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cogan syndrome

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https://www.readbyqxmd.com/read/29703835/fever-of-unknown-origin-bilateral-sensorineural-hearing-loss-with-canal-paresis-and-uveitis-with-iridocyclitis-and-episcleritis-a-case-of-cogan-s-syndrome
#1
Takashi Watari, Yasuharu Tokuda
No abstract text is available yet for this article.
April 27, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29557550/brainstem-dysgenesis-beyond-moebius-syndrome
#2
REVIEW
F Munell, M A Tormos, M Roig-Quilis
Brainstem dysgenesis designates all those patients with congenital dysfunction of cranial nerves and muscle tone due to prenatal lesions or malformations of the brainstem. This generic term has the advantage over the eponyms Moebius 'expanded' or 'unrestricted', Robin, Cogan or Carey-Fineman-Ziter syndromes in that it has a less restrictive view and provides a frame work that enables a systematic approach to diagnosis and research of most developmental disorders involving the brainstem. The review of the literature and our experience shows that infants with a predominant rombencephalic involvement are due to brainstem prenatal disruptive vascular accidents, while cases with midbrain and cerebellar involvement and widespread malformative syndromes have most likely an underlying genetic cause...
April 1, 2018: Revista de Neurologia
https://www.readbyqxmd.com/read/29550833/a-case-of-lown-ganong-levine-syndrome-due-to-an-accessory-pathway-of-james-fibers-or-enhanced-atrioventricular-nodal-conduction-eavnc
#3
Juanita Hunter, Emmanouil Tsounias, John Cogan, Ming-Lon Young
BACKGROUND Lown-Ganong-Levine syndrome, includes a short PR interval, normal QRS complex, and paroxysmal tachycardia. The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular (AV) node (James fiber), or between the atria and the His bundle (Brechenmacher fiber). Similar features are seen in enhanced atrioventricular nodal conduction (EAVNC), with the underlying pathophysiology due to a fast pathway to the AV node, and with the diagnosis requiring specific electrophysiologic criteria...
March 18, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29490187/interstitial-keratitis-in-cogan-s-syndrome
#4
Sandra Rodrigues-Barros, Sonia Parreira
No abstract text is available yet for this article.
March 1, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29489984/cogan-s-syndrome-a-rare-aortitis-difficult-to-diagnose-but-with-therapeutic-potential
#5
Raiza Colodetti, Guilherme Spina, Tatiana Leal, Mucio Oliveira, Alexandre Soeiro
The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control.
December 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/29341437/phenotypic-heterogeneity-of-zmpste24-deficiency
#6
Thomas A Cassini, Amy K Robertson, Anna G Bican, Joy D Cogan, Vickie L Hannig, John H Newman, Rizwan Hamid, John A Phillips
A 4-year-old girl was referred to the Undiagnosed Diseases Network with a history of short stature, thin and translucent skin, macrocephaly, small hands, and camptodactyly. She had been diagnosed with possible Hallerman-Streiff syndrome. Her evaluation showed that she was mosaic for uniparental isodisomy of chromosome 1, which harbored a pathogenic c.1077dupT variant in ZMPSTE24 which predicts p.(Leu362fsX18). ZMPSTE24 is a zinc metalloproteinase that is involved in processing farnesylated proteins and pathogenic ZMPSTE24 variants cause accumulation of abnormal farnesylated forms of prelamin A...
January 17, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29331589/the-iridocorneal-endothelial-syndrome
#7
REVIEW
Luis Silva, Ahmad Najafi, Yanin Suwan, Chaiwat Teekhasaenee, Robert Ritch
The iridocorneal endothelial syndrome (ICE) represents a unique group of ocular pathologies (Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome) characterized by the proliferation of corneal endothelial cells that migrate toward the iridocorneal angle and iris surface causing, to a degree varying according to the subtype, corneal edema and decompensation, and secondary glaucoma, whether by obstructing the angle or producing peripheral anterior synechiae by contraction of the basement membrane of the migrating cells over the surface of the iris...
January 10, 2018: Survey of Ophthalmology
https://www.readbyqxmd.com/read/29317827/optimal-management-of-cogan-s-syndrome-a-multidisciplinary-approach
#8
REVIEW
Vittorio D'Aguanno, Massimo Ralli, Marco de Vincentiis, Antonio Greco
Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Ménière-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism...
2018: Journal of Multidisciplinary Healthcare
https://www.readbyqxmd.com/read/29065093/cochlear-implantation-in-cochlear-ossification-retrospective-review-of-etiologies-surgical-considerations-and-auditory-outcomes
#9
Ashish Vashishth, Andrea Fulcheri, Sampath Chandra Prasad, Margherita Bassi, Gianluca Rossi, Antonio Caruso, Mario Sanna
OBJECTIVES: 1) To review the surgical and auditory outcomes and complications of cochlear implantation in cases with cochlear ossification. 2) To evaluate association between the extent and etiology of ossification to outcomes. STUDY DESIGN: Retrospective study. SETTING: Otology and skull base surgery center. SUBJECTS AND METHODS: Charts of 40 patients (42 ears) with cochlear ossification undergoing cochlear implantation were reviewed...
January 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29037902/cogan-syndrome-characteristics-outcome-and-treatment-in-a-french-nationwide-retrospective-study-and-literature-review
#10
REVIEW
Charlotte Durtette, Eric Hachulla, Matthieu Resche-Rigon, Thomas Papo, Thierry Zénone, Bertrand Lioger, Christophe Deligny, Marc Lambert, Cédric Landron, Jacques Pouchot, Jean Emmanuel Kahn, Christian Lavigne, Benoit De Wazieres, Robin Dhote, Guillaume Gondran, Edouard Pertuiset, Thomas Quemeneur, Mohamed Hamidou, Pascal Sève, Thomas Le Gallou, Anne Grasland, Pierre-Yves Hatron, Olivier Fain, Arsène Mekinian
BACKGROUND: Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments. PATIENTS AND METHODS: We conducted a French nationwide retrospective study of patients with Cogan syndrome (n=40) and a literature review of cases (n=22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists. RESULTS: We included 62 patients (31 females) (median age 37years [range 2-76]...
December 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28993974/unique-variations-and-characteristics-of-iridocorneal-endothelial-syndrome-in-china-a-case-series-of-58-patients
#11
Bo Feng, Xin Tang, Hong Chen, Xia Sun, Ningli Wang
PURPOSE: Iridocorneal endothelial (ICE) syndrome is a rare condition, and unique characteristics in Chinese patients can make diagnosis difficult. Our purpose was to describe the clinical characteristics and variations of ICE syndrome in 58 consecutive Chinese patients. METHODS: The clinical data of consecutive patients with ICE syndrome who were seen between 2008 and 2011 at the glaucoma clinic of our ophthalmology department were retrospectively reviewed. The diagnostic criteria for ICE syndrome were a "hammered-silver" appearance of the corneal endothelium and specular microscopy showing ICE cells characterized by the absence of a hexagonal appearance, dark areas within the cells, and a light-dark reversal pattern...
October 10, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28583785/late-onset-cogan-s-syndrome-associated-with-large-vessel-vasculitis
#12
Iván Cabezas-Rodríguez, Anahy Brandy-García, Carlos Rodríguez-Balsera, Paloma Rozas-Reyes, Belen Fernández-Llana, Luis Arboleya-Rodríguez
Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome...
June 2, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28471585/successful-pregnancy-in-a-patient-with-atypical-cogan-s-syndrome
#13
Francesca Riboni, Stefano Cosma, Pino Gino Perini, Chiara Benedetto
No abstract text is available yet for this article.
August 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28458810/autoimmune-ear-disease-clinical-and-diagnostic-relevance-in-cogan-s-sydrome
#14
Luigi Maiolino, Salvatore Cocuzza, Angelo Conti, Luisa Licciardello, Agostino Serra, Salvatore Gallina
The autoimmune inner ear disease is a clinical syndrome with uncertain pathogenesis that is often associated to rapidly progressive hearing loss that, especially at the early stages of disease, may be at monoaural localization, although more often it is at binaural localization. It usually occurs as a sudden deafness, or a rapidly progressive sensorineural hearing loss. In this study a particular form of autoimmune inner ear disease is described, Cogan's syndrome. Cogan's syndrome is a chronic inflammatory disorder that most commonly affects young adults...
February 1, 2017: Audiology Research
https://www.readbyqxmd.com/read/28345423/systemic-lupus-erythematosus-and-hearing-disorders-literature-review-and-meta-analysis-of-clinical-and-temporal-bone-findings
#15
A Di Stadio, Massimo Ralli
Objective This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Methods PubMed and Google Scholar searches were performed using the following keywords: "auto-immune disease," "systemic lupus erythematosus (SLE)," "hearing loss," "temporal bone study," "vertigo," "dizziness," "tinnitus," "ear symptoms," "treatment," "diagnosis," "symptoms," "etiopathogenesis," "Wegener granulomatosis," "Sjogren," "polyarteritis nodosa," "Cogan syndrome," and "granulomatosis...
October 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28300746/a-unique-case-of-keratoconus-with-cogan-reese-syndrome-and-secondary-glaucoma
#16
Lipi Chakrabarty
Keratoconus (KC), though one of the most common corneal degeneration, still continues to be a mystique regarding its pathogenesis, diagnosis, associations, and management; with newer discoveries and evolutions being reported. We report, what we believe to be another new association of KC- Cogan Reese syndrome with secondary glaucoma. A 32-year-old male, diagnosed as bilateral KC, presented for examination. Unilateral Cogan-Reese syndrome and associated secondary glaucoma was identified. These associations had been missed by previous ophthalmologists...
January 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28282413/glaucoma-associated-with-iridocorneal-endothelial-syndrome-in-203-indian-subjects
#17
Premanand Chandran, Harsha L Rao, Anil K Mandal, Nikhil S Choudhari, Chandra S Garudadri, Sirisha Senthil
PURPOSE: To report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with Iridocorneal endothelial (ICE) syndrome. METHODS: Retrospective review of 203 consecutive subjects with ICE syndrome at a tertiary eye care centre between January 1988 and June 2013. RESULTS: ICE syndrome was present in 223 eyes of 203 subjects, 124 (61%) were female and 79 (39%) were male. The median age at presentation was 43 years (1st (Q1) and 3rd (Q3) quartile; 34, 51 years)...
2017: PloS One
https://www.readbyqxmd.com/read/28160939/atypical-ulcerated-lesions-in-a-patient-with-cogan-syndrome
#18
G Solano-López, A Montes-Torres, E Tomero, J Fraga, D De Argila, E Daudén
No abstract text is available yet for this article.
May 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#19
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...
January 1, 2016: Lupus
https://www.readbyqxmd.com/read/27843668/rituximab-not-effective-for-hearing-loss-in-cogan-s-syndrome
#20
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
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