Alterations of panoramic radiomorphometric indices in children and adolescents with beta-thalassemia major:a fractal analysis study

Beta-thalassemia major is an inherited disorder that can cause bone deformity and loss of bone mineral density. The objective of this study is to evaluate the cortical and trabecular mandibular bone morphology of children and adolescents who have beta-thalassemia major (ß-TM) using a fractal dimension (FD) analysis and different panoramic radiomorphometric indices with digital panoramic radiographic images (DPRIs). The study included 80 patients (with 40 patients each of ß-TM and control). The mandibular cortical width (MCW), panoramic mandibular index (PMI), mandibular cortical index (MCI), and simple visual estimation (SVE) were evaluated, and an FD analysis of five regions of interest (ROIs) (ROI 1: in basal cortical bone; ROI 2: in premolar region; ROI 3: in molar region; ROI 4: in angulus mandible and ROI 5: in condyle region) was obtained in all DPRIs. Quantitative variables were analyzed using the student?s t-test , Kruskal?Wallis and Mann-Whitney U tests. When the ß-TM groups were compared with controls, there were no statistically significant differences found in the mean FD values, the ROIs of the trabecular bone, or the SVE. There was a significant correlation in the mean MCW, PMI, ROI of cortical bone (ROI 1), and MCI between ß-TM and control groups (p < 0.001, p < 0.001, p = 0.047, and p = 0.046, respectively). The mean MCW values correlated with the SVE in both the ß-TM and control groups (p = 0.031 and p < 0.001, respectively). While the mean MCW values correlated with the MCI (p = 0.04) in the control group, the mean MCW values were not correlated with the MCI (p = 0.493) in ß-TM group. The current study revealed lower MCW and PMI values in the ß-TM group. While the mean FD values of trabecular bone is similar to the control groups, the mean FD value is lower in cortical bone in the ß-TM group. MCW, PMI, FD of cortical bone and MCI may be key indicators in individuals with beta-thalassemia major

Influence of Paroxysmal Nocturnal Hemoglobinuria Clone Positivity on Outcome of Childhood Acquired Aplastic Anemia: A Multicenter Center Study

60th Annual Meeting of the American-Society-of-Hematology (ASH) -- DEC 01-04, 2018 -- San Diego, CAWOS: 000454842805371Amer Soc Hemato

Risks and outcomes of invasive fungal infections in pediatric allogeneic hematopoietic stem cell transplant recipients receiving fluconazole prophylaxis: a multicenter cohort study by the Turkish Pediatric Bone Marrow Transplantation Study Group

###EgeUn###Invasive fungal infections (IFIs) are a major cause of infection-related morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT). Data from pediatric settings are scarce. To determine the incidence, risk factors and outcomes of IFIs in a 180-day period post-transplantation, 408 pediatric patients who underwent allogeneic HSCT were retrospectively analyzed. The study included only proven and probable IFIs. The cumulative incidences of IFI were 2.7%, 5.0%, and 6.5% at 30, 100, and 180 days post-transplantation, respectively. According to the multivariate analysis, the factors associated with increased IFI risk in the 180-day period post-HSCT were previous HSCT history (hazard ratio [HR], 4.57; 95% confidence interval [CI] 1.42-14.71; P =.011), use of anti-thymocyte globulin (ATG) (HR, 2.94; 95% CI 1.27-6.80; P =.012), grade III-IV acute graft-versus-host-disease (GVHD) (HR, 2.91; 95% CI 1.24-6.80; P =.014) and late or no lymphocyte engraftment (HR, 2.71; 95% CI 1.30-5.62; P =.007). CMV reactivation was marginally associated with an increased risk of IFI development (HR, 1.91; 95% CI 0.97-3.74; P =.063). IFI-related mortality was 1.5%, and case fatality rate was 27.0%. The close monitoring of IFIs in pediatric patients with severe acute GVHD who receive ATG during conditioning is critical to reduce morbidity and mortality after allogeneic HSCT, particularly among those with prior HSCT and no or late lymphocyte engraftment

Evaluation Of The Achievement Of Hematologists To Transfusion Medicine Education With Self-Assessment Questionnaire In Turkey

Background: Proper clinical use of blood andblood products requires competent theoreticaland practical knowledge of transfusion medicine.The Curriculum Development and StandardDetermination System Medical SpecializationBoard is prepared Hematology SpecialistEducation Core Curriculum in Turkey. In thisstudy, we aimed to determine the access ofhematologists to the learning objectives definedby curriculum for the transfusion medicine andthe factors affecting it.Methods: Hematologists who have beenmembers of Turkish Hematology Society since2013 have been included in the study, Thesurvey questions were prepared based on thecurriculum for transfusion medicine. The studywas applied to hematologists with “surveymonkey” application. The questionnaireconsisted of a competence self-assessment withLikert scale and theoretical multiple-choiceknowledge questions.Results: Of the 213 hematologists, 54 (25%)were included in the study. Hematologists ratedtheir competences in the clinical competenceareas as 3,65 ± 0,73 (median 3,60) as “I knowbut not t a sufficient level”. The participants‘perception of competence was “I know, butnot at a sufficient level’” with an average of3.31 ± 0.84 (median3.5) in the blood bankingfield, while the average in hemapheresis andtransfusion medicine was 4.04 ± 0.63 (median4) as “enough”. In interventional procedures,hematologists stated that their vocationalcompetences were 2,79± 0,92 (median 2,93)on average as “I have an idea- I know, but notenough”. The correct answer to 13 theoreticalquestions was an average of 6,96 ± 1,89(median 7). Hematologists performing bloodrotation felt significantly more competent thanthe physicians who could not do the rotation inthe blood bank, blood banking t(52) = -3.9, p .001 , transfusion medicine and interventionalcompetence t(52) = -2.2, p = .04 . Physicianswho believed that they are sufficient in theblood banking area, were more confident intransfusion medicine r(54) = .67, p .001 andmanaging interventional procedures r(54) =.85, p .001.Conclusion: In this study, hematologistsgenerally felt more competent in subjects suchas transfusion and therapeutic apheresis,which they often think of as not having enoughknowledge in the area of blood banking.Hematologists have been more confident inthe field of transfusion medicine as their yearsof expertise increased, but they did not feelbetter equipped in the fields of blood bankingand interventional competence. The currentresults suggested that hematologists who areexpected to be the blood bank supervisors do notinternalize the area of blood banking, are notstrong in their competence, and do not want towork in this area unless they are required.In hematology education curriculum, positiverevisions in education can be achieved byrevising blood banking curriculum and learningobjectives, standardizing blood center rotationswith content and duration, and support fromonline distance education programs

Thalassemia-free and graft-versus-host-free survival: outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience

We report the national data on the outcomes of hematopoietic stem cell transplantation (HSCT) for thalassemia major (TM) patients in Turkey on behalf of the Turkish Pediatric Stem Cell Transplantation Group. We retrospectively enrolled 1469 patients with TM who underwent their first HSCT between 1988 and 2020 in 25 pediatric centers in Turkey. The median follow-up duration and transplant ages were 62 months and 7 years, respectively; 113 patients had chronic graft versus host disease (cGVHD) and the cGVHD rate was 8.3% in surviving patients. Upon the last visit, 30 patients still had cGvHD (2.2%). The 5-year overall survival (OS), thalassemia-free survival (TFS) and thalassemia-GVHD-free survival (TGFS) rates were 92.3%, 82.1%, and 80.8%, respectively. cGVHD incidence was significantly lower in the mixed chimerism (MC) group compared to the complete chimerism (CC) group (p < 0.001). In survival analysis, OS, TFS, and TGFS rates were significantly higher for transplants after 2010. TFS and TGFS rates were better for patients under 7 years and at centers that had performed over 100 thalassemia transplants. Transplants from matched unrelated donors had significantly higher TFS rates. We recommend HSCT before 7 years old in thalassemia patients who have a matched donor for improved outcomes