patients. In patient 1 (a boy aged 4.8 years, homozygote W402X), the treatment was always intended to be palliative because of the advanced stage of the disease. In patient 2 (a 17-month-old girl, heterozygote W402X) the treatment was initiated early with subsequent clinical stabilization without acquisition of regressive factors. Bone marrow transplantation from an unrelated donor was successful. Currently, because of the lack of histocompatible bone marrow donors, transplantation of hematopoietic stem cells from umbilical cord blood or peripheral blood are being performed with satisfactory results. In the future, gene therapy may be able to prevent the diseases associated with Hurler's syndrome and halt the neurocognitive deterioration characteristic of these patients

[Clinical characteristics of children hospitalized for influenza virus infection]. / Características clínicas de los niños hospitalizados por infección por virus Influenza.

BACKGROUND: The clinical manifestations of influenza virus are fairly nonspecific and are similar to those of other viral infections, respiratory processes due to other causes and even septic disease in neonates. Few studies have been performed of the clinical characteristics of influenza virus infection in hospitalized children. OBJECTIVE: To evaluate the clinical characteristics of children hospitalized for influenza virus infection in four consecutive epidemic waves (2000-2004). MATERIAL AND METHODS: We retrospectively reviewed the medical records of children hospitalized for influenza A and B virus infection confirmed by cell culture and polymerase chain reaction. Age, sex, symptoms on admission, complementary investigations, diagnosis and outcome were evaluated. RESULTS: Eighty-four children were hospitalized, of which 74 had influenza virus type AH3, five had influenza virus type AH1 and five had influenza B virus. A total of 42.8 % were aged less than 6 months. The main symptoms were fever (75 patients), cough (56 patients) and ENT involvement (53 patients). The most frequent causes of admission were febrile syndrome (75 patients), bronchiolitis (19 patients), pneumonia (13 patients) and bronchitis (8 patients). In 21 patients viral or bacterial coinfection was found, the most frequent of which was respiratory syncytial virus (10 patients). Few differences were found among age groups except for pneumonia and prolonged fever (more frequent in children older than 6 months) and lymphocytosis (in children younger than 6 months). The greatest number of complementary investigations in younger children was performed for acute febrile syndrome in neonates aged less than 6 months. Risk factors for hospitalization were found in only three children, all of whom were older than 6 months. Outcome was favorable in all children. CONCLUSIONS: Influenza virus infection in hospitalized children is most frequent in previously healthy neonates with fever and nonspecific signs and symptoms that are similar to those of other infectious processes. Specific microbiological diagnostic techniques are required for an early diagnosis. Healthy infants aged less than 24 months are most at risk for hospitalization for influenza virus infection.

Características clínicas de los niños hospitalizados por infección por virus Influenza / Clinical characteristics of children hospitalized for influenza virus infection

Antecedentes: Las manifestaciones clínicas de la gripe son bastante inespecíficas y similares a otras infecciones virales, a procesos respiratorios de otra etiología e incluso a cuadros sépticos en lactantes. Existen pocos estudios sobre las características clínicas de la infección por virus Influenza en niños hospitalizados. Objetivo: Evaluar las características clínicas de los niños hospitalizados por infección por virus Influenza en cuatro ondas epidémicas consecutivas (2000-2004). Material y métodos: Estudio retrospectivo por revisión de historias clínicas de los niños hospitalizados por infección confirmada por virus Influenza A y B, mediante cultivo celular y reacción en cadena de la polimerasa. Se registraron las variables: sexo, edad, clínica al ingreso, exploraciones complementarias, diagnóstico y evolución posterior. Resultados Fueron hospitalizados 84 niños, 74 casos debidos a infección por virus Influenza AH3, 5 casos por virus Influenza AH1 y cinco por Influenza B. El 42,8 % fueron menores de 6 meses. El cuadro clínico principal fue fiebre (75 casos), tos (56 casos), afectación otorrinolaringológica (53 casos). Los diagnósticos más frecuentes que motivaron el ingreso fueron síndrome febril (75 casos), bronquiolitis (19 casos), neumonía (13 casos) y bronquitis (8 casos). En 21 casos se objetivó coinfección viral o bacteriana, siendo la asociación con virus respiratorio sincitial la coinfección más frecuente (10 casos). Se han observado pocas diferencias entre los diferentes grupos de edad, excepto para neumonía y fiebre prolongada (más frecuentes en mayores de 6 meses) y linfocitosis (en menores de 6 meses). El cuadro febril agudo en menores de 6 meses ha originado mayor número de exploraciones complementarias en los de menor edad. Sólo en tres niños se encontraron factores de riesgo para hospitalización, todos ellos mayores de 6 meses. Todos los casos evolucionaron favorablemente. Conclusiones: La infección por virus Influenza en los niños que están hospitalizados predomina en lactantes sanos, con fiebre y signos y síntomas respiratorios poco específicos, similares a otros cuadros infecciosos. Es necesario aplicar técnicas diagnósticas microbiológicas específicas para el diagnóstico precoz. Los lactantes sanos y menores de 24 meses constituyen el grupo de mayor riesgo para la hospitalización por infección por virus Influenza

Background: The clinical manifestations of influenza virus are fairly nonspecific and are similar to those of other viral infections, respiratory processes due to other causes and even septic disease in neonates. Few studies have been performed of the clinical characteristics of influenza virus infection in hospitalized children. Objective: To evaluate the clinical characteristics of children hospitalized for influenza virus infection in four consecutive epidemic waves (2000-2004). Material and methods: We retrospectively reviewed the medical records of children hospitalized for influenza A and B virus infection confirmed by cell culture and polymerase chain reaction. Age, sex, symptoms on admission, complementary investigations, diagnosis and outcome were evaluated. Results: Eighty-four children were hospitalized, of which 74 had influenza virus type AH3, five had influenza virus type AH1 and five had influenza B virus. A total of 42.8% were aged less than 6 months. The main symptoms were fever (75 patients), cough (56 patients) and ENT involvement (53 patients). The most frequent causes of admission were febrile syndrome (75 patients), bronchiolitis (19 patients), pneumonia (13 patients) and bronchitis (8 patients). In 21 patients viral or bacterial coinfection was found, the most frequent of which was respiratory syncytial virus (10 patients). Few differences were found among age groups except for pneumonia and prolonged fever (more frequent in children older than 6 months) and lymphocytosis (in children younger than 6 months). The greatest number of complementary investigations in younger children was performed for acute febrile syndrome in neonates aged less than 6 months. Risk factors for hospitalization were found in only three children, all of whom were older than 6 months. Outcome was favorable in all children. Conclusions: Influenza virus infection in hospitalized children is most frequent in previously healthy neonates with fever and nonspecific signs and symptoms that are similar to those of other infectious processes. Specific microbiological diagnostic techniques are required for an early diagnosis. Healthy infants aged less than 24 months are most at risk for hospitalization for influenza virus infection

[Psychogenic cough: Another etiology for persistent cough]. / Tos psicógena: una causa de tos crónica.

The most common causes of persistent cough are upper respiratory tract disease (postnasal drip syndrome, infections) and asthma. In the last year, six patients (four boys and two girls), aged 7-12 years old, with a diagnosis of hard-to-manage asthma and/or persistent cough were referred to our department. All the patients had undergone treatment with multiple drugs for long periods without favorable clinical response. The findings of physical examination, radiology, basal pulmonary function and post-bronchodilation and fibrobronchoscopy were normal. Organic disease was ruled out and a psychiatric evaluation was performed. Intelligence quotient was in the lower normal range and generalized anxiety order was identified, thus establishing a diagnosis of psychogenic cough. Treatment consisted of relaxation techniques and psychopedagogic support with favorable outcome. To avoid diagnostic errors and inappropriate treatment, psychogenic cough should be included in the differential diagnosis of persistent cough and hard-to-manage asthma.

Tos psicógena: una causa de tos crónica / Psychogenic cough: another etiology for persistent cough

Las enfermedades de la vía aérea superior (síndrome de goteo nasal posterior, infecciones) y el asma son las causas más habituales de la tos crónica. Durante el último año, nos fueron remitidos 6 casos (4 varones y 2 mujeres) de 7 a 12 años de edad, con el diagnóstico de asma de difícil control y/o tos persistente. Todos habían recibido tratamientos con múltiples fármacos durante largos períodos de tiempo, sin respuesta clínica favorable. La exploración física, radiología, función pulmonar basal y posbroncodilatación y fibrobroncoscopia, fueron normales. Descartada la enfermedad orgánica se realizó estudio psiquiátrico, constatando un coeficiente de inteligencia en el límite bajo de la normalidad y un trastorno por ansiedad generalizada, estableciéndose el diagnóstico de tos psicógena. El tratamiento se efectuó con técnicas de relajación y tratamiento psicopedagógico de apoyo, y evolucionaron favorablemente. La tos psicógena es un proceso que debe incluirse en el diagnóstico diferencial de tos crónica y en el asma de difícil control, con el fin de evitar errores diagnósticos y tratamientos inadecuados (AU)