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Aim: To assess the relationship between air pollution and the mortality of IPF. Methods: The probability of death was analyzed from 32 IPF patients diagnosed in our ILD Unit from 2011 to 2019 and it was related to the values of (CO, NO2, PM2.5, PM10, O3 and SO2) in Madrid during that period. Results: Thirty-two patients were reviewed with a mean age of 66±10 years at the time of diagnosis, 7 (21.9%) of them were women. The average of the pollutants from the three months prior to the date of death of the 11 patients was (34.4%) patients who died was SO2=9.518±4.397μg/m2, NO2=58.910±8.819μg/m2, CO=0.390±0.104mg/m2, O3=38.778±15.309 μg/m2, PM2,5=12.371±2.138 μg/m2 PM10= 23.583±5.196 μg/m2 and the average air in the previous three months to the last clinic visit in 21 (65,6%) patients who are still alive was SO2 =8.736±1.719 μg/m2, NO2=56.216±9.586μg/m2, CO=0.237±0.106 mg/m2 O3=48.294±14,059 μg/m2 PM2,5=11.029±2,280 μg/m2 PM10=22.376±4,533 μg/m2 . Firstly, we observed that those 28 (87.5%) patients who were treated had no association with the probability of death [OR=7,500 (0.676-83.264)] (p=0.101) and after that we saw that pollution levels in all patients were not associated with the likelihood of dying: OR SO2= 1.154 (0.521-2.557) (p=0.723), OR CO=1.247 (0.970-1.602) (p=0.085), OR NO2 = 0.829 (0.468-1.468) (p=0.520), OR O3 = 0.944 (0.578-1.544) (p=0.820), OR PM2,5 = 0.646 (0.152-2.744) (p=0.554), OR PM10 = 1.302 (0.668-2.537) (p=0.439). Conclusion: There is no evidence to affirm that air pollution in Madrid is associated with the probability of death in IPF patients.

Tras la presentacion de un caso de enfermedad de Niemann-Pick (N-P) tipo B se realiza una revision de la literatura en la que se describen los hallazgos radiologicos, con especial hincapie en la afectacion pulmonar. Se trata de un paciente de 40 anos diagnosticado a los 6 anos de posible enfermedad de N-P sospechada por presentar hepatoesplenomegalia y patron reticular en radiografia de torax, con pruebas de funcion respiratoria inicialmente normales. Dicho diagnostico se confirmo mediante medicion de la actividad de la enzima esfingomielinasa acida (ASM) en cultivo de fibroblastos de piel y demostracion de la presencia de mutaciones en el gen de la ASM. Ante una enfermedad de deposito habria que tener siempre en cuenta la afectacion pulmonar, y la tecnica radiologica mas fiable es la tomografia computarizada de alta resolucion. Aunque el patron encrazy-paving no es el mas especifico de esta patologia, la enfermedad de N-P deberia estar en el diagnostico diferencial.

We have carried out a review of the literature after the presentation of a case of Niemann-Pick (N-P) disease type B reporting the radiological findings with special emphasis on lung affectation. The patient is 40 years old and was diagnosed at the age of 6 with possible N-P disease suspected due to the presentation of hepatosplenomegaly and reticular pattern on chest radiography, with initially normal lung function tests. Said diagnosis was confirmed by measuring the activity of acid sphingomyelinase (ASM) enzyme in skin fibroblast cultures and by demonstrating the presence of mutations in the ASM gene. In cases of storage disease, lung affectation should always be considered, and the most reliable radiological technique is high-resolution computed tomography. Although the “crazy-paving” pattern is not the most specific for this pathology, N-P disease should be included in the differential diagnosis.

We have carried out a review of the literature after the presentation of a case of Niemann-Pick (N-P) disease type B reporting the radiological findings with special emphasis on lung affectation. The patient is 40 years old and was diagnosed at the age of 6 with possible N-P disease suspected due to the presentation of hepatosplenomegaly and reticular pattern on chest radiography, with initially normal lung function tests. Said diagnosis was confirmed by measuring the activity of acid sphingomyelinase (ASM) enzyme in skin fibroblast cultures and by demonstrating the presence of mutations in the ASM gene. In cases of storage disease, lung affectation should always be considered, and the most reliable radiological technique is high-resolution computed tomography. Although the "crazy-paving" pattern is not the most specific for this pathology, N-P disease should be included in the differential diagnosis.