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Athyrium yokoscense is hypertolerant to cadmium (Cd) and can grow normally under a high Cd concentration despite Cd being a highly toxic heavy metal. To mitigate Cd stress in general plant species, Cd is promptly chelated with a thiol compound and is isolated into vacuoles. Generated active oxygen species (ROS) in the cytoplasm are removed by reduced glutathione. However, we found many differences in the countermeasures in A. yokoscense. Thiol compounds accumulated in the stele of the roots, although a long-term Cd exposure induced Cd accumulation in the aerial parts. Synchrotron radiation-based X-ray fluorescence (SR-XRF) analysis indicated that a large amount of Cd was localized in the cell walls of the roots. Overexpression of AyNramp5a, encoding a representative Fe and Mn transporter of A. yokoscense, increased both Cd uptake and Fe and Mn uptake in rice calli under the Cd exposure conditions. Organic acids are known to play a key role in reducing Cd availability to the plants by forming chelation and preventing its entry in free form into the roots. In A. yokoscense roots, Organic acids were abundantly detected. Investigating the chemical forms of the Cd molecules by X-ray absorption fine structure (XAFS) analysis detected many compounds with Cd-oxygen (Cd-O) binding in A. yokoscense roots, whereas in the aerial parts, the ratio of the compounds with Cd-sulfur (Cd-S) binding was increased. Together, our results imply that the strong Cd tolerance of A. yokoscense is an attribute of the following two mechanisms: Cd-O compound formation in the cell wall is a barrier to reduce Cd uptake into aerial parts. Thiol compounds in the region of root stele are involved in detoxication of Cd by formation of Cd-S compounds.
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Cadmio , Raíces de Plantas , Cadmio/toxicidad , Raíces de Plantas/efectos de los fármacos , Raíces de Plantas/metabolismo , Helechos/efectos de los fármacos , Helechos/fisiología , Helechos/metabolismo , Estrés Fisiológico , Componentes Aéreos de las Plantas/efectos de los fármacos , Componentes Aéreos de las Plantas/química , Componentes Aéreos de las Plantas/metabolismo , Hierro/metabolismo , Oryza/efectos de los fármacos , Oryza/metabolismo , Oryza/fisiología , Compuestos de Sulfhidrilo/metabolismoRESUMEN
Age-related hearing loss (ARHL) is one of the most prevalent types of sensory decline in a superaging society. Although various studies have focused on the effect of oxidative stress on the inner ear as an inducer of ARHL, there are no effective preventive approaches for ARHL. Recent studies have suggested that oxidative stress-induced DNA damage responses (oxidative DDRs) drive cochlear cell senescence and contribute to accelerated ARHL, and autophagy could function as a defense mechanism against cellular senescence in auditory cells. However, the underlying mechanism remains unclear. Sodium arsenite (NaAsO2) is a unique oxidative stress inducer associated with reactive oxygen species (ROS) that causes high-tone hearing loss similar to ARHL. Transcription factor EB (TFEB) functions as a master regulator of the autophagyâlysosome pathway (ALP), which is a potential target during aging and the pathogenesis of various age-related diseases. Here, we focused on the function of TFEB and the impact of intracellular ROS as a potential target for ARHL treatment in a NaAsO2-induced auditory premature senescence model. Our results suggested that short exposure to NaAsO2 leads to DNA damage, lysosomal damage and mitochondrial damage in auditory cells, triggering temporary signals for TFEB transport into the nucleus and, as a result, causing insufficient autophagic flux and declines in lysosomal function and biogenesis and mitochondrial quality. Then, intracellular ROS derived from damaged mitochondria play a role as a second messenger to induce premature senescence in auditory cells. These findings suggest that TFEB activation via transport into the nucleus contributes to anti-senescence activity in auditory cells and represents a new therapeutic target for ARHL. We have revealed the potential function of TFEB as a master regulator of the induction of oxidative stress-induced premature senescence and the senescence-associated secretion phenotype (SASP) in auditory cells, which regulates ALP and controls mitochondrial quality through ROS production.
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This case report describes rare concomitant allergic fungal rhinosinusitis (AFRS) and chronic granulomatous invasive fungal sinusitis (CGIFS) in a 34-year-old woman with acute lymphoblastic leukemia and graft-versus-host disease (GVHD) post bone marrow transplantation. Initially presenting with rhinorrhea and nasal obstruction, the patient was diagnosed with AFRS in the right maxillary sinus, followed by a postoperative course of CGIFS in the left nasal cavity, showcasing the unique occurrence. She was not immunocompromised during diagnosis. CGIFS may have occurred because of surgery; however, voriconazole led to significant improvement. This case highlights noninvasive and invasive fungal infections in patients with chronic rhinosinusitis and a history of GVHD and underscores the complexity of diagnosing and managing such cases.
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BACKGROUND: It is controversial whether transoral resection for early pharyngolaryngeal cancer preserves swallowing function and quality of life. We investigated swallowing function and quality of life before and after transoral videolaryngoscopic surgery (TOVS). METHODS: Seventy-three patients with pharyngolaryngeal cancer who underwent TOVS between July 2012 and July 2022 were enrolled in this prospective analysis. The Hyodo score and European Organization for Research and Treatment of Cancer Quality of Life Questionnaires were recorded preoperatively and at three, six, and 12 months postoperatively, in addition to the postoperative functional outcome swallowing scale (FOSS) at six months postoperatively. RESULTS: Although most patients could consume food orally without restrictions with a preferable FOSS score, 23 patients showed impaired Hyodo scores. Age ≥65 years significantly predicted impaired swallowing. Sub-scores of the impaired patient group showed worsening for the glottal closure reflex when the endoscope touched the epiglottis or arytenoid, as well as a reduction in the extent of pharyngeal clearance following the ingestion of blue-dyed water. CONCLUSION: After TOVS, swallowing function is generally well preserved. Elderly patients, especially those with laryngeal hypoesthesia and poor clearance, are at risk of swallowing dysfunction.
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Introduction: Vestibular migraine (VM) is a newly defined clinical condition. Several vestibular abnormalities have been reported in patients with VM. However, to date, no specific vestibular examinations are used to define VM. Therefore, the utility of vestibular examinations is limited. Currently, the role of vestibular examination has not been clearly defined. We speculated that the results of vestibular examinations could predict the prognosis of VM. We investigated the relationship between the vestibular examination results and clinical outcomes in patients with VM. Methods: This study included 25 patients with VM. Vestibular examinations, including the video head impulse test (V-HIT), cervical and ocular vestibular evoked myogenic potential (c-VEMP and o-VEMP), posturography, and several questionnaires, including the Dizziness Handicap Inventory (DHI), were conducted at the initial evaluation. Lifestyle modifications for VM and conventional pharmacological prophylactic treatments, including lomerizine, amitriptyline, and valproic acid, were performed. After 4 weeks of treatment, clinical improvements were evaluated using the Clinical Global Improvement Scale (CGI-s). The relationships among the CGI-S score, several clinical variables, and the results of several vestibular examinations were evaluated. Each patient was further classified into two subgroups according to treatment outcomes concerning vertigo and headache: CGI-S score from 0 to 2 (good response [GR]) and CGI-S score > 3 (poor response [PR]). Results: Overall, after treatment, most of the patients had improved dizziness and headache, and the CGI-s was 2.7 ± 1.3. There were 12 GRs, and 13 had PRs. Thus, neither V-HIT nor posturography predicted the prognosis. For c-VEMP, patients with GRs had significantly small AR concerning PR (19.2 ± 12.8 and 62.5 ± 42.5, respectively, [p < 0.01]). There were five normal, six unilateral, and 14 bilateral no response in 500hz o-VEMP. CGI-s of normal, unilateral, and bilateral no response was 1.4 ± 0.5, 2.8 ± 1.3, and 3.1 ± 1.2, respectively. There was a statistically significant difference between the normal and bilateral non-response o-VEMP groups (p < 0.05). Conclusion: Patients with VM had improvements in both headache and vertigo through a combination of lifestyle changes and prophylactic medications. Vestibular examinations, especially o- or c-VEMP, are beneficial for predicting the treatment outcomes of VM. The pathophysiology of VM is closely related to vestibular abnormalities, particularly the otolith-related pathways.
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Objectives: To study habituation disorders in auditory middle latency response (AMLR) to repetitive stimuli of persistent postural-perceptual dizziness (PPPD) patients. Subjects: Twenty-eight PPPD (10 men and 18 women, mean 59.5 years of age, 26-81 years of age) were enrolled. For comparison, data of 13 definite vestibular migraine (VM) patients (3 men, 10 women, mean age 45.5), 13 definite unilateral Meniere's disease (MD) patients (2 men, 11 women, mean age 50.6), and 8 healthy control (HC) subjects (2 men, 6 women, mean age 37.1) in the previous study were utilized. Methods: The electrodes were placed on the vertex and the spinal process of the fifth cervical vertebra. Clicks (0.1 msec, 70 dB nHL) were binaurally presented and averaged (800 times). Averaged responses were divided into 4 sets (S1 to S4) according to the temporal order. As peaks, Na, and Pa were identified, and relative Na-Pa amplitudes in S2-S4 to S1 were analyzed. Results: The mean relative amplitude of PPPD patients showed lack of habituation (potentiation) as shown in VM patients, although the extent of potentiation was weaker than VM. Comparison of relative S4 amplitudes showed significant differences among the 4 groups (p = 0.0013 one-way ANOVA), Multiple comparison revealed significant differences between PPPD and MD (p = 0.0337 Dunnet's test). Conclusion: PPPD patients showed lack of habituation (potentiation) of Na-Pa amplitude in AMLR to repetitive stimuli. Lack of habituation (potentiation) might be associated with sensory processing disorders in PPPD.
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OBJECTIVE: Previously, we used a nasal cavity model to analyze the intranasal airflow dynamics and numerically calculate the nasal resistance value. In this study, We attempted clarify the parameters influencing nasal resistance by newly developed computer model. METHODS: The computer simulation model was developed from the structures of nasal airway tract adopted from 1.0-mm slice computed tomography (CT) obtained from the 2 of the healthy volunteers. (model 1: the one at 35-year-old man, model 2: 25-year-old man.) We have calculated the nasal resistance by computer simulation calculations of both model 1 and model 2. These calculated values were compared with the values obtained from the established method of rhinomanometry. For the simulation, Fluent 17.2® (ANSYS, American) was employed for f luid a nalysis u sing the continuity equation for 3D incompressible flow and the Navies-Stokes equation for the basic equations. Both models were laminar models. The SIMPLE calculation method using the finite volume method was employed here, and the quadratic precision upwind difference method was used to discretize the convection terms. RESULTS: The measured (simulation) values in Model 1 were 0.69 (0.48), 1.10 (0.41), and 0.42 (0.22) Pa/cm3/s on the right, left, and both sides, whereas those in Model 2 were 0.72 (0.21), 0.32 (0.09), and 0.22 (0.06) Pa/cm3/s, respectively. CONCLUSION: Our results suggest that nasal resistance is possibly affected by the length of the inferior turbinate and the cross-sectional area of the choana and nasopharynx. Further experiments using additional nasal cavity and paranasal sinus models are warranted.
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Cavidad Nasal , Senos Paranasales , Masculino , Humanos , Adulto , Cavidad Nasal/diagnóstico por imagen , Simulación por Computador , Senos Paranasales/diagnóstico por imagen , Tomografía Computarizada por Rayos X , RinomanometríaRESUMEN
Magnetic resonance imaging (MRI) is a more useful diagnostic modality for detecting paranasal tumors compared with computed tomography (CT). We encountered a case of malignant lymphoma of the maxillary sinus. Although CT findings suggested malignancy, MRI findings indicated an inflammatory disease. The patient was a 51-year-old man with a chief complaint of right maxillary toothache. Edema in the right middle meatus and bloody rhinorrhea were observed. CT revealed r ight maxillary s inus shadow with partial bone loss, suggesting malignancy. However, MRI performed two weeks later showed an internal homogeneous lesion with neither contrast effect, nor invasion outside the maxillary sinus. The patient also had no fever, weight loss, or night sweats. Additionally, no palpable cervical lymphadenopathy was observed. Endoscopic sinus surgery was performed to confirm the diagnosis. Upon opening the maxillary sinus, highly viscous retention and a large amount of yellowish-white debris were observed. Allergic fungal rhinosinusitis was suspected. However, histopathological analysis of the debris established a diagnosis of malignant lymphoma. The debris exhibited pathological findings of necrosis. The patient remained in remission after undergoing radiochemotherapy. Malignant lymphomas of the paranasal sinuses, which have a minimal tendency for invasion but with considerable predominance of necrosis, may be diagnosed as an inflammatory disease, based on MRI findings. In cases in which a thorough physical examination could not rule out malignant lymphomas, an endoscopic biopsy should be immediately considered.
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Linfoma , Masculino , Humanos , Persona de Mediana Edad , Linfoma/diagnóstico por imagen , Linfoma/patología , Seno Maxilar/patología , Seno Maxilar/cirugía , Tomografía Computarizada por Rayos X , Imagen por Resonancia Magnética , NecrosisRESUMEN
Gardner syndrome with odontogenic sinusitis is rare but should be suspected in patients with multiple osteomas of the skull and facial bones, excess teeth, impacted teeth, and odontomas. Early diagnosis and treatment of GS may improve prognosis.
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The usefulness of nasal irrigation for chronic rhinosinusitis is recognized, and it is widely used as a topical treatment after endoscopic sinus surgery (ESS). Generally, there are few complications due to nasal irrigation, and it is recognized as a highly safe treatment. There are no reports of nasal septal perforation due to nasal irrigation. The objective of this study was to describe three cases of nasal septal perforation occurring during self-nasal irrigation after ESS. Case patient 1 was a 38-year-old woman who was admitted to our hospital with a complaint of nasal obstruction. Based on a diagnosis of chronic rhinosinusitis and allergic rhinitis, we performed bilateral ESS and bilateral inferior turbinectomy. At discharge, we instructed the patient in how to perform self-nasal irrigation twice a day at home. At the time of the third visit after discharge, a black crust had adhered to the bilateral anterior nasal septum. Crust formation continued at the same site, and nasal septal perforation was seen two and a half months after the operation. The other two cases showed similar courses. Postoperative nasal irrigation can cause nasal septal perforation. We need to educate patients on proper nasal irrigation and regularly check the nasal septum.
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Perforación del Tabique Nasal , Rinitis , Sinusitis , Adulto , Femenino , Humanos , Lavado Nasal (Proceso) , Perforación del Tabique Nasal/etiología , Perforación del Tabique Nasal/cirugía , Tabique Nasal/cirugía , Rinitis/terapia , Sinusitis/cirugíaRESUMEN
Most maxillary sinus papillomas are confirmed when they have extended beyond the nasal cavity and are rarely found while localized in the maxillary sinus. We experienced two cases of localized papilloma in the maxillary sinus. Case 1 was a 69-year-old man with a localized left maxillary sinus lesion detected during a routine imaging examination. As the lesion was likely to be papilloma, we recommended that the patient undergo diagnostic surgery, which he refused. He experienced bloody rhinorrhea 1 year and 9 months after the first visit, and computed tomography (CT) showed increased lesions and bone destruction. Histological examinations revealed squamous cell carcinoma ex inverted papilloma. He died 5 years after the first visit. Case 2 was a 46-year-old woman in whom positron emission tomography/CT showed a localized right maxillary sinus lesion. Tissue biopsy results indicated oncocytic papilloma. Endoscopic resection was performed later. On an imaging examination, sinonasal papilloma was determined accidentally to be a localized lesion of the maxillary sinus. A detailed interpretation of the CT scan was useful in estimating sinonasal papilloma. Tissue biopsy or diagnostic surgery should be performed when sinonasal papilloma is suspected during appropriate image evaluation.
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Hallazgos Incidentales , Neoplasias del Seno Maxilar/diagnóstico por imagen , Neoplasias del Seno Maxilar/cirugía , Papiloma/diagnóstico por imagen , Papiloma/cirugía , Anciano , Biopsia , Carcinoma de Células Escamosas/patología , Transformación Celular Neoplásica/patología , Resultado Fatal , Femenino , Humanos , Masculino , Neoplasias del Seno Maxilar/patología , Persona de Mediana Edad , Papiloma/patología , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Negativa del Paciente al TratamientoRESUMEN
Chemical burns of the paranasal sinus are rare; therefore, assessment methods for treatment of and prognoses for the exposure site are unknown. We experienced a case in which a hydrochloric acid burn of the paranasal sinuses caused irreversible tissue damage. Computed tomography is useful for identifying the exposure site and assessing tissue damage over time. Identification of the exposure site and proactive washing are recommended for patients with chemical burns of the paranasal sinuses. Laryngoscope, 131:E2490-E2493, 2021.
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Quemaduras Químicas/diagnóstico por imagen , Quemaduras Químicas/terapia , Senos Paranasales/diagnóstico por imagen , Senos Paranasales/lesiones , Tomografía Computarizada por Rayos X , Accidentes por Caídas , Endoscopía , Humanos , Ácido Clorhídrico , Masculino , Persona de Mediana EdadRESUMEN
Mycobacterium marinum is a free-living nontuberculous mycobacterium that is widely distributed in freshwater and seawater around the world. Granulomatous skin infection from M. marinum in people who are exposed to fish or aquatic environments is a rare condition known as fish tank granuloma. The granuloma mainly occurs on the skin of the upper limb, in a few cases on the face, and rarely in the nasal cavity. We describe a case of M. marinum infection that presented as a nasal cavity mass. A 57-year-old woman who was receiving infliximab for psoriatic arthritis visited our hospital with a complaint of right nasal obstruction. A granulomatous mass with an irregular surface was found in the anterior part of the right nasal cavity. Tissue biopsy revealed granulation tissue. Since the application of steroid ointment did not reduce the size of the mass, the tumor was resected under local anesthesia, and the base was cauterized. The pathological finding was an inflammatory granuloma with negative Ziehl-Neelsen staining. The granuloma recurred 3 months after resection. The interferon-gamma release assay (IGRA) test was positive, and therefore, a mycobacterial tissue culture test was performed because of suspected nasal tuberculosis, which identified M. marinum. The nasal cavity mass disappeared 2 months after the administration of minocycline, followed by clarithromycin, and subsequent discontinuation of infliximab. M. marinum infection can cause an intranasal mass. IGRA and the mycobacterial tissue culture test are useful for diagnosis. As in this case, the nasal lesion may be excised as an inflammatory nasal granuloma, and therefore, there may be many more "hidden" cases of M. marinum infection. If nasal granulation is present, the possibility of M. marinum infection should be considered.
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In Japan, there have been no examinations of tinnitus with respect to personality traits, and only a few studies have investigated sleep disorders in tinnitus. Understanding the association between these can aid in selecting and developing effective treatment options for patients with tinnitus. This study aimed to clarify the relationship between tinnitus severity, personality, and sleep disorders, in patients with chronic tinnitus in Japan. We retrospectively evaluated the personality factors of 56 patients presenting with tinnitus using the NEO Five-Factor Inventory (NEO-FFI), which examines five personality characteristics, including Neuroticism, Extraversion, Openness, Agreeableness, and Conscientiousness. We evaluated tinnitus severity, sleep disorders, depression, and anxiety using the Tinnitus Handicap Inventory, Pittsburgh Sleep Quality Index (PSQI), Self-Rating Depression Scale, and State-Trait Anxiety Inventory, respectively. Neuroticism and tinnitus severity were positively correlated (r = 0.548, p < 0.001). Sleep disorders were observed in 73.2% of patients; however, there was no correlation between the PSQI score and tinnitus severity or between PSQI score and scores of the five NEO-FFI items. Sleep disorders are frequently experienced by patients with chronic tinnitus, regardless of tinnitus severity or personality. Understanding the relationship between personality and tinnitus severity could lead to better management of tinnitus.
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Personalidad , Trastornos del Sueño-Vigilia/complicaciones , Trastornos del Sueño-Vigilia/fisiopatología , Acúfeno/complicaciones , Acúfeno/psicología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Japón , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Trastornos del Sueño-Vigilia/diagnóstico , Acúfeno/fisiopatología , Adulto JovenRESUMEN
OBJECTIVE: This study aimed to investigate the association among swallowing function, hand grip strength, and peak expiratory flow in elderly patients with dysphagia. METHODS: A total of 35 patients (26 men and 9 women, 80.7 ± 8.1 years) were included in the final analysis. They underwent videoendoscopy (VE) for the assessment of swallowing function, hand grip strength test, and peak expiratory flow test. We used the Hyodo score, the objective scale of VE developed by Hyodo et al. (2010), which consists of the following 4 parameters: salivary pooling degree at the vallecula and pyriform sinuses, glottal closure reflex induced by touching of the endoscope to the epiglottis or arytenoid, swallowing reflex initiation assessed by "white-out" timing, and pharyngeal clearance after blue-dyed water is swallowed. RESULTS: The Hyodo score significantly correlated to hand grip strength and peak expiratory flow, and hand grip strength was significantly correlated to peak expiratory flow. Although a significant correlation was noted between the Hyodo score and hand grip strength after adjustment for peak expiratory flow, no significant correlation was found between the Hyodo score and peak expiratory flow after adjustment for hand grip strength. CONCLUSIONS: This study indicated that unlike peak expiratory flow, hand grip strength is associated with swallowing function, which was evaluated by an objective scale, and can be used as an indicator for whole body muscle strength. This result may contribute to the investigation of the effect of muscle training on dysphagia.
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Trastornos de Deglución/fisiopatología , Fuerza de la Mano/fisiología , Ápice del Flujo Espiratorio , Anciano , Anciano de 80 o más Años , Comorbilidad , Deglución/fisiología , Femenino , Anciano Frágil , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Athyrium yokoscense shows strong tolerance to cadmium exposure, even at levels that are many times greater than the toxic levels in ordinary plants. To determine the mechanism of Cd tolerance in A. yokoscense, we grew these plants under high Cd conditions and observed the tissue-specific accumulation of Cd and generation of reactive oxygen species, which is one of the major physiological responses to Cd stress. Fuchsin staining indicated the existence of a casparian strip in A. yokoscense roots, which may participate in Cd hypertolerance in A. yokoscense. Moreover, we performed RNA-seq of RNA samples from A. yokoscense plants treated with or without Cd exposure and obtained comprehensive RNA sequences as well as the Cd-responsive expression patterns of individual genes. Through de novo transcriptome assembly and gene expression analysis, we found that A. yokoscense showed normal features with no significant change in the expression levels of any transporter genes, even under high Cd exposure conditions. Our results demonstrate that A. yokoscense has an unusual mechanism that allows the invading Cd to partition into the distal roots, thus avoiding translocation of Cd into the xylem.
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Cadmio/toxicidad , Resistencia a Medicamentos , Helechos/genética , Transcriptoma , Helechos/efectos de los fármacos , Proteínas de Transporte de Membrana/genética , Proteínas de Transporte de Membrana/metabolismo , Proteínas de Plantas/genética , Proteínas de Plantas/metabolismo , Estrés FisiológicoRESUMEN
Objectives: To compare habituation in auditory middle latency response (AMLR) to repetitive stimuli of vestibular migraine (VM) patients with Meniere's disease (MD) patients and healthy controls (HC) and to assess usefulness of AMLR for diagnosis of VM. Subjects: Thirteen unilateral definite MD patients (2 men, 11 women, mean age 50.6), 13 definite VM patients (3 men, 10 women, mean age 45.5), and 8 HC subjects (2 men, 6 women, mean age 37.1) were enrolled. Methods: The electrodes were placed on the vertex and the spinal process of the fifth cervical vertebra. Binaural click stimulation (0.1 ms, 70 dBnHL) was presented. A total of 800 responses were averaged. Averaged responses were divided into four sets (S1 to S4) according to the temporal order. No, Po, Na, and Pa were identified, and amplitudes and latencies were measured. Results: Concerning latencies, HC subjects showed a tendency of shorter latencies. However, there was no clear effect of repetitive stimulation. Concerning No-Po amplitudes, no significant differences were observed. Raw amplitudes of Na-Pa showed statistically significant differences in S1 and S2 among the groups (p < 0.01 one-way ANOVA). Differences were shown in MD vs. VM and HC vs. VM in S1 (smaller in VM) (p < 0.01 Bonferroni's test) and in MD vs. VM in S2 (smaller in VM) (p < 0.01 Bonferroni test). Relative amplitudes of Na-Pa to S1 showed statistically significant differences in S4 (p < 0.01 one-way ANOVA). Differences were shown in MD vs. VM and HC vs. VM (larger in VM) (p < 0.01 Bonferroni's test). Differences of Na-Pa amplitudes in S2 to S4 from Na-Pa amplitude in S1 were significant in S4 of VM patients (Dunnett's test). Conclusions: VM patients showed lack of habituation (potentiation) of Na-Pa amplitude in AMLR to repetitive stimuli while MD patients and HC subjects showed habituation. Observation of lack of habituation has high diagnostic accuracy for differential diagnosis of VM from MD.
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HYPOTHESIS: The phenotype of DFNA11 consists of specific features at diverse developmental and age stages. BACKGROUND: Only eight mutations have been identified for autosomal dominant non-syndromic hearing loss related to MYO7A (DFNA11), and the onset and progression of DFNA11 are poorly understood. METHODS: After linkage analysis and following Sanger sequencing in a family suspected to have autosomal dominant hereditary hearing loss, we analyzed the audiometric and vestibular functions and their long-term changes in the subjects carrying the variant. RESULTS: A reported variant of uncertain significance, NP_000251.3:p.Arg853His, in MYO7A was detected and cosegregation data of this large family provided evidence that the variant was likely pathogenic for DFNA11. Family members with the variant had no other symptoms associated with hearing loss and were confirmed to have autosomal dominant non-syndromic sensorineural hearing loss. Audiograms tended to show gently sloping configuration in childhood and flat configuration after the age of 30 years. Hearing loss at high frequencies progressed slowly, while hearing at low frequencies started to deteriorate later but progressed more rapidly. Some subjects showed partly abnormal results in the distortion products of otoacoustic emissions before the elevation of hearing thresholds. Vestibular function was within the normal range in all the subjects tested. CONCLUSION: We revealed that hearing loss at high frequencies was mainly noted in early developmental stages and that thresholds increased more rapidly in the low frequency range, resulting in changes in audiometric configuration. Deterioration of distortion product otoacoustic emissions (DPOAE) before the elevation of hearing thresholds was considered as a clinical feature of DFNA11.
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Pérdida Auditiva Sensorineural , Miosina VIIa/genética , Adulto , Envejecimiento , Niño , Pérdida Auditiva Sensorineural/diagnóstico , Pérdida Auditiva Sensorineural/genética , Humanos , Miosinas/genética , Emisiones Otoacústicas Espontáneas , LinajeRESUMEN
Peach (Prunus persica (L.) Batsch) leaves are sensitive to copper (Cu) exposure. The symptoms of Cu exposure are similar to those of bacterial spot disease; however, the mechanism underlying lesion formation caused by Cu exposure is not clear. Here, we investigated whether lesion formation caused by Cu exposure was related to the mechanism underlying plant resistance to microbial pathogens. When Cu was applied to the centre of a pinhole on peach leaves, a two-step process was observed. A pale green section in the shape of a doughnut, located far from a Cu treatment point, first appeared on a leaf treated with 2 mM CuSO4. Next, a yellow-white section gradually spread from the Cu treatment point to the pale green section. Finally, a gap was formed in the middle of the pale green section. The inner part of the pale green section contained 96% of the Cu applied, indicating that Cu is retained in the lesion area. Real-time PCR analysis of the expression of genes encoding pathogenesis-related proteins and enzymes involved in phytoalexin synthesis revealed that three genes (encoding chitinase, pathogenesis-related protein 4, and ß-1,3-glucanase-3) of the eight tested were upregulated by Cu treatment. Furthermore, treatment with caspase-1 inhibitors reduced lesion formation. These results show that Cu treatment of peach leaves causes cell death similar to that occurring during the biotic stress response.
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The oropharynx is examined with a light source such as an electric light, a penlight, or a forehead mirror based on an acquired visual field using a tongue depressor. However, it is extremely difficult to obtain objective and reproducible images of tissue within the pharynx required in recent years with these methods, and insufficient progress in the examination tools has been made. There is an increasing need to develop a method for display during oropharyngeal examination. We conducted the present study to develop a novel oropharyngeal endoscope as an objective observation method.