Αρχειοθήκη ιστολογίου

Αλέξανδρος Γ. Σφακιανάκης
ΩτοΡινοΛαρυγγολόγος
Αναπαύσεως 5
Άγιος Νικόλαος Κρήτη 72100
2841026182
6032607174

Παρασκευή 15 Δεκεμβρίου 2017

Comparison of TIVA with different combinations of ketamine–propofol mixtures in pediatric patients

Abstract

Purpose

Adding ketamine to propofol has been suggested to be useful for sedation and general anesthesia. This study aimed to determine the effect of TIVA with different ratios of ketofol on recovery in children.

Methods

Seventy-five children aged 3–12 years and undergoing adenoidectomy and/or tonsillectomy surgery were randomized into three groups. Ratios of 1:5, 1:6.7 and 1:10 ketamine–propofol mixture (ketofol) were prepared in the same syringe for groups I, II and III, respectively. Induction and maintenance of anesthesia were performed with 1:5, 1:6.7 and 1:10 ratios of ketofol in groups I, II and III, respectively. A McFarlan infusion dose regimen was used (15 mg/kg/h for 15 min, 13 mg/kg/h for 15 min, 11 mg/kg/h for 30 min) and infusion rates were decreased for the different ratios. Infusion rates were reduced to 67, 80 and 90% of the McFarlan dose regimen for groups I, II and III, respectively. Extubating time, length of stay in PACU, postoperative PAED and FLACC scores were recorded.

Results

Extubating time was significantly lower [mean 254.3 ± 92.7 s (95% CI 216.6–292.6, p = 0.001)] in group III than in groups I and II [371.3 ± 153 s (308.1–434.48) and 343.2 ± 123.7 s (292.2–394.3), respectively]. Length of stay in the PACU was lower in group III [median 15 min (interquartile range 15–20, p = 0.001)] than in groups I and II: 20 (15–27.5) and 20 min (20–27.5), respectively.

Conclusion

TIVA with a 1:10 ratio of ketofol admixture with a 90% reduction of McFarlan regimen can provide improved recovery conditions.

Trial registration

ClinicalTrials.gov identifier: NCT02848963.



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Prevalence of True Median Cleft of Upper Lip as Reflected from a Small Central Indian Population: Attempt to Report and Review the True Median Cleft of Upper Lip

Abstract

Aims and Objectives

The median clefts of the facial skeleton and soft tissue are least in occurrence. Here we have attempted to analyze and review the prevalence of median clefts in our center.

Materials and Methods

All the patients who reported with congenital deformities from January 2013 to June 2016 were included in the study. A detailed list of patients was then re-evaluated to preclude duplication of the record which could have occurred during the admission process. All the characteristics which can be evaluated epidemiologically were individually tabulated and evaluated in detail.

Results

Out of the 1184 patients included in the study only 2 were affected with median cleft. The prevalence of true median cleft was hence determined to be 0.17% among the cleft population.

Conclusion

The prevalence of the true median cleft lip is rare and hence makes it difficult to classify these clefts, and also the treatment protocol needs to be fabricated for the treatment of such clefts.



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Human c-SRC kinase (CSK) overexpression makes T cells dummy

Abstract

Adoptive cell therapy with T-cell receptor (TCR)-engineered T cells represents a powerful method to redirect the immune system against tumours. However, although TCR recognition is restricted to a specific peptide–MHC (pMHC) complex, increasing numbers of reports have shown cross-reactivity and off-target effects with severe consequences for the patients. This demands further development of strategies to validate TCR safety prior to clinical use. We reasoned that the desired TCR signalling depends on correct pMHC recognition on the outside and a restricted clustering on the inside of the cell. Since the majority of the adverse events are due to TCR recognition of the wrong target, we tested if blocking the signalling would affect the binding. By over-expressing the c-SRC kinase (CSK), a negative regulator of LCK, in redirected T cells, we showed that peripheral blood T cells inhibited anti-CD3/anti-CD28-induced phosphorylation of ERK, whereas TCR proximal signalling was not affected. Similarly, overexpression of CSK together with a therapeutic TCR prevented pMHC-induced ERK phosphorylation. Downstream effector functions were also almost completely blocked, including pMHC-induced IL-2 release, degranulation and, most importantly, target cell killing. The lack of effector functions contrasted with the unaffected TCR expression, pMHC recognition, and membrane exchange activity (trogocytosis). Therefore, co-expression of CSK with a therapeutic TCR did not compromise target recognition and binding, but rendered T cells incapable of executing their effector functions. Consequently, we named these redirected T cells "dummy T cells" and propose to use them for safety validation of new TCRs prior to therapy.



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Factors impacting hearing aid performance outcomes for Egyptian hearing impaired children

Publication date: Available online 15 December 2017
Source:Egyptian Journal of Ear, Nose, Throat and Allied Sciences
Author(s): Eman Abdel-Fattah Said
Despite recent technological advances in the audiologic assessment of children, their hearing aid fitting remains a daunting task.Aims of studyTo assess effectiveness of amplification in children using aided sound field tests and Auditory Behavior in Everyday Life (ABEL) questionnaire and to elucidate factors contributing to poor outcomes.MethodsDetailed medical history, otoscopic examination, basic audiological evaluation, aided sound field tests and ABEL questionnaire for 114 hearing impaired children aged 4–16 years.ResultsCongenital HL considered the commonest cause of HL (55.3%), 36% had unknown cause and 8.8% of HI had acquired cause. Profound loss in 67.5%, severe in 17% and 54.4% of them (54.4%) were fitted around the age of 3 years. Binaural HA in 88.6% and digital type for 61.4%. There were statistically significant differences between unaided and aided values in sound field tests for HI children.Poor performance in direct measures and ABEl in children with congenital and profound degree of hearing loss, better response when they were fitted earlier with digital aids. ABEL scores showed negative correlations with aided tonal sound field test and positive with aided speech discrimination score.ConclusionsInappropriate amplification, late age of fitting with no speech therapy were clinical red flags for poor outcomes. ABEL questionnaire was a valid procedure to assess the hearing aids appropriateness.



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Childhood hearing loss is a key feature of CAPOS syndrome: A case report

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Stéphanie Paquay, Elsa Wiame, Naima Deggouj, Antonella Boschi, Romolo Daniele De Siati, Yves Sznajer, Marie-Cécile Nassogne
CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is a rare neurological disorder, recently associated with the c.2452G > A hotspot mutation in the ATP1A3 gene, with sensorineural hearing loss as a prominent feature. We herein report on a girl who has experienced hearing loss for three years following an initial encephalitic episode when aged 15 months old. CAPOS was diagnosed only when she was six years old by targeted testing whilst she displayed optic atrophy, cerebellar signs and areflexia. CAPOS syndrome should be considered in the differential diagnosis of acquired childhood deafness, prompting clinicians to search for associated neurological features.



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Genetic analysis of CLDN14 in the Chinese population affected with non-syndromic hearing loss

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Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Yajie Lu, Jun Yao, Qinjun Wei, Jin Xu, Guangqian Xing, Xin Cao
ObjectiveThe CLDN14 gene, encoding the tight junction protein Claudin-14, has been proposed as a candidate causative gene affecting autosomal recessive non-syndromic hearing loss (ARNSHL). Genetic analysis of nonsynonymous single-nucleotide variations (nsSNVs) in CLDN14 has been performed in different populations. The role of CLDN14 nsSNVs in contributing to hearing loss in Chinese populations would be investigated in this study.MethodsTarget screening for CLDN14 variations were conducted in 500 unrelated patients diagnosed with non-syndromic hearing loss (NSHL).ResultsNo reported pathogenic CLDN14 nsSNVs in heterozygote or homozygote were detected in this study, however, we identified 4 heterozygous nsSNVs [c.11C > T, p.(Thr4Met); c.16G > A, p.(Val6Met); c.68T > C, p.(Ile23Thr); c.367A > C, p.(Thr123Pro)] in CLDN14. The 4 nsSNVs are located at claudin-14 transmembrane domains, but assessed to be poorly conservative and non-pathogenic via multiple in silico algorithms. The structure-based analysis also suggested that the 4 nsSNVs had less structural and functional impact on claudin-14.ConclusionOur findings indicated that CLDN14 might not be a major causative gene for NSHL in Chinese populations, which would contribute to fully understanding the genetic cause of NSHL in the East Asian populations.



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SLC52A2 mutations cause SCABD2 phenotype: A second report

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Mojgan Babanejad, Omid Ali Adeli, Nooshin Nikzat, Maryam Beheshtian, Hakimeh Azarafra, Farnaz Sadeghnia, Marzieh Mohseni, Hossein Najmabadi, Kimia Kahrizi
IntroductionAutosomal recessive cerebellar ataxias (ARCAs) are a large group of neurodegenerative disorders that manifest mainly in children and young adults. Most ARCAs are heterogeneous with respect to age at onset, severity of disease progression, and frequency of extracerebellar and systemic signs.MethodsThe phenotype of a consanguineous Iranian family was characterized using clinical testing and pedigree analysis. Whole-exome sequencing was used to identify the disease-causing gene in this family.Results and conclusionUsing whole exome sequencing (WES), a novel missense mutation in SLC52A2 gene is reported in a consanguineous Iranian family with progressive severe hearing loss, optic atrophy and ataxia. This is the second report of the genotype-phenotype correlation between this syndrome named spinocerebellar ataxia with blindness and deafness type 2 (SCABD2) and SLC52A2 gene.



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Pediatric Meniere's disease

Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Chi Wang, Chien-Hao Wu, Po-Wen Cheng, Yi-Ho Young
ObjectiveThis study adopted an inner ear test battery comprising audiometry, and ocular vestibular evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests to investigate the sequence of inner ear deficits in pediatric Meniere's disease (MD).MethodsFrom 2005 to 2016, a total of 24 MD children aged <15 years old underwent otoscopy, CT scan, blood test, and an inner ear test battery. Nine subjects were males and 15 were females, with mean age of 12 years. Right ear was affected in 3 patients, left ear in 5 patients, and both ears in 16 patients (67%). Eight (33%) of the 24 MD children had positive family history.ResultsInner ear deficits in MD children ran from abnormal hearing (25%) to abnormal cVEMP (20%), oVEMP (13%), and caloric (12%) tests, exhibiting a significantly declining sequence from the cochlea, to the saccule, utricle and semicircular canals. Analysis between the laterality and family history revealed a significantly positive correlation.ConclusionPediatric MD is rare, accounting for 2.3% prevalence in MD patients. The inner ear deficits in pediatric MD showed a significantly declining sequence from the cochlea, to the saccule, utricle and semicircular canals, mimicking the sequence in adult MD. One-third MD children had positive family history, which may explain the high rate of bilateral affliction and symmetrical hearing levels on both ears.



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Papillary thyroid carcinoma presenting as acute suppurative thyroiditis: A case report and review of the literature

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Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Shabana Kalladi Puthanpurayil, Gary L. Francis, Adele O. Kraft, Uma Prasad, Rajanya S. Petersson
Acute suppurative thyroiditis is a rare, potentially life-threatening condition. We report the case of a 17-year-old male who initially presented with a thyroid abscess. Due to persistent symptoms and lack of evidence for underlying predisposing factors, he was followed closely and subsequently diagnosed with papillary thyroid cancer. He was successfully managed with surgery. His clinical course, radiological evaluation, and pathology reports are presented here along with a review of the literature. This case of papillary thyroid cancer highlights the need for close follow-up of patients presenting with a thyroid abscess, when other predisposing risk factors are not evident.



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A novel technique for unilateral supraglottoplasty

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Harry H. Ching, Alycia G. Spinner, Nathaniel H. Reeve, T.J. O-Lee
ObjectivesTraditional supraglottoplasty for pediatric laryngomalacia is most commonly conducted with either CO2 laser or cold steel instruments. While the procedure enjoys high success rates, serious complications such as excessive bleeding, supraglottic stenosis and aspiration can occur. Unilateral coblation supraglottoplasty may reduce this risk, but data on respiratory and swallowing outcomes are lacking. This study reports our experiences with unilateral coblation supraglottoplasty.MethodsPediatric patients with severe congenital laryngomalacia who underwent unilateral supraglottoplasty at a single institution from 2013 to 2016 were retrospectively reviewed. Bipolar radiofrequency ablation (Coblation) was utilized with partial arytenoidectomy, aryepiglottoplasty, and advancement of mucosal flaps. Outcome measures included apnea-hypopnea index (AHI), weight-by-age percentile, and decannulation rate.ResultsTwelve patients were included with an average age of 13.1 months (range 2–28 months). In patients without tracheostomy, 88% had complete resolution of respiratory symptoms, while the remainder had significant improvement. In patients without gastrostomy tubes, there was an average increase in weight-age percentile of 6.1, 7.8, and 15.3 points at 1, 3, and 6 months postoperatively, respectively. Three patients had complete polysomnography data with a mean preoperative AHI of 19.3 and postoperative AHI of 4.0. Three of four patients with tracheostomy have been decannulated at a mean follow-up of 1.5 years. There were no early or late postoperative complications and no revision supraglottoplasty.ConclusionUnilateral supraglottoplasty with bipolar radiofrequency ablation can improve respiratory symptoms and decrease OSA severity in severe congenital laryngomalacia. This technique is safe and can lead to substantial improvement in AHI in patients with OSA.



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Total serum bilirubin levels and sensorineural hearing loss in the US adolescents: NHANES 2007–2010

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Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Guoli Zhou, Wenjiang Fu
ObjectiveWe aimed to investigate whether current levels of total serum bilirubin are associated with different subtypes of sensorineural hearing loss (SNHL) in adolescents.MethodsA set of cross-sectional data from the National Health and Nutrition Examination Survey (NHANES) (2007–2010) was used. A subset of 1404 adolescents was sampled for measurements of total serum bilirubin, tympanometry, and average pure tone threshold at low-frequencies (LPTA: 500, 1000, 2000 Hz) or high-frequencies (HPTA: 3000, 4000, 6000, and 8000 Hz). SNHL was defined as the hearing loss that had type A tympanograms with a peak admittance of 0.3 ml or greater. Associations between serum bilirubin (square-root transformed) and different subtypes of SNHL were evaluated using binary or multinomial logistic regression models with 4-year sampling weights. The bootstrap method was used for estimation of variance and 10-fold cross-validation for assessment of overfitting issue.ResultsTotal serum bilirubin levels were found to be associated with any high-frequency (HPTA>15 dB in at least one ear, adjusted odds-ratio (ORa)(bootstrap 95% confidence interval) = 3.29(1.31–8.19), p = 0.011), but not with any low-frequency (LPTA>15 dB in at least one ear), SNHL in the US adolescents. Furthermore, high-frequency SNHL with HPTA>15 dB in both ears (bilateral) or HPTA≥25 dB in at least one ear, compared to that with HPTA>15 dB in one ear only (unilateral) or HPTA = 15–25 dB in at least one ear, had a stronger association with total serum bilirubin levels (ORa = 5.37(1.27–22.65), p = 0.022 for bilateral; ORa = 2.64(0.84–8.25), p = 0.094 for unilateral; ORa = 5.00(0.95–26.58), p = 0.058 for HPTA≥25 dB in at least one ear; as well as ORa = 3.06(1.15–8.25), p = 0.025 for HPTA = 15–25 dB in at least one ear). No severe overfitting problems were found.ConclusionOur findings suggest that current levels of total serum bilirubin may be informative in predicting and/or targeting high-frequency SNHL in the US adolescents.



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Postoperative bradycardia following adenotonsillectomy in children: Does intraoperative administration of dexmedetomidine play a role?

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Benjamin Bush, Joseph D. Tobias, Chen Lin, James Ruda, Kris R. Jatana, Garth Essig, Jennifer Cooper, Dmitry Tumin, Charles A. Elmaraghy
IntroductionDexmedetomidine is a novel pharmacologic agent that has become a frequently used adjunct during care of pediatric patients with obstructive sleep apnea (OSA) undergoing tonsillectomy. While generally safe and effective, dexmedetomidine is associated with adverse effects of hypotension and bradycardia from its central sympatholytic effects. Due to safety concerns, our institution routinely admits patients with OSA for overnight cardiorespiratory monitoring following tonsillectomy. With such monitoring, we have anecdotally noted bradycardia in our patients and sought to investigate whether this was related to the increased use of intra-operative dexmedetomidine.MethodsWe retrospectively reviewed records over an 11-month period to compare the incidence of postoperative bradycardia following hospital admission for tonsillectomy in patients who received dexmedetomidine versus those who did not.ResultsThe study cohort included 921 patients (371 received dexmedetomidine and 550 did not). Bradycardia was asymptomatically noted in 66 patients (7.2%). No patient required medical intervention for the bradycardia or developed clinical symptoms. There was no association of bradycardia with the intra-operative administration of dexmedetomidine (8.9% of patients who received dexmetomidine vs. 9.4% who did not). In multivariable analysis, bradycardia was more common among older patients, with the administration of topical or injected lidocaine, and with specific associated procedures (inferior turbinate coblation with out-fracture or direct laryngoscopy and bronchoscopy).ConclusionThe increased incidence of asymptomatic bradycardia in our post-adenotonsillectomy patients seemed to relate more to increased utilization of postoperative cardiac telemetry, and did not appear associated with the use of dexmedetomidine use intra-operatively.



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Long-term outcomes of Universiti Kebangsaan Malaysia Cochlear Implant Program among pediatric implantees

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Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Bee-See Goh, Noraziana Fadzilah, Asma Abdullah, Basyariatul Fathi Othman, Cila Umat
ObjectivesCochlear implant (CI) greatly enhances auditory performance as compared to hearing aids and has dramatically affected the educational and communication outcomes for profoundly deaf children. Universiti Kebangsaan Malaysia (UKM) pioneered CI program in 1995 in the South East Asia. We would like to report the long-term outcomes of UKM paediatric cochlear implantation in terms of: the proportion of children who were implanted and still using the device, the children's modes of communication, their educational placements, and their functional auditory/oral performance. We also examined the factors that affected the outcomes measured.Study designThis was a cross sectional observational study.MethodsTwo sets of questionnaires were given to 126 parents or primary caregivers of the implantees. The first set of questionnaire contained questions to assess the children's usage of CI, their types of education placement, and their modes of communication. The second set of questionnaire was the Parent's Evaluation Of Aural/Oral Performance of Children (PEACH) to evaluate the children's auditory functionality.ResultsOur study showed that among the implantees, 97.6% are still using their CI, 69.8% communicating orally, and 58.5% attending mainstream education. For implantees that use oral communication and attend mainstream education, their mean age of implantation is 38 months. This is significantly lower compared to the mean age of implantation of implantees that use non-oral communication and attend non-mainstream education. Simple logistic regression analysis shows age of implantation reliably predicts implantees (N = 126) would communicate using oral communication with odds ratio of 0.974, and also predict mainstream education (N = 118) with odds ratio of 0.967. The median score of PEACH rating scale is 87.5% in quiet, and this significantly correlates with an earlier age of implantation (r = −0.235 p = 0.048).ConclusionsUKM Cochlear Implant Program has achieved reasonable success among the pediatric implantees, with better outcomes seen in those implanted at the age of less than 4 years old.



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Clinical role of vitamin D in prognosis of otitis media with effusion

Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Fatih Alper Akcan, Yusuf Dündar, Hümeyra Bayram Akcan, Ahmet Uluat, Derya Cebeci, Mehmet Ali Sungur, İlhan Ünlü
ObjectiveTo investigate the clinical role of Vitamin D in prognosis of Otitis media with effusion.MethodsThis prospective-controlled study was conducted at otolaryngology department in Duzce University, Turkey. The study group comprised children who were diagnosed with Otitis media with effusion between September 2016 and February 2017. Control group was conducted with children underwent circumcision or inguinal hernia repair operations that confirmed with ENT examination they do not have any sign of otitis media. After 3 months of follow-up without any treatment, unresolved cases who were accepted as chronic otitis media with effusion were operated under general anesthesia for ventilation tube application. Study and control groups were assessed depending on the serum 25(OH)Vitamin D levels at the end of 3 months; < 15 ng/mL was accepted as vitamin D deficiency. The results were compared with the control group in terms of vitamin D levels. Also, subgroup analysis was performed addressing to the complete recovery otitis media with effusion and chronic otitis media with effusion.ResultsOne-hundred-seventy-four children with otitis media with effusion and 80 control patients were included to the study. One-hundred-eight (62%) out of 174 patients with otitis media with effusion was completely recovered after a 3-months follow up. Of those 66 out of 174 children, they had persistent diseases, underwent ventilation tube insertion after a 3-months follow-up. The mean 25(OH)Vitamin D level was 18.98 ± 10.60 in otitis media with effusion group and 28.07 ± 14.10 in control group and the difference was statistically significant between the study and control group (p < 0.001). Vitamin D deficiency was observed in 33 out of 66 patients (50.0%) in chronic otitis media with effusion group whilst 35 out of 108 patients (32.4%) in complete recovery otitis media with effusion group (p = 0.021). The rate of 25(OH)Vitamin D deficiency was 25% in control group which was statistically different from chronic otitis media with effusion and recovery chronic otitis media with effusion groups (p = 0.006).ConclusionsThis study not only shows the relationship between Vitamin D and otitis media with effusion development, but also demonstrates the effects of Vitamin D on otitis media with effusion prognosis. There is a significant association between 25(OH)Vitamin D deficiency and follow-up outcomes of otitis media with effusion.



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Pre- and post-operative evaluation of the frequency of nocturnal enuresis and Modified Pediatric Epworth Scale in pediatric obstructive sleep apnea patients

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Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Kerem Sami Kaya, Bilge Türk, Zeynep Nur Erol, Pınar Akova, Berna Uslu Coşkun
ObjectiveTo investigate the beneficial effect of adenotonsillectomy (AT) on nocturnal enuresis (NE) in children with adenotonsillar hypertrophy and evaluate the Modified Pediatric Epworth Scale (MPES).MethodsThis was a prospective study comparing preoperative and postoperative evaluation of the frequency of NE and MPES in pediatric obstructive sleep apnea (OSA). A questionnaire on NE history was prepared based on the Turkish Enuresis Guidelines. NE histories were evaluated pre- and postoperatively on the first month and third month, respectively. MPES questions were asked to the parents of all the OSA patients pre- and postoperatively, and scores were noted.ResultsEighty-four (84) pediatric OSA patients were involved in the study. Preoperatively, 19 patients (27%) complained about nocturnal enuresis. After the surgery, 52% of the patients with NE had complete resolution of NE (p < 0.001). Postoperatively, the average Epworth scores of both groups significantly decreased (p < 0.001).ConclusionThere is a strong correlation between OSA and NE. In the present study, enuresis in pediatric OSA patients significantly decreased after surgery. Also, Epworth scores decreased significantly after surgery. In children with nocturnal enuresis, the presence of OSA symptoms should be questioned.



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Spontaneous retropharyngeal and mediastinal thoracic duct cyst in an infant with respiratory distress

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Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Vidur Bhalla, Thomas Schrepfer, Adam McCann, Pamela Nicklaus, Brenton Reading
Thoracic duct cysts (TDC) within the retropharyngeal space and mediastinum are exceedingly rare lesions, with the majority related to trauma or neoplasm. We describe a case of an otherwise healthy 8-month-old boy who presented with severe respiratory distress, which was found to be caused by a large, spontaneous TDC occupying most the retropharyngeal and mediastinal space. To our knowledge, this is the youngest patient to date presenting with TDC. Ultimately, his TDC was completely resolved with sclerotherapy, however the patient's age and size presented unique challenges to his medical management, which we describe below.



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Readability of online patient education materials for velopharyngeal insufficiency

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Deborah X. Xie, Ray Y. Wang, Sivakumar Chinnadurai
ObjectiveEvaluate the readability of online and mobile application health information about velopharyngeal insufficiency (VPI).MethodsTop website and mobile application results for search terms "velopharyngeal insufficiency", "velopharyngeal dysfunction", "VPI", and "VPD" were analyzed. Readability was determined using 10 algorithms with Readability Studio Professional Edition (Oleander Software Ltd; Vandalia, OH). Subgroup analysis was performed based on search term and article source – academic hospital, general online resource, peer-reviewed journal, or professional organization.Results18 unique articles were identified. Overall mean reading grade level was a 12.89 ± 2.9. The highest reading level among these articles was 15.47–approximately the level of a college senior. Articles from "velopharyngeal dysfunction" had the highest mean reading level (13.73 ± 2.11), above "velopharyngeal insufficiency" (12.30 ± 1.56) and "VPI" (11.66 ± 1.70). Articles from peer-reviewed journals had the highest mean reading level (15.35 ± 2.79), while articles from academic hospitals had the lowest (12.81 ± 1.66). There were statistically significant differences in reading levels between the different search terms (P < 0.01) and article source types (P < 0.05). Only one mobile application was identified with VPI information, with a readability of 10.68.ConclusionDespite published reading level guidelines, online patient education materials for VPI are disseminated with language too complex for most readers. There is also a lack of VPI-related mobile application data available for patients. Patients will benefit if future updates to websites and disseminated patient information are undertaken with health literacy in mind. Future studies will investigate patient comprehension of these materials.



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Sirolimus for management of complex vascular anomalies – A proposed dosing regimen for very young infants

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Publication date: February 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 105
Author(s): Josephine A. Czechowicz, Janel R. Long-Boyle, Kristina W. Rosbe, Erin F. Mathes, Ilona J. Frieden, Kristin A. Shimano
Neonates with vascular anomalies causing airway compromise and other complications require early initiation of medical therapy. Sirolimus has emerged as a safe and effective treatment, but standard recommendations for dosing start at seven months. Guidelines are needed for dosing in very young infants, who have reduced hepatic metabolism of sirolimus. We present our experience treating six neonates (mean age 14.8 days) with complicated vascular anomalies. Standard dosing caused supratherapeutic levels in this population. Our modified dosing regimen has resulted in safe therapeutic concentrations. Properly dosed, sirolimus is a viable and potentially lifesaving option for neonates with severe morbidity from vascular anomalies.



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Using simulators to teach pediatric airway procedures in an international setting

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Marissa A. Schwartz, Katherine R. Kavanagh, Steven J. Frampton, Iain A. Bruce, Tulio A. Valdez
IntroductionThere has been a growing shift towards endoscopic management of laryngeal procedures in pediatric otolaryngology. There still appears to be a shortage of pediatric otolaryngology programs and children's hospitals worldwide where physicians can learn and practice these skills. Laryngeal simulation models have the potential to be part of the educational training of physicians who lack exposure to relatively uncommon pediatric otolaryngologic pathology.ObjectivesThe objective of this study was to assess the utility of pediatric laryngeal models to teach laryngeal pathology to physicians at an international meeting.MethodsPediatric laryngeal models were assessed by participants at an international pediatric otolaryngology meeting. Participants provided demographic information and previous experience with pediatric airways. Participants then performed simulated surgery on these models and evaluated them using both a previously validated Tissue Likeness Scale and a pre-simulation to post-simulation confidence scale.ResultsParticipants reported significant subjective improvement in confidence level after use of the simulation models (p < 0.05). Participants reported realistic representations of human anatomy and pathology. The models' tissue mechanics were adequate to practice operative technique including the ability to incise, suture, and suspend models.ConclusionThe pediatric laryngeal models demonstrate high quality anatomy, which is easy manipulated with surgical instruments. These models allow both trainees and surgeons to practice time-sensitive airway surgeries in a safe and controlled environment.



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Germinal mosaicism of PAX3 mutation caused Waardenburg syndrome type I

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Kaitian Chen, Yuan Zhan, Xuan Wu, Ling Zong, Hongyan Jiang
ObjectivesWaardenburg syndrome mutations are most often recurrent or de novo. The rate of familial recurrence is low and families with several affected children are extremely rare. In this study, we aimed to clarify the underlying hereditary cause of Waardenburg syndrome type I in two siblings in a Chinese family, with a mother affected by prelingual mild hearing loss and a father who was negative for clinical symptoms of Waardenburg syndrome and had a normal hearing threshold.MethodsComplete characteristic features of the family members were recorded and genetic sequencing and parent-child relationship analyses were performed.ResultsThe two probands were found to share double mutations in the PAX3/GJB2 genes that caused concurrent hearing loss in Waardenburg syndrome type I. Their mother carried the GJB2 c.109G > A homozygous mutation; however, neither the novel PAX3 c.592delG mutation, nor the Waardenburg syndrome phenotype, was observed in either parent.ConclusionThese previously unreported digenic mutations in PAX3/GJB2 resulted in deafness associated with Waardenburg syndrome type I in this family. To our knowledge, this is the first report describing germinal mosaicism in Waardenburg syndrome. This concept is important because it complicates genetic counseling of this family regarding the risk of recurrence of the mutations in subsequent pregnancies.



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Systematic review of knowledge of, attitudes towards, and practices for newborn hearing screening among healthcare professionals

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Rohit Ravi, Dhanshree R. Gunjawate, Krishna Yerraguntla, Bellur Rajashekhar
IntroductionThe success of newborn hearing screening programs lies in the timely identification, diagnosis, and management of children with hearing loss accomplished via a multidisciplinary newborn hearing screening (NHS) team. The team is typically comprised of various healthcare professionals who act as decision makers as well as facilitators for different stages in the screening process. Team members' knowledge of, attitudes towards, and practices for early hearing detection and intervention programs are critical for success and prevention of loss to follow up. In this context, it becomes crucial to understand their knowledge of, attitudes towards, and practices for towards newborn hearing screening.MethodsA systematic review was conducted on the following databases; PubMed/Medline, Cumulative Index to Nursing and Allied Health Literature (CINAHL), Scopus, Web of Science, Science Direct and Cochrane Library. This search was carried out using various keywords such as practitioners, newborn hearing screening, knowledge, attitudes, and practices in different combinations. The review was conducted based on Preferred Reporting Items for Systematic Reviews and Meta-analyses statement guidelines.ResultsA total of 271 hits were obtained of which 20 articles were found suitable for inclusion in the final review. Overall, similar results were found regarding team members' knowledge of NHS programs, regardless of country of origin. Similarly, attitudes toward NHS programs were positive. Team members' experiences with NHS programs varied from country-to-country and across healthcare professionals. Results consistently showed gaps in team members' knowledge suggesting the need for outreach and professional education programs on NHS.ConclusionNHS teams members from different countries, healthcare systems, and early hearing detection and intervention programs show gaps in critical knowledge warranting outreach and educational programs.



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Identification of two novel pathogenic compound heterozygous MYO7A mutations in Usher syndrome by whole exome sequencing

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Ying Jia, Xiaoge Li, Dong Yang, Yi Xu, Ying Guo, Xin Li
The current study aims to identify the pathogenic sites in a core pedigree of Usher syndrome (USH). A core pedigree of USH was analyzed by whole exome sequencing (WES). Mutations were verified by polymerase chain reaction (PCR) amplification and Sanger sequencing. Two pathogenic variations (c.849+2T>C and c.5994G>A) in MYO7A were successfully identified and individually separated from parents. One variant (c.849+2T>C) was nonsense mutation, causing the protein terminated in advance, and the other one (c.5994G>A) located near the boundary of exon could cause aberrant splicing. This study provides a meaningful exploration for identification of clinical core genetic pedigrees.



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The anatomic applicability of transcanal endoscopic ear surgery in children

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Publication date: Available online 15 December 2017
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Wan-Hsuan Sun, Chin-Lung Kuo, Tzu-Chin Huang
ObjectiveThe feasibility of TEES in dealing with pediatric middle ear disease is still under investigation. The goal of this study was to compare anatomical dimensions of the EAC between children and adults, and to analyze the anatomic applicability of TEES in children.MethodsForty pediatric (18 years old and younger) and 20 adult patients who received TB-HRCT to evaluate middle ear conditions were enrolled from December 2010 to December 2015. Dimensions including the diameters of the EAC orifice, isthmus and tympanic membrane annulus, the length of EAC, the height of the middle ear, and the angle between tympanic membrane and EAC axis were determined according to the TB-HRCT data.ResultsThe diameters of the EAC orifice and isthmus and length of EAC in the pediatric patients were slightly smaller than those in the adult patients. The anatomical dimensions of middle ear were similar in both groups. Simple regression analysis indicated that the diameters of the EAC orifice and isthmus and the length of the EAC were positively correlated with the age of the pediatric patients. In the pediatric patients, 67 ears (83.75%) had an EAC isthmus diameter larger than 4 mm and are sufficient with a 3-mm endoscope manipulation.ConclusionTEES is applicable for most pediatric patients using an endoscope with a diameter of 3 mm or smaller. With an appropriate endoscope and instruments, TEES is a safe and effective alternative to treat pediatric middle ear disease.



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Designing a tissue-engineered tracheal scaffold for preclinical evaluation

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Publication date: January 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 104
Author(s): Cameron A. Best, Victoria K. Pepper, Devan Ohst, Kyle Bodnyk, Eric Heuer, Ekene A. Onwuka, Nakesha King, Robert Strouse, Jonathan Grischkan, Christopher K. Breuer, Jed Johnson, Tendy Chiang
ObjectiveRecent efforts to tissue engineer long-segment tracheal grafts have been complicated by stenosis and malacia. It has been proposed that both the mechanical characteristics and cell seeding capacity of TETG scaffolds are integral to graft performance. Our aim was to design a tracheal construct that approximates the biomechanical properties of native sheep trachea and optimizes seeding with bone marrow derived mononuclear cells prior to preclinical evaluation in an ovine model.MethodsA solution of 8% polyethylene terephthalate (PET) and 3% polyurethane (PU) was prepared at a ratio of either 8:2 or 2:8 and electrospun onto a custom stainless steel mandrel designed to match the dimensional measurements of the juvenile sheep trachea. 3D-printed porous or solid polycarbonate C-shaped rings were embedded within the scaffolds during electrospinning. The scaffolds underwent compression testing in the anterior-posterior and lateral-medial axes and the biomechanical profiles compared to that of a juvenile ovine trachea. The most biomimetic constructs then underwent vacuum seeding with ovine bone marrow derived mononuclear cells. Fluorometric DNA assay was used to quantify scaffold seeding.ResultsBoth porous and solid rings approximated the biomechanics of the native ovine trachea, but the porous rings were most biomimetic. The load-displacement curve of scaffolds fabricated from a ratio of 2:8 PET:PU most closely mimicked that of native trachea in the anterior-posterior and medial-lateral axes. Solid C-ringed scaffolds had a greater cell seeding efficiency when compared to porous ringed scaffolds (Solid: 19 × 104 vs. Porous: 9.6 × 104 cells/mm3, p = 0.0098).ConclusionA long segment tracheal graft composed of 2:8 PET:PU with solid C-rings approximates the biomechanics of the native ovine trachea and demonstrates superior cell seeding capacity of the two prototypes tested. Further preclinical studies using this graft design in vivo would inform the rational design of an optimal TETG scaffold.



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'Mutiny on the crown: two cases of rare cephalic malformations

The presentation and management of two bizarre congenital cephalic curiosities at the two extremes of the clinical spectrum are discussed herewith. Case 1: presented to us as a neonate with a scalp swelling mirror-imaging her head and face. The journey from clinics to wards and to the operation theatre and to her home is introspected. Case 2: presented to us as dicephalous dibrachius dipus parapagus conjoined twins. The detailed work-up of individual organ systems, the multidisciplinary approach to management and the final outcome are discussed. This is an unsolved mystery for the anatomists, paediatric surgeons, radiologists and the medical fraternity at large.



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Constrictive bronchiolitis presenting with a mixed obstructive and restrictive pattern, associated with acid reflux

A previously healthy 55-year-old woman presented with worsening dyspnoea on exertion. The patient lived at altitude, did not smoke and had no exposure to occupational or environmental toxins. Her physical examination, including pulmonary, was unremarkable. Pulmonary function tests showed forced expiratory volume in 1 s/forced vital capacity ratio 74% predicted, diffusing capacity for carbon monoxide (DLCO) 92% predicted and residual volume 213% predicted. Rheumatological workup was negative. Chest radiograph showed hyperinflation without consolidation, and high-resolution chest CT showed mosaic attenuation with air trapping on expiratory imaging. A decreasing DLCO lead to transbronchial biopsies that were inconclusive. A video-assisted thoracic surgery lung biopsy showed small airway disease suggestive of constrictive bronchiolitis. Oesophagram and a barium swallow showed a hiatal hernia with large volume gastro-oesophageal reflux to the level of the clavicles. The development of constrictive bronchiolitis in this patient was possibly secondary to hiatal hernia and silent gastroesophageal reflux disease (GERD). In the face of presumably idiopathic lung disease, clinicians should perform a GERD workup even in the absence of GERD symptoms.



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Three-vessel coronary artery aneurysmal disease complicated by multivessel thrombosis and cardiogenic shock: the saving role of intracoronary thrombolysis

The benefit of intracoronary thrombolytics in ST-elevation myocardial infarction (STEMI) is not well established. Mainstays of STEMI management include intravenous thrombolytics, percutaneous coronary interventions and surgical revascularisation. However, in cases of STEMI secondary to coronary artery aneurysmal disease (CAAD), standard treatment options may not be suitable due to high thrombus burden, perioperative risk and factors unique to each patient. Thus, STEMI management in CAAD can represent a therapeutically challenging clinical scenario. Here, we describe a patient with severe three-vessel CAAD complicated by multivessel thrombosis and cardiogenic shock for whom traditional management options including placement of haemodynamic support devices were not feasible. As an alternative measure, the patient was treated with intracoronary thrombolysis with remarkable clinical stabilisation and angiographic resolution of thrombosis. He remains clinically stable several years later without recurrent events. This case serves to demonstrate the potential lifesaving benefit of intracoronary thrombolysis in complicated multivessel CAAD.



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Necrotising soft tissue infection without systemic toxicity in a patient with rheumatoid arthritis treated with tocilizumab

A Japanese woman aged 76 years with rheumatoid arthritis treated with prednisolone and tocilizumab presented with a 2-day history of redness and pain in her right thigh. She was hospitalised with a primary diagnosis of cellulitis and antimicrobial therapy was initiated. She had been stable until the fourth day of admission, when the swelling of her right thigh rapidly worsened and demonstrated purpura; she was subsequently unable to walk because of the pain. A diagnosis of necrotising soft tissue infection (NSTI) was made and extensive debridement was performed. Over the next 4 months, additional debridement was performed four times. Her condition improved significantly and she was able to walk later. Physicians should recognise that tocilizumab can mask systemic toxicities and inflammatory findings even in severe infections. To avoid delays in diagnosis and surgical intervention, clinicians should consider NSTIs when they encounter patients treated with tocilizumab, even if it mimics cellulitis.



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Enlarging hypermetabolic nodule: benign non-functional adrenocortical adenoma

Description

A 70-year-old woman with controlled type 2 diabetes mellitus and hypertension presented for evaluation of chronic abdominal discomfort. An incidental 3.2x3.5x3 cm left adrenal mass was identified on CT. Physical examination revealed obesity stage 2 without a Cushingoid appearance and controlled hypertension with a regular heart rate. Biochemical evaluations for Cushing syndrome, primary aldosteronism and pheochromocytoma were negative. A 1-year follow-up CT of the adrenal glands with washout showed a larger left-sided adrenal mass, measuring 4.3x3.4x3 cm (figure 1A). The attenuation values of the mass were indeterminate: non-contrast, 15 Hounsfield unit (HU); portal venous, 70 HU; delayed, 42 HU; absolute washout, 51%. MRI of the abdomen confirmed the left adrenal mass (figure 1B,C). Given the rapidly increasing size, malignancy was suspected and a whole-body positron emission tomography/CT scan using 18F-fluorodeoxyglucose (18F-FDG) was performed, which demonstrated (figure 1D,E) an 18F-FDG avid adrenal mass (standardized uptake...



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High fat diet exacerbates imiquimod-induced psoriasis-like dermatitis in mice

Abstract

Psoriasis, a chronic inflammatory skin disease, is closely related to systemic metabolism. An elevated body mass index (BMI) is a risk factor for psoriasis; inflammasomes are activated by adipose tissue macrophages in obese subjects. We hypothesized that hyperlipidemia is involved in the pathogenesis of psoriasis and examined the role of a high fat diet (HFD) in the development of psoriasis in imiquimod (IMQ)-treated mice. The body weight and serum level of cholesterol were significantly higher in mice fed an HFD than a regular diet (RD). HFD mice had higher psoriasis skin scores and the number of neutrophils infiltrating into the lesional skin was elevated. IL-17A mRNA expression was significantly increased in the skin of IMQ-treated HFD mice; the expression of IL-22, IL-23, and TNF-α mRNA was not enhanced. Caspase-1 and IL-1β were activated in the skin of IMQ-treated HFD mice and their serum level of IL-17A, TNF-α, and IL-1β was significantly upregulated. Our findings strongly suggest that hyperlipidemia is involved in the development and progression of psoriasis via systemic inflammation and inflammasome activation.

This article is protected by copyright. All rights reserved.



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Altered natural killer cell cytokine profile in type 2 autoimmune hepatitis

Publication date: Available online 9 December 2017
Source:Clinical Immunology
Author(s): Dalila Mele, Grazia Bossi, Giuseppe Maggiore, Barbara Oliviero, Stefania Mantovani, Beatrice Bonelli, Mario U. Mondelli, Stefania Varchetta
Type 2 autoimmune hepatitis (AIH-2) is a rare disease presenting in early childhood. The immunopathogenetic mechanisms are poorly characterized, although a defect of regulatory T cells (Treg) has been shown. There is virtually no information on innate immune responses and natural killer (NK) cells in particular. We have performed an extended immunophenotypic and functional analysis of NK cells in children with AIH-2. We show that NK cell frequency is reduced in this setting and that the balance between NK activating and inhibitory receptors is skewed toward activation. More importantly, NK cells display an altered cytokine pattern characterized by increased IFNγ and reduced IL2 production which could contribute to impaired Treg function. Exposure of mononuclear cells to IL2 resulted in normalization of NK IFNγ production. Thus, our findings support treatment of AIH-2 with low-dose IL2, which would result in normalization of NK cell function and expansion of the Treg cell subset.

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GM-CSF producing autoreactive CD4+ T cells in type 1 diabetes

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Publication date: Available online 8 December 2017
Source:Clinical Immunology
Author(s): Jan Knoop, Anita Gavrisan, Denise Kuehn, Julia Reinhardt, Melanie Heinrich, Markus Hippich, Anne Eugster, Christian Ockert, Anette-Gabriele Ziegler, Ezio Bonifacio
The phenotype of autoreactive T cells in type 1 diabetes is described as Th1, Th17 and/or Th21, but is largely uncharacterized. We combined multi-parameter cytokine profiling and proliferation, and identified GM-CSF producing cells as a component of the response to beta cell autoantigens proinsulin and GAD65. Overall cytokine profiles of CD4+ T cell were not altered in type 1 diabetes. In contrast, patients with recent onset type 1 diabetes had increased frequencies of proinsulin-responsive CD4+CD45RA- T cells producing GM-CSF (p=0.002), IFNγ (p=0.004), IL-17A (p=0.008), IL-21 (p=0.011), and IL-22 (p=0.007), and GAD65-responsive CD4+CD45RA- T cells producing IL-21 (p=0.039). CD4+ T cells with a GM-CSF+IFNγ-IL-17A-IL-21-IL-22- phenotype were increased in patients for responses to both proinsulin (p=0.006) and GAD65 (p=0.037). GM-CSF producing T cells are a novel phenotype in the repertoire of T helper cells in type 1 diabetes and consolidate a Th1/Th17 pro-inflammatory pathogenesis in the disease.



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The epidermal growth factor receptor inhibitor AG1478 inhibits eosinophilic inflammation in upper airways

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Publication date: Available online 26 November 2017
Source:Clinical Immunology
Author(s): Shino Shimizu, Kumiko Takezawa-Yasuoka, Takao Ogawa, Ichiro Tojima, Hideaki Kouzaki, Takeshi Shimizu
Mucus hypersecretion and eosinophil infiltration are important characteristics of eosinophilic inflammation in upper airways, such as allergic rhinitis and chronic rhinosinusitis with nasal polyp. EGFR transactivation induces mucus and inflammatory cytokine secretion from airway epithelial cells. However, the roles of EGFR in eosinophilic inflammation in upper airways are still unknown. The purpose of the study is to elucidate the effects of the EGFR inhibitor AG1478 on eosinophilic airway inflammation. AG1478 significantly inhibited thrombin-induced GM-CSF secretion from nasal epithelial cells and thrombin-induced secretion of eotaxin-1 and RANTES from nasal fibroblasts. Intranasal instillation of AG1478 inhibited OVA-induced goblet cell metaplasia, mucus production and eosinophil/neutrophil infiltration in rat nasal epithelium, as did intraperitoneal injection of AG1478. These results indicate that EGFR transactivation plays an important role in eosinophilic airway inflammation. Intranasal instillation of an EGFR inhibitor may be a new therapeutic approach for the treatment of intractable eosinophilic inflammation in upper airways.



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Corrigendum to “Klinefelter's syndrome (47,XXY) is in excess among men with Sjögren's syndrome” [Clin. Immunol. 168 (2016) 25–29]

Publication date: Available online 7 December 2017
Source:Clinical Immunology
Author(s): Valerie M. Harris, Rohan Sharma, Joshua Cavett, Biji T. Kurien, Ke Liu, Kristi A. Koelsch, Astrid Rasmussen, Lida Radfar, David Lewis, Donald U. Stone, C. Erick Kaufman, Shibo Li, Barbara Segal, Daniel J. Wallace, Michael H. Weisman, Swamy Venuturupalli, Jennifer A. Kelly, Marta E. Alarcon-Riquelme, Bernardo Pons-Estel, Roland Jonsson, Xianglan Lu, Jacques-Eric Gottenberg, Juan-Manuel Anaya, Deborah S. Cunninghame-Graham, Andrew J.W. Huang, Michael T. Brennan, Pamela Hughes, Ilias Alevizos, Corinne Miceli-Richard, Edward C. Keystone, Vivian P. Bykerk, Gideon Hirschfield, Gang Xie, Katherine A. Siminovitch, Wan-Fai Ng, Gunnel Nordmark, Sara Magnusson Bucher, Per Eriksson, Roald Omdal, Nelson L. Rhodus, Maureen Rischmueller, Michael Rohrer, Marie Wahren-Herlenius, Torsten Witte, Xavier Mariette, Christopher J. Lessard, John B. Harley, Kathy L. Sivils, R. Hal Scofield




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Chronic Granulomatous Disease in children: a single center experience

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Publication date: Available online 7 December 2017
Source:Clinical Immunology
Author(s): Alessandra Beghin, Marta Comini, Annarosa Soresina, Luisa Imberti, Michela Zucchi, Alessandro Plebani, Alessandro Montanelli, Fulvio Porta, Arnalda Lanfranchi
Chronic Granulomatous Disease (CGD) is caused by the failure of the phagocytes to kill pathogens. We carried out a retrospective analysis of cellular, molecular and clinical features of 14 young patients (mean age at the onset of symptoms and diagnosis: 10 and 25months, respectively), 7 with autosomal recessive and 7 X-linked form, referred to the Children's Hospital of Brescia between 1999 and 2016. Two new mutations were found, one localized in the CYBB and one in the NCF1 genes. Twelve patients were followed in our institution; the average length of their follow-up after diagnosis was 66months in X-linked patients and 126months in autosomal recessive inheritance. The overall survival was 67%, 40% in X-linked and 86% in autosomal recessive form. Eight patients were treated with HSCT. We did not find a clear correlation between the clinical symptoms and the type of mutation, but the fine characterization of the patients was mandatory for therapeutic option, genetic counseling and prenatal diagnosis.



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STAT3-mediated epigenetic silencing of FOXP3 in LADA T cells is regulated through HDAC5 and DNMT1

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Publication date: Available online 6 December 2017
Source:Clinical Immunology
Author(s): Can Hou, Yanjun Zhong, Zhen Wang, Zhao Ming, Gan Huang, Lin Ouyang, Yijun Li, Qianjin Lu, Zhiguang Zhou
In LADA patients, Tregs are reduced and FOXP3 is downregulated in CD4+ T cells, but the etiology remains unclear. Our study included in 20 LADA patients and 20 healthy control patients. qRT-PCR results showed that STAT3, HDAC3, HDAC5, SIRT1, DNMT1 and DNMT3b mRNAs were significantly upregulated in LADA CD4+ T cells than controls, while FOXP3 mRNA significantly decreased. p-STAT3, STAT3, DNMT1 and DNMT3b expressions were increased demonstrated by western blot. ChIP-PCR suggested that p-STAT3 binds to the Foxp3 promoter, meanwhile, histone H3 acetylation at K9 and K14 of FOXP3 promoter were significantly lower than controls. Luciferase reporter assay showed that ectopic STAT3 expression significantly reduced FOXP3 promoter activities. The Foxp3 promoter was significantly hypermethylated in LADA than controls. LADA patients showed stronger binding of p-STAT3, HDAC5 and DNMT1 than controls using CHIP. These findings reveal a crucial role of STAT3 in regulating the epigenetic status of T cells in LADA.



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Modulation the expression of natural killer cell activating receptor (NKp44) in the peripheral blood of diffuse large B-cell lymphoma patients and the correlation with clinic pathological features

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Publication date: Available online 13 December 2017
Source:Clinical Immunology
Author(s): Enas Said Essa, Gehan Abd-Elfatah Tawfeek, Suzan Ahmed El Hassanin, Khloud Gamal Mohammed Emara
NK cell activation is one strategy to improve the immunotherapy of non-Hodgkin's lymphoma. So, we aimed to investigate expression of Natural killer cell activating receptor NKp44 in patients with diffuse large B-cell lymphoma (DLBCL) and its correlation with clinic pathological data. In this study, 30 new cases with DLBCL in addition to 20 healthy control were involved. All were submitted to full history, clinical examination, histopathology, Routine laboratory investigations including CBC, LDH, β2microgloubine and bone marrow examination. Cell culture of peripheral blood mononuclear cells and expression of CD56 and NKp44 by flowcytometry was done. We demonstrated increased NK cell populations (CD 56 +ve NKp44 –ve, CD 56 –veNKp44 +ve, total CD 56 +ve) and NKp44 MFI after in-vitro activation in both healthy control and DLBCL cases except for CD 56 +ve NKp44 +ve which significantly increased in patients not in healthy control (p=0.005, 0.601) respectively. No significant difference between the DLBCL and healthy control regarding all NK cell populations without PHA stimulation. However, the culture with PHA in DLBCL showed significant increase in NK cell populations than the healthy control (CD 56 +ve NKp44 +ve 12.37±7.52vs 6.80±4.07, p=0.008), (Total CD 56 +ve 18.80±8.74vs 12.66±5.17, p=0.017), (MFI of NKp44 10.95±6.18vs 5.58±1.70, p=0.001). Regarding the association with clinic pathologic features, increased expression of NKp44 was associated with lower values of LDH and earlier stages of DLBCL (p<0.05). So, activating receptor NKp44 can be modulated by in-vitro activation, hence improvement of its function as an approach of immunotherapy of DLBCL.



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Novel biallelic TRNT1 mutations resulting in sideroblastic anemia, combined B and T cell defects, hypogammaglobulinemia, recurrent infections, hypertrophic cardiomyopathy and developmental delay

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Publication date: Available online 21 November 2017
Source:Clinical Immunology
Author(s): Vassilios Lougaris, Janet Chou, Manuela Baronio, Luisa Gazzurelli, Tiziana Lorenzini, Annarosa Soresina, Daniele Moratto, Raffaele Badolato, Michael Seleman, Massimo Bellettato, Raif S. Geha, Alessandro Plebani




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An optimized whole blood assay measuring expression and activity of NLRP3, NLRC4 and AIM2 inflammasomes

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Publication date: Available online 26 November 2017
Source:Clinical Immunology
Author(s): Lev Grinstein, Hella Luksch, Felix Schulze, Avril A.B. Robertson, Matthew A. Cooper, Angela Rösen-Wolff, Stefan Winkler
The proinflammatory protease caspase-1 plays pivotal roles in central pathways of innate immunity, thereby contributing to pathogen clearance. Beside its physiological role, dysregulated activity of caspase-1 is known to contribute to an increasing number of diseases. In this study we optimized and validated a low-volume human whole blood assay facilitating the measurement of caspase-1 activation and inflammasome-related gene expression upon stimulation of the NLRP3, NLRC4 or AIM2 inflammasome. Using the NLRP3 inflammasome specific inhibitor MCC950 we were able to measure the activity of canonical or alternative NLRP3 pathways, AIM2 and NLRC4 inflammasomes in whole blood. Based on our data we assume a superposition of NLRP3 and NLRC4 inflammasome activities in human whole blood following stimulation with S. typhimurium. The optimized whole blood assay may be suitable for diagnostic and research purposes for pediatric patients who can only donate small amounts of blood.



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A novel de novo activating mutation in STAT3 identified in a patient with common variable immunodeficiency (CVID)

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Publication date: Available online 24 November 2017
Source:Clinical Immunology
Author(s): Mark A. Russell, Manuela Pigors, Maha E. Houssen, Ania Manson, David Kelsell, Hilary Longhurst, Noel G. Morgan
Common variable immunodeficiency (CVID) is characterised by repeated infection associated with primary acquired hypogammaglobulinemia. CVID frequently has a complex aetiology but, in certain cases, it has a monogenic cause. Recently, variants within the gene encoding the transcription factor STAT3 were implicated in monogenic CVID. Here, we describe a patient presenting with symptoms synonymous with CVID, who displayed reduced levels of IgG and IgA, repeated viral infections and multiple additional co-morbidities. Whole-exome sequencing revealed a de novo novel missense mutation in the coiled-coil domain of STAT3 (c.870A>T; p.K290N). Accordingly, the K290N variant of STAT3 was generated, and a STAT3 responsive dual-luciferase reporter assay revealed that the variant strongly enhances STAT3 transcriptional activity both under basal and stimulated (with IL-6) conditions. Overall, these data complement earlier studies in which CVID-associated STAT3 mutations are predicted to enhance transcriptional activity, suggesting that such patients may respond favourably to IL-6 receptor antagonists (e.g. tocilizumab).



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Novel anti-suprabasin antibodies may contribute to the pathogenesis of neuropsychiatric systemic lupus erythematosus

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Publication date: Available online 21 November 2017
Source:Clinical Immunology
Author(s): Kunihiro Ichinose, Kaname Ohyama, Kaori Furukawa, Osamu Higuchi, Akihiro Mukaino, Katsuya Satoh, Shunya Nakane, Toshimasa Shimizu, Masataka Umeda, Shoichi Fukui, Ayako Nishino, Hideki Nakajima, Tomohiro Koga, Shin-ya Kawashiri, Naoki Iwamoto, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Mari Yoshida, Naotaka Kuroda, Atsushi Kawakami
Neuropsychiatric systemic lupus erythematosus (NPSLE) is often difficult to diagnose and distinguish from other diseases, because no NPSLE-specific antibodies have been identified. We developed a novel proteomic strategy for identifying and profiling antigens in immune complexes in the cerebrospinal fluid (CSF), and applied this strategy to 26 NPSLE patients. As controls, we also included 25 SLE patients without neuropsychiatric manifestations (SLE), 15 with relapsing remitting multiple sclerosis (MS) and 10 with normal pressure hydrocephalus (NPH). We identified immune complexes of suprabasin (SBSN) in the CSF of the NPSLE group. The titer of anti-SBSN antibodies was significantly higher in the CSF of the NPSLE group compared to those of the SLE, MS and NPH groups. Microarray data showed that the senescence and autophagy pathways were significantly changed in astrocytes exposed to anti-SBSN antibodies. Our findings indicate that SBSN could be a novel autoantibody for the evaluation of suspected NPSLE.



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RAG deficiency with ALPS features successfully treated with TCRαβ/CD19 cell depleted haploidentical stem cell transplant

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Publication date: Available online 2 November 2017
Source:Clinical Immunology
Author(s): Emma Westermann-Clark, Alice Grossi, Francesca Fioredda, Stefano Giardino, Enrico Cappelli, Paola Terranova, Elena Palmisani, Jocelyn R. Farmer, Zsofia Foldvari, Yasuhiro Yamazaki, Maura Faraci, Edoardo Lanino, Luigi D. Notarangelo, Carlo Dufour, Isabella Ceccherini, Jolan E. Walter, Maurizio Miano




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Up-regulation of chemokine CXCL13 in systemic candidiasis

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Publication date: Available online 1 December 2017
Source:Clinical Immunology
Author(s): Congya Li, Ju Cao, Lifang Wang, Xiaojiong Jia, Jianchun He, Liping Zhang
Candida albicans is the leading cause of healthcare associated bloodstream infections. Chemokine CXCL13 is well-known involved in inflammation, but its role in candidemia has not been assessed. Our study firstly demonstrated that serum CXCL13 levels were significantly elevated in candidemic patients compared with bacteremic patients and control subjects by ELISA, and CXCL13 concentrations were positively and significantly correlated with clinical Sequential Organ Failure Assessment (SOFA) scores and several laboratory parameters in patients. Moreover, ROC curve analysis showed the diagnostic efficiency of CXCL13 was superior to CRP and PCT. To further study the role of CXCL13, a mouse model was established. Importantly, the data showed the dramatically elevated levels of CXCL13 in mice serum and infected kidney, were significantly correlated with renal fungal burden and pathology scores. In conclusion, our results indicated that CXCL13 had strong potential as a novel biomarker of diagnosis and prognosis for candidemia.



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Intrathecal anti-suprabasin antibodies in SLE, a cause of local concern?

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Publication date: Available online 29 November 2017
Source:Clinical Immunology
Author(s): Florencia Rosetti, José C. Crispín




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Circulating IL-17-producing mucosal-associated invariant T cells (MAIT) are associated with symptoms in children with asthma

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Publication date: Available online 28 November 2017
Source:Clinical Immunology
Author(s): Guillaume Lezmi, Rola Abou Taam, Céline Dietrich, Lucienne Chatenoud, Jacques de Blic, Maria Leite-de-Moraes
IL-17 and mucosal-associated invariant T (MAIT) cells have been involved in asthma pathogenesis. However, IL-17-producing MAIT cells (MAIT-17) were not evidenced. We aimed to determine whether circulating MAIT-17 were detectable in children with asthma, and whether they correlated with asthma symptoms or lung function. Children from the SPASM cohort of preschoolers with severe wheeze were reassessed for asthma at school age, and categorized as exacerbators (1 or more severe exacerbations in the previous 12months) or non-exacerbators. Nineteen children (10.9years) were included (9 non-exacerbators, 10 exacerbators). Circulating MAIT-17 were detected by flow cytometry. Their frequency was higher in exacerbators than in non-exacerbators (1.9 [1.01–3.55] vs 0.58 [0.46–1.15], p<0.01). MAIT-17 correlated with the number of severe exacerbations (r=0.68, p<0.001), and correlated negatively with the ACT score (r=−0.55, p=0.01). In summary, MAIT-17 are present in children with asthma and associated with asthma symptoms.



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Subcutaneous immunoglobulins in patients with multiple myeloma and secondary hypogammaglobulinemia

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Publication date: Available online 28 November 2017
Source:Clinical Immunology
Author(s): Angelo Vacca, Carolina Marasco, Assunta Melaccio, Azzurra Sportelli, Ilaria Saltarella, Antonio Solimando, Franco Dammacco, Roberto Ria
Multiple myeloma is commonly associated with a reduction of non-paraprotein immunoglobulins, resulting in a higher risk of infections that represent the leading cause of the patients' death. Therefore, immunoglobulin replacement therapy appears a logical approach.A total number of 46 myeloma patients were enrolled: 24 of them were assigned to receive subcutaneous immunoglobulins, and 22 were controls. The primary endpoint was the evaluation of the annual rate of severe infections in immunoglobulins-receiving patients as compared with those untreated. Subcutaneous immunoglobulins-treated patients showed a significantly lower number of severe infections per year. Adverse events were limited to the site of infusion and were easily manageable. Health-related quality of life was significantly better in subcutaneous immunoglobulins-receiving patients.By decreasing the rate of infections, the prophylactic administration of SCIg improves both adherence to chemotherapy and health-related quality of life, and is cost-effective by reducing the need of hospitalization and the use of antibiotics.



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EAACI/ESCD Skin Allergy Meeting 2017 (SAM 2017)



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Chronic Long-Standing Temporomandibular Joint Dislocation: Report of Three Cases and Review of Literature

Abstract

Purpose

To present three cases of chronic long-standing TMJ dislocation and discuss our treatment protocol with other options in the literature.

Patients and methods

Three cases of chronic TMJ dislocation (more than 4 months) that has never been reduced previously were treated by open reduction, meniscectomy or meniscoplasty and lateral pterygoid muscle myotomy. After a short period of MMF, TMJ physiotherapy was performed.

Results

During 3 years of follow-up, the condition had not recurred at all and all patients were functional and symptom free.

Conclusion

Based on other therapeutic options in the literature, our treatment protocol seems to be an effectual operation with fewer complications.



http://ift.tt/2yHszaZ

Comment on “Low-level laser therapy with 940 nm diode laser on stability of dental implants: a randomized controlled clinical trial”



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Pityriasis rubra pilaris: Algorithms for diagnosis and treatment

Abstract

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease that affects men and women of all ages and also children. The clinical appearance of PRP is highly variable, as is the individual prognosis. Therefore, stratifying PRP into six disease subtypes represents a first step to personalized medicine for this rare inflammatory skin disease. The next step should be to associate specific therapeutic strategies with these subtypes of PRP. However, no randomized, controlled trials on the treatment of PRP have been performed. Consequently, the actual treatment algorithm for PRP will be based on clinical experience, small case series, and case reports. The majority of published evidence is on type I PRP, whereas the treatment experience for other clinical types of PRP is still sparse and has to be gained. Nevertheless, it is now time to start developing valid algorithms as a basis for the diagnosis and treatment of PRP based on the data available.

This review makes use of algorithms developed in psoriasis and atopic eczema and puts together recent insights into the pathogenesis, diagnosis and treatment experience of PRP. The innovative intention of this appraisal is to develop a structured algorithm for PRP treatment that should be further developed going forward.

This article is protected by copyright. All rights reserved.



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The many faces of tuberculosis of the oral mucosa- Three cases with distinct pathomechanisms

Abstract

Tuberculosis (TB) is a chronic infectious disease caused by the acid-fast bacillus Micobacterium tuberculosis. Increased risk of infection occurs with HIV disease, intravenous drug abuse, malignancies, and treatment with immunosuppressants and immunobiologics 1

This article is protected by copyright. All rights reserved.



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Presenting symptoms and long term survival in Head and Neck cancer

Abstract

Objectives

To assess how type and number of symptoms are related to survival in head and neck cancer patients.

Design

Patients were followed-up for over 10 years from the Scottish Audit of Head and Neck Cancer; (national cohort of head and neck cancer patients in Scotland 1999 – 2001). September 2013, cohort was linked to national mortality data. First, second and third presenting symptoms were recorded at diagnosis.

Setting

National prospective audit – Scotland.

Participants

A subset of 1589 patients, from the original cohort of 1895, who had cancer arising from one of the four main subsites; larynx, oropharynx, oral cavity and hypopharynx.

Main outcome measures

Median survival in relation to patients' presenting symptoms.

Results

1146 (72%) males and 443 (28%) females, mean age at diagnosis 64 years (13 – 95). There was a significant difference in survival in relation to the number of the patient's presenting symptoms; one symptom had a median survival of 5.3 years compared with 1.1 years for three symptoms. Patients who presented with weight loss had a median survival of 0.8 years, compared to 4.2 years if they did not (p<0.001). Patients who presented with hoarseness had a median survival of 5.9 years compared to 2.6 years without (p<0.001). There was no significant difference in long term survival for patients who presented with an ulcer, compared to those that did not (p=0.105).

Conclusions

This study highlights the importance of patients' presenting symptoms, giving valuable information in highlighting appropriate "red flag" symptoms and subsequent treatment planning and prognosis.

This article is protected by copyright. All rights reserved.



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Epidemiology of basal cell carcinoma: a 10-year comparative study

Basal cell carcinoma (BCC) is a slow-growing and locally aggressive skin cancer. Despite its high incidence, good quality epidemiological data are sparse. We therefore organised a retrospective study of two separate years' incidence of BCC in one county within the United Kingdom (Dorset) with an interval of 10 years between them. There were 2455 patients in 2006, and 3797 in 2016, who had a new diagnosis with corresponding crude incidences of 459.99 and 491.92/100,000 person-years. The male:female ratio was 1:071 for both years.

http://ift.tt/2ASyr78

Can dietary supplements improve a clinician’s well-being and health?

Many people use dietary supplements to improve their physical and mental well-being and their general health, but do not know if they really have any benefit. To our knowledge, little has been published on their use in the clinical environment, so we evaluated the evidence for their benefits in people whose work is physically and mentally challenging. Studies on nutrition and supplementation in athletes and military personnel have clearly shown that several compounds improve cognition, mental well-being, and physical performance.

http://ift.tt/2yFOEGI

Grafts of autogenous coronoid process to reconstruct the mandibular condyle in children with unilateral ankylosis of the temporomandibular joint: long-term effects on mandibular growth

Injury to the mandibular condyle in children usually leads to malocclusion and disharmony of facial growth. Our aim was to study the facial growth after reconstruction of the mandibular condyle using autogenous coronoid process grafts in children with unilateral ankylosis of the temporomandibular joint (TMJ). We followed up 10 growing patients with unilateral bony ankylosis of the TMJ who had been admitted to the West China Hospital of Stomatology, Sichuan University (Chengdu, China) between 1 January 2008 and 31 December 2012.

http://ift.tt/2AR5t7w

From shortened dental degree to OMFS consultancy: Evaluation of the outcome of the first 5 years of the King's College London Dentistry Programme for Medical Graduates course

Introduction: The Dentistry Programme for Medical Graduates (DPMG) course at King's College London (KCL) remains the only 3-year dental degree for medical graduates seeking to pursue a career in OMFS. Since its inception in 2006 it has enrolled 8-10 students per year with the first of these now taking up consultancy posts in OMFS. Our aim was to evaluate the outcome of this programme with regard the number of graduates entering OMFS specialist training.

http://ift.tt/2yGAWU1

How new NICE guidelines will impact service provision in patients treated for early oral cavity squamous cell carcinoma – a ten-year single centre retrospective review

Introduction: Historically, patients with early oral cavity squamous cell carcinoma (oSCC) without evidence of neck disease have not all been treated with cervical node surgery. New NICE guidelines recommends all patients are offered surgical intervention in the neck (elective neck dissection, ND or sentinel lymph node biopsy (SLNB).

http://ift.tt/2AR5rwq

Dermoscopy – Dermatologists only?

NICE Guidelines for Melanoma, July 2015 recommend "all pigmented lesions that are either referred for assessment or identified during follow-up in secondary or tertiary care" should be assessed "using dermoscopy carried out by healthcare professionals trained in this technique". The aim of this cross- specialty study was to establish the extent and frequency of dermoscopy use in Maxillofacial(OMFS) and Plastic Surgery(PS) clinics/theatres and to also establish how much dermoscopy training the providers had received.

http://ift.tt/2AVQr0j

Plasma Cell Gingivitis – Does it still exist?

We present the case of a rarely seen mucosal pathology, previously referred to in the literature as "plasma cell gingivitis", in an otherwise fit and healthy Caucasian 23-year-old male. This condition was thought to be associated with historical components of toothpaste or chewing gum, and, was thought to be no longer existent with removal of the causative ingredient from these products. However, we would like to highlight that this entity is still present today and colleagues in our specialty can come across these cases being referred for surgical excision.

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Synovial chondromatosis of the temporomandibular joint with intracranial extension: a case report

Synovial chondromatosis (SC) is an uncommon proliferative condition with unknown aetiology, generally affecting the larger joints of the body. The formation of benign cartilaginous nodules occupies the joint space causing symptoms such as pain and swelling. The clinical presentation can be similar to more common TMJ pathologies, meaning SC can get overlooked during the differential diagnosis. The use of image modalities, in particular Computed Tomography and Magnetic Resonance Imaging are essential in the planning of these cases.

http://ift.tt/2AVQoBF

Is it justifiable to admit Orthognathic surgical patients for less than 23hours? A single surgeon's experience

Introduction: To assess how long, on average orthognathic patients are admitted to a ward following their surgery and to investigate if there were any immediate re-admissions or complications after discharging these patients.

http://ift.tt/2yGGmhU

Adenoid Cystic Carcinoma of the head and neck – a survey of current UK practice

Background: Adenoid cystic carcinomas (ACC) comprise

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A service evaluation measuring compliance to conservative management with soft splint therapy and outcomes in patients suffering from temporomandibular disorder (TMD)

Introduction: TMD is a common problem affecting up to 33% of individuals within their lifetime. Only 5% to 10% of patients with TMD symptoms require treatment, with 40% of patients experiencing spontaneous resolution. A long-term follow-up study showed 50% to 90% of patients had significant improvement in their symptoms with conservative management and splint therapy.

http://ift.tt/2yFrGQe

Recurrent pleomorphic salivary adenoma in submandibular gland

Case presentation: A 26-year-old woman presented to our department via her GP with two distinct lumps in her right anterior neck. Clinical history revealed she had surgery in the right submandibular gland in Pakistan several years previously. Ultrasound and fine needle aspiration cytology confirmed a recurrent pleomorphic salivary adenoma in the right submandibular gland. An MRI scan was carried out to aid surgical planning and visualised two separate areas of recurrence. The largest was at the anterior inferior aspect of the submandibular gland, and a smaller focus was as the posterolateral aspect, still within submandibular triangle, but separate from gland.

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Awake emergency tracheostomy - Is it an ethical treatment modality for end of life patients?

Background: Awake tracheostomies are indicated for severe acute severe airway obstruction when other means have failed or are inappropriate. Indications for elective tracheostomies are currently defined by the American Academy of Otolaryngology; however, emergency awake tracheostomies are not routinely performed with only a few studies reported in the literature.

http://ift.tt/2yG3Bsv

Splintless Orthognathic Surgery using Virtual Planning and Patient Specific Printed Titanium Cutting Guides and Osteosynthesis Plates: A case report and review of the technology

Virtual planning is increasingly used in orthognathic surgery and offers a number of advantages over conventional techniques. One of the difficulties however is reproducing these planned movements in accurately executed surgery. A number of techniques have previously been proposed in the literature to remove the reliance on occlusal wafers when positioning osteotomised bone segments, these include the use of tooth or tissue born cutting and or positioning guides. We describe the use of patient specific tissue born cutting guides and osteosynthesis plates in a segmental maxillary osteotomy and genioplasty.

http://ift.tt/2AT1fMP

Intravascular Papillary Endothelial Hyperplasia of the oral cavity – uncommon or undiagnosed?

Background: Oral Intravascular Papillary Endothelial Hyperplasia (IPEH) also known as Masson's pseudoangiosarcoma or vegetant intravascular haemangioendothelioma is a benign, non-neoplastic disorder of vascular origin caused by abnormal proliferation of endothelial cells. It commonly presents as a cutaneous lesion, however, only a few cases of oral manifestations have been reported to date.

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Indicative Numbers for Maxillofacial Training in the UK - Are we at Par?

Introduction: Standardisation of surgical training is a statutory requirement of the GMC Postgraduate board. To ensure that the training is properly structured and balanced, trainees have minimum indicative numbers for procedures that encompass the speciality of OMFS. However, currently there is some variation with the number of procedures done in different hospitals across the country. As a result, trainees may have to travel to units out of their parent deanery to comply with minimum required numbers.

http://ift.tt/2ASysrI

Cutaneous Squamous Cell Carcinoma (CSCC) of the Head and Neck: An analysis of 691 patients over 6 years

Introduction: CSCC are the second most common skin cancer in the head and neck area. Our aim was to delineate the clinical and pathological characteristics to identify the rate of local recurrence and metastasis.

http://ift.tt/2yGuGLW

Creation of a Care Pathway for Temporomandibular Joint Dysfunction (TMD) at a Tertiary Referral Centre

Introduction: Temporomandibular joint dysfunction is a complex disorder, which encompasses a wide range of conditions. There are various conservative and surgical treatment options available, but a lack of evidence and no 'gold standard' protocol can often inhibit consistent and effective patient care.

http://ift.tt/2ATWZg4

An unusual cause of a parotid mass; Osteomyelitis and sequestra of the mandibular ramus secondary to recurrent pericoronitis

Introduction: Osteomyelitis with bony sequestra in the mandible can be seen secondary to dental infections and mandibular fractures. It is unreported that a case of osteomyelitis secondary to pericoronitis should present as a parotid mass.

http://ift.tt/2yG3zRp

A Review into the utilisation of the 2-week-waiting oncology pathway for intra-oral lesions in the Maxillofacial Surgery Department

Background: Due to the limited clinical resources available to maxillofacial departments, correct use of referral pathways is of paramount importance to allow prioritisation of high risk cases and the delivery of optimal care.

http://ift.tt/2AVQ99J

Comparison with clinicoradiologic findings between patients with recovering and those with residual diplopia after surgery of pure orbital blowout fracture

Diplopia is a common symptom of blowout fracture (BOF), and can persist after proper surgical management. We compared clinicoradiological findings between patients with recovering diplopia and those with residual diplopia after surgery for orbital BOF.

http://ift.tt/2CAUz3a

Rearthroscopy of The Temporomandibular Joint: a Retrospective study of 600 Arthroscopies

Arthroscopic surgery is an effective treatment for patients with temporomandibular disorders, releasing symptoms and restoring the mandibular function. In patients with poor arthroscopic outcomes, several options of treatment can be considered such as conservative nonsurgical therapy, open surgery, or a second arthroscopy. The purpose of this study was to evaluate our results after 619 arthroscopies.

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Practical Immunodermatology



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Clinical and Basic Immunodermatology, 2nd edition Anthony A. Gaspari, Stephen K. Tyring and Daniel H. Kaplan, (Eds.). 2017, Springer. 895 pages. $249.00 (eBook $189.00). ISBN 978-3-319-29785-9



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Emerging Therapies for Atopic Dermatitis: TRPV1 Antagonists

Transient receptor potential (TRP) ion channels are important mediators of somatosensory signaling throughout the body. Our understanding of the contribution of TRPs to a multitude of cutaneous physiologic processes has grown substantially in the past decade. TRPV1, one of the better-understood members of this large family of ion channels, impacts multiple pathways involved in pruritus. Further, TRPV1 appears to play a role in maintaining skin barrier function. Together, these properties make TRPV1 a ripe target for new therapies in atopic dermatitis.

http://ift.tt/2j7UsUc

Hibernoma: a rare case of adipocytic tumor in head and neck.

Hibernoma is a rare soft tissue tumor stem from persistent fetal brown fat tissue. This benign tumor may occasionally occur in head and neck area and, in most cases, is characterized by an asymptomatic slow gr...

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FDA Approves Ozenoxacin Cream for Impetigo

The US Food and Drug Administration has approved ozenoxacin cream (Xepi Cream, Medimetriks Pharmaceuticals Inc) 1% for impetigo.
FDA Approvals

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BAD Guidelines for Biologic Therapy for Psoriasis 2017

The British Association of Dermatologists provides evidence-based recommendations on the use of biologic therapies for the treatment of psoriasis and psoriatic arthritis in adults and children.
The British Journal of Dermatology

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FDA OKs Eskata for Raised Seborrheic Keratoses

Eskata is a proprietary, high-concentration hydrogen peroxide-based topical solution applied by a healthcare provider directly to raised seborrheic keratoses using a penlike applicator.
FDA Approvals

http://ift.tt/2yED12Y

Transoral robotic surgery for residual and recurrent oropharyngeal cancers: Exploratory study of surgical innovation using the IDEAL framework for early-phase surgical studies

Abstract

Background

The purpose of this study was to identify the role of transoral robotic surgery (TORS) in the management of residual and recurrent oropharyngeal cancer.

Methods

IDEAL (Idea, Development, Exploration, Assessment, Long-term Follow-up) 2a framework.

Results

Of 26 patients assessed for TORS, 21 underwent the procedure, 5 underwent open resection (4 due to unsuitable anatomy/tumor extent and 1 on the basis of patient choice). Three patients underwent intraoperative ultrasound-assisted robotic resection, and 3 received robotic-assisted free flap inset. A technical refinement for TORS of residual and recurrent oropharyngeal cancer of the tongue base is described. Actuarial plots showed estimated overall survival of 48.2%, local control of 76.6%, and disease-specific survival of 77.1% at 42.6 months.

Conclusion

TORS is a valid management option for residual and recurrent oropharyngeal cancer. Oncologic outcomes are comparable to open surgery and transoral laser microsurgery, with the added advantages of en bloc resections, facility for intraoperative ultrasound imaging, and inset of free flaps without mandibular split.



http://ift.tt/2jYUQW1

Transoral robotic surgery for oropharyngeal squamous cell carcinoma in the era of human papillomavirus

Abstract

Background

The emergence of transoral robotic surgery (TORS) ignited the debate between surgical and nonsurgical strategies on oropharyngeal squamous cell carcinoma (SCC) management; a question further complicated by human papillomavirus (HPV). We evaluated the survival by treatment strategy independently in HPV-related and HPV-nonrelated oropharyngeal SCC.

Methods

The National Cancer Database was queried for patients with oropharyngeal SCC with known HPV status who underwent primary TORS or primary radiotherapy. The overall survival (OS) was compared by treatment strategy, including propensity matching to control for confounders.

Results

Of 1873 patients, 73% were HPV-positive and 30% were treated with TORS. The propensity-matched patients with HPV-positive disease displayed no significant difference in 3-year survival; 95% versus 91% (P = .116) for the TORS versus primary radiotherapy. In the HPV-negative cohort, TORS was associated with superior survival; 84% versus 66% (P = .01).

Conclusion

The TORS-based approach was associated with superior survival in patients with HPV-negative oropharyngeal SCC; similar difference was not observed in patients with HPV-positive disease.



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Intrakranielle Blutung nach Sinusvenenthrombose bei Cochleaimplantation

Zusammenfassung

Die Cochleaimplantation stellt ein sicheres operatives Therapieverfahren mit geringen Komplikationsraten dar. Vor allem intrakranielle Komplikationen sind selten. Wir präsentieren den Fall einer Patientin, bei der es bei schwierigen anatomischen Verhältnissen während der Cochleaimplantation zur Eröffnung des Sinus sigmoideus kam. In der Folge trat eine Sinusvenenthrombose und intrazerebrale Blutung auf. Sowohl die Diagnostik als auch die Therapie dieser Komplikationen werden im vorliegenden Fallbericht erläutert.



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EMLA cream does not influence efficacy and pain reduction during pulsed-dye laser treatment of port-wine stain: a prospective side-by-side comparison

Abstract

EMLA cream was developed to reduce pain during pulsed-dye laser (PDL) treatment; however, no standard assessment for the therapeutic outcomes of PDL with EMLA creams thus far available. This comparative, prospective clinical trial evaluates laser efficacy and pain reduction during PDL treatment with EMLA cream for local topical anesthesia. Nineteen patients with untreated port-wine stain (PWS) were treated using PDL and examined in this study. Treatment specifications included Vbeam® PDL (Candela Corp.), 595-nm wavelength, 9 J/cm2 radiant exposure, 0.45 ms pulse duration, 10 mm spot size, and cryogen spray cooling (40 ms cooling plus a 20 ms delay). A topical anesthetic (EMLA cream: 2.5% lidocaine and 2.5% prilocaine) and a placebo were applied to two respective testing areas on all patients prior to treatment. The visual analog scale (VAS) was used for pain assessment. Clinical therapeutic outcomes were evaluated by visual evaluation and with the use of a chromameter 2 months after 3PDL treatments. The average VAS scores were 3.15 ± 0.95 and 8 ± 0.57 for the EMLA cream site and the placebo site, respectively, at a significance level p < 0.001. The EMLA cream site and the placebo site had clearance or fading rates of 45.08 and 44.12%, respectively (p < 0.05). No serious side effects were reported. Patients reported a consistent decrease in pain during PDL treatment when the topical anesthetic EMLA cream was administered. Treatment of PWS by PDL with EMLA cream does not lead to a decrease in efficacy or an increase in side effects; instead, it significantly reduces pain during treatment. EMLA cream is a safe and effective local topical anesthetic for PWS treatment by PDL.



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Self-medication and access to care in Global Health

By Nathan Douthit

I am a local village doctor. I came to the eye hospital escorted by two people in view of my severe eye condition and blindness. Fortunately, I was immediately relieved of my symptoms and my vision was restored after treatment. I am now completely aware of the ill effects of using home remedies. I will spread awareness about these ill effects to all people in my village. I will also bring other blind people from my village for eye treatment.           

            Above is the case "Self-medication complicating pseudo membranous conjunctivitis" by Singh et al described in the words of the patient. The patient had an episode of conjunctivitis 20 days prior to presentation, "for which he had instilled ghee (clarified butter) as well as goat's milk in his eyes as a remedial measure. Also, he had instilled some antibiotic–steroid combination drops." His condition had worsened to the point of near blindness before he sought out care at a medical center, where he was diagnosed with pseudo-membranous conjunctivitis and treated appropriately.

One aspect of this problem is Non-Degree Allopathic Practitioners (NDAPs). These practitioners practice allopathy, however very few have any formal allopathic training and by definition, none have allopathic degrees. Some are trained in traditional medicine (for example, the AYUSH practitioners—Ayurveda, Yoga and Naturopathy, Unani, Siddha and Homeopathy) while some lack formal training entirely. For many rural residents in India and other countries, these are the first point of contact for acute illnesses.[1] This is likely secondary to the inadequate access to appropriate care, particularly in rural areas, with several other confounding factors including illiteracy, insufficient women's rights, food insecurity and other variables.

This is especially a problem with ophthalmologic problems.[2] While traditional medicine and traditional practitioners certainly play a role in global health, these delays in presentation can prove costly to the patient and their local community.[3] It can lead to decreased public health and long-term morbidity and mortality from treatable illness.

Education and proper care play a key role in scenarios like this. Since this patient had a high status in the local community, he is using his experience to help educate others:           

            Being a local village doctor, he has better access and hold over the local populace, and now, following positive outcomes of eye treatment based on the principles of mainstream medicine, he wants to extend the same advantage to others who require help—particularly the blind and the visually impaired in his locality—by bringing them to our secondary eye care centre for treatment and vision rehabilitation. He is now voluntarily spreading awareness among people and of his own accord discouraging the use of home-made remedies for different ailments, especially of the eyes.

BMJ Case Reports invites authors to publish cases regarding self-medication, delays in care, and inadequate access to care. Global health case reports can emphasize:

-The effects of educational interventions for patients and providers

-Training methods for non-degree allopathic practitioners, and how to incorporate them into the local healthcare system

-Disease spread or exacerbation as a result of self medication

-Successes in treating cases complicated by early inadequate intervention.

Manuscripts may be submitted by students, physicians, nurses or other medical professionals to BMJ Case Reports. For more information, review the blog on how to write a global health case report.

Read more about cultural competence and humility at BMJCR

Consequences of low birth weight, maternal illiteracy and poor access to medical care in rural India: infantile iatrogenic Cushing syndrome

Factors affecting illness in the developing world: chronic disease, mental health and traditional medicine cures

Why tuberculosis control programmes fail? Role of microlevel and macrolevel factors: an analysis from India.

Read more about cultural competence and humility from other sources

-May C, Roth K, Panda P. Non-degree allopathic practitioners as first contact points for acute illness episodes: insights from a qualitative study in rural northern India. BMC health services research. 2014 Apr 23;14(1):182.

-Carvalho RS, Kara-José N, Temporini ER, Kara-Junior N, Noma-Campos R. Self-medication: initial treatments used by patients seen in an ophthalmologic emergency room. Clinics. 2009;64(8):735-41.

-Cambanis A, Yassin MA, Ramsay A, Bertel Squire S, Arbide I, Cuevas LE. Rural poverty and delayed presentation to tuberculosis services in Ethiopia. Tropical Medicine & International Health. 2005 Apr 1;10(4):330-5.

 

[1] May C, Roth K, Panda P. Non-degree allopathic practitioners as first contact points for acute illness episodes: insights from a qualitative study in rural northern India. BMC health services research. 2014 Apr 23;14(1):182

[2] Carvalho RS, Kara-José N, Temporini ER, Kara-Junior N, Noma-Campos R. Self-medication: initial treatments used by patients seen in an ophthalmologic emergency room. Clinics. 2009;64(8):735-41.

[3] Cambanis A, Yassin MA, Ramsay A, Bertel Squire S, Arbide I, Cuevas LE. Rural poverty and delayed presentation to tuberculosis services in Ethiopia. Tropical Medicine & International Health. 2005 Apr 1;10(4):330-5.

Competing Interests

None Declared



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Passende Verordnungsmenge und Applikation von Topika

Zusammenfassung

Hintergrund

Die Abschätzung der notwendigen Verordnungsmenge sowie der Anwendungsmenge von Topika durch den Patienten bereitet in der Versorgung nicht selten Schwierigkeiten. Unzureichende oder unwirtschaftliche Versorgung kann die Folge sein. Um beide Fehlerbereiche zu minimieren, sind einfache Methoden und eine hinreichende Erfahrung in der Abschätzung passender Verordnungsmengen und der Vermittlung verständlicher Applikationsvorgaben wichtig.

Ziel der Arbeit

Es erfolgten die Aufzeigung des aktuellen Publikationsstandes und die Prüfung der Evidenz zur sachgerechten topischen Verordnungsmenge und deren Anwendung durch den Patienten.

Material und Methoden

Es wurde ein Literaturreview auf der Basis einer strukturierten Literaturrecherche in PubMed mit den einzeln und gepoolt eingesetzten Suchbegriffen „local therapy", „topical treatment", „prescription", „amount of ointment needed", „involved area", „BSA", „finger-tip-unit", „Rule of Hand", „calculated dosage" und „rule of nines" durchgeführt. Eingeschlossen wurden wissenschaftliche Originalarbeiten nach manuellem Screening des Titels und Abstracts hinsichtlich der Relevanz.

Ergebnisse

Die Suchstrategie identifizierte nach manueller Selektion 19 relevante Publikationen. Als Maß der genaueren Applikation wurde am häufigsten das Prinzip der Fingertip-Unit (Fingerspitzeneinheit; FTU) eingesetzt. Die sachgerechte Verordnungsmenge wird in Abhängigkeit des Bedarfs pro befallener Fläche berechnet.

Diskussion

Es besteht in Deutschland weiterhin Klärungsbedarf, inwieweit in der Versorgungsroutine optimierte Mengen verordnet und zur Anwendung empfohlen werden. Für die Patienteninstruktion ist die FTU in Verbindung mit der Handflächenregel („Rule of Hand") ein geeignetes Maß zur Selbstapplikation.



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Auswirkungen von Phonationsverdickungen bei professionellen Sängerinnen

Zusammenfassung

Hintergrund

Der Einfluss funktioneller Phonationsverdickungen auf das Schwingungsverhalten der Stimmlippen bei stimmlich herausfordernden Aufgaben ist noch nicht im Detail verstanden.

Material und Methoden

In dieser Studie wurden Glissandi von 220 bis 440 Hz und von 440 bis 880 Hz auf dem Vokal [a:] bei je 4 professionellen Sängerinnen zum einen ohne organischen Befund und ohne Dysphonie (Gruppe A), zum anderen mit funktionellen Phonationsverdickungen (Gruppe B) und zum Dritten mit organischer Dysphonie (Gruppe C) mittels Hochgeschwindigkeitsendoskopie („high speed digital imaging", HSDI, 20.000 Bilder/s), akustischen und elektroglottographischen (EGG-)Signalen untersucht. Anhand der EGG-Stichprobenentropie („sample entropy") wurden Zeitfenster zur Analyse von Registerübergangsphänomen gebildet. Ferner wurden alle Stimmsignale („glottal area waveform", GAW; akustisches und EGG-Signal) einer perzeptiven Bewertung hinsichtlich der Wahrnehmbarkeit von Registrierungsvorgängen unterzogen.

Ergebnisse

Die absolute „sample entropy" zeigte Maxima in Grundfrequenzbereichen, in denen typischerweise Registerübergänge zu finden sind. Gruppe B unterschied sich weder im Rating noch in den Werten der „sample entropy" deutlich von Gruppe A. Die absoluten Werte der „sample entropy" lagen für die Gruppe C nur für das untere Glissando oberhalb der beiden anderen Gruppen. Die größeren Irregularitäten der Stimmlippenschwingungen waren in dieser Gruppe jedoch im oberen Glissando zu beobachten.

Schlussfolgerung

Funktionelle Phonationsverdickungen wirken sich nicht negativ in Hinblick auf die Biomechanik in stimmtechnisch herausfordernden Bereichen wie Registerübergängen aus. Die Verwendung der „sample entropy" als Kriterium zur Detektion von Registerübergängen ist vielversprechend, bedarf jedoch weiterer Validierung.



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