The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies.

RATIONALE: Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS. OBJECTIVES: To elucidate the long-term outcome of ARS-ILD with and without PM/DM and compare it to that of IPF. METHODS: A two-center retrospective study was conducted. The study population comprised 36 patients with ARS-ILD (8 with PM, 12 with DM, and 16 without myositis throughout the course), 100 patients with IPF without anti-ARS, and 7 patients with NSIP without anti-ARS. The presence of anti-ARS was determined by RNA immunoprecipitation using the sera obtained at the time of diagnosis before specific treatment. MEASUREMENTS AND MAIN RESULTS: During the observational period (median 49 months; range, 1-114 months), 7 patients with ARS-ILD (19%; 3 with PM, 1 with DM, and 3 without PM/DM) and 51 patients with IPF (51%) died. Patients with ARS-ILD had better overall survival than those with IPF (log-rank test, P < 0.001) and similar survival compared to those with NSIP (log-rank test, P = 0.59). The prognosis for patients with ARS-ILD was similar between those with and without myositis (log-rank test, P = 0.91). At the median follow-up time of 76.5 months, 14 of the 36 patients with ARS-ILD had deteriorated. Both a decline in forced vital capacity or an initiation of long-term oxygen therapy during the course (odds ratio [OR], 5.34) and acute exacerbation (OR, 28.4) significantly increased the mortality risk. CONCLUSIONS: The long-term outcome of ARS-ILD was significantly better than that of IPF regardless of the presence or absence of myositis.

The influence of environmental exposure on the response to antimicrobial treatment in pulmonary Mycobacterial avium complex disease.

BACKGROUND: Environmental exposure is a likely risk factor for the development of pulmonary Mycobacterium avium complex (MAC) disease. The influence of environmental exposure on the response to antimicrobial treatment and relapse is unknown. METHODS: We recruited 72 patients with pulmonary MAC disease (male [female], 18 [54]; age, 61.7 ± 10.3 years) who initiated and completed standard three-drug regimens for more than 12 months between January 2007 and December 2011. The factors associated with sputum conversion, relapse and treatment success without relapse were retrospectively evaluated after adjustments for confounding predictors. RESULTS: Fifty-two patients (72.2%) demonstrated sputum conversion, and 15 patients (28.8%) relapsed. A total of 37 patients (51.4%) demonstrated treatment success. Sputum conversion was associated with negative smears (odds ratio [OR], 3.89; 95% confidence interval [CI], 1.27-12.60; P = 0.02). A relapse occurred in patients with low soil exposure after the start of treatment less frequently than in patients with high soil exposure (7/42 [16.7%] vs. 8/10 [80.0%], P = 0.0003). Treatment success was associated with low soil exposure after the beginning of treatment (OR, 13.46; 95% CI, 3.24-93.43; P = 0.0001) and a negative smear (OR, 2.97; 95% CI, 1.02-9.13; P = 0.047). CONCLUSION: Low soil exposure was independently associated with better microbiological outcomes in patients with pulmonary MAC disease after adjusting for confounding clinical, microbiological and radiographic findings.

Assessing binaural/bimodal advantages using auditory event-related potentials in subjects with cochlear implants.

OBJECTIVE: To assess the advantage of binaural and bimodal hearing for subjects with cochlear implant (CI) using auditory event-related potentials as well as speech perception tests. SUBJECTS AND METHODS: The subjects comprised four binaural CI users (CI/CI group) and eleven bimodal CI users, who wore a hearing aid (HA) contralaterally (CI/HA group). All subjects had used binaural or bimodal fitting for over 6 months. Their speech perception was examined in a quiet environment using monosyllabic words. Late cortical waves were measured while subjects were engaged in an oddball task of 1kHz frequent and 2kHz rare tone stimuli. The latencies of event-related potential (N1, N2, P3) were compared for monaural, binaural, and bimodal hearing conditions. RESULTS: Significantly (p<0.01, paired t-test) better speech perception for monosyllabic words was found for both binaural and bimodal hearing than monaural hearing. The latency of N1 did not significantly change for either binaural or bimodal hearing. On the other hand, the latency of N2 was significantly (p<0.01, paired t-test) shorter for binaural and bimodal hearing than for monaural hearing. The latency of P3 was shorter for binaural and bimodal hearing than monaural hearing in all subjects, and the difference was statistically significant in both CI/CI and CI/HA groups (p<0.01, paired t-test). CONCLUSIONS: Better speech perception was obtained for binaural and bimodal hearing than for monaural hearing in CI subjects. The results obtained in the comparison of P3 latency agreed with that of speech perception. Thus, using bilateral hearing devices is recommended for CI subjects. We also found that event-related potentials were useful as an objective tool to assess the advantage of binaural and bimodal hearing for CI subjects.

Prognostic Value of FDG-PET in patients with oropharyngeal carcinoma treated with concurrent chemoradiotherapy.

PURPOSE: The purpose of this study was to evaluate the predictive value of 2-deoxy-2-[F-18]fluoro-D-glucose-positron emission tomography (FDG-PET) following concurrent chemoradiotherapy (CRT) on survival in patients with carcinoma of the oropharynx (OPC). METHODS: Eighteen patients with primary OPC who underwent PET pre- and post-CRT were evaluated prospectively for survival. The prognostic performance of post-CRT PET and CT for recurrence was compared. RESULTS: Patients with positive post-CRT PET exhibited significantly lower 2-year cause-specific survival and disease-free survival (50% vs. 91%, P < 0.05 and 0% vs. 83%, P < 0.0001); however, patients with positive post-CRT CT did not exhibit any significant difference (67% vs. 83%, P = 0.416 and 50% vs. 75%, P = 0.070). Other factors, such as clinical and pre-CRT PET variables, also did not indicate any significant difference. The accuracy of prediction of residual and local recurrence for post-CRT PET and CT (local%/regional%) was 83%/94% and 83%/78%, respectively. CONCLUSION: OPC patients with positive post-CRT PET exhibit poor survival. The prognostic accuracy of post-CRT PET is superior to that of CT. The results of post-CRT FDG-PET should be included in the management of the OPC patients.

Progressive hearing loss in mice carrying a mutation in the p75 gene.

The neurotrophin receptor p75 (p75NTR) is expressed in the developmental stage of the cochlea. However, the role of the p75NTR in the inner ear remains to be established. In this study, we conducted electrophysiological and morphological analyses of the auditory function of mice carrying a mutation in the p75 gene at different longitudinal stages. The mice carrying a mutation in the p75 gene showed an age-related progressive hearing loss. At 1 month, there was no obvious morphological change in the cochlea of the mice carrying a mutation in the p75 gene compared to wild-type mice, except for a slight loss of spiral ganglion neurons (SGNs). Auditory function was not significantly different between both genotypes from 1 to up to 4 months of age. The mice carrying a mutation in the p75 gene started to show progressive hearing loss at 4 months, when both SGN degeneration and hair cell (HC) loss were observed at the basal turn. These results suggest that the neurotrophin receptor p75 may play a significant role in the maintenance of cochlear function, and that mice carrying a mutation in the p75 gene could be a good animal model of early onset progressive hearing loss.

Transcatheter arterial embolization for tumor seeding in the chest wall after radiofrequency ablation for hepatocellular carcinoma.

Tumor seeding in the chest wall was depicted at follow-up CT obtained 9 months after radiofrequency ablation for hepatocellular carcinoma. Transcatheter arterial embolization was successfully performed, injecting emulsion of 10 mg of epirubicin and 1 ml of iodized oil followed by gelatin sponge particles via the microcatheter placed in the right eleventh intercostal artery. The patient died of tumor growth in the liver one year after the embolization, but no progression of the tumor seeding was noted during the follow-up period. We conclude that transcatheter arterial embolization was effective for the control of tumor seeding after radiofrequency ablation for hepatocellular carcinoma.

Up-regulation of cochlear aquaporin-3 mRNA expression after intra-endolymphatic sac application of dexamethasone.

The final aim of the present study is to see if the endolymphatic sac is really available as a drug delivery system to have effect on the inner ear organs. In the present study, we examined effects of a single insertion of dexamethasone into the rat unilateral endolymphatic sac on mRNA expression of the inner ear aquaporin (AQP) family, transmembrane water transporters and putative endolymphatic fluid modulators, by means of real-time quantitative PCR. Only AQP-3 mRNA expression in the ipsilateral cochlea was significantly up-regulated in comparison with controls and the up-regulation was demonstrated both in dose-dependent and time-dependent manners. These findings suggest that the intra-endolymphatic sac steroids could make regulatory effects on the inner ear AQP-3 expression via vestibular aqueduct and modulate the homeostasis of endolymphatic fluids, encouraging the possibility that the endolymphatic sac could be a therapeutic window for the inner ear disease.

Evaluation of provocation test monitoring palmoplantar temperature with the use of thermography for diagnosis of focal tonsillar infection in palmoplantar pustulosis.

BACKGROUND: Since focal tonsillar infections are often associated with palmoplantar pustulosis (PPP), provocation tests have been performed for preoperative evaluation of tonsillectomy. However, these tests have not been fully established. OBJECTIVES: To introduce a more sensitive operative indication for tonsillectomy to the patients with PPP, we have monitored the temperature after provocation tests at palmoplantar sites, as measured by thermography, and we hypothesized that this methodology may lead to a more sensitive marker for tonsillectomy. METHODS: Twenty-two PPP patients with/without clinical tonsillitis were included in this study. After mechanical tonsillar massage, using infrared thermography, we have monitored the surface temperature at palmoplantar sites of 22 patients with PPP, five chronic tonsillitis patients without PPP, and four healthy controls, to compare the findings with the skin lesional outcome after tonsillectomy. RESULTS: There was a significant relationship between the effects of tonsillectomy and the results of provocation tests assessed by thermography. The sensitivity, specificity, and efficiency of the provocation tests with thermography of detecting a favorable outcome of tonsillectomy were 75.0, 83.3, and 77.3%, respectively, while those of the provocation tests as estimated with the conventional criteria were 37.5, 83.3, and 50.0%, respectively. CONCLUSION: Our results suggest that a new indicator using non-invasive thermography for the provocation tests is useful in predicting the effects of tonsillectomy for PPP.