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Langerhans cell histiocytosis (LCH) is a rare inflammatory disorder of myeloid dendritic cells with mutations involving KRAS, BRAF and/or NRAS, and MAP2K1 genes. We describe the case of a 58-year-old female previous smoker with multifocal LCH involving the lungs, pituitary gland and mandibular bone. Initial treatment with 6 cycles of cladribine showed improvement in her extrapulmonary lesions, however, her lung disease progressed and after qualification and assessment tests she underwent uncomplicated double lung transplant surgery and was discharged home. We highlight that in select patients with well managed and controlled extrapulmonary LCH, such an invasive procedure as lung transplant is possible.
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Histiocitosis de Células de Langerhans , Trasplante de Pulmón , Cladribina/uso terapéutico , Femenino , Histiocitosis de Células de Langerhans/diagnóstico , Histiocitosis de Células de Langerhans/tratamiento farmacológico , Histiocitosis de Células de Langerhans/patología , Humanos , Trasplante de Pulmón/efectos adversos , Persona de Mediana Edad , Mutación , Proteínas Proto-Oncogénicas B-raf/genética , Proteínas Proto-Oncogénicas B-raf/metabolismo , Proteínas Proto-Oncogénicas B-raf/uso terapéuticoRESUMEN
OBJECTIVE: To evaluate short- and long-term changes in quality of life (QoL) in patients undergoing transcatheter aortic valve implantation (TAVI) and to assess differences in patient QoL when using the TAVI transaortic (TAVI TAo) approach compared with the transfemoral approach (TAVI TF) and surgical aortic valve replacement (SAVR). METHODS: Ninety-seven patients were assessed. Thirty-two patients underwent TAVI TAo, 31 underwent TAVI TF and 34 patients underwent SAVR. QoL was assessed using the EQ-5D-3L questionnaire at baseline, after one month and one year. RESULTS: Mean patient age was 80 years (range, 61-92 years) and the mean logistic EuroSCORE was 12.45% (range, 1.39%-78.98%). Declared health state at baseline was significantly lower in TAVI TF (P < 0.001) and after one month there were no differences between the three groups (P = 0.99). After one year, SAVR patient results of the EQ-5D-3L index value were lower in comparison to both TAVI patient groups (P < 0.05). The analysis also showed significant differences between the results of EQ-5D-3L index value over the one month and one year follow-up (TAVI TAo, P < 0.001; TAVI TF, P < 0.05; SAVR, P < 0.05). In all groups, the values significantly increased after one-month and one-year of follow-up in comparison to baseline value. Significant differences were also demonstrated between Visual Analogue Scale values (VAS). CONCLUSIONS: A significant improvement in QoL was observed in all three patient groups. Regardless of the TAVI approach, EQ-5D-3L and VAS values were significantly increased after one-month and one-year follow up; the SAVR patients however, reported lower health status when compared to the TAVI patients.
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INTRODUCTION: Pulmonary hypertension (PH) is common complication in advanced lung disease. Echocardiography provides additional information and may be useful to assess PH probability. OBJECTIVES: The usefulness of combination of well-known echocardiographic parameters in detecting PH in patients with advanced lung disease referred for lung transplantation was evaluated. METHODS: The study population consisted of 37 consecutive patients with idiopathic pulmonary fibrosis (IPF), 20 patients with chronic obstructive pulmonary disease (COPD), and 8 patients with other interstitial lung diseases. PH was defined as mean pulmonary arterial pressure (mPAP) ≥25 mm Hg diagnosed by cardiac catheterization. RESULTS: PH was present in 67.6% of enrolled IPF patients, 30% of enrolled COPD patients, and 75% of patients with other interstitial lung diseases. The receiver operating characteristics (ROC) curve analysis demonstrated right ventricular systolic pressure (RVSP) ≥43 mm Hg to be the threshold for PH prediction (n = 37, sensitivity 92.3%, specificity 81.8%, area under curve (AUC) 0.84, 95% confidence interval (CI) 0.67-1.0; P = .019). Right ventricular outflow tract (RVOT) diameter ≥34 mm and tricuspid annular plane systolic excursion (TAPSE) ≤18 mm had acceptable sensitivity, specificity and AUC (n = 65, 62.2%, 89.3%, 0.77, 95% CI 0.66-0.89; P = .11 and n = 62, 77.1%, 66.7%, 0.74, CI 0.61-0.87; P = .27, respectively). Combination of RVSP, RVOT and TAPSE, obtained in 36 patients, increased the sensitivity and negative predictive value (NPV) to 100%. CONCLUSIONS: In patients with advanced lung diseases referred for lung transplantation the combination of RVSP, RVOT diameter, and TAPSE may be helpful in PH exclusion.
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Ecocardiografía/métodos , Ventrículos Cardíacos/diagnóstico por imagen , Hipertensión Pulmonar/diagnóstico por imagen , Enfermedades Pulmonares/diagnóstico , Válvula Tricúspide/diagnóstico por imagen , Adulto , Cateterismo Cardíaco/métodos , Femenino , Ventrículos Cardíacos/anatomía & histología , Ventrículos Cardíacos/fisiopatología , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/fisiopatología , Fibrosis Pulmonar Idiopática/complicaciones , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/fisiopatología , Enfermedades Pulmonares/complicaciones , Enfermedades Pulmonares/fisiopatología , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Trasplante de Pulmón/normas , Masculino , Persona de Mediana Edad , Enfermedad Pulmonar Obstructiva Crónica/complicaciones , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Pruebas de Función Respiratoria/métodos , Estudios Retrospectivos , Válvula Tricúspide/anatomía & histología , Válvula Tricúspide/fisiopatología , Prueba de Paso/métodosRESUMEN
INTRODUCTION: Epiaortic ultrasound scanning (EAS) extended the use of ultrasound to the intraoperative diagnosis of aortic pathology. Surgical palpation of the ascending aorta underestimates the presence and severity of atherosclerotic plaques. Epiaortic ultrasound scanning has been used as an adjunct to transesophageal echocardiography (TEE) or as a primary direct diagnostic tool for imaging the ascending aorta as well as the aortic arch, which gained prominence as part of a multipronged intraoperative strategy to reduce atherosclerotic emboli. AIM: To compare the epiaortic examination with transthoracic and transesophageal echo (transthoracic echocardiography (TTE) and TEE), X-ray, surgical intraoperative palpation, and postoperative neurological status. MATERIAL AND METHODS: The analyzed group consisted of 35 patients (mean age: 81.3 years) treated with aortic valve replacement (AVR), either alone (60%) or combined with coronary artery bypass grafting (CABG; 22.8%) or aortic aneurysm replacement (11.42%). In 2 patients, only CABG was performed because intraoperatively reevaluated strategy. Thierteen patients have got a history of diabetes, 10 chronic renal failure and 3 of cerebral stroke. RESULTS: In more than 80% of patients, positive EAS results had an influence on the choice of aortic clamping site and in 50% of patients on the site of cannulation. Female sex, peripheral vascular disease, history of previous stroke, and calcifications in the ascending aorta in TTE have significant predictive value for recognizing atherosclerotic changes in EAS and the risk of postoperative neurological complications in octogenarians treated with AVR. CONCLUSIONS: Epiaortic ultrasound scanning imaging is superior to TTE and manual palpation in the detection and localization of ascending atherosclerosis. This technique should be introduced as a standard perioperative examination in older patients at risk of neurological complications.
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Over the last twenty years, minimally invasive aortic valve replacement (MIAVR) has evolved into a safe, well-tolerated and efficient surgical treatment option for aortic valve disease. It has been shown to reduce postoperative morbidity, providing faster recovery and rehabilitation, shorter hospital stay and better cosmetic results compared with conventional surgery. A variety of minimally invasive accesses have been developed and utilized to date. This concise review demonstrates and discusses surgical techniques used in contemporary approaches to MIAVR and presents the most important results of MIAVR procedures.
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The human coronavirus NL63 is generally classified as a common cold pathogen, though the infection may also result in severe lower respiratory tract diseases, especially in children, patients with underlying disease, and elderly. It has been previously shown that HCoV-NL63 is also one of the most important causes of croup in children. In the current manuscript we developed a set of polymer-based compounds showing prominent anticoronaviral activity. Polymers have been recently considered as promising alternatives to small molecule inhibitors, due to their intrinsic antimicrobial properties and ability to serve as matrices for antimicrobial compounds. Most of the antimicrobial polymers show antibacterial properties, while those with antiviral activity are much less frequent. A cationically modified chitosan derivative, N-(2-hydroxypropyl)-3-trimethylammonium chitosan chloride (HTCC), and hydrophobically-modified HTCC were shown to be potent inhibitors of HCoV-NL63 replication. Furthermore, both compounds showed prominent activity against murine hepatitis virus, suggesting broader anticoronaviral activity.
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Antivirales/farmacología , Quitosano/farmacología , Coronavirus Humano NL63/efectos de los fármacos , Animales , Antivirales/química , Cationes/química , Cationes/farmacología , Línea Celular , Quitosano/química , Coronavirus Humano NL63/fisiología , Humanos , Macaca mulatta , Virus de la Hepatitis Murina/efectos de los fármacos , Replicación Viral/efectos de los fármacosRESUMEN
Rapid and accurate detection and identification of viruses causing respiratory tract infections is important for patient care and disease control. Despite the fact that several assays are available, identification of an etiological agent is not possible in ~30% of patients suffering from respiratory tract diseases. Therefore, the aim of the current study was to develop a diagnostic set for the detection of respiratory viruses with sensitivity as low as 1-10 copies per reaction. Evaluation of the assay using a training clinical sample set showed that viral nucleic acids were identified in ~76% of cases. To improve assay performance and facilitate the identification of novel species or emerging strains, cultures of fully differentiated human airway epithelium were used to pre-amplify infectious viruses. This additional step resulted in the detection of pathogens in all samples tested. Based on these results it can be hypothesized that the lack of an etiological agent in some clinical samples, both reported previously and observed in the present study, may result not only from the presence of unknown viral species, but also from imperfections in the detection methods used.