Tıp Fakültesi / Faculty of Medicine

Permanent URI for this collectionhttps://hdl.handle.net/11727/1403

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    Graft-Versus-Host Disease and Relapse/Rejection-Free Survival After Allogeneic Transplantation for Idiopathic Severe Aplastic Anemia: A Comprehensive Analysis from the SAAWP of the EBMT
    (2023) Devillier, Raynier; Eikema, Dirk-Jan; Dufour, Carlo; Aljurf, Mahmoud; Wu, Depei; Maschan, Alexei; Kulagin, Alexander; Halkes, Constantijn J. M.; Collin, Matthew; Snowden, John; Renard, Cecile; Ganser, Arnold; Sykora, Karl-Walter; Gibson, Brenda E.; Maertens, Johan; Itala-Remes, Maija; Corti, Paola; Cornelissen, Jan; Bornhaeuser, Martin; Colorado Araujo, Mercedes; Ozdogu, Hakan; Risitano, Antonio; Socie, Gerard; de latour, Regis Peffault; 36951165
    Survival after allogeneic hematopoietic stem cell transplantation (allo-HSCT) for severe idiopathic aplastic anemia (SAA) has improved in recent years, approaching 75% at 5 years. However, an SAA-adapted composite endpoint, graft-versus-host disease (GvHD) and relapse/rejection-free survival (GRFS), may more accurately assess patient outcomes beyond survival. We analyzed GRFS to identify risk factors and specific causes of GRFS failure. Our retrospective analysis from the Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation included 479 patients with idiopathic SAA who underwent allo-HSCT in two conventional situations: i) upfront allo-HSCT from a matched related donor (MRD) (upfront cohort), and ii) allo-HSCT for relapsed or refractory SAA (rel/ref cohort). Relevant events for GRFS calculation included graft failure, grade 3-4 acute GvHD, extensive chronic GvHD, and death. In the upfront cohort (n=209), 5-year GRFS was 77%. Late allo-HSCT (i.e., >6 months after SAA diagnosis) was the main poor prognostic factor, specifically increasing the risk of death as the cause of GRFS failure (hazard ratio [HR]=4.08; 95% confidence interval [CI]: 1.41-11.83; P=0.010). In the rel/ref cohort (n=270), 5-year GRFS was 61%. Age was the main factor significantly increasing the risk of death (HR=1.04; 95% CI: 1.02-1.06; P<0.001), acute GvHD (HR=1.03; 95% CI: 1.00-1.07; P=0.041), and chronic GvHD (HR=1.04; 95% CI: 1.01-1.08; P=0.032) as the cause of GRFS failure. GRFS after upfront MRD allo-HSCT was very good, notably with early allo-HSCT, confirming that younger patients with an MRD should be transplanted immediately. GRFS was worse in cases of salvage allo-HSCT, most notably in older patients, questioning the utility of allo-HSCT earlier in the disease course.
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    Familial Mediterranean fever associated frosted branch angiitis, retinal vasculitis and vascular occlusion
    (2021) Mansour, Hana A.; Ozdal, Pinar C.; Kadayifcilar, Sibel; Tugal-Tutkun, Ilknur; Eser-Ozturk, Hilal; Yalcindag, F. Nilufer; Petrushkin, Harry; Chan, Errol W.; Belfaiza, Soukaina; Karadag, Remzi; Gungor, Sirel Gur; Parodi, Maurizio Battaglia; Mansour, Ahmad M.; 0000-0001-6178-8362; 34725467; AAD-5967-2021
    Objectives To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF). Methods Retrospective collaborative case series using invitation by email to uveitis specialists around the Mediterranean basin. This series was combined with a literature review. Exclusion criteria included infectious diseases, Behcet's disease or other autoimmune diseases. Results A total of 16 patients (21 eyes) had FMF and retinal vasculitis (FBA 11 patients, mild retinal vasculitis 5 patients). The mean age at onset of vasculitis was 29.5 +/- 13.4 (range 9-62) with a female to male ratio of 9 to 7. In 19 eyes treated with various forms of corticosteroid and/or immunosuppression, the mean initial spectacle-corrected visual acuity improved from 6/194 to 6/10.5 at the last mean follow-up of 29.0 +/- 34.9 months (p < 0.001). The most common FEVR mutations were M680I and M694V. In addition, retinal vascular occlusions included one case of central retinal artery occlusion and one case of branch retinal artery occlusion. Conclusion FBA and milder forms of retinal vasculitis are associated with FMF. Therapy involves an increase in colchicine dosage in early cases, a long period of oral corticosteroid, intravitreal dexamethasone implant or periocular corticosteroid in select cases, and combination therapy with systemic immunosuppression in severe cases. FMF needs to be included in the differential diagnosis of retinal vasculitis.
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    Perioperative considerations and anesthesia management in patients with obstructive sleep apnea undergoing ophthalmic surgery
    (2019) Cok, Oya Y.; Seet, Edwin; Kumar, Chandra M.; Joshi, Girish P.; 31174989
    Obstructive sleep apnea (OSA) is a disorder characterized by breathing cessation caused by obstruction of the upper airway during sleep. It is associated with multiorgan comorbidities such as obesity, hypertension, heart failure, arrhythmias, diabetes mellitus, and stroke. Patients with OSA have an increased prevalence of ophthalmic disorders such as cataract, glaucoma, central serous retinopathy (detachment of retina, macular hole), eyelid laxity, keratoconus, and nonarteritic anterior ischemic optic neuropathy; and some might require surgery. Given that OSA is associated with a high incidence of perioperative complications and more than 80% of surgical patients with OSA are unrecognized, all surgical patients should be screened for OSA (eg, STOP-Bang questionnaire) with comorbidities identified. Patients suspected or diagnosed with OSA scheduled for ophthalmic surgery should have their comorbid conditions optimized. This article includes a review of the literature and highlights best perioperative anesthesia practices in the management of ophthalmic surgical patients with OSA. (C) 2019 ASCRS and ESCRS