Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2 and SLC26A4 mutations

Conclusion. We have demonstrated differences in cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. The differences in cortical processing patterns between these two cases may have been influenced by the differing clinical courses and pathogenesis of hearing loss due to genetic mutations. Our results suggest the importance of hearing during early childhood for the development of a normal cortical language network. Objectives. To investigate the cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. Methods: The cortical activity of two adult patients with known genetic mutations (GJB2, SLC26A4) was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task and compared with that of normal-hearing controls. Results: A patient with a GJB2 mutation showed activation in the right auditory association area [BA21, BA22], and the left auditory association area [BA42] even with visual language task; in contrast, a patient with an SLC26A4 mutation showed no significant activation in the corresponding area.ArticleACTA OTO-LARYNGOLOGICA. 131(11):1232-1236 (2011)journal articl

Impact of Parenting Style on Clinically Significant Behavioral Problems Among Children Aged 4–11 Years Old After Disaster: A Follow-Up Study of the Great East Japan Earthquake

Objective: The purpose of this study was to investigate the influence of parenting style on children's behavior problems after the Great East Japan Earthquake in 2011.Methods: Participants were children exposed to the 2011 disaster at preschool age (n = 163). Data were collected from August 2012 to March 2013, and from July 2014 to March 2015 (2 and 4 years, respectively, after the earthquake), thus participants were aged 4–11 years when assessed. Parenting style was assessed by caregivers using the Alabama Parenting Questionnaire (APQ), which measures parental involvement, positive parenting, poor monitoring/supervision, inconsistent discipline, and corporal punishment in the second year after the disaster. Behavior problems were assessed by caregivers using the Child Behavior Checklist (CBCL), which identifies internalizing, externalizing, and total problems in the second and fourth year after the disaster.Results: The results show that corporal punishment in the second year after the disaster had negative influence on CBCL internalizing score (coefficient: 0.78, 95%CI: 0.12–1.45, p = 0.023), externalizing score (coefficient: 0.74, 95%CI: 0.09–1.39, p = 0.025), and total score in the fourth year after the disaster (coefficient: 0.85, 95%CI: 0.16–1.55, p = 0.016), after adjusted for children's age, sex, the number of trauma experiences, maternal education, the number of siblings, temporally housing experience, and CBCL each scores in the second year after the disaster. Other parenting style did not affect children's behavioral problems.Conclusion: The result suggests that inadequate rearing after a natural disaster had negative impact on the behavior problems of the affected children in 4 years later of the disaster. Specifically, corporal punishment had negative influence on children's behavior problems

Two Relapsed Stage III Childhood Anaplastic Large Cell Lymphoma Patients with NPM-ALK Fusion in Bone Marrow from Initial Diagnosis

Childhood anaplastic large cell lymphoma (ALCL) accounts for approx. 10–30 of cases of non-Hodgkin lymphoma, and the ALCL99 study reported 60–75 disease-free survival; however, a relatively high relapse rate was observed (25–30 ). We report 2 patients with Stage III ALCL who relapsed 6–18 months after the end of ALCL99 chemotherapy. A retrospective molecular analysis identified the nucleophosmin (NPM)-anaplastic lymphoma kinase (ALK) fusion gene in the first diagnostic bone marrow samples taken from both patients. However, antibodies against the ALK protein appeared to be relatively low in the serum of both patients (×100 and ×750). An increase in chemotherapy intensity may be beneficial if Stage III ALCL patients are shown to be NPM-ALK chimera-positive in the first diagnostic bone marrow sample

Two Relapsed Stage III Childhood Anaplastic Large Cell Lymphoma Patients with NPM-ALK Fusion in Bone Marrow from Initial Diagnosis

Childhood anaplastic large cell lymphoma (ALCL) accounts for approx. 10–30 of cases of non-Hodgkin lymphoma, and the ALCL99 study reported 60–75 disease-free survival; however, a relatively high relapse rate was observed (25–30 ). We report 2 patients with Stage III ALCL who relapsed 6–18 months after the end of ALCL99 chemotherapy. A retrospective molecular analysis identified the nucleophosmin (NPM)-anaplastic lymphoma kinase (ALK) fusion gene in the first diagnostic bone marrow samples taken from both patients. However, antibodies against the ALK protein appeared to be relatively low in the serum of both patients (×100 and ×750). An increase in chemotherapy intensity may be beneficial if Stage III ALCL patients are shown to be NPM-ALK chimera-positive in the first diagnostic bone marrow sample

Pleiotropic effects of levofloxacin, fluoroquinolone antibiotics, against influenza virus-induced lung injury

© 2015 Enoki et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Reactive oxygen species (ROS) and nitric oxide (NO) are major pathogenic molecules produced during viral lung infections, including influenza. While fluoroquinolones are widely used as antimicrobial agents for treating a variety of bacterial infections, including secondary infections associated with the influenza virus, it has been reported that they also function as anti-oxidants against ROS and as a NO regulator. Therefore, we hypothesized that levofloxacin (LVFX), one of the most frequently used fluoroquinolone derivatives, may attenuate pulmonary injuries associated with influenza virus infections by inhibiting the production of ROS species such as hydroxyl radicals and neutrophil-derived NO that is produced during an influenza viral infection. The therapeutic impact of LVFX was examined in a PR8 (H1N1) influenza virus-induced lung injury mouse model. ESR spin-trapping experiments indicated that LVFX showed scavenging activity against neutrophil-derived hydroxyl radicals. LVFX markedly improved the survival rate of mice that were infected with the influenza virus in a dose-dependent manner. In addition, the LVFX treatment resulted in a dose-dependent decrease in the level of 8-hydroxy-2'-deoxyguanosine (a marker of oxidative stress) and nitrotyrosine (a nitrative marker) in the lungs of virus-infected mice, and the nitrite/nitrate ratio (NO metabolites) and IFN-? in BALF. These results indicate that LVFX may be of substantial benefit in the treatment of various acute inflammatory disorders such as influenza virus-induced pneumonia, by inhibiting inflammatory cell responses and suppressing the overproduction of NO in the lungs

SLC26A4 p.Thr410Met homozygous mutation in a patient with a cystic cochlea and an enlarged vestibular aqueduct showing characteristic features of incomplete partition type I and II.

Mutations of SLC26A4 are associated with incomplete partition type II (IP-II) and isolated enlargement of the vestibular aqueduct (EVA). We experienced a congenitally deaf 6-year-old boy with a rare p.Thr410Met homozygous mutation in SLC26A4 who underwent bilateral cochlear implantation. He had bilateral inner ear malformation, in which the dilated vestibule and EVA were identical to those in IP-II, but the cochlea lacking a bony modiolus resembled that in incomplete partition type I. These results suggest that homozygous mutations in SLC26A4 are always associated with EVA, while the severity of cochlear malformation may vary depending on the type of SLC26A4 mutation