Αρχειοθήκη ιστολογίου

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

Δευτέρα 17 Ιουλίου 2017

Resectable adenocarcinoma developing in the remnant pancreas 7 years after partial pancreatoduodenectomy for invasive ductal adenocarcinoma of the pancreas: a case report

Pancreatic adenocarcinoma still has an excessively high mortality rate and resection is the only potentially curative treatment. The postoperative 5-year survival rate is approximately 20% and recurrence devel...

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Granulomatous reaction to hyaluronic acid filler material in oral and perioral region: A case report and review of literature

Summary

The use of cosmetic fillers agents in orofacial region has become more often used for esthetic concern. Although adverse effects are rare, some patients may develop foreign body reaction to such fillers. Hyaluronic acid (HA) is a biomaterial in the spotlight, because it is normally present in several tissues of human body. The aim of this study was to report a case of a 54-year-old white woman with granulomatous reaction to the HA located in the lips. In addition, a review of the English-language literature of all previously described cases of this condition in oral and perioral region was performed. The location, clinical features, symptoms, time between injection and reaction, type of treatment and treatment outcome of 17 cases were summarized. The clinical and histopathological examination along with a detailed history about this condition is very important to management of patients with nodular lesions in maxillofacial region.



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New imaging tools for an old disease: Secondary syphilis



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When is a sentinel node biopsy indicated for patients with primary melanoma? An update of the ‘Australian guidelines for the management of cutaneous melanoma’

Abstract

A sentinel lymph node biopsy is a surgical staging procedure performed for patients with primary cutaneous melanoma who are clinically lymph-node negative to determine whether there is low volume nodal metastasis in the draining lymph node field. A systematic review was recently performed to update the Australian clinical practice guidelines for the diagnosis and management of melanoma, addressing the question, 'When is a sentinel lymph node biopsy indicated?' This article discusses the findings of the systematic review and the evidence base for the updated guidelines.



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Plaque psoriasis is associated with subclinical atherosclerosis in a Chinese population



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Intermittent administration of parathyroid hormone ameliorated alveolar bone loss in experimental periodontitis in streptozotocin-induced diabetic rats

Publication date: November 2017
Source:Archives of Oral Biology, Volume 83
Author(s): Helin Chen, Taozi Fu, Yuanyuan Ma, Xiangnan Wu, Xianxian Li, Xinyi Li, Jiefei Shen, Hang Wang
ObjectiveIntermittent administration of parathyroid hormone (PTH) has been demonstrated to have anabolic effects on bone metabolism and is approved for use in the treatment of osteoporosis. This study evaluates the role of intermittent PTH administration on alveolar bone loss in streptozotocin (STZ)-induced diabetic rats.DesignFifty male Sprague Dawley rats were randomly divided into the following five groups: (1) a control group (saline placebo without ligature and STZ injection), (2) a PTH group (PTH administration without ligature and STZ injection), (3) an L group (saline placebo with ligature), (4) an L+STZ group (saline placebo with ligature and STZ injection), and (5) an L+STZ+PTH group (PTH administration with ligature and STZ injection). PTH was administered at 75μg/kg per dose four times a week for 28days. Subsequently, all rats were sacrificed, and their mandibles were extracted for micro-computed tomography (micro-CT) scanning, as well as histological and immunochemical evaluation.ResultsMicro-CT scanning demonstrated the anabolic effect of PTH on alveolar bone metabolism in STZ-induced diabetic rats (P<0.05), and histomorphometry indicated that PTH inhibited inflammation of the periodontium and increased the level of osteoblastic activity (P<0.05). Immunochemical evaluation showed that rats subjected to both ligature placement and STZ injection had the highest receptor activator of nuclear factor kappa B ligand (RANKL)/osteoprotegerin (OPG) ratio and that PTH administration decreased this ratio.ConclusionIntermittent systemic PTH administration effectively reduced alveolar bone loss and ameliorated the manifestation of experimental periodontitis in STZ-induced diabetic rats.



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Intermittent administration of parathyroid hormone ameliorated alveolar bone loss in experimental periodontitis in streptozotocin-induced diabetic rats

Publication date: November 2017
Source:Archives of Oral Biology, Volume 83
Author(s): Helin Chen, Taozi Fu, Yuanyuan Ma, Xiangnan Wu, Xianxian Li, Xinyi Li, Jiefei Shen, Hang Wang
ObjectiveIntermittent administration of parathyroid hormone (PTH) has been demonstrated to have anabolic effects on bone metabolism and is approved for use in the treatment of osteoporosis. This study evaluates the role of intermittent PTH administration on alveolar bone loss in streptozotocin (STZ)-induced diabetic rats.DesignFifty male Sprague Dawley rats were randomly divided into the following five groups: (1) a control group (saline placebo without ligature and STZ injection), (2) a PTH group (PTH administration without ligature and STZ injection), (3) an L group (saline placebo with ligature), (4) an L+STZ group (saline placebo with ligature and STZ injection), and (5) an L+STZ+PTH group (PTH administration with ligature and STZ injection). PTH was administered at 75μg/kg per dose four times a week for 28days. Subsequently, all rats were sacrificed, and their mandibles were extracted for micro-computed tomography (micro-CT) scanning, as well as histological and immunochemical evaluation.ResultsMicro-CT scanning demonstrated the anabolic effect of PTH on alveolar bone metabolism in STZ-induced diabetic rats (P<0.05), and histomorphometry indicated that PTH inhibited inflammation of the periodontium and increased the level of osteoblastic activity (P<0.05). Immunochemical evaluation showed that rats subjected to both ligature placement and STZ injection had the highest receptor activator of nuclear factor kappa B ligand (RANKL)/osteoprotegerin (OPG) ratio and that PTH administration decreased this ratio.ConclusionIntermittent systemic PTH administration effectively reduced alveolar bone loss and ameliorated the manifestation of experimental periodontitis in STZ-induced diabetic rats.



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Table of contents



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Masthead



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Hatchery Vaccination Against Poultry Viral Diseases: Potential Mechanisms and Limitations

Viral Immunology , Vol. 0, No. 0.


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Re: “Thyroglobulin as a Functional Biomarker of Iodine Status in a Cohort Study of Pregnant Women in the United Kingdom” by Bath et al. (Thyroid 2017;27:426–433)

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Thyroid , Vol. 0, No. 0.


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A case of acquired epidermodysplasia verruciformis in a renal transplant recipient clearing with multimodal treatment including HPV (Gardasil) vaccination



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Erratum to: The German Lipoprotein Apheresis Registry (GLAR) – almost 5 years on



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BCG — old workhorse, new skills

M Gengenbacher | NE Nieuwenhuizen | SHE Kaufmann

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Non-human primates as a model for understanding the mechanism of action of toll-like receptor-based vaccine adjuvants

Elizabeth A .Thompson | Karin Loré

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Counterclockwise maxillomandibular advancement surgery and disc repositioning: can condylar remodeling in the long-term follow-up be predicted?

This study investigated predictive risk factors of condylar remodeling changes after counterclockwise maxillomandibular advancement (CCW-MMA) and disc repositioning surgery. Forty-one female patients (75 condyles) treated with CCW-MMA and disc repositioning had cone beam computed tomography (CBCT) scans taken pre-surgery, immediately after surgery, and at an average 16 months post-surgery. Pre- and post-surgical three-dimensional models were superimposed using automated voxel-based registration on the cranial base to evaluate condylar displacements after surgery.

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Comment on comparing the outcomes of different compression screw systems in the fixation of mandibular fractures

We read with interest the article by Kotrashetti and Singh1, comparing the use of Herbert type headless compression screws (HCS) with lag screws for the fixation of mandibular fractures, and wish to make a few critical observations.

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Aesthetic outcome and complications of simple interrupted versus running subcuticular sutures in facial surgery: A randomized controlled trial

The suturing technique and its associated complications could affect cosmetic outcome after facial surgery. Literature on this topic is limited.

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Discectomy without replacement improves function in patients with internal derangement of the temporomandibular joint

Treatment of internal derangement is controversial. This study assessed the effectiveness of discectomy without replacement in improving jaw function and decreasing pain.

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Temporal Envelope Coding by Inferior Colliculus Neurons with Cochlear Implant Stimulation

Abstract

Modulations in temporal envelopes are a ubiquitous property of natural sounds and are especially important for hearing with cochlear implants (CIs) because these devices typically discard temporal fine structure information. With few exceptions, neural temporal envelope processing has been studied in both normal hearing (NH) and CI animals using only pure sinusoidal amplitude modulation (SAM) which poorly represents the diversity of envelope shapes contained in natural sounds because it confounds repetition rate and the width of each modulation cycle. Here, we used stimuli that allow independent manipulation of the two parameters to characterize envelope processing by inferior colliculus (IC) neurons in barbiturate-anesthetized cats with CIs. Specifically, the stimuli were amplitude modulated, high rate pulse trains, where the envelope waveform interleaved single cycles ("bursts") of a sinusoid with silent intervals. We found that IC neurons vary widely with respect to the envelope parameters that maximize their firing rates. In general, pure SAM was a relatively ineffective stimulus. The majority of neurons (60 %) preferred a combination of short bursts and low repetition rates (long silent intervals). Others preferred low repetition rates with minimal dependence on envelope width (17 %), while the remainder responded most strongly to brief bursts with lesser sensitivity to repetition rate (23 %). A simple phenomenological model suggests that a combination of inhibitory and intrinsic cellular mechanisms suffices to account for the wide variation in optimal envelope shapes. In contrast to the strong dependence of firing rate on envelope shape, neurons tended to phase lock precisely to the envelope regardless of shape. Most neurons tended to fire specifically near the peak of the modulation cycle, with little phase dispersion within or across neurons. Such consistently precise timing degrades envelope coding compared to NH processing of real-world sounds, because it effectively eliminates spike timing as a cue to envelope shape.



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Temporal Envelope Coding by Inferior Colliculus Neurons with Cochlear Implant Stimulation

Abstract

Modulations in temporal envelopes are a ubiquitous property of natural sounds and are especially important for hearing with cochlear implants (CIs) because these devices typically discard temporal fine structure information. With few exceptions, neural temporal envelope processing has been studied in both normal hearing (NH) and CI animals using only pure sinusoidal amplitude modulation (SAM) which poorly represents the diversity of envelope shapes contained in natural sounds because it confounds repetition rate and the width of each modulation cycle. Here, we used stimuli that allow independent manipulation of the two parameters to characterize envelope processing by inferior colliculus (IC) neurons in barbiturate-anesthetized cats with CIs. Specifically, the stimuli were amplitude modulated, high rate pulse trains, where the envelope waveform interleaved single cycles ("bursts") of a sinusoid with silent intervals. We found that IC neurons vary widely with respect to the envelope parameters that maximize their firing rates. In general, pure SAM was a relatively ineffective stimulus. The majority of neurons (60 %) preferred a combination of short bursts and low repetition rates (long silent intervals). Others preferred low repetition rates with minimal dependence on envelope width (17 %), while the remainder responded most strongly to brief bursts with lesser sensitivity to repetition rate (23 %). A simple phenomenological model suggests that a combination of inhibitory and intrinsic cellular mechanisms suffices to account for the wide variation in optimal envelope shapes. In contrast to the strong dependence of firing rate on envelope shape, neurons tended to phase lock precisely to the envelope regardless of shape. Most neurons tended to fire specifically near the peak of the modulation cycle, with little phase dispersion within or across neurons. Such consistently precise timing degrades envelope coding compared to NH processing of real-world sounds, because it effectively eliminates spike timing as a cue to envelope shape.



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The “Dangers” of Chronic Proton Pump Inhibitor Use

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Publication date: Available online 17 July 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Rena Yadlapati, Peter J. Kahrilas




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Curvature height and distance of MB canal of mandibular molar with Schneider angle and its comparison with canal access angle

Publication date: Available online 17 July 2017
Source:Journal of Oral Biology and Craniofacial Research
Author(s): Manjunath H. Malur, Anil Chandra
AIMTo measure the proposed curvature height and distance of MB canal of mandibular molar with Schneider angle and its comparison with canal access angle both before and after instrumentation, and to compare among each other with multiple linear regression analysis.METHODOLOGYOne hundred human mandibular first and second molars were used for the study. After endodontic access, a size 10 k-file was placed in the mesiobuccal canal extending to the apical foramen and radiographs were taken by using Radiovisiography. Both Schneider and canal access angle with its respective linear counterpart (Curvature height and curvature distance) was measured and calculated by using PLANMECA romexis (Helsinki Finland) software connected to the Intraoral x-ray unit both before and after biomechanical preparation with mesiobuccal canal till size F2. Statistical analysis was done by using multiple linear regression analysis.RESULTSA significant difference was observed between before and after enlargement with respect to Schneider angle and Canal access angle with a t-value being (t=11.0629, p<0.05) (t=10.5773, p<0.05) for before and after enlargement of canals respectively. Percent of change is observed more with curvature height (29.62%) as compared with the curvature distance (8.87%)CONCLUSIONSSchneider angle with its proposed curvature height and distance reduced after instrumentation. Whereas canal access angle decreased and its curvature height and distance have increased after instrumentation.



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Discectomy without replacement improves function in patients with internal derangement of the temporomandibular joint

Publication date: Available online 17 July 2017
Source:Journal of Cranio-Maxillofacial Surgery
Author(s): Michael Miloro, Matthew McKnight, Michael D. Han, Michael R. Markiewicz
PurposeTreatment of internal derangement is controversial. This study assessed the effectiveness of discectomy without replacement in improving jaw function and decreasing pain.Materials and MethodsA retrospective cohort study sample of subjects with internal derangement underwent discectomy without replacement by one surgeon at a single academic medical center. The primary predictor variables were preoperative maximal incisal opening (MIO) and Helkimo Clinical Dysfunction Index (CDI) score. The primary outcome variable was postoperative MIO and CDI score. A paired student's T-test assessed the difference between pre- and post-operative MIO and CDI scores.ResultsPreoperatively, all patients had a clinical dysfunction index of DiIII, indicating severe dysfunction. Postoperatively 14 of 17 subjects (82%) showed marked improvement in mandibular function, and reduction in pain characterized as clinically symptom-free or only small dysfunction (DiO or DiI). One subject improved to DiII and two remained DiIII due to poor compliance. Preoperatively all subjects had an anamnestic index of AiII, representing TMJ locking or severe TMJ or muscle pain. Postoperatively 15 of 17 patients (88%) improved to AiO or AiI, and the two patients with poor compliance remained at AiII, but with marked pain reduction. Of the 17 subjects, the mean pre- and post-operative MIO was 24.2mm and 34.9mm, respectively (p < 0.001). There was a significant difference in pre- and post-operative MIO in subjects with Wilkes III (p = 0.005) and Wilkes IV (p = 0.008), but not Wilkes V (p = 0.7). Mean pre- and post-CDI scores were 17.3 and 3.8, respectively (p < 0.001). When stratifying by Wilkes stage, there was a significant difference in pre- and post-operative CDI in subjects with Wilkes III (p < 0.001) and Wilkes IV (p < 0.001), but not Wilkes V (p = 0.1).ConclusionsIn Wilkes III and IV subjects, discectomy without replacement is effective in improving MIO based upon improvement in objective and subjective assessments.



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Application of phototherapy for the healing of the navels of neonatal dairy calves

Abstract

The present work evaluated the effects of LED light irradiation on the healing of the navels of neonatal dairy calves. Fifty-seven neonatal calves were divided into two groups. Animals had their umbilical stumps immersed in 10% iodine tincture for 60 s, and this process was repeated every 24 h for three consecutive days. The 29 animals in the first group did not receive LED therapy. The 28 animals in the second group received LED light irradiation at 640 nm with 300 mW power, 46.8 J/cm2 energy density, 60 s irradiation time, and 0.385 cm2 spot size. The animals were irradiated at four points (46.8 J/cm2 per point) evenly distributed around the insertion site of the umbilical stump every 24 h for three consecutive days. Irradiation with LED light was applied before the umbilical stumps were immersed in the iodine solution. The time after birth at which the umbilical stump fell off of each calf was noted. The umbilical stumps of all animals fell off by the 25th day of age. After the umbilical stump fell off, the healing of the remnant wound was followed up to the 30th day after birth. The area of the wound was measured on the 15th, 20th, and 25th day after birth using digital photographs and computer-assisted area measurements. A two-tailed unpaired t test was applied to analyze the falling off the umbilical stump, whereas a Kruskal-Wallis one-way ANOVA test with a Dunn's multiple comparison test was used for the wound size evolution. GraphPad Prisma 5.0® and GraphPad StatMate 2.00® were used for the statistical analysis. The results revealed that phototherapy hastened the falling off the umbilical stump, accelerated navel healing, and reduced the mortality rate in newborn calves. Therefore, this study introduced a preventive and adjuvant after birth treatment that proved to be effective in reducing the incidences of omphalitis and newborn mortality.



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The Origins of Oral Medicine in the Hippocratic Collected Works

Abstract

The collected works of Hippocrates describe for the first time in a systematic way a large number of oral diseases, such as ulcers, inflammations, abscesses and tumours from the epiglottis, mouth, tongue, palate, uvula and the sublingual area. Several of these case reports are remarkable for the accurate observation of clinical symptoms and signs, the aetiology, the pathogenesis and their therapeutic approach in relation to prognosis. The Hippocratic authors report cases of 'aphthae' as part of a polysystemic disease, described many centuries later by Behçet and Adamantiades, while they associate features of splenomegaly from endemic malaria with gingivitis ('ulitis'). Benign lip ulcers, caused by sharp teeth bites, were distinguished from the difficult to treat herpes labialis ('herpes') and from the necrotising 'nomae'. Although staphylitis and angina ('kynanche') were attributed to phlegm accumulation, they were recognised as true emergencies when they were associated with a swollen tongue and uvula. Several cases of 'kynanche' with forward displacement of the first cervical vertebrae, atrophy of uvula and oedema of the jaws are illustrated. A fatal outcome was anticipated in cases of 'phagedaenic' ulcers of the teeth, causing necrosis and abscess formation. The therapeutic approach of oral diseases proceeded step by step, starting with simple regimens and progressing to invasive techniques, such as phlebotomy, surgical incisions for fluid drainage and cauterisation. With the aim to avoid adverse events, special attention was paid to the correct timing of surgery and the maintenance of a patent airway with the insertion of small pharyngeal tubes.

This article is protected by copyright. All rights reserved.



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Newcomers in paediatric GI pathology: childhood enteropathies including very early onset monogenic IBD

Abstract

Childhood enteropathies are a group of diseases causing severe chronic (>2–3 weeks) diarrhoea often starting in the first week of life with the potential for fatal complications for the affected infant. Early identification and accurate classification of childhood enteropathies are, therefore, crucial for making treatment decisions to prevent life-threatening complications. Childhood enteropathies are classified into four groups based on the underlying pathology: (i) conditions related to defective digestion, absorption and transport of nutrients and electrolytes; (ii) disorders related to enterocyte differentiation and polarization; (iii) defects of enteroendocrine cell differentiation; and (iv) disorders associated with defective modulation of intestinal immune response. While the intestinal mucosa is usually normal in enteropathies related to congenital transport or enzyme deficiencies, the intestinal biopsy in other disorders may reveal a wide range of abnormalities varying from normal villous architecture to villous atrophy and/or inflammation, or features specific to the underlying disorder including epithelial abnormalities, lipid vacuolization in the enterocytes, absence of plasma cells, lymphangiectasia, microorganisms, and mucosal eosinophilic or histiocytic infiltration. This review intends to provide an update on small intestinal biopsy findings in childhood enteropathies, the "newcomers", including very early onset monogenic inflammatory bowel disease (IBD), in particular, for the practicing pathologist.



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DRESS compliqué d’un syndrome d’activation macrophagique chez un nourrisson traité pour une toxoplasmose congénitale

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Publication date: Available online 17 July 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): S. Rioualen, J. Dufau, C. Flatres, P. Lavenant, L. Misery, J.-M. Roué
IntroductionLe syndrome d'hypersensibilité médicamenteuse, ou drug reaction with eosinophilia and systemic symptoms (DRESS), est une affection grave qui peut se compliquer d'un syndrome d'activation macrophagique (SAM) ; cette association est toutefois rarement décrite chez l'enfant.ObservationNous rapportons le cas d'un nourrisson âgé de cinq semaines hospitalisé pour un syndrome d'activation macrophagique manifesté par une fièvre prolongée, une splénomégalie, une bicytopénie, une hypofibrinogénémie, une hyperferritinémie et une élévation des récepteurs solubles de l'IL-2. La présence d'un exanthème cutané diffus, associé à une hyperéosinophilie majeure et une atteinte multiviscérale, a permis d'évoquer l'association avec un DRESS. Les premières manifestations cliniques étaient apparues 3 semaines après l'instauration d'un traitement par sulfadiazine, pyriméthamine et folinoral pour une toxoplasmose congénitale.DiscussionL'exposition à la sulfadiazine reste le principal facteur imputable au déclenchement du DRESS dans ce cas. Il s'agit probablement de l'événement princeps qui s'est secondairement compliqué du SAM, même si dans notre observation le diagnostic a été évoqué dans un deuxième temps. Le jeune âge de l'enfant était un facteur de risque supplémentaire.ConclusionLe clinicien doit être alerté de la possibilité d'une association DRESS-SAM d'apparition précoce, en lien avec un traitement instauré en période néonatale, dès la fin du premier mois de vie, pour éviter un retard de prise en charge préjudiciable au pronostic.BackgroundDrug reaction with eosinophilia and systemic symptoms (DRESS) is a severe disease that may be complicated by hemophagocytic lymphohistiocytosis but this is rarely described in children.Patients and methodsWe report the case of a 5-week old infant hospitalized in a pediatric intensive care unit for hemophagocytic lymphohistiocytosis with prolonged fever, splenomegaly, cytopenia, fibrinogen≤1.5g/L, ferritin≥500μg/L, and soluble IL-2 receptor≥2400U/mL. As a result of the presence of a diffuse skin rash, eosinophilia and multiple organ failure that started three weeks after the initiation of a congenital toxoplasmosis treatment, association with DRESS was suggested.DiscussionExposure to sulfadiazine remains the main factor leading to DRESS in this case. This is probably the trigger event, secondarily complicated by hemophagocytic lymphohistiocytosis, although in our case the diagnosis was made subsequently. The unfortunately poor outcome of this association is probably exacerbated in fragile patients such as young infants.ConclusionClinicians should be aware of the possibility of DRESS of every early onset associated with hemophagocytic lymphohistiocytosis linked to a treatment started during the neonatal period to avoid any delay in care that might adversely affect the prognosis.



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Vitiligo dévéloppé sur une mélanocytose dermique (nævus de Ota) chez une patiente à peau foncée

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Publication date: Available online 17 July 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): K.-C. Ahogo, I.-P. Gbery, B. Vagamon, O.-A. Ouattara, K.-A. Kouassi, H.-S. Kourouma, Y.-I. Kouassi
IntroductionLe nævus de Ota est une pathologie rare, observée plus fréquemment chez des personnes d'ascendance asiatique. Il se présente cliniquement comme une hyperpigmentation maculeuse gris-bleu unilatérale du visage. La transformation en mélanome et le glaucome sont les principaux risques. L'apparition de lésions de vitiligo avec poliose au sein d'un nævus de Ota est exceptionnelle.ObservationUne femme de 22 ans, aux antécédents de nævus de Ota, consultait pour une dépigmentation des cils. L'examen physique trouvait une hyperpigmentation en nappe orbitofrontale droite, des macules achromiques ainsi qu'une poliose des cils. Le diagnostic de vililigo developpé sur nævus de Ota était retenu. L'examen ophtalmologique trouvait une hyperpigmentation de la sclérotique. Un suivi régulier en dermatologie et en ophtalmologie était institué.DiscussionLe vitiligo est une affection caractérisée par le développement de lésions dépigmentées secondaires à une disparition mélanocytaire chronique dans l'épiderme et le follicule pileux. Sa survenue sur des nævus congénitaux et sur des mélanomes a déjà été rapportée, mais son apparition au sein d'une mélanocytose dermique est très rare. Depuis la première observation de cette association en 1979, seuls 4 autres cas ont été rapportés. Les mécanismes pathogéniques de cette association sont encore mal élucidés. L'examen histopathologique montre en général une perte des mélanocytes épidermiques, alors que les mélanocytes dermiques ne sont pas affectés. Le vitiligo développé sur mélanocytose dermique résulterait ainsi de différences entre les mélanocytes normaux (épidermiques) et ectopiques (dermiques).ConclusionL'association vitiligo/nævus de Ota est exceptionnelle. Nous en rapportons un nouveau cas sur peau foncée.BackgroundNevus of Ota is a rare disease most frequently found in Asians. It presents clinically as a bluish gray hyperpigmentation of one side of the face. Transformation into melanoma and glaucoma are the main risks. The appearance of vitiligo lesions with poliosis within a nevus of Ota is exceptional.Patients and methodsA 22-year-old female patient with a nevus of Ota consulted for depigmentation of the eyelashes. Physical examination revealed hyperpigmentation in the right orbitofrontal part of her face, achromic macules and eyelash poliosis. A diagnosis was made of vitiligo developing on a nevus of Ota. Ophthalmologic examination showed hyperpigmentation of the sclera. Regular dermatologic and ophthalmologic follow-up was instituted.DiscussionVitiligo is a condition characterized by the development of depigmented lesions secondary to chronic degradation of the melanocytes of the epidermis and the follicles. Its occurrence on congenital nevus and melanoma has already been reported. However, its appearance in dermal melanocytosis is very rare. Since the first observation of this association in 1979, only 4 other cases have been reported. The pathogenic mechanisms of this association are still poorly understood. Histopathological examination generally shows a loss of epidermal melanocytes, especially in the basal layer, while dermal melanocytes remain unaffected. In this context, vitiligo developed on dermal melanosis appears to result from the difference between the properties of normal (epidermal) melanocytes and ectopic (dermal) melanocytes.ConclusionAssociation of vitiligo with nevus of Ota is rare. Herein, we report a new case in a dark-skinned subject.



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Tumeurs à cellules granuleuses multiples chez un enfant atteint d’un syndrome de Noonan compliqué de leucémie myélomonocytaire juvénile

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Publication date: Available online 17 July 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): J. Castagna, J. Clerc, A.-S. Dupond, C. Laresche
IntroductionLa tumeur à cellules granuleuses (TCG) est une tumeur rare constituée de cellules de Schwann. Nous rapportons le cas d'un enfant atteint d'un syndrome de Noonan compliqué de leucémie myélomonocytaire juvénile (LMMJ) et qui a développé aussi une forme multiple de TCG. Les mécanismes moléculaires qui pourraient expliquer une telle association sont discutés.ObservationUn garçon de 6 ans atteint de syndrome de Noonan compliqué de LMMJ présentait trois nodules sous-cutanés asymptomatiques du dos, de l'avant-bras et du cou. L'analyse histologique montrait des TCG. Une revue de la littérature montre que sept cas de syndrome de Noonan présentant des TCG ont été décrits, aucun en association à une LMMJ. La mutation du gène PTPN11, via l'hyperactivation du signal Ras intracellulaire, pourrait être responsable du développement des TCG et de la LMMJ chez les enfants souffrant d'un syndrome de Noonan.DiscussionUn examen clinique détaillé est recommandé chez un enfant présentant une TCG, pour rechercher une forme multiple et des signes malformatifs orientant vers un syndrome génétique. Notre observation correspond au premier cas décrit de syndrome de Noonan compliqué de LMMJ associé à des TCG multiples. Cette association soulève une fois de plus l'importance de la voie de signalisation Ras-MAPK dans le développement des tumeurs bénignes ou malignes, solides ou sanguines, associées aux syndromes génétiques.BackgroundGranular cell tumour (GCT) is a rare form of tumour comprising Schwann cells. Herein, we report a case of a child presenting Noonan syndrome complicated by juvenile myelomonocytic leukaemia (JMML) and who also developed a multiple form of GCT. We discussed the molecular mechanisms that might account for this association.Patients and methodsA six-year-old boy with Noonan syndrome complicated by JMML presented three asymptomatic subcutaneous nodules on his back, forearm and neck. Histological analysis revealed GCT. A literature review revealed seven cases of Noonan syndrome presenting GCT, none of which were associated with JMML. Mutation of gene PTPN11, via hyperactivation of intracellular Ras signalling may cause the development of GCT and JMML in children presenting Noonan syndrome.DiscussionDetailed clinical examination is recommended in children presenting GCT to screen for multiple forms and for signs of malformation suggestive of a genetic syndrome. Ours is the first case to be described of Noonan syndrome complicated by JMML associated with multiple GCT. This association once again raises the important question of the role of the Ras-MAPK signalling pathway in the development of benign and malignant tumours of solid organs or blood, associated with genetic syndromes.



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X-Linked Agammaglobulinaemia: Outcomes in the modern era

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Publication date: Available online 17 July 2017
Source:Clinical Immunology
Author(s): Ben Shillitoe, Andrew Gennery
Colonel Ogden Bruton reported X-Linked Agammaglobulinaemia in 1952 and treated the child with replacement immunoglobulin therapy. Over 60years later, the treatment for XLA has largely remained unchanged. Replacement immunoglobulin lacks the isotypes IgA and IgM, leading to concerns that patients continue to experience recurrent sinopulmonary tract infections and be at increased risk of bronchiectasis. There is potential hope of earlier diagnosis with newborn screening, and a potential cure for these patients, in the form of gene therapy. However, it is first necessary to evaluate current management and outcomes to aid decisions regarding further research and clinical trials. This article reviews current management and outcomes of XLA, whilst identifying gaps in our knowledge base that may need answering before we proceed with novel diagnostic methods and treatment for XLA.



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Erratum zu: SOP – Darmpassagestörung in der Palliativmedizin



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A Study of MGD013 in Patients With Unresectable or Metastatic Neoplasms

Conditions:   Advanced Solid Tumors;   Non-small Cell Lung Cancer;   Squamous Cell Carcinoma of the Head and Neck;   Small Cell Lung Cancer;   Diffuse Large B Cell Lymphoma;   Epithelial Ovarian Cancer
Intervention:   Biological: MGD013
Sponsor:   MacroGenics
Not yet recruiting - verified July 2017

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Otolaryngology externships and the match: Productive or futile?

Objectives/Hypothesis

The objectives of this study were to summarize externship experiences among recent graduates and current residents in otolaryngology residency programs and determine whether externships affect the match process.

Study Design

Cross-sectional survey.

Methods

A survey was distributed to otolaryngology residents in allopathic US residency programs and otolaryngology graduates from the past 5 years (2011–2015). There were 2,141 surveys distributed.

Results

There were 654 subjects who responded, for a 30.5% response rate. Most respondents were residents (n = 438, 67%). Of the residents, 85.6% had completed at least one externship compared to 75.9% of graduates (P = .002). The most common reasons for selecting a particular externship were geographic location (74.2%) and program reputation (71.1%), whereas the most common reason for not completing an externship was being advised not to (59.1%). Furthermore, 82.6% of respondents received at least one interview from their externships, 90% went to those interviews, and 89.1% reported that externship experiences affected their rank list. Respondents had a 32.7% match rate to the externship residency program if it was ranked versus a 48.8% match rate if the program was ranked first. Respondents who matched at the externship residency program matched higher on their rank list (P < .001). Of the respondents, 90.7% found externships to be valuable, and 74.5% recommended completing one.

Conclusions

Externships are beneficial because they influence the rank list of applicants and are viewed as valuable experiences. Completing an externship is advisable for the experience, but applicants should weigh the benefits versus the possible risk of being judged more harshly during a month-long rotation.

Level of Evidence

NA Laryngoscope, 2017



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Issue Information



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Announcement



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Comparison of two stroke volume variation-based goal-directed fluid therapies for supratentorial brain tumour resection: a randomized controlled trial

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Abstract
Background: The optimal volume status for neurosurgery has yet to be determined. We compared two fluid protocols based on different stroke volume variation (SVV) cut-offs for goal-directed fluid therapy (GDFT) during supratentorial brain tumour resection.Methods: A randomized, single-blind, open-label trial was conducted. Eighty adult patients undergoing elective supratentorial brain tumour resection were randomly divided into a low SVV and a high SVV group. The SVV cut-offs were used to determine when to initiate colloid infusion. Clinical outcomes and perioperative changes in serum neuronal biomarkers, including S100β, neurone-specific enolase (NSE) and glial fibrillary acidic protein (GFAP), were compared.Results: Patients in the low SVV group received a higher volume of colloid [869 (SD 404) vs 569 (453) ml; P=0.0025], had a higher urine output [3.4 (2.4) vs 2.5 (1.7) ml kg–1 h–1; P=0.0416] and a higher average cardiac index [3.2 (0.7) vs 2.8 (0.6) litres min–1 m–2; P=0.0204]. Patients in the low SVV group also had a shorter intensive care unit stay [1.4 (0.7) vs 2.6 (3.3) days, P=0.0326], fewer postoperative neurological events (17.5 vs 40%, P=0.0469), attenuated changes in the NSE and GFAP levels, lower intraoperative serum lactate and a higher Barthel index at discharge (all P<0.05).Conclusions: During GDFT for supratentorial brain tumour resection, fluid boluses targeting a lower SVV are more beneficial than a restrictive protocol.Clinical trial registration. NCT02113358.

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What do recent human studies tell us about the association between anaesthesia in young children and neurodevelopmental outcomes?

Abstract
Anaesthetic and sedative drugs transiently disrupt normal neural activity to facilitate healthcare procedures in children, but they can also cause long-term brain injury in experimental animal models. The US Food and Drug Administration (FDA) has recently advised that repeated or lengthy exposures to anaesthetic and sedative drugs prior to 3 yr of age have the potential to harm the development of children's brains and added warnings to these drug labels. Paediatric anaesthesia toxicity could represent a significant public health issue, and concern about this potential injury in children has become an important issue for families, paediatric clinicians and healthcare regulators. Since late 2015, important new data from five major clinical studies have been published. This narrative review aims to provide a brief overview of the preclinical and clinical literature, including a comprehensive review of these recent additions to the human literature. We integrate these new data with prior studies to provide further insights into how these clinical findings can be applied to children.

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Duration of general anaesthetic exposure in early childhood and long-term language and cognitive ability

Abstract
Background. The anaesthetic dose causing neurotoxicity in animals has been evaluated, but the relationship between duration of volatile anaesthetic (VA) exposure and neurodevelopment in children remains unclear.Methods. Data were obtained from the Western Australian Pregnancy Cohort (Raine) Study, with language (Clinical Evaluation of Language Fundamentals: Receptive [CELF-R] and Expressive [CELF-E] and Total [CELF-T]) and cognition (Coloured Progressive Matrices [CPM]) assessed at age 10 yr. Medical records were reviewed, and children divided into quartiles based on total VA exposure duration before age three yr. The association between test score and exposure duration quartile was evaluated using linear regression, adjusting for patient characteristics and comorbidity.Results. Of 1622 children with available test scores, 148 had documented VA exposure and were split into the following quartiles: ≤25, >25 to ≤35, >35 to ≤60 and >60 min. Compared with unexposed children, CELF-T scores for children in the first and second quartiles did not differ, but those in the third and fourth quartiles had significantly lower scores ([3rd quartile – Unexposed] -5.3; 95% confidence interval [CI], (-10.2 – -0.4), [4th quartile – Unexposed] -6.2; 95% CI, (-11.6 – -0.9). CELF-E showed similar findings, but significant differences were not found in CELF-R or CPM for any quartile.Conclusions. Children with VA exposures ≤35 min did not differ from unexposed children, but those with exposures >35 min had lower total and expressive language scores. It remains unclear if this is a dose-response relationship, or if children requiring longer exposures for longer surgeries have other clinical reasons for lower scores.

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How big data shape paediatric anaesthesia

Paediatric anaesthesia practise is changing. This change is primarily the result of new evidence emerging from analyses of large datasets and represents a shift in focus from prevention of perioperative mortality towards a reduction of perioperative morbidity. Children do offer the unique opportunity to study the lifelong consequences of any early intervention. The effects of early-life surgical and anaesthetic exposure may resonate through adolescence and into adulthood. Therefore, it appears necessary to perform long-term follow-up over several years until higher cognitive and other physiological functions have been fully matured and developed.

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Dexmedetomidine pharmacokinetic–pharmacodynamic modelling in healthy volunteers: 1. Influence of arousal on bispectral index and sedation

Abstract
Background. Dexmedetomidine, a selective α2-adrenoreceptor agonist, has unique characteristics, such as maintained respiratory drive and production of arousable sedation. We describe development of a pharmacokinetic–pharmacodynamic model of the sedative properties of dexmedetomidine, taking into account the effect of stimulation on its sedative properties.Methods. In a two-period, randomized study in 18 healthy volunteers, dexmedetomidine was delivered in a step-up fashion by means of target-controlled infusion using the Dyck model. Volunteers were randomized to a session without background noise and a session with pre-recorded looped operating room background noise. Exploratory pharmacokinetic–pharmacodynamic modelling and covariate analysis were conducted in NONMEM using bispectral index (BIS) monitoring of processed EEG.Results. We found that both stimulation at the time of Modified Observer's Assessment of Alertness/Sedation (MOAA/S) scale scoring and the presence or absence of ambient noise had an effect on the sedative properties of dexmedetomidine. The stimuli associated with MOAA/S scoring increased the BIS of sedated volunteers because of a transient 170% increase in the effect-site concentration necessary to reach half of the maximal effect. In contrast, volunteers deprived of ambient noise were more resistant to dexmedetomidine and required, on average, 32% higher effect-site concentrations for the same effect as subjects who were exposed to background operating room noise.Conclusions. The new pharmacokinetic–pharmacodynamic models might be used for effect-site rather than plasma concentration target-controlled infusion for dexmedetomidine in clinical practice, thereby allowing tighter control over the desired level of sedation.Clinical trial registration. NCT01879865.

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Ultrasound-guided percutaneous cryoneurolysis for treatment of acute pain: could cryoanalgesia replace continuous peripheral nerve blocks?

Local anaesthetics, delivered percutaneously through a needle, have been used for over a century to provide perioperative anaesthesia and analgesia. However, the duration of a single-injection peripheral nerve block is usually limited to less than 24 hr, leaving untreated surgical pain that may last for weeks—or in some cases months. While prolonged analgesia may be provided using a perineural catheter and repeated/continuous administration of local anaesthetic, the duration of this modality is still usually limited to less than one week because of the risk of infection, rapid consumption of the local anaesthetic, and the burden of carrying an infusion pump and anaesthetic reservoir.1 An analgesic modality with a prolonged duration of action could be advantageous for various surgical procedures that are associated with a typically prolonged postoperative period of pain.

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Inhibition of p75 neurotrophin receptor does not rescue cognitive impairment in adulthood after isoflurane exposure in neonatal mice

Abstract
Background: Isoflurane is widely used for anaesthesia in humans. Isoflurane exposure of rodents prior to post-natal day 7 (PND7) leads to widespread neurodegeneration in laboratory animals. Previous data from our laboratory suggest an attenuation of apoptosis with the p75 neurotrophin receptor (p75NTR) inhibitor TAT-Pep5. We hypothesized that isoflurane toxicity leads to behavioural and cognitive abnormalities and can be rescued with pre-anaesthesia administration of TAT-Pep5.Methods: Neonatal mouse pups were pretreated with either TAT-Pep5 (25 μl, 10 μM i.p.) or a scrambled control peptide (TAT-ctrl; 25 μl, 10 μM i.p.) prior to isoflurane exposure (1.4%; 4 h) or control (n = 15–26/group). Three to 5 months after exposure, behavioural testing and endpoint assays [brain volume (stereology) and immunoblotting] were performed.Results: No significant difference was observed in open field, T-maze, balance beam or wire-hanging testing. The Barnes maze revealed a significant effect of isoflurane (P = 0.019) in errors to find the escape tunnel during the day 5 probe trial, a finding indicative of impaired short-term spatial memory. No difference was found for brain volumes or protein expression. TAT-Pep5 treatment did not reverse the effects of isoflurane on neurocognitive behaviour.Conclusion: A single isoflurane exposure to early post-natal mice caused a hippocampal-dependent memory deficit that was not prevented by pre-administration of TAT-Pep5, although TAT-Pep5, an inhibitor of p75NTR, has been shown to reduce isoflurane-induced apoptosis. These findings suggest that neuronal apoptosis is not requisite for the development of cognitive deficits in the adults attendant with neonatal anaesthetic exposure.

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Variations in assessment and certification in postgraduate anaesthesia training: a European survey

Abstract
Background: Postgraduate specialty training has traditionally been based on a time- and rotation-based model, but competency-based models are emerging. Because anaesthesia training evolves differently across Europe, variations in assessment and certification processes are expected, but the extent of similarities and differences is unknown. The aim of this study was to compare anaesthesia training programmes in Europe, focusing on assessment and certification processes.Methods: We performed an online survey among national representatives of the Union of European Medical Specialists/European Board of Anaesthesiology. Results: All 36 countries participated. Duration of training had a median of 5 yr (range 2.75–7). Mean number of different assessment tools was 7.45 (range 4–13), with more tools being used in competency-based programmes [mean 9.1 (sd 2.97) vs 7.0 (sd 1.97); P=0.03]. Most countries had a nationally uniform certification process. Based on a qualitative analysis of the survey findings, a categorization of countries emerged, reflecting the approach to assessment and certification. We observed two main streams of countries with an underlying knowledge or procedural focus within a time- and rotation-based apprenticeship model. These main streams are evolving, to different extents, towards a third orientation, competency-based training.Conclusions: Assessment and certification processes in European anaesthesia training are diverse. In many countries, a time-based apprenticeship model is evolving towards a competency-based certification process. This diversity precludes comparison of competence of graduating anaesthetists across Europe.

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Acute rehabilitation after resuscitative endovascular balloon occlusion of the aorta (REBOA) in major trauma

We report a 23-year-old woman admitted post cyclist versus heavy goods vehicle accident in December 2014. This was the second case the life-saving procedure, that is, resuscitative endovascular balloon occlusion of the aorta (REBOA) was performed on at the roadside. This advanced procedure was performed due to the extensive haemorrhage from this patient's complex pelvic fracture . As a result of REBOA, the patient consequently lost her left lower limb and underwent a variety of complex pelvic and lower limb surgeries.

The patient was admitted to the acute critical care unit and underwent repeated operations and was not ready to start active rehabilitation until 12 days into her admission. Prior to this she was on movement restrictions and received physiotherapy for limb care and dietetics in order to meet her nutritional requirements. The patient was stepped down to a ward setting and started on an extensive physiotherapy programme and was then transferred to the rehabilitation unit for amputees at Roehampton.



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Uncommon cause of cervicobrachial neuralgia: epidural abscess complicating tuberculous arthritis

Description

A 59-year-old patient was admitted to the rheumatology department with cervicobrachial neuralgia in the upper extremities and paresthesia. The symptoms appeared 3 months ago.

Physical examination showed neck stiffness with decreased sensitivity in the territory of the C4 root in both arms. There was no motor weakness or fever. The inflammatory blood markers were increased.

Spinal MRI was performed showing posterior epidural collection hyperintense in T2-weighted images (figure 1) with intense peripheral enhancement extending from C3-C4 to C4-C5 (figure 2). A facet joint arthritis at the level of C3-C4 was also noticed with a T2 hyperintense lesion of the spinal cord (figure 1).

Figure 1

T2-weighted sagittal and axis images showing posterior epidural mass with mass effect on the spinal cord at C3-C4 and medullar oedema.

Figure 2

T1FAT SAT post-gadolinium axial images showing intense...



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Customised crescentic corneal transplant with conjunctival resection in an atypical case of malignant Moorens ulcer

Description

Mooren's ulcer is a painful peripheral ulcerative keratitis of unknown aetiology. It is rapidly progressive inflammatory destruction of the juxtalimbal corneal stroma with no associated scleritis. Mooren's ulcer is a diagnosis of exclusion, after ruling out all the relevant systemic and local causes that lead to peripheral ulcerative keratitis. It usually present as bilateral disease with male to female distribution of 1:0.74 being more common in older age group.1 Mooren's ulcer has been seen to be associated with human leucocyte antigen (HLA)-DR17(3) and DQ2.2

A 20-year-old male patient presented with complaints of sudden onset pain, redness and watering in the left eye. History revealed recurrent episodes of similar complaints for the last 2 years. On examination, the visual acuity in the left eye was counting finger at 2 m and 20/20 in the right eye. The slit-lamp biomicroscopic examination showed juxtalimbal severe congestion associated with 2x2 mm of...



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Social considerations of inflammatory bowel disease in Southern Israel

Chronic diseases pose unique social challenges beyond traditional health considerations that require specific attention. In this report, we examine the case of a middle-aged woman with ulcerative colitis, living in Southern Israel. Trust between the patient and physician is shown to positively influence a variety of therapeutic outcomes and should be considered a fundamental component of successful care. In context of the military conflict between Israel and Gaza, the needs of patients with chronic diseases cannot be forgotten. The work environment is also identified as an area of particular concern, as a supportive work environment is essential in order to maintain satisfaction in the workplace and sustain a high quality of life. Out-of-pocket costs for medications are confirmed to be a significant barrier to adherence. Better understanding of patientsâ™ financial capabilities, along with affordable therapeutic interventions, will alleviate healthcare-related financial burdens and improve health outcomes.



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Increased risk for thionamide-induced agranulocytosis in elderly patients: a case presentation and literature review

Thionamides, such as methimazole and propylthiouracil, are used for the management of hyperthyroidism. Agranulocytosis is a rare adverse effect of thionamides and elderly patients are especially vulnerable. Here we discuss a case of an 80-year-old woman who developed agranulocytosis and pneumonia approximately 4 weeks after starting low dose methimazole therapy. Despite aggressive treatment with broad-spectrum antibiotics and granulocyte colony stimulating factor, she developed multiorgan failure and died. Our goals are to identify risk factors common to elderly patients and hopefully improve outcomes in this population when prescribed thionamides.



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Thrombotic microangiopathy associated with intravenous injection of extended-release oxycodone

We describe the case of a 35-year-old man presenting with thrombotic microangiopathy (TMA) and renal impairment following, as he later disclosed, intravenous injection of oral formulation tamper-resistant extended-release oxycodone hydrochloride (Oxycontin). Recurrent misuse of this agent was associated with relapsing TMA despite treatment with terminal complement inhibitor eculizumab. Cases of TMA have been reported in the USA in association with intravenous misuse of extended-release oxymorphone (Opana ER) after the introduction of a new non-crushable formulation in 2012. There are two reported accounts of TMA associated with tamper-resistant Oxycontin, which became available in Australia in 2014. This is the first documented case in which eculizumab was used. This case illustrates the practical diagnostic challenges in identifying TMA disorders, and the importance of a detailed drug history. It also highlights the need to clarify what role, if any, eculizumab therapy has in cases of drug-associated TMA.



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Chewing-induced facial dystonia: a rare presentation of task-specific dystonia

This case is an addition to scarce literature available for a rare condition, chewing-induced task-specific dystonia. The patient was a 63-year-old woman who presented with a 4-year history of progressive difficulty in eating food only during chewing associated with abnormal facial grimaces without any difficulty in drinking, swallowing, speaking or singing. Examination revealed dystonia of facial muscles every time she chewed but absent during drinking and speaking. As movements were consistent and reproducible with the specific task, other differential diagnosis like motor tics, psychogenic disorder, tardive dystonia and parkinsonism syndrome were excluded leading to a diagnosis of task-specific facial dystonia triggered by chewing. Treatment was started with trihexyphenidyl and later on tetrabenazine was also added but she got only mild relief of symptoms. As she did not agreed for botulinum toxin therapy, so we continued with the same treatment without much improvement.



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Localised pneumoperitoneum following liver mass biopsy in the setting of post-ERCP pneumobilia

Description

A 72-year-old woman presented with a 1-week history of fatigue, weight loss and painless jaundice. Liver chemistries confirmed cholestasis with hyperbilirubinaemia of 25.6 mg/dL. An abdominal CT scan revealed multifocal liver lesions and dilation of intrahepatic and extrahepatic bile ducts. On endoscopic retrograde cholangiopancreatography (ERCP), a common bile duct stricture was found requiring dilation and insertion of a biliary stent. Cholangiogram demonstrated free bile drainage without contrast medium leakage. The patient then underwent CT-guided biopsy of a left lobe liver mass. On the next day, she complained of constant abdominal pain. She was afebrile and there were no peritoneal signs. Abdominal CT scan demonstrated localised free air in the peritoneal cavity (figure 1). The stent was at the proper position and pneumobilia of the common bile duct and intrahepatic bile ducts was noted. The occurrence of free air in the peritoneal cavity post-ERCP raised significant concerns...



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A rare consequence of very common osteoporotic pubic rami fracture in a patient with myeloproliferative disease

Description

An 80-year-old female with janus kinase 2 positive myeloproliferative disease with stable blood parameters on aspirin and anagrelide admitted to the emergency department after a fall over the kerb complaining of pain limiting mobility. Initial assessment was stable without any external injuries noted. Pelvic X-ray demonstrated a stable pubic rami fracture (figure 1). Later on, her haemoglobin dropped 106 g/dL to 67 g/dL for no apparent cause. She was also noted to have an abdominal fullness and examination revealed a suprapubic mass. CT scan showed a well-defined hypointense collection measuring 85x60x105 mm extending superiorly from the symphysis pubis compressing bladder (figure 2-4). It was compatible with a late development of a haematoma. There was no contrast extravasation suggesting active bleeding. Her platelets and coagulation were normal. She was managed conservatively with blood transfusions but there was a place for angiography if she was...



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Multiple Osteolytic Lesions Causing Hypercalcemia: A Rare Presentation of Acute Lymphoblastic Leukemia

Acute lymphoblastic leukemia is characterized by unchecked proliferation of malignant lymphoblasts which replaces the normal bone marrow culminating in anemia due to red blood cells inadequacy as well as in easy bruising/bleeding secondary to insufficient platelets production. Even the white blood cells which are produced excessively are immature and abnormal. ALL is the most common hematological malignancy in children. Most commonly, patients present with lymphadenopathy, recurrent infections, bleeding, fatigue, and bone pains. Bone pains, often particularly involving long bones, occur in about 21–38% of cases and are due to overcrowding of bone marrow with malignant cells. Vast majority of children with ALL have thrombocytopenia and/or anemia with a normal or mildly elevated white blood cells count with the presence of lymphoblasts on peripheral smear. About 50% of children present with bleeding while about 75% of patients have platelet count 100,000/microL. Visceromegaly is not uncommon but osteolytic lesions and hypercalcemia are rather uncommon. We present a 22-year-old gentleman with generalized fatigue and bone pains without visceromegaly. There was severe hypercalcemia with normal parathyroid levels but multiple osteolytic lesions. Peripheral smear showed anemia without blasts, whereas a bone marrow biopsy revealed > 30% blasts with interspersed CD 10 positive cells.

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Mechanical performance of cement– and screw–retained all–ceramic single crowns on dental implants

Abstract

Objectives

This in-vitro study was performed to compare the contact wear, fracture strength and failure mode of implant-supported all-ceramic single crowns manufactured with various fabrication and fixation concepts.

Materials and methods

Fifty dental implants (Conelog Ø 4,3mm/L11mm, Camlog Biotechnologies AG) were embedded and treated with all-ceramic molar single-crowns. Three groups received hand-layered zirconia crowns (IPS e.max Ceram/ IPS e.max ZirCAD, Ivoclar Vivadent AG): CZL (cement-retained zirconia-based layered) group crowns were cemented conventionally, SZL (screw-retained zirconia-based layered) group crowns were screw-retained, MZL (modified zirconia-based layered) group crowns showed a different coping design with screw retention. The specimens of SST (screw-retained sintering-technique) and SFL (screw-retained full-contour lithium-disilicate) group were CAD/CAM (Computer-aided design/computer-aided manufacturing) fabricated in the sintering technique (IPS e.max ZirCAD/IPS e.max CAD, Ivoclar Vivadent AG) and full-contour of lithium disilicate (IPS e.max CAD, Ivoclar Vivadent AG) respectively and screw-retained. All specimens underwent artificial aging, load until failure and a scanning electron microscopy (SEM) analysis. The received data were statistically compared (one-way ANOVA; Student-Newman-Keuls test; Mann-Whitney U-test) at a significance level of 5%.

Results

Mouth-motion fatigue testing caused two abutment fractures (SST group and SZL group) and two chipping events (CZL group). Specimens of MZL group showed statistically significant less contact wear compared to the other groups (p<0.001). There was no statistical difference between the groups in terms of the maximum fracture load. SEM-analysis showed a more homogenous structure and surface of CAD/CAM fabricated specimens towards manually veneered components.

Conclusions

The mode of retention did not influence the fracture resistance but the failure patterns of the specimens. CAD/CAM milled lithium-disilicate crowns seemed to be a preserving factor for dental implants.

Clinical relevance

The mode of retention and veneering influences the mechanical performance of implant-supported single crowns.



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Adjustable thermoplastic oral appliance versus positive airway pressure for obstructive sleep apnea

Objectives/Hypothesis

To compare outcomes of continuous positive airway pressure (CPAP) and the adjustable thermoplastic mandibular advancement splint (AT-MAS) for obstructive sleep apnea treatment.

Study Design

Randomized crossover, noninferiority, tertiary center setting.

Methods

Fifty patients with a mean age of 49.5 ± 10.6 years were enrolled. Inclusion criteria were age ≥18 years, apnea-hypopnea index (AHI) ≥5 events/hour, and oxygen saturation ≥70%. Exclusion criteria were temporomandibular joint disorders, severe periodontitis, inadequate teeth, and unstable medical diseases. Treatment intolerance was considered a failure. Two-week periods without treatment were followed by questionnaires and randomization into two groups: CPAP/AT-MAS (25) and AT-MAS/CPAP (25). After 6 weeks of intervention, questionnaires and home WatchPAT monitoring were performed. Following each 2-week washout period, patients crossed over to the other treatment followed by similar procedures. Primary outcomes involved the scores from the Functional Outcomes of Sleep Questionnaire (FOSQ). Secondary outcomes were AHI, side effects, and treatment adherence.

Results

Seven patients withdrew from this study: five (AT-MAS intolerance) and two (lost follow-up). There was no significant difference among FOSQ scores, particularly on global scores, between both treatments (0.57, 95% confidential interval of difference: −0.15 to 1.29). Mean AHI decreased from pretreatment 39.2 ± 2.53 to 2.56 ± 0.49 and 12.92 ± 2.05 events/hour while using CPAP and the AT-MAS, respectively (P < .05). Most common side effects of CPAP were dry throat and inconvenience to carry, whereas those of the AT-MAS were jaw pain and excessive salivation.

Conclusions

Both devices improved short-term quality of life similarly; however, the AT-MAS was not as efficacious as CPAP on resolving sleep-test parameters. The AT-MAS might be considered only a temporary treatment alternative.

Level of Evidence

1b. Laryngoscope, 2017



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Effect of psychosocial distress on outcome for head and neck cancer patients undergoing radiation

Objectives/Hypothesis

To determine the impact of pretreatment psychosocial distress on compliance to radiation therapy (RT) and clinical outcomes for patients with head and neck cancer

Study Design

Self-reported responses to the mood and anxiety domains of the University of Washington Quality of Life instrument were reviewed among 133 patients with newly diagnosed head and neck cancer prior to initiating RT.

Methods

Varying definitions were used (total number of unexpectedly missed RT days, >5 days continuous interruption of RT outside of weekends, >10 days continuous interruption of RT, and failure to complete prescribed course of RT) to analyze the effect of psychosocial disruption on compliance. Survival was determined using the Kaplan-Meier method.

Results

The prevalence of pretreatment depression and anxiety was 23% and 47%, respectively. Continuous RT breaks >5 days occurred in 46%, 33%, 10%, 9%, and 0% of patients whose mood was rated as "extremely depressed," "somewhat depressed," "neither in a good mood or depressed," "generally good," and "excellent," respectively (P = .0016). The corresponding proportion of patients who did not complete their planned RT was 23%, 11%, 5%, and 3%, and 0%, respectively (P = .043). The 2-year overall survival of patients who were "extremely depressed" or "somewhat depressed" at baseline was 71% versus 86% for all others (P = .026). Depression was independently associated with decreased overall survival on logistical regression analysis.

Conclusions

Pretreatment depression predicted for decreased RT compliance and inferior survival for head and neck cancer. Additional research to overcome potential barriers to treatment in this setting may be warranted.

Level of Evidence

4. Laryngoscope, 2017



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Safety and efficacy of functional laryngectomy for end-stage dysphagia

Objectives/Hypothesis

To evaluate functional outcomes and complication rate after total laryngectomy (TL) for dysfunctional larynx with end-stage dysphagia.

Study Design

Retrospective chart review.

Methods

Chart review was performed on all patients who underwent TL from January 2008 to July 2016 at a single tertiary academic medical center. Patients who underwent TL for dysfunctional larynx without preoperative evidence of malignancy were included. Main outcome measures were post-TL functional swallowing and speech outcomes, and complication rate.

Results

The study included 19 patients from a cohort of 278 consecutive patients. All patients were previously treated with radiotherapy (RT), whereas 13/19 (68%) previously received chemoradiotherapy. The median time from RT to TL was 10.98 years (range, 0.67–23.94 years). Aspiration was evident preoperatively in 17/19 (89%) patients, with 11 experiencing recurrent aspiration pneumonia. Seventeen of 19 (89%) patients were nil per os (NPO) requiring enteral nutrition. Six of 19 (32%) patients had surgical complications, including three (16%) pharyngocutaneous fistulas. At 3-month and 1-year postoperative follow-up, there was significant improvement in mean Functional Oral Intake Scale (FOIS) score and aspiration, recurrent pneumonia, enteral nutrition, and NPO status rates (P < .05). At 1-year follow-up, no patients were NPO, and only one patient required gastrostomy tube supplementation. Mean FOIS score increased from 1.3 to 6.1 (P = .001). Eight of 13 patients (62%) were actively using a tracheoesophageal prosthesis at 1-year follow-up.

Conclusions

Laryngectomy for dysfunctional larynx eliminates the morbidity of aspiration while improving diet and reducing gastrostomy tube dependence with an acceptable complication rate.

Level of Evidence

4. Laryngoscope, 2017



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Surgical outcome for empty nose syndrome: Impact of implantation site

Objective/Hypothesis

Endonasal submucosal implantation has been confirmed to be beneficial for patients with empty nose syndrome (ENS). However, the optimal implantation site has not been defined. This study aimed to evaluate whether lateral nasal wall implantation is superior to inferior nasal wall implantation in terms of clinical benefits and improvements in quality of life.

Study Design

Retrospective study in a tertiary medical center.

Methods

Consecutive ENS patients between 2010 and 2015 with operative histories of inferior turbinectomies and indicated for surgical implantation were enrolled, with at least 1-year follow-up. Patients were divided into lateral and inferior nasal wall groups. SinoNasal Outcome Test (SNOT)-22, Beck Depression Inventory (BDI)-II, and Beck Anxiety Inventory (BAI) were applied before and 1 year after implantation.

Results

Of the total 30 ENS patients analyzed, 14 were in the inferior nasal wall group and 16 were in the lateral nasal wall group. There were no significant intergroup differences in demographic data and preoperative SNOT-22, BDI-II, and BAI scores. Postoperative assessment revealed that the lateral nasal wall group had significantly better SNOT-22 score improvements than the inferior nasal wall group, particularly regarding rhinological symptoms and sleep function.

Conclusion

Lateral nasal wall implantation may provide significantly better clinical outcomes than inferior nasal wall implantation, and thus may be the preferred, more optimal site for implant placement in ENS patients.

Level of Evidence

4. Laryngoscope, 2017



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Immunologic responses to a novel DNA vaccine targeting human papillomavirus-11 E6E7

Objectives/Hypothesis

Recurrent respiratory papillomatosis (RRP) is a benign disease caused by human papillomavirus (HPV) types 6 and 11. Although a prophylactic vaccine against RRP is available, a therapeutic vaccine is needed to treat those already infected. The objective of our study was to design and test a DNA vaccine targeting HPV11 proteins.

Study Design

Preclinical scientific investigation.

Methods

A DNA vaccine encoding the HPV11 E6 and E7 genes linked to calreticulin (CRT) was generated. Immunologic response to the HPV11 CRT/E6E7 vaccine was measured by vaccinating C57BL/6 mice via electroporation and measuring CD8 + T cell responses from harvested splenocytes. A tumor cell line containing HPV11-E6E7 was created, and the ability of novel DNA vaccine to control tumor growth was measured in vivo.

Results

Our vaccine generated a significant and specific CD8 + T-cell response against the HPV11-E6aa41-70 peptide. The CD8 + T-cell responses did not recognize E7 epitopes, indicating E6 immunodominance. CD8 + responses were augmented in the CRT-linked vaccine compared to a control non-CRT vaccine. The HPV11 CRT/E6E7 vaccine was used to treat mice inoculated with a HPV11 E6E7 expressing tumor cell line after temporary CD3 depletion to facilitate tumor growth. Vaccinated mice had a significantly lower tumor growth rate (P = .029) and smaller tumor volumes compared to control mice, indicating an augmented immunologic response in vaccinated mice.

Conclusions

A DNA vaccine targeting HPV11 E6E7 generates a specific HPV11 CD-8 + T-cell response capable of reducing the growth of HPV11-expressing tumors. DNA vaccines are a promising immunologic strategy for treating RRP.

Level of Evidence

NA Laryngoscope, 2017



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Laryngeal distribution of recurrent respiratory papillomatosis in a previously untreated cohort

Objectives/Hypothesis

To describe the distribution of recurrent respiratory papillomatosis (RRP) lesions across 21 laryngeal anatomic regions in previously untreated patients at initial presentation to provide insight regarding the natural history of RRP.

Study Design

Multi-institutional, retrospective case series.

Methods

Initial laryngoscopic examination videos of 83 previously untreated patients with adult-onset RRP were reviewed. Papilloma locations were recorded using a 21-region laryngeal schematic. Multivariate analyses by anatomic subsite were conducted for the entire population and for subgroups stratified by sex, age, and proton pump inhibitor (PPI) usage. Heat maps were generated, hierarchically color coding the anatomic distribution of disease.

Results

In this cohort, RRP was most likely to occur on the true vocal folds (TVFs) and anterior commissure (P < .0001, odds ratio [OR]: 7.02); within the TVFs, the membranous vocal folds (MVFs) were most likely to be affected (P < .0001, OR: 3.56). The cohort was predominantly male (80.7%); males had a higher average number of affected sites (P = .005) and were more likely to have lesions in any laryngeal subsite (P < .0001, OR: 2.88,) compared to females. PPI users were more likely than nonusers to have disease in any laryngeal subsite (P = .0037, OR: 1.62), particularly in the posterior and subglottic regions (P = .0061, OR: 2.53). Age was not correlated with lesion prevalence or distribution.

Conclusions

In untreated patients presenting to three laryngology clinics, the MVFs were most likely to be affected by RRP. Males had more anatomic sites affected by papilloma than females. The influence of PPI use on RRP distribution warrants further investigation.

Level of Evidence

4. Laryngoscope, 2017



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Full-night measurement of level of obstruction in sleep apnea utilizing continuous manometry

Objectives/Hypothesis

To determine the average proportion of retropalatal or retroglossal obstruction occurring during a whole night in single patients with obstructive sleep apnea.

Study Design

Retrospective analysis.

Methods

Patients who underwent full-night polysomnography and upper airway pressure manometry at the same time were retrospectively enrolled. Airway obstruction levels were categorized into retropalatal or retroglossal obstruction according to the pressure change shown during pressure manometry. The proportion of retropalatal/retroglossal obstruction was calculated for each patient. Independent factors associated with the proportion were assessed.

Results

A total of 218 patients (198 men) with obstructive sleep apnea were included in the study (mean age, 46.8 ± 1.6 years; body mass index, 26.7 ± 3.1 kg/m2). The average proportion of retroglossal and retropalatal obstruction in individuals was 36.2% and 63.8%, respectively. The apnea-hypopnea index was not significantly correlated with the proportion of retroglossal obstruction (P = .219) after adjustment for age, sex, and body mass index. Meanwhile, the proportion of retroglossal obstruction was significantly correlated with oxygen desaturation index (P = .036), average oxygen saturation (P = .006), lowest oxygen saturation (P = .028), and time below 90% oxygen saturation (P = .021).

Conclusions

To our knowledge, this is the first study showing the average proportions of retropalatal/retroglossal obstruction occurring in single individuals with obstructive sleep apnea during a whole night. The proportion of upper airway obstruction site was significantly associated with oxygen desaturation.

Level of Evidence

4 Laryngoscope, 2017



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Salivary duct stenosis: Short-term symptom outcomes after sialendoscopy-assisted salivary duct surgery

Objectives

To prospectively assess short-term symptom change after sialendoscopy-assisted salivary duct surgery (SASDS) for salivary duct stenosis.

Study Design

Prospective cohort study.

Methods

Patients with obstructive sialadenitis from duct stenosis completed the 20-item Chronic Obstructive Sialadenitis Symptoms (COSS) Questionnaire (scored 0–100) prior to SASDS and 3 months postoperatively.

Results

Thirty glands in 19 patients with endoscopically confirmed salivary duct stenosis showed overall symptom improvement, with a mean COSS score reduction of 12.9 points (standard deviation [SD] 13.1) to a mean postoperative score of 25.1 (range 0–75.5) (P < 0.001) with six (20%) glands (5 patients) achieving complete symptom resolution. Symptoms improved significantly for parotid glands (n = 20) by 16.6 points (SD 15.9) (P < 0.0001). For distal duct stenoses (n = 25), significant symptom improvement was seen in cases treated with dilation only (n = 17; partial stenoses) with a mean 20.6 point COSS reduction (SD 19.0) (P < 0.0005) and in cases treated with sialodochoplasty (n = 5; 4 complete, 1 partial stenosis) with a mean 13.8 point reduction (SD 4.7) (P < 0.005). Symptom scores did not improve after SASDS in proximal stenoses (n = 3) and distal stenoses cases not amenable to treatment (n = 3).

Conclusion

SASDS for salivary duct stenosis often can improve obstructive salivary symptoms; however, many patients report persistent symptoms after surgery. Partial duct stenoses or distal duct stenoses are associated with the greatest improvements in COSS scores after SASDS.

Level of Evidence

4. Laryngoscope, 2017



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Establishing and Prioritising Research Questions for the Prevention, Diagnosis and Treatment of Hair Loss (excluding Alopecia Areata): The Hair Loss Priority Setting Partnership

Summary

Background

Hair and scalp problems are common. Unfortunately, many uncertainties exist around the most effective management and treatments strategies for these disorders.

Objectives

To identify uncertainties in hair loss management, prevention, diagnosis and treatment that are important to both people with hair loss and healthcare professionals.

Methods

A hair loss priority setting partnership was established between patients, their carers and relatives, and healthcare professionals to identify the most important uncertainties in hair loss. The methodology of the James Lind Alliance was followed to ensure a balanced, inclusive and transparent process.

Results

In total 2747 treatment uncertainties were submitted by 912 participants; following exclusions 884 uncertainties relating to hair loss (excluding alopecia areata) were analyzed. Questions were combined into "indicative uncertainties" following a structured format. A series of ranking exercises further reduced this list to a top 25 that was taken to a final prioritization workshop where the top 10 priorities were agreed.

Conclusions

We present the top 10 research priorities for hair loss (excluding alopecia areata) to guide researchers and funding bodies to support studies important to both patients and clinicians.

This article is protected by copyright. All rights reserved.



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The first sporadic case of DFNA11 identified by next-generation sequencing

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Publication date: September 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 100
Author(s): Yuka Kaneko, Atsuko Nakano, Yukiko Arimoto, Kiyomitsu Nara, Hideki Mutai, Tatsuo Matsunaga
We report the first sporadic case of nonsyndromic autosomal dominant hearing loss (DFNA11). The patient was a 5-year-old boy with moderate bilateral hearing loss. Targeted next-generation sequencing analysis of patient DNA identified a known heterozygous DFNA11 mutation, c.689C > T, in MYO7A, encoding p.Ala230Val. The mutation was not detected in the parents of the patient and is considered to be de novo. This mutation is identical to the one reported previously in an Italian family. Accumulation of mutation data increases the feasibility of identifying autosomal dominant mutations in sporadic sensorineural hearing loss.



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