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BACKGROUND: Progressive pulmonary fibrosis is the symptomatic, physiological, and radiological progression of interstitial lung diseases. The aim of this study was to examine the relationship between progressive pulmonary fibrosis and demographic characteristics and to evaluate the effect on clinical outcomes and mortality. METHODS: This cross-sectional study included 221 patients diagnosed with non-idiopathic pulmonary fibrosis interstitial lung diseases who were followed in the last 5 years. Patient symptoms, clinical, radiological, and demographic data were examined. Risk factors for the development of progressive pulmonary fibrosis and the relationship with clinical outcomes and mortality were examined. RESULTS: Of the patients, 33.0% (n = 73) had fibrotic idiopathic nonspecific interstitial pneumonia (iNSIP), 35.7% (n = 79) had fibrotic hypersensitivity pneumonia (HP), 18.1% (n = 40) had fibrotic connective tissue disease (CTD) interstitial lung diseases (ILD), and 13.1% (n = 29) had postinfectious fibrotic ILD. The progressive pulmonary fibrosis development rates of the subtypes were 46.5% iNSIP (n = 34), 86.0% fibrotic HP (n = 68), 42.5% fibrotic CTD-ILD (n = 17), and 20.7% postinfectious ILD (n = 6). The presence of progressive pulmonary fibrosis was associated with the development of respiratory failure and mortality (odds ratio [OR]: 2.70, 95% CI: 1.04-7.05 and OR: 2.13, 95% CI: 1.23-3.69). Progressive pulmonary fibrosis development was higher in hypersensitivity pneumonia patients with farmer's lung (OR: 5.06, 95% CI: 1.02-25.18). CONCLUSION: Progressive pulmonary fibrosis was more prevalent in older patients. Farming was an important risk factor in the development of hypersensitivity pneumonia-progressive pulmonary fibrosis. Respiratory failure and mortality were higher in those who developed progressive pulmonary fibrosis.
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Progresión de la Enfermedad , Enfermedades Pulmonares Intersticiales , Fibrosis Pulmonar , Humanos , Estudios Transversales , Masculino , Femenino , Persona de Mediana Edad , Anciano , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/epidemiología , Fibrosis Pulmonar/epidemiología , Fibrosis Pulmonar/complicaciones , Fibrosis Pulmonar/mortalidad , Factores de Riesgo , Alveolitis Alérgica Extrínseca/complicaciones , Alveolitis Alérgica Extrínseca/patología , Alveolitis Alérgica Extrínseca/epidemiología , Fibrosis Pulmonar Idiopática/complicaciones , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/epidemiología , Fibrosis Pulmonar Idiopática/patología , Adulto , Enfermedades del Tejido Conjuntivo/complicacionesRESUMEN
INTRODUCTION: Coronavirus disease 2019 (COVID-19) has a 1-2% fatality rate, where no specific treatment has yet been defined. Although corticosteroids are recommended for selected COVID-19 patients without acute respiratory distress syndrome (ARDS) and septic shock, there is no consensus regarding patient subgroups, dose, and duration. In this study, it was aimed to examine the contribution of corticosteroid treatment to the management of COVID-19 pneumonia without ARDS, septic shock both in acute and recovery setting. MATERIALS AND METHODS: The study population was divided into two as those who used corticosteroids during the recovery phase (who did not develop sufficient radiological or clinical improvement) and those who did so during the activation phase (non-ARDS/septic shock condition, clinical, laboratory or radiological progression). RESULT: We identified 47 patients, 26 of which were males, and mean age was 60.5 ± 16.5 years. Seventeen patients were found to receive corticosteroids during the recovery phase and the rest (n= 30) during the activation period. After corticosteroid therapy, we found reduction of increased pre-treatment levels of D-dimer, ferritin, fibrinogen, CRP, increment of decreased pre-treatment lymphocyte count and saturation. Complete symptomatic improvement was detected in 6.9% and 17.6% of the patients in the activation phase and recovery phase, respectively. Complete radiological improvement was found in 11.5% and 35.3% of the patients in the activation phase and recovery phase, respectively. While corticosteroid treatment was initiated on day 4.2 ± 2.6 and continued for a mean of 5.9 ± 2.8 days in the activation group, it was started on day 8.1 ± 11.3 and administered for 7.8 ± 3.8 days in the recovery group. In both groups, methylprednisolone was given at a median dose of 40 mg/day. CONCLUSIONS: Short-term low-dose corticosteroid therapy may improve clinical, radiological, laboratory outcomes in the management of COVID-19 pneumonia during the activation period without ARDS and non-septic shock and during recovery period with no satisfactory response. Further randomized controlled studies will be useful in demonstrating its efficacy.
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Corticoesteroides/administración & dosificación , Tratamiento Farmacológico de COVID-19 , Pandemias , SARS-CoV-2 , COVID-19/diagnóstico , COVID-19/epidemiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Turquía/epidemiologíaRESUMEN
In this review, we aimed to determine the etiopathogenesis, diagnosis and treatment of non-cystic fibrosis bronchiectasis in the light of scientific studies conducted in our country. For this purpose, Pubmed, Web of Science ve Ulakbim TR Dizin were searched and the publications available as of July 2019 were evaluated and the findings of these studies were summarized and presented.
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Bronquiectasia/epidemiología , Bases de Datos Factuales , Bronquiectasia/diagnóstico , Enfermedad Crónica , Humanos , Turquía/epidemiologíaRESUMEN
INTRODUCTION: Intensive care physicians are increasingly involved in decision making about the prognosis of intensive care unit ICU patients. With this study; we aimed to evaluate the power of clinician foresight at prediction of mortality in patient at triage to intensive care and patient follow-up. MATERIALS AND METHODS: This study was conducted in ICUs located in various geographical regions of Turkey between January 1, 2017-April 30, 2017.The clinical research was planned as observational, multicenter, cross-sectional. RESULT: A total of 1169 intubated patients were followed in 37 different ICU. At the beginning of the follow-up we asked the physician who will follow the patient in the ICU to give a score for the probability of survival of the patients. Scoring included a total of 6 scores from 0 to 5, with the "0" the worst probability "5" being the best. According to this distribution, only 1 (0.9%) of 113 patients who were given 0 points survived. Three (6.1%) of 49 with the best score of 5 died. Survival rates were significantly different in each score group (r: -0.488; p<0.001). After the combined mortality estimation scores based on the clinical observations of the physicians (0 and 1 point score was combined as non-survive, 4 and 5 score was combined as survived) 320 of the 545 patients were estimated to be dead and 225 were predicted survival. Sensitivity and spesifity of scoring system to predict mortality was 91.56% (95% CI: 87.96-94.37), 76.89% (95% CI: 70.82-82.23) respectively. CONCLUSIONS: In this study, we concluded that the physicians who follow the patients in the ICU can predict the poor prognosis at the time of admission and the high mortality rate. The physician's opinion on mortality estimation should be considered in intensive care mortality scoring in addition to other laboratory and clinical parameters.
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Enfermedad Crítica/mortalidad , Mortalidad Hospitalaria/tendencias , Unidades de Cuidados Intensivos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Índice de Severidad de la Enfermedad , Adulto , Anciano , Cuidados Críticos/estadística & datos numéricos , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Prospectivos , TurquíaRESUMEN
INTRODUCTION: Influenza subtypes vary by clinical, radiological, and prognostic courses and may go along with viral pneumonia. We aimed to identify clinical, radiological, and prognostic aspects of influenza epidemic during years 2016-2017. MATERIALS AND METHODS: Influenza cases reported to the Public Health Directorate in our city was assessed retrospectively. Clinical, radiological, and prognostic parameters were compared based on influenza subtypes. RESULT: We analyzed samples from 197 cases with suspected influenza. Mean age of the subjects was 51.17 ± 26.74. We found influenza A/H1N1, influenza A/H3N2, and influenza B in 59 (30.0%), 29 (14.7%), and 3 (1.5%) cases, respectively. Comorbidity was present in 48 (24.4%) cases. Most common radiological finding was interstitial pattern. Seventy-one and 79 per cent of H1N1 and H3N2 cases were influenza pneumonia, respectively. The prevalence of overall mortality was 5.5% with a predominance in H1N1 over H3N2. Influenza vaccination had been performed in 6.8% and 3.4% of H1N1 and H3N2 cases, respectively. We detected no mortality in any vaccinated patient. We identified 6 pregnant women, 2 of which ended up with preterm birth, and another one with abortion. CONCLUSIONS: Often manifested as lower respiratory tract infection, influenza may cause epidemics with increased mortality rate. Influenza should be suspected when interstitial pattern was seen on radiological images. H1N1 cases course worse. Since the prognosis is better in vaccinated patients, seasonal influenza vaccination among the community needs to be elevated. In addition, protective measures like vaccination should be taken in pregnancy to avoid preterm delivery or abortion.
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Subtipo H1N1 del Virus de la Influenza A/inmunología , Subtipo H3N2 del Virus de la Influenza A/inmunología , Vacunas contra la Influenza/uso terapéutico , Gripe Humana/diagnóstico , Vacunación/métodos , Femenino , Humanos , Incidencia , Gripe Humana/epidemiología , Gripe Humana/prevención & control , Masculino , Persona de Mediana Edad , Pronóstico , Tasa de Supervivencia/tendencias , Turquía/epidemiologíaRESUMEN
Depression and mood disorders occur commonly following emergent cardiac surgery. Selective serotonin reuptake inhibitors are commonly used antidepressants. We report the development of severe hyponatremia leading to adverse clinical effects due to escitalopram and thiazide diuretic use concomitantly in a patient with depression after emergency coronary artery bypass grafting.
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Antidepresivos/efectos adversos , Citalopram/efectos adversos , Puente de Arteria Coronaria , Depresión/tratamiento farmacológico , Hiponatremia/inducido químicamente , Trastornos del Humor/tratamiento farmacológico , Complicaciones Posoperatorias/tratamiento farmacológico , Inhibidores Selectivos de la Recaptación de Serotonina/efectos adversos , Inhibidores de los Simportadores del Cloruro de Sodio/efectos adversos , Antidepresivos/uso terapéutico , Citalopram/uso terapéutico , Quimioterapia Combinada , Urgencias Médicas , Humanos , Masculino , Persona de Mediana Edad , Inhibidores Selectivos de la Recaptación de Serotonina/uso terapéutico , Índice de Severidad de la Enfermedad , Inhibidores de los Simportadores del Cloruro de Sodio/uso terapéuticoRESUMEN
INTRODUCTION: Non-invasive mechanical ventilation provides early improvement in most of the patients with acute hypercapnic respiratory failure. The aim of our study was to determine the risk factors for late failure of non-invasive mechanical ventilation in patients with acute hypercapnic respiratory failure. MATERIALS AND METHODS: Ninety three patients were prospectively evaluated. Non-invasive mechanical ventilation was accepted to be successful if the patient was discharged from the hospital without the need for intubation (group 1) and to be late failure if a deterioration occurred after an initial improvement of blood gases tension and general conditions (group 2). RESULTS: Non-invasive mechanical ventilation was successful in 62 (66.7%) patients. In 25 (26.9%) patients a late failure was observed. There was no difference between groups 1 and 2 in terms of pretreatment pH, PaCO2 and PaO2/FiO2. However, serum C-reactive protein level, Acute Physiology and Chronic Health Evaluation II (APACHE II) score and frequency of bronchiectasis and pneumonia were significantly higher and serum albumin level, Glasgow Coma Score, cough strength and compliance to non-invasive mechanical ventilation were significantly lower in group 2. CONCLUSION: The pretreatment high APACHE II Score and C-reactive protein level, low Glasgow Coma Score, albumin level, cough strength, bad compliance to non-invasive mechanical ventilation, the presence of bronchiectasis and pneumonia and absence of significance improvement in PaO2/FiO2 after treatment were determined as risk factors for non-invasive mechanical ventilation late failure.
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Hipercapnia/terapia , Complicaciones Posoperatorias/epidemiología , Respiración Artificial/efectos adversos , Insuficiencia Respiratoria/terapia , APACHE , Anciano , Análisis de los Gases de la Sangre , Femenino , Hospitales Universitarios , Humanos , Masculino , Complicaciones Posoperatorias/etiología , Estudios Prospectivos , Factores de Riesgo , Resultado del Tratamiento , Turquía/epidemiologíaRESUMEN
INTRODUCTION AND STUDY OBJECTIVE: In Turkey, a total of 269 earthquakes took place from 1900 through 2023. The most devastating earthquakes in terms of casualties and extensive destruction occurred at 4:17am and 1:24pm local time on February 6, 2023 with the epicenters located in Pazarcik (Kahramanmaras) and Ekinozu (Kahramanmaras) and magnitudes of 7.7Mw and 7.6Mw, respectively. The aim of this study was to define the frequency of lung complications that occurred directly and/or developed during the intensive care follow-up of individuals affected by the Kahramanmaras earthquakes. METHOD: A retrospective evaluation was conducted on the files of 69 patients who were rescued from the debris of collapsed buildings after the Kahramanmaras earthquakes and followed up in the intensive care unit in terms of the time under the debris, demographic data, vital signs, and lung complications that were present at the time of admission and developed during follow-up. SPSS for Windows v. 20.0 was used for data analysis. RESULTS: The study included a total of 69 patients, of whom 29 (42%) were female and 40 (58%) were male. The mean age was 39.9 (SD = 16.9) years. The mean time under the debris was 53.9 (SD = 52) hours, and the mean time from rescue to the intensive care unit admission was 18.7 (SD = 12.8) hours. One or more pulmonary complications were detected in 52.2% (n = 36) of the patients at the time of admission. During the follow-up, 30.4% (n = 21) of the patients developed pulmonary congestion, 13.0% (n = 9) pneumonia, 1.5% (n = 1) alveolar hemorrhage, and 1.5% (n = 1) atelectasis, while no additional lung complications developed in the remaining 37 patients (53.6%). CONCLUSION: Severe cases of individuals recovered from the debris can have a high prevalence of earthquake-related lung disorders and chest trauma, which may be associated with high mortality. The timely identification and effective intervention of pulmonary complications that may develop during follow-up can reduce mortality.
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Terremotos , Unidades de Cuidados Intensivos , Humanos , Masculino , Turquía/epidemiología , Femenino , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Adulto Joven , Adolescente , Anciano , Enfermedades Pulmonares/epidemiologíaRESUMEN
Background: This study aimed to provide nuanced insights in managing patients with a history of coronavirus disease 2019 (COVID-19) pneumonia undergoing coronary artery bypass grafting. Methods: This retrospective cohort study involved 168 patients (131 males, 37 females; mean age: 61.2±9.7 years; range, 51 to 72 years) undergoing isolated coronary artery bypass grafting surgery between December 2021 and December 2023. The study examined factors such as age, sex, comorbidities, blood test results, vaccination status, operative parameters, and postoperative complications. Patients' health records were reviewed to confirm the presence of previous COVID-19 pneumonia and vaccination status. Patients were divided into two groups based on their history of COVID-19 pneumonia: Group 1 included 140 who had not been diagnosed with COVID-19 pneumonia, and Group 2 included 28 patients who had a documented history of COVID-19 pneumonia. Postoperative pulmonary complications, including atelectasis, pleural effusion, acute respiratory distress syndrome, and pneumonia, were noted. Results: Patients with a history of COVID-19 pneumonia (Group 2, n=28) demonstrated significantly higher seropositivity for COVID-19 (89.3% vs. 29.3%, p=0.001) compared to those without a history (Group 1, n=140). Although pulmonary complications were higher in Group 2 (17.9% vs. 3.6%, p=0.013), postoperative mortality rates did not differ significantly between the groups. Pleural effusion was markedly higher in Group 2 (14.3% vs. 2.1%, p=0.015). Vaccination did not significantly affect perioperative and postoperative outcomes, except for a minor difference in postoperative drainage volume. Conclusion: This study highlights the impact of prior COVID-19 pneumonia on postoperative outcomes in coronary artery bypass grafting patients. Although there was a rise in pulmonary complications, the mortality rates stayed similar among individuals with and without a prior history of COVID-19 pneumonia. Vaccination did not significantly influence outcomes, emphasizing the need for further research with larger cohorts to validate and expand upon these findings.
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Pulmonology is one of the branches that frequently receive consultation requests from the emergency department. Pulmonology consultation (PC) is requested from almost all clinical branches due to the diagnosis and treatment of any respiratory condition, preoperative evaluation, or postoperative pulmonary problems. The aim of our study was to describe the profile of the pulmonology consultations received from emergency departments in Turkiye. A total of 32 centers from Turkiye (the PuPCEST Study Group) were included to the study. The demographic, clinical, laboratory and radiological data of the consulted cases were examined. The final result of the consultation and the justification of the consultation by the consulting pulmonologist were recorded. We identified 1712 patients, 64% of which applied to the emergency department by themselves and 41.4% were women. Eighty-five percent of the patients had a previously diagnosed disease. Dyspnea was the reason for consultation in 34.7% of the cases. The leading radiological finding was consolidation (13%). Exacerbation of preexisting lung disease was present in 39% of patients. The most commonly established diagnoses by pulmonologists were chronic obstructive pulmonary disease (19%) and pneumonia (12%). While 35% of the patients were discharged, 35% were interned into the chest diseases ward. The majority of patients were hospitalized and treated conservatively. It may be suggested that most of the applications would be evaluated in the pulmonology outpatient clinic which may result in a decrease in emergency department visits/consultations. Thus, improvements in the reorganization of the pulmonology outpatient clinics and follow-up visits may positively contribute emergency admission rates.
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Servicios Médicos de Urgencia , Enfermedades Pulmonares , Médicos , Humanos , Femenino , Masculino , Estudios Transversales , Turquía , Pulmón , Servicio de Urgencia en Hospital , Enfermedades Pulmonares/diagnóstico , Enfermedades Pulmonares/terapia , Derivación y ConsultaRESUMEN
Introduction: The aim of this study was to assess the potential of subcutaneous adipose tissue volume, mediastinal adipose tissue volume, lung density, and lung volume (as measured on high-resolution computed tomography) to predict disease progression in patients with idiopathic pulmonary fibrosis (IPF). Additionally, the study aimed to evaluate the changes in these semiquantitative measures over time. Materials and Methods: The HRCT images of 57 patients diagnosed with IPF were retrospectively screened. Subcutaneous adipose tissue volume, mediastinal adipose tissue volume, and mean lung density and volume were measured at the time of diagnosis and at the 12th month. The ability of these parameters to predict progression was evaluated using the univariate and multivariate Cox regression analyses. Results: Low mediastinal adipose tissue volume at diagnosis had a 0.991-fold effect [odds ratio (OR)= 0.991, 95% confidence interval (CI)= 0.984-0.997, p< 0.001] on progression. Low mediastinal adipose tissue volume at diagnosis had a 0.993-fold effect [odds ratio (OR)= 0.993, 95% confidence interval (CI)= 0.975-1.011, p< 0.001] and progression development at the 12th month had a 6.5-fold effect [odds ratio (OR)= 6.516, 95% confidence interval (CI)= 1.594-26.639, p< 0.009] on mortality. Conclusion: This study indicate that the prognosis was better in those with a large mediastinal adipose tissue volume among the patients with IPF.
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Fibrosis Pulmonar Idiopática , Humanos , Estudios Retrospectivos , Pronóstico , Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Tejido Adiposo/diagnóstico por imagen , Tomografía Computarizada por Rayos X , PulmónRESUMEN
OBJECTIVE: This study aimed to evaluate attitude and practice toward use of regular tobacco cigarettes and electronic cigarettes among pregnant women. MATERIAL AND METHODS: A total of 1123 pregnant women participated on a voluntary basis in this questionnaire survey. Maternal characteristics, cigarette consumption parameters, and personal opinions regarding the adverse effects of smoking during pregnancy were evaluated. RESULTS: Active smokers composed 12.4% (9.4%: regular tobacco cigarettes, 3.0%: electronic cigarettes) of the study population. Smoking during the current pregnancy, particularly via regular tobacco cigarettes, was more likely for women with smoking during previous pregnancies (56.0% vs. 7.8%, P < .001), previous history of low birth weight infant delivery (16.1% vs. 8.6%, P = .013), premature delivery (16.7% vs. 7.0%, P < .001), and stillbirth (22.8% vs. 11.7%, P = .002). The presence versus absence of smoking during pregnancy was associated with a lower likelihood of being a housewife (70.5% vs. 80.5%, P = .010) and a higher likelihood of having an actively smoking mother (25.9% vs. 11.2%, P < .001) or partner (65.7% vs. 46.9%, P < .001). Regular tobacco cigarette users considered electronic cigarettes to have a higher risk of adverse impacts (11.1% vs. 2.9%, P = .012), while electronic cigarette users considered regular cigarettes to have a higher risk of nicotine exposure (55.9% vs. 13.0%, P < .001). CONCLUSION: Our findings indicate being employed, having an actively smoking mother or partner, as well as smoking in previous pregnancies, to be the risk factors for increased likelihood of smoking during pregnancy.
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Background Standard treatment for deep venous thromboembolism involves parenteral anticoagulation overlapping with a vitamin K antagonist, an approach that is effective but associated with limitations including the need for frequent coagulation monitoring. The direct oral anticoagulant rivaroxaban is similarly effective to standard therapy as a single-drug treatment for venous thromboembolism and does not require routine coagulation monitoring. The aim of this analysis was to project the long-term costs and outcomes for rivaroxaban compared to standard of care (tinzaparin/warfarin). Methods A total of 184 patients who were under anticoagulant therapy with warfarin or rivaroxaban for extended deep venous thromboembolism were retrospectively evaluated; 59 received rivaroxaban and 125 received warfarin therapy. Assessments were made on age, gender, place of residence, the duration of anticoagulation, mean international normalized ratio value, the effective rate of international normalized ratio (time in the therapeutic range), bleeding-related complication rate, duration of hospitalization due to complications, the number of annual outpatient department admission, cost for drug, cost for hospitalization, cost for outpatient department admission and international normalized ratio measurements. Results The annual outpatient cost is higher in warfarin group (147.09 ± 78 vs. 62.32 ± 19.79 USD p < 0.001). But annual drug cost is higher in rivaroxaban group (362.6 vs. 71.55 ± 31.01 USD p < 0.001). Overall cost of rivaroxaban group is higher than warfarin group (476.25 ± 36.78 vs. 364.82 ± 174.44 USD). Warfarin is not cost-effective when non-drug costs (342.5 ± 174.44 vs. 113.65 ± 36.77) and hospital costs (173.85 ± 122.73 vs. 64.9 ± 23.55 USD) were analyzed. Conclusion This analysis suggests that rivaroxaban has lower costs than warfarin in terms of outpatient department admission and hospital costs due to complications; however, warfarin was more economic when all cost parameters were considered. Time in the therapeutic range was found as 56% for warfarin that should be taken into account while analyzing costs and benefits.
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Anticoagulantes/economía , Anticoagulantes/uso terapéutico , Inhibidores del Factor Xa/economía , Inhibidores del Factor Xa/uso terapéutico , Costos de la Atención en Salud , Rivaroxabán/economía , Rivaroxabán/uso terapéutico , Tromboembolia/tratamiento farmacológico , Tromboembolia/economía , Trombosis de la Vena/tratamiento farmacológico , Trombosis de la Vena/economía , Warfarina/economía , Warfarina/uso terapéutico , Adulto , Anciano , Atención Ambulatoria/economía , Anticoagulantes/efectos adversos , Coagulación Sanguínea/efectos de los fármacos , Ahorro de Costo , Análisis Costo-Beneficio , Costos de los Medicamentos , Monitoreo de Drogas/economía , Monitoreo de Drogas/métodos , Inhibidores del Factor Xa/efectos adversos , Femenino , Hemorragia/inducido químicamente , Hemorragia/economía , Costos de Hospital , Humanos , Relación Normalizada Internacional/economía , Masculino , Persona de Mediana Edad , Modelos Económicos , Estudios Retrospectivos , Factores de Riesgo , Rivaroxabán/efectos adversos , Tromboembolia/sangre , Tromboembolia/diagnóstico , Factores de Tiempo , Resultado del Tratamiento , Trombosis de la Vena/sangre , Trombosis de la Vena/diagnóstico , Warfarina/efectos adversosRESUMEN
BACKGROUND/AIM: Alpha-1 antitrypsin deficiency may be a potential predisposing factor for interstitial lung fibrosis. We investigated alpha-1 antitrypsin levels and its polymorphisms in patients with interstitial lung disease. MATERIALS AND METHODS: A total of 103 interstitial lung disease patients were compared. RESULTS: The mean alpha-1 antitrypsin level in idiopathic interstitial pneumonia patients was 1.67 ± 0.33 g/L, and it was 1.54 ± 0.37 g/L in patients with nonidiopathic interstitial pneumonia (P = 0.13). Low alpha-1 antitrypsin levels were more frequently observed in nonidiopathic interstitial pneumonia patients compared with idiopathic interstitial pneumonia, but the difference was not statistically significant (8.9% vs. 0%, respectively, P = 0.4). In 100 patients, the normal PiMM genotype was detected, while abnormal ones (PiMZ, n = 2, 1.9%; PiMS, n = 1, 0.97%) were determined in three cases. When the frequency of alpha-1 antitrypsin polymorphism in interstitial lung disease patients was compared with the data of the healthy population, no significant difference was detected for the PiMZ and PiMS variants (P = 0.15 and P = 0.44, respectively). CONCLUSION: Lower levels of serum alpha-1 antitrypsin were more frequent in nonidiopathic interstitial pneumonia patients than idiopathic interstitial pneumonia without an increase in genetic polymorphism. The difference was not statistically significant.
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Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/genética , Polimorfismo Genético , Deficiencia de alfa 1-Antitripsina/diagnóstico , Deficiencia de alfa 1-Antitripsina/genética , alfa 1-Antitripsina/sangre , alfa 1-Antitripsina/genética , Adulto , Anciano , Femenino , Predisposición Genética a la Enfermedad , Humanos , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/fisiopatología , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Medición de Riesgo , Factores de Riesgo , Turquía/epidemiología , Deficiencia de alfa 1-Antitripsina/epidemiología , Deficiencia de alfa 1-Antitripsina/fisiopatologíaRESUMEN
A familial aggregation of Behçet's disease (BD) has long been noted. These studies have supported the direct role of HLA-B5 in the pathogenesis of BD. Despite the fact that familial clustering is characterized by genetic anticipation, accounting for the earlier disease onset in successive generations, we present two brothers and two cousins from the same family who were diagnosed when they were over twenty years old.We report these young adult patients to introduce the characteristics of familial aggregation of BD. In this article HLA-B*51 and Cw*16 positivities with adult onset were demonstrated.