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https://www.readbyqxmd.com/read/27906097/a-10-year-old-child-presenting-with-syndromic-paucity-of-bile-ducts-alagille-syndrome-a-case-report
#1
Girish Kumar Pati, Ayaskanta Singh, Preetam Nath, Jimmy Narayan, Pradeep Kumar Padhi, Prasanta Kumar Parida, Kaumudee Pattnaik, Chittaranjan Panda, Shivaram Prasad Singh
BACKGROUND: Alagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests with five major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects, and peripheral pulmonary stenosis. Globally, only 500 cases have so far been reported, with only five cases reported in the Indian subcontinent. Rarely, Alagille syndrome also presents with skin manifestations and early-onset chronic liver disease, which was found in our case...
November 30, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27890105/use-of-postmortem-computed-tomography-angiography-to-detect-vascular-injuries-accompanying-skull-base-fracture
#2
Hikaru Kuninaka, Yoichiro Takahashi, Rie Sano, Keiko Takahashi, Rieko Kubo, Yoshihiko Kominato, Hiroyuki Takei, Susumu Kobayashi, Takehiro Shimada, Hiroyuki Tokue, Sachiko Awata, Satoshi Hirasawa
A 58-year-old woman who had presented for upper gastrointestinal barium examination accidently slipped from the movable bed, and her head became compressed between the end of the bed and the side wall. She suffered massive bleeding from her nose and ear followed by cardiac arrest, and subsequent attempts at cardiopulmonary resuscitation failed. A medicolegal autopsy was performed to reveal the cause of death, as part of the investigation of the accident. During the autopsy, postmortem cerebral CT angiography was carried out by injection of 5% gelatin-barium emulsion as a radiopaque contrast medium into the bilateral common carotid arteries, demonstrating transudation of the contrast medium into the right acoustic meatus and the sphenoidal sinus cavity...
November 2016: Legal Medicine
https://www.readbyqxmd.com/read/27878613/intermittent-low-dose-bevacizumab-in-hereditary-hemorrhagic-telangiectasia-a%C3%A2-case-report
#3
Florian Huemer, Martin Dejaco, Christoph Grabmer, Thomas Melchardt, Daniel Neureiter, Georg Mayer, Alexander Egle, Richard Greil, Lukas Weiss
BACKGROUND: Hereditary hemorrhagic telangiectasia is an inherited autosomal dominant disease presenting with recurrent bleeding episodes and iron deficiency anemia due to vascular malformations. Hereditary hemorrhagic telangiectasia is associated with an increased risk of stroke, gastrointestinal bleeding and pulmonary hypertension and life expectancy is significantly reduced. Excess vascular endothelial growth factor (VEGF) plays a key role in the pathophysiology of the disease. CASE PRESENTATION: Here we report about a male patient with hereditary hemorrhagic telangiectasia presenting with pulmonary and central nervous system involvement experiencing repetitive nosebleeds, necessitating frequent local cauterization and transfusion of more than 100 units of packed red blood cells...
November 23, 2016: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/27869377/physical-modalities-for-the-treatment-of-rosacea
#4
Maja A Hofmann, Percy Lehmann
Rosacea is characterised by a wide variety of vascular changes. Apart from telangiectasia and erythema, often so-called flushing occurs. These vascular abnormalities can be targeted with specific light and laser devices. In addition to KTP laser, pulsed dye laser (PDL) and Nd:YAG laser, also intense pulsed light devices (IPLs) are used. The described therapeutic effects include the reduction of vascular abnormalities and even improvement of papulopustular changes. While the KTP laser shows very good results in telangiectasia, the dye laser and IPL devices are used preferably in erythema...
December 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/27863666/nasal-powders-of-thalidomide-for-local-treatment-of-nose-bleeding-in-persons-affected-by-hereditary-hemorrhagic-telangiectasia
#5
G Colombo, F Bortolotti, V Chiapponi, F Buttini, F Sonvico, R Invernizzi, F Quaglia, C Danesino, F Pagella, P Russo, R Bettini, P Colombo, A Rossi
In this work nasal powder formulations of thalidomide were designed and studied to be used by persons affected by hereditary hemorrhagic telangiectasia as a complementary anti-epistaxis therapy, with the goal of sustaining the effect obtained with thalidomide oral treatment after its discontinuation for adverse effects. Three nasal powders were prepared using as carriers β-CD or its more hydrophilic derivatives such as hydropropyl-β-CD and sulphobutylether-β-CD and tested with respect to technological and biopharmaceutical features after emission with active and passive nasal powder devices...
November 30, 2016: International Journal of Pharmaceutics
https://www.readbyqxmd.com/read/27837213/-prosthetic-treatment-of-nasal-septal-perforations-results-with-custom-made-silicone-buttons
#6
K Zaoui, M H Schneider, O Neuner, P A Federspil
BACKGROUND: Perforations of the nasal septum can be very disturbing for patients due to increased crust formation, nose bleeds, obstruction of nasal breathing and whistling sounds during nasal breathing. The aim of this study was to evaluate how the symptom burden can be alleviated by custom-made silicone buttons derived from an impression mold. MATERIAL AND METHODS: A retrospective study was carried out to evaluate 45 patients with symptomatic septal perforations, who have been treated over a period of 8 years...
December 2016: HNO
https://www.readbyqxmd.com/read/27813078/bleeding-erythematous-papules-over-nose-in-a-middle-aged-man
#7
Neetu Bhari, Kanika Sahni, Sudheer Arava
No abstract text is available yet for this article.
November 4, 2016: International Journal of Dermatology
https://www.readbyqxmd.com/read/27813041/-one-year-treatment-of-nose-bleeding-in-the-ent-emergency-departments-of-east-thuringia
#8
K Weigel, G F Volk, A Müller, O Guntinas-Lichius
Objective: Aim of the study was to analyse the medical care situation of patients suffering from epistaxis in everyday clinical practice in ENT emergency departments. Material and Methods: In the year 2009, 690 patients with 862 occurrences of epistaxis sought help in the 2 East Thuringian ENT emergency departments in Jena and Gera (60% male, average age: 60 years). The patients' characteristics were evaluated retrospectively with a focus on comorbidity, long-term medication and treatment measures. Results: The incidence of epistaxis treatment in the ENT emergency departments was 121 28 per 100 000 habitants of East Thuringia...
November 3, 2016: Laryngo- Rhino- Otologie
https://www.readbyqxmd.com/read/27811840/notes-from-the-field-rift-valley-fever-response-kabale-district-uganda-march-2016
#9
Annabelle de St Maurice, Luke Nyakarahuka, Lawrence Purpura, Elizabeth Ervin, Alex Tumusiime, Stephen Balinandi, Jackson Kayondo, Sophia Mulei, Anne Marion Namutebi, Patrick Tusiime, Steven Wiersma, Stuart Nichol, Pierre Rollin, John Klena, Barbara Knust, Trevor Shoemaker
On March 9, 2016, a male butcher from Kabale District, Uganda, aged 45 years, reported to the Kabale Regional Referral Hospital with fever, fatigue, and headache associated with black tarry stools and bleeding from the nose. One day later, a student aged 16 years from a different sub-county in Kabale District developed similar symptoms and was admitted to the same hospital. The student also had a history of contact with livestock. Blood specimens collected from both patients were sent for testing for Marburg virus disease, Ebola virus disease, Rift Valley fever (RVF), and Crimean Congo Hemorrhagic fever at the Uganda Virus Research Institute, as part of the viral hemorrhagic fevers surveillance program...
November 4, 2016: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/27755410/the-rate-of-epistaxis-incidence-in-new-generation-anticoagulants-and-perioperative-approach-in-otorhinolaryngological-practices
#10
Ozan Gökdoğan, Ilker Akyildiz, Begum Yetis Sayin, Sercan Okutucu, Ali Cevat Tanalp, Necmi Arslan
Nose bleeding is a common situation seen in otorhinolaryngological practices. One of the greatest risk factors in nose bleeding is the use of anticoagulant medicine. With the medicine developed in recent years, the risk of nose bleeding due to the frequent use of anticoagulant and antiagregant is gradually increasing.The purpose of this study is to determine the effects of especially new-generation anticoagulants on nose bleeding. In addition, the use and complications of new-generation anticoagulants and antiagregants have been compiled in light of information obtained from the literature...
October 14, 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27714705/predictive-factors-of-bleeding-events-in-adults-undergoing-extracorporeal-membrane-oxygenation
#11
Cécile Aubron, Joris DePuydt, François Belon, Michael Bailey, Matthieu Schmidt, Jayne Sheldrake, Deirdre Murphy, Carlos Scheinkestel, D Jamie Cooper, Gilles Capellier, Vincent Pellegrino, David Pilcher, Zoe McQuilten
BACKGROUND: Bleeding is the most frequent complication associated with extracorporeal membrane oxygenation (ECMO) support in critically ill patients. Nonetheless, risk factors for bleeding have been poorly described especially those associated with coagulation anomalies and anticoagulant therapy during ECMO support. The aim of this study is to describe bleeding complications in critically ill patients undergoing ECMO and to identify risk factors for bleeding events. METHODS: We retrospectively analysed ICU charts of adults who received either veno-venous (VV) or veno-arterial (VA) ECMO support in two participating ICUs between 2010 and 2013...
December 2016: Annals of Intensive Care
https://www.readbyqxmd.com/read/27651480/primary-open-rhinoplasty
#12
Arash Momeni, Ronald P Gruber
Rhinoplasty is perhaps the most complex cosmetic surgery procedure performed today. It is characterized by an intricate interplay between form and function, with patient satisfaction being dependent not only on improvement of nasal appearance but also resolution of preexisting airway symptoms. The prerequisite for successful execution of this challenging procedure is a thorough understanding of nasal anatomy and physiology. Hence, a thorough preoperative evaluation is at least as important and the surgical skill in performing the operation...
October 2016: Aesthetic Surgery Journal
https://www.readbyqxmd.com/read/27631244/postural-orthostatic-tachycardia-syndrome-is-associated-with-platelet-storage-pool-deficiency
#13
William T Gunning, Beverly L Karabin, Thomas M Blomquist, Blair P Grubb
Mechanisms have been postulated to explain postural orthostatic tachycardia syndrome (POTS), however, the etiology of this often debilitating disorder remains unknown. We conducted a retrospective case-control study of 181 POTS patients who exhibited/reported bleeding symptoms for a specific platelet (PL) dysfunction disorder, delta granule storage pool deficiency (δ-SPD).Patients were included only if results of blood tests for δ-SPD were available. Electron microscopy was utilized to diagnose δ-SPD. An ELISA assay was used to determine serotonin (5HT) concentration in PLs and medical record review was employed to collect patients' clinical symptoms...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27624510/phosphodiesterase-5-inhibitor-use-an-under-reported-cause-of%C3%A2-profuse-nasal-bleeding
#14
Petros V Vlastarakos, Thomas P Nikolopoulos
BACKGROUND: Phosphodiesterase-5 (PDE-5) inhibitors enhance penile erection and have gained popularity not only for erectile dysfunction, but also in recreational settings. Nevertheless, adverse effects have been associated with their use, with nasal bleeding among them. PDE-5 inhibitor action is materialized through the inhibition of the cyclic guanosine monophosphate (cGMP) enzyme. cGMP is present at several sites of the human body in addition to the corpus cavernosum, leading to the adverse effects associated with its nonselective inhibition...
November 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27617522/pigmented-hidrocystoma-of-nasal-epithelium-phone-report-of-a-man-with-a-pigmented-hidrocystoma-of-his-nose-and-literature-review
#15
Katherine C Rappazzo, Philip R Cohen
BACKGROUND: Hidrocystomas are benign tumors of apocrine or eccrine epithelium. They most commonly occur on the head and neck, especially periorbitally. Albeit rare, these adnexal tumors may present as pigmented lesions. PURPOSE: To describe a patient with a pigmented eccrine hidrocystoma of his nose and to review the features of other individuals with pigmented hidrocystoma of the nasal epithelium. MATERIAL AND METHODS: PubMed was used to search the follow terms: hidrocystoma and pigmented...
2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/27607121/arterio-venous-malformations-of-the-nose-combined-approach-for-a-successful-strategy
#16
Daria Almesberger, Francesco Manna, Gianni Franco Guarneri, Andrea Marchesi, Pier Camillo Parodi
BACKGROUND AND PURPOSE: The purpose of the present study is to evaluate the vascular malformations of the head and neck. They are uncommon lesions, but some areas have a significant potential for fatality, due to their massive bleeding. The vascular anatomy of the nose carries a high recurrence rate, due to the unique characteristic of the nose, especially if deep vascular connection is present. METHODS: The present article describes 2 patients of nasal dorsum arteriovenous malformations, both using a combined procedure: preoperative selective embolization, en-bloc tumor resection, and reconstruction with a forehead flap...
September 2016: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/27599329/effect-of-topical-intranasal-therapy-on-epistaxis-frequency-in-patients-with-hereditary-hemorrhagic-telangiectasia-a-randomized-clinical-trial
#17
RANDOMIZED CONTROLLED TRIAL
Kevin J Whitehead, Nathan B Sautter, Justin P McWilliams, Murali M Chakinala, Christian A Merlo, Maribeth H Johnson, Melissa James, Eric M Everett, Marianne S Clancy, Marie E Faughnan, S Paul Oh, Scott E Olitsky, Reed E Pyeritz, James R Gossage
IMPORTANCE: Epistaxis is a major factor negatively affecting quality of life in patients with hereditary hemorrhagic telangiectasia (HHT; also known as Osler-Weber-Rendu disease). Optimal treatment for HHT-related epistaxis is uncertain. OBJECTIVE: To determine whether topical therapy with any of 3 drugs with differing mechanisms of action is effective in reducing HHT-related epistaxis. DESIGN, SETTING, AND PARTICIPANTS: The North American Study of Epistaxis in HHT was a double-blind, placebo-controlled randomized clinical trial performed at 6 HHT centers of excellence...
September 6, 2016: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/27591836/a-systematic-review-of-anti-thrombotic-therapy-in-epistaxis
#18
K M Musgrave, J Powell
There is limited guidance available to clinicians regarding the management of antithrombotic therapy during epistaxis, whilst there has been an increase in the use of anticoagulation and antiplatelet therapy. In addition, the introduction of direct oral anticoagulants (DOACs), such as dabigatran and rivaroxaban, over the last decade has significantly increased the complexity of managing the anticoagulated epistaxis patient. We undertook a systemic literature review investigating potential management strategies for each class of anti-thrombotic therapy during epistaxis...
September 4, 2016: Rhinology
https://www.readbyqxmd.com/read/27500577/-the-blockade-of-sphenopalatineganglionthrough-the-palatal-approachin-the-present-day-rhinological-practice
#19
V G Borodulin, S V Filimonov
This article deals with the application of the sphenopalatine ganglion blockade in the present-day rhinological practice. The blockade is known to arrest the propagation of pain impulses from the nose and break the rhinocardiac reflex arc. Moreover, it is involved in bleeding control during nasal surgery. The method for the blockade via the palatal route using the modern equipment and imaging techniques is described. The objective of the present study was to evaluate the effectiveness and safety of the blockade of sphenopalatine ganglionthrough the palatal approach in the patients who had undergone septoplasty under general and local anesthesia...
2016: Vestnik Otorinolaringologii
https://www.readbyqxmd.com/read/27490981/the-use-of-floseal-haemostatic-sealant-in-the-management-of-epistaxis-a-prospective-clinical-study-and-literature-review
#20
O C Wakelam, P A Dimitriadis, J Stephens
INTRODUCTION It is standard practice in the UK that if conservative measures or chemical cautery fail to control epistaxis, patients receive nasal packing which is often uncomfortable, requires admission and has well documented associated morbidity. Our study aims to evaluate the use of FloSeal haemostatic sealant in managing patients with epistaxis. MATERIALS AND METHODS Patients were identified from those referred with active epistaxis. A successful outcome was defined as complete haemostasis with FloSeal alone, with no further significant bleeding requiring admission or further interventions in the subsequent 7 days...
August 4, 2016: Annals of the Royal College of Surgeons of England
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