Browsing by Author "Oner, Ahmet F."
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Article Brachial Plexus Paralysis Due To Giant Cavernous Hemangioma With Kasabach-Merritt Syndrome: Successful Management With Interferon Alpha(Taylor & Francis Ltd, 2009) Peker, Erdal; Kirimi, Ercan; Tuncer, Oguz; Ceylan, Abdullah; Oner, Ahmet F.Giant hemangiomas are rare in newborn babies and may cause some life-threatening complications such as congestive heart failure and coagulopathies including severe thrombocytopenia, microangiopathic hemolytic anemia and bleeding such as Kasabach-Merritt syndrome. We have presented here one infant case with a right lower brachial plexus paralysis due to giant hemangioma treated successfully with interferon alpha and have discussed the treatment. This is the first case of Kasabach-Merritt syndrome associated with a right lower brachial plexus paralysis treated with interferon in the literature to our knowledge. The administration of interferon-alpha might be indicated as a therapy for severe, life-threatening and giant hemangiomas such as Kasabach-Merritt syndrome, especially when there is resistance to steroid. However, more extensive data and new patients are needed to clarify this issue.Article A Case of Chediak-Higashi Syndrome Presented With Hemophagocytic Lymphohistiocytosis(Akad Doktorlar Yayinevi, 2011) Akbayram, Sinan; Akgun, Cihangir; Basaranoglu, Murat; Kaya, Avni; Balta, Gunay; Ustyol, Lokman; Oner, Ahmet F.Chediak Higashi syndrome, is a rare autosomal recessive disorder characterised by oculocutaneus albinism, recurrent respiratory system infections and other pyogenic infections. Hemophagocytic lymphohistiocytosis can develop in any time of the life in patients with Chediak Higashi syndrome. A 14-month-old girl patient was diagnosed as hemophagocytic lymphohistiocytosis with the laboratory findings of pancytopenia, high levels of triglyceride and ferritin, hypofibrinogenemia, low ratio of natural killers in lymphocyte subtypes, and with determined macrophages that made hemophagocytosis in recurrent bone marrow aspirates. The treatment protocol of hemophagocytic lymphohistiocytosis 2004 was administered. During the maintenance treatment, recurrence was developed. In the second bone marrow examination, the diagnosis of Chediak Higashi syndrome was made with determined intracytoplasmic giant granules. Hair analysis result was meaningful for Chediak Higashi syndrome. In this report, we would like to emphasize the condition that especially in early infants, Chediak Higashi syndrome presenting with hemophagocytic lymphohistiocytosis may be misdiagnosed because of the uncertain clinical findings and this can be the result of resistance to treatment.Article Combinatorial Antibody Libraries From Survivors of the Turkish H5n1 Avian Influenza Outbreak Reveal Virus Neutralization Strategies(Natl Acad Sciences, 2008) Kashyap, Arun K.; Steel, John; Oner, Ahmet F.; Dillon, Michael A.; Swale, Ryann E.; Wall, Katherine M.; Lerner, Richard A.The widespread incidence of H5N1 influenza viruses in bird populations poses risks to human health. Although the virus has not yet adapted for facile transmission between humans, it can cause severe disease and often death. Here we report the generation of combinatorial antibody libraries from the bone marrow of five survivors of the recent HSN1 avian influenza outbreak in Turkey. To date, these libraries have yielded >300 unique antibodies against H5N1 viral antigens. Among these antibodies, we have identified several broadly reactive neutralizing antibodies that could be used for passive immunization against H5N1 virus or as guides for vaccine design. The large number of antibodies obtained from these survivors provide a detailed immunochemical analysis of individual human solutions to virus neutralization in the setting of an actual virulent influenza outbreak. Remarkably, three of these antibodies neutralized both H1 and H5 subtype influenza viruses.Article Concurrent Celiac Disease, Idiopathic Thrombocytopenic Purpura and Autoimmune Thyroiditis: a Case Report(Sage Publications inc, 2011) Dogan, Murat; Sal, Ertan; Akbayram, Sinan; Peker, Erdal; Cesur, Yasar; Oner, Ahmet F.Celiac disease (CD) is a disease of the small intestine caused by an immune response to ingested gluten. Idiopathic thrombocytopenic purpura (ITP) is a common acquired bleeding disorder of childhood. It may follow a viral infection or immunization and is caused by an inappropriate response of the immune system. Autoimmune thyroiditis (AT) is a disease that occurs due to autoimmune mechanisms. Celiac disease associated with autoimmune thyroid disease is well known, but the association of CD, autoimmune thyroid disease, and ITP has been reported very rarely in the literature. In the current report, we have presented a case with CD, AT, and acute ITP, because this association is rarely seen, and to emphasize that CD and AT should be kept in mind in patients with ITP.Article Evaluation of Renal Function Disorder With Urinary Neutrophil Gelatinase-Associated Lipocalin Level in Patients With Β-Thalassemia Major(Lippincott Williams & Wilkins, 2019) Karaman, Kamuran; Sahin, Serdar; Geylan, Hadi; Yasar, Akkiz Sahin; Cetin, Mecnun; Komuroglu, Ahmet U.; Oner, Ahmet F.Background and Aim: Neutrophil gelatinase-associated lipocalin (NGAL) is found in the granules of human neutrophils, with many diverse functions. Expression of NGAL is induced under various pathophysiological conditions, for example, infection, inflammation, kidney injury, cardiovascular disease, burn injury, and intoxication, and it has an important antiapoptotic and anti-inflammatory role. Patients and Methods: A total of 37 patients with thalassemia disease beta-thalassemia major (24 male and 13 female individuals) and 37 healthy controls (17 male and 20 female) were enrolled in this study. All patients were regularly transfused, and all of them were using oral chelator, deferasirox. Results: NGAL and cystatin C levels were significantly higher in the case group than in the control group. Conclusion: In patients with beta-thalassemia major, renal damage can occur owing to regular blood transfusion. Urinary NGAL levels in these patients may be considered as a marker for early renal injury.Conference Object Familial Hemophagocytic Lymphohistiocytosis Type Iv: Possible Founder Effect in Syntaxin 11 Gene Common Mutation Endemic To Turkish Population(Amer Soc Hematology, 2008) Caliskan, Umran; Guler, Elif; Patiroglu, Turkan; Oner, Ahmet F.; Gurgey, AytemizArticle Frequency of the P.gly262asp Mutation in Congenital Factor X Deficiency(Wiley-blackwell, 2015) Epcacan, Serdar; Menegatti, Marzia; Akbayram, Sinan; Cairo, Andrea; Peyvandi, Flora; Oner, Ahmet F.Introduction Congenital factor X (FX) deficiency is a rare bleeding disorder inherited as an autosomal recessive trait with an incidence of 1 :500 000-1 000 000. A total or partial deficiency of FX causes an impairment of clot formation, leading to a haemorrhagic disease, which manifests with bleeding symptoms of different severity, also unprovoked. Aim We analysed the clinical manifestations, laboratory phenotype and genotype in 12 patients from Turkey affected with severe FX deficiency. Methods Prothrombin time (PT), activated partial thromboplastin time (APTT), FX activity (FX:C) and FX antigen level (FX:Ag) were measured, and mutation analysis was performed for all patients. Results The most frequent bleeding episodes in patients were epistaxis and easy bruising (11/12, 91%), followed by haemarthroses (10/12, 83%). FX:C was <1% in 11 patients, and 4% in one. FX:Ag was reduced in all patients, consistent with type II deficiency. Direct sequencing of the factor X gene (F10) identified two different mutations:the novel 33 bp in-frame deletion p.Thr176_Gln186, c.526_558del, which seems to be associated with milder bleeding symptoms and the c.785G>A, p.Gly262Asp missense mutation (previously reported as Gly222Asp), which is associated with severe bleeding symptoms. Conclusion The p.Gly262Asp missense mutation was identified in 11 of the 12 patients in this study. Previously published cases on the same p.Gly262Asp mutation were Iranian patients originating from the border between Turkey and Iran suggesting that this mutation may be candidate as a good tool for mutational screening analysis in this area.Article Hematologic Findings in Children With Brucellosis: Experiences of 622 Patients in Eastern Turkey(Lippincott Williams & Wilkins, 2016) Karaman, Kamuran; Akbayram, Sinan; Bayhan, Guelsuem I.; Dogan, Murat; Parlak, Mehmet; Akbayram, Hatice T.; Oner, Ahmet F.The aim of the present study was to present the hematologic findings of 622 cases diagnosed with acute brucellosis. Medical records of children with brucellosis, admitted to Yuzuncu Yil University Hospital over a 6-year period, were analyzed retrospectively. Three hundred ninety-seven (63.8%) patients were male and 225 (36.2%) were female. The mean age was 11 years (range, 1 to 16 y). The mean hematologic values were as follows: hemoglobin 9.4 (3.4 to 17) g/dL, hematocrite 28% (12% to 50%), leukopenia 2.8 (1.1 to 33)x10(9)/L, leukocytosis 13.7 (1.1 to 33)x10(9)/L, and platelets 79 (3 to 972)x10(9)/L. An overall 28.6% of patients were found to have anemia, alone or in combination with leukopenia or thrombocytopenia. Sixteen percent of patients had thrombocytopenia, 13.9% had leukopenia, 7.7% had pancytopenia, and 4% had both leukopenia and thrombocytopenia. Hematologic complications such as anemia, thrombocytopenia, and leukopenia are more frequently seen in acute brucellosis cases. However, acute brucellosis should also be considered in the differential diagnosis in the presence of other hematologic abnormalities such as severe thrombocytopenia, bicytopenia, and pancytopenia.Letter Immune Thrombocytopenic Purpura in a Case of Propionic Acidemia: Case Report(Lippincott Williams & Wilkins, 2014) Bulan, Keziban; Dogan, Murat; Kaba, Sultan; Dogan, Sekibe Zehra; Akbayram, Sinan; Oner, Ahmet F.Article Static Postural Balance Evaluation and an Investigation of the Relationship With Joint Health in Children With Severe Haemophilia: a Controlled Cross-Sectional Study(Wiley, 2021) Tat, Necati M.; Tat, Ayse M.; Oner, Ahmet F.; Karaman, Kamuran; Kaplan, Seyhmus; Can, FilizIntroduction Recurrent hemarthroses disturbing force/movement control are likely to impair postural balance. Aim To investigate the possible changes in static postural balance parameters in children with haemophilia (CwH) and to reveal its relationship with Haemophilia Joint Health Score (HJHS). Methods Twenty-one CwH aged 6-18 who had haemophilic arthropathy in at least one lower limb joint and 21 healthy peers were evaluated by using a force platform. Centre of pressure (CoP) signals were displayed as a map in both anteroposterior direction (APD) and mediolateral direction (MLD) by statokinesigram. Accordingly, the amplitude, velocity and standard deviation of CoP displacements along with the perimeter and ellipse area were measured. Assessments were made under eyes opened and eyes closed in bipedal stance for 60 s. Joint health and muscle strength were evaluated with HJHS and digital dynamometer, respectively. Results Velocity and amplitude of CoP displacements in MLD were increased in CwH (p<0.05). It was also found that these parameters were moderate correlated with the clinical score of the lower limb joints (p<0.05). In CwH, standard deviation of CoP displacements in the APD was significantly higher in eyes closed, while standard deviation in MLD was significantly higher in eyes opened (p<0.05). Conclusion Disturbances during bipedal stance reveal the need for balance evaluation in CwH. Increased mediolateral oscillations may be an early sign of disorders of the musculoskeletal system in CwH. In addition to improving joint health, postural balance exercises that increase MLD stability have to be included in the rehabilitation programme of CwH.Article Thrombocytopenia Associated With Galsulfase Treatment(Sage Publications Ltd, 2011) Dogan, Murat; Cesur, Yasar; Peker, Erdal; Oner, Ahmet F.; Dogan, Sekibe ZehraMucopolysaccharidosis type VI (MPS VI), or Maroteaux-Lamy syndrome, is a lysosomal storage disorder that results from a deficiency of the enzyme N-acetylgalactosamine-4-sulfatase or arylsulfatase B (ASB). It is a relatively rare disorder, with an estimated incidence of 1 in 248,000 to 1 in 300,000. The diagnosis is made on the basis of findings of elevated urine glycosaminoglycans and a deficiency of ASB activity in leukocytes or cultured fibroblasts. In treatment of MPS VI, enzyme replacement therapy (galsulfase; human recombinant ASB enzyme) became available. Infusions of galsulfase were generally well tolerated. But in some patients, infusion-associated reactions including rash, urticaria, headache, hypotension, nausea, and vomiting were documented and were managed successfully by interrupting or slowing the rate of infusion and/or by the administration of antihistamines, antipyretics, corticosteroids, or oxygen. Here, we report a case with MPS VI who developed thrombocytopenia after third dose of therapy. To the best of our knowledge, this is the first report about thrombocytopenia associated with galsulfase therapy in the literature. Additionally, with this report, we want to share our approach for this case.Article Treatment of Chronic Immune Thrombocytopenic Purpura With Rituximab in Children(Springer india, 2009) Dogan, Murat; Oner, Ahmet F.; Acikgoz, Mehmet; Uner, AbdurrahmanTo evaluate the rituximab treatment in children with chronic immune thrombocytopenic purpura This study included ten children with chronic immune thrombocytopenic purpura, which were nonresponsive to Steroid (S), IVIG and anti-D treatments. Rituximab was given with a dosage of 375 mg/m2 weekly for 4-6 weeks. Initial platelet count was less than 30x109/L and responses were assessed in follow-up. The patients' groups were categorized as complete remission (CR);a platelet count a parts per thousand yen150x10(9)/L,partial remission (PR);a platelet count ranging from 50x10(9)/L to 150x10(9)/L, minimal remission (MR); a platelet count ranging from 30x10(9)/L to 50x10(9)/L and no response (NR); a platelet count less than 30x10(9)/L. Of our patients, four female and six male, their ages ranged from 39 mth to 13 yr and the mean age was 83.4 +/- 44.58 mth. None of the patients was splenectomized. The follow-up period after rituximab treatment ranged between 12 to 42 mth and the mean follow-up period was 25.10 +/- 13.03 months. While on this treatment, we had a CR in two patients, a PR in one, a MR in three, but no response in four. The patients in CR/PR are still being followed as in remission and they have 40 mth of mean follow-up period. The three patients in MR had a decrease in values of platelets earliest in one mth and the latest in four mth. Adverse effects of rituximab, such as itching and scraps that were not clinically significant were observed in three patients during rituximab infusion. There were no increase in infections after rituximab in any patient. CR was found in 20% of our patients, PR in 10% and MR in 30% with rituximab. On this treatment, while some series had good outcomes with this treatment (72%-100%, remission ratios), but many series, such as ours, had a poor response rate contrast to many reported case series in the literature. This condition may be associated with the age of our most patients who were young at the time of commenced rituximab. However, we believe that more studies are required to elucidate the reasons for different results in different case series reported in literature.Article Typhoid Fever Accompanied With Hematopoetic Lymphohistiocytosis and Rhabdomyolysis in a Refugee Child(Lippincott Williams & Wilkins, 2019) Yasar, Akkiz Sahin; Karaman, Kamuran; Geylan, Hadi; Cetin, Mecnun; Guven, Burcu; Oner, Ahmet F.Typhoid fever is an acute and systemic infectious disease that is specific to humans and is caused by Salmonella typhi. Patients may present with different findings ranging from simple fever to multiple organ failure. Hemophagocytosis and rhabdomyolysis are rare complications of typhoid fever. In this study, we presented a case of hemophagocytic lymphohistiocytosis and rhabdomyolysis in a 14-year-old girl who migrated from Mosul, Iraq, 1 month ago.Article Use of a High-Purity Factor X Concentrate in Turkish Subjects With Hereditary Factor X Deficiency: Post Hoc Cohort Subanalysis of a Phase 3 Study(Galenos Yayincilik, 2018) Oner, Ahmet F.; Celkan, Tiraje; Timur, Cetin; Norton, Miranda; Kavakli, KaanHereditary factor X (FX) deficiency is a rare bleeding disorder more prevalent in countries with high rates of consanguineous marriage. In a prospective, open-label, multicenter phase 3 study, 25 IU/kg plasma-derived factor X (pdFX) was administered as on-demand treatment or short-term prophylaxis for 6 months to 2 years. In Turkish subjects (n=6), 60.7% of bleeds were minor. A mean of 1.03 infusions were used to treat each bleed, and mean total dose per bleed was 25.38 IU/kg. Turkish subjects rated pdFX efficacy as excellent or good for all 84 assessable bleeds; investigators judged overall pdFX efficacy to be excellent or good for all subjects. Turkish subjects had 51 adverse events; 96% with known severity were mild/moderate, and 1 (infusion-site pain) was possibly pdFX-related. These results demonstrate that 25 IU/kg pdFX is safe and effective in this Turkish cohort