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1.
Eur Respir J ; 2020 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-32139456

RESUMO

INTRODUCTION: Sarcoidosis associated pulmonary hypertension (SAPH) is associated with reduced survival in single center studies. An international registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients. METHODS: ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrollment. Information analyzed includes right heart catheterization data, pulmonary function testing, chest x-ray Scadding stage, six minute walk distance (6MWD) among others. Cox regression models were used to identify independent predictors of transplant-free survival. RESULTS: Data from a total of 215 patients followed for a mean of 2.5±1.9 years were available for analysis. In the 159 pre-capillary patients, the KM adjusted 1, 3 and 5 year transplant free survival was 89.2%, 71.7% and 62.0%, respectively. In the incident and prevalent groups, KM adjusted 1, 3 and 5 year transplant free survival was 83.5%, 70.3% and 58.3% and 94.7%, 72.2%, and 66.3% respectively. Patients with reduced DLCO (<35% predicted) and 6MWD <300 m in the pre-capillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved FEV1/FVC ratio were identified as independent risk factors for reduced transplant-free survival in the pre-capillary cohort. CONCLUSION: Reduced diffusion capacity (<35% of predicted) and 6MWD <300 m at the time of registry enrollment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was also identified as an independent risk factor for worsened outcomes.

2.
Transpl Infect Dis ; 21(6): e13166, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31487755

RESUMO

BACKGROUND: Cytomegalovirus (CMV) infection is common in thoracic organ transplant recipients. Valganciclovir and ganciclovir are used for both prophylaxis and treatment of this infection, but intolerance and treatment failure are common. Letermovir has been demonstrated to reduce the risk of CMV infection when used for prophylaxis in allogeneic hematopoietic cell transplantation. However, there are no data on its efficacy in thoracic organ transplantation. METHODS: We examined the use of letermovir for either CMV prophylaxis (primary and secondary) or treatment in heart and lung transplant recipients at our institution from February 1, 2018, through December 31, 2018. RESULTS: Nine total patients received letermovir at our institution (8 lung transplant, 1 heart transplant) during the study period. Letermovir was prescribed for CMV prophylaxis in eight patients (primary prophylaxis in two patients and secondary prophylaxis in 6 patients), and for treatment of CMV DNAemia in two cases. One patient received letermovir for both secondary prophylaxis and treatment on separate occasions. Three out of 8 (37.5%) patients receiving letermovir for prophylaxis developed CMV DNAemia during prophylaxis. One patient treated for CMV disease had clinical failure with a sharp rise in serum CMV DNA PCR. The other patient treated for low-grade CMV DNAemia initially had a slight rise in CMV DNA PCR, but has since had a sustained response. No major side effects were experienced, and 2 patients reported minor side effects. CONCLUSION: Letermovir was well tolerated with only minor side effects reported; however, the rate of development of CMV DNAemia on prophylaxis was considerable. Further study of the dosing and efficacy of letermovir for CMV prophylaxis or treatment in thoracic organ transplant recipients is warranted.

4.
Respir Med ; 139: 72-78, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29858005

RESUMO

BACKGROUND: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients. METHODS: Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), chest x-ray, and 6-min walk distance (6MWD). RESULTS: A total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DLCO percent predicted (% pred) andmPAP (Rho = -0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = -0.362, p < 0.0001). PVR was significantly higher in stage 4 disease than in stage 0 or 1 disease (p < 0.05 for both comparisons). About two-thirds of the SAPH patients came from the United States (US). There was a significant difference in the rate of treatment between US (67.5%) versus non-US (86%) (Chi Square 11.26, p = 0.0008) sites. CONCLUSIONS: The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers.


Assuntos
Hipertensão Pulmonar/fisiopatologia , Sarcoidose Pulmonar/complicações , Sarcoidose Pulmonar/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Cateterismo Cardíaco , Europa (Continente) , Feminino , Hemodinâmica , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Oriente Médio , Sistema de Registros , Sarcoidose Pulmonar/diagnóstico por imagem , Sarcoidose Pulmonar/etiologia , Estados Unidos , Capacidade Vital , Teste de Caminhada , Raios X
6.
Chest ; 153(3): e53-e56, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29519311

RESUMO

CASE PRESENTATION: A 24-year-old woman with ΔF508/Y1092X cystic fibrosis (CF) complicated by severe obstructive lung disease (FEV1 of 30% predicted) was admitted for IV antibiotics for planned sinus surgery resulting from severe chronic sinusitis causing frequent exacerbations and declining lung function. She had persistent airway infection with multidrug-resistant Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and growth of a fungus presumed to be an airway colonizer, identified as Stephanoascus ciferrii 1 year before presentation. Two days after surgery, she developed acute respiratory failure requiring mechanical ventilation. On day 4 of mechanical ventilation, venovenous-extracorporeal membrane oxygenation (VV-ECMO) was initiated for refractory respiratory failure. The following day, she was listed for bilateral lung transplant and was transplanted 4 days later. Following transplantation, she was decannulated from ECMO; however, over the next 12 hours, oxygenation deteriorated requiring reinstitution of VV-ECMO for presumed severe primary graft dysfunction. Despite treatment with broad spectrum antimicrobial coverage with piperacillin/tazobactam, ciprofloxacin, linezolid, micafungin, voriconazole, and ganciclovir, she failed to improve and developed complex bilateral pleural effusions.


Assuntos
Antifúngicos/uso terapêutico , Criptococose/tratamento farmacológico , Criptococose/microbiologia , Cryptococcus/isolamento & purificação , Fibrose Cística/cirurgia , Transplante de Pulmão/efeitos adversos , Síndrome do Desconforto Respiratório do Adulto/tratamento farmacológico , Síndrome do Desconforto Respiratório do Adulto/microbiologia , Fibrose Cística/complicações , Oxigenação por Membrana Extracorpórea , Feminino , Humanos , Hospedeiro Imunocomprometido , Respiração Artificial , Cirurgia Torácica Vídeoassistida , Adulto Jovem
10.
Chest ; 152(5): 980-989, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-28527879

RESUMO

BACKGROUND: Pulmonary hypertension (PH) is diagnosed and classified through right-sided heart catheterization, with a number of hemodynamic markers used to help guide treatment decisions. These markers may not reflect the complex remodeling of the right ventricle or the interplay between ventricles and struggle to predict treatment response. This study investigates the use of a novel marker: the ratio of left ventricular end-diastolic pressure to right ventricular end-diastolic pressure (LVEDP/RVEDP) in predicting treatment outcomes in a cohort of patients with PH. METHODS: We performed a retrospective analysis of patients with PH at INOVA Fairfax Hospital's advanced lung disease program with simultaneous left-sided and right-sided heart catheterization. The primary end point was the time to clinical improvement, defined by an improvement in distance walked on the 6-min walk test (6MWT) of > 35 m in a year without interceding hospitalization for worsening PH or the need for additional PH therapy. RESULTS: A total of 51 patients were included in the final analysis, 21 of whom (41.2%) had a salutary treatment effect with a mean improvement in the 6MWT of 75 m. Treatment responders were more likely to have a lower LVEDP/RVEDP ratio (1.08 vs 1.62; P = .051). This association persisted in the final multivariate regression model after adjustment for age and sex and controlling for severity of PH (OR, 0.17; 95% CI, 0.03-0.65; P = .024). CONCLUSIONS: The LVEDP/RVEDP ratio is a novel marker for therapeutic response in patients with PH treated with pulmonary vasodilator medications and may offer robust predictive value independent of existing markers of disease severity.


Assuntos
Anti-Hipertensivos/uso terapêutico , Ventrículos do Coração/fisiopatologia , Hipertensão Pulmonar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Resistência Vascular/fisiologia , Função Ventricular Direita/fisiologia , Pressão Ventricular/fisiologia , Cateterismo Cardíaco , Diástole , Teste de Esforço , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Pressão Propulsora Pulmonar/fisiologia , Estudos Retrospectivos , Volume Sistólico
11.
Clin Transplant ; 31(7)2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28425132

RESUMO

INTRODUCTION: Little data exist regarding optimal therapeutic strategies postoperatively after lung transplant (LTx). Current practice patterns rely on expert opinion and institutional experience resulting in nonuniform postoperative care. To better define current practice patterns, an international survey of LTx clinicians was conducted. METHODS: A 30-question survey was sent to transplant clinicians via email to the International Society of Heart and Lung Transplantation open forum mailing list and directly to the chief transplant surgeon and pulmonologist of all LTx centers in the United States. RESULTS: Fifty-two clinicians representing 10 countries responded to the survey. Sedatives use patterns included: opiates + propofol (57.2%), opiates + dexmedetomidine (18.4%), opiates + intermittent benzodiazepines (14.3%), opiates + continuous benzodiazepines (8.2%), and opiates alone (2%). About 40.4% reported no formal sedation scale was followed and 13.5% of programs had no formal policy on sedation and analgesia. A lung protective strategy was commonly employed, with 13.8%, 51.3%, and 35.9% of respondents using tidal volumes of <6 mL/kg ideal body weight (IBW), 6 mL/kg IBW, and 8 mL/kg IBW, respectively. CONCLUSION: Practice patterns in the early postoperative care of lung transplant recipients differ considerably among centers. Many of the reported practices do not conform to consensus guidelines on management of critically ill patients.


Assuntos
Transplante de Pulmão/métodos , Cuidados Pós-Operatórios/normas , Guias de Prática Clínica como Assunto/normas , Padrões de Prática Médica/normas , Protocolos Clínicos , Gerenciamento Clínico , Humanos , Agências Internacionais , Inquéritos e Questionários
12.
Chest ; 151(1): 204-214, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27554299

RESUMO

Pulmonary hypertension (PH) can be triggered by any number of disease processes that result in increased pulmonary vascular resistance. Although historically associated with idiopathic pulmonary arterial hypertension (PAH), most patients with PH do not have the idiopathic subtype, but rather PH associated with another underlying diagnosis, such as left heart or lung disease. The World Health Organization (WHO) classification of PH helps conceptualize the different categories based on presumed etiology. WHO group 3 is PH associated with lung disease. This review focuses on PH in diffuse parenchymal lung diseases (DPLDs), such as the idiopathic interstitial pneumonias and other more rare forms of DPLD. Although there are clear associations of PH with DPLD, the exact pathophysiologic mechanisms and full clinical significance remain uncertain. Treatment of PH related to DPLD remains investigational, but an area of great interest given the negative prognostic implications and the growing number of available pulmonary vasoactive agents.


Assuntos
Hipertensão Pulmonar , Doenças Pulmonares Intersticiais , Gerenciamento Clínico , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Prognóstico
13.
Multidiscip Respir Med ; 11: 40, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27872748

RESUMO

BACKGROUND: Hemothorax after lung transplantation may result in increased post-operative morbidity and mortality. Risk factors for developing hemothorax and the outcomes of patients who develop hemothorax have not been well studied. METHODS: A retrospective chart review was performed on all patients who underwent lung transplantation at a single center between March 2009 and July 2014. Comparison was made between patients with and without hemothorax post-transplant. RESULTS: There were 132 lung transplantations performed during the study period. Hemothorax was a complication in 17 (12.9 %) patients, occurring an average of 9 days after transplant. No difference was found between the hemothorax and non-hemothorax groups with respect to age, preoperative anticoagulation, lung allocation score, prior thoracotomy, coagulation profile, use of cardiopulmonary bypass, ischemic time, or postoperative P/F ratio. There was a trend towards a higher incidence of hemothorax in patients with underlying sarcoidosis and re-transplantation (p = 0.13 and 0.17, respectively). Hemothorax developed early (<48 h post-operatively) in 5 patients and presented in a delayed manner (≥48 h post-operatively) in 12 patients. Delayed hemothorax occurred primarily in the setting of anticoagulation (10 out of 12 patients). The hemothorax group had decreased ventilator-free days (p = 0.006), increased ICU length of stay (p = 0.01) and increased hospital length of stay (p = 0.005). Hemothorax was also associated with reduced 90-day survival (p = 0.001), but similar 1, 3, and 5-year survival (p = 0.63, p = 0.30, and p = 0.25), respectively). CONCLUSION: The development of hemothorax is associated with increased morbidity and decreased short-term survival. Hemothorax may present either within the first 48 h after surgery or in a delayed fashion, most commonly in the setting of anticoagulation.

14.
Eur Respir J ; 47(5): 1445-51, 2016 05.
Artigo em Inglês | MEDLINE | ID: mdl-26846836

RESUMO

UNLABELLED: IPF patients have heightened propensity for pulmonary hypertension, which portends a worse outcome. Presence of pulmonary hypertension may be reflected in an enlarged pulmonary artery. We investigated pulmonary artery size measured on high-resolution computed tomography (HRCT) as an outcome predictor in IPF.We retrospectively reviewed all IPF patients evaluated at a tertiary-care centre between 2008 and 2013. Pulmonary artery and ascending aorta diameters were measured from chest HRCT with pulmonary artery:ascending aorta diameter (PA:A) ratio calculations. Outcome analysis defined by either death or lung transplant based on pulmonary artery size and PA:A ratio over 60 months was performed. Independent effects of different variables on overall outcomes were evaluated using the Cox proportional hazards model.98 IPF patients with available HRCT scans had a mean pulmonary artery diameter and PA:A ratio of 32.8 mm and 0.94, respectively. Patients with a PA:A ratio >1 had higher risk of death or transplant compared with a PA:A ratio ≤1 (p<0.001). A PA:A ratio >1 was also an independent predictor of outcomes in unadjusted and adjusted outcomes analyses (hazard ratio 3.99, p<0.001 and hazard ratio 3.35, p=0.002, respectively).A PA:A ratio >1 is associated with worse outcomes in patients with IPF. HRCT PA: A ratio measurement may assist in risk stratification and prognostication of IPF patients.


Assuntos
Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Hipertensão Pulmonar/diagnóstico por imagem , Fibrose Pulmonar Idiopática/diagnóstico , Artéria Pulmonar/diagnóstico por imagem , Idoso , Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/fisiopatologia , Feminino , Volume Expiratório Forçado , Humanos , Hipertensão Pulmonar/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Pulmão/fisiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Artéria Pulmonar/fisiopatologia , Análise de Regressão , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Capacidade Vital
15.
Chest ; 147(1): 173-179, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25188694

RESUMO

OBJECTIVE: The outcomes of patients with idiopathic pulmonary fibrosis (IPF) who undergo hospitalization have not been well characterized. We sought to determine the frequency of all-cause and respiratory-related hospitalizations and to evaluate their impact on the subsequent course and survival of patients with IPF. METHODS: The records of patients with IPF evaluated at a tertiary center were examined for the cause and duration of hospitalization. Data on subsequent patient outcomes were collated. RESULTS: The IPF cohort consisted of 592 patients, 25.3% of whom were hospitalized subsequent to their IPF diagnosis. A respiratory-related cause accounted for 77.3% of these hospitalizations. The median transplant-free survival for all patients was 23.3 months (interquartile range [IQR], 7.6-63.6 months) from the time of consultation. Transplant-free survival after hospital admission was much lower for patients with a respiratory hospitalization compared with those with a nonrespiratory hospitalization (median survival, 2.8 months [IQR, 0.63-16.2 months] vs 27.7 months [IQR, 7.4-59.6 months]; P = .0004). Multivariate analyses demonstrated that both all-cause and respiratory-related hospitalizations were strongly associated with mortality after adjusting for baseline demographics. Among patients with a respiratory hospitalization, 22.4% died while in the hospital, whereas 16.4% eventually went on to lung transplantation. CONCLUSIONS: Hospitalizations are common events in patients with IPF. Most hospitalizations are respiratory-related and are associated with high in-hospital mortality and limited survival beyond discharge. Both all-cause and respiratory hospitalizations are associated with mortality, and therefore, either could be used as an end point in IPF clinical trials.


Assuntos
Hospitalização , Fibrose Pulmonar Idiopática/terapia , Idoso , California/epidemiologia , Causas de Morte/tendências , Feminino , Seguimentos , Mortalidade Hospitalar/tendências , Humanos , Fibrose Pulmonar Idiopática/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Taxa de Sobrevida/tendências , Fatores de Tempo
16.
Respir Med ; 108(11): 1626-32, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25225149

RESUMO

RATIONALE: The relationship between pulmonary artery size with underlying pulmonary hypertension and mortality remains to be determined in COPD. We sought to evaluate the relationships in a cohort of patients with advanced COPD. METHODS: A retrospective study of advanced COPD patients evaluated between 1998 and 2012 was conducted at a tertiary care center. Patients with chest computed tomography images and right heart catheterizations formed the study cohort. The diameters of the pulmonary artery and ascending aorta were measured by independent observers and compared to pulmonary artery pressures. Intermediate-term mortality was evaluated for the 24-month period subsequent to the respective studies. Cox proportional hazards model was used to determine independent effects of variables on survival. RESULTS: There were 65 subjects identified, of whom 38 (58%) had pulmonary hypertension. Patients with and without pulmonary hypertension had mean pulmonary artery diameters of 34.4 mm and 29.1 mm, respectively (p = 0.0003). The mean PA:A ratio for those with and without pulmonary hypertension was 1.05 and 0.87, respectively (p = 0.0003). The PA:A ratio was an independent predictor of mortality with a reduced survival in those with a PA:A >1 (p = 0.008). CONCLUSIONS: The PA:A ratio is associated with underlying pulmonary hypertension in patients with COPD and is an independent predictor of mortality. This readily available measurement may be a valuable non-invasive screening tool for underlying pulmonary hypertension in COPD patients and appears to impart important independent prognostic information.


Assuntos
Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/patologia , Artéria Pulmonar/patologia , Doença Pulmonar Obstrutiva Crônica/complicações , Aorta/patologia , Pressão Arterial/fisiologia , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/diagnóstico por imagem , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X
17.
Crit Care Clin ; 30(3): 475-98, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24996606

RESUMO

Right ventricular failure complicates several commonly encountered conditions in the intensive care unit. Right ventricular dilation and paradoxic movement of the interventricular septum on echocardiography establishes the diagnosis. Right heart catheterization is useful in establishing the specific cause and aids clinicians in management. Principles of treatment focus on reversal of the underlying cause, optimization of right ventricular preload and contractility, and reduction of right ventricular afterload. Mechanical support with right ventricular assist device or veno-arterial extracorporeal membrane oxygenation can be used in select patients who fail to improve with optimal medical therapy.


Assuntos
Cuidados Críticos/métodos , Estado Terminal/terapia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/terapia , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/terapia , Insuficiência Cardíaca/complicações , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/terapia , Guias de Prática Clínica como Assunto , Síndrome do Desconforto Respiratório do Adulto/complicações , Síndrome do Desconforto Respiratório do Adulto/diagnóstico , Síndrome do Desconforto Respiratório do Adulto/terapia , Disfunção Ventricular Direita/complicações
18.
Chest ; 144(6): 1900-1905, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-23975232

RESUMO

BACKGROUND: The goal of the 6-min walk test (6MWT) is to enable patients to walk "as far as possible" as a measure of their functional ability. The impact of the specific walk instructions on patient 6MWT performance is unknown. METHODS: Patients with pulmonary arterial hypertension (PAH), idiopathic pulmonary fibrosis (IPF), and other forms of interstitial lung disease (ILD) were recruited to perform four identical 6MWTs with one differing instructional phrase. The standard instruction to walk "as far as possible" was substituted in random order with "as fast as possible," "at your normal pace," or "at a leisurely pace." RESULTS: Twenty-four patients (10 with PAH, eight with IPF, six with other ILD) were enrolled and completed all four 6MWTs. Patients attained the greatest distance with the fast instruction, exceeding the standard instruction distance by a mean of 52.7 m (P < .001). The mean difference between the fast and standard walks was 41.5 m in the PAH group, 66.5 m in the IPF group, and 53 m in the other ILD group. CONCLUSIONS: Patients do not walk as far as they are able with the standard American Thoracic Society instruction for 6MWT. Changing the wording from "far" to "fast" may facilitate a better effort and greater distance during the test. It is possible that this modified 6MWT instruction may result in improved accuracy and reproducibility, thereby enhancing its clinical and research trial usefulness.


Assuntos
Teste de Esforço/métodos , Tolerância ao Exercício/fisiologia , Resistência Física/fisiologia , Caminhada/fisiologia , Adulto , Idoso , Feminino , Humanos , Hipertensão Pulmonar/fisiopatologia , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Análise e Desempenho de Tarefas
19.
Chest ; 143(6): 1692-1698, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23238641

RESUMO

BACKGROUND: The course of idiopathic pulmonary fibrosis (IPF) is characterized by variable patterns of disease progression. The red cell distribution width (RDW) is a parameter that is routinely reported with all CBC counts. We sought to test the prognostic usefulness of this parameter in a well-defined cohort of patients with IPF. METHODS: CBCs, demographics, and pulmonary function data from patients with IPF evaluated between January 1997 and June 2011 were collated. Patient outcomes were ascertained from the program's database and the Social Security Death Index. RESULTS: There were 319 patients with IPF evaluated in whom baseline CBCs were available. The range in the RDW was 11.9 to 21.9 (median 14.1). There were 228 subjects with RDW values ≤ 15 (normal) and 91 patients with RDW values > 15. Patients with normal RDW values had a median survival of 43.1 months compared with 16.3 months for those whose RDW was > 15 (P = .001). There were 198 patients with available serial RDW data. Those patients who had a change in the RDW of less or greater than +0.010/mo had median survivals of 43.0 and 23.9 months, respectively (P = .0246). CONCLUSIONS: The RDW is a readily available laboratory test result that may provide important, independent prognostic information at baseline and follow-up in patients with IPF. Further studies are warranted to validate this as a biomarker for IPF outcomes, as well as to define the biologic basis for this association.


Assuntos
Índices de Eritrócitos , Fibrose Pulmonar Idiopática/sangue , Idoso , Biomarcadores/sangue , Distribuição de Qui-Quadrado , Progressão da Doença , Feminino , Humanos , Masculino , Prognóstico , Modelos de Riscos Proporcionais , Testes de Função Respiratória , Estatísticas não Paramétricas , Análise de Sobrevida
20.
Chest ; 142(4): 1005-1010, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22797563

RESUMO

BACKGROUND: The characteristics of long-term survivors with idiopathic pulmonary fibrosis (IPF) have never been fully elucidated. We sought to illustrate the attenuated mortality and describe the characteristics of patients with IPF who survived at least 5 years beyond their initial presentation. METHODS: Patients with IPF evaluated between 1997 and 2006 were identified through the clinic database. Patients who survived beyond 5 years from the time of their evaluation were compared with those who died or underwent lung transplantation within 5 years. Survival analyses were performed from the time of initial evaluation and contingent on annualized survival thereafter. RESULTS: Eighty-seven patients who survived at least 5 years formed the comparator group to whom other patients were contrasted. These patients had a higher BMI, FVC % predicted, FEV1 % predicted, total lung capacity % predicted, and diffusing capacity of lung for carbon monoxide % predicted, but a lower FEV1/FVC ratio and lower mean pulmonary artery pressures. More than one-half of these patients had moderate or severe disease at the time of presentation. Our annualized contingent survival analyses revealed a progressively increasing median survival dependent on the duration of the disease. CONCLUSIONS: Although we were able to demonstrate differences in our 5-year survivors, rather than being a distinct group, these patients appear to exist within a continuum of improving survival dependent on prior disease duration. This progressively improving time-dependent prognosis mandates the serial reevaluation of an individual patient's projected outcomes. The implementation of dynamic counseling is an important concept in more accurately predicting life expectancy for patients with IPF who are frequently haunted by the prospects of a dismal survival.


Assuntos
Aconselhamento/métodos , Fibrose Pulmonar Idiopática/diagnóstico , Transplante de Pulmão , Adulto , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Feminino , Seguimentos , Volume Expiratório Forçado/fisiologia , Humanos , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/cirurgia , Masculino , Pessoa de Meia-Idade , Prognóstico , Testes de Função Respiratória , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Capacidade Pulmonar Total , Estados Unidos/epidemiologia
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