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Deafblindness is a condition of combined vision and hearing loss that is extremely rare in children and young adults, as well as being a highly heterogeneous condition, with over 70 specific etiologies. Due to these features, sporadic clinical experiences have not been collated, which has hampered medical progress. Genetics plays a major role in the pathogenesis of deafblindness in children and young adults, with more than 50 hereditary syndromes and disorders associated with the condition, including CHARGE, Usher, Down, Stickler, and Dandy-Walker syndromes, which are the most common. Clinical diagnosis of deafblindness is often difficult, and a significant proportion of patients are undiagnosed. No curative therapy is currently available for the majority of patients with hereditary deafblindness; however, experimental studies using animal models have shown promising results by targeting specific genes that cause vision or hearing loss. In Japan, the Rare Disease Data Registry of Japan (RADDAR-J) has been established as a national registry of rare and intractable diseases. Diseases of deafblindness have been elected as a disease category in RADDAR-J. Currently, clinical and genomic data are being collected and analyzed using this system, with the aim of generating an overview of deafblindness to improve medical practice.
Nasal irrigation is an important step of functional endoscopic sinus surgery (FESS) postoperative care. This study was performed to compare the effects of diluted baby shampoo (BS) and normal saline solution (NSS) irrigation on patients' quality of life (QoL) and surgical outcomes after FESS.
This study included 77 patients who underwent FESS to treat chronic rhinosinusitis with nasal polyps. Lund-Mackay score, Lund-Kennedy endoscopic score (LKES), synechia score and QoL (using the Sinonasal Outcome Test (SNOT-22)) were evaluated.
LKES was significantly better in the BS group (p=0.001), especially in terms of nasal discharge and crust formation (p=0.024 and p=0.030, respectively) at 1 month postoperatively. However, no significant difference was found at 3, 6 or 12 months postoperatively (p=0.833, p=0.263, and p=0.346, respectively). The reduction of SNOT-22 score (between preoperative assessment and 1 month postoperatively) was significantly better in the BS than in the NSS group (p=0.025). However, no statistically significant differences were found between groups at 3, 6, or 12 months postoperatively (p=0.312, p=0.280, and p=0.285, respectively). In the evaluation of SNOT-22 subdomains, changes in psychological, rhinological and extranasal rhinological subdomains were significantly better in the BS group at 1 month postoperatively (p=0.019, p=0.010 and p=0.002, respectively).
Compared to irrigation with NSS, BS usage following FESS led to reductions of crusting, nasal discharge and synechia formation; moreover, it was associated with improved SNOT-22 scores, especially in psychological, rhinological and extranasal rhinological subdomains.
Compared to irrigation with NSS, BS usage following FESS led to reductions of crusting, nasal discharge and synechia formation; moreover, it was associated with improved SNOT-22 scores, especially in psychological, rhinological and extranasal rhinological subdomains.
Current interventions of dysphagia are not generalizable, and treatments are commonly used in combination. We conducted a questionnaire survey on nurses and speech therapists regarding dysphagia rehabilitation to understand the current situation in Japan.
The questionnaire was sent to 616 certified nurses in dysphasia nursing and 254 certified speech-language-hearing therapists for dysphagia. Based on "Summaries of training methods in 2014" by JSDR, 24 local indirect exercises, 11 general indirect exercises, and 13 direct exercises were selected. The Likert scale "How do you feel about each method" was used as follows A; Frequency, B; Ease, C; Adherence, D; Effectiveness (1-5))?".
Two hundred fifty (40%) nurses and 145 (57%) speech-language-hearing therapists (ST) responded to the questionnaire. The direct exercise was associated with a significantly high score in every question. In indirect exercises, "Cervical range of motion exercise," "Orofacial myofunctional exercise," "Lip closure exercise." "Ice massage of pharynx" and "Huffing" were used relatively frequently. selleck chemicals "Balloon dilatation therapy" and "Tube exercise" was associated with a relatively high discrepancy for two questions. Frequency" and the sum of "Ease," "Adherence," and "Effectiveness." was significantly correlated for local indirect exercises (r2=0.928, P < 0.01), general indirect exercises (r2=0.987, P < 0.01), and direct exercises (r2=0.996, P < 0.01) (Fig. 5).
This study examined the current situation of dysphagia rehabilitation in Japan. Our results aid to increase understanding and selection of rehabilitative treatments for dysphagia patients in Japan.
This study examined the current situation of dysphagia rehabilitation in Japan. Our results aid to increase understanding and selection of rehabilitative treatments for dysphagia patients in Japan.Vertical partial laryngectomy is a well-established surgical procedure for early glottic cancers with acceptable functional and oncological outcomes. However, on a long-term basis, aspiration might be a serious problem with aging. Here we presented two cases of refractory aspiration pneumonia after vertical laryngectomy. Case 1 A 76-year old gentleman with a past history of malignant lymphoma treated by chemotherapy and radiotherapy had glottic cancer, which was treated by repeated vertical partial laryngectomies. Although glottic caner had been well controlled, he started to suffer from refractory aspiration pneumonia. Since his cervical skin was very thin and hard and his general condition was poor, we employed modified Kano's method for glottic closure. Case 2 A 87-year old Japanese male had a past history of glottic cancer treated by radiotherapy and vertical partial laryngectomy. He was repeatedly hospitalized for severe aspiration pneumonia. At the age of 87, he had second primary oropharyngeal cancer. Kano's method was simultaneously performed at the time of resection of oropharyngeal cancer.
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