Seguimiento de niños con diagnóstico de asma grave antes y durante la pandemia por COVID-19 / Follow-up of children diagnosed with severe asthma before and during the COVID-19 pandemic

En la pandemia por COVID-19 se exploraron estrategias de atención para garantizar el seguimiento de niños con asma grave. Estudio prospectivo, observacional, comparativo. Se incluyeron pacientes del programa de asma grave de un hospital pediátrico de tercer nivel (n 74). Se evaluó el grado de control, exacerbaciones y hospitalizaciones durante un período presencial (PP), marzo 2019-2020, y uno virtual (PV), abril 2020-2021. En el PP, se incluyeron 74 pacientes vs. 68 (92 %) del PV. En el PP, el 68 % (46) de los pacientes presentaron exacerbaciones vs. el 46 % (31) de los pacientes en el PV (p 0,003). En el PP, se registraron 135 exacerbaciones totales vs. 79 en el PV (p 0,001); hubo una reducción del 41 %. En el PP, el 47 % (32) de los pacientes tuvieron exacerbaciones graves vs. el 32 % (22) de los pacientes en el PV (p 0,048). Hubo 91 exacerbaciones graves en el PP vs. 49 en el PV (p 0,029), reducción del 46 %. No hubo diferencias en las hospitalizaciones (PP 10, PV 6; p 0,9). La telemedicina fue efectiva para el seguimiento de pacientes con asma grave

During the COVID-19 pandemic, health care strategies were explored to ensure the follow-up of children with severe asthma. This was a prospective, observational, and comparative study. Patients in the severe asthma program of a tertiary care children's hospital were included (n: 74). The extent of control, exacerbations, and hospitalizations during an in-person period (IPP) (March 2019­2020) and an online period (OP) (April 2020­2021) was assessed. A total of 74 patients were enrolled in the IPP compared to 68 (92%) in the OP. During the IPP, 68% (46) of patients had exacerbations versus 46% (31) during the OP (p = 0.003). During the IPP, 135 total exacerbations were recorded compared to 79 during the OP (p = 0.001); this accounted for a 41% reduction. During the IPP, 47% (32) of patients had severe exacerbations versus 32% (22) during the OP (p = 0.048). A total of 91 severe exacerbations were recorded during the IPP compared to 49 during the OP (p = 0.029); the reduction was 46%. No differences were observed in terms of hospitalization (IPP: 10, OP: 6; p = 0,9). Telemedicine was effective for the follow-up of patients with severe asthma.

Follow-up of children diagnosed with severe asthma before and during the COVID-19 pandemic. / Seguimiento de niños con diagnóstico de asma grave antes y durante la pandemia por COVID-19.

During the COVID-19 pandemic, health care strategies were explored to ensure the follow-up of children with severe asthma. This was a prospective, observational, and comparative study. Patients in the severe asthma program of a tertiary care children's hospital were included (n: 74). The extent of control, exacerbations, and hospitalizations during an in-person period (IPP) (March 2019-2020) and an online period (OP) (April 2020-2021) was assessed. A total of 74 patients were enrolled in the IPP compared to 68 (92%) in the OP. During the IPP, 68% (46) of patients had exacerbations versus 46% (31) during the OP (p = 0.003). During the IPP, 135 total exacerbations were recorded compared to 79 during the OP (p = 0.001); this accounted for a 41% reduction. During the IPP, 47% (32) of patients had severe exacerbations versus 32% (22) during the OP (p = 0.048). A total of 91 severe exacerbations were recorded during the IPP compared to 49 during the OP (p = 0.029); the reduction was 46%. No differences were observed in terms of hospitalization (IPP: 10, OP: 6; p = 0,9). Telemedicine was effective for the follow-up of patients with severe asthma.

En la pandemia por COVID-19 se exploraron estrategias de atención para garantizar el seguimiento de niños con asma grave. Estudio prospectivo, observacional, comparativo. Se incluyeron pacientes del programa de asma grave de un hospital pediátrico de tercer nivel (n 74). Se evaluó el grado de control, exacerbaciones y hospitalizaciones durante un período presencial (PP), marzo 2019-2020, y uno virtual (PV), abril 2020-2021. En el PP, se incluyeron 74 pacientes vs. 68 (92 %) del PV. En el PP, el 68 % (46) de los pacientes presentaron exacerbaciones vs. el 46 % (31) de los pacientes en el PV (p 0,003). En el PP, se registraron 135 exacerbaciones totales vs. 79 en el PV (p 0,001); hubo una reducción del 41 %. En el PP, el 47 % (32) de los pacientes tuvieron exacerbaciones graves vs. el 32 % (22) de los pacientes en el PV (p 0,048). Hubo 91 exacerbaciones graves en el PP vs. 49 en el PV (p 0,029), reducción del 46 %. No hubo diferencias en las hospitalizaciones (PP 10, PV 6; p 0,9). La telemedicina fue efectiva para el seguimiento de pacientes con asma grave.

Bronchogenic Cyst: Lessons Learned in 20 Years of Experience at a Tertiary Pediatric Center.

BACKGROUND: Bronchogenic cysts are benign congenital malformations of the primitive ventral foregut. The aim of this study is to analyze and report 20 years of experience in the diagnosis and treatment of bronchogenic cysts at a tertiary pediatric center. METHODS: A retrospective review was conducted of all patients diagnosed with a bronchogenic cyst between 2000-2020. Presence of symptoms, cyst location, surgical technique, postoperative complications, need for pleural drainage, and recurrence were reviewed. RESULTS: Forty-five children were included in the study. In 37 patients a partial resection of the cyst was done, followed by cauterization or chemical obliterateration with iodopovidone of the mucosa of the remaining cyst wall that was adherent to the airway. A lobectomy was done in patients who had intrapulmonary cysts (n = 8). Cyst location was subcarinal in 23 (51.1%), paratracheal in 14 (31.1%), and intrapulmonary in eight patients (17.8%). The majority of subcarinal and paratracheal cysts (90%) were approached by thoracoscopy. Complications occurred in seven patients (15%): subcutaneous emphysema after pleural drain removal in one, extubation failure in two, reoperation due to bleeding in one, surgical site infection in one, bronchopleural fistula in one, and pneumothorax in one. Reoperation due to cyst recurrence was necessary in two patients (4.4%). Mean follow-up was 56 months (range, 0-115). CONCLUSION: A minimally invasive approach is a safe option for the management of paratracheal and subcarinal bronchogenic cysts with no history of infection in specialized pediatric surgery center. Thoracoscopic partial resection is a feasible option in most patients with subcarinal and paratracheal bronchogenic cysts with a low complication and reoperation rate. LEVEL OF EVIDENCE: IV.

Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso / Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report

El sarcoma sinovial pleuropulmonar (SSPP) es un tumor primario de pulmón, maligno, infrecuente en pediatría (prevalencia 0,1-0,5 %) que afecta predominantemente a adolescentes y adultos jóvenes. Se ha descrito una sobrevida global cercana al 30 % a los 5 años. Se reporta el caso de un paciente de 12 años de edad, previamente sano, que presentó tos, dolor torácico y disnea de comienzo súbito, como manifestación inicial de neumotórax izquierdo, el que persistió a los 4 días y requirió resección quirúrgica de lesión bullosa pulmonar. Se realizó diagnóstico histológico de sarcoma sinovial pleuropulmonar confirmado por estudio molecular, que evidenció la translocación cromosómica entre el cromosoma X y el 18: t(X;18) (p11.2;q11.2) de la pieza quirúrgica extirpada. Ante pacientes con neumotórax persistente o recidivante, es importante descartar causas secundarias, entre ellas, sarcoma sinovial pleuropulmonar. Su ominoso pronóstico determina la necesidad de arribar a un diagnóstico temprano e implementar un tratamiento agresivo

Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1­0.5%) that predominantly affects adolescents and young adults. Overall survival has been reported to be close to 30% at 5 years. Here we report the case of a previously healthy 12-year-old male patient who presented with cough, chest pain, and dyspnea of sudden onset as initial manifestation of left pneumothorax, which persisted after 4 days and required surgical resection of pulmonary bullous lesion. A histological diagnosis of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, which showed chromosomal translocation between chromosomes X and 18: t(X;18) (p11.2;q11.2) in the surgical specimen removed. In patients with persistent or recurrent pneumothorax, it is important to rule out secondary causes, including pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for early diagnosis and aggressive treatment.

Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report. / Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso.

Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1-0.5%) that predominantly affects adolescents and young adults. Overall survival has been reported to be close to 30% at 5 years. Here we report the case of a previously healthy 12-year-old male patient who presented with cough, chest pain, and dyspnea of sudden onset as initial manifestation of left pneumothorax, which persisted after 4 days and required surgical resection of pulmonary bullous lesion. A histological diagnosis of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, which showed chromosomal translocation between chromosomes X and 18: t(X;18) (p11.2;q11.2) in the surgical specimen removed. In patients with persistent or recurrent pneumothorax, it is important to rule out secondary causes, including pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for early diagnosis and aggressive treatment.

El sarcoma sinovial pleuropulmonar (SSPP) es un tumor primario de pulmón, maligno, infrecuente en pediatría (prevalencia 0,1-0,5 %) que afecta predominantemente a adolescentes y adultos jóvenes. Se ha descrito una sobrevida global cercana al 30 % a los 5 años. Se reporta el caso de un paciente de 12 años de edad, previamente sano, que presentó tos, dolor torácico y disnea de comienzo súbito, como manifestación inicial de neumotórax izquierdo, el que persistió a los 4 días y requirió resección quirúrgica de lesión bullosa pulmonar. Se realizó diagnóstico histológico de sarcoma sinovial pleuropulmonar confirmado por estudio molecular, que evidenció la translocación cromosómica entre el cromosoma X y el 18: t(X;18) (p11.2;q11.2) de la pieza quirúrgica extirpada. Ante pacientes con neumotórax persistente o recidivante, es importante descartar causas secundarias, entre ellas, sarcoma sinovial pleuropulmonar. Su ominoso pronóstico determina la necesidad de arribar a un diagnóstico temprano e implementar un tratamiento agresivo.

[Pulmonary tuberculosis presenting as a lung mass in children: case report]. / Masa pulmonar como manifestación primaria de tuberculosis en pediatría: reporte de un caso.

Tuberculosis is one of the main causes of death due to infection around the world. Although tuberculosis frequently involves lung parenchyma, tuberculoma is a rare complication. We describe an atypical pulmonary presentation of tuberculosis. A 15-month-old girl, previously healthy, was referred to the pulmonology department due to fever, poor weight gain, and a 2-months persistent lung image on chest x-ray despite antibiotic therapy. She had been in frequent contact with a respiratory symptomatic subject. She was admitted to the hospital with a TST of 13 mm and a heterogeneous bulky mass in the left upper lobe at chest computed tomography. Three gastric lavages were Masa pulmonar como manifestación primaria de tuberculosis en pediatría: reporte de un caso Pulmonary tuberculosis presenting as a lung mass in children: case report done and the patient underwent exploratory thoracoscopy and lung biopsy, with positive cultures for Mycobacterium tuberculosis. The diagnosis of pulmonary tuberculoma was confirmed, and the patient received standard anti- tuberculosis therapy with a favorable evolution.

La tuberculosis es una de las principales causas infecciosas de muerte en el mundo y es endémica en Argentina. La mayoría de los casos de tuberculosis son de localización pulmonar; el tuberculoma una complicación infrecuente. Se describe un caso clínico de presentación pulmonar atípica de tuberculosis. Se trata de una niña de 15 meses, previamente sana, derivada a neumología por fiebre, mal progreso de peso e imagen persistente por 2 meses en la radiografía de tórax a pesar de haber recibido antibioticoterapia. Antecedente de contacto estrecho con persona sintomática respiratoria. Se internó para estudio, mostró una PPD de 13 mm y una masa voluminosa heterogénea en el lóbulo superior izquierdo en la tomografía computada de tórax. Se realizaron tres lavados gástricos y toracoscopia exploratoria con biopsia pulmonar con rescate de Mycobacterium tuberculosis en cultivos. Se diagnosticó tuberculoma pulmonar y realizó tratamiento estándar con fármacos antituberculosos con evolución favorable.

Masa pulmonar como manifestación primaria de tuberculosis en pediatría: reporte de un caso / Pulmonary tuberculosis presenting as a lung mass in children: case report

La tuberculosis es una de las principales causas infecciosas de muerte en el mundo y es endémica en Argentina. La mayoría de los casos de tuberculosis son de localización pulmonar; el tuberculoma una complicación infrecuente. Se describe un caso clínico de presentación pulmonar atípica de tuberculosis. Se trata de una niña de 15 meses, previamente sana, derivada a neumología por fiebre, mal progreso de peso e imagen persistente por 2 meses en la radiografía de tórax a pesar de haber recibido antibioticoterapia. Antecedente de contacto estrecho con persona sintomática respiratoria. Se internó para estudio, mostró una PPD de 13 mm y una masa voluminosa heterogénea en el lóbulo superior izquierdo en la tomografía computada de tórax. Se realizaron tres lavados gástricos y toracoscopia exploratoria con biopsia pulmonar con rescate de Mycobacterium tuberculosis en cultivos. Se diagnosticó tuberculoma pulmonar y realizó tratamiento estándar con fármacos antituberculosos con evolución favorable.

Tuberculosis is one of the main causes of death due to infection around the world. Although tuberculosis frequently involves lung parenchyma, tuberculoma is a rare complication. We describe an atypical pulmonary presentation of tuberculosis. A 15-month-old girl, previously healthy, was referred to the pulmonology department due to fever, poor weight gain, and a 2-months persistent lung image on chest x-ray despite antibiotic therapy. She had been in frequent contact with a respiratory symptomatic subject. She was admitted to the hospital with a TST of 13 mm and a heterogeneous bulky mass in the left upper lobe at chest computed tomography. Three gastric lavages were done and the patient underwent exploratory thoracoscopy and lung biopsy, with positive cultures for Mycobacterium tuberculosis. The diagnosis of pulmonary tuberculoma was confirmed, and the patient received standard anti- tuberculosis therapy with a favorable evolution.

Biological versus synthetic patch for the repair of congenital diaphragmatic hernia: 8-year experience at a tertiary center.

PURPOSE: The aim of this study is to compare outcome between small intestinal submucosal (SIS) and polytetrafluoroethylene (PTFE) patches in patients with congenital diaphragmatic hernia (CDH) with large muscular defects. MATERIAL AND METHODS: We performed a retrospective review of prospectively collected data of patients who underwent CDH repair from January 2011 to June 2019, with a minimum follow-up of 1 year. Data were collected regarding length of stay (LOS), mortality and recurrence. Kaplan-Meier analyses was used. RESULTS: In the study period, 195 patients with CDH were admitted, 176 underwent surgical repair and 19 died before surgery. Sixty-two (35%) required a patch. Over the last 5 years, our institution transitioned from the use of all SIS to all PTFE. SIS was used in 18 cases and PTFE in 25. Overall survival rate was 89% (174/195). There was no significant difference regarding LOS and mortality. Nine patients (50%) of SIS and 1 (4%) of PTFE repairs recurred (P < 0.01; log-rank test P <  0.01), with a time course of recurrence of 18 (SD = 13) and 8 months, respectively. Median follow-up was 60 months (SD: 23) for the SIS group and 26 (SD: 10) for the PTFE group. CONCLUSION: A significant lower recurrence was found using PTFE patches instead of SIS for the repair of large CDH. LEVEL OF EVIDENCE: Level 3. Retrospective comparative study.

Impacto clínico del tratamiento con omalizumab en niños con asma grave: Reporte de una experiencia local / Clinical impact of omalizumab treatment in children with severe asthma: Report of a local experience

El anticuerpo monoclonal anti-IgE omalizumab está indicado para tratamiento del asma grave. Estudio longitudinal (pre-posintervención), observacional y analítico con el objetivo de evaluar la evolución clínica y funcional de pacientes con asma grave no controlada, 16 semanas antes y después del tratamiento. En los 17 casos, se controló el asma (p= 0,00001). Se redujeron en un 48,5 % las exacerbaciones (p= 0,009) y en un 100 % las crisis graves (p= 0,001). Dieciséis pacientes (el 94 %) tuvieron exacerbaciones pretratamiento y 10 (el 59 %), luego del omalizumab (p= 0,005). No hubo hospitalizaciones (p= 0,007). Se redujo en un 20 % la dosis de corticoides inhalados (0,002) y el número de casos con corticoides orales continuos (p= 0,01); disminuyó el uso de salbutamol (p= 0,001) y de corticoides orales (p= 0,003). No se modificó la función pulmonar. Dos casos presentaron reacciones adversas leves. El omalizumab permitió un adecuado control de la enfermedad en pacientes con asma grave.

Omalizumab, an anti-IgE monoclonal antibody, is indicated for the treatment of severe asthma. A longitudinal (pre-/post-intervention), observational, analytical study was conducted to assess the clinical and functional course of patients with uncontrolled severe asthma, 16 weeks before and after treatment. Asthma was controlled in 17 cases (p = 0.00001). Exacerbations were reduced by 48.5 % (p = 0.009) and severe crises, by 100 % (p = 0.001). Before omalizumab treatment, 16 patients (94 %) had exacerbations, whereas 10 (59 %) had them after treatment (p = 0.005). None of the patients was hospitalized (p = 0.007). The dose of inhaled corticosteroids was reduced by 20 % (0.002); the number of patients using continuous oral corticosteroids (p = 0.01), salbutamol (p = 0.001), and oral corticosteroids (p=0.003) also decreased. Pulmonary function was not affected. Two patients had mild adverse reactions. Omalizumab achieved an adequate asthma control in patients with severe asthma.

Clinical impact of omalizumab treatment in children with severe asthma: Report of a local experience. / Impacto clínico del tratamiento con omalizumab en niños con asma grave: Reporte de una experiencia local.

Omalizumab, an anti-IgE monoclonal antibody, is indicated for the treatment of severe asthma. A longitudinal (pre-/post-intervention), observational, analytical study was conducted to assess the clinical and functional course of patients with uncontrolled severe asthma, 16 weeks before and after treatment. Asthma was controlled in 17 cases (p = 0.00001). Exacerbations were reduced by 48.5 % (p = 0.009) and severe crises, by 100 % (p = 0.001). Before omalizumab treatment, 16 patients (94 %) had exacerbations, whereas 10 (59 %) had them after treatment (p = 0.005). None of the patients was hospitalized (p = 0.007). The dose of inhaled corticosteroids was reduced by 20 % (0.002); the number of patients using continuous oral corticosteroids (p = 0.01), salbutamol (p = 0.001), and oral corticosteroids (p=0.003) also decreased. Pulmonary function was not affected. Two patients had mild adverse reactions. Omalizumab achieved an adequate asthma control in patients with severe asthma.

El anticuerpo monoclonal anti-IgE omalizumab está indicado para tratamiento del asma grave. Estudio longitudinal (pre-posintervención), observacional y analítico con el objetivo de evaluar la evolución clínica y funcional de pacientes con asma grave no controlada, 16 semanas antes y después del tratamiento. En los 17 casos, se controló el asma (p= 0,00001). Se redujeron en un 48,5 % las exacerbaciones (p= 0,009) y en un 100 % las crisis graves (p= 0,001). Dieciséis pacientes (el 94 %) tuvieron exacerbaciones pretratamiento y 10 (el 59 %), luego del omalizumab (p= 0,005). No hubo hospitalizaciones (p= 0,007). Se redujo en un 20 % la dosis de corticoides inhalados (0,002) y el número de casos con corticoides orales continuos (p= 0,01); disminuyó el uso de salbutamol (p= 0,001) y de corticoides orales (p= 0,003). No se modificó la función pulmonar. Dos casos presentaron reacciones adversas leves. El omalizumab permitió un adecuado control de la enfermedad en pacientes con asma grave.

Asma grave en pediatría: resultados de la implementación de un protocolo especial de atención / Severe asthma in pediatrics: Outcomes of the implementation of a special health care protocol

Introducción. El asma grave representa 5-7% del total de asmáticos. La OMS propuso un protocolo de seguimiento para categorizarlos como asma grave resistente al tratamiento (AGRT) o asma grave de difícil control (AGDC). Objetivo. Analizar las características clínicas, funcionales y terapéuticas de pacientes con AGRT o AGDC. Métodos. Estudio transversal, observacional y analítico para evaluar el diagnóstico, grado de control (clínico y funcional), comorbilidades, adherencia al tratamiento, técnica inhalatoria y factores ambientales en pacientes con asma grave. Resultados. Se incluyeron 69 pacientes: AGRT (n= 33) y AGDC (n= 36). El 100% del grupo con AGRT fue hospitalizado previamente por asma vs. 87,8% del grupo con AGDC (p= 0,03). El 63% del grupo AGRT requirió cuidados intensivos (UCI)), 82%, asistencia ventilatoria y uno fue traqueostomizado. En el AGDC, 54% requirió internación en la UCI , y 33%, asistencia ventilatoria (p= 0,03). La espirometría basal fue normal en el AGDC; se observó incapacidad ventilatoria obstructiva leve en el AGRT (p= 0,009). En el AGDC, hubo menor cumplimiento del tratamiento (p= 0,01). Se requirieron dosis mayores de corticoides inhalados en AGRT (p= 0,0001). Omalizumab fue indicado en AGRT (p= 0,0001). A los 6 meses de seguimiento, más del 75% de los niños en ambos grupos presentó asma controlada. Conclusiones. Se observó significativa falta de adherencia al tratamiento en el grupo AGDC. Se redujeron las dosis de tratamiento en este grupo. Se logró controlar la enfermedad en un alto porcentaje de niños con AGRT y AGDC.

Introduction. Severe asthma accounts for 5-7% of all asthma cases. The World Health Organization proposed a follow-up protocol to classify cases into severe, treatment-resistant asthma (STRA) or severe, difficult-to-control asthma (SDCA). Objective. To analyze the clinical, functional, and therapeutic characteristics of patients with STRA or SDCA. Methods. Cross-sectional, observational, and analytical study to assess the diagnosis, the extent of control (clinical and functional), comorbidities, treatment adherence, inhalation technique, and environmental factors in patients with severe asthma. Results. A total of 69 patients were included: STRA (n= 33) and SDCA (n= 36). In the group with STRA, 100% of patients had been previously hospitalized due to asthma compared to 87.8% in the group with SDCA (p= 0.03). In the group with STRA, 63% required admission to the intensive care unit (ICU); 82%, ventilatory support; and 1 patient, tracheostomy. In the group with SDCA, 54% required admission to the ICU; and 33%, ventilatory support (p= 0.03). The baseline spirometry was normal in the SDCA group; a mild obstructive ventilatory defect was observed in the STRA group (p= 0.009). In the SDCA group, treatment adherence was lower (p= 0.01). Higher inhaled corticosteroid doses were required in the STRA group (p= 0.0001). Omalizumab was indicated in the case of STRA (p= 0.0001). After 6 months of follow-up, more than 75% of children in both groups achieved asthma control. Conclusions. A significant lack of treatment adherence was observed in the SDCA group. In this group, treatment doses were reduced. Asthma was controlled in a high percentage of children with STRA and SDCA.

Severe asthma in pediatrics: Outcomes of the implementation of a special health care protocol. / Asma grave en pediatría: resultados de la implementación de un protocolo especial de atención.

INTRODUCTION: Severe asthma accounts for 5-7% of all asthma cases. The World Health Organization proposed a follow-up protocol to classify cases into severe, treatment-resistant asthma (STRA) or severe, difficult-to-control asthma (SDCA). OBJECTIVE: To analyze the clinical, functional, and therapeutic characteristics of patients with STRA or SDCA. METHODS: Cross-sectional, observational, and analytical study to assess the diagnosis, the extent of control (clinical and functional), comorbidities, treatment adherence, inhalation technique, and environmental factors in patients with severe asthma. RESULTS: A total of 69 patients were included: STRA (n= 33) and SDCA (n= 36). In the group with STRA, 100% of patients had been previously hospitalized due to asthma compared to 87.8% in the group with SDCA (p= 0.03). In the group with STRA, 63% required admission to the intensive care unit (ICU); 82%, ventilatory support; and 1 patient, tracheostomy. In the group with SDCA, 54% required admission to the ICU; and 33%, ventilatory support (p= 0.03).The baseline spirometry was normal in the SDCA group; a mild obstructive ventilatory defect was observed in the STRA group (p= 0.009).In the SDCA group, treatment adherence was lower (p= 0.01). Higher inhaled corticosteroid doses were required in the STRA group (p= 0.0001). Omalizumab was indicated in the case of STRA (p= 0.0001). After 6 months of follow-up, more than 75% of children in both groups achieved asthma control. CONCLUSIONS: A significant lack of treatment adherence was observed in the SDCA group. In this group, treatment doses were reduced. Asthma was controlled in a high percentage of children with STRA and SDCA.

INTRODUCCIÓN: El asma grave representa 5-7% del total de asmáticos. La OMS propuso un protocolo de seguimiento para categorizarlos como asma grave resistente al tratamiento (AGRT) o asma grave de difícil control (AGDC). OBJETIVO: Analizar las características clínicas, funcionales y terapéuticas de pacientes con AGRT o AGDC. MÉTODOS: Estudio transversal, observacional y analítico para evaluar el diagnóstico, grado de control (clínico y funcional), comorbilidades, adherencia al tratamiento, técnica inhalatoria y factores ambientales en pacientes con asma grave. RESULTADOS: Se incluyeron 69 pacientes: AGRT (n= 33) y AGDC (n= 36). El 100% del grupo con AGRT fue hospitalizado previamente por asma vs. 87,8% del grupo con AGDC (p= 0,03). El 63% del grupo AGRT requirió cuidados intensivos (UCI)), 82%, asistencia ventilatoria y uno fue traqueostomizado. En el AGDC, 54% requirió internación en la UCI , y 33%, asistencia ventilatoria (p= 0,03). La espirometría basal fue normal en el AGDC; se observó incapacidad ventilatoria obstructiva leve en el AGRT (p= 0,009).En el AGDC, hubo menor cumplimiento del tratamiento (p= 0,01). Se requirieron dosis mayores de corticoides inhalados en AGRT (p= 0,0001). Omalizumab fue indicado en AGRT (p= 0,0001). A los 6 meses de seguimiento, más del 75% de los niños en ambos grupos presentó asma controlada. CONCLUSIONES: Se observó significativa falta de adherencia al tratamiento en el grupo AGDC. Se redujeron las dosis de tratamiento en este grupo. Se logró controlar la enfermedad en un alto porcentaje de niños con AGRT y AGDC.

Series de Pediatría Garrahan. El niño con problemas respiratorios / Garrahan Pediatrics Series. The child with respiratory problems

La patología respiratoria presenta un gran desafío para las instituciones de salud, por su frecuencia, su complejidad diagnóstica y terapéutica y la carga que representa en costos económicos y vitales. Estos conceptos se extienden a todos los grupos etarios y sus características han ido cambiando a lo largo del tiempo ante los avances producidos en las inmunizaciones, los métodos diagnósticos y los tratamientos. Aún así, las infecciones respiratorias bajas son todavía la causa más frecuente de consulta, internación, morbilidad crónica, discapacidad y mortalidad en pediatría. Este nuevo volumen aborda esta temática especial y entre sus características se destacan: El estudio de las patologías más frecuentes en los diferentes ámbitos de atención pero en particular en el primer nivel, con una exposición centrada en los aspectos que facilitan el diagnóstico rápido y el tratamiento adecuado, con el menor uso de recursos y con pautas que fijan la derivación oportuna hacia el especialista o hacia una institución de mayor complejidad. El desarrollo de importantes temas, como la patología obstructiva de la vía aérea superior, su estudio diagnóstico y sus formas recurrentes; bronquiolitis; las intercurrencias respiratorias en pacientes con condiciones clínicas especiales; y la supuración pleuropulmonar. La inclusión, en todos los capítulos, de casos clínicos con su evolución y desenlace, textos destacados con los principales conceptos y puntos claves para recordar. Una obra sólida y práctica, que transmite la experiencia de los profesionales de una institución del prestigio internacional del Hospital dePediatría Prof. Dr. Juan P. Garrahan, dedicada a todos los pediatras, dondequiera que trabajen al servicio de la salud de los niños.

Risk factors for morbidities and mortality in children following pneumonectomy.

BACKGROUND AND OBJECTIVE: Pneumonectomy (PNE) is a procedure infrequently performed in children. A high morbidity/mortality rate associated with PNE has been described. Few series have been published in the last 15 years. Risk factors associated with morbidity/mortality after PNE were evaluated. Indications, course, survival and complications of PNE in children were also analized. METHODS: In a case series of 51 children who underwent PNE, death within 30 days of surgery, pneumonia, empyema, sepsis, adult respiratory distress syndrome, bronchopleural fistula, bleeding, pneumothorax and post-PNE syndrome were considered major morbidities. Scoliosis, wound infection and atelectasis were considered minor morbidities. RESULTS: Median age at PNE was 7.4 years; 45% were males. Indications of pneumonectomy were postinfectious bronchiectasis (61%), tumours (17%), pulmonary malformations (17%), aspiration syndrome (14%), cystic fibrosis (6%), immunodeficiency (4%) and trauma (2%). Mortality rate was 4% at 1 month. Major and minor morbidities were present in 23% and 27% of patients, respectively. Risk factors for development of morbidities after PNE were age ≤ 3 years (OR: 16.7; 95% CI: 2.4-117) and the need for mechanical ventilation for at least 4 days (OR: 8; 95% CI: 1.5-43.6). CONCLUSION: Children are at high risk of death, major and minor morbidities following PNE. Caution is recommended for this group of patients.

Primary lung tumors in children: 24 years of experience at a referral center.

PURPOSE: Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years. METHODS: A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990-2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome. RESULTS: Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91-16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology. CONCLUSION: Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.

Post-infectious bronchiolitis obliterans and mannose-binding lectin insufficiency in Argentinean children.

BACKGROUND AND OBJECTIVE: Post-infectious bronchiolitis obliterans (PIBO) is a severe disorder following acute lower pulmonary infection in young children, especially caused by adenovirus. Mannose-binding lectin (MBL) deficiency arising from polymorphisms in the coding and non-coding region on the MBL2 gene has been associated with more frequent and severe respiratory infections. Our aim was to evaluate the influence of MBL variants in the susceptibility and evolution of children with PIBO. METHODS: One hundred eleven children with PIBO diagnosis were studied. The coding A, B, D and X promoter variants of MBL2 gene were assessed by PCR-RFLP. B and D alleles were pooled as O. The combined genotypes A/A and YA/O were grouped as sufficient MBL (sMBL), and O/O and XA/O as insufficient MBL (iMBL) groups. To evaluate the frequency of MBL2 polymorphisms in the general population, we studied DNA samples from 127 healthy donors from the blood bank of the hospital (control group). RESULTS: iMBL variants were significantly more frequent in PIBO children compared with controls (21.6% vs 10.2%, P = 0.01). PIBO patients with iMBL required intensive care unit (P = 0.001) and mechanical assistance at the moment of viral injury (P = 0.001) more frequently than those with sMBL. CONCLUSIONS: Insufficiency of MBL was more common in PIBO children than in healthy controls. This genetic condition was significantly associated with more severe initial disease, illustrating the relevance of innate immune defence factors prior to the maturation of the adaptative immune system.

Series de Pediatría Garrahan. El niño que fue prematuro

A 27 años del nacimiento del Hospital Garrahan, sentimos la necesidad de ponernos en contacto con todos los pediatras para compartir con ellos las estrategias de atención del niño y su familia utilizadas en nuestra institución, destacar la importancia del trabajo interdisciplinario y colaboración permanente. En este volumen desarrollamos el tema del niño que fue prematuro, ya que estos pacientes presentan al pediatra desafíos específicos y novedosos. Si bien la prematurez es la principal causa de mortalidad infantil, la sobrevida de los prematuros ha aumentado significativamente en los últimos años, y por lo tanto estos niños acceden al cuidado pediátrico con creciente frecuencia y vulnerabilidad. Algunos de ellos pueden sufrir secuelas de distinto grado y en diferentes aspectos de su desarrollo, crecimiento o función respiratoria. Dado que el rol del pediatra es fundamental en el diagnóstico, la prevención y el tratamiento de posibles secuelas, el reconocimiento de sus necesidades especiales y cuidado centrado en la familia, el profesional encontrará aquí aspectos epidemiológicos, de identificación y prevención de riesgos, comunicación con la familia, oportunidades de interconsultas y abordaje de la discapacidad mediante el análisis de casos clínicos.

Malformaciones pulmonares congénitas / Congenital lung malformations

Congenital lung malformations (CLM) comprise a heterogeneous group of lung diseases. They vary widely in their clinical presentation and severity, depending on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Patients with CLM can present respiratory symptoms at birth or can remain asymptomatic for long periods. There has been an increase in early diagnosis of CLM attributable to the routine use of prenatal ultrasound. Management of these lesions depends on the type of malformation and symptoms. Treatment of asymptomatic patients is controversial, because the prognosis of these diseases is unpredictable. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. This review describes the principal CLM, their diagnosis, and the controversies regarding treatment.

Las malformaciones pulmonares congénitas (MPC) constituyen un grupo heterogéneo de enfermedades. Varían en su presentación clínica y gravedad en función al grado de afectación pulmonar y a su ubicación en tórax. Se pueden manifestar a cualquier edad y pueden condicionar morbilidad y mortalidad significativa en lactantes y niños. Los pacientes pueden presentar síntomas respiratorios desde el nacimiento o permanecer asintomáticos durante largos períodos. Se ha observado un aumento en el diagnóstico precoz de las MPC atribuible al uso rutinario de la ecografía prenatal. El manejo de estas lesiones depende del tipo de malformación y de los síntomas. El tratamiento de pacientes asintomáticos es controvertido debido a que el pronóstico de estas enfermedades es desconocido. La mayoría de los autores recomiendan la resección de la lesión en el momento del diagnóstico debido al riesgo de complicaciones. Esta revisión describe las principales malformaciones congénitas del pulmón, su diagnóstico y las estrategias de tratamiento.

Postinfectious bronchiolitis obliterans in children: the South American contribution.

UNLABELLED: Postinfectious bronchiolitis obliterans (PIBO) is an infrequent chronic lung that causes irreversible obstruction and, or, obliteration of the smaller airways. This review particularly focuses on more than 30 studies from South America. CONCLUSION: The initial PIBO event occurs in the early years of life and is strongly associated with adenovirus infection and the need for mechanical ventilator support. Treatment requires a multidisciplinary strategy. Multicentre studies are needed to determine progression, optimal management and long-term follow-up.