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

Introduction: We 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. Methods: We 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. Results: We 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 < 0.001 vs. CF controls). Selenium deficiency (Se < 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. Conclusion: 10% 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.

Mycobacterial infection after COVID-19 pneumonia / Infección por micobacterias tras neumonía por COVID-19

A 69-year-old man with a history of hypertrophic cardiomyopathy and major depressive disorder was admitted to the Emergency Department with fever, weakness, and shortness of breath. He was diagnosed with acute respiratory distress syndrome due to COVID-19 and received oxygen and steroids during a one-month hospital stay. After discharge, he continued steroids and home oxygen therapy for nearly two years. CT scans revealed bronchiectasis and ground glass opacities related to COVID-19. He developed pulmonary nodules and M. intracellulare infection, which were treated with rifampicin, ethambutol, and azithromycin. After six months of treatment, the patient showed clinical and radiological improvement (AU)

Un hombre de 69 años con antecedentes de miocardiopatía hipertrófica y trastorno depresivo mayor acudió a urgencias por fiebre, debilidad y dificultad respiratoria. Se le diagnosticó síndrome de distrés respiratorio agudo debido a COVID-19 y fue ingresado en planta de neumología, donde recibió oxígeno y esteroides durante 1 mes. Después del alta continuó con esteroides y oxigenoterapia domiciliaria durante casi 2 años. Las tomografías objetivaron bronquiectasias y opacidades en vidrio deslustrado relacionadas con la COVID-19. Desarrolló nódulos pulmonares e infección por Mycobacterium intracellulare, siendo tratado con rifampicina, etambutol y azitromicina. Después de 6 meses de tratamiento, el paciente mostró mejoría clínica y radiológica (AU)

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.

Real Life With Tezacaftor and Ivacaftor in Adult Patients With Cystic Fibrosis: Spanish Multicenter Study

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

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.

Desaturations During 6-Minute Walk Test and Predicting Nocturnal Desaturations in Adult Subjects With Cystic Fibrosis.

BACKGROUND: Nocturnal desaturation in cystic fibrosis (CF) may have prognostic implications because a significant and maintained nocturnal desaturation can contribute to the development and progression of pulmonary hypertension with cor pulmonale. Its relation with the desaturation in exercise has not been sufficiently studied. We aimed to determine whether desaturation during 6MWT can be an indicator of nocturnal desaturation in adult subjects with CF. METHODS: 57 subjects were included: 50.9% male, 27.5 ± 7.7 y old, mean FEV1 = 2.37 ± 0.74 L, and %FEV1 67 ± 18.1%. Desaturation during 6MWT was defined as oxygen saturation (SpO2 ) ≤ 90% or a decline of > 4 points in SpO2 from baseline, and nocturnal desaturation as a desaturation index > 4 or > 5% of sleep time with SpO2 ≤ 90%. RESULTS: Desaturation observed during 6MWT in adult subjects with CF did not correlate with nocturnal desaturation (P = .27). Subjects with %FEV1 ≤ 55% and diffusion capacity of carbon monoxide (DLCO) ≤ 50 mmol/min/mm Hg were at higher risk of 6MWT desaturation. Nocturnal desaturation was more frequent in males, with PaO2 ≤ 71 mm Hg in blood gas analysis. CONCLUSIONS: Desaturation observed in 6MWT cannot predict desaturation at night in adults with CF. Other parameters were identified as predictors of desaturation.

[Inhaled antibiotic therapy in cystic fibrosis]. / Antibioterapia inhalada en la fibrosis quística.

Cystic fibrosis is the most frequent fatal genetically-transmitted disease among Caucasians. Chronic bronchial infection, especially by Pseudomonas aeruginosa, is the main cause of morbidity and mortality in this disease. Aerosolized antibiotic therapy achieves high drug concentrations in the airway with low toxicity, allowing chronic use. Currently, two antibiotics have been approved for inhalation therapy, tobramycin inhalation solution and colistimethate sodium aerosol. There is less evidence from clinical trials for the latter. The main indication for these drugs is chronic bronchial colonization by P. aeruginosa, although there is increasing evidence of the importance of the primary infection by this bacterium, whether treated by oral or intravenous antibiotics or not. More controversial is the use of aerosolized antibiotic therapy in bacterial prophylaxis or respiratory exacerbations. For many years, intravenous formulations of distinct antibiotics for aerosolized use have been employed, which are in distinct phases of research for use in nebulizer therapy. In addition to being used to treat P. aeruginosa infection, aerosolized antibiotics have been used to treat other pathogens such as methicillin-resistant Staphylococus aureus, Mycobacterium abscessus and Aspergillus fumigatus.

Antibioterapia inhalada en la fibrosis quística / Inhaled antibiotic therapy in cystic fibrosis

La fibrosis quística es la enfermedad genética letal más frecuente en la población caucasiana. La infecciónbronquial crónica, especialmente por Pseudomonas aeruginosa, es la principal causa de morbimortalidad deesta patología. El tratamiento antibiótico por aerosol alcanza altas concentraciones en la vía aérea con bajatoxicidad, por lo que permite el empleo crónico. En la actualidad hay 2 antibióticos aprobados para su usoinhalatorio, la tobramicina en solución para inhalación y el colistimetato de sodio, existiendo con este últimomenos evidencias en estudios clínicos. La indicación fundamental es la colonización bronquial crónica por P.aeruginosa, aunque cada vez se demuestra más relevancia en la primoinfección por esta bacteria, acompañadao no de antibióticos por vía oral o intravenosos. Más controvertido es el uso de la aerosolterapia antibiótica enla profilaxis bacteriana o en la exacerbación respiratoria. Durante muchos años se han estado empleandoformulaciones intravenosas de distintos antibióticos en aerosol, las cuales están en distintas fases de investigaciónpara su lanzamiento como presentación por vía nebulizada. Además de su indicación en el tratamientode la infección por P. aeruginosa se han empleado otros antibióticos en aerosol para otros patógenos comoStaphylococus aureus resistentes a meticilina, Mycobacterium abscessus o Aspergillus fumigatus(AU)

Cystic fibrosis is the most frequent fatal genetically-transmitted disease among Caucasians. Chronic bronchialinfection, especially by Pseudomonas aeruginosa, is the main cause of morbidity and mortality in this disease.Aerosolized antibiotic therapy achieves high drug concentrations in the airway with low toxicity, allowingchronic use. Currently, two antibiotics have been approved for inhalation therapy, tobramycin inhalationsolution and colistimethate sodium aerosol. There is less evidence from clinical trials for the latter. The mainindication for these drugs is chronic bronchial colonization by P. aeruginosa, although there is increasingevidence of the importance of the primary infection by this bacterium, whether treated by oral or intravenousantibiotics or not. More controversial is the use of aerosolized antibiotic therapy in bacterial prophylaxis orrespiratory exacerbations. For many years, intravenous formulations of distinct antibiotics for aerosolized usehave been employed, which are in distinct phases of research for use in nebulizer therapy. In addition to beingused to treat P. aeruginosa infection, aerosolized antibiotics have been used to treat other pathogens such asmethicillin-resistant Staphylococus aureus, Mycobacterium abscessus and Aspergillus fumigatus(AU)

Aislamiento de Pandoraea pulmonicola en paciente con fibrosis quística / Pandoraea pulmonicola isolation in patients with cystic fibrosis

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