Update in postinfectious bronchiolitis obliterans.

Postinfectious bronchiolitis obliterans (PiBO) is a rare and severe form of chronic obstructive lung disease caused by an infectious injury to the lower respiratory tract. The most commonly recognized inciting stimuli leading to PiBO are airway pathogens, such as adenovirus and Mycoplasma. PiBO is characterized by persistent and nonreversible airway obstruction, with functional and radiological evidence of small airway involvement. The literature has limited information on the aetiology, clinical profile, treatment, and outcome of PiBO.

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.

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.

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.

Función pulmonar en bronquiolitis obliterante postinfecciosa / Pulmonary function in post-infectious bronchiolitis obliterans

Bronchiolitis obliterans is a rare and severe chronic lung disease resulting from a lower respiratory tract injury. It may occur after a bone marrow or lung transplantation, infectious diseases, or less frequently after inhaling toxic substances or connective tissue diseases. Pathogenesis and molecular biology, as well as the best treatment of bronchiolitis obliterans, remain the subject of ongoing research. This review discusses our current knowledge of lung function of post-infectious bronchiolitis obliterans.

La bronquiolitis obliterante es una enfermedad pulmonar crónica rara y grave que resulta de una lesión del tracto respiratorio inferior. Puede ocurrir después de un trasplante de médula ósea o pulmón, enfermedades infecciosas, o menos frecuentemente después de inhalar sustancias tóxicas o después de enfermedades del tejido conectivo. La patogénesis y la biología molecular, así como el mejor tratamiento de la bronquiolitis obliterante, siguen siendo objeto de investigación. Esta revisión analiza nuestro conocimiento actual sobre la función pulmonar de los pacientes con bronquiolitis obliterante secundaria a infecciones.

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.

Post-infectious bronchiolitis obliterans.

Bronchiolitis obliterans is a rare and severe chronic lung disease resulting from a lower respiratory tract lesion. It may occur after a bone marrow or lung transplantation, infectious diseases, or less frequently after inhaling toxic substances or after connective tissue diseases. Pathology, pathogenesis, and molecular biology, as well as the best treatment of bronchiolitis obliterans, remain the subject of ongoing research. This review discusses our current knowledge of different areas of bronchiolitis obliterans associated with infectious lesions.

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.

Bronquiolitis obliterante post-infecciosa, los primeros adultos jóvenes / Post-infectious bronchiolitis obliterans, the first young adults

En cuanto al diagnóstico: Este desafío diagnóstico se plantea al comienzo de la enfermedad, es decir, cuando el paciente es menor de un año de edad. El objetivo de los métodos diagnósticos consistirá en demostrar la obliteración de la pequeña vía aérea, que es la lesión anatomopatológica de esta entidad. De esta manera, el mayor rendimiento es para la biopsia pulmonar, luego para la función pulmonar que demuestre la obstrucción severa y fija de la vía aérea y finalmente los signos indirectos de las imágenes pulmonares. Como fue expresado en el ateneo, los criterios validados para el diagnostico de esta enfermedad son la historia clínica típica, el antecedente de infección por adenovirus y el patrón en mosaico en la TAC. Con 2 de los 3 criterios la posibilidad del diagnóstico es elevada, si el score no es positivo no lo descarta. Interpreto, según lo expresado en el ateneo, que no se cumplen con los 2 primeros criterios y que el score es negativo. Por otro lado, el paciente presenta una función pulmonar compatible con BO, por lo que la sospecha diagnóstica de BO es elevada

Association between the Asthma Predictive Index and levels of exhaled nitric oxide in infants and toddlers with recurrent wheezing.

It is difficult to make an early identification of which children with recurrent wheezing will develop asthma in the following years. The Asthma Predictive Index (API) is a questionnaire based on clinical and laboratory parameters used for this end. The measurement of fractional exhaled nitric oxide (FE NO) has been used as a marker of eosinophilic airway infammation in asthma patients. Objective. To determine the association between the Asthma Predictive Index and FE NO levels in children younger than 3 years old with recurrent wheezing. Materials and methods. Observational, cross sectional study. Children younger than 36 months old with 3 or more episodes of bronchial obstruction in the past year who were inhaled corticosteroid-naive or leukotriene receptor antagonist-naive were included. After recording clinical data, FE NO was measured by a chemiluminescence analyzer during tidal breathing (online method). Results. A total of 52 children aged 5-36 months old were included. Patients with a positive API accounted for 60% of the population and had higher levels of FE NO than those with a negative API, with a median (range) of 13.5 ppb (0.7-31) versus 5.6 ppb (0.1-20.8), respectively (p <0.01). A high FE NO (>8 ppb) was observed in 74% of children with a positive API and in 26% of those with a negative API (p <0.01). Conclusions. This study found an association between high levels of exhaled nitric oxide and a positive Asthma Predictive Index in children younger than 3 years old with recurrent wheezing.

Asociación entre el índice de predicción de asma y el óxido nítrico exhalado en niños pequeños con sibilancias recurrentes / Association between the Asthma Predictive Index and levels of exhaled nitric oxide in infants ans toddlers with recurrent wheezing

Introducción. Es difícil identificar de manera temprana qué niños con sibilancias recurrentes desarrollarán asma en el futuro. El índice predictor de asma (API) es un cuestionario basado en parámetros clínicos y de laboratorio aplicado para este fin. La medición de la fracción exhalada de óxido nítrico (FE NO) se utiliza como un marcador de inflamación eosinofílica en las vías aéreas de los pacientes asmáticos. Objetivo. Determinar la asociación entre el índice predictor de asma y los valores de FE NO en menores de 3 años con sibilancias recurrentes. Materiales y métodos. Estudio observacional de corte transversal. Se incluyeron niños menores de 36 meses con tres o más episodios de obstrucción bronquial en el último año sin tratamiento previo con corticosteroides inhalados o antagonistas de los receptores de leucotrienos. Después de obtener los datos clínicos, se realizó la determinación de FE NO mediante un analizador de quimioluminiscencia mientras el paciente respiraba a volumen corriente (técnica on line). Resultados. Se incluyeron 52 niños de entre 5 y 36 meses de edad. Los pacientes con un índice (+) constituyeron el 60% de la población y presentaron valores de FE NO más elevados que los niños con un índice (-), mediana (rango) 13,5 (0,7 a 31) contra 5,6 (0,1 a 20,8) ppb, respectivamente (p <0,01). Se observó FE NO elevado (>8 ppb) en el 74% de los niños con API (+) y en el 26% de los niños con API (-) (p <0,01). Conclusiones. En el presente estudio se encontró una asociación entre los niveles elevados de óxido nítrico exhalado y un índice predictor de asma positivo en niños menores de 3 años con sibilancias recurrentes.(AU)

It is difficult to make an early identification of which children with recurrent wheezing will develop asthma in the following years. The Asthma Predictive Index (API) is a questionnaire based on clinical and laboratory parameters used for this end. The measurement of fractional exhaled nitric oxide (FE NO) has been used as a marker of eosinophilic airway infammation in asthma patients. Objective. To determine the association between the Asthma Predictive Index and FE NO levels in children younger than 3 years old with recurrent wheezing. Materials and methods. Observational, cross sectional study. Children younger than 36 months old with 3 or more episodes of bronchial obstruction in the past year who were inhaled corticosteroid-naive or leukotriene receptor antagonist-naive were included. After recording clinical data, FE NO was measured by a chemiluminescence analyzer during tidal breathing (online method). Results. A total of 52 children aged 5-36 months old were included. Patients with a positive API accounted for 60% of the population and had higher levels of FE NO than those with a negative API, with a median (range) of 13.5 ppb (0.7-31) versus 5.6 ppb (0.1-20.8), respectively (p <0.01). A high FE NO (>8 ppb) was observed in 74% of children with a positive API and in 26% of those with a negative API (p <0.01). Conclusions. This study found an association between high levels of exhaled nitric oxide and a positive Asthma Predictive Index in children younger than 3 years old with recurrent wheezing.(AU)

Asociación entre el índice de predicción de asma y el óxido nítrico exhalado en niños pequeños con sibilancias recurrentes / Association between the Asthma Predictive Index and levels of exhaled nitric oxide in infants ans toddlers with recurrent wheezing

Introducción. Es difícil identificar de manera temprana qué niños con sibilancias recurrentes desarrollarán asma en el futuro. El índice predictor de asma (API) es un cuestionario basado en parámetros clínicos y de laboratorio aplicado para este fin. La medición de la fracción exhalada de óxido nítrico (FE NO) se utiliza como un marcador de inflamación eosinofílica en las vías aéreas de los pacientes asmáticos. Objetivo. Determinar la asociación entre el índice predictor de asma y los valores de FE NO en menores de 3 años con sibilancias recurrentes. Materiales y métodos. Estudio observacional de corte transversal. Se incluyeron niños menores de 36 meses con tres o más episodios de obstrucción bronquial en el último año sin tratamiento previo con corticosteroides inhalados o antagonistas de los receptores de leucotrienos. Después de obtener los datos clínicos, se realizó la determinación de FE NO mediante un analizador de quimioluminiscencia mientras el paciente respiraba a volumen corriente (técnica on line). Resultados. Se incluyeron 52 niños de entre 5 y 36 meses de edad. Los pacientes con un índice (+) constituyeron el 60% de la población y presentaron valores de FE NO más elevados que los niños con un índice (-), mediana (rango) 13,5 (0,7 a 31) contra 5,6 (0,1 a 20,8) ppb, respectivamente (p <0,01). Se observó FE NO elevado (>8 ppb) en el 74% de los niños con API (+) y en el 26% de los niños con API (-) (p <0,01). Conclusiones. En el presente estudio se encontró una asociación entre los niveles elevados de óxido nítrico exhalado y un índice predictor de asma positivo en niños menores de 3 años con sibilancias recurrentes.

It is difficult to make an early identification of which children with recurrent wheezing will develop asthma in the following years. The Asthma Predictive Index (API) is a questionnaire based on clinical and laboratory parameters used for this end. The measurement of fractional exhaled nitric oxide (FE NO) has been used as a marker of eosinophilic airway infammation in asthma patients. Objective. To determine the association between the Asthma Predictive Index and FE NO levels in children younger than 3 years old with recurrent wheezing. Materials and methods. Observational, cross sectional study. Children younger than 36 months old with 3 or more episodes of bronchial obstruction in the past year who were inhaled corticosteroid-naive or leukotriene receptor antagonist-naive were included. After recording clinical data, FE NO was measured by a chemiluminescence analyzer during tidal breathing (online method). Results. A total of 52 children aged 5-36 months old were included. Patients with a positive API accounted for 60% of the population and had higher levels of FE NO than those with a negative API, with a median (range) of 13.5 ppb (0.7-31) versus 5.6 ppb (0.1-20.8), respectively (p <0.01). A high FE NO (>8 ppb) was observed in 74% of children with a positive API and in 26% of those with a negative API (p <0.01). Conclusions. This study found an association between high levels of exhaled nitric oxide and a positive Asthma Predictive Index in children younger than 3 years old with recurrent wheezing.

Association between the Asthma Predictive Index and levels of exhaled nitric oxide in infants and toddlers with recurrent wheezing. / Association between the Asthma Predictive Index and levels of exhaled nitric oxide in infants and toddlers with recurrent wheezing.

It is difficult to make an early identification of which children with recurrent wheezing will develop asthma in the following years. The Asthma Predictive Index (API) is a questionnaire based on clinical and laboratory parameters used for this end. The measurement of fractional exhaled nitric oxide (FE NO) has been used as a marker of eosinophilic airway infammation in asthma patients. Objective. To determine the association between the Asthma Predictive Index and FE NO levels in children younger than 3 years old with recurrent wheezing. Materials and methods. Observational, cross sectional study. Children younger than 36 months old with 3 or more episodes of bronchial obstruction in the past year who were inhaled corticosteroid-naive or leukotriene receptor antagonist-naive were included. After recording clinical data, FE NO was measured by a chemiluminescence analyzer during tidal breathing (online method). Results. A total of 52 children aged 5-36 months old were included. Patients with a positive API accounted for 60

of the population and had higher levels of FE NO than those with a negative API, with a median (range) of 13.5 ppb (0.7-31) versus 5.6 ppb (0.1-20.8), respectively (p <0.01). A high FE NO (>8 ppb) was observed in 74

of children with a positive API and in 26

of those with a negative API (p <0.01). Conclusions. This study found an association between high levels of exhaled nitric oxide and a positive Asthma Predictive Index in children younger than 3 years old with recurrent wheezing.

[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.

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

La hemosiderosis pulmonar idiopática (HPI) es una enfermedad grave y potencialmente fatal caracterizada por episodios recurrentes de hemorragia alveolar, hemoptisis y anemia. Su asociación con enfermedad celíaca (EC), descripta como síndrome de Lane-Hamilton, podría deberse a que ambas entidades comparten una misma vía inmunopatogénica. Se presentan dos pacientes de 13 años que consultaron por hemoptisis y anemia grave que no habían respondido al tratamiento inmunosupresor con pulsos de metilprednisolona, meprednisona e hidroxicloroquina. En ambos niños se destaca la ausencia de síntomas gastrointestinales al momento de la consulta, pero el dosaje de anticuerpos antiendomisio y antitransglutaminasa fue positivo, y la biopsia de intestino confirmó la presencia de enteropatía. En el plan de estudios de pacientes con síndrome de hemorragia alveolar difusa, aún en ausencia síntomas gastrointestinales, corresponde evaluar la presencia concomitante de enfermedad celíaca. En su presencia, la incorporación de una dieta libre de gluten favorece el control de los síntomas, permite reducir el tratamiento inmunosupresor y mejora la evolución clínica de ambas entidades.

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.

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

La hemosiderosis pulmonar idiopática (HPI) es una enfermedad grave y potencialmente fatal caracterizada por episodios recurrentes de hemorragia alveolar, hemoptisis y anemia. Su asociación con enfermedad celíaca (EC), descripta como síndrome de Lane-Hamilton, podría deberse a que ambas entidades comparten una misma vía inmunopatogénica. Se presentan dos pacientes de 13 años que consultaron por hemoptisis y anemia grave que no habían respondido al tratamiento inmunosupresor con pulsos de metilprednisolona, meprednisona e hidroxicloroquina. En ambos niños se destaca la ausencia de síntomas gastrointestinales al momento de la consulta, pero el dosaje de anticuerpos antiendomisio y antitransglutaminasa fue positivo, y la biopsia de intestino confirmó la presencia de enteropatía. En el plan de estudios de pacientes con síndrome de hemorragia alveolar difusa, aún en ausencia síntomas gastrointestinales, corresponde evaluar la presencia concomitante de enfermedad celíaca. En su presencia, la incorporación de una dieta libre de gluten favorece el control de los síntomas, permite reducir el tratamiento inmunosupresor y mejora la evolución clínica de ambas entidades.(AU)

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.(AU)

Clinical prediction rule to diagnose post-infectious bronchiolitis obliterans in children.

RATIONALE: Infant pulmonary function testing has a great value in the diagnosis of post-infectious bronchiolitis obliterans (BOs), because of characteristic patterns of severe and fixed airway obstruction. Unfortunately, infant pulmonary function testing is not available in most pediatric pulmonary centers. OBJECTIVE: To develop and validate a clinical prediction rule (BO-Score) to diagnose children under 2 years of age with BOs, using multiple objectively measured parameters readily available in most medical centers. METHODS: Study subjects, children under 2 years old with a chronic pulmonary disease assisted at R. Gutierrez Children's Hospital of Buenos Aires. Patients were randomly divided into a derivation (66%) and a validation (34%) set. ROC analyses and multivariable logistic regression included significant clinical, radiological, and laboratory predictors. The main outcome measure was a diagnosis of BOs. The performance of the BO-Score was tested on the validation set. RESULTS: Hundred twenty-five patients were included, 83 in the derivation set and 42 in the validation set. The BO-Score (area under ROC curve = 0.96; 95% CI, 0.9-1.0%) was developed by assigning points to the following variables: typical clinical history (four points), adenovirus infection (three points), and high-resolution computed tomography with mosaic perfusion (four points). A Score > or =7 predicted the diagnosis of BOs with a specificity of 100% (95% CI, 79-100%) and a sensitivity of 67% (95% CI, 47-80%). CONCLUSIONS: The BO-Score is a simple-to-use clinical prediction rule, based on variables that are readily available. A BO-Score of 7 or more predicts a diagnosis of post-infectious BOs with high accuracy.

[Postinfectious bronchiolitis obliterans]. / Bronquiolitis obliterante posinfecciosa.

Bronchiolitis obliterans is an uncommon and severe form of chronic obstructive lung disease that results from an insult to the lower respiratory tract. The bronchiolitis obliterans was described as a complication of graft versus host disease in bone marrow or lung transplant recipients. Bronchiolitis obliterans is most commonly seen in children after severe viral lower respiratory tract infections. The understanding of pathology, pathogenesis and molecular pathology, as well as the best treatment in bronchiolitis obliterans remain the subject of ongoing investigations. This review discusses our current knowledge on the different areas of bronchiolitis obliterans associated to infectious disease.

Bronquiolitis obliterante posinfecciosa / Postinfectious bronchiolitis obliterans

La bronquiolitis obliterante es una enfermedad pulmonar crónica infrecuente y grave producto de una lesión del tracto respiratorio inferior.Puede ocurrir luego de un trasplante de médula ósea o de pulmón, de enfermedades infecciosas o, menos frecuentemente, luego de inhalaciones tóxicas o enfermedades del tejido conectivo. La patología, patogenia y biología molecular, como así también el mejor tratamiento para la bronquiolitisobliterante son áreas aún en investigación. Esta revisión discute el estado actual del conocimiento en las diferentes áreas de la bronquiolitis obliterante secundaria a una lesión infecciosa.

Bronchiolitis obliterans is an uncommon and severe form of chronic obstructive lung disease that results from an insult tothe lower respiratory tract. The bronchiolitis obliterans was described as a complication of graft versus host disease in bone marrow or lung transplant recipients. Bronchiolitis obliterans is most commonly seen in children after severe viral lower respiratory tract infections. The understanding of pathology, pathogenesis and molecular pathology, as well as the best treatment in bronchiolitis obliterans remain the subject of ongoing investigations. This review discusses our current knowledge on the different areas of bronchiolitisobliterans associated to infectious disease.