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.

Cystic fibrosis transmembrane regulator (CFTR) modulators treat defective CFTR protein. Our objective is to describe the course of children with cystic fibrosis treated with lumacaftor/ivacaftor. This is a case series of 13 patients aged 6 to 18 years with ≥ 6 months of treatment. Forced expiratory volume in the first second (FEV1), body mass index (BMI) Z-score, antibiotic therapy/year, before treatment and for 24 months after treatment were analyzed.At 12 months (9/13) and 24 months (5/13), the median change in the percent predicted FEV1 (ppFEV1) was 0.5 pp (-2-12) and 15 pp (8.7-15.2) and the BMI Z-score was 0.32 points (-0.2-0.5) and 1.23 points (0.3-1.6). In the first year, in 11/13 patients, the median number of days of antibiotic use decreased from 57 to 28 (oral) and from 27 to 0 (intravenous). Two children had associated adverse events.

Reporte de casos RESUMENLa 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.