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1.
Pulm Circ ; 13(3): e12274, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37609358

RESUMEN

Chronic thromboembolic pulmonary hypertension (CTEPH) is successfully treatable with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty, and medical therapy. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management risk score (RRS) is able to predict long-term outcome in inoperable patients or in patients with residual PH after surgery. We performed a post hoc analysis of RRS in patients who were enrolled in the CTREPH study (NCT01416636), a randomized, double-blind clinical trial comparing high-dose and low-dose subcutaneous (SC) treprostinil in patients with severe CTEPH that was classified by an interdisciplinary CTEPH team as nonoperable, or as persistent or recurrent pulmonary hypertension after PEA. Baseline mean RRS was similar in both treatment groups (8.7 in high-dose arm vs. 8.6 in low-dose arm), but mean RRS change from baseline to Week 24 was greater in the high-dose treprostinil group than in the low-dose treprostinil group (-0.88 vs. -0.17). The difference in RRS change from baseline to Week 24 between high dose versus low dose was statistically significant with mean difference of -0.70 (95% confidence interval: -1.36 to -0.05, p = 0.0352), and was driven mainly by improvement of World Health Organization functional class and N-terminal pro-brain natriuretic peptide concentration. SC treprostinil therapy administered in standard dose had positive effect on the risk profile measured by RRS in patients with inoperable or persistent/recurrent severe CTEPH. Although our study was limited by the small sample size and post hoc nature, assessment of risk profile is of great importance to this particular patient population with very poor prognosis.

2.
Int J Cardiol ; 384: 31-36, 2023 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-37087053

RESUMEN

BACKGROUND: The exact interaction of factors leading to myocardial dysfunction and fibrosis of the systemic right ventricle (SRV) is not completely understood. Myocardial ischemia and injury associated with a supply-demand mismatch of the pressure overloaded SRV are thought to play an important role, however studies confirming this are lacking. METHODS: Adult SRV patients were included in this single centre cohort study. All patients underwent a comprehensive diagnostic and imaging workup. A two-day stress-rest SPECT was performed to assess myocardial perfusion. SRV ischemia was defined as decreased segmental tracer uptake during exercise with significant improvement at rest. Contrast enhanced cardiac magnetic resonance imaging (CMR) was also performed in a subgroup of patients without contraindication, to assess focal myocardial fibrosis. Differences between patients with and without SRV ischemia were assessed. RESULTS: Twenty-three SRV patients (15 with transposition of the great arteries after atrial switch procedure and 8 with congenitally corrected transposition of the great arteries; 5 (22%) females; mean age 38 ± 11 years) were included. Seven (30%) patients had SRV ischemia on SPECT. Late gadolinium enhancement on CMR was more common in patients with SRV ischemia (p = 0.002). However, there was no association between SRV ischemia and different echocardiographic or CMR parameters of SRV systolic function, laboratory markers (high-sensitivity troponin I and NT-proBNP) and exercise capacity. CONCLUSIONS: Our multimodality study showed that SRV ischemia in adult SRV patients was associated with more focal myocardial fibrosis, but not with functional or imaging markers of SRV function.


Asunto(s)
Enfermedad de la Arteria Coronaria , Isquemia Miocárdica , Transposición de los Grandes Vasos , Femenino , Humanos , Adulto , Persona de Mediana Edad , Masculino , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/cirugía , Ventrículos Cardíacos/diagnóstico por imagen , Estudios de Cohortes , Medios de Contraste , Gadolinio , Isquemia Miocárdica/diagnóstico por imagen , Fibrosis
3.
Pulm Circ ; 12(1): e12008, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-35506097

RESUMEN

Congenital extrahepatic portocaval shunt (CEPS) is a rare condition in which a rare congenital vascular anomaly of the portal system is present. CEPS may manifest as pulmonary arterial hypertension (PAH). When diagnosed and treated early, PAH can be reversible. We report a case of a previously asymptomatic woman, who manifested with severe pulmonary hypertension during pregnancy and was consequently diagnosed with CEPS. After unsuccessful medical treatment, urgent lung transplantation was done.

4.
Eur Heart J Suppl ; 22(Suppl H): H112-H114, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32884487

RESUMEN

Elevated blood pressure (BP) is a growing burden worldwide, contributing to over 10 million deaths each year. May Measurement Month (MMM) is a global initiative organized by the International Society of Hypertension aimed to raise awareness of high BP. In May 2018, we carried out an opportunistic cross-sectional survey of volunteers from different parts of the country aged ≥18 years. Blood pressure measurement followed the standard MMM protocol and statistical analysis mean of the last 2 of 3 readings was used, where these were unavailable additional imputations were performed. In total, 4883 individuals (61.0% female) were screened during the whole month of May in 91 primary and secondary health facilities, pharmacies and through an online survey. After multiple imputation, 2841 (58.2%) had HTN. Of individuals not receiving antihypertensive medication, 850 (29.4%) were hypertensive. Of those receiving antihypertensive medication, 1025 (51.5%) had uncontrolled BP. MMM18 was the largest BP screening campaign undertaken in Slovenia. A substantial number of people with possible HTN were identified and referred to general practitioners for further management. The high number of individuals with HTN, with newly diagnosed HTN and with uncontrolled BP despite medication, confirms a real need for such screening programmes in our country.

6.
ESC Heart Fail ; 7(3): 1161-1167, 2020 06.
Artículo en Inglés | MEDLINE | ID: mdl-32212326

RESUMEN

AIMS: Non-compaction cardiomyopathy (NCM) is a congenital heart disease characterized by an arrest of the myocardial compaction process. Although NCM patients have impaired formation of microvasculature, the functional impact of these changes remains undefined. We sought to analyse a potential correlation between myocardial ischemia and heart failure severity in NCM patients. METHODS AND RESULTS: We enrolled 41 NCM patients (28 male and 13 female), aged 21-70 years. In all patients, we have determined left ventricular end-diastolic volume (LVEDV), left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS) by echocardiography. At the same time, serum levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) have been measured, and myocardial single-photon emission computed tomography at rest and on stress was used to define significant myocardial ischemia defined as summed difference score ≥ 2. Myocardial ischemia has been demonstrated in 11 patients (27%, Group A), and 30 patients showed no significant ischemic changes (73%, Group B). The groups did not differ in sex, age, kidney, or liver function. When compared with Group B, Group A had significantly lower LVEF (35 ± 15% in Group A vs. 53 ± 11% in Group B, P < 0.001), higher LVEDV (188 ± 52 mL vs. 136 ± 52 mL, P = 0.007), lower GLS (-9.9 ± 5.2% vs. -14.5 ± 4.1%, P = 0.001), and higher NT-proBNP levels (1691 ± 1883 pg/mL vs. 422 ± 877 pg/mL, P = 0.006). Overall, higher summed difference score was associated with lower LVEF (r = -0.48, P = 0.001), higher LVEDV (r = 0.39, P = 0.012), lower GLS (r = 0.352, P = 0.024), and higher levels of NT-proBNP (r = 0.66, P < 0.001). CONCLUSIONS: The presence of myocardial ischemia in patients with NCM is associated with worse left ventricular function, dilation of the left ventricle, and more pronounced neurohumoral activation.


Asunto(s)
Cardiomiopatías , Insuficiencia Cardíaca , Cardiomiopatías/complicaciones , Cardiomiopatías/diagnóstico , Femenino , Insuficiencia Cardíaca/diagnóstico , Humanos , Masculino , Perfusión , Volumen Sistólico , Función Ventricular Izquierda
7.
Lancet Respir Med ; 7(3): 239-248, 2019 03.
Artículo en Inglés | MEDLINE | ID: mdl-30477763

RESUMEN

BACKGROUND: Treprostinil, a prostacyclin analogue, is effective for the treatment of pulmonary arterial hypertension. However, information is scarce regarding treprostinil for treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to examine the efficacy and safety of subcutaneous treprostinil in this setting. METHODS: In this 24-week, randomised, double-blind controlled trial, we enrolled patients with CTEPH, classified as non-operable, or with persistent or recurrent pulmonary hypertension after pulmonary endarterectomy, in six European expert centres in Austria, Czech Republic, Germany, and Poland. Patients in WHO functional class III or IV with a 6-min walk distance of 150-400 m were randomly assigned at a 1:1 allocation ratio to continuous high-dose subcutaneous treprostinil (target dose around 30 ng/kg per min at week 12) or low-dose subcutaneous treprostinil (target dose around 3 ng/kg per min at week 12). The primary endpoint was the change from baseline in 6-min walk distance at week 24. All patients who received at least one dose of the study drug were included in the intention-to-treat efficacy and safety analyses based on assessment of adverse events. The trial was registered at ClinicalTrialsRegister.eu EudraCT number 2008-006441-10 and ClinicalTrials.gov, number NCT01416636. FINDINGS: From March 9, 2009, to June 9, 2016, 105 patients were enrolled with 53 (50%) patients randomly assigned to high-dose and 52 (50%) patients to low-dose subcutaneous treprostinil. At week 24, marginal mean 6-min walk distance improved by 44·98 m (95% CI 27·52 to 62·45) in the high-dose group, and by 4·29 m (95% CI -13·34 to 21·92) in the low-dose group (treatment effect 40·69 m, 95% CI 15·86 to 65·53, p=0·0016). 12 serious adverse events were reported in ten (19%) of 52 patients from the low-dose group and 16 serious adverse events were reported in nine (17%) of 53 patients from the high-dose group. The most common treatment-related adverse events in both groups were infusion site pain and other infusion site reactions. INTERPRETATION: Treatment with subcutaneous treprostinil was safe, and improved exercise capacity in patients with severe CTEPH. Subcutaneous treprostinil provides a parenteral treatment option for patients of WHO functional class III or IV and those who do not tolerate other therapies or need combination treatment. FUNDING: SciPharm Sàrl.


Asunto(s)
Epoprostenol/análogos & derivados , Tolerancia al Ejercicio/efectos de los fármacos , Hipertensión Pulmonar , Embolia Pulmonar/complicaciones , Antihipertensivos/administración & dosificación , Antihipertensivos/efectos adversos , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Monitoreo de Drogas/métodos , Epoprostenol/administración & dosificación , Epoprostenol/efectos adversos , Femenino , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Inyecciones Subcutáneas , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Prueba de Paso/métodos
9.
Sarcoidosis Vasc Diffuse Lung Dis ; 34(2): 130-135, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-32476834

RESUMEN

Background: Previous studies have demonstrated a relationship between biomass of fungi exposure in the home and the risk of sarcoidosis. ß-glucan was present in the bronchial alveolar lavage fluid (BALF) of patients with sarcoidosis. The Kveim-Siltzbach test reagent (KSTR) induces a sarcoidosis specific, granulomatous, cutaneous response and was used to establish the diagnosis. To date, the granuloma-inducing component of KSTR is still unknown. The present study was undertaken to investigate the presence of ß-glucan in the lymph nodes of patients with sarcoidosis and to determine the relationship between the amounts of this agent with disease severity and to investigate the presence of ß-glucan in KSTR. Materials and methods: Lymph node aspirations were collected by transbronchial needle aspiration (TBNA) in region R4 or 7 from patients with newly diagnosed sarcoidosis. The samples were treated to isolate ß-glucan and analyzed using a Limulus-based assay. Cultures of Propionibacterium ac. and Mycobacterium gordonae as well as samples of Kveim-Siltzbach test reagent were analyzed to determine ß-glucan content. Results: A significant relationship was observed between the amount of the ß-glucan in the lymph nodes and the extent of granuloma formation in the lung parenchyma, and the size of the lymph nodes in the mediastinum (r=0.787, p=0.0001 and r=0.664, p<0.001 respectively, Spearman's test). The samples of Kveim-Siltzbach test reagent contained high levels of ß-glucan. Cultures of Propionibacterium ac. and Mycobacterium gordonae contained ß-glucan, the levels of which were lower in the Mycobacterium cultures. Comments: The results support the hypothesis that ß-glucan, and thus fungal exposure, are involved in the pathogenesis of sarcoidosis. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 130-135).

10.
J Nucl Cardiol ; 24(2): 507-517, 2017 04.
Artículo en Inglés | MEDLINE | ID: mdl-27796852

RESUMEN

OBJECTIVE: To test the hypothesis that, in the initial evaluation of patients with suspected coronary artery disease (CAD), stress myocardial perfusion imaging (MPI) would result in less downstream testing than coronary computed tomographic angiography (CCTA). METHODS: In this international, randomized trial, mildly symptomatic patients with an intermediate likelihood of having CAD, and asymptomatic patients at intermediate risk of cardiac events, underwent either initial stress-rest MPI or CCTA. The primary outcome was downstream noninvasive or invasive testing at 6 months. Secondary outcomes included cumulative effective radiation dose (ERD) and costs at 12 months. RESULTS: We recruited 303 patients (151 MPI and 152 CTA) from 6 centers in 6 countries. The initial MPI was abnormal in 29% (41/143) and CCTA in 56% (79/141) of patients. Fewer patients undergoing initial stress-rest MPI had further downstream testing at 6 months (adjusted OR 0.51, 95% CI 0.28-0.91, P = 0.023). There was a small increase in the median cumulative ERD with MPI (9.6 vs. 8.8 mSv, P = 0.04), but no difference in costs between the two strategies at 12 months. CONCLUSION: In the management of patients with suspected CAD, a strategy of initial stress MPI is substantially less likely to require further downstream testing than initial testing with CCTA. TRIAL REGISTRATION: clinicaltrials.gov identification number NCT01368770.


Asunto(s)
Angiografía por Tomografía Computarizada/métodos , Angiografía Coronaria/métodos , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Enfermedad de la Arteria Coronaria/patología , Pruebas de Función Cardíaca/métodos , Tamizaje Masivo/métodos , Imagen de Perfusión Miocárdica/métodos , Diagnóstico Precoz , Femenino , Humanos , Internacionalidad , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Reproducibilidad de los Resultados , Sensibilidad y Especificidad
11.
J Comput Assist Tomogr ; 41(3): 360-363, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-27861199

RESUMEN

OBJECTIVE: Our prospective, randomized, open-label study assessed the efficacy of a heart rate-lowering, adjusted-dose protocol with ivabradine prior to coronary computed tomographic angiography (CCTA). METHODS: Patients undergoing CCTA were randomized to 7 days of adjusted-dose ivabradine or standard care (ie, no additional medication). Heart rate and ß-blocker and antianxiety medication use on the day of the CCTA were recorded. RESULTS: One hundred one patients were randomized (mean age, 60 [SD, 13] years; 66% women). Significantly more patients on ivabradine had heart rates of 60 beats per minute or less at the time of the CCTA scan (48% vs 8%, P < 0.01); accordingly, fewer patients on ivabradine needed additional heart rate lowering with ß-blockers (40% vs 86%, P < 0.01), as well as antianxiety medication (18% vs 39%, P < 0.05), and also required lower doses of intravenous ß-blockers (4 [SD, 2] vs 7 [SD, 5] mg, P < 0.05). CONCLUSIONS: A 7-day premedication protocol with ivabradine effectively lowers heart rate in patients undergoing CCTA.


Asunto(s)
Benzazepinas/farmacología , Fármacos Cardiovasculares/farmacología , Angiografía Coronaria/métodos , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Frecuencia Cardíaca/efectos de los fármacos , Tomografía Computarizada por Rayos X/métodos , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Ivabradina , Masculino , Persona de Mediana Edad , Estudios Prospectivos
12.
J Occup Med Toxicol ; 11: 46, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27688795

RESUMEN

BACKGROUND: Composition of organic dust is very complex, involving particles of microbial, animal and plant origin. Several environmental exposure studies associate microbial cell wall agents in organic dust with various respiratory symptoms and diseases. The aim of the present study was to investigate the in vitro effects of the co-exposure of fungal cell wall agents (FCWAs) and bacterial lipopolysaccharide (LPS) on inflammatory immune responses of peripheral blood mononuclear cells (PBMCs) from patients with pulmonary sarcoidosis. METHODS: PBMCs from 22 patients with pulmonary sarcoidosis and 20 healthy subjects were isolated and stimulated in vitro with FCWAs (soluble and particulate (1 → 3)-ß-D-glucan, zymosan and chitosan) and/or LPS. Subsequently, cytokines were measured by ELISA and the mRNA expression of dectin-1, toll-like receptor 2 (TLR2), TLR4 and mannose receptor (MR) was analysed by real-time RT-PCR. RESULTS: Patients with sarcoidosis had a significantly higher secretion of inflammatory cytokines tumour necrosis factor-alpha (TNF-α), interleukin-6 (IL-6), IL-10 and IL-12 (1.7-fold, 2.0-fold, 2.2-fold, and 2.8-fold, respectively; all p < 0.05) after in vitro co-stimulation of PBMCs with FCWAs and LPS. We showed that PBMCs from patients with sarcoidosis had a higher baseline mRNA expression of dectin-1, TLR2, TLR4 and MR (6-fold, 11-fold, 18-fold, and 4-fold, respectively). Furthermore, we found a reduced expression of dectin-1, TLR2 and TLR4 after stimulation with FCWAs and/or LPS, although the reduction was significantly weaker in patients than in healthy subjects. CONCLUSIONS: In conclusion, co-stimulation with FCWAs and LPS of PBMC from patients with sarcoidosis caused a weaker reduction of dectin-1, TLR2, TLR4 receptors expression, which could increase the sensitivity of PBMCs, leading to excessive inflammatory cytokine responses and result in the development or progression of pulmonary sarcoidosis.

13.
Sarcoidosis Vasc Diffuse Lung Dis ; 32(4): 313-7, 2016 Jan 18.
Artículo en Inglés | MEDLINE | ID: mdl-26847098

RESUMEN

BACKGROUND: Chitotriosidase (CTO) was shown to be a good biomarker of sarcoidosis. Increased levels in bronchoalveolar lavage fluid (BALF) were reported and associated with more severe forms of the disease. OBJECTIVES: The aim of the study was to evaluate the value of CTO in BALF as a routine biomarker of sarcoidosis. METHODS: The study included 85 patients in 9 control subjects in whom serum and BALF CTO were measured. RESULTS: Significantly higher CTO levels were detected in BALF of sarcoidosis patients than in control subjects (p < 0.001). There was good correlation between serum and BALF CTO levels in sarcoidosis patients (Spearman's Rho 0.481, p < 0.001). Serum but not BALF CTO had good correlation with clinical parameters. Only in a group of patients with BALF CTO above upper normal range there was association of BALF CTO with impaired FVC (p = 0.020) and chest radiograph score (0-2 vs. 3-4, p = 0.016). CONCLUSIONS: In comparison to serum CTO no additional benefit of determining CTO in BAL for routine sarcoidosis workup was shown.


Asunto(s)
Pruebas Enzimáticas Clínicas , Hexosaminidasas/análisis , Sarcoidosis Pulmonar/diagnóstico , Adulto , Anciano , Biomarcadores/análisis , Biomarcadores/sangre , Líquido del Lavado Bronquioalveolar/química , Estudios de Casos y Controles , Femenino , Hexosaminidasas/sangre , Humanos , Pulmón/fisiopatología , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Sarcoidosis Pulmonar/sangre , Sarcoidosis Pulmonar/fisiopatología , Índice de Severidad de la Enfermedad , Regulación hacia Arriba , Capacidad Vital , Adulto Joven
14.
Sarcoidosis Vasc Diffuse Lung Dis ; 32(3): 194-9, 2015 Sep 14.
Artículo en Inglés | MEDLINE | ID: mdl-26422563

RESUMEN

BACKGROUND: Chitotriosidase has been found to be useful as a sarcoidosis biomarker. In patients with better outcome lower values were observed. Some subjects have 24-base pair duplication in the chitotriosidase gene (CHIT1) that results in the production of inactive enzyme. This might influence the outcome of sarcoidosis and account for described observations. OBJECTIVES: The aim of this study was to correlate common CHIT1 duplication polymorphism and clinical outcome status in sarcoidosis (COS). METHODS: This retrospective study comprised 180 patients with sarcoidosis. COS at 3, 5 and 10 years was determined and correlated with CHIT1 24-base pair duplication polymorphism. CHIT1 genotyping was done by the PCR method. RESULTS: There was no significant correlation between CHIT1 24-base pair duplication polymorphism and COS at 3, 5 or 10 years but a subgroup analysis showed higher frequency of patients with Loefgren's syndrome (50% vs. 17.1%) and better COS in CHIT1 24-base pair duplication homozygotes vs. all other subjects in major COS groups (no, minimal and persistent disease) at 3 years (p=0.025) and borderline significant at 5 years (p = 0.090). CONCLUSIONS: In this study no correlation between CHIT1 24-base pair duplication polymorphism and COS was shown, but possible protective role of homozygous condition for CHIT1 24-base pair duplication polymorphism is suggested.


Asunto(s)
Duplicación de Gen , Hexosaminidasas/genética , Polimorfismo Genético , Sarcoidosis/genética , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Heterocigoto , Homocigoto , Humanos , Masculino , Persona de Mediana Edad , Fenotipo , Reacción en Cadena de la Polimerasa , Valor Predictivo de las Pruebas , Pronóstico , Factores Protectores , Estudios Retrospectivos , Factores de Riesgo , Sarcoidosis/diagnóstico , Sarcoidosis/enzimología , Sarcoidosis/terapia , Adulto Joven
15.
Int J Cardiol Heart Vasc ; 7: 40-48, 2015 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-28785643

RESUMEN

BACKGROUND: Chronic inflammation, the fundamental pathogenetic process of atherosclerosis, can be modified by pharmacological and non-pharmacological measures as a part of secondary prevention after acute myocardial infarction (AMI). The aim of our study was to determine the effect of diet, rich with natural antioxidants, added to physical activity (as a part of cardiac rehabilitation (CR) program) on inflammatory markers and ox-LDL, a marker of oxidative stress, closely involved in the process of chronic inflammation. METHODS: 41 male patients after AMI undergoing CR were divided into a diet group (supervised cardioprotective diet throughout the CR), and control group (CR without diet). We measured hsCRP, leucocytes, neutrophils, IL-6, oxLDL, exercise capacity and classic risk factors before and after CR program. RESULTS: Patients from the diet group presented with a significant decline in classic risk factors (BMI, waist circumference, waist to hip ratio, systolic blood pressure, heart rate, blood glucose, total cholesterol, LDL, TAG) and inflammatory markers (hsCRP, leucocytes, neutrophils) compared to control group. Furthermore, when studying nonsmokers, we observed significant decline of oxLDL in the diet group. CONCLUSIONS: The addition of cardioprotective diet, rich with natural antioxidants, to physical activity as a part of a CR program, positively modifies not just classic risk factors and exercise capacity, but also diminishes chronic inflammation markers. These effects, and oxLDL decline were most prominent in nonsmoking patients.

16.
Pulm Med ; 2014: 739673, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25548666

RESUMEN

INTRODUCTION: The aim of the study was to compare treatment of sarcoidosis with antifungal or corticosteroid medication. METHODS: In patients with sarcoidosis antifungal medication (n = 29), corticosteroids (n = 21) or a combination (n = 27) was given. Nine patients allotted to antifungal medication were later given corticosteroids because of the lack of regression of the disease. X-ray scores for the severity of granuloma infiltration were determined. Chitotriosidase and angiotensin converting enzyme were determined. The time in months till remission was observed as well as the number of recurrences.


Asunto(s)
Corticoesteroides/uso terapéutico , Antifúngicos/uso terapéutico , Sarcoidosis/tratamiento farmacológico , Corticoesteroides/administración & dosificación , Adulto , Antifúngicos/administración & dosificación , Quimioterapia Combinada , Femenino , Humanos , Masculino , Radiografía , Recurrencia , Sarcoidosis/diagnóstico por imagen , Resultado del Tratamiento
17.
Pulm Med ; 2014: 164565, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25180094

RESUMEN

BACKGROUND AND OBJECTIVES: Sarcoidosis is an inflammatory disease with increased levels of inflammatory cytokines. Previous studies have shown a relation between the degree of granuloma infiltration and serum cytokine levels, except for interleukin- (IL-) 10. The aim of the study was to further investigate the serum levels of IL-10 in patients with sarcoidosis and relate them to fungal exposure in terms of the amount of fungi in the air of their homes and ß-glucan in bronchoalveolar lavage (BAL) fluid. METHODS: Patients with sarcoidosis (n = 71) and healthy controls (n = 27) were enrolled. IL-10 was determined in serum. BAL was performed and the amount of ß-glucan was measured. Domestic exposure to fungi was determined by measuring airborne ß-N-acetylhexosaminidase (NAHA) in the bedrooms. RESULTS: At high levels of fungal exposure (domestic fungal exposure and ß-glucan in BAL), serum IL-10 values were lower than at low and intermediate exposure levels. CONCLUSION: The low serum IL-10 values at high fungal exposure suggest that fungal cell wall agents play a role in granuloma formation in sarcoidosis by inhibiting the secretion of the anti-inflammatory cytokine IL-10.


Asunto(s)
Exposición a Riesgos Ambientales/efectos adversos , Microbiología Ambiental , Hongos/inmunología , Interleucina-10/sangre , Sarcoidosis/sangre , Líquido del Lavado Bronquioalveolar/química , Estudios de Casos y Controles , Contaminantes Ambientales/análisis , Femenino , Humanos , Masculino , Persona de Mediana Edad , Sarcoidosis/inmunología , beta-Glucanos/análisis , beta-N-Acetilhexosaminidasas/análisis
18.
Respir Med ; 108(5): 775-82, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24594143

RESUMEN

INTRODUCTION: Chitotriosidase (CTO) is a human chitinolytic enzyme secreted by activated macrophages and polymorphonuclear neutrophils. Albeit not specific for sarcoidosis, it is increased in over 90% of patients with active disease. The aims of this study were to correlate CTO measurements with clinical assessment of sarcoidosis and to test CTO as a marker of sarcoidosis relapse. METHODS: 95 patients were followed-up for 24-60 months. Serial CTO measurements were performed every 3-6 months and correlated to clinical symptoms, lung function (FVC and DLco) and chest X-ray. In 38 patients clinical outcome status (COS) at 5 years was determined. RESULTS: Initial CTO levels were significantly higher in patients with impaired FVC/DLco (p = 0.011 for both) but there was no correlation with standard chest X-ray stages. Patients with Loefgren's syndrome had significantly lower initial and control CTO level compared to other patients (p = 0.011 and p = 0.001, respectively). At follow-up there was a positive correlation of CTO and deterioration of clinical symptoms (p < 0.001), chest X-ray (p < 0.001) and FVC/DLco (p = 0.012 and p = 0.086, respectively). Control CTO levels were significantly lower in no disease groups versus minimal or persistent disease group as defined by COS (p = 0.003 and p < 0.001, respectively). At relapse CTO increased for 100% or more from baseline value in 12/14 patients. CONCLUSIONS: It was shown that CTO correlates with certain sarcoidosis phenotypes (Loefgren's syndrome, COS) and that serial measurements of CTO correlate with clinical symptoms, chest radiographs and lung function.


Asunto(s)
Hexosaminidasas/sangre , Sarcoidosis/diagnóstico , Adulto , Anciano , Antifúngicos/uso terapéutico , Biomarcadores/sangre , Recolección de Muestras de Sangre/métodos , Pruebas Enzimáticas Clínicas/métodos , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Glucocorticoides/uso terapéutico , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , Capacidad de Difusión Pulmonar/fisiología , Radiografía , Recurrencia , Sarcoidosis/diagnóstico por imagen , Sarcoidosis/tratamiento farmacológico , Sarcoidosis/fisiopatología , Capacidad Vital/fisiología , Adulto Joven
19.
Respirology ; 19(2): 225-230, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24372709

RESUMEN

BACKGROUND AND OBJECTIVE: Previous studies have demonstrated increases of inflammatory mediators in sarcoidosis while epidemiological studies have also demonstrated an association with increased fungi exposure. This study measured the level of ß-glucan in the lungs and of inflammatory mediators in serum, and correlated both with the extent of pulmonary granuloma infiltration. METHODS: This is a cross-sectional study of 98 patients with sarcoidosis and 26 controls. ß-glucan, a cell wall constituent of fungi, was measured in bronchoalveolar lavage. Inflammatory mediator levels were determined in serum. The extent of granuloma infiltration was estimated on the chest X-ray. Exposure to fungi at home was determined by taking air samples in bedrooms and analysing for the presence of ß-N-acetylhexosaminidase. RESULTS: Significantly, higher levels of ß-glucan were found in broncho-alveolar lavage in subjects with sarcoidosis as compared with controls. There were significant positive relationships between the extent of granuloma infiltration and the levels of the different inflammatory mediators, except for interleukin-10. Domestic fungal exposure was higher among subjects with sarcoidosis. CONCLUSIONS: This is the first time that a specific agent, previously suspected to be related to the risk of sarcoidosis, has been detected in the lung of subjects with sarcoidosis and related to the levels of inflammatory mediators and the degree of home exposure to fungi. The results suggest that exposure to fungi should be explored when investigating patients with sarcoidosis.


Asunto(s)
Biomarcadores/metabolismo , Líquido del Lavado Bronquioalveolar/química , Granuloma/metabolismo , Inflamación/metabolismo , Sarcoidosis Pulmonar/metabolismo , Adulto , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico
20.
Metab Syndr Relat Disord ; 12(2): 149-55, 2014 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24266733

RESUMEN

BACKGROUND: High-sensitivity C-reactive protein (hsCRP) is an important biomarker of risk for coronary heart disease morbidity and mortality. We investigated the influence of short-term cardiac rehabilitation (CR) after acute myocardial infarction (AMI) on values of hsCRP and classical risk factors, including metabolic syndrome. METHODS: hsCRP and classical risk factors were measured before and after completed 2-week CR program in 30 men after AMI. The comparison group comprised 30 age-balanced healthy men, with no risk factors for coronary heart disease. RESULTS: As expected, in comparison to healthy individuals, patients had higher values of hsCRP; furthermore, smokers had significantly higher hsCRP values than nonsmokers. Patients had more expressed markers of metabolic syndrome and due to pharmacological therapy lower blood pressure, total cholesterol and low-density lipoprotein cholesterol (LDL-C). After CR was completed, a significant drop in hsCRP (P=0.006) and improvement of metabolic syndrome parameters (lower body mass index, blood pressure, LDL-C, triglycerides) was observed in nonsmokers, whereas no such changes occurred in smokers. CONCLUSIONS: Our study revealed that hsCRP and metabolic syndrome parameters can be substantially reduced by a 2-week CR program; however, this effect is present only in nonsmokers. Thus, all patients entering the CR program after AMI should be advised to quit smoking before entering the program to achieve optimal benefits.


Asunto(s)
Proteína C-Reactiva/metabolismo , Infarto del Miocardio/sangre , Infarto del Miocardio/rehabilitación , Adulto , Anciano , Terapia Combinada , Humanos , Masculino , Persona de Mediana Edad , Infarto del Miocardio/cirugía , Modalidades de Fisioterapia , Cuidados Posoperatorios , Fumar/sangre , Factores de Tiempo , Resultado del Tratamiento
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