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Öğe Co-existing mild haemophilia a with mild type 1 von willebrand disease: Case report [Hafif tip von willebrand hastali{dotless}gi{dotless} ile hafif hemofili a birlikteligi](2011) Akin M.; Karapinar D.Y.; Balkan C.; Ay Y.; Kavakli K.Von 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. We believe that the co-existence of VWD and haemophilia A is underappreciated, under-diagnosed, and under-reported.Öğe DDAVP treatment in children with haemophilia B [2](2001) Kavakli K.; Aydinok Y.; Karapinar D.; Balkan C.[No abstract available]Öğe An evaluation of the DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in children(2011) Akin M.; Karapinar D.Y.; Balkan C.; Ay Y.; Kavakli K.von Willebrand disease (vWD) is classified into partial (type 1), qualitative (type 2), and total deficiency (type 3).The aims of the study were to evaluate prospectively the potency of the DDAVP infusion test together with von Willebrand factor (vWF) ristocetin cofactor (vWF:RCo), vWF antigen (vWF:Ag), factor VIII coagulant activity (FVIII:C), and platelet function analyzer (PFA)-100 to distinguish vWD types. Genetic analysis and multimeric analysis of vWF was not applied. We classified the 112 patients and 47 healthy children phenotypically according to the laboratory test results and bleeding severity score. PFA-100 closure times (CT), FVIII:C, vWF:RCo, vWF:Ag, ristocetin-induced platelet aggregation (RIPA), and the response of FVIII:C and vWF parameters to desmopressin (DDAVP) were used to define types 1, 2, and 3 vWD. Type 1 vWD is mild in 34 cases (vWF:RCo % 40-55), moderate in 29 (vWF:RCo %27-40), severe type 1 vWD or nonclassical type 2 vWD in 12 cases (vWF:RCo % 4-16), and type 2 vWD in 23 cases (vWF:RCo %4-38).The response to DDAVP of vWF parameters is normal in all patients with mild/moderate type 1 vWD, 6 patients with severe type 1 vWD or nonclassical type 2 vWD and 11 patients with type 2 vWD. In conclusion, this study showed that measurement of vWF:RCo, vWF:Ag, FVIII:C, and PFA-100 parameters can differentiate vWD types but not severe type 1 vWD or nonclassical type 2 vWD. In the differentiation of severe type 1 vWD and nonclassical type 2 vWD, DDAVP response may be used. © SAGE Publications 2011.Öğe Experience of pandemic influenza with H1N1 in children with leukemia(2011) Karapinar D.Y.; Ay Y.; Karzaoglu Z.; Balkan C.; Ergin F.; Vardar F.; Kavakli K.It is not exactly known the risks from infection with pandemic influenza (H1N1) 2009 in children with leukemia. Here the authors present their experience in 5 children with leukemia. Pandemic influenza (H1N1) 2009 was detected in 5 patients (F/M: 3/2) at their institution. The ages of these patients were between 2 and 16 years. Four had acute lymphoblastic leukemia (ALL) and 1 acute myeloblasic leukemia (AML). Three of the ALL patients had the diagnosis of pandemic influenza (H1N1) 2009 at the same time as they were diagnosed with ALL. The remaining 2 patients were receiving intensive chemotherapy. All patients had fever, rhinorrhea, and cough. Although bronchopneumonia was seen in 3 patients, only 1 revealed respiratory distress. Stomachache and diarrhea was seen in the patient who had no pneumonia. All treated as inpatients, but none of them required hospitalization in intensive care unit. One to 3 days after the symptoms of influenza appeared, oseltamivir (Tamiflu) was given to all patients in combination with broad-spectrum antibiotics. Fever declined to normal ranges in 1 to 3 days after treatment was started. The patients received oseltamivir for 5 to 7 days. Cell culture tests were found to be positive for influenza A and polymerase chain reaction (PCR) revealed H1N1 for all 5 patients. Although this is a very small case series, pandemic influenza (H1N1) 2009 did not seem to be very dangerous for children with leukemia if the oseltamivir treatment was given early when symptoms of influenza appeared. © 2011 Informa Healthcare USA, Inc.Öğe Extended half-life coagulation factors: A new era in the management of hemophilia patients [Uzatılmış yarı ömürlü koagülasyon faktörleri: Hemofili tedavisinde yeni bir dönem](Turkish Society of Hematology, 2019) Ar M.C.; Balkan C.; Kavaklı K.Despite effective factor replacement and various treatment schedules, there remain several challenges and unmet needs in the prophylactic treatment of hemophilia limiting its adoption and thereby posing an increased risk of spontaneous bleeding. In this regard, extended half-life (EHL) recombinant factor VIII (rFVIII) and factor IX (rFIX) products promise optimal prophylaxis by decreasing the dose frequency, increasing the compliance, and improving the quality of life without compromising safety and efficacy. EHL products might lead to higher trough levels without increasing infusion frequency, or could facilitate the ability to maintain trough levels while reducing infusion frequency. This paper aims to provide a comprehensive review of the rationale for developing EHL coagulation factors and their utility in the management of hemophilia, with special emphasis on optimal techniques for half-life extension and criteria for defining EHL coagulation factors, as well as indications, efficacy, and safety issues of the currently available EHL-rFVIII and EHL-rFIX products. Potential impacts of these factors on quality of life, health economics, and immune tolerance treatment will also be discussed alongside the challenges in pharmacokinetic-driven prophylaxis and difficulties in monitoring the EHL products with laboratory assays. © 2019 by Turkish Society of Hematology.Öğe Feasibility of using thrombin generation assay (TGA) for monitoring bypassing agent therapy in patients with hemophilia having inhibitors(2013) Ay Y.; Balkan C.; Karapinar D.Y.; Akin M.; Bilenoglu B.; Kavakli K.Background: Monitoring bypassing agent therapy and observing concordance with clinical hemostasis is crucial in vital hemorrhages and major surgeries in patients with hemophilia having inhibitor. Objective: We aimed to investigate the value of the thrombin generation assay (TGA) and thromboelastography (TEG) for monitoring hemostasis in patients with hemophilia having inhibitor, during supplementation therapy with bypassing agents. Patients and Methods: The study group consisted of 7 patients with hemophilia having factor VIII inhibitor. All patients were male. The median age of the participants was 10 years. Age range was 6 to 32 years. The median inhibitor level was 10 Bethesda units (BU), with a range of 5 to 32 BU. A total of 17 bleeding episodes were evaluated. Both TEG and TGA tests were assessed in addition to clinical responses. Assessments were made prior to bypass agent therapy such as recombinant factor VIIa (rFVIIa) or activated prothrombin complex concentrate (aPCC) for bleeding episodes, during the first hour and 24 hours after either intervention in patients. Results: No relation between clinical response and TGA or TEG parameters was found in patients. There was no difference between clinical responses after rFVIIa and aPCC treatments. However, after aPCC treatment, endogenous thrombin potential and peak thrombin levels and also TEG R, K, and alpha angle degrees were significantly higher. Conclusions: In conclusion, we found that the clinical effectiveness of bypass therapy in hemophilia cannot be assessed by TGA and TEG. © 2012 The Author(s).Öğe A further Turkish case of Griscelli syndrome with new RAB27A mutation(2008) Onay H.; Balkan C.; Cogulu O.; Aydinok Y.; Karapinar D.Y.; Özkınay F.[No abstract available]Öğe FVIII inhibitor development according to concentrate: Data from the EUHASS registry excluding overlap with other studies(Blackwell Publishing Ltd, 2016) Fischer K.; Iorio A.; Hollingsworth R.; Makris M.; Watson H.; Rae J.; Platokouki H.; Pergantou H.; Katsarou O.; Theodossiades G.; Nomikou E.; Parra R.; Alonso S.; Klamroth R.; Kubicek C.; Wilde J.; Dunkley T.; Oldenburg J.; Schmickler D.; Ackroyd S.; Mirza L.; Batorova A.; Jankovicova D.; Auerswald G.; Buehrlen M.; Penka M.; Smejkal P.; Blatny J.; Zapletal O.; Hermans C.; Lambert C.; Tagariello G.; Radossi P.; Nolan B.; Brady B.; O'Donnell J.; Singleton E.; Thomas A.; Shea I.; Morfini M.; Linari S.; de Moerloose P.; Boehlen F.; Campbell Tait R.; Brodie N.; Chalmers E.; Gibson A.; Meijer K.; Tamminga R.; Lassila R.; Armstrong E.; Zulfikar B.; Ozdemir N.; Kavakli K.; Balkan C.; Jurgutis R.; Gailiute N.; Peerlinck K.; Geet C.V.; Goudemand J.; Wibaut B.; Diniz M.J.; Antunes M.; Liesner R.; Khair K.; Laffan M.; Patel S.; Thynn Yee T.; Harrington C.; Pasi J.; Bowles L.; Bevan D.; Madan B.; Negrier C.; Ringenbach S.; Mingot Castellano M.E.; Astermark J.; Lindvall K.; Gatt A.; Hay C.R.M.; Grey P.; Will A.; Hague A.; Peyvandi F.; Mancuso M.E.; Schved J.F.; Rousseau F.; Kurnik K.; Bidlingmaier C.; Rocino A.; Hanley J.; Talks K.; Antoniades M.; Zanon E.; Bon E.D.; Lambert T.; Rothschild C.; Matingou M.; Tagliaferri A.; Rivolta G.F.; Iorio A.; Marchesini E.; Fraga C.; Cunha-Ribeiro L.; Carvalho M.; Komrska V.; Pindurova E.; Lejniece S.; Kluce G.; Leebeek F.; Cnossen M.; Makris M.; Lockley C.; Payne J.; Vidler V.; Lissitchkov T.; Raia P.; Holmstre om M.; Garipidou V.; Vakalopoulou S.; Serban M.; Mihailov M.-D.; Schinco P.; Valeri F.; Schutgens R.; Bonanad S.; Cid A.R.; Castaman G.; Pabinger I.; Reitter S.-E.; Male C.; Thom K.; Windyga J.; Piorowski M.; Schmugge M.; Albisetti M.[No abstract available]Öğe Hypertension and life-threatening bleeding in children with relapsed acute myeloblastic leukemia treated with FLT3 inhibitors [FLT3 İnhibitörleri ile tedavi edilen nüks akut miyeloblastik lösemili çocuklarda hipertansiyon ve hayatı tehdit eden kanama](Turkish Society of Hematology, 2015) Yılmaz Karapınar D.; Karadaş N.; Siviş Z.Ö.; Balkan C.; Kavaklı K.; Aydınok Y.Experiences with new multikinase inhibitors are limited, especially in children. In this report we summarize our experience with 2 patients with relapsed acute myeloblastic leukemia (AML), one with FMS-like tyrosine kinase- 3-internal tandem duplication mutation and the other with a single base mutation (D835Y). Both patients received sorafenib, one for 19 days and the other for 42 days, with clofarabine-including chemotherapy. One additionally received sunitinib for a total of 20 days. Both patients developed severe pancytopenia, hypertension, life-threatening bleedings from the gastrointestinal system, and, finally, intrapulmonary hemorrhage. Although both reached severe aplasia of the bone marrow without blastic infiltration, death occurred with neutropenic sepsis. © 2015, Turkish Society of Hematology. All rights reserved.Öğe Imatinib treatment alone in philadelphia-positive acute lymphoblastic leukemia: Is it enough?(2011) Ay Y.; Yilmaz D.; Balkan C.; Karapinar B.; Karadas N.; Kavakli K.BCR-ABL fusion gene t(9;22)(q34;q11) occurs in only 3% of pediatric acute lymphoblastic leukemia (ALL) cases. Previously, less than 40% of Philadelphia-positive ALL patients were cured with intensive chemotherapy. The use of imatinib (340 mg/m2/day) added to an intensive chemotherapy regimen has improved the outcome in this population at 3 years to an event-free survival of 80%. Imatinib treatment alone was administered after remission induction chemotherapy to a patient with Philadelphia-positive ALL who presented with serious chemotherapy toxicity, so that intensive chemotherapy could not be maintained. This is the only patient in the literature who survived remission for more than 2.5 years with imatinib treatment only. Copyright © 2011 S. Karger AG, Basel.Öğe Immune haemolytic anaemia induced by allopurinol after liver transplantation [2](2006) Tumgor G.; Balkan C.; Arikan C.; Kavakli K.; Aydogdu S.[No abstract available]Öğe Incidence and clinical importance of lupus anticoagulant in children with recurrent upper respiratory tract infection(2011) Peker E.; Kavakli K.; Balkan C.; Karapinar D.; Aydemir B.Background: This study aims to understand the incidence and presence of lupus anticoagulant (LA) in children with recurrent upper respiratory tract infection (URTI). Methods: One hundred and sixty-five patients who were admitted to Departments of Pediatrics and Otolaryngology at Ege University Faculty of Medicine during the last 2 years and 120 age-matched healthy children as a control group were enrolled in the study. Results: The presence of LA in serum was positive in 8 (4.8%) cases in the patient group while only 2 (1.6%) cases in the healthy control group (P =.03). Mean age of patients with LA positive was significantly lower than those of negative cases (P =.02). Of the patients, 92 (55.8%) had adenoid hypertrophy. The annual frequency of URTI did not differ significantly between the LA patients and the LA-negative patients (7.5/year and 6.9/year, respectively). None of the patients with LA positive had adenoid hypertrophy (P =.009). Activated partial thromboplastin time was prolonged in 6 (3.6%) of 165 patients. Of these 6 patients, 2 were also LA positive. The presence of LA disappeared in all the 8 patients 2 months after the diagnosis. Lupus anticoagulant was found negative in all patients at the end of the second month. Conclusion: We found that the ratio of the presence of LA is higher in children with recurrent URTI than healthy children. However, the presence of LA does not lead to bleeding and/or thrombosis, and it disappears in a short period of time. © The Author(s) 2011.Öğe An infant with Imersland-Gräsbeck syndrome.(2012) Bulut I.K.; Mutlubas F.; Mir S.; Balkan C.The Imersland-Gräsbeck Syndrome (IGS) is a rare inherited disorder characterized by megaloblastic anemia due to a selective Vitamin B12 malabsorption in association with mild proteinuria. This syndrome can be diagnosed and treated easily. Herein, we describe an infant with IGS as a rare etiology of growth retardation with diarrhea, vomiting and therapy-resistant proteinuria.Öğe The influence of the ABO blood type on the distribution of von Willebrand factor in healthy children with no bleeding symptoms(2012) Akin M.; Balkan C.; Karapinar D.Y.; Kavakli K.The purpose of this study was to determine the effect of ABO blood groups on von Willebrand factor-ristocetin cofactor activity (vWF-RCo) and on vWF-antigen (vWF-Ag) in children who have no personal or familial history of bleeding. Material and methods: A survey and testing were performed on 200 children with no personal or familial history of bleeding. In all, 100 of them belonged to blood group O, and the remaining 100 belonged to other blood groups. The blood samples were stored at ?80°C for a maximum period of 2 weeks to detect vWF-RCo and vWF-Ag levels. Results: The mean vWF-Ag (±2 standard deviation [SD]) level in children with blood group O was 86% (±20%); and for those with non-O blood group, it was 98.8% (±25%). There was a significant difference between the 2 groups (P <.001). The mean vWF-RCo (±2 SD) level in children with blood group O was 89% (±23%); and for those with non-O blood group, it was 103% (±17%). There was a significant difference between those in the 2 groups (P <.001). The lowest value of vWF-Ag and vWF-RCo levels in children with blood group O was found to be 50%. In conclusion, we showed that the selection of normal ranges based on the ABO group might influence the clinical diagnosis of vWD and that while the approach of using ABO group ranges for a vWF-Ag level lower than 50 IU/dL is scientifically sound, it might not be useful to assist a clinician in identifying people at increased risk of bleeding. © 2012 The Author(s).Öğe Inhibitor screening for patients with hemophilia in Turkey(2006) Kavakli K.; Aktuglu G.; Kemahli S.; Başlar Z.; Ertem M.; Balkan C.; Ar C.; Yilmaz Karapinar D.; Bilenoglu B.; Gülseven M.; Gürman C.Development of factor VIII inhibitors remains the most serious and life-threatening complication of hemophilia therapy. The aim of this study was to determine the prevalence of inhibitor development in Turkish patients with hemophilia. Totally 1226 patients were screened [HA: 1057, HB: 105, von Willebrand's disease (vWD): 641. Ages ranged from 1 to 55 years (mean: 16.5 years). Sixty-two percent of patients (657/1057) were severe hemophilia. This study showed that inhibitor prevalence in Turkish hemophiliacs exposed to factor concentrates and fresh frozen plasma (FFP) is 11.2% for all HA and 15.8% for severe HA versus 1.9% for HB after eliminating transient inhibitors. Totally 122 patients were found inhibitor positive [high responder (HR) inhibitor= 60 and low responder (LR) inhibitor= 59 for HA/2 LR for HB/1 LR for vWD]. Thanks to this project, patients with inhibitor development can be treated with specific products such as recombinant factor VIIa or activated protrombin complex concentrates for their bleeding episodes or in their elective operations.Öğe Murine and math models for the level of stable mixed chimerism to cure ß-thalassemia by nonmyeloablative bone marrow transplantation(New York Academy of Sciences, 2005) Roberts C.; Kean L.; Archer D.; Balkan C.; Hsu L.L.Stable mixed chimeric stem cell transplantation in hemoglobinopathies exploits shorter erythroid survival in hemolytic anemias, providing normal donor red blood cells with a competitive survival advantage. This study examined the level of stable mixed chimerism necessary for complete hematological cure of the thalassemic phenotype, using a nonmyeloablative busulfan chemotherapeutic preparation. Thalassemic mice transplanted from congenic wild-type donors developed partial mixed chimerism. Hematologic cure required >80% donor red blood cells and only >13% donor white blood cells. Murine and human transplant results were compared with a math model for survival advantage of donor peripheral blood cells produced by steady-state chimeric marrow. © 2005 New York Academy of Sciences.Öğe Outcome of joint functions in hemophilic patients: A twelve year retrospective study(2010) Çapaci K.; Balkan C.; Yilmaz D.; Yilmaz A.Y.; Kirazli Y.; Durmaz B.; Kavakli K.In hemophilic patients, recurrent intra-articular bleeding results in hemophilic arthropathy. The frequently affected joints are knee, elbow, and ankle. Prevention of recurrent joint bleedings is of great significance in avoiding hemophilic arthropathy. Bleedings may occur as a result of minor traumas or spontaneously in severe hemophilic patients. In this study, data belonging to 31 hemophilic patients were analyzed after a 12-year retrospective observation. Joints of knee, elbow, and ankle were assessed with Gilbert score and swelling, atrophy, axial deformity, crepitus, range of motion, and flexion contracture were assessed in terms of instability. Patients were grouped based on the severity of hemophilia. Severe hemophiliacs were grouped depending on whether they received long term prophylaxis or on-demand therapy. As a conclusion, it was observed that joint assessment results of severe hemophiliacs who received on-demand therapy were rather poor compared to those of mild or moderate hemophiliacs. Joint assessment results obtained from hemophilia patients who received prophylaxis, on the other hand, were almost as good as those of mild hemophiliacs. Based on swelling, atrophy, axial deformity, crepitus, flexion contracture and loss of full range of motion, there was a significant difference between the on-demand and prophylaxis groups in severe hemophiliacs (p< 0.05). In the on-demand group there was a significant difference between the baseline and final evaluations based on all parameters (p< 0.05). Consequently, it was determined that prophylactic treatment has a crucial role in the prevention of arthropathy in severe hemophiliacs. Copyright © 2010 by Türk Tibbi Rehabilitasyon Kurumu Dernegi.Öğe Radioisotope synovectomy with rhenium186 in haemophilic synovitis for elbows, ankles and shoulders(2008) Kavakli K.; Aydogdu S.; Taner M.; Duman Y.; Balkan C.; Karapinar D.Y.; Saydam, G..; Capaci K.; Oktay A.We have performed 221 radioisotope synovectomy (RS) in more than 150 children and young adults with haemophilia, age ranging 3-30years (mean 15) in Ege Hemophilia Center, Izmir, Turkey for last 7years. We always preferred to use Yttrium 90 (Y90) for knees; however, since 2005, we started using rhenium 186 (Re186) for medium-sized joints with respect to safety. In this article, we have evaluated long-term experience ranging from 6months to 3years (mean 18months) with Re186 for elbows (n = 35), ankles (n = 26) and shoulders (n = 2) in total of 63 RS procedures for 49 patients. Their age range was 3-30 years and mean age was 15.5. Two mCi of Re186 intra-articularly injected for treating target joints and chronical synovitis. After RS, joint bleedings were decreased for all patients. The best results were obtained for all joints in patients with grade-II synovitis as like earlier experience with Y90. Excellent rates (no bleeding) were observed in grade-II synovitis in 81% and 46% for elbows vs. 86% and 57% for ankles after 6months and after 1 year follow-up of patients, respectively. In grade-III synovitis, excellent rates were 53% and 25% for elbows and 44% and 29% for ankles, respectively. In five joints for five patients, repeated injections were needed for better outcome. No adverse events such as radioisotope leakage, local inflamatory reactions or malignancy development were observed during and after RS. For medium-sized joints, RS with Re186 seems to be either effective or safe treatment method. Our results confirm those previously published by others on the value of Re186 synoviorthesis in medium-sized joints in haemophilia patients. After this experience, we changed our protocol and we use Re 186 for all medium-sized joints for treating chronical synovitis. © 2008 Blackwell Publishing Ltd.Öğe Rare coagulation disorders: A retrospective analysis of 156 patients in Turkey [Nadir koagülasyon bozukluklari{dotless}: Türkiye'deki 156 olgunun retrospektif i·ncelenmesi](2012) Fişgin T.; Balkan C.; Celkan T.; Kilinç Y.; Türker M.; Timur C.; Gürsel T.; Kürekçi E.; Duru F.; Küpesiz A.; Olcay L.; Yilmaz S.; Özgen U.; Ünüvar A.; Ören H.; Kavakli K.Objective: To retrospectively evaluate the clinical findings, laboratory data, management, and outcome in a group of Turkish children diagnosed with rare coagulation deficiencies (RCDs) between January 1999 and June 2009. Material and Methods: The Turkish Society of Pediatric Hematology-Hemophilia-Thrombosis-Hemostasis subcommittee designed a Microsoft Excel-based questionnaire for standardized data collection and sent it to participating institutions. Results: In total, 156 patients from 12 pediatric referral centers were included in the study. The cost common RCDs were as follows: FVII (n = 53 [34%]), FV (n = 24 [15.4%]), and FX (n = 23 [14.7%]) deficiency. The most common initial finding in the patients was epistaxis, followed by ecchymosis, and gingival bleeding. Conclusion: Initial symptoms were mucosal bleeding, and fresh frozen plasma (FFP) and tranexamic acid were the most commonly used treatments. We think that prophylactic treatment used for hemophilia patients should be considered as an initial therapeutic option for patients with rare factor deficiencies and a severe clinical course, and for those with a factor deficiency that can lead to severe bleeding.Öğe Relation between NT-proBNP levels, iron overload, and early stage of myocardial dysfunction in ß-thalassemia major patients(2012) Balkan C.; Tuluce S.Y.; Basol G.; Tuluce K.; Ay Y.; Karapinar D.Y.; Gurgun C.; Bayindir O.; Kavakli K.Background: Heart failure (HF) secondary to myocardial iron loading remains the leading cause of death in ß-thalassemia major (ß-TM) patients. The early diagnosis and treatment of HF in these patients is related to survival. We aimed to evaluate myocardial performance using conventional and tissue Doppler echocardiography and its relation to plasma NT-proBNP levels and iron overload indices in ß-TM patients with preserved systolic function. Methods: The study population included 49 ß-TM patients (24.0 ± 4.2 years) and 48 age-matched healthy controls. Doppler-echocardiographic study was performed and blood samples for NT-proBNP measurements were drawn on the third day following blood transfusion. Patients were divided as group-1, without diastolic dysfunction: E/E' ratio < 9 and group-2, with suspected diastolic dysfunction: E/E' ratio ? 9. Results: NT-proBNP levels and E/E' ratio were increased in patients compared with controls (P < 0.001 and P < 0.001) but did not correlate with each other. A strong positive correlation was detected between NT-proBNP levels and mean ferritin levels in ß-TM patients (r s= 0.939; P < 0.001). Median NT-proBNP levels were significantly higher in group-1 in comparison to controls [51.2 (41.51-113.5) vs 30.1 (17.97-68.16) ng/mL, P < 0.01]. NT-proBNP levels were also increased in group-2 in comparison to group-1 but this increase was not statistically significant. Conclusion: NT-proBNP secretion begins in the early phase of the disease before the increase in diastolic pressure becomes overt. While there was a strong correlation between the plasma NT-proBNP levels and iron overload, there was no correlation between NT-proBNP levels and diastolic dysfunction parameters in patients in the third decade of life. © 2011 Wiley Periodicals, Inc.
