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cogan's Syndrome

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https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#1
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27843668/rituximab-not-effective-for-hearing-loss-in-cogan-s-syndrome
#2
Daniel R Bunker, Leslie Dubin Kerr
Importance. Rituximab was not effective in ameliorating the hearing loss in a patient with atypical Cogan's syndrome. Observations. We report the case of a patient who developed acute bilateral uveitis and sensorineural hearing loss. A diagnosis of atypical Cogan's syndrome was made. The patient's hearing loss did not improve despite high dose steroids and azathioprine. Rituximab was administered given a recent report of its efficacy in a patient with refractory disease; however, our patient's hearing loss did not improve...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27765438/power-injection-through-ultrasound-guided-intravenous-lines-safety-and-efficacy-under-an-institutional-protocol
#3
Michael D Witting, Siamak Moayedi, Kathy Dunning, Lisa S Babin, Brad M Cogan
BACKGROUND: After an index case of contrast-associated compartment syndrome, an urban hospital instituted a protocol limiting high-speed injection to intravenous (IV) lines started proximal to the forearm and testing those lines before contrast injection. OBJECTIVE: In this article, we estimate the safety and efficacy of high-speed injection using this protocol in patients with IV lines inserted under ultrasound guidance. METHODS: In an ambispective study, we enrolled prospective cohorts of ED patients requiring high-speed radiographic contrast media injection (≥3...
October 17, 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27585567/cystoid-macular-edema-associated-with-iridocorneal-endothelial-syndrome-a-case-report
#4
Keita Suzuki, Tadashi Mizuguchi, Yui Seno, Atsuhiro Tanikawa, Masayuki Horiguchi
BACKGROUND: Iridocorneal endothelial (ICE) syndrome occurs mainly in young and middle-aged women and typically presents as a unilateral disease characterized by abnormalities of the iris and corneal endothelium. While the ICE syndrome is known to be associated with glaucoma and bullous keratopathy, to our knowledge, only two cases of ICE syndrome complicated with cystoid macular edema (CME) have been reported to date. In this paper, we report a case of ICE syndrome complicated with CME treated at our institution...
2016: BMC Ophthalmology
https://www.readbyqxmd.com/read/27473762/nosological-delineation-of-congenital-ocular-motor-apraxia-type-cogan-an-observational-study
#5
Sarah Wente, Simone Schröder, Johannes Buckard, Hans-Martin Büttel, Florian von Deimling, Wilfried Diener, Martin Häussler, Susanne Hübschle, Silvia Kinder, Gerhard Kurlemann, Christoph Kretzschmar, Michael Lingen, Wiebke Maroske, Dirk Mundt, Iciar Sánchez-Albisua, Jürgen Seeger, Sandra P Toelle, Eugen Boltshauser, Knut Brockmann
BACKGROUND: The nosological assignment of congenital ocular motor apraxia type Cogan (COMA) is still controversial. While regarded as a distinct entity by some authorities including the Online Mendelian Inheritance in Man catalog of genetic disorders, others consider COMA merely a clinical symptom. METHODS: We performed a retrospective multicenter data collection study with re-evaluation of clinical and neuroimaging data of 21 previously unreported patients (8 female, 13 male, ages ranging from 2 to 24 years) diagnosed as having COMA...
2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27330475/cogan-syndrome-with-severe-medium-and-large-vessel-vasculitis
#6
Duriye D Sevgi, Lucia Sobrin, George N Papaliodis
Cogan syndrome is a rare disease characterized by coexisting audiovestibular and ocular symptoms. Almost half of patients develop systemic manifestations. We report the case of a 38-year-old woman who presented with severe medium and large vessel vasculitis as a systemic manifestation of Cogan syndrome.
2016: Digital Journal of Ophthalmology: DJO
https://www.readbyqxmd.com/read/27319272/-peripheral-artery-disease-in-patients-younger-than-50%C3%A2-years-old-which-etiology
#7
S Cotard, A Nouni, V Jaquinandi, G Gladu, A Kaladji, G Mahé
Peripheral arterial disease (PAD) encompasses disease of all arteries of the body except the coronary arteries. The main etiology whatever the patient's age is atherosclerosis. Different etiologies can induce PAD especially when patients are younger than 50 years old and have no cardiovascular risk factors (smoking, hypertension, diabetes…). PAD that appears before 50 years old can be named juvenile PAD (JPAD) although there is no consensus about the definition. The aim of this work is to present the different etiologies of JPAD according to their hereditary, acquired or mixed origins...
September 2016: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/27049822/the-role-the-frailty-syndrome-can-play-in-advocacy-and-resource-allocation-for-our-ageing-population-findings-in-a-dublin-day-hospital
#8
O Ntlholang, R E Kelly, R Romero-Ortuno, S Cosgrave, D Kelly, M Crowe, O Collins, J J Barry, L Cogan, G Hughes, D O'Shea
Frail individuals are at higher risk of adverse outcomes, and need identification and priority access to Comprehensive Geriatric Assessment (CGA). We prospectively collected data on new referrals to our day hospital. Levels of frailty were measured with the SHARE Frailty Instrument for Primary Care (SHARE-FI). Of 257 patients assessed (90 men, 167 women), 110 (43%) were non-frail, 66 (26%) pre-frail and 81 (32%) frail. Mean age was 82 years for the non-frail, 83 for the pre-frail and 84 for the frail. Forty-one percent of the frail reported two or more falls in the preceding year, compared to 38% of the pre-frail and 21% of the non-frail (P for trend = 0...
2014: Journal of Frailty & Aging
https://www.readbyqxmd.com/read/26978875/ice-syndrome-case-report
#9
Valeria Coviltir, Dinu Valentin
Iridocorneal endothelial (ICE) syndrome encompasses a group of rare ocular pathologies with unilateral involvement, frequently affecting young women. The disease complex includes essential iris atrophy, Chandler's syndrome, and Cogan-Reese syndrome. In the following article, we present a case of Iridocorneal endothelial syndrome in which a late diagnosis was made and who underwent surgery for advanced glaucoma.
April 2015: Romanian Journal of Ophthalmology
https://www.readbyqxmd.com/read/26928685/-not-available
#10
Erika Hedin, Helge Lyckberg, Per-Inge Carlsson
Cogans syndrome is a rare systemic inflammatory disease characterized by a combination of audiovestibular and ocular symptoms. In some cases, systemic complications occur with vascular inflammation. Aortitis and large vessel vasculitis are the most common forms, but medium-sized and small vessel involvement has also been described. The autoimmune reaction can lead to blindness, deafness and in worst case death, if these patients remain untreated or if treatment is delayed. There is no specific blood test or imaging method available and the diagnosis is clinical...
2016: Läkartidningen
https://www.readbyqxmd.com/read/26693453/otolaryngologic-manifestations-of-various-rheumatic-diseases-awareness-and-practice-among-otolaryngologists
#11
Chanchal Gera, Navneet Kumar
Wegener's granulomatosis, Sarcoidosis, Sjögren's Syndrome, Behçet disease, Systemic lupus erythematosus, relapsing polychondritis, Churg-Strauss syndrome and Cogan syndromes are rheumatic diseases commonly encountered by otolaryngologists. The present study was designed to assess the understanding and practice about various ENT problems of rheumatic diseases amongst otolaryngologists. A group of 29 otolaryngologists was asked to fill questionnaire which was based on knowledge and practice of otolaryngologists about various otolaryngologic manifestations of rheumatic diseases...
December 2015: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/26451377/diagnosis-and-management-of-iridocorneal-endothelial-syndrome
#12
REVIEW
Marta Sacchetti, Flavio Mantelli, Marco Marenco, Ilaria Macchi, Oriella Ambrosio, Paolo Rama
The iridocorneal endothelial (ICE) syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and proliferative abnormalities of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. The main subtypes of this syndrome are the progressive iris atrophy, the Cogan-Reese syndrome, and the Chandler syndrome. ICE syndrome is usually diagnosed in women in the adult age...
2015: BioMed Research International
https://www.readbyqxmd.com/read/26434452/surgical-treatment-in-a-case-of-cogan-s-syndrome-complicated-with-proximal-aortic-vasculitis
#13
Mehmet Kaya, Korhan Erkanlı, Furkan Kılınç, Mehmet Sar, İhsan Bakır
Cogan's syndrome is a rare idiopathic inflammatory disease with involvement of the eye and inner ear. This syndrome can be associated with aortic root vasculitis. We report the case of a young man with a history of uveitis and bilateral hearing loss who presented with proximal aortic vasculitis and dilatation after aortic valve replacement. Cogan's syndrome was diagnosed on the basis of large artery vasculitis with typical vestibuloauditory and ocular involvement. The patient underwent successful a Bentall operation after aortic valve replacement...
October 2015: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/26426408/serous-neurosensory-retinal-detachment-associated-with-atypical-cogan-syndrome-a-case-report
#14
James R Singer, Rahul K Reddy
PURPOSE: To present a case of a 71-year-old woman with possible focal choroiditis and secondary serous neurosensory retinal detachment associated with atypical Cogan syndrome. METHODS: Retrospective case report. RESULTS: On initial evaluation, the patient had decreased vision with photopsias and distortion in the affected eye. Her history revealed 3 years of preexisting neurosensory hearing loss and corneal scarring in the affected eye. Examination findings were significant for unilateral interstitial keratitis and focal macular neurosensory retinal detachment...
2015: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/26361386/ultrasound-guided-trigger-point-injection-for-serratus-anterior-muscle-pain-syndrome-description-of-technique-and-case-series
#15
Grisell Vargas-Schaffer, Michal Nowakowsky, Marzieh Eghtesadi, Jennifer Cogan
Chronic chest pain is a challenge, and serratus anterior muscle pain syndrome (SAMPS) is often overlooked. We have developed an ultrasound-guided technique for infiltrating local anesthetics and steroids in patients with SAMPS. In 8 patients, the duration of chronic pain was approximately 19 months. Three months after treatment, all patients had experienced a significant reduction in pain. Infiltration for SAMPS confirms the diagnosis and provides adequate pain relief.
September 15, 2015: A & A Case Reports
https://www.readbyqxmd.com/read/26350991/lamotrigine-induced-tubulointerstitial-nephritis-and-uveitis-atypical-cogan-syndrome
#16
Anton M Kolomeyer, Shyam Kodati
PURPOSE: To report a case of lamotrigine-induced tubulointerstitial nephritis and uveitis (TINU)-atypical Cogan syndrome. METHODS: Case report. RESULTS: A 16-year-old boy with traumatic brain injury and seizures presented to the emergency department with facial swelling, rash, and back pain several days after increasing lamotrigine dose secondary to a breakthrough seizure. Creatinine, urine β2 microglobulin, and eosinophils were elevated...
January 2016: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/26257951/development-of-a-low-grade-lymphoma-in-the-mastoid-bone-in-a-patient-with-atypical-cogan-s-syndrome-a-case-report
#17
Chris Kalogeropoulos, Dimitrios Karachalios, George Pentheroudakis, Aikaterini Tsikou-Papafrangou, Lydia Abou-Asabeh, Maria Argyropoulou, Alexandros Drosos, Nicholas Pavlidis
Cogan's syndrome is a rare disorder characterized by ocular and audiovestibular manifestations in its typical form and caries a wide variety of atypical manifestations. It is considered as an autoimmune disease. We present the first case in the literature of a 67 year old woman with the development of low grade non-Hodgkin lymphoma (NHL) in the mastoid bone in a pre-existing history of atypical Cogan's syndrome. The anatomical development of NHL was to a "target" organ of Cogan's syndrome, which is the inner ear...
May 2015: Journal of Advanced Research
https://www.readbyqxmd.com/read/26195577/histopathology-of-the-inner-ear-in-a-case-with-recent-onset-of-cogan-s-syndrome-evidence-for-vasculitis
#18
David H Jung, Joseph B Nadol, Rebecca D Folkerth, Joseph F Merola
The association of sensorineural hearing loss and vertigo with inflammatory eye disease, usually interstitial keratitis, has been called Cogan's syndrome. The pathogenesis of Cogan's syndrome is unknown, but it has been assumed to be an immune mediated disorder with vasculitis. The histopathology of the inner ear in Cogan's syndrome has been described in 6 case reports. Although common pathologic findings in these reports include degeneration of the auditory and vestibular neuroepithelium, endolymphatic hydrops, fibrosis, and new bone formation, direct pathologic evidence of a vasculitis has not been published...
January 2016: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/26188351/cogan-s-syndrome-in-patients-with-inflammatory-bowel-disease-a-case-series
#19
Stephan R Vavricka, Thomas Greuter, Michael Scharl, Gerassimos Mantzaris, Ariella B Shitrit, Rafal Filip, Konstantinos Karmiris, Christoph K Thoeringer, Hubert Boldys, Anne V Wewer, Henit Yanai, Cristina Flores, Carsten Schmidt, Revital Kariv, Gerhard Rogler, Jean-François Rahier
BACKGROUND: Cogan's syndrome (CSy) is a very rare autoimmune disorder, mainly affecting the inner ear and the eye, and is associated with inflammatory bowel disease (IBD). METHODS: This was a European Crohn's and Colitis Organisation (ECCO) retrospective observational study, performed as part of the CONFER project. A call to all ECCO members was made to report concomitant CSy and inflammatory bowel disease (IBD) cases. Clinical data were recorded in a standardized questionnaire...
October 2015: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/26181159/-description-of-the-case-of-cogan-syndrome
#20
Elżbieta Semik-Grabarczyk, Antoni Hrycek
Cogan syndrome is a persistent disease with the autoimmunologic background connected with vasculitis. It can be diagnosed by symptoms such as interstitial keratitis, optic nerve dysfunction and nervus acusticus dysfunction with subsequent hearing impairment or deafness. In its course, such systemic symptoms frequently occur: exhaustion, weight loss and joint pain. Due to the rare occurrence of Cogan syndrome and vast obstacles in diagnosing it, the case of34 years old patient with Cogan syndrome is discussed...
2015: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
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