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Bu çalışmanın amacı Bursa'da yaşayan 1-16 yaş arası çocuklarda demir eksikliği (DE), demir eksikliği anemisi (DEA) prevalansını belirlemek ve beslenme alışkanlıkları ve aile eğitim düzeyinin demir eksikliği anemisi gelişimine etkilerini araştırmaktır. Bu çalışma düşük sosyoekonomik bir bölgede bulunan 3 farklı aile sağlığı merkezine başvuran 1-16 yaş arası 500 sağlıklı çocukta yapılmıştır. Çalışmaya alınan çocuklar sırasıyla 1-1,9, 2-5, 6-10,9 ve 11-16 yaş grubundaki çocuklar olmak üzere dört gruba ayrıldı.Tam kan sayımı, serum demir (µg/dl), demir bağlama kapasitesi (µg/dl), ferritin (ng /ml), transferrin satürasyonu (%) çalışıldı. Araştırmaya alınan çocukların beslenme ve anne-baba eğitim durumlarını araştırmak için anket uygulandı. Bu çalışmada anemi, DE ve DEA National Health and Nutrition Examination Survey (NHANES III) kriterlerine göre araştırıldı. Mentzer indeksi talasemi minör ve DEA'yı ayırt etmek için kullanıldı. Araştırmaya alınan 247 erkek (%49) ve 253 kız (%51)olgularımızın yaş ortalaması 7,4±3,9 yıl idi. NHANES III kriterlerine göre anemi prevalansı %31(n=155) idi. Grup I ve Grup IV'te anemi görülme sıklığı diğer gruplara göre daha yüksekti(p, This study aimed to determine the prevalence of iron deficiency(ID) and iron deficiency anemia(IDA),and to investigate the effects of nutritional habits and family education level on the development of IDA in children aged between 1-16 years living in Bursa.This study is performed in 3 different primary health care center in a low socioeconomic region.Five hundred healthy subjects aged between 1-16 years was admitted for the study.The study population was divided into 4 different age groups as including children1-1.9, 2-5,6-10.9,and11-16years old,respectively.Blood count,serum iron(µg/dl),iron-binding capacity(µg/dl),ferritin(ng/ml),and transferrin saturation(%)were measured.A questionnaire was performed for investigating the nutritional and parental education status of the children included in the study.Anemia,ID,and IDA were investigated according to the National Health and Nutrition Examination Survey(NHANESIII) criteria.Mentzer Index was used to differentiate thalassemia minor and IDA in anemic children.There were 247 male(49%), 253 females(51%) in study. The mean age was7.4±3.9 years.The prevalence of anemia according to the NHANES III criteria was found 31%(n=155).The prevalence of anemia was higher in Group I and Group IV compared to other groups(p

Assestment of minimal residual disease (MRD) in childhood acute lymphoblastic leukemia (ALL) is of utmost importance both for risk classification and tailoring of the therapy. The data of pediatric ALL patients that received treatment with Berlin-Frankfurt-Munster (BFM) protocols were retrospectively collected from 5 university hospitals in Turkey. Of the 1388 patients enrolled in the study 390 were treated according to MRD-based protocols. MRD assestment was with real time quantitative polymerase chain reaction (qPCR) in 283 patients and with multiparametric flow cytometry (MFC)-MRD in 107 patients. MRD monitoring had upstaged a total of 8 patients (2%) from intermediate risk group to high-risk group. Univariate analysis revealed age 10 years or above, prednisone poor response, PCR-MRD >= 10(-3) on day 33 and on day 78 as poor prognostic factors affecting event-free survival (EFS). Detection of >10% blasts on day 15 with MFC (MFC-high-risk group) was not shown to affect EFS and/or overall survival (log-rank P=0.339). Multiple logistic regression analysis revealed PCR-MRD >= 10(-3) on day 78 as the only poor prognostic factor affecting EFS (odds ratio: 8.03; 95% confidence interval: 2.5-25; P=0.000). It is very important to establish the infrastructure and ensure necessary standardization for both MRD methods for optimal management of children with ALL.

Giriş: Lösemi, multifaktöriyel bir hastalıktır. Bazı genetik sendromların lösemi sıklığını artırdığı iyi bilinmektedir. Biz çalışmamızda sendromik olmayanmalformasyon ile lösemi ilişkisini değerlendirdik.Gereç ve Yöntem: Akut lösemi tanısı almış 288 hasta çalışmaya dahil edildi. Malign olmayan hematolojik hastalığı olan 201 hasta kontrol grubu olarak kabuledildi. Sendromik çocuklar her iki grupta da dışlandı. Tüm çocuklar ICD-10, Bölüm XVII’ye göre konjenital malformasyon açısından muayene edildi. Her ikigrupta malformasyonların tipi ve sayısı karşılaştırıldı.Bulgular: Tanı anındaki yaş, cinsiyet, ebeveynler arasında akrabalık, anne babanın doğum yaşı, ailede kanser öyküsü ve annenin önceki gebelikleri açısından lösemi ve kontrol grubu arasında fark yoktu. Lösemili popülasyonda konjenital malformasyonlar daha fazla görüldü (p, Introduction:Leukemia is a multifactorial disease. Some genetic syndromes is well known related to leukemia. We evaluated non-syndromic malformation andleukemia relation. Materials and Methods: 288 patients diagnosed with acute leukemia are included the study. 201 patients with non-malign hematologic disease are accepted as acontrol. Syndromic children were excluded both group. All children were examined according to ICD-10th, Chapter XVII for congenital malformation. The type andnumber of malformations were compared both group. Results: There were no differences between leukemia and control group in terms of age at diagnosis, gender, consanguinity between parents, parents age at birth,family history of cancer and pregnancies of mother. Congenital malformations were more observed in leukemic population (p

INTRODUCTION: Hemophilia is expected to adversely affect the child's psycho-social development and mothers’ mood and attitudes toward the ill children. The aim of the study was to investigate the psychological problems of both mother and adolescent and to found out if there was a relationship between mother’s mood, overprotection and adolescents’ problem behaviors.MATERIALS and METHODS: The study group consisted of 19 male adolescents with severe Hemophilia A and B (between 11-16 years) and their mothers. 22 volunteer medical staff mothers and their sons (between 11-16 years) enrolled in the study as a control group. Data were collected from both mother and son with self-rated psychometric tools in one session.RESULTS: There were positive correlation between diagnosed hemophilia and anxiety, depression and oppositional and defiant behavior. Over-protective behavior of mother and sons’ anxiety, depression, oppositional and defiant behavior, and mothers’ depression were positively correlated.When comparing the both group’s total score, mother’s depression, over-protective attitudes, were found significantly different. And adolescents’ oppositional and defiant behavior, depression and anxiety score were found significantly different between groups.CONCLUSIONS: Some suggestions were made in order to decrease the problematic behavious in both hemophilic adolescents and their mothers. Mothers should be informed about the disease, treatment and their overprotective attitudes and mothers must be supported to share responsibilities in care of hemophilic children. The family and the hemophiliac child should focus on what can be done, taking into account the risks of hemophilia., GİRİŞ ve AMAÇ: Hemofili, çocuğun psiko-sosyal gelişimini ve annelerin duygu durumu ile çocuğa karşı tutumlarını olumsuz etkilemesi beklenen kronik hastalıklardandır. Bu çalışmanın amacı, ağır hemofili tanısıyla izlenen ergen ve annesinin psikolojik problemlerini incelemek ve annenin duygu durumu ile aşırı koruyucu tutumlarının ergenin davranışları üzerindeki etkisini incelemektir.YÖNTEM ve GEREÇLER: Çalışma grubu 19 ağır hemofili A ve B hastası olan erkek ergen (11-16 yaş arası) ve annelerinden oluşmaktadır. 22 gönüllü sağlık personeli anne ve oğulları (11-16 yaş arası) çalışmaya kontrol grubu olarak alınmıştır. Veriler hem anne hem de ergenlerden tek oturumda psikometrik araçlarla toplanmıştır.BULGULAR: Hemofili ile anksiyete, depresyon ve karşıt olma-karşıt gelme davranışları arasında pozitif korelasyon bulunmuştur. Annenin aşırı koruyucu tutumları ile ergenlerin anksiyete, depresyon, karşıt olma-karşıt gelme davranışları ve annenin depresyon puanları arasında pozitif bir korelasyon bulunmuştur. Her iki grubun toplam puanları karşılaştırıldığında, annelerin depresyon puanları ve aşırı koruyucu tutumları çalışma grubunda anlamlı olarak fazladır. Ergenlerin ise karşıt olma karşıt gelme, depresyon ve anksiyete puanları çalışma grubunda anlamlı olarak fazla bulunmuştur.TARTIŞMA ve SONUÇ: Hemofili tanısı almış ergen ve annelerinde problem davranışların azaltılması için bazı önerilerde bulunulmuştur. İlk olarak anne, hastalık, tedavi ve aşırı koruyucu tutumları ile bilgilendirilmeli, sorumluluklarını paylaşmalıdır. Aile ve hasta çocuk, hemofili risklerini göz önünde bulundurarak, yapılabileceklere odaklanmalıdır.

Objective: We investigated the health-related quality of life (HRQL)in survivors of pediatric acute lymphoblastic leukemia (ALL) andevaluated the perceptions of the children, their siblings, and theirparents.Materials and Methods: Seventy ALL survivors, who were between7 and 17 years of age and had completed therapy ≥2 years, wereincluded. The control group consisted of their healthy siblings. HRQLwas assessed by the age-specific KINDLR questionnaire.Results: No significant differences could be found among HRQL scoresof ALL survivors with respect to variables such as sex, risk group, andhaving chronic illness. HRQL scores for physical well-being, emotionalwell-being, family, and social functioning of the patient and siblingself-reports and parent proxy reports were lower than the expectedvalues for healthy and chronically ill children.Conclusion: These results demonstrate that both ALL survivors andtheir families need help via psychological counseling programs toimprove their HRQL even after completion of therapy.

WOS:000426572200004, PubMed ID: 28179213, Amaç: Türkiye'deki juvenil miyelomonositik lösemi (JMML) hastalarının durumunu, tanı zamanı, klinik özellikler, mutasyon çalışmaları, klinik gidiş ve tedavi stratejileri açısından ortaya koymaktır.Gereç ve Yöntemler: Ülkemizdeki pediatrik hematoloji ve onkoloji kliniklerinden veri istenerek, JMML tanısı ile takip ve tedavisi yapılan hastaların klinik ve laboratuvar bulguları geriye dönük olarak değerlendirildi.Bulgular: On sekiz merkezden, 2002-2016 tarihleri arasında JMML tanısı alan toplam 65 hasta çalışmaya dahil edildi. Ortanca tanı yaşı 17 ay idi (2-117 ay). Splenomegali tanıda %92 hastada vardı. Ortanca lökosit, monosit ve trombosit sayıları sırasıyla 32,9x109/L, 5,4x109/L ve 58,3x109/L idi. Monozomi 7, %18 hastada saptanmıştı. JMML mutasyonları 32 hastada (%49) çalışılmış olup, en sık rastlanan mutasyon PTPN11 idi. Hematopoetik kök hücre nakli (HKHN) 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 3017.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p0.019). Older age at diagnosis (2 years), platelet count of less than 40x109/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. Conclusion: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results.

WOS: 000426572200004, PubMed ID: 28179213, Objective: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies. Materials and Methods: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. Results: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. The median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. The median white blood cell, monocyte, and platelet counts were 32.9x10(9)/L, 5.4x10(9)/L, and 58.3x10(9)/L, respectively. Monosomy 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 30 +/- 17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). Older age at diagnosis (>2 years), platelet count of less than 40x10(9)/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. Conclusion: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results are not satisfactory and it is necessary to put more effort into this issue in Turkey.

Trombosit sayısının450.000/mm3 üzerinde olması trombositoz olarak kabul edilir. Nedenlerinegöre sekonder ya da primer olarak gruplandırılır. Çocukluk çağında genellikle trombositozunnedeni sekonderdir ve neden olan hastalığın kontrol altına alınması iletrombositoz geriler. Sekonder trombositoz kendini sınırlayan bir durumdur. Nadirenve özellikle altta yatan bir risk faktörü var ise tromboemboliye neden olur.Myeloproliferatif hastalıklar içinde gruplandırılan primer trombositozlar çocuklukçağında çok nadirdir. Primer trombositozda tromboembolik olaylar en önemlimortalite ve morbidite nedenidir. Primer trombositozlu hastaların bir kısmıkanama bulguları ile gelebilir.APPROACH TO CHILDREN WITHTHROMBOCYTOSISSUMMARY:The platelet countabove 450.000/mm3 is defined as thrombocytosis. According to theunderlying causes, thrombocytosis is classified as primary (essential) orsecondary (reactive) thrombocytosis. In childhood, the most common causes ofthrombocytosis are secondary reasons. Secondary thrombcytosis is a self-limiteddisease. It resolves with control of the underlying disorder. Secondary thrombocytosisusually does not result in thromboembolic events. Thrombembolic complications occurusually in patients who have additional risk factors. Primary thrombocytosis isgrouped under the myeloproliferative diseases and is extremely rare in childhood.Thromboembolic events in primary thrombocytosisare the most important causes of mortality and morbidity. Some cases withprimary thrombocytosis may present with bleeding symptoms. Keywords: Thrombocytosis, Childhood