Inherited pulmonary surfactant metabolism disorders in Argentina: Differences between patients with SFTPC and ABCA3 variants.

BACKGROUND: Patients with inherited pulmonary surfactant metabolism disorders have a wide range of clinical outcomes and imaging findings. Response to current anti-inflammatory therapies has been variable and efficacy is unclear. OBJECTIVE: To describe and compare genetic, clinical, histological, and computed tomography (CT) outcomes in a cohort of patients with variants in the genes encoding surfactant protein C (SP-C) or adenosine triphosphate-binding cassette transporter A3 (ABCA3) in Argentina. METHODS: Observational cohort retrospective study. Patients carrying variants in genes encoding SP-C and ABCA3 proteins were included. RESULTS: Fourteen patients met the inclusion criteria: SFTPC n = 6, ABCA3 n = 8 (seven were heterozygous and one compound heterozygous). Neonatal respiratory distress was more frequent and severe in neonates with variants in the ABCA3 gene. The onset of the disease occurred in infancy before the age of 20 months in all cases. Patients with ABCA3 pathogenic variants had a severe clinical course, while long-term outcomes were more favorable in individuals with SFTPC variants. Initial CT findings were ground glass opacities and intraparenchymal cysts in both groups. Over time, signs of lung fibrosis were present in 57% of patients with ABCA3 variants and in 33% of the SFTPC group. The efficacy of anti-inflammatory interventions appears to be poor, especially for patients with ABCA3 pathogenic variants. CONCLUSIONS: Clinical, histological, and radiological features are similar in patients with SFTPC and ABCA3 variants; however, the latter have more severe clinical course. Current anti-inflammatory regimens do not appear to stop the progression of the disease.

Paciente con quistes pulmonares y diagnóstico de coinfección con tuberculosis e hidatidosis: reporte de un caso pediátrico / Patient with pulmonary cysts and diagnosis of co-infection with tuberculosis and hydatidosis. Pediatric cases report

Existen numerosas entidades en la población pediátrica que pueden presentarse en forma de quistes o como lesiones de similares características. De estas patologías, las infecciosas son las más frecuentes. Se presenta el caso de una paciente oriunda de Bolivia con migración reciente a la Argentina que presentó una coinfección con tuberculosis e hidatidosis pulmonar. Ambas infecciones se pueden presentar con signos y síntomas similares y, aunque la asociación citada es poco frecuente en la bibliografía, ciertos mecanismos inmunitarios podrían intervenir en la coinfección de parásitos helmintos y micobacterias. Ambas patologías son infecciones prevalentes en nuestra región y deben ser tenidas en cuenta entre los diagnósticos diferenciales ante pacientes con imágenes quísticas o cavitarias pulmonares.

Numerous entities in the pediatric population can present in the form of cysts or as lesions with similar characteristics. Of the pathologies that can cause these images in children, infectious diseases are the most frequent. We present the case of a native of Bolivia with recent immigration to Argentina who presented a pulmonary co-infection with tuberculosis and hydatidosis. Both infections can present with similar signs and symptoms and although this association is rarely reported in the literature, certain immunological mechanisms could intervene in the causal association of co-infection between helminth parasites and mycobacteria. Both pathologies are very prevalent infections in our region and should be taken into account among the differential diagnoses in patients with cystic or cavitary pulmonary diseases.

[Patient with pulmonary cysts and diagnosis of co-infection with tuberculosis and hydatidosis. Pediatric cases report]. / Paciente con quistes pulmonares y diagnóstico de coinfección con tuberculosis e hidatidosis: reporte de un caso pediátrico.

Numerous entities in the pediatric population can present in the form of cysts or as lesions with similar characteristics. Of the pathologies that can cause these images in children, infectious diseases are the most frequent. We present the case of a native of Bolivia with recent immigration to Argentina who presented a pulmonary co-infection with tuberculosis and hydatidosis. Both infections can present with similar signs and symptoms and although this association is rarely reported in the literature, certain immunological mechanisms could intervene in the causal association of co-infection between helminth parasites and mycobacteria. Both pathologies are very prevalent infections in our region and should be taken into account among the differential diagnoses in patients with cystic or cavitary pulmonary diseases.

Existen numerosas entidades en la población pediátrica que pueden presentarse en forma de quistes o como lesiones de similares características. De estas patologías, las infecciosas son las más frecuentes. Se presenta el caso de una paciente oriunda de Bolivia con migración reciente a la Argentina que presentó una coinfección con tuberculosis e hidatidosis pulmonar. Ambas infecciones se pueden presentar con signos y síntomas similares y, aunque la asociación citada es poco frecuente en la bibliografía, ciertos mecanismos inmunitarios podrían intervenir en la coinfección de parásitos helmintos y micobacterias. Ambas patologías son infecciones prevalentes en nuestra región y deben ser tenidas en cuenta entre los diagnósticos diferenciales ante pacientes con imágenes quísticas o cavitarias pulmonares.

Taquipnea persistente de la infancia: hiperplasia de células neuroendocrinas, una revisión / Persistent tachypnea of infancy: neuroendocrine cell hyperplasia, a review

La hiperplasia de células neuroendocrinas de la infancia (HCNEI) constituye una de las enfermedades intersticiales más frecuentes en pediatría. Tanto su etiología como los mecanismos fisiopatológicos involucrados son inciertos. Suele presentarse en pacientes por lo demás sanos, durante los primeros meses de vida con taquipnea, retracciones costales, rales e hipoxemia. En la tomografía axial computada de tórax de alta resolución (TACAR) presenta imágenes características en vidrio esmerilado de distribución central y zonas de atrapamiento aéreo. Para el diagnóstico, además de la clínica y la TACAR, podemos recurrir a la biopsia en casos atípicos. Los hallazgos histológicos reflejan una arquitectura pulmonar normal y un aumento en el número de células neuroendocrinas. El manejo global es con medidas de sostén, ya que no se cuenta con un tratamiento específico. La sintomatología suele mejorar con la edad y el pronóstico es favorable.

Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common interstitial lung diseases of childhood. The etiology and pathophysiological mechanisms involved are uncertain. It usually presents in otherwise healthy patients during the first months of life with tachypnea, rib retractions, crackles, and hypoxemia. High-resolution chest computed tomography (HRCT) shows ground-glass opacities of central distribution and areas of air trapping. For diagnosis purposes, in addition to clinical and HRCT features, a lung biopsy is indicated for atypical cases. Histological findings reflect normal architecture and an increased number of neuroendocrine cells. The management consists of supportive and preventive care, since there is no specific treatment. Symptoms usually improve with age and the prognosis is favorable.

Algunos desafíos en mucopolisacaridosis tipo I / A few challenges in mucopolysaccharidosis type I

Se describen como desafíos actuales en mucopolisacaridosis I la necesidad de una clasificación adecuada, vinculándola a las indicaciones terapéuticas; el diagnóstico temprano desde la pesquisa neonatal, sus ventajas y dificultades hasta la sospecha clínica de las formas grave y atenuada; el cuidado de la patología espinal y oftalmológica, desde el diagnóstico, el seguimiento y el tratamiento; las reacciones alérgicas por terapia de reemplazo enzimático, su diagnóstico y tratamiento. Por último, la transición hacia el cuidado adulto

Here we describe the current challenges of mucopolysaccharidosis type I: the need for an adequate classification, establishing its relationship to therapeutic indications; an early diagnosis, from neonatal screening, its advantages and barriers, to clinical suspicion of severe and attenuated forms; spinal and eye disease care, from diagnosis to follow-up and treatment; allergic reactions caused by enzyme replacement therapy, their diagnosis and treatment. And lastly, transition to adult care

Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

A few challenges in mucopolysaccharidosis type I. / Algunos desafíos en mucopolisacaridosis tipo I.

Here we describe the current challenges of mucopolysaccharidosis type I: the need for an adequate classification, establishing its relationship to therapeutic indications; an early diagnosis, from neonatal screening, its advantages and barriers, to clinical suspicion of severe and attenuated forms; spinal and eye disease care, from diagnosis to follow-up and treatment; allergic reactions caused by enzyme replacement therapy, their diagnosis and treatment. And lastly, transition to adult care.

Se describen como desafíos actuales en mucopolisacaridosis I la necesidad de una clasificación adecuada, vinculándola a las indicaciones terapéuticas; el diagnóstico temprano desde la pesquisa neonatal, sus ventajas y dificultades hasta la sospecha clínica de las formas grave y atenuada; el cuidado de la patología espinal y oftalmológica, desde el diagnóstico, el seguimiento y el tratamiento; las reacciones alérgicas por terapia de reemplazo enzimático, su diagnóstico y tratamiento. Por último, la transición hacia el cuidado adulto.

[Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report]. / Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico.

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood. A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/ day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization. Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente. Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5- 4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalidad.

Nuevas recomendaciones para el cuidado de los pacientes con mucopolisacaridosis tipo I / New recommendations for the care of patients with mucopolysaccharidosis type I

Dados los avances sobre mucopolisacaridosis Icon posterioridad al consenso publicado en la Argentina por un grupo de expertos en 2008, se revisan recomendaciones respecto a estudios genéticos, seguimiento cardiológico, cuidado de la vía aérea, alertas sobre aspectos auditivos, de la patología espinal y neurológica. Se hace revisión de la terapéutica actual y se enfatiza en la necesidad de un diagnóstico y tratamiento precoces, así como de un seguimiento interdisciplinario

Considering the advances made on mucopolysaccharidosis type I after the consensus study published by a group of experts in Argentina in 2008, recommendations about genetic testing, cardiological follow-up, airway care, hearing impairment detection, spinal and neurological conditions, as well as current treatments, were reviewed. Emphasis was placed on the need for early diagnosis and treatment, as well as an interdisciplinary follow-up

New recommendations for the care of patients with mucopolysaccharidosis type I. / Nuevas recomendaciones para el cuidado de los pacientes con mucopolisacaridosis tipo I.

Considering the advances made on mucopolysaccharidosis type I after the consensus study published by a group of experts in Argentina in 2008, recommendations about genetic testing, cardiological follow-up, airway care, hearing impairment detection, spinal and neurological conditions, as well as current treatments, were reviewed. Emphasis was placed on the need for early diagnosis and treatment, as well as an interdisciplinary follow-up.

Dados los avances sobre mucopolisacaridosis I con posterioridad al consenso publicado en la Argentina por un grupo de expertos en 2008, se revisan recomendaciones respecto a estudios genéticos, seguimiento cardiológico, cuidado de la vía aérea, alertas sobre aspectos auditivos, de la patología espinal y neurológica. Se hace revisión de la terapéutica actual y se enfatiza en la necesidad de un diagnóstico y tratamiento precoces, así como de un seguimiento interdisciplinario.

Clinical, functional, and computed tomography findings in a cohort of patients with neuroendocrine cell hyperplasia of infancy.

INTRODUCTION: Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common interstitial lung diseases in children. Both the etiology and pathophysiological mechanisms of the disease are still unknown. Prognosis is usually favorable; however, there are significant morbidities during the early years of life. OBJECTIVE: To describe the clinical course, infant pulmonary function tests and computed tomography (CT) findings in a cohort of patients with NEHI in Argentina. METHODS: This is a observational multicenter cohort study of children diagnosed with NEHI between 2011 and 2020. RESULTS: Twenty patients participated in this study. The median age of onset of symptoms was 3 months and the median age at diagnosis was 6 months. The most common clinical presentation was tachypnea, retractions and hypoxemia. The chest CT findings showed central ground glass opacities and air trapping. Infant pulmonary function tests revealed an obstructive pattern in 75% of the cases (10/12). Most patients (75%) required home oxygen therapy for 17 months (interquartile range 12-25). In 85% of them, tachypnea and hypoxemia spontaneously resolved between the second and third years of life. CONCLUSION: In this cohort, the first symptoms appeared during the early months of life. The typical clinical, CT, and functional findings allowed the diagnosis without the need of a lung biopsy. Although most patients required home oxygen therapy, they showed a favorable evolution.

Clinical, functional, and radiological outcome in children with pleural empyema.

INTRODUCTION: Few studies have prospectively evaluated recovery process and long-term consequences of pleural space infections. OBJECTIVE: To evaluate clinical, pulmonary, and diaphragmatic function and radiological outcome in patients hospitalized with pleural empyema. MATERIAL AND METHODS: Previously healthy patients from 6 to 16 years were enrolled. Demographic, clinical, and treatment data were registered. At hospital discharge, and every 30 days or until normalization, patients underwent a clinical evaluation, diaphragmatic ultrasound, and lung function testing. Chest radiographs were performed at subsequent visits only if abnormalities persisted. RESULTS: Thirty patients were included. Nineteen (63%) were male, with an age of (mean ± SD) 9.7 ± 3.2 years, and body mass index (mean ± SD) 18.6 ± 3. Twelve patients (40%) were treated with chest tube drainage only, 12 (40%) exclusively with surgery, and 6 (20%) completed treatment with surgery due to an ineffective chest tube drainage. At hospital discharge, 26 (87%) of patients had abnormal breath sounds at the site of infection, 28 (93%) had a spirometric restrictive pattern, 19 (63%) diaphragmatic motion impairment, and 29 (97%) presented radiological involvement of pleural space, mainly pleural thickening. All patients had recovered diaphragmatic motion and were asymptomatic at 90- and 120-day follow-up control, respectively. Then, with a great individual variability, radiological findings, and lung function returned to normal at 60 days (range 30-180) and 90 days (range 30-180) after hospital discharge, respectively. CONCLUSION: Patients with pleural empyema had a complete and progressive recovery, with initial clinical and diaphragmatic motion normalization followed by radiological and lung function recovery.

Pulmonary function of a paediatric cohort of patients with postinfectious bronchiolitis obliterans. A long term follow-up.

BACKGROUND: Postinfectious bronchiolitis obliterans (BO) is a chronic respiratory disease that usually follows a severe adenovirus infection. OBJECTIVE: To determine the evolution of pulmonary function and clinical outcome of children with postinfectious BO during childhood. METHODS: The study included patients diagnosed with postinfectious BO in whom at least two spirometries were performed within a minimum interval of 3 months. RESULTS: 46 met the inclusion criteria. The mean (±SD) follow-up period was 12.5 (±3.5) years. 197 spirometries and 41 plethysmographies were performed. Initial (9±3 years old) lung function was as follows (z score, mean±SD): forced vital capacity (FVC) -3.8±1; forced expiratory volume in 1 s (FEV1) -4.4±1; FEV1/FVC -2.2±1; forced expiratory flow (FEF)(25-75) -3.7±1; total lung capacity (TLC) 120±26%; residual volume (RV) 309±108%; and RV/TLC 55±13. During childhood, FVC and FEV1 increased by a mean of 11%/year (95% CI 9.3% to 12.6%; p<0.0001) and 9%/year (95% CI 7.7% to 10.2%; p<0.0001), and the FEV1/FVC ratio decreased by 1.9%/year (95% CI 1% to 2.8; p<0.001). The z score for FVC, FEV1 and FEV1/FVC decreased by 0.07 z score/year (95% CI 0.1 to 0.01; p<0.05), 0.09 z score/year (95% CI 0.1 to 0.05; p<0.01) and 0.04 z score/year (95% CI 0.09 to 0.001; p<0.02), respectively. During the follow-up period, 69% of patients required at least one hospital readmission and five required mechanical ventilation. Nine patients developed a thoracic deformity, and seven whose bronchiectasis did not respond to clinical treatment underwent a lobectomy. CONCLUSIONS: After a 12 year follow-up period, pulmonary function remained severely impaired, showing an obstructive pattern with air trapping that slowly improved during childhood. An unequal growth of lung parenchyma over the airways suggests dysinaptic growth. Patients required frequent readmission due to recurrent respiratory infections, and hypoxaemia improved slowly over time.

Original salbutamol versus similar salbutamol in children with asthma exacerbation: a randomized, controlled, double-blind study.

INTRODUCTION: There is clinical evidence suggesting that original salbutamol is more effective than a similar salbutamol product to revert symptoms in acute asthma exacerbation.. OBJECTIVE: To evaluate the bronchodilator response of both salbutamol medicinal products in children with asthma and to establish, based on the forced expiratory volume, if there is a difference between the group treated with the original salbutamol and the group treated with similar salbutamol. DESIGN: Prospective, randomized, controlled, double-blind study. MATERIAL AND METHODS: One hundred and twenty six children (63 boys, age 9.18 ± 2.83 years old) were included. They were administered a dose of 20 drops (5 mg) of the original salbutamol or similar salbutamol product in nebulizing solution diluted only once in 2 ml saline solution. Pre- and post-bronchodilator, intra- and inter-group forced expiratory volume was compared at baseline and at 30 minutes. The weight of salbutamol drops was determined by gravimetry, the concentration by chromatography and the number of drops by bottle. RESULTS: The bronchodilator response between the pre- and post-bronchodilator forced expiratory volume was 225 ml (95% CI: 164-286) and 224 ml (95% CI: 163-284) for original salbutamol and similar salbutamol, respectively (p < 0.001). The Delta difference was 1.3 ml (95% CI: -86+83) (p = 0.97). The mean, standard deviation and variation coefficient percentage of the weight of the drop was 364.75 mg (± 6.01, 1.65) and 543.88 mg (± 56.09, 10.31) (p < 0.001) for original salbutamol and similar salbutamol, respectively. CONCLUSION: There were no differences in the bronchodilator response measured by FEV1 between the original salbutamol and a similar salbutamol product.

[Pulmonary hemorrhage associated with celiac disease]. / Hemorragia pulmonar asociada a enfermedad celíaca.

Idiopathic pulmonary hemosiderosis is a severe and potentially fatal disease characterized by recurrent episodes of alveolar hemorrhage, hemoptysis, and anemia. His association with celiac disease, described as Lane- Hamilton syndrome, could be due to the fact that both entities share a common pathogenic immune pathway. We report two patients of 13 years who consulted for hemoptysis and severe anemia that had not responded to immunosuppressive treatment with pulses of methyl prednisolone, oral meprednisone and hydroxychloroquine. Although both children highlight the absence of gastrointestinal symptoms at the time of consultation, the dosage of anti-endomysial and anti-transglutaminase antibodies was positive and biopsy confirmed the presence of intestinal enteropathy. It is emphasized that in patients with diffuse alveolar hemorrhage, even in the absence of gastrointestinal symptoms, the concomitant presence of celiac disease should be evaluated. If celiac disease is present, the incorporation of a gluten-free diet helps to control the symptoms, allows reducing the immunosuppressive treatment and improves the clinical course of both entities.

Original salbutamol versus similar salbutamol in children with asthma exacerbation: A randomized, controlled, double-blind study / Salbutamol original contra salbutamol similar en niños con crisis de asma: Estudio aleatorizado, controlado y doble ciego

Introduction.There is clinical evidence suggesting that original salbutamol is more effective than a similar salbutamol product to revert symptoms in acute asthma exacerbation.. Objective. To evaluate the bronchodilator response of both salbutamol medicinal products in children with asthma and to establish, based on the forced expiratory volume, if there is a difference between the group treated with the original salbutamol and the group treated with similar salbutamol. Design. Prospective, randomized, controlled, double-blind study. Material and Methods. One hundred and twenty six children (63 boys, age 9.18 ± 2.83 years old) were included. They were administered a dose of 20 drops (5 mg) of the original salbutamol or similar salbutamol product in nebulizing solution diluted only once in 2 ml saline solution. Preand post-bronchodilator, intra- and inter-group forced expiratory volume was compared at baseline and at 30 minutes. The weight of salbutamol drops was determined by gravimetry, the concentration by chromatography and the number of drops by bottle. Results. The bronchodilator response between the pre- and post-bronchodilator forced expiratory volume was 225 ml (95% CI: 164-286) and 224 ml (95% CI: 163-284) for original salbutamol and similar salbutamol, respectively (p < 0.001). The Delta difference was 1.3 ml (95% CI: -86+83) (p = 0.97). The mean, standard deviation and variation coefficient percentage of the weight of the drop was 364.75 mg (± 6.01, 1.65) and 543.88 mg (± 56.09, 10.31) (p < 0.001) for original salbutamol and similar salbutamol, respectively. Conclusion. There were no differences in the bronchodilator response measured by FEV1 between the original salbutamol and a similar salbutamol product.(AU)

Introducción. Existe evidencia clínica que sugiere que el salbutamol original sería más eficaz que el salbutamol similar para revertir los síntomas en el episodio agudo de asma. Objetivo. Evaluar la respuesta broncodilatadora de ambas especialidades farmacéuticas de salbutamol en niños con asma y establecer, mediante el volumen espiratorio forzado, si difiere entre los grupos tratados con salbutamol original y similar. Diseño. Estudio prospectivo, aleatorizado, controlado, a doble ciego. Material y métodos. Se incluyeron 126 niños (63 varones, edad 9,18 ± 2,83 años), que recibieron una dosis de 20 gotas (5 mg) de salbutamol original o similar en solución para nebulizar diluida en 2 ml de solución fisiológica por única vez. Se comparó el volumen espiratorio forzado prebroncodilatador y posbroncodilatador, intragrupos e intergrupos, al inicio y a los 30 minutos. Se determinó el peso de las gotas de salbutamol por gravimetría, la concentración por cromatografía y el número de gotas por envase. Resultados. La respuesta broncodilatadora entre el volumen espiratorio forzado prebroncodilatador y posbroncodilatador fue de 225 ml (IC 95%: 164-286) y 224 ml (IC 95%: 163-284) para salbutamol original y similar, respectivamente (p <0,001). El delta de la diferencia fue de 1,3 ml (IC 95%: -86+83) (p= 0,97). La media, desvío estándar y porcentaje del coeficiente de variación del peso de las gotas fue de 364,75 mg (± 6,01, 1,65) y 543,88 mg (± 56,09, 10,31) (p <0,001) para salbutamol original y similar, respectivamente. Conclusión. No hubo diferencias en la respuesta broncodilatadora medida por el VEF1 entre salbutamol original y similar.(AU)