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Epidemiological Survey of Japanese Patients with Idiopathic Pulmonary Fibrosis and Investigation of Ethnic Differences.

Idiopathic pulmonary fibrosis (IPF) has an unknown etiology and poor prognosis. Several large-scale epidemiological studies have been conducted predominantly in Western countries. There are few studies reported from Asian countries. It remains unclear whether ethnic difference exists in IPF. It is important to determine the current IPF status in Asian populations and compare it with that of Western populations.

Objectives: To provide the epidemiological status of IPF in Japan and to investigate ethnic differences.

Methods: We selected Hokkaido prefecture (population, 5.6 million) as the epidemiological cohort of IPF among Japanese. On the basis of the clinical records of 553 IPF patients who were accepted based on the application of the Certificate of Medical Benefit between 2003 and 2007, we conducted a retrospective epidemiological and prognostic analysis.

Measurements and Main Results: The prevalence and cumulative incidence of IPF was 10.0 and 2.23 per 100,000 population, respectively, with 72.7% predominance of males and an increase in frequency with age. The median survival time was 35 months, and the most common (40%) cause of death was acute exacerbation. The most important factor influencing IPF prognosis was the percent vital capacity.

Conclusions: The status of IPF in the Japanese population was clarified for the first time through our study. Our results showed that in men, the incidence of death due to acute exacerbation was higher and that due to cardiovascular disease was lower in Japan than in Western countries. These results may suggest ethnic differences in IPF.

The role of the microbiome in exacerbations of chronic lung diseases.

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The role of the microbiome in exacerbations of chronic lung diseases.

Lancet. 2014 Aug 23;384(9944):691-702

Authors: Dickson RP, Martinez FJ, Huffnagle GB

Abstract
Culture-independent microbiological techniques have shown a previously unappreciated complexity to the bacterial microbiome of the respiratory tract that forces reconsideration of the interactions between host, bacteria, and the pathogenesis of exacerbations of chronic lung disease. The composition of the lung microbiome is determined by microbial immigration, elimination, and relative growth rates of its members. All these factors change dramatically in chronic lung disease and further during exacerbations. Exacerbations lack the features of bacterial infections, including increased bacterial burden and decreased diversity of microbial communities. We propose that exacerbations are occasions of respiratory tract dysbiosis--a disorder of the respiratory tract microbial ecosystem with negative effects on host biology. Respiratory tract dysbiosis provokes a dysregulated host immune response, which in turn alters growth conditions for microbes in airways, promoting further dysbiosis and perpetuating a cycle of inflammation and disordered microbiota. Differences in the composition of baseline respiratory tract microbiota might help to explain the so-called frequent-exacerbator phenotype observed in several disease states, and might provide novel targets for therapeutic intervention.

PMID: 25152271 [PubMed - in process]

Sleep in Patients with Restrictive Lung Disease.

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Sleep in Patients with Restrictive Lung Disease.

Clin Chest Med. 2014 Sep;35(3):505-512

Authors: Won CH, Kryger M

Abstract
Restrictive lung disease leads to ventilatory defects and diffusion impairments. These changes may contribute to abnormal nocturnal pathophysiology, including sleep architecture disruption and impaired ventilation and oxygenation. Patients with restrictive lung disease may suffer significant daytime fatigue and dysfunction. Hypercarbia and hypoxemia during sleep may impact progression of lung disease and related symptoms. Little is known about the impact of treatment of sleep disruption on sleep quality and overall prognosis in restrictive lung disease. This review discusses the pathophysiology of sleep and comorbid sleep disorders in restrictive lung diseases including interstitial lung disease, neuromuscular disease, and obesity hypoventilation syndrome.

PMID: 25156766 [PubMed - as supplied by publisher]

Comparison of the yield of different diagnostic procedures for cellular differentiation and genetic profiling of non-small-cell lung cancer.

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Comparison of the yield of different diagnostic procedures for cellular differentiation and genetic profiling of non-small-cell lung cancer.

J Thorac Oncol. 2014 Aug;9(8):1120-5

Authors: Albanna AS, Kasymjanova G, Robitaille C, Cohen V, Brandao G, Pepe C, Small D, Agulnik J

Abstract
INTRODUCTION: As treatments for non-small-cell lung cancer (NSCLC) become personalized, cellular and molecular differentiation of the tumor is becoming the standard of care. Our objective is to compare the yield of different diagnostic procedures for cellular differentiation of NSCLC and analysis of epidermal growth factor receptor (EGFR) mutation.
METHODS: We evaluated all patients diagnosed with NSCLC from January 2004 to September 2010 at the Jewish General Hospital, Montreal. Diagnostic procedures included surgical biopsies, nonsurgical histologic biopsies (endobronchial and core needle), transbronchial needle aspirate (TBNA) and transthoracic needle aspirate (TTNA), bronchoalveolar lavage (BAL), and pleural fluid samples.
RESULTS: We included 702 subjects investigated for histopathologic differentiation of NSCLC. Of these, 269 were also investigated for EGFR mutation. Failure to ascertain the cellular subtype and EGFR mutation status was least likely with surgical specimens (0% and 1.8%, respectively); followed by TTNA (14% and 10%, respectively) and histologic biopsy (18% for both); and was frequent with TBNA (39% and 30%, respectively). Although BAL and pleural fluid specimens provided reasonable yield for cellular differentiation (20 % and 11%, respectively), their results were not accurate in 6% of their samples when compared with concurrent or subsequent surgical specimens (reference standard) performed in a subgroup of patients.
CONCLUSION: Radiologically guided TTNA and histologic biopsies provided high yield for both molecular and histologic analyses. The yield of unguided TBNA was relatively low. Further studies are needed to assess the adequacy of BAL and pleural fluid samples for EGFR mutation analysis and accurate characterization of cellular subtypes of NSCLC.

PMID: 25157765 [PubMed - in process]

Surgery for small cell lung cancer: a retrospective analysis of 243 patients from Japanese lung cancer registry in 2004.

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Surgery for small cell lung cancer: a retrospective analysis of 243 patients from Japanese lung cancer registry in 2004.

J Thorac Oncol. 2014 Aug;9(8):1140-5

Authors: Takei H, Kondo H, Miyaoka E, Asamura H, Yoshino I, Date H, Okumura M, Tada H, Fujii Y, Nakanishi Y, Eguchi K, Dosaka-Akita H, Kobayashi H, Sawabata N, Yokoi K, Japanese Joint Committee of Lung Cancer Registry

Abstract
INTRODUCTION: Indications for surgical resection for small cell lung cancer (SCLC) have been very limited. Because early-stage SCLC is a rare presentation of lung cancer, studies comparing surgical resection among a large number of patients are unlikely to be conducted. This study reports the most recent surgical outcomes of a large number of SCLC patients who underwent surgery in 2004.
METHODS: In 2010, the Japanese Joint Committee of Lung Cancer Registry performed a nationwide retrospective registry study regarding the prognosis and clinicopathologic profiles of 11,663 patients who underwent resection for primary lung cancer in 2004. Of the 11,663 patients, 243 patients with SCLC (2.1%) were included in this study. The registry data of the patients with SCLC were analyzed, and the clinicopathologic profiles and surgical outcomes of the patients were evaluated.
RESULTS: The 5-year survival rate for all cases (n = 243, 213 males, mean age 68.2 years) was 52.6%. The 5-year survival rates by c-stage and p-stage were as follows: IA, 64.3% (n = 132) and 72.3% (n = 93); IB, 45.7% (n = 36) and 61.1% (n = 51); IIA, 50.5% (n =25); and 44.8% (n = 27); IIB, 33.3% (n = 10) and 40.3% (n = 17); IIIA, 30.5% (n = 30) and 23.4% (n = 45); and IV, 0% (n = 7) and 0% (n = 9), respectively. A multivariate analysis showed that the significant prognostic factors were age, gender, c-stage, and surgical curability. A kappa value was moderate conformity between c-stage and p-stage in all cases.
CONCLUSIONS: Surgical resection in selected patients with early-stage SCLC, especially stage I, had favorable results.

PMID: 25157766 [PubMed - in process]

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