Fungal microbiota dynamics and its geographic, age and gender variability in patients with cystic fibrosis

[Objectives] In cystic fibrosis (CF), there is a predisposition to bronchial colonization by potentially pathogenic microorganisms, such as fungi. Our aims were to describe the dynamics of respiratory mycobiota in patients with CF and to evaluate the geographic, age and gender variability in its distribution.[Methods] Cohort study in which 45 patients with CF from four hospitals in three Spanish cities were followed up during a 1-year period, obtaining spontaneous sputum samples every 3 to 6 months. Fungal microbiota were characterized by Internal Transcribed Spacer sequencing and Pneumocystis jirovecii was identified by nested PCR in a total of 180 samples.[Results] The presence of fungi were detected in 119 (66.11%) of the 180 samples and in 44 (97.8%) of the 45 patients: 19 were positive and 1 negative throughout all follow-ups and the remaining 25 presented alternation between positive and negative results. A total of 16 different genera were identified, with Candida spp. (50/180, 27.78%) and Pneumocystis spp. (44/180, 24.44%) being the most prevalent ones. The distribution of fungal genera was different among the evaluated centres (p < 0.05), by age (non-adults aged 6–17 years vs. adults aged ≥18 years) (p < 0.05) and by gender (p < 0.05).[Discussion] A high prevalence of fungal respiratory microbiota in patients with CF was observed, whose dynamics are characterized by the existence of multiple cycles of clearance and colonization, reporting the existence of geographic, age and gender variability in the distribution of fungal genera in this disease.The study was supported by the Spanish Ministry of Science and Innovation (grant number FIS-PS09/00957), by Plan Andaluz de Investigación, Desarrollo e Innovación, Consejería de Economía Conocimiento, Empresas y Universidad de la Junta de Andalucía (grant number PS20_00894), and by Consejería de Salud y Familia, Junta de Andalucía (grant number CSyF Exp. RH-0061/2021).Peer reviewe

The cardiomyopathy of cystic fibrosis: a modern form of Keshan disease

IntroductionWe conducted a study to determine the prevalence of structural heart disease in patients with CF, the characteristics of a cardiomyopathy not previously described in this population, and its possible relationship with nutritional deficiencies in CF.MethodsWe studied 3 CMP CF patients referred for heart-lung transplantation and a prospective series of 120 adult CF patients. All patients underwent a clinical examination, blood tests including levels of vitamins and trace elements, and echocardiography with evaluation of myocardial strain. Cardiac magnetic resonance imaging (CMR) was performed in patients with CMP and in a control group. Histopathological study was performed on hearts obtained in transplant or necropsy.ResultsWe found a prevalence of 10% (CI 4.6%–15.4%) of left ventricular (LV) dysfunction in the prospective cohort. Myocardial strain parameters were already altered in CF patients with otherwise normal hearts. Histopathological examination of 4 hearts from CF CMP patients showed a unique histological pattern of multifocal myocardial fibrosis similar to Keshan disease. Four of the five CF CMP patients undergoing CMR showed late gadolinium uptake, with a characteristic patchy pattern in 3 cases (p &lt; 0.001 vs. CF controls). Selenium deficiency (Se &lt; 60 µg/L) was associated with more severe LV dysfunction, higher prevalence of CF CMP, higher NTproBNP levels, and more severe pulmonary and digestive involvement.Conclusion10% of adults with CF showed significant cardiac involvement, with histological and imaging features resembling Keshan disease. Selenium deficiency was associated with the presence and severity of LV dysfunction in these patients

Empiema pleural asociado a lipoma endobronquial

Los tumores benignos broncopulmonares representan menos del 4% de las neoplasias de origen pulmonar. El lipoma endobronquial es una neoplasia benigna extremadamente rara, cuya incidencia oscila entre el 0,1 y el 0,5% de todos los tumores del pulmón. Los síntomas clínicos dependen de su localización, del grado de obstrucción bronquial y de las consecuencias morfofuncionantes de dicha obstrucción sobre el parénquima distal. La tomografía axial computarizada suele poner de manifiesto el contenido adiposo del tumor. Presentamos el caso clínico de un varón de 83 años diagnosticado de neumonía adquirida en la comunidad, que se complicó con un empiema pleural por Haemophilus influenzae y atelectasia de lóbulos medio e inferior derecho, secundaria a obstrucción endobronquial por lipoma. Se realizó extirpación del lipoma bronquial mediante fotorresección con láser

Polirradiculitis y enfermedad de Wegener

La enfermedad de Wegener es una vasculitis granulomatosa sistémica de etiología desconocida, que afecta sobre todo al tracto respiratorio superior e inferior y al riñón, aunque se distingue una forma limitada al pulmón. Es una entidad poco frecuente, con una incidencia estimada de 8,5 por 1.000.000 de habitantes/año. Los principales síntomas de comienzo suelen ser los derivados de la afectación de las vías aéreas superior e inferior. El sistema nervioso central se implica en alrededor de un 22% de los casos, pero es infrecuente que la clínica neurológica forme parte de la sintomatología inicial. Los anticuerpos anticitoplasma del neutrófilo específicos contra la proteinasa 3 constituyen marcadores serológicos de gran utilidad para el establecimiento del diagnóstico, aunque éste debe confirmarse por la biopsia tisular del órgano afectad

Impact of SARS-CoV-2 infection in patients with cystic fibrosis in Spain: Incidence and results of the national CF-COVID19-Spain survey

CF-COVID19-Spain Registry Group.[Background] Given the high incidence of confirmed infection by SARS-CoV-2 and mortality by COVID-19 in the Spanish population, its impact was analysed among persons with Cystic Fibrosis (CF) as a group at risk of a worse evolution. The possible causes of the incidence observed in them are explained and how CF Units have faced this health challenge is detailed.[Methods] Retrospective descriptive observational study, for which a Spanish CF Patients with Confirmed COVID-19 Registry is created, requesting information on number of people affected between 8 March–16 May 2020 and their clinical-demographic characteristics from the CF Units participating in the European Cystic Fibrosis Society Patient Registry (ECFSPR). The accumulated incidence is calculated, compared with that of the general population. Additionally, a survey (CF-COVID19-Spain) is carried out on prevention of SARS-CoV-2 infection, workings of CF Units and possible reasons for the incidence observed.[Results] COVID-19 was diagnosed in eight CF patients, one of whom had received a lung transplant. The accumulated incidence was 32/10000 in CF patients and 49/10000 in the general population. General death rate was 5.85/10000 while no CF patients included in the ECFSPR died. The characteristics of those affected and the results of the survey are described.[Conclusions] Despite being considered a disease at high risk of severe COVID-19, the low incidence and mortality in CF patients in Spain contrasts with the figures for the general population. The possible factors that would explain such findings are discussed, with the help of the results of the CF-COVID19-Spain survey