Unilateral telangiectasia macularis eruptiva perstans with an unusual clinical presentation

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Pruritic arthropod bite-like papules in T-cell large granular lymphocytic leukaemia and chronic myelomonocytic leukaemia

Summary

T-cell large granular lymphocytic leukaemia (T-LGLL) is a clinically indolent mature T-cell neoplasm characterized by a monoclonal population of CD3+ CD8+ cytotoxic T cells, which usually presents as neutropenia, anaemia and thrombocytopenia. Chronic myelomonocytic leukaemia (CMML) is a clonal haematopoietic disorder with features of both a myeloproliferative neoplasm and myelodysplastic syndrome (MDS). Patients with CMML exhibit a persistent peripheral blood monocytosis in addition to myelodysplastic features. Because of the rarity of T-LGLL, its cutaneous manifestations are poorly documented, but include vasculitis, vasculopathy, persistent ulcerations, generalized pruritus and disseminated granuloma annulare. Various types of skin lesions have been observed in patients with CMML and reportedly occur in approximately 10% of cases. We report the extraordinary case of a patient with MDS who developed T-LGLL, and subsequently the MDS progressed to CMML. The patient then developed diffuse arthropod bite-like papules and intractable pruritus.

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Involvement of spinal cannabinoid receptors in the antipruritic effects of WIN 55,212-2, a cannabinoid receptor agonist

Summary

Background

Cannabinoids have been used for their analgesic and euphoric effects for millennia, but recently the antipruritic effects of cannabis have been discovered. Considering the similarities between pain and itch sensations, we hypothesized that cannabinoid receptors may play a role in the antipruritic effects of cannabinoids.

Aim

To analyse the role of the spinal cannabinoid receptors, CB1 and CB2, in the antipruritic effects of the cannabinoid agonist WIN 55,212-2.

Methods

Male Balb/c mice weighing 20–30 g were used. Scratching behaviour in the mice was produced by injection of serotonin 5 μg/50 μL intradermally into the nape of the neck. Scratching of the site of injection by the hind paws was video-recorded for 30 min. After testing different doses of WIN 55,212-2 [1, 3 and 10 mg/kg intraperitoneally (IP)], the effects of the CB1 receptor antagonist, AM-251 [1 μg/mouse administered intrathecally (IT)] and the CB2 receptor antagonist AM-630 (4 μg/mouse IT) on the antipruritic effects of WIN 55,212-2 were studied using a rotarod apparatus.

Results

WIN 55,212-2 (1, 3 or 10 mg/kg IP) dose-dependently decreased serotonin-induced scratches. The receptor antagonist CB1 partially reversed the effects of WIN 55,212-2 (P < 0.05); whereas CB2 had no statistically significant effect. WIN 55,212-2 impaired motor function only at the highest dose given (10 mg/kg, P < 0.05).

Conclusions

Our findings support prior researches indicating that cannabinoids exert antipruritic effects. Moreover, our results show that the antipruritic effects of cannabinoids are partially mediated by spinal CB1 receptors.

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A 27-Year-Old Woman With Facial Rash: USMLE Question

A 27-year-old woman presents with fatigue, joint pain, and facial rash. What study or investigation is the best next step?
Osmosis

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Erythema elevatum diutinum-like vasculitis secondary to cocaine adulterated with levamisole

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Asymptomatic hyperkeratotic plaque on the vulva of a patient with lichen sclerosus

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Alternative activation of hedgehog pathway induced by ultraviolet B radiation: preliminary study

Summary

Background

There is still much ambiguity in studies of Sonic hedgehog (Shh) pathways and its dysregulation. Some studies concerning the role of the Shh pathway in basal cell carcinoma (BCC) have been conducted, but there is a lack of studies about Shh pathway dysregulation under the influence of ultraviolet (UV)B radiation.

Aim

To evaluate skin expression of Shh, Ptch1, Ptch2, Smo and Gli1 proteins in BCCs with and without the influence of UVB radiation.

Methods

In total, 34 healthy controls (HCs) and 42 patients with nodular BCC were recruited into the study. Patients were divided into five groups (A–E), depending on UVB dose received and BCC status. In all skin specimens, expression of Shh, Ptch1, Ptch2, Smo and Gli1 protein was evaluated.

Results

Comparing the BCC group with the HC group, there was significantly higher expression of Shh, Ptch1, Ptch2, Smo and Gli1 proteins. Expression of Ptch2, Smo and Gli1 was increased in response to UVB doses of 3 MED (minimal erythema dose), whereas expression of Ptch1 and Shh was unaffected.

Conclusion

The lack of change in expression of Shh and Ptch1 after exposure to UVB suggests that the Shh pathway may be activated through a noncanonical pathway under the influence of strong UVB doses.

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ONDAM, T2A, PMSI, SROS, MIGAC, MERRI, SIGAPS & IF : des acronymes financiers médicaux français très liés…

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): O. Laccourreye, F. Rubin




http://ift.tt/2FVXWlS

Contrôle des vertiges dans la maladie de Menière par injections trans-tympaniques de dexaméthasone

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): A. Weckel, M. Marx, M.-J. Esteve-Fraysse
ButL'objectif principal était d'évaluer l'efficacité des injections trans-tympaniques (ITT) de dexaméthasone sur le contrôle des vertiges, dans des maladies de Menière unilatérales, réfractaires aux traitements médicamenteux.Matériel et méthodesIl s'agit d'une étude rétrospective, portant sur 25 patients, présentant une maladie de Menière unilatérale, définie selon les critères de l'American Academy of Otorhinolaryngology-Head and Neck Surgery (AAO-HNS), invalidante. Les patients ont bénéficié d'ITT de dexaméthasone durant la période de suivi. Le contrôle des vertiges a été évalué selon l'index de contrôle des vertiges définis en classe selon l'AAO-HNS, à 6 mois, 1 an, et 2 ans après le début de cette thérapeutique. La survenue d'éventuelles complications, et l'évolution de l'audition ont également été évaluées.ObjectifsL'objectif principal était d'évaluer l'efficacité des ITT de dexamethasone dans le contrôle des vertiges dans la maladie de Menière.RésultatsÀ 6 mois un contrôle satisfaisant (classe A ou B) était obtenu chez 92 % des patients (n=23/25), à 1 an le contrôle était satisfaisant chez 68 % des patients (n=17/25), et à 2 ans chez 70 % des patients (n=16/23). Il n'y a pas eu d'aggravation de l'audition chez les patients bien contrôlés par cette thérapeutique. Aucune complication locale ni systémique n'a été observée durant le suivi.ConclusionLa dexaméthasone fait partie de la stratégie de prise en charge des patients présentant une maladie de Menière réfractaire aux traitements conventionnels, avant les traitements destructeurs, et permet un contrôle des vertiges dans 70 % des cas à 2 ans.



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Intérêt de la mesure du débit inspiratoire de pointe dans l’indication de trachéotomie

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): M. Lesnik, J. Sanchez-Guerrero, O. De Crouy Chanel, C. Hervé, J. Guerlain, S. Périé
ObjectifsL'évaluation quantitative du degré d'obstruction des voies aériennes supérieures ne peut pas être réalisée lors d'une dyspnée aiguë, notamment en cas de cancer des voies aérodigestives supérieures. La décision de réaliser ou non un geste de contrôle des voies aériennes peut être difficile à prendre. Le débit inspiratoire de pointe (DIP) est un outil dont l'intérêt a été démontré en situation de décanulation après chirurgie pour cancer des VADS. L'objectif de ce travail a été de montrer son intérêt en tant qu'outil non invasif permettant de mesurer la sévérité des dyspnées inspiratoires nécessitant une trachéotomie en urgence.Matériels et méthodeÉtude pilote, prospective observationnelle et monocentrique. Nous avons analysé les mesures du DIP chez 22 patients présentant une dyspnée aiguë par obstruction des voies aériennes supérieures. La décision de réaliser ou non une trachéotomie était prise avant la réalisation des mesures du DIP. Les valeurs du DIP étaient mesurées par un dispositif portable (In-Check method), et la nasofibroscopie réalisée ensuite. La sévérité de l'obstruction était définie par les valeurs du DIP.RésultatsLa mesure du DIP a pu être réalisée avant la trachéotomie (imminente chez 21 patients, reportée chez un patient) chez tous les patients. Les valeurs de DIP inférieures à 53,1 L/min (i.e 18,3 % de la valeur théorique) semblent être corrélées à la nécessité de réaliser un geste en urgence. Cette valeur est cohérente avec la valeur seuil précédemment retrouvée et au-delà de laquelle la décanulation est possible (60L/min).Conclusionsle DIP est un paramètre quantitatif non invasif permettant d'évaluer la sévérité de l'obstruction des voies aériennes supérieures, utile dans la prise de décision de trachéotomie. Il est simple, rapide et reproductible.



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Évaluation de l’impact médico-économique de la chirurgie ambulatoire en rhinologie

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): G. Mortuaire, D. Theis, R. Fackeure, D. Chevalier, I. Gengler
ObjectifsCaractériser l'impact médical et économique de la chirurgie rhinologique ambulatoire en termes d'efficacité clinique et de maîtrise des dépenses.Matériel et méthodesUne analyse rétrospective était menée de janvier 2014 à janvier 2016 incluant l'ensemble des patients traités en chirurgie ambulatoire pour une pathologie rhinologique. Les données cliniques étaient recueillies par croisement des dossiers informatiques médical et anesthésique. Les coûts relatifs aux charges logistiques et médicotechniques étaient calculés en s'appuyant sur la « Comptabilité analytique hospitalière 2014 ». Les recettes pour l'établissement étaient estimées sur les dernières versions tarifaires définies pour les groupes homogènes de séjours.RésultatsSur 2 ans, 927 interventions étaient effectuées en chirurgie ambulatoire. Le taux de conversion en hospitalisation conventionnelle était de 2,9 %. Les patients contactés à j1 considéraient la prise en charge comme très satisfaisante dans 85 % des cas. L'ensemble des séjours ambulatoires s'accompagnait d'une réduction statistiquement significative des charges par comparaison à une estimation financière d'un séjour d'une nuit en hospitalisation conventionnelle pour les mêmes patients. Les recettes n'étaient pas modifiées entre les deux modes de séjour.ConclusionCette étude confirme l'efficacité de la prise en charge ambulatoire. La réduction des charges constitue une économie potentielle pour l'assurance-maladie par les réajustements tarifaires calculés sur les coûts. L'estimation précise de l'impact médico-économique de la chirurgie ambulatoire nécessite une approche plus fine dite de « micro-costing » à l'échelle de l'établissement de santé et une évaluation des conséquences sur l'activité chirurgicale conventionnelle et la demande de soins en ville une fois le patient sorti.



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Editorial Board

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1





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Atteintes otologiques du syndrome de Turner

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): E. Bois, M. Nassar, D. Zenaty, J. Léger, T. Van Den Abbeele, N. Teissier
IntroductionLes filles porteuses d'un syndrome de Turner (ST) présentent des malformations craniofaciales (hypoplasie et dysfonctionnement des trompes d'Eustache, dysfonctionnement vélo-palatin) qui favorisent la survenue d'otites moyennes aiguës (OMA). L'objectif de cette étude a été d'étudier les affections otologiques à l'âge pédiatrique du syndrome de Turner lors de la première consultation ORL dans notre centre.Patients et méthodesNous avons repris les données des consultations ORL de patientes en âge pédiatrique suivies pour syndrome de Turner dans notre institution entre 2005 et 2015 et relevé : l'otoscopie, le seuil auditif, les antécédents d'OMA et chirurgicaux ORL. Nous avons comparé ces données selon le caryotype des patientes [monosomie (45,X), mosaïque (45,X/46,XX), isochromosome (46,Xi(Xq)), chromosome X en anneau (XrX), contenant du matériel Y et « autres » caryotypes].RésultatsQuatre-vingt-dix patientes, avec un âge moyen de 11,9 ans (± 4,8 ans) lors de la première consultation ORL dans notre institution, ont été incluses : 29 % présentaient une anomalie tympanique à l'otoscopie, 21 % une hypoacousie, et 24 % des antécédents d'OMA à répétition. D'un point de vue chirurgical, 18 % ont été opérées d'une adénoïdectomie, 24 % ont eu une pose d'ATT, 5,6 % ont eu une tympanoplastie. Aucun caryotype ne présentait plus qu'un autre un risque de surdité ou d'OMA.ConclusionCes résultats montrent que les patientes porteuses d'un ST présentent une forte prévalence d'affections otologiques à l'âge pédiatrique et nécessitent un suivi ORL régulier et rapproché.



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Rhinophyma : notre expérience à propos de 12 cas

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): P. Clarós, M.-C. Sarr, F.-B. Nyada, A. Clarós
IntroductionLe rhinophyma est une pathologie rare, bénigne et inesthétique de la peau du nez. Son existence a été signalée il y a fort longtemps, comme l'illustre le portrait du nez bosselé du vieillard tenant son petit-fils peint par Ghirlandaio en 1490, et sa description par Ferdinando Hebra Von (1816–1880), comme 3^e stade d'acné rosacé. L'objectif de cette étude est de rapporter l'expérience de l'auteur et de proposer une nouvelle option thérapeutique dans sa prise en charge.Matériel et méthodesNous décrivons notre expérience sur ce sujet. Nous avons mené une étude rétrospective sur étude des dossiers.RésultatsNous avons recensé 12 cas en 12 ans, largement dominés par le sexe masculin. Le procédé thérapeutique a été le même chez l'ensemble des patients, à savoir l'association dermabrasion, décortication et application de colle de fibrine. L'évolution a été favorable pour tous avec épidermisation complète.ConclusionLe rhinophyma est une affection rare dont la physiopathologie reste incertaine. La prise en charge est chirurgicale et de multiples techniques et procédés ayant été proposés, nous prônons le slogan « à chacun sa technique » en attendant l'adoption d'un consensus. Notre technique associant dermabrasion, décortication et application de colle de fibrine a donné de très bons résultats.



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Les revues « prédatrices » à l’assaut de la presse médicale scientifique

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): O. Laccourreye, F. Rubin, H. Maisonneuve
Dans cet article les auteurs détaillent les révolutions (classement des revues – structuration des articles scientifiques et de leur relecture – impact des technologies de l'information et transformation du modèle économique de la presse médicale) qui, en un temps très court, ont transformé le monde de la rédaction médicale scientifique désormais assailli par les revues « prédatrices ».



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Symptômes de rhinite allergique à Parakou, Bénin : prévalence, sévérité et facteurs associés

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): M.-C. Flatin, S. Ade, S.-H.-R. Hounkpatin, B. Ametonou, U.-B. Vodouhe, W. Adjibabi
ButsLa rhinite allergique (RA) est une affection extrêmement fréquente. Le but de ce travail était de déterminer la prévalence des symptômes de RA, leur sévérité et les facteurs potentiellement associés à Parakou, une ville du Bénin.Matériel et méthodeIl s'agissait d'une étude transversale menée entre avril et juillet 2016, portant sur les habitants de la ville, âgés entre 6 et 65 ans. Un échantillonnage en grappes était réalisé. Les symptômes de RA étaient définis par la présence simultanée d'une rhinorrhée aqueuse, d'obstruction nasale et d'éternuements sans relation évidente avec une infection respiratoire. Les données étaient analysées dans EpiData Analysis v2.2.2.183 puis R.3.2.1.RésultatsAu total, 395 sujets ont fait partie de l'enquête, le sex-ratio était de 1,32, l'âge médian 19 ans. Parmi eux, 141 (35,7 %) avaient des symptômes de RA, dont 47 (33,3 %) une histoire familiale d'atopie, 87 (61,7 %) une conjonctivite allergique. Les principaux facteurs déclenchants étaient la poussière de maison (139 ; 98,5 %) et la pollution atmosphérique (111 ; 78,7 %). Après analyse multivariée, les facteurs associés étaient un âge inférieur à 18 ans (p<0,05), une exposition à la poussière de maison (p<0,001), une histoire personnelle d'urticaire (p<0,001) ou de conjonctivite allergique (p<0,01). Vingt-huit sujets (19,9 %) avaient des symptômes persistants, 43 (30,5 %), modérés à sévères. Ces derniers étaient associés à un âge supérieur à 18 ans (p<0,01), à une atopie familiale (p<0,01), à une histoire personnelle d'eczéma, de dermatite atopique (p<0,01), et à la présence dans la maison de chien/chat (p=0,01).ConclusionLa prévalence des symptômes de rhinite allergique est élevée. Ces symptômes sont sévères dans une proportion non négligeable. Elle justifie une attention particulière dans la ville.



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Évaluation du retentissement de la surdité chez l’adulte : validation d’un questionnaire de qualité de vie

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): E. Ambert-Dahan, C. Laouénan, M. Lebredonchel, S. Borel, C. Carillo, D. Bouccara, O. Sterkers, E. Ferrary, I. Mosnier
ObjectifsL'évaluation de l'impact de la surdité et de sa réhabilitation (aides auditives, implant cochléaire) sur la qualité de vie est fondamentale. Les questionnaires couramment utilisés (Nijmegen, Aphab, GBI) sont longs, compliqués à remplir et peu sensibles aux faibles améliorations/détériorations. L'objectif de cette étude est de valider une échelle de qualité de vie destinée aux patients adultes présentant une perte auditive qu'elle soit réhabilitée ou non.Matériel et méthodesLe questionnaire d'évaluation du retentissement de la surdité chez l'adulte (Ersa) est un questionnaire auto-administré, divisé en quatre sous-domaines comprenant chacun cinq questions notées de 1 à 10. Les questions sont simples et formulées de façon à ce que le patient réponde à l'instant présent. La fiabilité test-retest d'Ersa a été mesurée chez 38 patients. La cohérence interne et la validité par rapport au questionnaire Aphab et aux performances auditives ont été mesurées chez 122 patients lors du bilan de surdité. La sensibilité au changement a été mesurée chez 36 patients avant et 6 à 12 mois après l'implantation cochléaire.RésultatsLa fiabilité du questionnaire Ersa (test/retest) est très satisfaisante (ρ=0,88). La cohérence interne est bonne pour l'ensemble des questions. La validité de structure externe, étudiée en comparant, chez les patients sourds non implantés, les scores Ersa à ceux du questionnaire Aphab, est bonne (ρ=0,52). Le score Ersa est corrélé aux performances auditives dans des situations d'écoute difficiles (mots monosyllabiques : ρ=0,22 ; phrases dans le bruit : ρ=0,19). Chez les patients testés avant et après implantation, l'amélioration des performances auditives dans le silence et dans le bruit est corrélée à l'amélioration du score Ersa (ρ=0,37 à 0,59 selon les tests), mais pas au score GBI.ConclusionLe questionnaire Ersa est un outil facile, rapide d'utilisation, fiable et sensible au changement des performances auditives après une implantation cochléaire.



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Dysfonctionnement nasal chronique

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): R. Jankowski, D.T. Nguyen, A. Russel, B. Toussaint, P. Gallet, C. Rumeau
Le dysfonctionnement nasal chronique (DNC) est un concept clinique de prise en charge diagnostique et thérapeutique de la pathologie rhino-sinusienne, qui s'appuie sur la théorie évo-dévo de formation du nez selon laquelle le nez n'est pas un organe unique mais l'association d'un nez olfactif, d'un nez respiratoire, et de sinus paranasaux. Dans la théorie du DNC le diagnostic étiologique tient compte de l'indépendance physiopathologique possible des symptômes, liée aux origines différentes et à la physiologie propre des trois organes qui composent le nez. Le raisonnement diagnostique dans le concept de DNC a pour objet de démembrer la pathologie en vue d'un traitement adapté à la pathologie du/des organe(s) malade(s) et aux possibilités de récupération physiologique des dysfonctionnements induits par la pathologie de l'un sur les autres organes composant le nez. L'ethmoïde n'étant pas un sinus, la chirurgie endoscopique endonasale fonctionnelle (Functional Endoscopic Endonasal Surgery ou FEES [fi:z]) ne peut se résumer dans le concept de chirurgie endoscopique fonctionnelle des sinus (Functional Endoscopic Sinus Surgery (FESS [fɛs]). La théorie évo-dévo du nez et le concept de DNC offrent une alternative à la conception de rhinosinusite chronique avec ou sans polypose dans la prise en charge des maladies naso-sinusiennes.



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Stimulation magnétique transcrânienne et acouphène subjectif. Revue 2014–2016

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): A. Londero, P. Bonfils, J.P. Lefaucheur
L'acouphène subjectif est un symptôme présent dans de nombreuses pathologies ORL pour lequel il n'existe pas de thérapie curative. Il peut être mal toléré par certains patients qui développent des troubles attentionnels ou du sommeil voire des réactions anxio-dépressives majeures entraînant une altération importante de la qualité de vie. Dans la genèse et l'entretien du symptôme acouphène, les modèles physiopathologiques actuels mettent en avant l'importance de phénomènes maladaptatifs de plasticité cérébrale provoqués par un déficit auditif périphérique. Bien qu'imparfaitement élucidées, ces altérations de l'activité neuronale sont la cible de tentatives de neuromodulation, en particulier grâce à la stimulation magnétique transcrânienne répétée (SMTr ou rTMS en acronyme anglais) qui a fait l'objet de différentes études cliniques et méta-analyses. Un consensus récent (Lefaucheur, 2014) a fait état d'un niveau de preuve de grade C (efficacité possible) pour l'utilisation de la SMTr en stimulation tonique basse fréquence (1Hz) ciblant le cortex temporal gauche. De nombreuses questions restent néanmoins en suspens concernant l'emploi de cette technique en pratique quotidienne. Cet article propose une revue de la littérature récente en utilisant les mots clés « tinnitus » et « rTMS » sur les bases de données PubMed et Cochrane entre avril 2014 et décembre 2016.



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Syndrome de Kindler compliqué d’un carcinome épidermoïde agressif du palais

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): H. Souldi, M.Y. Bajja, M. Mahtar
IntroductionLe syndrome de Kindler est une génodermatose autosomique récessive très rare. Il est caractérisé par une fragilité et une photosensibilité cutanées qui débutent pendant l'enfance, et par une poïkilodermie progressive.ObservationNous rapportons l'observation d'une jeune femme suivie depuis son enfance pour un syndrome de Kindler, caractérisé par des lésions importantes de la muqueuse oro-pharyngée. La patiente s'est présentée en raison d'une lésion ulcéro-bourgeonnante du palais. Le scanner et la biopsie réalisés ont conclu à un carcinome épidermoïde agressif et non réséquable du palais dur. Une chimiothérapie néoadjuvante a été proposée chez cette patiente, qui est décédée après la première cure de chimiothérapie avec un tableau de mucite digestive sévère compliquée d'un sepsis général.DiscussionDes formes d'atteinte des muqueuses dans le syndrome de Kindler ont été décrites dans la littérature. Cependant, la transformation en carcinome épidermoïde est très peu rapportée, même s'il s'agit d'une complication à long terme connue de ce syndrome. Nous décrivons le second cas de syndrome de Kindler compliqué d'un carcinome épidermoïde du palais.



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Liposarcome bien différencié pédiculé de l’hypopharynx

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): C. Eyermann, T. Raguin, P. Hemar, C. Debry
IntroductionLes liposarcomes de l'hypopharynx sont extrêmement rares. Seuls 28 cas sont décrits dans la littérature. Le premier signe clinique est une dysphagie d'aggravation progressive.Présentation du casNous rapportons le cas d'un homme de 71 ans ayant présenté une dysphagie avec une perte de poids importante en quelques semaines et un épisode d'extériorisation d'une masse charnue par la bouche lors d'un épisode de vomissement. L'imagerie retrouvait une masse endo-oesophagienne de structure graisseuse. La panendoscopie associée à une oesogastroscopie a permis de visualiser la tumeur, pédiculée au niveau du sinus piriforme gauche, et de l'extérioriser par la cavité orale. La tumeur a ensuite été réséquée par voie endoscopique, au niveau de son pédicule d'insertion hypopharyngé. L'examen anatomopathologique de la pièce opératoire concluait à un liposarcome bénin bien différencié.DiscussionLe liposarcome bien différencié est le plus fréquent des liposarcomes, mais exceptionnellement décrit au niveau hypopharyngé. Les principaux symptômes sont liés à la compression des structures adjacentes. L'imagerie est aspécifique. Seul l'examen histologique permet de le différencier d'autres tumeurs œsophagiennes bénignes. Le traitement de référence est l'exérèse complète et large, pas toujours réalisable au niveau cervical. Un suivi au long cours des patients est indispensable pour détecter précocement une récidive.



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Modification du lambeau libre fascio-cutané antébrachial radial pour réaliser une fermeture en deux plans lors de la reconstruction de pertes de substance circonférentielles de l’hypopharynx

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): M. Abu-Serriah, S. Loughran
Les pertes de substance circonférentielles de l'hypopharynx sont des défis de reconstruction. Différents lambeaux locaux, régionaux et libres ont été décrits avec pour chacun des avantages et des inconvénients en termes de résultats fonctionnels. Le lambeau libre fascio-cutané antébrachial radial (LLFAR) est l'un des lambeaux libres les plus fréquemment utilisés pour reconstruire les pertes de substance circonférentielles de l'hypopharynx. La palette cutanée est souple et ressemble à la paroi hypopharyngée d'origine. Il est facile à prélever, possède une anatomie vasculaire prévisible et un long pédicule. Contrairement au lambeau antérolatéral de la cuisse (LALC), le LLFAR est associé à des taux plus élevés de fistule pharyngo-cutanée. On a pensé que cela était dû à la difficulté de réaliser une fermeture en deux plans. Cependant, dans un cou post-traitement ou chez un patient corpulent, l'utilisation du LALC ou d'autres lambeaux libres peut ne pas être possible, faisant du LLFAR la seule option viable. Afin de réduire le risque de fistule et de déhiscence de la plaie, nous décrivons une nouvelle méthode de LLFAR avec une fermeture en deux plans. Nous croyons que notre méthode offre au chirurgien reconstructeur une alternative dont il serait intéressant de tenir compte dans les cas difficiles de pertes de substance circonférentielles de l' hypopharynx.



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Oropharyngectomie par voie cervico-transorale assistée par robot et lambeau libre antérolatéral de cuisse fin

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): P. Gorphe, S. Temam, F. Kolb, Q. Qassemyar
La technique classique de l'oropharyngectomie latérale par voie assistée par robot a été décrite pour l'exérèse simple des lésions T1 et T2 de loge amygdalienne avec une perte de substance le plus souvent laissée en cicatrisation dirigée. Nous proposons une technique modifiée adaptée à la chirurgie de rattrapage et à la chirurgie en terrain irradié, issue de l'expérience des voies d'abord ouvertes. Elle consiste en une résection en monobloc de l'ensemble de l'espace parapharyngé pré- et rétro-stylien avec la pièce opératoire d'oropharyngectomie latérale par une voie d'abord combinée cervicale et transorale assistée par robot. La reconstruction est réalisée par un lambeau libre antérolatéral de cuisse fin disséqué dans le plan du fascia superficialis.



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Une masse linguale

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): A. Nurdoğan




http://ift.tt/2ESvOku

Une colonisation du cornet moyen

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): C. Djian, A.-L. Gaultier, S. Chartier, O. Laccourreye




http://ift.tt/2EaG2LT

Abcès avec ostéomyélite du clivus après adénoïdectomie : Une complication exceptionnelle d’un geste fréquent

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): E. Moreddu, C. Le Treut, J.-M. Triglia, R. Nicollas




http://ift.tt/2EPNZaj

Rhinorrhée unilatérale : une étiologie atypique

Publication date: February 2018
Source:Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, Volume 135, Issue 1
Author(s): C. Aussedat, C. Dorbeau, D. Bakhos




http://ift.tt/2EcZYxP

Systemic allergic contact dermatitis associated with topical diltiazem and/or cinchocaine

Abstract

Topical anesthetics for the treatment of anal fissures or hemorrhoids are a frequent cause of allergic contact dermatitis (ACD) in the perianal region¹. Since 2009, a few cases have been reported also to the calcium channel blocker diltiazem^2,3,4 used as a first-line agent in the treatment of anal fissures. We present a case of ACD from diltiazem and/or cinchocaine followed by systemic contact dermatitis (SCD).

This article is protected by copyright. All rights reserved.

http://ift.tt/2sniIJA

A retrospective case series of referrals to our psychodermatology clinic 2009-2016

Abstract

Psychodermatology is a relatively new subspecialty exploring the interaction between skin and the mind. Managing patients in this cohort can be challenging^1,2,3. The aetiology of psychocutaneous disease is often complex, ranging from solely functional skin-directed symptoms (psychogenic pruritis), to thought disorders manifesting in a cutaneous fashion (delusional parasitosis), to factitious disorders with cutaneous consequences (e.g. dermatitis artefacta). In addition, purely psychiatric conditions (i.e. substance misuse, depression or obsessive compulsive disorder), may occur alongside dermatological disease, further complicating successful management.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EwhQH6

Die endonasale endoskopische Resektion von Choanalatresien – wie groß ist der Langzeiterfolg?

Laryngo-Rhino-Otol
DOI: 10.1055/s-0044-101464

Einleitung Die Choanalatresie gehört zur Gruppe der seltenen Fehlbildungen. Sie stellt bei beidseitigem Auftreten einen akut lebensbedrohlichen Notfall dar, da Neugeborene obligate Nasenatmer sind. Die endoskopische Resektion ist eine etablierte Therapie, die Operationstechnik ist bisher jedoch nicht standardisiert. Methodik Die endonasal endoskopische Resektion von kongenitalen Choanalatresien bei 11 Kindern wurde retrospektiv in einem Nachbeobachtungszeitraum von 9 bis 87 Monaten analysiert. Vier Kinder wiesen eine bilaterale, sieben eine unilaterale Atresie auf. Neben der endoskopischen Resektion der Choanalatresie erfolgten die Erweiterung der Choane durch endoskopisches Abtragen des Knochens Richtung Schädelbasis und das Abtragen der hinteren Septumkante. Das intraoperative Handling, intra- und postoperative Komplikationen und die Rezidivrate im Verlauf wurden ermittelt. Eltern bzw. Patienten wurden zur subjektiven Einschätzung der Nasenatmung befragt. Ergebnisse Intraoperative Komplikationen wurden keine beobachtet. Drei Frühgeborene wurden innerhalb der ersten Lebenswoche bei einem durchschnittlichen Geburtsgewicht von 2540 g operiert. Postoperativ kam es zweimal zu Schmierblutungen. Die langfristige Rezidivfreiheit lag bei 73 % (unilateral) bzw. 100 % (bilateral). Schlussfolgerungen Die endonasale endoskopische Resektion von Choanalatresien mit Erweiterung der Choane Richtung Schädelbasis, Reduktion des Nasenbodens und Abtragen der hinteren Septumanteile stellt eine schonende und erfolgreiche Therapieoption dar.
[...]

© Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text

http://ift.tt/2BixyUf

Origins of antidromic activity in sensory afferent fibers and neurogenic inflammation

Abstract

Neurogenic inflammation results from the release of biologically active agents from the peripheral primary afferent terminal. This release reflects the presence of releasable pools of active product and depolarization-exocytotic coupling mechanisms in the distal afferent terminal and serves to alter the physiologic function of innervated organ systems ranging from the skin and meninges to muscle, bone, and viscera. Aside from direct stimulation, this biologically important release from the peripheral afferent terminal can be initiated by antidromic activity arising from five anatomically distinct points of origin: (i) afferent collaterals at the peripheral-target organ level, (ii) afferent collaterals arising proximal to the target organ, (iii) from mid-axon where afferents lacking myelin sheaths (C fibers and others following demyelinating injuries) may display crosstalk and respond to local irritation, (iv) the dorsal root ganglion itself, and (v) the central terminals of the afferent in the dorsal horn where local circuits and bulbospinal projections can initiate the so-called dorsal root reflexes, i.e., antidromic traffic in the sensory afferent.

http://ift.tt/2shsHzM

Chitotriosidase inhibits allergic asthmatic airways via regulation of TGF-β expression and Foxp3+ Treg cells

Abstract

Background

Chitotriosidase (Chitnase 1, Chit1), a major true chitinase in humans, is induced in childhood asthma and has been implicated in the pathogenesis of a variety of inflammatory and tissue remodeling responses. We hypothesized that Chit1 plays a significant role in the pathogenesis of allergic asthma. To identify the role of Chit1, the mechanisms that underlie these contributions and the relevance of these murine findings to childhood asthma.

Methods

Wild type and Chit1-deficient mice and cells in culture were used to define the roles of Chit1 in models of allergic adaptive Th2 inflammation. In addition, the levels of sputum Chit1 were evaluated in pediatric asthma patients and compared to control.

Results

The levels of sputum Chit1 were significantly increased in the patients with childhood asthma. Mice with Chit1 null mutation demonstrated enhanced allergic Th2 inflammatory and cytokine and IgE responses to OVA or house dust mite allergen sensitization and challenge. However, the expression levels of TGF-β1 were significantly decreased with a diminished number of Foxp3⁺ regulatory T cells (Treg) in the lungs of Chit1^-/- mice compared to WT controls. In vitro, the absence of Chit1 significantly reduced TGF-β-stimulated conversion of CD4⁺CD25^- naïve T cells to CD4⁺Foxp3⁺ Treg cells, suggesting Chit1 is required for optimal effect of TGF-β1 in Treg cell differentiation.

Conclusion

Chit1 plays a protective role in the pathogenesis of allergic inflammation and asthmatic airway responses via regulation of TGF-β expression and Foxp3⁺ Treg cells.

This article is protected by copyright. All rights reserved.

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Alterations of Global DNA Methylation and DNA Methyltransferase Expression in T and B Lymphocytes from Patients with Newly Diagnosed Autoimmune Thyroid Diseases After Treatment: A Follow-Up Study

Thyroid , Vol. 0, No. 0.


http://ift.tt/2BlCpUC

Preparation, Purification, and Identification of a Monoclonal Antibody Against the C-Terminal Domain of Semaphorin3F

Monoclonal Antibodies in Immunodiagnosis and Immunotherapy , Vol. 0, No. 0.


http://ift.tt/2EpAhNR

Onabotulinum Toxin A in Adductor Spasmodic Dysphonia and Laryngeal Tremor

This cohort study examines the outcomes of onabotulinum toxin A treatment in patients with adductor spasmodic dysphonia with or without lateral laryngeal tremor.

http://ift.tt/2ERh6tZ

Medical Student Knowledge of Human Papillomavirus–Positive Head and Neck Cancer

This survey examines knowledge of the association of human papillomavirus and head and neck cancer among students at 10 New York State medical schools.

http://ift.tt/2EcSOti

The 'Ugly Duckling' Rule in Melanoma Self-exam

Compared with the more traditional ABCD rule, does the 'ugly duckling' sign improve melanoma detection?
Medscape Dermatology

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Hepatotoxicity Associated With Vismodegib

An 82-year-old Caucasian woman with a history of basal cell carcinoma on vismodegib presented with nausea, vomiting and intermittent abdominal pain. Laboratory results were remarkable for the elevation of liver enzymes. Endoscopic retrograde cholangiopancreatography and percutaneous transhepatic cholangiogram (PTC) did not show evidence of intrahepatic or extrahepatic obstruction of the biliary tract. During PTC external biliary catheter was placed; however, bilirubin continued to rise. Further, laboratory work-up and imaging studies ruled out other possible aetiologies for hepatotoxicity such as infections, autoimmune hepatitis and other drugs known to be hepatotoxic thus leaving vismodegib the most likely cause of hepatotoxicity.

http://ift.tt/2BNryUK

Intracaval misplacement of a double-J ureteral stent

Double-J ureteral stent (DJUS) is an important therapeutic tool in endourology. There are well-known frequent complications associated with DJUS placement such as distal and proximal migration within the urinary tract. However, perforation and stent misplacement are uncommon but serious complications of this technique. We present a case of a 63-year-old man who had a misplacement of a DJUS into the inferior vena cava during an elective procedure of ureteral catheterisation. The stent placement was performed under fluoroscopic control and it seemed well positioned. Actually, the DJUS was misplaced in the inferior vena cava after drilling at the level of the crossing of the ureter with the ipsilateral iliac vessels. Diagnosis was incidentally made 3 months after the placement of the stent in a renal CT scan. The patient was always asymptomatic. We performed an endoscopic removal of the ureteral stent, which took place without complications.

http://ift.tt/2siU6Bp

Double invasive fungal infection due to dematiaceous moulds in a renal transplant patient

Alternaria and Verruconis are two dematiaceous moulds that occasionally cause disease in immunocompromised hosts. We present the case of a 58-year-old man with history of deceased donor renal transplantation 14 months prior, who presented with fevers and cough. He was found to have right upper lobe pneumonia and a non-healing eschar of his right knee. Dematiaceous fungi grew from bronchoalveolar lavage (BAL) and was sent to reference lab for identification. Meanwhile, the eschar on his right knee was biopsied and grew Alternaria spp. Pathology was consistent with invasive mould infection and he was treated as having disseminated Alternaria infection with voriconazole and amphotericin B lipid complex. Later on, the dematiaceous mould from a BAL specimen was identified as Verruconis gallopava. The patient was discharged on voriconazole awaiting minimal inhibitory concentrations for V. gallopava but was readmitted 2 days later with high fevers and died from acute respiratory failure.

http://ift.tt/2BOFkq7

Pathological fracture of femoral neck in a middle-aged woman: a rare presentation of primary hydatid cyst disease in humans

Hydatid disease in humans is caused by Echinococcus granulosus. It most commonly involves the liver and, to a lesser extent, the lungs and spleen; however, it is known to involve other areas, too. Involvement of bone by hydatid cyst is rare. Here, we describe the case of a 37-year-old woman who presented with pain in the left groin and swelling in the left thigh. The radiological imaging showed a fracture of the femoral neck and cysts in the shaft of the femur. Diagnosis of hydatid cyst was confirmed on the basis of histopathology of biopsy specimens. The patient recovered after surgical excision of the cyst. This case illustrates the various sites and presentations of hydatid cyst disease, and the need to investigate for it if cystic bony lesions are encountered especially in endemic regions, as a delay in diagnosis can lead to long-term morbidity and even death.

http://ift.tt/2shD9rd

Autoimmune fasciitis triggered by the anti-programmed cell death-1 monoclonal antibody nivolumab

A 43-year-old woman with a history of recently diagnosed metastatic melanoma was commenced on systemic therapy with nivolumab, an anti-programmed cell death-1 monoclonal antibody and one of an increasing group of the so-called 'immune checkpoint inhibitors'. She experienced a dramatic complete response within 6 months of initiation. However, in addition to developing incident autoimmune hypothyroidism, she also developed progressive fatigue, proximal weakness, myalgia and dysphagia. Initial investigations with blood tests, electrophysiology and a muscle biopsy were non-specific or normal. Subsequent examination revealed 'woody' thickening of the subcutaneous tissues of the forearms, thighs and calves consistent with fasciitis. MRI and a full-thickness skin–muscle biopsy were ultimately diagnostic of a likely iatrogenic autoimmune myofasciitis. The clinical manifestations only responded partly to prednisolone 30 mg orally and treatment was escalated to include intravenous immunoglobulin. At 3 months, this has only resulted in a modest incremental improvement.

http://ift.tt/2BNTeZK

Follicular dendritic cell sarcoma of the porta hepatis

Follicular dendritic cell (FDC) sarcoma is a very rare neoplasm which commonly involves the lymph nodes and less commonly involves extranodal organs such as the liver. Most cases of FDC sarcoma are idiopathic, however some cases are associated with other disease states. Management of FDC sarcoma is primarily focused on surgical resection of the mass, and secondarily focused on radiotherapy, chemotherapy and/or biologic pharmacotherapy. We report the case of a patient who was found to have FDC sarcoma presenting as an obstructing mass of the porta hepatis, a manifestation which does not appear to be reported in the literature.

http://ift.tt/2sgKXJT

21st century obstetrics: a 50-year-old nullip--walk in the park?

We discuss the case of a 50-year-old nulliparous woman who conceived after in vitro fertilisation. She had multiple medical comorbidities and presented an obstetric and medical challenge. She was carefully managed through pregnancy and had a successful outcome. In this report, we explore the medical complexity, as well as ethical and logistic issues involved.

http://ift.tt/2BNT6cI

Emphysematous gastritis secondary to Sarcina ventriculi

Description

An 87-year-old man with history of dementia, coronary artery disease on dual antiplatelet therapy and oesophagitis presented to the emergency department with acute-onset coffee ground emesis and left upper quadrant abdominal pain of 1-day duration. Vital signs were normal. Exam was notable for diffuse abdominal pain without peritoneal signs.

Medical work-up was remarkable for haemoglobin of 8.2 g/dL, lactate of 6.3 mmol/L and white blood cell count of 21.84x10⁹/L. CT with angiography of the abdomen and pelvis revealed extensive portal and mesenteric venous gas as well as gastric emphysema (figures 1 and 2). The bowel was normal.

Figure 1

Axial CT image of the upper abdomen with intravenous contrast demonstrates extensive portal venous gas (blue arrow). Multiple locules of gas (red arrows) are present in the wall of the stomach.

Figure 2

Coronal CT of the upper abdomen...

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Giant granadillas seeds phytobezoar rectal impactation: a very unusual case of intestinal obstruction

A 78-year-old Caucasian man presented to the emergency department with bloody diarrhoea, diffuse abdominal pain and fever with 1-week duration. He had just returned from Angola where he had been treated for a presumed Clostridium difficile infection without improvement. He had no relevant medical or familiar history except for hypertension and prostate benign hyperplasia. He was drowsy, feverish and eupnoeic. His oxygen saturation on pulse oximetry was 92%, blood pressure was 173/99 mm Hg and pulse rate 100 beats per minute. Except for a distended, silent and painful abdomen, particularly on lower quadrants, the rest of the examination was unremarkable. A CT showed a mesh-like mass inside the rectum conditioning colonic obstruction and distention. This turned to be a giant granadilla's seeds phytobezoar and was removed endoscopically. Five days later, the patient had a colonic perforation requiring total colectomy. He made a full recovery after rehabilitation for 3 months.

http://ift.tt/2BLMxat

Successful management of benign paroxysmal positional vertigo (BPPV) in a patient who was ventilated with a C3 complete spinal injury on a regional spinal unit intensive care

Benign paroxysmal positional vertigo (BPPV) occurs in 14.5% of patients with spinal cord injury (SCI) and may require intervention on intensive care unit (ICU). A 61-year-old man was admitted to a spinal injury ICU with a traumatic C3 complete SCI following a mountain bike accident. Ventilated but stable he complained of severe dizziness on rolling, during personal cares, which lasted for 40 s. Clinical examination was limited due to the injury and ventilation. Subjective questioning, visio-ocular control and a modified Dix-Hallpike and roll tests confirmed a right posterior canalithiasis BPPV. A modified right Epley was performed with assistance of four people, medical supervision, monitoring of tracheal ventilation and vital signs. No adverse reaction was observed. Resolution of dizziness on rolling was achieved with no recurrence at 1 year. BPPV can be successfully and safely managed on ICU.

http://ift.tt/2sm29h2

Ventricular tachyarrhythmia in a 78-year-old woman with essential thrombocythaemia

Anagrelide is a phosphodiesterase-3 inhibitor used in the treatment of essential thrombocythaemia. Cardiovascular side effects such as ventricular tachycardia and cardiomyopathy are rare but potentially fatal and should be made known to patients before starting the medication. It usually arises within the first 6 months after initiation of therapy and may be dose related. The elderly population are particularly susceptible. These cardiotoxicities result from an increase in cyclic AMP that induces positive inotropic and chronotropic effects and are often reversible with cessation of use. We report a case of a 78-year-old woman with essential thrombocythaemia and recently started on anagrelide who presented with syncope and multiple bruises and facial trauma and found to have developed ventricular tachyarrhythmia.

http://ift.tt/2BMLfMd

Tinea of vellus hair: a diagnostic and therapeutic challenge

Tinea infection is a common superficial fungal infection of the skin, hair and nails. Tinea of vellus hair is a rare form of dermatophytosis that is difficult to diagnose and treat. Herein, the authors report the case of a patient who had an itchy rash on the cheek. Microscopic and mycological studies confirmed the diagnosis of tinea of vellus hair. The patient was treated with systemic antifungal therapy with clinical improvement. This report aims to describe an infrequent subtype of common disease and review clinical clues, tools for diagnosis as well as treatment plans.

http://ift.tt/2sm2bWc

Successful treatment of non-tuberculous mycobacterial pleurisy with acute fever by antimycobacterial therapy

We report two cases of pleurisy caused by non-tuberculous mycobacteria followed by pneumothorax. The onset of pleurisy was accompanied by acute fever. Cultured samples of the pleural effusions from the two patients, an 80-year-old man and an 87-year-old woman, were ultimately found to contain Mycobacterium intracellulare and Mycobacterium kansasii, respectively. Both patients were initially administered antibiotics, but their fevers persisted. Therefore, different combinations of antimycobacterial drugs were used, which reduced the fever in a few days. In these patients, pleurisy caused by non-tuberculous mycobacteria followed by pneumothorax was characterised by acute fever and improvement in the fever after administration of antimycobacterial drugs; however, the aetiology remains to be clarified.

http://ift.tt/2BN2JIt

Reversible bioprosthetic valve thrombosis from eosinophilia

A 31-year-old man with a mitral bioprosthetic valve presented with recent worsening of exertional dyspnoea 7 years after the mitral valve replacement. Evaluation revealed an increased gradient across the thickened mitral bioprosthetic valve leaflets. Marked eosinophilia was present and was considered as a putative cause for bioprosthetic valve thrombosis. The treatment with systemic corticosteroids and oral anticoagulation led to complete resolution of symptoms with significant decrease in mitral bioprosthetic valve gradient and leaflet thinning. The case is reported to highlight the fact that eosinophilia may cause reversible bioprosthetic valve thrombosis.

http://ift.tt/2sfjqIF

Acute haemorrhage from a retroperitoneal liposarcoma: a rare presentation

A previously healthy 61-year-old Caucasian woman presented to the emergency department after collapsing at home with associated abdominal pain radiating to her back. An urgent CT angiogram was requested to rule out a ruptured aortic aneurysm. This showed a large 21 cm fat-containing lesion arising from the mid-pole of the left kidney, with an adjacent 4 cm perirenal haematoma. An initial diagnosis of a ruptured angiomyolipoma was made. Her haemoglobin was 105 g/L, with a creatinine of 104 mmol/L and an eGFR of 47 mL/min. Her clotting profile was normal. Following resuscitation, she was taken to the operating room and underwent an emergency open left radical nephrectomy via a left flank incision. Her recovery was uneventful and she was discharged home after 6 days. The histopathology confirmed a well-differentiated liposarcoma.

http://ift.tt/2BMi3VO

Down syndrome with posterior cortical atrophy

Down syndrome (DS) is caused by trisomy of chromosome 21. The average age of onset of Alzheimer's disease (AD) ranged from 50 to 55 years in DS, with early symptoms usually characterised by changes in behaviour and executive dysfunction. On the other hand, posterior cortical atrophy (PCA) is a rare neurodegenerative syndrome characterised by progressive impairment of visual functions in the absence of visual deficits and a pattern of atrophy involving posterior cortex. This syndrome is mostly caused by AD pathology. We report the case of patient with DS who developed PCA. While atypical variants of AD are commonly associated with an early age at onset, all focal forms of AD may potentially appear in DS. Specifying the phenotype has an impact on the care of DS patients and could help us to know the evolution. It could also provide a better understanding of the underlying mechanisms of focal forms.

http://ift.tt/2shD3Qn

Nasopharyngeal angiofibroma in a 32-year-old man

Nasopharyngeal angiofibroma (NA) is a benign, highly vascularised tumour of the nasopharynx, which typically occurs in young males aged 14–25 years. We report an interesting case of an NA arising de novo in a 32-year-old male. He was referred to our facility for severe nasal haemorrhage after biopsy of a left nasopharyngeal mass. In the operating room, extensive bleeding was noted, and an endoscopic medial maxillectomy was performed, and the left internal maxillary artery was ligated allowing for near total resection of the lesion. The pathological specimen confirmed the diagnosis of NA. To our knowledge, this is one of the oldest patients presenting with a de novo NA, in his fourth decade of life, confirming that this diagnosis must be considered in all those with large nasopharyngeal masses.

http://ift.tt/2BNC7Hi

Melioidosis: the great mimicker presenting as spondylodiscitis

Melioidosis, a syndrome with protean clinical manifestations, is caused by Gram-negative soil saprophyte Burkholderiapseudomallei. Among its diverse clinical presentations, the involvement of spine is a rare phenomenon and can mimic tuberculosis on presentation. A 65-year-old female with a known case of diabetes presented with fever with lower back pain. Blood culture grew Staphylococcus aureus, and as per sensitivity report, clindamycin and cefazolin were started. X-ray and MRI lumbosacral spine showed spondylodiscitis (likely Koch's). Decompression and biopsy were done, and a sample was sent for microbiological investigations that showed no growth of any significant pathogen; furthermore, all tests for tuberculosis diagnosis also remained negative. Active Melioidosis Detect Lateral Flow Assay was used on the tissue sample, which was positive for B. pseudomallei Capsular Polysaccharide (CPS) antigen; the case was confirmed by typethree secretion system 1 PCR for melioidosis. Antibiotics were changed to parenteral ceftazidime for 2 weeks followed by oral cotrimoxazole. A dedicated team of microbiologists and physicians is required to identify and treat the disease.

http://ift.tt/2shCWUX

Bleeding or clotting: an intracranial dilemma

Description

We present a complex diagnosis of a 25-year-old woman with a background of ulcerative colitis (UC) at risk of both intracranial haemorrhage and thrombosis.

The patient presented to the emergency department having collapsed at work, obtaining a head injury while falling to the floor and suffering an 8 min seizure in the immediate aftermath. The patient had an acute flare of her UC 2 weeks previously, and her medical history included a deep vein thrombosis following a long-haul flight and a pulmonary embolism while taking contraceptive pill/oral contraceptives.

The patient denied a headache preceding her collapse, although she admitted her head had 'felt fuzzy' during the previous 2 days. CT of the head was performed (figure 1A,B), revealing patchy haemorrhagic changes in the right frontal lobe. Abnormal hyperdensity of the superior sagittal sinus and of a cortical vein was also noted; the patient underwent MRI of the head and CT...

http://ift.tt/2BPdMkg

Isolated splenic mucormycosis in a case of aplastic anaemia

Mucormycosis, a rare opportunistic infection seen in immunocompromised hosts, is caused by fungi of Mucorales family. It may be confined to the organs, such as rhinocerebral and pulmonary mucormycosis, or may cause disseminated infection. A 14-year-old boy presented to our clinic with fever and left upper quadrant abdominal pain, and on evaluation was found to have pancytopaenia, and imaging revealed ill-defined splenic collection with thrombus in the splenic vein. He was started on empirical intravenous antibiotics, followed by antifungals empirically as he did not show any improvement clinically. Eventually, splenectomy was done, which on histopathological examination revealed mucormycosis. The patient finally succumbed to his illness as he developed peritonitis and refractory shock. To date, only two cases of isolated splenic mucormycosis have been reported. Aggressive treatment is needed, which includes the use of antifungals (amphotericin B) and surgical debridement or resection of the involved tissues or organs.

http://ift.tt/2sgKU0F

Pretreatment neutrophil-to-lymphocyte ratio is associated with outcome of advanced-stage cancer patients treated with immunotherapy: a meta-analysis

Abstract

Background

To investigate the association between pretreatment blood neutrophil-to-lymphocyte ratio (NLR) and clinical outcomes for advanced-stage cancer patients treated with immunotherapy.

Methods

We conducted a comprehensive literature search to assess the relationship between pretreatment blood NLR and overall survival (OS) or progression-free survival (PFS) in advanced-stage cancer patients treated with immunotherapy. Published data including hazard ratios (HRs) and related 95% confidence interval (CI) were extracted. Pooled estimates of treatment outcomes were calculated using RevMan 5.3.5.

Results

Twenty-seven studies with 4647 patients were included in the current study. The pooled results suggested that high pretreatment blood NLR was correlated with significant shorter OS (HR = 1.98, 95% CI 1.66–2.36, P < 0.001) and PFS (HR = 1.78, 95% CI 1.48–2.15, P < 0.001). Subgroup analysis stratified by study targets revealed that anti-VEGF/VEGFR therapy (HR = 2.04, 95% CI 1.61–2.60, P < 0.001) and immune checkpoints blockade (HR = 2.16, 95% CI 1.86–2.51, P < 0.001) were significantly associated with inferior OS while other targets (HR = 1.63, 95% CI 0.89–2.99, P = 0.120) were not associated with OS. There was no correlation between distinct NLR cutoff values and OS ( \({r^}}\) = 0.218, P = 0.329) or PFS benefit ( \({r^}}\) = − 0.386, P = 0.140). Of note, HRs of PFS showed significant correlation with HRs of OS ( \({r^}}\) = 0.656, P = 0.015).

Conclusion

Elevated pretreatment blood NLR was a promising prognostic and predictive biomarker for advanced-stage cancer patients treated with immunotherapy.

http://ift.tt/2ES8rY4

Ocular Injury due to Potassium Permanganate Granules

Purpose: We report a rare case of ocular injury due to potassium permanganate (KMnO₄) granules in a child. Methods: This is a retrospective case report. Results: A 2-year-old boy was transferred to our emergency room with severe pain in his right eye, inflamed eyelids, and brownish stains on his fingers. Chemical injury was suspected. Copious eye irrigation was immediately performed. Diffuse brownish splotches were then observed at the inferior bulbar conjunctiva. Otherwise, systemic organs were intact. Complete eye exam under general anesthesia revealed a 5-mm epithelial defect at the central cornea, along with generalized conjunctival injection and limbal ischemia, inferiorly. Multiple semi-dissolved granules of KMnO₄ trapped in the inferior fornix were identified. The chemical particles were gradually washed out and removed; however, the brownish stains remained. The patient received preservative-free steroid, antibiotic eye drops, and lubricants as regular management for mild to moderate degree of ocular burn. Pseudomembrane developed early and transformed into symblepharon within a few days after the injury. Membrane adhesion was lysed, and more aggressive medications were then substituted. Commercial amniotic membrane (PROKERA®) was also applied to promote wound healing and to prevent recurrence of symblepharon. The ocular surface was eventually restored, and corneal transparency was preserved. Conclusion: Ocular injury with the granular form of KMnO₄ is rare. Its toxicity is comparable to concentrated KMnO₄ solution. However, the dissolved particles that had been absorbed in the stained conjunctiva were continuously released and damaged the ocular surface more than we primarily anticipated. Awareness of this condition and prompt management yield a good treatment outcome.
Case Rep Ophthalmol 2018;9:132–137

http://ift.tt/2FY230O

Isolated Medial Orbital Wall Fracture Associated with Enophthalmos in a Paediatric Patient: An Unusual Presentation

Purpose: To report a case of isolated medial orbital wall fracture with enophthalmos in a paediatric patient and describe the clinical presentation and findings by means of computed tomography (CT) of the head and eyes. Methods: We looked at the patient's medical and ophthalmologic history, and an ophthalmologic examination and a CT of the head were performed at baseline. Results: A 14-year-old boy was admitted to the emergency department of our institution with ecchymosis of his right eyelids secondary to a sport accident. Physical examination revealed a moderate limitation of upgaze without diplopia. CT showed a medial orbital wall fracture without haemorrhage and a gross accumulation of air in the right eyelid with pressure exertion over the right globe and enophthalmos. The patient was treated conservatively with oral antibiotics and steroids showing dramatic improvement within 1 week. Enophthalmos and periorbital emphysema were completely resolved within 3 months after the accident as indicated by CT. Conclusions: We conclude that surgical intervention and intravenous treatment are not warranted in similar cases of medial orbital wall fracture. Medical history, clinical and paraclinical evaluations, and a regular follow-up, including CT, are needed though to avoid complications such as painful abduction, horizontal diplopia, pseudo sixth nerve paresis, or pseudo Duane.
Case Rep Ophthalmol 2018;9:126–131

http://ift.tt/2H0R08A

Evaluation of the success of obstructive sleep apnea surgery using criteria based on long-term symptoms and incident hypertension

Abstract

Objective

To identify appropriate success criteria, based on long-term symptoms and incident hypertension, after surgery for obstructive sleep apnea (OSA).

Methods

This observational cohort study included 97 adult OSA patients (90 men) who underwent surgical treatment at our tertiary medical center. Subjective symptoms [witnessed sleep apnea and snoring, and Epworth sleepiness scale (ESS) scores] were evaluated through a telephone survey, and incident hypertension was assessed from medical records. The subjects were divided into success and failure groups according to seven different criteria, and data were analyzed to identify the criteria that could significantly differentiate the success from failure groups.

Results

The participants had a mean age of 48.8 ± 11.9 years and a mean preoperative body mass index of 26.5 ± 3.5 kg/m². The mean preoperative and postoperative apnea–hypopnea index (AHI) values were 36.1/h and 19.4/h, respectively. The mean follow-up duration was 77.0 ± 31.1 months. Postoperative witnessed apnea, snoring, and the ESS scores decreased significantly compared to preoperative scores in both the success and failure groups based on most of the seven criteria. Among the seven criteria, success and failure groups based on a postoperative AHI cutoff of 15 or 20/h differed significantly in witnessed apnea, snoring, or ESS scores. Kaplan–Meier survival analysis based on incident hypertension revealed that no criterion could significantly distinguish between the two groups.

Conclusions

Our results suggest that some of the success criteria analyzed may be more useful in differentiating between success and failure groups after surgery, in terms of long-term improvement of subjective OSA-related symptoms.

http://ift.tt/2nU5kqB

Swallowing evaluation after surgery for obstructive sleep apnea syndrome: uvulopalatopharyngoplasty vs. expansion pharyngoplasty

Abstract

Purpose

To evaluate the effect of pharyngeal surgery on swallowing pattern in patients with obstructive sleep apnea syndrome (OSAS), and to compare two surgical techniques: uvulopalatopharyngoplasty (UPPP) and expansion pharyngoplasty (EP), through videofluoroscopy.

Methods

Longitudinal prospective cohort, in a tertiary referral center. 17 adult patients were enrolled this study, divided into two groups: patients who underwent UPPP (n = 10) or EP (n = 7). Swallowing videofluoroscopy (for both liquid and pasty consistences) was assessed at three different periods: before surgery, and at 14 and 28 days following surgery. Comparisons were performed between pre- and post-operative (PO) swallowing conditions in the same patient, and between surgical techniques.

Results

Asymptomatic OSAS patients already presented altered swallowing pattern before surgery. Both surgical procedures led to an increased hyoid movement time and an increased frequency of laryngeal penetration in early PO during liquid ingestion. For pasty consistency, both techniques reduced velum movement time and increased pharyngeal transit time and the rate of stasis in hypopharynx. All these parameters reached or tended to reach the pre-operative indices at day PO 28.

Conclusions

OSAS patients show sub-clinical changes in swallowing pattern before surgery. Both surgical techniques are related to transitory changes in swallowing biomechanics. Complete or partial reversal to pre-operative swallowing parameters occurs 1 month after both surgery techniques.

http://ift.tt/2nNz4WT

Issue Information

http://ift.tt/2EOHYuv

Differentiating Simple Hepatic Cysts from Mucinous Cystic Neoplasms: Radiological Features, Cyst Fluid Tumour Marker Analysis and Multidisciplinary Team Outcomes

Labib, PLZ; Aroori, S; Bowles, M; Stell, D; Briggs, C; (2016) Differentiating Simple Hepatic Cysts from Mucinous Cystic Neoplasms: Radiological Features, Cyst Fluid Tumour Marker Analysis and Multidisciplinary Team Outcomes. Digestive Surgery , 34 pp. 36-42. 10.1159/000447308 .

http://ift.tt/2BOyrVT

Speech, stone tool-making and the evolution of language

Cataldo, DM; Migliano, AB; Vinicius, L; (2018) Speech, stone tool-making and the evolution of language. PLoS One , 13 (1) , Article e0191071. 10.1371/journal.pone.0191071 . Green open access

http://ift.tt/2Bg8Qns

Matrix Single Stage Distributed Amplifier Design for Ultra Wideband Application

Odedeyi, TO; Darwazeh, I; (2018) Matrix Single Stage Distributed Amplifier Design for Ultra Wideband Application. In: (Proceedings) 24th IEEE International Conference on Electronics, Circuits and Systems (ICECS). IEEE (In press).

http://ift.tt/2BLsybN

R.S. Thomas's Poetics of Insomnia

Beaumont, M; (2018) R.S. Thomas's Poetics of Insomnia. Essays in Criticism , 68 (1) pp. 74-107. 10.1093/escrit/cgx027 .

http://ift.tt/2BkdK38

Regulating Offense, Nurturing Offense

Simpson, RM; (2018) Regulating Offense, Nurturing Offense. Politics, Philosophy and Economics 10.1177/1470594X17741228 . (In press).

http://ift.tt/2BLVJvE

Multiple Levels of Control Determine How E4bp4/Nfil3 Regulates NK Cell Development

Kostrzewski, T; Borg, AJ; Meng, Y; Filipovic, I; Male, V; Wack, A; DiMaggio, PA; Kostrzewski, T; Borg, AJ; Meng, Y; Filipovic, I; Male, V; Wack, A; DiMaggio, PA; Brady, HJM; - view fewer (2018) Multiple Levels of Control Determine How E4bp4/Nfil3 Regulates NK Cell Development. Journal of Immunology , 200 (4) pp. 1370-1381. 10.4049/jimmunol.1700981 .

http://ift.tt/2BjgEFh

On coercivity of boundary integral functionals in high-frequency scattering

Smyshlyaev, VP; (2017) On coercivity of boundary integral functionals in high-frequency scattering. Presented at: Oberwolfach Workshop ID 1729: Material Theories, Oberwolfach, Germany.

http://ift.tt/2BLxBcp

The algal chloroplast as a synthetic biology platform for production of therapeutic proteins

Dyo, YM; Purton, S; (2018) The algal chloroplast as a synthetic biology platform for production of therapeutic proteins. Microbiology , 164 pp. 113-121. 10.1099/mic.0.000599 .

http://ift.tt/2BkYin0

Manufactured Exoticism and Retelling The Story of a Crime: The Case of Sjöwall and Wahlöö’s Reception in France

Grydehoj, A; (2017) Manufactured Exoticism and Retelling The Story of a Crime: The Case of Sjöwall and Wahlöö's Reception in France. Australian Journal of Crime Fiction , 1 (1)

http://ift.tt/2BLFZbZ

Indirect epistemic reasons and religious belief

Lougheed, K; Simpson, RM; (2017) Indirect epistemic reasons and religious belief. Religious Studies , 53 (2) pp. 151-169. 10.1017/S0034412516000202 . Green open access

http://ift.tt/2Bjge1F

Fine table linen in England, 1450-1750: The supply, ownership and use of a luxury commodity

Mitchell, David Malcolm; (1999) Fine table linen in England, 1450-1750: The supply, ownership and use of a luxury commodity. Doctoral thesis (Ph.D), UCL (University College London). Green open access

http://ift.tt/2BOhURF

Temporal trends of transmitted HIV drug resistance in a multinational seroconversion cohort

Olson, A; Bannert, N; Soennerborg, A; de Mendoza, C; Price, M; Zangerle, R; Chaix, M-L; ... Porter, K; + view all Olson, A; Bannert, N; Soennerborg, A; de Mendoza, C; Price, M; Zangerle, R; Chaix, M-L; Prins, M; Kran, A-MB; Gill, J; Paraskevis, D; Porter, K; - view fewer (2018) Temporal trends of transmitted HIV drug resistance in a multinational seroconversion cohort. AIDS , 32 (2) pp. 161-169. 10.1097/QAD.0000000000001689 .

http://ift.tt/2Biuh7x

Climate change, cooperation and moral bioenhancement

Handfield, T; Huang, PH; Simpson, RM; (2016) Climate change, cooperation and moral bioenhancement. Journal of Medical Ethics , 42 (11) pp. 742-747. 10.1136/medethics-2016-103593 . Green open access

http://ift.tt/2BM26io

Permissivism and the Arbitrariness Objection

Simpson, RM; (2017) Permissivism and the Arbitrariness Objection. Episteme , 14 (4) pp. 519-538. 10.1017/epi.2016.35 . Green open access

http://ift.tt/2BM1OrO

Short courses of daily prednisolone during upper respiratory tract infections reduce relapse frequency in childhood nephrotic syndrome

Abeyagunawardena, AS; Thalgahagoda, RS; Dissanayake, PV; Abeyagunawardena, S; Illangasekera, YA; Karunadasa, UI; Trompeter, RS; (2017) Short courses of daily prednisolone during upper respiratory tract infections reduce relapse frequency in childhood nephrotic syndrome. Pediatric Nephrology , 32 (8) pp. 1377-1382. 10.1007/s00467-017-3640-5 .

http://ift.tt/2Bi1wba

Effective Principals Serving in High Poverty Schools in Chile: Managing Competing Realities

Riley, K; Montecinos, C; Ahumada, L; (2017) Effective Principals Serving in High Poverty Schools in Chile: Managing Competing Realities. Procedia - Social and Behavioral Sciences , 237 pp. 843-849. 10.1016/j.sbspro.2017.02.181 . Green open access

http://ift.tt/2BNnaoE

NODAL/Activin signalling to chromatin: mechanisms of SMAD2-regulated transcription

Coda, Davide Martino; (2018) NODAL/Activin signalling to chromatin: mechanisms of SMAD2-regulated transcription. Doctoral thesis (Ph.D), UCL (University College London).

http://ift.tt/2Bg8Fsi

Defining 'Speech': Subtraction, Addition, and Division

Simpson, RM; (2016) Defining 'Speech': Subtraction, Addition, and Division. Canadian Journal of Law and Jurisprudence , 29 (2) pp. 457-494. 10.1017/cjlj.2016.20 . Green open access

http://ift.tt/2BOhRoX

Economic structure and energy savings from energy efficiency in households

Freire-González, J; Font Vivanco, D; Puig-Ventosa, I; (2016) Economic structure and energy savings from energy efficiency in households. Ecological Economics , 131 pp. 12-20. 10.1016/j.ecolecon.2016.08.023 . Green open access

http://ift.tt/2BkXT3Y

Disciplining and Governing Headteachers? Exploring Headteachers' Administrative Placement in the Local Education Department in Taiwan

Chen, Hung-Chang; (2018) Disciplining and Governing Headteachers? Exploring Headteachers' Administrative Placement in the Local Education Department in Taiwan. Doctoral thesis (Ph.D), UCL (University College London).

http://ift.tt/2BM19Xm

Early laser intervention to reduce scar formation: a systematic review

Abstract

The ability of laser treatment to affect wound healing and subsequently minimize scar formation has been investigated in recent years. However, no systematic review links these clinical trials. The aim of this study is to systematically review and evaluate clinical evidence for early laser intervention to reduce scar formation in studies where laser treatment were introduced less than 3 months after wounding. We searched PubMed using relevant key words in June 2017. Titles, abstracts and articles were sorted according to inclusion and exclusion criteria. Methodological quality was evaluated according to Cochrane Collaborations risk-of-bias assessment guideline by two independent authors. Twenty-five articles met the inclusion criteria. In total 22 of 25 studies were controlled studies and 17 of 25 studies compared laser treatment versus untreated control scars. The following laser devices have been investigated; pulsed-dye laser (PDL) laser, potassium-titanyl-phosphat (KTP) laser, fractional Erbium:Glass 1540 nm/1550 nm, fractional/full-ablation erbium-doped-yttrium-aluminium-garnet (Er:YAG) laser, or fractional CO₂-laser. Eighteen studies applied laser treatments 2-4 times with 2-8 weeks intervals, while 7 studies applied only one laser treatment. Follow-up time ranged from 1-12 months with 18 studies using a follow-up time ≤3 months. In general, laser treated wounds and scars showed benefit from laser intervention, though not always reaching significance. Significant scar improvement were found in: 3 of 4 studies using laser treatment in inflammation phase, in 6 of 16 studies with laser initiated in the proliferation phase and in 2 of 5 studies in the remodeling phase. High risk-of- bias were found in randomization and allocation concealment, and low risk-of-bias with regard to blinding of outcome assessment and lost to follow-up. In conclusion, laser intervention when introduced in inflammation, proliferation or remodeling phase has the potential to reduce cutaneous scar formation. Further high quality studies are needed before standard protocols can be implemented in clinical practice.

This article is protected by copyright. All rights reserved.

http://ift.tt/2Edp7rV

Predatory conferences: Caveat emptor!

Abstract

It is a dream of every young researcher or postgraduate student to publish an article in an indexed journal and to present an oral paper or poster at a reputed conference. Unfortunately these young researchers are commonly fallen prey to predatory publications which have become a major hazard in recent years because of their rapid multidisciplinary proliferation. In fact many dermatologists daily come across with such fraudulent journals which have quite matured and rooted and now days, flourished with conference version i.e. 'Predatory conferences'. In the name of greater good of Open Access, these fraudulent journals and organizers are prospering by trading on the naïve doctors and academicians, who look to enhance their academic resume and to earn tenure and promotion.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EQ86FA

Granulomatous Tattoo reaction with Associated Uveitis successfully treated with methotrexate

Abstract

Few reports describe tattoo granulomas with the simultaneous development of intraocular inflammation without any evidence of systemic sarcoidosis at the time of presentation. Here, we present a patient with no prior diagnosis of sarcoidosis who developed inflammation of tattooed skin and shortly after bilateral uveitis.A 52-year-old previously healthy woman presented with a 8 months history of swelling and itching located in all of her 11 black tattoos. Approximately 5 months later she developed complaints of blurred vision, diagnosed as recurrent uveitis anterior by an ophthalmologist.

This article is protected by copyright. All rights reserved.

http://ift.tt/2Eakn6v

Normoglycemic glucagonoma syndrome associated with necrolytic migratory erythema

Abstract

Glucagonoma is an extremely rare tumor of the pancreatic alpha-cells. Its estimated annual incidence is 1 case per 20 million individuals. Necrolytic migratory erythema (NME) is the hallmark clinical sign. We report a patient with normoglycemic glucagonoma and NME.

A 44-year-old male patient presented with a 4-year history of unexplained exanthema and unintentional weight loss. Skin examination revealed a mildly pruritic rash on the trunc, the extremities and the face (fig 1). One found erythematous polycyclic migratory lesions with scaling and crusting margins and central resolution. Histological examination showed parakeratotic epidermis and upper epidermal pallor with vacuolated keratinocytes (fig 2). The glucagon level was elevated at 444,5 pg/mL (normal 5,1-62,3 pg/mL). Fasting blood sugar and HbA1c where within normal range. The insulin level was raised (112 μU/mL, normal 3,21-16,32 μU/mL). CT scan of the abdomen showed two masses in the tail of pancreas, measuring 3,8 cm and 2,6 cm in diameter and thrombosis of the left common femoral vein. In the somatostatin receptor scintigraphy, the pancreatic masses stained positive. Multiple endocrine neoplasia type 1 syndrome (MEN 1) was ruled out by genetic analysis. Surgical resection of the pancreatic tail with local lymphadenectomy and splenectomy was performed. Pathological examination confirmed two low-grade neuroendocrine neoplasms consistent with glucagonoma. Tumor cells were also found in 1 out of 19 removed lymph nodes. After surgery, the patients general condition improved rapidly and the skin lesions completely disappeared.

This article is protected by copyright. All rights reserved.

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Once weekly targeted excimer light produced modest re-pigmentation of vitiligo over a 20-week period

Abstract

Vitiligo is an acquired disorder of depigmentation resulting from the loss of functional epidermal melanocytes. It has a worldwide prevalence of 0.5-2%. Vitiligo can have a major negative impact on patients' quality of life.Current treatments include topical corticosteroids and immunomodulators, systemic immunosuppressants, cosmetic camouflage, ultraviolet radiation and surgical procedures, e.g. punch grafting. Recently, targeted phototherapy using the 308-nm xenon-chloride (XeCl) monochromatic excimer light (MEL) delivered by a laser or a lamp emitting non-coherent monochromatic light has been used.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EchEcM

The regular use of an emollient improves symptoms of atopic dermatitis in children: a randomized controlled study

Abstract

Background

Emollients are considered as a first-line therapy for the treatment of atopic dermatitis (AD). However, evidence-based proof that the regular use of emollients reduces AD severity is lacking.

Objective

To assess whether the regular use of emollients results in a reduction of AD severity in children with AD.

Methods

In this multicenter randomized, parallel group, open-label study, children with mild to moderate AD were recruited during a flare. After flare resolution with a topical corticosteroid, patients were randomized to V0034CR emollient, reference emollient, or no emollient (1:1:1 ratio), for 12 weeks. AD severity was assessed regularly by physicians (SCORAD and subcomponents, IGA) and by parents (PO-SCORAD and POEM).

Results

335 patients were randomized to V0034CR (n=111), reference emollient (n=116) or no emollient (n=108). After 12 weeks of treatment, SCORAD score was reduced by 5.28 points in the V0034CR group and by 3.36 points in the reference emollient group compared with the no emollient group (+4 points; p<0.001 in both emollient groups vs no emollient group). In a similar manner, PO-SCORAD score was reduced by 4.88 and 2.67 points in the V0034CR and reference emollient groups, respectively, but increased by 2.90 points in the no emollient group (p<0.001). Similar results were observed for POEM. A continuous decrease in all scores was observed over the 12-week treatment period. At the end of the study, the percentage of patients in complete remission (i.e. without a new flare over the treatment period) was higher in the V0034CR (59.5%) and reference emollient (44.3%) groups than in the no emollient group (29.8%) (p<0.001).

Conclusion

These results demonstrate that the regular use of emollients in children with mild to moderate AD reduces the severity of symptoms and, therefore, support their use as a first-line treatment for these patients.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EQMngP

Variation in the diagnosis and clinical management of Lentigo Maligna across Europe: a survey study among EADV members

Abstract

Background

Lentigo maligna (LM), a form of melanoma in situ, is treated to prevent progression to lentigo maligna melanoma (LMM). Surgical treatment is the gold standard. However, treatment guidelines are based on expert opinion and comparative studies are lacking.

Objective

The objective of this study is to assess the diagnostic methods and clinical management of LM patients among European dermatologists and residents.

Methods

A survey consisting of 29 questions about diagnostic methods and treatment options used for LM patients was sent to 3308 members of the European association of Dermatologists and Venereologists (EADV).

Results

Most questions were multiple choice, and multiple answers could be ticked per question.

A total of N = 415 (12.5%) completed surveys were included into the analyses. A combination of clinical diagnosis 65.7%, dermatoscopy 83.4% and histopathology 88.2% is used by most respondents to diagnose LM. Tissue for histopathological evaluation was collected using most often by a single punch biopsy in 61.0%, The most common treatment for LM patients <60 years of age is surgery (97,6%). For LM patients >70 years of age, 66.8% of the respondents preferred surgical treatment. Non-surgical options such as radiotherapy (17.0%), topical imiquimod (30.6%), watchful waiting (19.6%) or cryotherapy (20.4%) were used in this elderly group. Sub-analysis showed that respondents who take into account patient preference, used topical imiquimod, radiotherapy and watchful waiting more often.

Conclusion

In conclusion, the results of this survey show that there is a variance in the diagnostic methods and treatment modalities used for LM across Europe. Surgery remains the most utilized option. However, non-surgical options, such as topical imiquimod and radiotherapy, are most often used for elderly patients. We recommend that future studies focus on patient preference and compare surgical to non-surgical therapy.

This article is protected by copyright. All rights reserved.

http://ift.tt/2Eak8Z9

Autosomal recessive hyper-IgE syndrome in two brothers of a Chinese family with a novel mutation in DOCK8 gene

Abstract

The hyper-IgE syndromes (HIES) are complex primary immunodeficiency disorders, including autosomal dominant form (AD-HIES) and recessive form (AR-HIES). Here we describe two boys with AR-HIES in a Chinese family and a novel mutation of DOCK8 gene was identified.The index patient was an 8-year-old boy. He presented with recurrent flexural skin lesions (similar with atopic dermatitis (AD)) for more than 5 years (Fig. 1a,b), and a 6-months history of severe molluscum contagiosum(Fig. 1c) and verruca plana(Fig. 1d). He had repeated respiratory tract infection, nasosinusitis and otitis media every year, with a history of asthma and peanut allergy.

This article is protected by copyright. All rights reserved.

http://ift.tt/2ERVgX6

Skin manifestations in patients with hcv–related chronic liver disease

Abstract

Hepatitis C virus (HCV) is able to trigger both hepatic and extra-hepatic manifestations (1,2).

Among the latter, the most investigated is mixed cryoglobulinemia vasculitis (MCV), especially of type II (3, 4), usually detected in 40-60% of patients chronically infected with enhanced the risk to develop lymphomas (5). Autoimmune disorders including thyroid disease, and diabetes mellitus have also been associated with chronic HCV infection (6). Skin manifestations may occur in up to 17% of HCV positive patients (7) and are the result of the immuno-mediated damage induced by the virus itself, and/or the associated liver injury (8). Cutaneous palpable purpura is the most frequent (70-90%). Porfiria cutanea tarda (PCT) is detected in 50% of HCV-infected patients (8). Observational and cohort studies described an association between psoriasis and HCV infection independently from exposure to interferon treatments (9). Instead, cutaneous Kaposi's sarcoma was reported only once in association with HCV (10). In this retrospective study, we aimed to evaluate the frequency and clinical features of skin disorders in a large cohort of patients with HCV-related liver disease.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EajZ83

Cytokeratin 19 expression is a risk factor for metastasis in cutaneous squamous cell carcinoma

Abstract

Cytokeratin 19 (CK19) expression has been reported to be a risk factor for metastasis in oral squamous cell carcinoma. However, the significance of CK19 expression in cutaneous squamous cell carcinoma (cSCC) is unclear. Although some studies have reported CK19 expression in cSCC, no study has focused on the relationship between CK19 expression and metastasis in cSCC. Herein, we evaluate whether CK19 expression is related to metastasis in cSCC.

This article is protected by copyright. All rights reserved.

http://ift.tt/2EMY7kd

Case 4-2018: A Newborn with Thrombocytopenia, Cataracts, and Hepatosplenomegaly

Presentation of Case. Dr. Madeleine I. Matthiesen (Medicine and Pediatrics): A newborn boy was transferred to the neonatal intensive care unit (ICU) of this hospital because of thrombocytopenia and abnormal results on an eye examination for red reflex and on a hearing screening. The patient was…

http://ift.tt/2BhqgjB

Radiotherapy With Double Checkpoint Blockade of Locally Advanced HNSCC

Condition:   Locally Advanced Head and Neck Squamous Cell Carcinoma
Intervention:   Combination Product: Durvalumab + Tremelimumab + RT
Sponsor:   University of Erlangen-Nürnberg Medical School
Not yet recruiting

http://ift.tt/2nLSH1D

Burkitt’s lymphoma with placental invasion diagnosed at cesarean delivery: a case report

Burkitt's lymphoma is a highly aggressive B cell non-Hodgkin lymphoma subtype. Its occurrence in pregnancy is rare and often results in a delayed diagnosis. The treatment plan and prognosis depend on a number ...

http://ift.tt/2EQHAvS

Relationship between swallowing-related quality of life and fiberoptic endoscopic evaluation of swallowing in patients who underwent open partial horizontal laryngectomy

Abstract

Background

Several studies have previously analyzed the relationship between QOL and signs of dysphagia in patients treated for head and neck cancer and have reported heterogeneous findings. To the best of our knowledge, no study has previously investigated this relationship among patients who underwent open partial horizontal laryngectomy (OPHL). The aim of the study is to determine if patient-reported swallowing-related QOL can discriminate between safe and unsafe swallowing in OPHL patients.

Methods

92 type I, type II, and type III OPHL patients at least 6 months postoperatively were recruited. Fiberoptic endoscopic evaluation of swallowing (FEES) was conducted using liquids, semisolids, and solids. FEES recordings were assessed through the penetration–aspiration scale, the pooling score and the dysphagia outcome and severity scale. All patients completed the MD Anderson dysphagia inventory (MDADI). Kruskal–Wallis test and post-hoc Mann Whitney U test were performed to compare MDADI scores among different level of airway invasion, post-swallow pharyngeal residue's degree and overall dysphagia severity. ROC curves were generated to determine diagnostic accuracy of the MDADI.

Results

Statistically significant differences in MDADI scores were found between level of airway invasion with semisolids and solids, degree of pharyngeal residue with solids, and severity of dysphagia. MDADI showed significant diagnostic accuracy only in the detection of moderate/severe pharyngeal residue and severe dysphagia; however, sensitivity and specificity were low.

Conclusions

Investigating patients' perception of swallowing impairment and swallowing-related QOL is not sufficient to discriminate safe and unsafe swallowing in OPHL patients.

http://ift.tt/2BLrJjn

Lights, Camera, Action! New Endomicroscopic Probes Visualize Living Animal Cell Activity

Johns Hopkins researchers report they have developed two new endoscopic probes that significantly sharpen the technology's imaging resolution and permit direct observation of fine tissue structures and cell activity in small organs in sheep, rats and mice

http://ift.tt/2FWCRrx

IMPACT OF TACROLIMUS INTRA-PATIENT VARIABILITY ON LONG-TERM OUTCOME AFTER LIVER TRANSPLANTATION

Misas, MG; De Luca, L; Gros, B; Thorburn, D; Patch, D; Aguilar, P; Costan, G; ... Rodriguez-Peralvarez, M; + view all Misas, MG; De Luca, L; Gros, B; Thorburn, D; Patch, D; Aguilar, P; Costan, G; Montero, JL; O'Beirne, J; Tsochatzis, E; De la Mata, M; Rodriguez-Peralvarez, M; - view fewer (2017) IMPACT OF TACROLIMUS INTRA-PATIENT VARIABILITY ON LONG-TERM OUTCOME AFTER LIVER TRANSPLANTATION. Presented at: 18th Congress of the European Society for Organ Transplantation, Barcelona, Spain.

http://ift.tt/2H0g3IH

Transformations of Scylla and Charybdis: Encounters with Otherness and Ancient Greek Myth in Post-Classical Perspective

Carbone, Marco Benoît; (2018) Transformations of Scylla and Charybdis: Encounters with Otherness and Ancient Greek Myth in Post-Classical Perspective. Doctoral thesis (Ph.D), UCL (University College London).

http://ift.tt/2FZ1Fzs

Freshwater Vulnerability beyond Local Water Stress: Heterogeneous Effects of Water-Electricity Nexus Across the Continental United States

Wang, R; Zimmerman, JB; Wang, C; Font Vivanco, D; Hertwich, EG; (2017) Freshwater Vulnerability beyond Local Water Stress: Heterogeneous Effects of Water-Electricity Nexus Across the Continental United States. Environmental Science & Technology , 51 (17) pp. 9899-9910. 10.1021/acs.est.7b01942 .

http://ift.tt/2GXUgl5

Reply to the letter to the author concerning: ‘Does adenoid hypertrophy affect disease severity in children with allergic rhinitis?’

http://ift.tt/2Eav5Or

The Rehabilitation Effectiveness for Activities for Life (REAL) study: a national programme of research into NHS inpatient mental health rehabilitation services across England

Killaspy, H; King, M; Holloway, F; Craig, TJ; Cook, S; Mundy, T; Leavey, G; ... Bhanbhro, S; + view all Killaspy, H; King, M; Holloway, F; Craig, TJ; Cook, S; Mundy, T; Leavey, G; McCrone, P; Koeser, L; Omar, R; Marston, L; Arbuthnott, M; Green, N; Harrison, I; Lean, M; Gee, M; Bhanbhro, S; - view fewer (2017) The Rehabilitation Effectiveness for Activities for Life (REAL) study: a national programme of research into NHS inpatient mental health rehabilitation services across England. Programme Grants for Applied Research , 5 (7) i-xxvi-1-284. 10.3310/pgfar05070 . Green open access

http://ift.tt/2FXTYcq

In reply to “Commentary to: Endoscopic and clinical benefits of hyaluronic acid in children with chronic adenoiditis and middle ear disease”, by Zhengcai Lou

Abstract

Some considerations will be expressed in consideration of the commentary previously published. In particular, we underline that no other medications were administered to the patients during the study period and any clinical evaluation was postponed in case of acute upper respiratory tract infection in the previous 14 days. We strongly advocate antibiotic treatment during any acute otitis media episode, and we agree that topically administered hyaluronic acid should be considered as a supporting treatment, "complementary to traditional therapies" in children with recurrent disease.

http://ift.tt/2C4jL0r

Using pressurised gyration to generate mucoadhesive progesterone-loaded fibres for drug delivery applications

Brako, Francis Asamoah; (2018) Using pressurised gyration to generate mucoadhesive progesterone-loaded fibres for drug delivery applications. Doctoral thesis (Ph.D), UCL (University College London). Green open access

http://ift.tt/2GVQJDQ

Sequencing analysis of the SCA6 CAG expansion excludes an influence of repeat interruptions on disease onset.

Wiethoff, S; O'Connor, E; Haridy, NA; Nethisinghe, S; Wood, N; Giunti, P; Bettencourt, C; Wiethoff, S; O'Connor, E; Haridy, NA; Nethisinghe, S; Wood, N; Giunti, P; Bettencourt, C; Houlden, H; - view fewer (2018) Sequencing analysis of the SCA6 CAG expansion excludes an influence of repeat interruptions on disease onset. J Neurol Neurosurg Psychiatry 10.1136/jnnp-2017-317253 . (In press). Green open access

http://ift.tt/2FWyrBc

Developing Highly Multiplexed Technology for High-throughput Super-resolution Fluorescence Microscopy

Bento Carvalho Almada, Pedro Manuel; (2018) Developing Highly Multiplexed Technology for High-throughput Super-resolution Fluorescence Microscopy. Doctoral thesis (Ph.D), UCL (University College London). Green open access

http://ift.tt/2GY7Hl6

Prosodic focus in three northern Wu dialects: Wuxi, Suzhou and Ningbo

Wang, B; Zhang, Y; Xu, Y; Ding, H; (2017) Prosodic focus in three northern Wu dialects: Wuxi, Suzhou and Ningbo. In: Botinis, Antonis, (ed.) Proceedings of 8th Tutorial and Research Workshop on Experimental Linguistics. (pp. pp. 117-120). National and Kapodistrian University of Athens: Athens, Greece.

http://ift.tt/2FZ050p

Some secret language: how toddlers learn to understand movies

Bazalgette, Cary; (2018) Some secret language: how toddlers learn to understand movies. Doctoral thesis (Ph.D), UCL (University College London). Green open access

http://ift.tt/2H08h1Q

Feasibility of VERDICT-MRI for non-invasive characterisation of rectal cancer microstructure

Tariq, M; Liao, C; Bonet-Carne, E; Plumb, AA; Rodriguez-Justo, M; Alexander, DC; Chand, M; ... Panagiotaki, E; + view all Tariq, M; Liao, C; Bonet-Carne, E; Plumb, AA; Rodriguez-Justo, M; Alexander, DC; Chand, M; Atkinson, D; Panagiotaki, E; - view fewer (2018) Feasibility of VERDICT-MRI for non-invasive characterisation of rectal cancer microstructure. In: (Proceedings) ISMRM 2018 (International Society for Magnetic Resonance in Medicine). Wiley (In press).

http://ift.tt/2FXu74q

Sexualized drug use ('chemsex') and high-risk sexual behaviours in HIV-positive men who have sex with men.

Pufall, EL; Kall, M; Shahmanesh, M; Nardone, A; Gilson, R; Delpech, V; Ward, H; Pufall, EL; Kall, M; Shahmanesh, M; Nardone, A; Gilson, R; Delpech, V; Ward, H; Positive Voices study group, ; - view fewer (2018) Sexualized drug use ('chemsex') and high-risk sexual behaviours in HIV-positive men who have sex with men. HIV Med 10.1111/hiv.12574 . (In press). Green open access

http://ift.tt/2GVYwlb

Posh accent and vocal attractiveness in British English

Jiao, L; Wang, C; Hsu, C; Birkholz, P; Xu, Y; (2017) Posh accent and vocal attractiveness in British English. In: Botinis, Antonis, (ed.) Proceedings of 8th Tutorial and Research Workshop on Experimental Linguistics. (pp. pp. 45-48). National and Kapodistrian University of Athens: Athens, Greece.

http://ift.tt/2FVA6a6

Surgical Management of Thoracolumbar Kyphosis in Patients with Mucopolysaccharidosis

Williams, N; Cundy, PJ; Eastwod, DM; (2017) Surgical Management of Thoracolumbar Kyphosis in Patients with Mucopolysaccharidosis. Presented at: British Orthopaedic Association Annual Congress 2017, Liverpool, United Kingdom.

http://ift.tt/2GWThS2

PDGF signalling during Neural Crest Cell migration

Bahm, I; (2018) PDGF signalling during Neural Crest Cell migration. Doctoral thesis (Ph.D), UCL (University College London). Green open access

http://ift.tt/2FYCQU9

Fully integrated silicon probes for high-density recording of neural activity

Jun, JJ; Steinmetz, NA; Siegle, JH; Denman, DJ; Bauza, M; Barbarits, B; Lee, AK; ... Harris, TD; + view all Jun, JJ; Steinmetz, NA; Siegle, JH; Denman, DJ; Bauza, M; Barbarits, B; Lee, AK; Anastassiou, CA; Andrei, A; Aydin, C; Barbic, M; Blanche, TJ; Bonin, V; Couto, J; Dutta, B; Gratiy, SL; Gutnisky, DA; Hausser, M; Karsh, B; Ledochowitsch, P; Lopez, CM; Mitelut, C; Musa, S; Okun, M; Pachitariu, M; Putzeys, J; Rich, PD; Rossant, C; Sun, W-L; Svoboda, K; Carandini, M; Harris, KD; Koch, C; O'Keefe, J; Harris, TD; - view fewer (2017) Fully integrated silicon probes for high-density recording of neural activity. [Letter]. Nature , 551 (7679) pp. 232-236. 10.1038/nature24636 .

http://ift.tt/2H0ocNm

The anatomy of ICT policies for development and education implementation - the case of secondary schools in Ghana

Awuku, Samuel Kwaku; (2018) The anatomy of ICT policies for development and education implementation - the case of secondary schools in Ghana. Doctoral thesis (Ph.D), UCL (University College London).

http://ift.tt/2FZ1zI6

The effects of zinc supplementation on autophagy in primary human fetal retinal pigment epithelial cells – new molecular target pathway

Emri, E; Yang, X; Kriston-Vizi, J; Ketteler, R; Lengyel, I; (2017) The effects of zinc supplementation on autophagy in primary human fetal retinal pigment epithelial cells – new molecular target pathway. Presented at: 2017 ARVO Annual Meeting, Baltimore, MD, USA.

http://ift.tt/2H1cn9U

Individual differences and romantic compatibility: the relationship between personality traits, eligibility and ideal partner preference

Anderson, Beth Emma; (2018) Individual differences and romantic compatibility: the relationship between personality traits, eligibility and ideal partner preference. Doctoral thesis (Ph.D), UCL (University College London). Green open access

http://ift.tt/2FZ1q7w

Law, Poetics and Legal Fictions. 'Law and Poetics' conference, Cambridge University

Young, CSR; (2018) Law, Poetics and Legal Fictions. 'Law and Poetics' conference, Cambridge University. [Lecture]. Presented at: Law and Poetics, Cambridge, United Kingdom.

http://ift.tt/2GVkc0B

The impact of the flipped classroom instructional model on MPharm students in two pharmacy schools in the UK

Almanasef, Mona Abdulaziz H; (2018) The impact of the flipped classroom instructional model on MPharm students in two pharmacy schools in the UK. Doctoral thesis (Ph.D), UCL (University College London).

http://ift.tt/2FZ0a4d

Ultrasound-guided bilateral rectus sheath block reduces early postoperative pain after laparoscopic gynecologic surgery: a randomized study

Abstract

Purpose

Rectus sheath block (RSB) is an anterior abdominal wall block that reduces postoperative pain associated with midline incisions. This study aims to investigate the effect of ultrasound-guided bilateral RSB (US-BRSB) on postoperative pain and analgesic consumption in patients undergoing laparoscopic gynecologic surgery.

Methods

Sixty patients who underwent laparoscopic gynecologic surgery were allocated to RSB (n = 30) or control (n = 30) group. A bilateral US-BRSB procedure (30 ml of 0.25% ropivacaine) was performed after induction of general anesthesia in the RSB group. The control group proceeded the surgery without sham block. All patients received fentanyl-based intravenous patient-controlled analgesia and rescue analgesics upon demand. Pain was scored by a blinded observer using a verbal numerical rating scale (VNRS) at rest while coughing at 0, 1, 6, 12, 24, and 48 h after postanesthesia care unit (PACU) admission. The primary outcome was the total number of rescue analgesics used in the 48-h postoperative period.

Results

At 0 h, VNRS were lower in the RSB group than in the control, both at rest (median VNRS 4.5 vs. 5, p = 0.02) and while coughing (median VNRS 6 vs. 7, p = 0.004). At 6 h, VNRS scores were lower in the RSB group than in the control while coughing (median VNRS 3 vs. 5, p = 0.01). Fentanyl use as rescue analgesics in the PACU was significantly lower in the RSB group than in the control (27.7 ± 32.1 vs. 53.3 ± 33.7 µg, respectively; p = 0.004). At 48 h postoperatively, the total number of rescue analgesics administered were significantly fewer in the RSB group than in the control (2.5 ± 2.5 vs. 3.9 ± 2.6, respectively; p = 0.04).

Conclusion

US-BRSB reduces the immediate postoperative pain and opioid consumption during the early postoperative period.

ClinicalTrials.gov identifier

NCT02476799, https://clinicaltrials.gov/ct2/show/NCT02476799.

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Acknowledgement to referees 2017

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Multiple bilateral adnexal tumours of the ear: Dermoscopic clues

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Onychoscopy of trachyonychia: An analysis of 30 patients and comparison with onychomycosis

The diagnosis of trachyonychia is often challenging, and misdiagnoses, particularly as onychomycosis, are common.^1-3 The correct diagnosis of trachyonychia is crucial because treatment options for trachyonychia include observation/active non-intervention, topical and systemic steroids, or immunomodulating agents, while onychomycosis should be treated with antifungal agents^{1, 2}. Onychoscopy (nail dermoscopy) is a useful tool for various nail disorders.^4-8 However, a detailed morphologic analysis of trachyonychia has not been performed. Here, we aim to describe the characteristic dermoscopic features of trachyonychia and to explore whether these features are significantly different from those in onychomycosis.

This article is protected by copyright. All rights reserved.

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