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Introduction

Pirfenidone was the first antifibrotic drug approved in Argentina for idiopathic pulmonary fibrosis (IPF). Outcomes in real life may differ from the results of clinical trials. The primary endpoint was to study the tolerance of pirfenidone in real life. Secondary endpoints were to analyze effectiveness and reasons for discontinuation.

Materials and methods

Retrospective observational study conducted in four specialized centers in Argentina. We analyzed the medical records of patients with IPF who received pirfenidone between June 2013 and September 2016. Adverse events (AE) and the variables that could influence these results were analyzed. Forced vital capacity (FVC%) parameters were also compared between the pre-pirfenidone and post-pirfenidone periods.

Results

Fifty patients were included, 38 (76%) men, with mean age (SD) 67.8 (8.36) years. Mean (SD) exposure to pirfenidone was 645.68 (428.19) days, with a mean daily dose (SD) of 2064.56 mg (301.49). Nineteen AEs in 15 patients (30%) were reported: nausea (14%), asthenia (10%) and skin rash (8%). A total of 18 patients (36%) interrupted treatment, only 1 definitively. The most frequent reason for discontinuation was failure of suppliers to provide the drug (9 subjects; 18%). We compared the evolution of FVC% between the pre-pirfenidone and post-pirfenidone periods, and found a mean (SD) FVC% decline of 4.03% (7.63) pre-pirfenidone and 2.64% (7.1) post-pirfenidone (P=.534).

Conclusions

In our study, pirfenidone was well tolerated and associated with a reduction in FVC decline, although without reaching statistical significance.  相似文献   
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PURPOSE: The aim of this study was to compare the value of endoluminal ultrasonography (ELUS) with magnetic resonance imaging (MRI) for preoperative staging of rectal carcinoma. METHODS: Thirty-seven consecutive patients were examined by ELUS and MRI. Imaging results were compared with pathohistologic studies. A tumor extending beyond the bowel wall was considered to be positive and one within the bowel wall was considered negative. Lymph node involvement was considered present if nodes equal to or greater than 5 mm in diameter were found in the perirectal tissue. For evaluating the differences between the two methods, the Mc Nemar test was performed. RESULTS: T-Staging was correct in 88.2 percent (30/34) of patients by ELUS and in 82.3 percent (28/34) by MRI (difference not significant). N-Staging was correct in 80 percent (20/25) by ELUS and in 60 percent (15/25) by MRI (difference of borderline significance). A comprehensive preoperative staging (T + N) was made correctly in 68 percent (17/25) by ELUS and in 48 percent only (12/25) by MRI (difference not significant). CONCLUSIONS: We suggest that ELUS and MRI must be evaluated within the framework of established parameters when treatment modalities such as preoperative radiation therapy and local or radical surgical approach must be decided.  相似文献   
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Clinical Rheumatology - Kawasaki disease (KD) is the most frequent cause of acquired heart disease in children in high-income countries because of coronary artery involvement. Risk factors for...  相似文献   
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The absence or very low levels of plasminogen cause a rare disabling disease called ligneous conjunctivitis, characterized by the growth of fibrin-rich pseudomembranes in the conjunctiva and on other mucosal surfaces. Several mutations have been detected in the plasminogen gene of patients affected with ligneous conjunctivitis. The human plasminogen gene, located on chromosome 6, has a marked homology with the genes belonging to the plasminogen-apo(a) family, and with a number of pseudogenes and plasminogen-like genes located on chromosome 2. This work describes a series of nucleotide variations related to genes other than the plasminogen one, found during the genetic characterization of plasminogen defect in two unrelated patients with ligneous conjunctivitis. The results of automated sequences of each exon and intron-exon boundaries were compared with those of the human plasminogen gene from the NCBI gene bank. In particular, a co-amplified gene on chromosome 2 mimicking a 14 bp deletion in exon 5 of the plasminogen gene was identified by sequencing two different bands obtained from a long run of the PCR exon 5 product in NuSieve agarose gel, and by PstI restriction enzyme analysis of the same amplicons. Moreover, 21 single nucleotide exchanges due to plasminogen-like genes co-amplification were observed, namely one in exon 1, two in exon 4, three in exons 3, 5 and 16, four in exon 13, and five in exon 17. In conclusion, these data confirm the difficulty of plasminogen genetic analysis and may help researchers to better identify the true plasminogen gene mutations causing molecular defects.  相似文献   
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