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Öğe Accuracy and clinical role of digital templating for total knee arthroplasty performed on haemophilic knees(Wiley, 2024) Vahabi, Arman; Er, Erdem; Bicer, Elcil Kaya; Sahin, Fahri; Kavakli, Kaan; Aydogdu, SemihIntroductionIn total knee arthroplasty (TKA), choosing the correct implant size is important. There is lack of data on accuracy of templating on haemophilic knees. Our aim was to test the accuracy of 2D digital templating for TKA on haemophilic arthropathy (HA) of knee.Materials and MethodsTKAs performed on HA between January 2011 and January 2022 were screened. Osteoarthritis (OA) group was created as control group by a one-to-one matching regarding type of implant used. Intra- and interobserver correlations were measured in HA, then correlation between templated and implanted sizes was investigated in four assessments (femur AP, femur lateral, tibia AP, tibia lateral), then compared with OA group. Fifty-eight knees in each group included.ResultsRegarding intraobserver correlation in HA, there was excellent correlation for femur AP [.93 (.73-.98)], femur lateral [.98 (.91-.99)], and tibia AP (1.0) templating. Regarding interobserver correlation in HA, excellent correlation was observed for femur lateral [.93 (.74-.98)] and tibia AP templating [.90 (.65-.97)]. Regarding correlation of templated and applied sizes in HA; tibia AP, tibia lateral and femur lateral templating showed good correlation [.81 (.70-.89), .86 (.77-.91), .79 (.67-.87) while femur AP templating showed moderate correlation [.67 (.50-.79)]. Comparing HA and OA, there was no difference in correlation levels regarding femur AP, femur lateral, tibia AP and tibia lateral templating (p = .056, p = .781, p = .761, p = .083, respectively).ConclusionAlthough 2D digital templating shows comparable correlation in HA and OA, clinical applicability of templating on HA appears to be limited in its current state.Öğe Anaphylaxis due to rFVIIa in an infant suffering from severe congenital FVII deficiency(Wiley-Blackwell, 2016) Sahin, Akkiz; Balkan, Can; Akinci, Burcu; Karapinar, Deniz; Aydinok, Yesim; Kavakli, KaanÖğe Are all licensed haemostatic agents for haemophilia therapy accessible to patients?(Wiley, 2022) Berger, Karin; Batorova, Angelika; Bohn, Rhonda; di Minno, Matteo; Mannucci, Pier Mannuccio; Kavakli, Kaan; Schramm, Wolfgang[No Abstract Available]Öğe Arterial ischemic stroke in childhood: Risk factors and outcome in old versus new era(Sage Publications Inc, 2007) Goekben, Sarenur; Tosun, Ayse; Bayram, Nuri; Serdaroglu, Gul; Polat, Muzaffer; Kavakli, Kaan; Tekgul, HasanRisk factors of children with arterial ischemic stroke were retrospectively evaluated. The children were grouped according to values on developing diagnostic tools: 13 in the old era (19871994) and 18 in the new era (1995-2004). The old era battery included 5 tests: protein C, protein S, antithrombin, lupus anticoagulants, and anticardiolipin antibodies. The new era battery added 5 more tests: homocystine level, factor VIII level, mutations for factor V Leiden and prothrombin G20210A, and lipoprotein (a) level. At least I risk factor was found in 5 of 13 children (38.5%) in the old era and in 8 of 18 (44.4%) in the new era. The extended battery for prothrombotic disorders revealed 7 risk factors in 4 children (22.2%) in the new era, whereas the limited battery identified a single risk factor in I child (7.7%) in the old era. For the correct etiologic identification, prothrombotic risk factors should be extensively evaluated in patients with arterial ischernic stroke.Öğe THE ASSOCIATION BETWEEN HLA CLASS I AND II ALLELES AND THE OCCURRENCE OF INHIBITORS IN TURKISH PATIENTS WITH HEMOPHILIA A: A PILOT STUDY(Wiley, 2019) Patiroglu, Turkan; Cansever, Murat; Akbayram, Sinan; Gulen, Huseyin; Oncel, Kahraman; Borst, Ozcan; Oymak, Yesim; Aral, Yusuf Ziya; Ay, Yilmaz; Kilinc, Yurdanur; Oren, Hale; Kavakli, KaanÖğe Characterizing a Cohort of Patients with Hemophilia B Treated with Fidanacogene Elaparvovec from the Phase 3 Benegene-2 Study Who Returned to Factor IX Prophylaxis(Amer Soc Hematology, 2023) Frenzel, Laurent; Kavakli, Kaan; Klamroth, Robert; Chiou, Shyh-Shin; Shapiro, Amy D.; Sun, Pengling; Fuiman, Joanne[Abstarct Not Available]Öğe Clinical, Laboratory and Molecular Approach to Ten Children with Congenital Neutropenia(Galenos Yayincilik, 2016) Karaca, Neslihan Edeer; Aksu, Guzide; Gulez, Nesrin; Azarsiz, Elif; Kavakli, Kaan; Klein, Christoph; Kutukculer, NecilAim: Severe congenital neutropenia is a rare immunodeficiency disease characterized by lack of mature neutrophils. We evaluated the association between the molecular, clinical and laboratory findings together with genotype-phenotype relationship in 10 patients with neutropenia. Materials and Methods: The clinical and laboratory findings of ten patients with severe congenital neutropenia were obtained and the diagnosis was confirmed by mutation analysis. Results: The mutation analysis by DNA sequencing revealed HAX-1 mutation in 3 patients from the same family and ELANE/ELA-2 mutation in 1 patient. We compared the patients who had normalization in neutrophil counts and clinical findings spontaneously by age with the patients with HAX1 and ELANE/ELA2 defects and observed that patients with known genetic defects had higher monocyte and immunoglobulin levels on admission. Conclusion: The risk of persistence of neutropenia and the chance to reach a genetic diagnosis is higher in neutropenic patients who have accompanying eosinophilia, monocytosis and hypergammaglobulinemia at the time of initial investigation.Öğe Co-existing mild Haemophilia A with Mild Type 1 Von Willebrand Disease: Case Report(Akad Doktorlar Yayinevi, 2011) Akin, Mehmet; Karapinar, Deniz Yilmaz; Balkan, Can; Ay, Yilmaz; Kavakli, KaanVon Willebrand disease and haemophilia A are the two most common inherited bleeding disorders. Despite the relatively high frequency of those two bleeding disorders in the general population, reports of their coexistence together or of combined coagulopathies in general are rare. We describe a 1-year-old male with confirmed mild haemophilia A co-existing with mild type 1 VWD. The 1- year old male was admitted to our hospital with a history of excessive bleeding following circumcision. Initial laboratory evaluation revealed a prolonged activated partial thromboplastin time (APTT) of 46.2 s (normal range 23.2-34.7), and low FVIII activity level of 5.5% of normal. His subsequent evaluation, was also consistent with mild type 1 VWD with a decreased VWF antigen (VWF:Ag) of 50%, decreased ristocetin cofactor activity (VWF:RCo) of 44%. The DNA testing detected a C2 domain R2304H mutation of the FVIII gene.Öğe A Common VWF Exon 28 Haplotype in the Turkish Population(Sage Publications Inc, 2013) Berber, Ergul; Pehlevan, Funda; Akin, Mehmet; Capan, Ozlem Yalcin; Kavakli, Kaan; Caglayan, S. HandeAn increasing number of mutations and polymorphisms have been identified in the von Willebrand factor (VWF) gene of patients with von Willebrand disease (VWD). Most of the sequence alterations are within exon 28, duplicated in the VWF pseudogene on chromosome 22. Genetic recombination causing the gene conversion between the VWF gene and its pseudogene is associated with multiple substitutions in the VWF gene and with VWD. In the present study, VWF gene exon 28 was analyzed in 33 patients with VWD by DNA sequencing. A total of 73% of the patients were heterozygous for p.D1472H, p.V1485L, p.1500A, p.1501F, p.L1503P, and p.S1506L single-nucleotide polymorphisms. Family analysis revealed that the gene conversion occurred between the VWF gene and its pseudogene in 3 patients. Case-control association analysis by Haploview 4.2 did not show an association between the haplotype and VWD. In conclusion, a common exon 28 haplotype in the Turkish population, which might have arisen from the gene conversion events in the founder population, was identified.Öğe Concizumab restores thrombin generation potential in patients with haemophilia: Pharmacokinetic/pharmacodynamic modelling results of concizumab phase 1/1b data(Wiley, 2019) Eichler, Hermann; Angchaisuksiri, Pantep; Kavakli, Kaan; Knoebl, Paul; Windyga, Jerzy; Jimenez-Yuste, Victor; Delff, Philip Harder; Chowdary, PratimaIntroduction Concizumab enhances thrombin generation (TG) potential in haemophilia patients by inhibiting tissue factor pathway inhibitor (TFPI). In EXPLORER3 (phase 1b), a dose-dependent pharmacokinetic/pharmacodynamic (PK/PD) relationship was confirmed between concizumab dose, free TFPI and TG potential. Aim Determine the association between concizumab exposure, PD markers (free TFPI; peak TG) and bleeding episodes to establish the minimum concizumab concentration for achieving sufficient efficacy. Methods Free TFPI predictions were generated using an estimated concizumab-free TFPI exposure-response (E-max) model based on concizumab phase 1/1b data for which simultaneously collected concizumab and free TFPI samples were available. Concizumab concentration at the time of a bleed was predicted using a PK model, based on available data for concizumab doses >50 mu g/kg to <= 9 mg/kg. Peak TG vs concizumab concentration analyses and an E-max model were constructed based on EXPLORER3 observations. Results The E-max model showed a tight PK/PD relationship between concizumab exposure and free TFPI; free TFPI decreased with increasing concizumab concentration. A strong correlation between concizumab concentration and peak TG was observed; concizumab >100 ng/mL re-established TG potential to within the normal reference range. Estimated EC50 values for the identified concizumab-free TFPI and concizumab-TG potential models were very similar, supporting free TFPI as an important biomarker. A correlation between bleeding episode frequency and concizumab concentration was indicated; patients with a concizumab concentration >100 ng/mL experienced less frequent bleeding. The PK model predicted that once-daily dosing would minimize within-patient concizumab PK variability. Conclusion Concizumab phase 2 trials will target an exposure >= 100 ng/mL, with a once-daily regimen.Öğe Correlation between HJHS and HEAD-US Scores during Prophylaxis in Turkish Patients with Hemophilia: National Prospective Observational Study(Wiley, 2020) Kavakli, Kaan; Antmen, Bulent; Eyupoglu, S. Selin Aytac; Sahin, Fahri; Zulfikar, Bulent; Sonmez, Mehmet; Bulakci, Mesut[No abstract available]Öğe Cost of hemophilia A in Turkey: an economic disease burden analysis(Taylor & Francis Ltd, 2021) Malhan, Simten; Oksuz, Ergun; Antmen, Bulent; Ar, Muhlis Cem; Balkan, Can; Kavakli, KaanObjective: Hemophilia A is the second most common bleeding disorder causing patients to have lifelong follow-up and treatment. Despite being a rare disease, hemophilia A has a high economic burden on individuals and the public. The purpose of this study was to estimate the total disease cost of hemophilia A in Turkey. Materials and Methods: Data used in this analysis were collected through literature review, including studies conducted in Turkey in December 2018. A disease burden analysis was performed by modeling hemophilia A-related costs among patients, their relatives, and the social security system. Two expert panels were held to evaluate real-world data sources and to provide further information. All direct medical and non-medical costs were calculated annually from the Social Security Institution of the Republic of Turkey perspective, while indirect costs were estimated from the patient and community perspective. Results: For the calendar year of 2018, the number of hemophilia A patients in Turkey were estimated to be 5,055, with an average weight of 64.7 kg. The average annual direct medical, direct non-medical, and indirect costs of hemophilia A were calculated as euro93,268 ($109,286; (sic)502,717), euro2,533 ($2,968; (sic)13,655), and euro7,957 ($9,323; (sic)42,888) per patient, respectively, with a total annual cost of euro103,759 ($121,578; (sic)559,259). For the management of patients with inhibitors (4.9%), the average annual total cost was calculated to be euro325,439 ($381,330; (sic)1,754,117) per patient. The total annual disease burden of hemophilia A in 2018 was estimated to be about euro524 million ($614 million; (sic)2.82 billion), which corresponded to 1.6% of the total health expenditure in Turkey. Conclusion: The most important reason hemophilia A has a significant economic burden in Turkey is that replacement therapy is expensive. The major cost contributor was identified as factor replacement therapy. With inhibitor development, the average annual cost increased more than 3-fold.Öğe Current childhood chronic myeloid leukemia management under tyrosine kinase inhibitor treatment(Springer Japan Kk, 2022) Karadas, Nihal; Goktepe, Serife Sebnem Onen; Bas, Ilke; Ece, Dilek; Ozdemir, Hamiyet Hekimci; Balkan, Can; Kavakli, KaanChronic myeloid leukemia (CML) is very rare during childhood. Tyrosine kinase inhibitors (TKI) provide very good results in terms of survival. The medical records of 15 chronic phase (CP)-CML patients in a university hospital pediatric hematology department between 1997 and 2022 were reviewed retrospectively. Complete hematological response was documented in all patients between 20 and 68 (median 30) days of treatment. Major molecular response was achieved in seven patients within 6 months. Median follow-up for the study group was 79 (range 3-330) months and overall survival was 100%. Three patients (2 blastic transformation, 1 therapy resistant) underwent bone marrow transplantation (BMT) and one with blastic transformation is scheduled to undergo BMT. TKI were discontinued in three patients after a median of 86 (range 73-177) months. The complete molecular remission maintenance period before discontinuation of TKI was 81 (range 62-122) months. While no molecular relapse was seen before the last follow-up, the median overall follow-up period was 152 (range 131-300) months. In conclusion, recent advances have led to a very good prognosis for children with CP-CML. With TKI treatment, most patients continue their lives without disease progression. Additionally, in selected patients TKI can be discontinued without molecular relapse.Öğe A developing aspect of hemophilia nursing: the role nurses play in clinical trials(Wiley-Blackwell, 2014) Senol, Selmin; Bayro, Idil; Kavakli, KaanÖğe Diagnosis, therapeutic advances, and key recommendations for the management of factor X deficiency(Churchill Livingstone, 2021) Peyvandi, Flora; Auerswald, Guenter; Austin, Steven K.; Liesner, Ri; Kavakli, Kaan; Roman, Maria Teresa Alvarez; Millar, Carolyn M.Factor X deficiency is a rare coagulation disorder that can be hereditary or acquired. The typology and severity of the associated bleeding symptoms are highly heterogeneous, adding to the difficulties of diagnosis and man-agement. Evidence-based guidelines and reviews on factor X deficiency are generally limited to publications covering a range of rare bleeding disorders. Here we provide a comprehensive review of the literature on factor X deficiency, focusing on the hereditary form, and discuss the evolution in disease management and the evidence associated with available treatment options. Current recommendations advise clinicians to use single-factor replacement therapy for hereditary disease rather than multifactor therapies such as fresh frozen plasma, cry-oprecipitate, and prothrombin complex concentrates. Consensus in treatment guidelines is still urgently needed to ensure optimal management of patients with factor X deficiency across the spectrum of disease severity.Öğe Does Digital Templating of total knee arthroplasties reliable in hemophilic arthropathies?(Wiley, 2021) Er, Erdem; Bicer, Elcil Kaya; Sahin, Fahri; Kavakli, Kaan; Aydogdu, Semih[No Abstract Available]Öğe The Effect of Self-infusion and Patient Education on Treatment Compliance in Hemophilia Patients(Dr Behcet Uz Cocuk Hastaliklari Ve Cerrahisi, 2019) Kazanci, Elif Guler; Ugur, Mehmet Can; Oymak, Yesim; Kavakli, KaanObjective: Problematic compliance to treatment regimens is a current issue in the treatment of hemophilia. These problems have been widely reported. We aimed to investigate the effect of self-infusion and patient education on treatment compliance in hemophilia patients. Method: A questionnaire developed by Hemophilia Federation was sent to Hemophilia and von Willebrand (vWH) patients who participated in the Workshop of Hemophilia Federation in 2019. Within fifteen days, patients were asked to answer the questionnaires, and survey data were evaluated by appropriate statistical methods. Results: Patients with diagnosis of severe hemophilia A (n=74), severe hemophilia B (n=20) and vWH (n=3) were included in the survey study. Patients were participated from six different regions of Turkey. Eight patients could not complete secondary education. Sixty patients completed or continued their secondary education. 29 of them were graduated from a university. Sixty-four patients were complaint and 33 patients noncomplaint to the treatment. The treatment compliance rate was 66%. No significant relationship was found between the educational status and treatment compliance of the patients (p=0.516). The median ages of treatment-compliant, and noncompliant patients were 21.5, and 20.6 years, respectively (p=0.015). There was no statistically significant relationship between treatment compliance and selfinfusion, self-infusion learning source and longevity of internet usage. Conclusion: The success in the follow-up and treatment of chronic diseases such as hemophilia is enhanced by the training of the physicians and other health personnel, as well as the education level of patients and their relatives.Öğe Efficacy and safety of a new human fibrinogen concentrate in patients with congenital fibrinogen deficiency: an interim analysis of a Phase III trial(Wiley, 2018) Lissitchkov, Toshko; Madan, Bella; Khayat, Claudia Djambas; Zozulya, Nadezhda; Ross, Cecil; Karimi, Mehran; Kavakli, Kaan; De Angulo, Guillermo R.; Almomen, Abdulkareem; Schwartz, Bruce A.; Solomon, Cristina; Knaub, Sigurd; Peyvandi, FloraBACKGROUNDFibrinogen concentrate is the preferred choice for fibrinogen replacement in congenital fibrinogen deficiency. This study investigated hemostatic efficacy of a new plasma-derived, double virus-inactivated (using two dedicated virus inactivation/elimination steps) human fibrinogen concentrate for on-demand treatment of bleeding episodes (BEs) and surgical prophylaxis. STUDY DESIGN AND METHODSIn this planned interim analysis of a prospective, multinational Phase III study (NCT02267226), 13 patients with afibrinogenemia (12 years) received fibrinogen concentrate (FIBRYGA, Octapharma AG). Hemostatic efficacy was assessed by investigators and an independent data monitoring and endpoint adjudication committee (IDMEAC) using objective four-point criteria and by thromboelastometry maximum clot firmness (MCF). RESULTSFibrinogen concentrate was used on-demand to treat 23 BEs in 11 patients, with 21 (91.3%) requiring a single infusion only. Treatment success was 95.7% (90% confidence interval [CI], 0.81-1.00; assessment missing for one BE) by investigators and 100% (90% CI, 0.88-1.00) by IDMEAC. Mean MCF increased significantly from 0.0 to 6.5 mm (95% CI, 5.65-7.40; p<0.0001) at 1 hour postinfusion of a median (range) dose of 58.8 (33.9-101.7) mg/kg per BE. Four patients received fibrinogen concentrate as surgical prophylaxis, with intraoperative and postoperative treatment success rated 100% (90% CI, 0.50-1.00) by investigators and IDMEAC (median [range] dose per surgery 93.5 [34.1-225.4] mg/kg). No additional hemostatic interventions were required. No deaths, thromboses, or seroconversions were reported. CONCLUSIONThese data showed that the new fibrinogen concentrate was efficacious for on-demand treatment of acute bleeding and surgical prophylaxis in congenital afibrinogenemia patients.Öğe Efficacy and Safety of Fitusiran Prophylaxis, an siRNA Therapeutic, in a Multicenter Phase 3 Study (ATLAS-INH) in People with Hemophilia A or B, with Inhibitors (PwHI)(Amer Soc Hematology, 2021) Young, Guy; Srivastava, Alok; Kavakli, Kaan; Ross, Cecil; Sathar, Jameela; Tran, Huyen; Wu, Runhui[No Abstract Available]Öğe Evaluation of Bleeding Phenotype of Inherited Factor VII Deficiency in Children With a Bleeding Assessment Tool and Global Assays(Lippincott Williams & Wilkins, 2020) Toret, Ersin; Ay, Yilmaz; Karapinar, Tuba H.; Oymak, Yesim; Kavakli, Kaan; Vergin, Raziye C.Introduction:Inherited factor VII (FVII) deficiency is the most common of the rare bleeding disorders and shows a heterogenous distribution of bleeding phenotypes independent of factor activity level. the bleeding score (BS) evaluates the phenotype of patients with rare bleeding disorders. Thromboelastography (TEG) and thrombin generation assays (TGAs) are 2 methods to evaluate global hemostasis, and controversially both tests are useful for identifying different bleeding tendency phenotypes. the purpose of this study was to investigate the use of the BS and global assays (TEG and TGAs) to predict the bleeding phenotype of inherited FVII deficiency.Materials and Methods:A total of 27 patients with FVII deficiency were evaluated with the BS and global hemostasis assays.Results:The BS was compatible with disease severity according to the FVII activity level (P<0.05) but the BS and bleeding grade of patients did not show a statistically significant correlation with factor activity level (P>0.05). No significant correlation was observed between the factor activity level and any TEG parameter (P>0.05). the factor activity level was negatively correlated with the lag time of the TGA on the contrary positively correlated with the peak thrombin time of the TGA (P<0.05).Conclusions:The global assays do not successfully predict the bleeding phenotype. the BS is a more suitable tool than conventional and global assays for predicting the bleeding phenotype.
