ARZU AKYAY - Academia.edu (original) (raw)

Papers by ARZU AKYAY

JAMA network open, May 23, 2023

IMPORTANCE Cancer was a common noncommunicable disease in Syria before the present conflict and i... more IMPORTANCE Cancer was a common noncommunicable disease in Syria before the present conflict and is now a major disease burden among 3.6 million Syrian refugees in Turkey. Data to inform health care practice are needed. OBJECTIVE To explore sociodemographic characteristics, clinical characteristics, and treatment outcomes of Syrian patients with cancer residing in the southern border provinces of Turkey hosting more than 50% of refugees. DESIGN, SETTING, AND PARTICIPANTS This was a retrospective hospital-based cross-sectional study. The study sample consisted of all adult and children Syrian refugees diagnosed and/or treated

Hematology, Transfusion and Cell Therapy, Oct 1, 2020

hematol transfus cell ther. 2 0 2 0;4 2(S 1):S17-S34 S31 components of red cell membranes. It is ... more hematol transfus cell ther. 2 0 2 0;4 2(S 1):S17-S34 S31 components of red cell membranes. It is characterized by anemia, jaundice, splenomegaly and cholelithiasis. The clinical manifestations vary widely, ranging from nearly asymptomatic to transfusion-dependent or severe life-threatening anemia. It is difficult to identify atypical cases with classical approaches. The known HS gene mutations are SPTA1 gene, SPTB gene, ANK1 gene, SLC4A1 gene and EPB42 gene. In this report, the next-generation sequencing (NGS) was used to analyze our patients with HS and we identified mutations responsible for HS. Methodology: Patients who were diagnosed with hereditary spherocytosis with osmotic fragility testing between 2007-2019; ten were further tested for molecular background. Diagnosed in our center were analyzed retrospectively. Either NGS or ANK1 Sanger testing were used. Results: The 10 cases of HS comprised 8 males and 2 females. The age of patients ranged from 5 months to 17 years. Hemolytic anemia, jaundice and splenomegaly were the most common findings in our cases. Gallstones were detected in four patients (40%). The family history was positive in 5 (50%) patients. Splenectomy and cholecystectomy was performed in two cases and three cases, respectively. The results corfirmed ANK1 gene mutation in 50%; SPTB gene mutation in 20%, EBP42 gene mutation in 10%; SPTA1 gene mutation in 10%. The clinical features of the patients are summarized in the Table 1. Table 1. Patient Age Sex Age of diagnosis Family history Splenomegaly Gallstone Splenectomy/Cholecystectomy Mutated gene 1 1 Female 1 year Yes + − −/− SPTB 2 10 Female 10 years Yes + − +/− ANK1 3 12 Female 7 years Yes + + +/+ ANK1 4 12 Female 2 years Yes − + −/+ ANK1 5 10 Male 6 years No − + −/− ABCG8 6 2 Female 5 months No + − −/− ANK1 7 13 Female 15 years Yes + − −/− ANK1 8 8 Female 7 years No + + −/+ EBP42 9 2.5 Male 2 years No + − −/− SPTB 10 19 Female 17 years No + − −/− SPTA1. Conclusion: Consistent with the literature, the most common gene mutated was ANK1. Collectively, our results suggest that mutation analyses will complement other conventional tests for accurate diagnosis of HS, especially in those who are under transfusion programme and are followed with a diagnosis of unspecified hemolytic anemia.

Pediatric Transplantation, Oct 7, 2022

Purpose of review This review describes the current use of hematopoietic stem cell transplantatio... more Purpose of review This review describes the current use of hematopoietic stem cell transplantation (HSCT) and aims to assess recent trends, to analyze factors associated with use and trends, and to discuss potential implications of such developments on future use. Recent findings The one millionth HSCT worldwide was announced in early 2013. More than 35 000 HSCT (40% allogeneic and 60% autologous) were reported in 2011 to the European Group for Blood and Marrow Transplantation, with more than 60 000 worldwide. Total numbers increased in Europe over the last 10 years by 50%, with the highest increase being for allogeneic HSCT from an unrelated donor. Change in HSCT numbers was different for each indication with novel indications emerging. There were significant differences in absolute numbers and increase in transplant rates between countries concerning main indication, donor type, and stem cell source or transplant technology. Trends showed a widening gap between countries with high or low national income. The high costs of HSCT impact on its use; availability of resources, presence of a national registry, and numbers of registered donors are the most closely associated with unrelated donor transplant rates.

Scandinavian Journal of Clinical and Laboratory Investigation, 2021

Abstract Introduction: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inher... more Abstract Introduction: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inherited rare bleeding disorders. Other determinants of clinical severity apart from FVII level (FVIIL) include genetic and environmental factors. We aimed to identify the cut-off FVIILs for general and severe bleedings in patients with FVIID by using an online national registry system including clinical, laboratory, and demographic characteristics of patients. Methods: Demographic, clinical, and laboratory data of patients with FVIID extracted from the national database, constituted by the Turkish Society of Hematology, were examined. Bleeding phenotypes, general characteristics, and laboratory features were assessed in terms of FVIILs. Bleeding rates and prophylaxis during special procedures/interventions were also recorded. Results: Data from 197 patients showed that 46.2% of patients had FVIIL< 10%. Most bleeds were of mucosal origin (67.7%), and severe bleeds tended to occur in younger patients (median age: 15 (IQR:6-29)). Cut-off FVIILs for all and severe bleeds were 16.5% and 7.5%, respectively. The major reason for long-term prophylaxis was observed as central nervous system bleeding (80%). Conclusion: Our data are consistent with most of the published literature in terms of cut-off FVIIL for bleeding, as well as reasons for prophylaxis, showing both an increased severity of bleeding and younger age at diagnosis with decreasing FVIIL. However, in order to offer a classification similar to that in Hemophilia A or B, data of a larger cohort with information about environmental and genetic factors are required.

Hematology, Transfusion and Cell Therapy, Oct 1, 2020

hematol transfus cell ther. 2 0 2 0;4 2(S 1):S35-S78 more effective diagnosis of patients with su... more hematol transfus cell ther. 2 0 2 0;4 2(S 1):S35-S78 more effective diagnosis of patients with suspected CDA and congenital hemolytic anemia.

Indian Journal of Pediatrics, May 5, 2023

Pediatric Blood & Cancer

BackgroundIn patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increa... more BackgroundIn patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increases due to hemostatic changes secondary to the primary disease and due to treatment‐related factors. In this multicenter study, we aimed to research the frequency of central nervous system (CNS) thrombosis occurring during treatment, hereditary and acquired risk factors, clinical and laboratory features of patients with thrombosis, treatment approaches, and thrombosis‐related mortality and morbidity rates in pediatric ALL patients.ProcedurePediatric patients who developed CNS thrombosis during ALL treatment from 2010 to 2021 were analyzed retrospectively in 25 different Pediatric Hematology Oncology centers in Türkiye. The demographic characteristics of the patients, symptoms associated with thrombosis, the stage of the leukemia treatment during thrombosis, the anticoagulant therapy applied for thrombosis, and the final status of the patients recorded through electronic medical records w...

Indian Journal of Pediatrics

Turkiye Klinikleri Journal of Pediatrics, 2013

of hematological malignancies. Particularly, when ARA-C is given in high doses, it provokes vario... more of hematological malignancies. Particularly, when ARA-C is given in high doses, it provokes various adverse effects, including fever, severe myelosupression, infection and toxic reactions involving the central nervous system, liver, gastrointestinal tract, eyes and skin. Dermatologic side effects of cytosine arabinoside therapy are often described as rare. Nevertheless, there are differences in the observed frequency of toxic skin events ranging from 2% to 53%. Moreover, idarubicine and etoposide are very rarely associated with adverse skin reactions.1,2

Neurophysiologie Clinique, 2021

OBJECTIVES In children, cancer chemotherapy may impair the functioning of the cardiac autonomic n... more OBJECTIVES In children, cancer chemotherapy may impair the functioning of the cardiac autonomic nervous system. Moreover, it is not known whether there are any differences between intensive and maintenance phases of chemotherapy. Therefore, the aim of the current study was to assess autonomic nervous system activity using heart rate variability, in children receiving intensive or maintenance cancer chemotherapy. METHODS For that purpose, children who were healthy (healthy control, n = 30), receiving intensive chemotherapy (chemotherapy, n = 30), and receiving maintenance chemotherapy (maintenance, n = 25) were included in the study. Autonomic nervous system activity was measured by means of heart rate variability. Electrocardiogram recordings were used to calculate time- and frequency-domain heart rate variability parameters. RESULTS Time-domain parameters such as standard deviation of NN intervals and frequency-domain parameters such as total power were lower during the intensive chemotherapy but not during maintenance phase (standard deviation of NN intervals: 50±5, 33±3, and 48±3 ms, and total power: 2613±504, 1379±296 and 2295±264 ms2, respectively for healthy control, chemotherapy and maintenance groups, P<0.001 for both standard deviation of NN intervals and total power). DISCUSSION The present results indicate that intensive chemotherapy perturbs the function of heart rate variability in children, with recovery during the maintenance phase. This suggests that intensive chemotherapy is likely to affect the autonomic nervous system but this effect does not appear to be permanent.

Turkish Journal of Hematology, 2020

Trends in Pediatrics, 2022

Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disease characterized by bone marr... more Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disease characterized by bone marrow dysfunction, exocrine pancreatic insufficiency and skeletal abnormalities. Persistent or intermittent neutropenia caused by bone marrow hypoplasia is the most common hematological abnormality in SDS. It can be difficult to diagnose the disease that usually occurs in early childhood. SDS should be kept in mind in the differential diagnosis of neutropenic patients. If the signs of pancreatic insufficiency are not observed, the diagnosis may be missed. The article wanted to present a patient with pancreatic insufficiency and SDS with the biallelic mutation who presented with neutropenia in a newborn.

Bone Marrow Transplantation, 2022

We report the national data on the outcomes of hematopoietic stem cell transplantation (HSCT) for... more 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.

Journal of Turgut Ozal Medical Center, 2015

Objectives: Metamizole sodium (Novalgin ® ) is an effective, widely used analgesic and antipyreti... more Objectives: Metamizole sodium (Novalgin ® ) is an effective, widely used analgesic and antipyretic agent in several countries. In spite of its availability and common usage, it has serious and potentially fatal adverse effects like agranulocytosis and aplastic anemia. Objectives of this study are to evaluate incidence, severity, and clinical significance of metamizole related neutropenia and agranulocytosis, and to study recovery duration of neutropenia and agranulocytosis in children exposed to metamizole. Material and Methods: Thirteen cases of neutropenia and/or agranulocytosis secondary to metamizole usage have been evaluated retrospectively. Duration and cumulative doses of metamizole, degree of neutropenia, recovery from neutropenia, and other complications related to neutropenia were evaluated. Results: Overall, thirteen granulocytopenic patients were recorded for 12 months after metamizol usage. Mean metamizole exposure duration was 4,64 (2-7) days; mean daily total metamizo...

Journal of Paediatrics and Child Health, 2021

The aim of this study is to investigate the effects of vitamin B12 deficiency on hearing in schoo... more The aim of this study is to investigate the effects of vitamin B12 deficiency on hearing in school‐aged children by pure‐tone audiometry.

Journal of Pediatric Nursing, 2021

Background: Caregivers of hemodialysis patients endure a significant caring pressure as a result ... more Background: Caregivers of hemodialysis patients endure a significant caring pressure as a result of caring for patients with chronic illness, which can affect their quality of life. Disruptions in the quality of life of these caregivers impose double pressure on them and disrupt the care process. Therefore, the present study aimed to determine the level of care burden and its relationship with quality of life of caregivers of hemodialysis patients. Methods: In this descriptive-analytical study, 246 caregivers of hemodialysis patients were enrolled by census method, so that, all patients and caregivers who attended the study environment at morning, evening and night participated in the study. The study tool was a three-part questionnaire, which included personal information, Novak & Guest Care burden Questionnaire, and WHOQOL-BREF Quality of Life Questionnaire. Data were analyzed by descriptive, statistical and inferential tests. Results: In total, 37.4% of caregivers were experiencing high and very high levels of care burden and 42.7% of them were experiencing a moderate level of care burden. The mean and standard deviation of the quality of life of caregivers was 76.27 ± 13.67 out of 130. There was a significant and negative correlation between the total scores of care burden and quality of life (r = − 0. 436, P < 0.001). The factors influencing care burden included variables such as; level of patient's caring capability, the patient's incidence of other chronic diseases, and the age of the caregiver. So that, in case of reduced patient's capability in self-care, the patient's incidence of other chronic diseases, and the increased age of the caregiver, the level of care burden on the caregivers would be increased. Conclusions: The caregivers of hemodialysis patients endure high level of care burden and this pressure has a negative effect on their quality of life. Therefore, it is recommended to pay more attention to the needs of caregivers and provide adequate social, economic, physical and psychological support for them.

Turkish Journal of Hematology, 2021

Patients with solid malignancies are more vulnerable to SARS-CoV-2 infection than healthy populat... more Patients with solid malignancies are more vulnerable to SARS-CoV-2 infection than healthy population. The outcome of SARS-CoV-2 infection in highly immunosupressed populations, such as in patients with hematological malignancies is a point of interest.We aimed to analyze the symptoms, complications, intensive care unit (ICU) admission and mortality rates of patients with hematological malignancies infected with SARS-CoV-2 in Turkey. In this multicenter study, we included 340 adult and pediatric patients diagnosed as COVID-19 from March to November. Forty four (12.9%) patients were asymptomatic at diagnosis. In symptomatic patients, fever, cough and dyspnea were observed in 62.6%, 48.8 % and 41.8% of the patients, respectively. Sixty nine (20%) patients had mild SARS-Cov-2 disease, whereas moderate, severe and critical diseases were reported in 101 (29%), 71 (20%) and 55 (16%) patients, respectively. Two hundered fifty one (73.8%) patients were hospitalized for SARS-Cov2. Mortality related to SARS-CoV-2 infection was 26.5% in all cohort; 4.4% in those patients with mild disease, 12.4% in moderate disease and 83% in severe and critical disease. Active hematological disease, lower life expectancy related with primary hematological disease, neutropenia at diagnosis of SARS-CoV-2, ICU admission and firstline therapy used for COVID 19 treatment were found to be related to higher mortality rates. Treatments with hydroxychloroquine alone or in combination with azitromycin were associated with a higher rate of mortality in comparison with favipiravir use.In conclusion, patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.

Transfusion and Apheresis Science, 2021

Thalassemia major (TM) is a severe transfusion-dependent anemia. Regular erytrocyte transfusion i... more Thalassemia major (TM) is a severe transfusion-dependent anemia. Regular erytrocyte transfusion is required for the treatment of thalassemia patients. However, repeated transfusion may result in impairements in heart function. In this study, we aimed to investigate short-term effects of erythrocyte transfusion on autonomic control of heart in children with thalassemia major. For that purpose heart rate variability (HRV), which is a non-invasive method used to evaluate the effects of the autonomic nervous system on the heart rhythym, was measured before and after erythrocyte transfusion and compared to the healthy controls. Children diagnosed with TM (n = 17) and sex and age matched healthy children (HC, n = 30) were included in the study. HRV values of TM patients were measured 5 min before erythrocyte transfusion (BET, n = 17) and 5 min after erythrocyte transfusion (AET, n = 17). Parameters of time-domain and frequency-domain of HRV were evaluated in all participants. Heart rate (HR) was higher in the BET than AET (P = 0.002) but there was no difference between AET and HC groups (P > 0.05). HRV parameters were lower in BET than AET (P < 0.05) but there were no statistical difference between AET and HC (P> 0.05). The data suggest that, in thalassemia major patients, erythrocyte transfusion restores HR and HRV parameters to the levels observed in healthy controls and, thus, in short-terms, appears to be beneficial for the autonomic control of the heart.

Pediatric Blood & Cancer, 2021

To the Editor: Adultswith cancer are reported tohave ahigher risk for coronavirus disease (COVID-... more To the Editor: Adultswith cancer are reported tohave ahigher risk for coronavirus disease (COVID-19) infection and more severe disease and mortality than the general population.1,2 Although children seem to be at a lower risk for COVID-19 than adults,3–5 data specifically addressing children with cancer are limited.6–12 Weconducted a retrospective,multicenter, cross-sectional studyon behalf of the Turkish Pediatric Hematology Society (TPHD) and Turkish Pediatric Oncology Group (TPOG) Society to analyze the characteristics of COVID-19 in all patients with cancer and stem cell transplant (SCT) recipients in all centers in Turkey, during March 11-May 31, 2020. Approval for the study was obtained by Turkish Ministry of Health (MoH), Istanbul University COVID Scientific Research Committee, and Istanbul University Ethics Committee. The study was carried out through the analysis of a questionnaire with 62 questions, which was sent to all members of the TPOG and TPHD Societies working in all 66 pediatric hematology/oncology departments in university, state, and private hospitals in Turkey. All replied and 53 patients were reported from the 24 centers. Following the national recommendations and guidelines of the MoH,13,14 centers tested all symptomatic patients or patients with contact history or patients who were planned to undergo transplantation or surgery. All patients and caregivers were questioned at the entrance of the hospital/oncology center and if there were any symptoms or contact history they were sent to the special clinics within the hospital that were allocated for suspected/proven COVID-19 patients. If a patient was suspected of having COVID-19 and found positive while in the oncology clinic, she/hewas transferred to theCOVID clinic and all staff, patients, and accompanying persons with whom she/he was in contact were tested for COVID-19. Samples from the naso-oropharyngeal swabs were tested for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by polymerase chain reaction (PCR). Confirmed cases were defined as PCR-positive patients. Probable cases were defined according to guidelines of World Health Organization15 and MoH,13 as those who had typical symptoms and chest CT findings, or who had typical symptoms and contact history but negative PCR. Patients were classified into four groups regarding the severity of infection as asymptomatic/mild, moderate, severe, and critical on the basis of the clinical, laboratory, and radiological features.3,15 Patients were treated according to recommendations of the MoH.13,14 Statistical analyses were performed by IBM SPSS Statistics version 21.0. For comparison of mean of numerical variables Mann-Whitney U test was used. Categorical nominal variableswere comparedwith Fisher’s exact tests. There were 51 children with cancer, six of whom (four leukemia/lymphomas, two solid tumors) had undergone SCT (Table 1). The median age was 6 (0.3-17.8) years and 64.7% of the patients were male. Additional two patients with thalassemia major who underwent SCT were not included in the analyses. Chemotherapy courses were interrupted in 32 (62.7%) patients and delayed with a median of 15 (3-45) days. The most common presenting signs were fever and cough, while 37.25% of patients were asymptomatic. COVID-19 pneumonia was detected in 26 (50.9%) patients. Five of them had hypoxemia. Gastrointestinal system (17.6%) was the second most commonly involved site followed by central nervous, musculoskeletal systems and skin. Twenty-five patients had asymptomatic/mild, 17 patients moderate/severe, and nine patients critical disease. Thirty-eight patients were hospitalized and treated according to the severity of illness, six of whom were already hospitalized for reasons such as surgery or diagnostic workup when diagnosed with COVID-19. Nine patients with critical disease were in the intensive care unit (ICU) and three were intubated. Treatment consisted of hydroxychloroquine, azitromycine, antivirals either as a single agent or in combination (Table 1). Convalescent plasma was used in three patients, one of whom additionally received mesenchymal stem cell, tocizulumab, and granulocyte transfusions and was intubated. At the time of COVID-19 diagnosis, 26 patients had neutropenia and among them 15 had fever. In all patients with febrile neutropenia, broad-spectrum empirical antibiotics were initiated. In addition, 12 patients received antimicrobial therapy due to clinically and/or microbiologically documented infections. All patients, but one, fully recovered and the PCR tests became negative at a median of 7 (2-17) days. The patient who had received allogeneic SCT for relapsed leukemia/lymphoma and had progressive disease and fungal infection died due to COVID-19 infection. The incidence of critical care disease and need for ICU care were found tobehigher inpatientswithhematologicmalignancies (P= .012), patients post SCT (P = .001), patients with other infections (P = .005), and…

Turkiye Klinikleri Journal of Case Reports, 2021

JAMA network open, May 23, 2023

IMPORTANCE Cancer was a common noncommunicable disease in Syria before the present conflict and i... more IMPORTANCE Cancer was a common noncommunicable disease in Syria before the present conflict and is now a major disease burden among 3.6 million Syrian refugees in Turkey. Data to inform health care practice are needed. OBJECTIVE To explore sociodemographic characteristics, clinical characteristics, and treatment outcomes of Syrian patients with cancer residing in the southern border provinces of Turkey hosting more than 50% of refugees. DESIGN, SETTING, AND PARTICIPANTS This was a retrospective hospital-based cross-sectional study. The study sample consisted of all adult and children Syrian refugees diagnosed and/or treated

Hematology, Transfusion and Cell Therapy, Oct 1, 2020

hematol transfus cell ther. 2 0 2 0;4 2(S 1):S17-S34 S31 components of red cell membranes. It is ... more hematol transfus cell ther. 2 0 2 0;4 2(S 1):S17-S34 S31 components of red cell membranes. It is characterized by anemia, jaundice, splenomegaly and cholelithiasis. The clinical manifestations vary widely, ranging from nearly asymptomatic to transfusion-dependent or severe life-threatening anemia. It is difficult to identify atypical cases with classical approaches. The known HS gene mutations are SPTA1 gene, SPTB gene, ANK1 gene, SLC4A1 gene and EPB42 gene. In this report, the next-generation sequencing (NGS) was used to analyze our patients with HS and we identified mutations responsible for HS. Methodology: Patients who were diagnosed with hereditary spherocytosis with osmotic fragility testing between 2007-2019; ten were further tested for molecular background. Diagnosed in our center were analyzed retrospectively. Either NGS or ANK1 Sanger testing were used. Results: The 10 cases of HS comprised 8 males and 2 females. The age of patients ranged from 5 months to 17 years. Hemolytic anemia, jaundice and splenomegaly were the most common findings in our cases. Gallstones were detected in four patients (40%). The family history was positive in 5 (50%) patients. Splenectomy and cholecystectomy was performed in two cases and three cases, respectively. The results corfirmed ANK1 gene mutation in 50%; SPTB gene mutation in 20%, EBP42 gene mutation in 10%; SPTA1 gene mutation in 10%. The clinical features of the patients are summarized in the Table 1. Table 1. Patient Age Sex Age of diagnosis Family history Splenomegaly Gallstone Splenectomy/Cholecystectomy Mutated gene 1 1 Female 1 year Yes + − −/− SPTB 2 10 Female 10 years Yes + − +/− ANK1 3 12 Female 7 years Yes + + +/+ ANK1 4 12 Female 2 years Yes − + −/+ ANK1 5 10 Male 6 years No − + −/− ABCG8 6 2 Female 5 months No + − −/− ANK1 7 13 Female 15 years Yes + − −/− ANK1 8 8 Female 7 years No + + −/+ EBP42 9 2.5 Male 2 years No + − −/− SPTB 10 19 Female 17 years No + − −/− SPTA1. Conclusion: Consistent with the literature, the most common gene mutated was ANK1. Collectively, our results suggest that mutation analyses will complement other conventional tests for accurate diagnosis of HS, especially in those who are under transfusion programme and are followed with a diagnosis of unspecified hemolytic anemia.

Pediatric Transplantation, Oct 7, 2022

Purpose of review This review describes the current use of hematopoietic stem cell transplantatio... more Purpose of review This review describes the current use of hematopoietic stem cell transplantation (HSCT) and aims to assess recent trends, to analyze factors associated with use and trends, and to discuss potential implications of such developments on future use. Recent findings The one millionth HSCT worldwide was announced in early 2013. More than 35 000 HSCT (40% allogeneic and 60% autologous) were reported in 2011 to the European Group for Blood and Marrow Transplantation, with more than 60 000 worldwide. Total numbers increased in Europe over the last 10 years by 50%, with the highest increase being for allogeneic HSCT from an unrelated donor. Change in HSCT numbers was different for each indication with novel indications emerging. There were significant differences in absolute numbers and increase in transplant rates between countries concerning main indication, donor type, and stem cell source or transplant technology. Trends showed a widening gap between countries with high or low national income. The high costs of HSCT impact on its use; availability of resources, presence of a national registry, and numbers of registered donors are the most closely associated with unrelated donor transplant rates.

Scandinavian Journal of Clinical and Laboratory Investigation, 2021

Abstract Introduction: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inher... more Abstract Introduction: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inherited rare bleeding disorders. Other determinants of clinical severity apart from FVII level (FVIIL) include genetic and environmental factors. We aimed to identify the cut-off FVIILs for general and severe bleedings in patients with FVIID by using an online national registry system including clinical, laboratory, and demographic characteristics of patients. Methods: Demographic, clinical, and laboratory data of patients with FVIID extracted from the national database, constituted by the Turkish Society of Hematology, were examined. Bleeding phenotypes, general characteristics, and laboratory features were assessed in terms of FVIILs. Bleeding rates and prophylaxis during special procedures/interventions were also recorded. Results: Data from 197 patients showed that 46.2% of patients had FVIIL< 10%. Most bleeds were of mucosal origin (67.7%), and severe bleeds tended to occur in younger patients (median age: 15 (IQR:6-29)). Cut-off FVIILs for all and severe bleeds were 16.5% and 7.5%, respectively. The major reason for long-term prophylaxis was observed as central nervous system bleeding (80%). Conclusion: Our data are consistent with most of the published literature in terms of cut-off FVIIL for bleeding, as well as reasons for prophylaxis, showing both an increased severity of bleeding and younger age at diagnosis with decreasing FVIIL. However, in order to offer a classification similar to that in Hemophilia A or B, data of a larger cohort with information about environmental and genetic factors are required.

Hematology, Transfusion and Cell Therapy, Oct 1, 2020

hematol transfus cell ther. 2 0 2 0;4 2(S 1):S35-S78 more effective diagnosis of patients with su... more hematol transfus cell ther. 2 0 2 0;4 2(S 1):S35-S78 more effective diagnosis of patients with suspected CDA and congenital hemolytic anemia.

Indian Journal of Pediatrics, May 5, 2023

Pediatric Blood & Cancer

BackgroundIn patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increa... more BackgroundIn patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increases due to hemostatic changes secondary to the primary disease and due to treatment‐related factors. In this multicenter study, we aimed to research the frequency of central nervous system (CNS) thrombosis occurring during treatment, hereditary and acquired risk factors, clinical and laboratory features of patients with thrombosis, treatment approaches, and thrombosis‐related mortality and morbidity rates in pediatric ALL patients.ProcedurePediatric patients who developed CNS thrombosis during ALL treatment from 2010 to 2021 were analyzed retrospectively in 25 different Pediatric Hematology Oncology centers in Türkiye. The demographic characteristics of the patients, symptoms associated with thrombosis, the stage of the leukemia treatment during thrombosis, the anticoagulant therapy applied for thrombosis, and the final status of the patients recorded through electronic medical records w...

Indian Journal of Pediatrics

Turkiye Klinikleri Journal of Pediatrics, 2013

of hematological malignancies. Particularly, when ARA-C is given in high doses, it provokes vario... more of hematological malignancies. Particularly, when ARA-C is given in high doses, it provokes various adverse effects, including fever, severe myelosupression, infection and toxic reactions involving the central nervous system, liver, gastrointestinal tract, eyes and skin. Dermatologic side effects of cytosine arabinoside therapy are often described as rare. Nevertheless, there are differences in the observed frequency of toxic skin events ranging from 2% to 53%. Moreover, idarubicine and etoposide are very rarely associated with adverse skin reactions.1,2

Neurophysiologie Clinique, 2021

OBJECTIVES In children, cancer chemotherapy may impair the functioning of the cardiac autonomic n... more OBJECTIVES In children, cancer chemotherapy may impair the functioning of the cardiac autonomic nervous system. Moreover, it is not known whether there are any differences between intensive and maintenance phases of chemotherapy. Therefore, the aim of the current study was to assess autonomic nervous system activity using heart rate variability, in children receiving intensive or maintenance cancer chemotherapy. METHODS For that purpose, children who were healthy (healthy control, n = 30), receiving intensive chemotherapy (chemotherapy, n = 30), and receiving maintenance chemotherapy (maintenance, n = 25) were included in the study. Autonomic nervous system activity was measured by means of heart rate variability. Electrocardiogram recordings were used to calculate time- and frequency-domain heart rate variability parameters. RESULTS Time-domain parameters such as standard deviation of NN intervals and frequency-domain parameters such as total power were lower during the intensive chemotherapy but not during maintenance phase (standard deviation of NN intervals: 50±5, 33±3, and 48±3 ms, and total power: 2613±504, 1379±296 and 2295±264 ms2, respectively for healthy control, chemotherapy and maintenance groups, P<0.001 for both standard deviation of NN intervals and total power). DISCUSSION The present results indicate that intensive chemotherapy perturbs the function of heart rate variability in children, with recovery during the maintenance phase. This suggests that intensive chemotherapy is likely to affect the autonomic nervous system but this effect does not appear to be permanent.

Turkish Journal of Hematology, 2020

Trends in Pediatrics, 2022

Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disease characterized by bone marr... more Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disease characterized by bone marrow dysfunction, exocrine pancreatic insufficiency and skeletal abnormalities. Persistent or intermittent neutropenia caused by bone marrow hypoplasia is the most common hematological abnormality in SDS. It can be difficult to diagnose the disease that usually occurs in early childhood. SDS should be kept in mind in the differential diagnosis of neutropenic patients. If the signs of pancreatic insufficiency are not observed, the diagnosis may be missed. The article wanted to present a patient with pancreatic insufficiency and SDS with the biallelic mutation who presented with neutropenia in a newborn.

Bone Marrow Transplantation, 2022

We report the national data on the outcomes of hematopoietic stem cell transplantation (HSCT) for... more 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.

Journal of Turgut Ozal Medical Center, 2015

Objectives: Metamizole sodium (Novalgin ® ) is an effective, widely used analgesic and antipyreti... more Objectives: Metamizole sodium (Novalgin ® ) is an effective, widely used analgesic and antipyretic agent in several countries. In spite of its availability and common usage, it has serious and potentially fatal adverse effects like agranulocytosis and aplastic anemia. Objectives of this study are to evaluate incidence, severity, and clinical significance of metamizole related neutropenia and agranulocytosis, and to study recovery duration of neutropenia and agranulocytosis in children exposed to metamizole. Material and Methods: Thirteen cases of neutropenia and/or agranulocytosis secondary to metamizole usage have been evaluated retrospectively. Duration and cumulative doses of metamizole, degree of neutropenia, recovery from neutropenia, and other complications related to neutropenia were evaluated. Results: Overall, thirteen granulocytopenic patients were recorded for 12 months after metamizol usage. Mean metamizole exposure duration was 4,64 (2-7) days; mean daily total metamizo...

Journal of Paediatrics and Child Health, 2021

The aim of this study is to investigate the effects of vitamin B12 deficiency on hearing in schoo... more The aim of this study is to investigate the effects of vitamin B12 deficiency on hearing in school‐aged children by pure‐tone audiometry.

Journal of Pediatric Nursing, 2021

Background: Caregivers of hemodialysis patients endure a significant caring pressure as a result ... more Background: Caregivers of hemodialysis patients endure a significant caring pressure as a result of caring for patients with chronic illness, which can affect their quality of life. Disruptions in the quality of life of these caregivers impose double pressure on them and disrupt the care process. Therefore, the present study aimed to determine the level of care burden and its relationship with quality of life of caregivers of hemodialysis patients. Methods: In this descriptive-analytical study, 246 caregivers of hemodialysis patients were enrolled by census method, so that, all patients and caregivers who attended the study environment at morning, evening and night participated in the study. The study tool was a three-part questionnaire, which included personal information, Novak & Guest Care burden Questionnaire, and WHOQOL-BREF Quality of Life Questionnaire. Data were analyzed by descriptive, statistical and inferential tests. Results: In total, 37.4% of caregivers were experiencing high and very high levels of care burden and 42.7% of them were experiencing a moderate level of care burden. The mean and standard deviation of the quality of life of caregivers was 76.27 ± 13.67 out of 130. There was a significant and negative correlation between the total scores of care burden and quality of life (r = − 0. 436, P < 0.001). The factors influencing care burden included variables such as; level of patient's caring capability, the patient's incidence of other chronic diseases, and the age of the caregiver. So that, in case of reduced patient's capability in self-care, the patient's incidence of other chronic diseases, and the increased age of the caregiver, the level of care burden on the caregivers would be increased. Conclusions: The caregivers of hemodialysis patients endure high level of care burden and this pressure has a negative effect on their quality of life. Therefore, it is recommended to pay more attention to the needs of caregivers and provide adequate social, economic, physical and psychological support for them.

Turkish Journal of Hematology, 2021

Patients with solid malignancies are more vulnerable to SARS-CoV-2 infection than healthy populat... more Patients with solid malignancies are more vulnerable to SARS-CoV-2 infection than healthy population. The outcome of SARS-CoV-2 infection in highly immunosupressed populations, such as in patients with hematological malignancies is a point of interest.We aimed to analyze the symptoms, complications, intensive care unit (ICU) admission and mortality rates of patients with hematological malignancies infected with SARS-CoV-2 in Turkey. In this multicenter study, we included 340 adult and pediatric patients diagnosed as COVID-19 from March to November. Forty four (12.9%) patients were asymptomatic at diagnosis. In symptomatic patients, fever, cough and dyspnea were observed in 62.6%, 48.8 % and 41.8% of the patients, respectively. Sixty nine (20%) patients had mild SARS-Cov-2 disease, whereas moderate, severe and critical diseases were reported in 101 (29%), 71 (20%) and 55 (16%) patients, respectively. Two hundered fifty one (73.8%) patients were hospitalized for SARS-Cov2. Mortality related to SARS-CoV-2 infection was 26.5% in all cohort; 4.4% in those patients with mild disease, 12.4% in moderate disease and 83% in severe and critical disease. Active hematological disease, lower life expectancy related with primary hematological disease, neutropenia at diagnosis of SARS-CoV-2, ICU admission and firstline therapy used for COVID 19 treatment were found to be related to higher mortality rates. Treatments with hydroxychloroquine alone or in combination with azitromycin were associated with a higher rate of mortality in comparison with favipiravir use.In conclusion, patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.

Transfusion and Apheresis Science, 2021

Thalassemia major (TM) is a severe transfusion-dependent anemia. Regular erytrocyte transfusion i... more Thalassemia major (TM) is a severe transfusion-dependent anemia. Regular erytrocyte transfusion is required for the treatment of thalassemia patients. However, repeated transfusion may result in impairements in heart function. In this study, we aimed to investigate short-term effects of erythrocyte transfusion on autonomic control of heart in children with thalassemia major. For that purpose heart rate variability (HRV), which is a non-invasive method used to evaluate the effects of the autonomic nervous system on the heart rhythym, was measured before and after erythrocyte transfusion and compared to the healthy controls. Children diagnosed with TM (n = 17) and sex and age matched healthy children (HC, n = 30) were included in the study. HRV values of TM patients were measured 5 min before erythrocyte transfusion (BET, n = 17) and 5 min after erythrocyte transfusion (AET, n = 17). Parameters of time-domain and frequency-domain of HRV were evaluated in all participants. Heart rate (HR) was higher in the BET than AET (P = 0.002) but there was no difference between AET and HC groups (P > 0.05). HRV parameters were lower in BET than AET (P < 0.05) but there were no statistical difference between AET and HC (P> 0.05). The data suggest that, in thalassemia major patients, erythrocyte transfusion restores HR and HRV parameters to the levels observed in healthy controls and, thus, in short-terms, appears to be beneficial for the autonomic control of the heart.

Pediatric Blood & Cancer, 2021

To the Editor: Adultswith cancer are reported tohave ahigher risk for coronavirus disease (COVID-... more To the Editor: Adultswith cancer are reported tohave ahigher risk for coronavirus disease (COVID-19) infection and more severe disease and mortality than the general population.1,2 Although children seem to be at a lower risk for COVID-19 than adults,3–5 data specifically addressing children with cancer are limited.6–12 Weconducted a retrospective,multicenter, cross-sectional studyon behalf of the Turkish Pediatric Hematology Society (TPHD) and Turkish Pediatric Oncology Group (TPOG) Society to analyze the characteristics of COVID-19 in all patients with cancer and stem cell transplant (SCT) recipients in all centers in Turkey, during March 11-May 31, 2020. Approval for the study was obtained by Turkish Ministry of Health (MoH), Istanbul University COVID Scientific Research Committee, and Istanbul University Ethics Committee. The study was carried out through the analysis of a questionnaire with 62 questions, which was sent to all members of the TPOG and TPHD Societies working in all 66 pediatric hematology/oncology departments in university, state, and private hospitals in Turkey. All replied and 53 patients were reported from the 24 centers. Following the national recommendations and guidelines of the MoH,13,14 centers tested all symptomatic patients or patients with contact history or patients who were planned to undergo transplantation or surgery. All patients and caregivers were questioned at the entrance of the hospital/oncology center and if there were any symptoms or contact history they were sent to the special clinics within the hospital that were allocated for suspected/proven COVID-19 patients. If a patient was suspected of having COVID-19 and found positive while in the oncology clinic, she/hewas transferred to theCOVID clinic and all staff, patients, and accompanying persons with whom she/he was in contact were tested for COVID-19. Samples from the naso-oropharyngeal swabs were tested for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by polymerase chain reaction (PCR). Confirmed cases were defined as PCR-positive patients. Probable cases were defined according to guidelines of World Health Organization15 and MoH,13 as those who had typical symptoms and chest CT findings, or who had typical symptoms and contact history but negative PCR. Patients were classified into four groups regarding the severity of infection as asymptomatic/mild, moderate, severe, and critical on the basis of the clinical, laboratory, and radiological features.3,15 Patients were treated according to recommendations of the MoH.13,14 Statistical analyses were performed by IBM SPSS Statistics version 21.0. For comparison of mean of numerical variables Mann-Whitney U test was used. Categorical nominal variableswere comparedwith Fisher’s exact tests. There were 51 children with cancer, six of whom (four leukemia/lymphomas, two solid tumors) had undergone SCT (Table 1). The median age was 6 (0.3-17.8) years and 64.7% of the patients were male. Additional two patients with thalassemia major who underwent SCT were not included in the analyses. Chemotherapy courses were interrupted in 32 (62.7%) patients and delayed with a median of 15 (3-45) days. The most common presenting signs were fever and cough, while 37.25% of patients were asymptomatic. COVID-19 pneumonia was detected in 26 (50.9%) patients. Five of them had hypoxemia. Gastrointestinal system (17.6%) was the second most commonly involved site followed by central nervous, musculoskeletal systems and skin. Twenty-five patients had asymptomatic/mild, 17 patients moderate/severe, and nine patients critical disease. Thirty-eight patients were hospitalized and treated according to the severity of illness, six of whom were already hospitalized for reasons such as surgery or diagnostic workup when diagnosed with COVID-19. Nine patients with critical disease were in the intensive care unit (ICU) and three were intubated. Treatment consisted of hydroxychloroquine, azitromycine, antivirals either as a single agent or in combination (Table 1). Convalescent plasma was used in three patients, one of whom additionally received mesenchymal stem cell, tocizulumab, and granulocyte transfusions and was intubated. At the time of COVID-19 diagnosis, 26 patients had neutropenia and among them 15 had fever. In all patients with febrile neutropenia, broad-spectrum empirical antibiotics were initiated. In addition, 12 patients received antimicrobial therapy due to clinically and/or microbiologically documented infections. All patients, but one, fully recovered and the PCR tests became negative at a median of 7 (2-17) days. The patient who had received allogeneic SCT for relapsed leukemia/lymphoma and had progressive disease and fungal infection died due to COVID-19 infection. The incidence of critical care disease and need for ICU care were found tobehigher inpatientswithhematologicmalignancies (P= .012), patients post SCT (P = .001), patients with other infections (P = .005), and…

Turkiye Klinikleri Journal of Case Reports, 2021