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MERAL GÜNEŞ, ADALET

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    Characteristics, treatment and outcome of 15 to 18 years-old adolescents with Chronic Myeloid Leukemia (CML): The experience of the International Registry of Childhood CML (I-CML-Ped Study)
    (American Society of Hematology, 2021-11-23) Froment, Morgane; Suttorp, Meinolf; Ragot, Stephanie; Deutsch, Helene; Goyeau, Violaine; De Moerloose, Barbara; Brandalise, Silvia Regina; Borisevich, Marina; Sedlacek, Petr; Gunes, Adalet Meral; Lausen, Birgitte; Molines Honrubia, Antonio; Jakovljevic, Gordana; Versluys, Birgitta; Kalwak, Krzysztof; Hraskova, Andrea; Millot, Frederic; MERAL GÜNEŞ, ADALET; Uludağ Üniversitesi/Tıp Fakültesi; EXD-8400-2022
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    Evaluation of micafungin use in children
    (Ankara Microbiology, 2020-01-01) Hacimustafaoglu, Mustafa; Yeşil, Edanur; YEŞİL, EDANUR; Çelebi, Solmaz; ÇELEBİ, SOLMAZ; Sezgin Evim, Melike; SEZGİN EVİM, MELİKE; Özer, Arife; Turan, Cansu; TURAN, CANSU; Timur, Demet; TİMUR, DEMET; Çakır, Salih Cağrı; ÇAKIR, SALİH ÇAĞRI; Bülbül, Beyhan; BÜLBÜL, BEYHAN; Ener, Beyza; ENER, BEYZA; Güneş, Adalet Meral; MERAL GÜNEŞ, ADALET; Koksal, Nilgun; Özkan, Hilal; ÖZKAN, HİLAL; Sevinir, Betul; SEVİNİR, BETÜL BERRİN; Düzcan Kilimci, Duygu; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Hematoloji Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Onkoloji Anabilim Dalı.; 0000-0002-8926-9959; 0000-0003-3146-6391; 0000-0001-5761-4757; 0000-0002-5720-1212; 0000-0002-3232-7652; 0000-0003-4646-660X; AAG-8523-2021; AEZ-2469-2022; GSO-3630-2022; AAH-1570-2021; HJZ-4508-2023; AAE-6201-2021; AAG-8393-2021; JCD-9679-2023
    Micafungin is recommended especially in patients with liver and kidney failure and in the presence of other side effects due to antifungals apart from its known priority indications such as invasive candidiasis. The aim of this study was to evaluate the children who have received micafungin treatment. In the study, 125 children who were hospitalized in the pediatric wards and intensive care units of our hospital and had used micafungin between November 2016 and January 2019 were analyzed retrospectively. Clinical data, micafungin indication, blood values on the first and fourth days of the treatment, side effects of the drug and efficacy were evaluated. Sixty percent (75/125) of the patients were male and the mean age of all the patients were 58 +/- 67 (0-215, 30) months. Approximately half of the cases (48%) had malignancy and 13% of them were premature. Sixty-two percent (n= 37) of the malignencies were hematological (27 acute lymphocytic leukemia, nine acute myeloid leukemia, one myelodysplastic syndrome) and 38% (n= 23) were oncological (six neuroblastoma, four Hodgkin lymphoma, two Non-Hodgkin's lymphoma, five sarcomas, one hepatoblastoma, five others) malignencies. The major cause of hospitalization was sepsis (53%). The patients had several risk factors like immunosuppressive therapy (n= 68, 54%), neutropenia (n= 61, 49%), central venous catheter (n= 102, 82%), nasogastric tube (n= 63, 50%), endotracheal intubation tube (n= 49, 39%), urinary catheter (n= 14, 11%) and total parenteral nutrition (n= 81, 65%). Thirteen percent (n= 16) of the cases were post-operative patients. Candida species were cultivated in 97 clinical specimens (blood, endotracheal aspirate, sputum, urine, etc.) among 23 (18%) of the patients. Thirteen (10%) of the patients had candidemia and 62% of them were non-albicans strains. In all candidemias, strains were echinocandin susceptible, and blood cultures were negative within four days. When all the patients (n= 125) were evaluated, a significant decrease in C-reactive protein, an increase in sodium, and a decrease in alanine aminotransferase were observed on the fourth day of micafungin treatment (p< 0.05). A total of 39 (31%) patients underwent various antifungal treatments for median seven (1-60) days prior to micafungin treatment. Fourteen (36%) of these 39 patients, had elevated liver function tests (LFT), 10 (26%) of them had hypokalemia, and five (13%) of them had elevated renal function tests. Ten (26%) patients had antifungal-induced hypokalemia previously; and potassium levels were normalized after micafungin treatment (p= 0.0001). The patients for which micafungin treatment was chosen due to elevated liver function tests (n= 47, 38%), whether the antifungalinduced or not; alanine aminotransferase and aspartate aminotransferase levels were decreased after micafungin treatment (p= 0.0001 and p= 0.0001, respectively). Nineteen (15%) of the patients have died within the first 30 days of micafungin treatment and one of them had candidemia. No micafungin treatment related significant side effects were observed in any of the patients. Our study showed that micafungin could be a safe and effective option in pediatric cases including newborns with high liver and kidney function tests.
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    A rare case of recurrence presenting with bilateral exudative retinal detachment in a child with acute lymphoblastic leukemia
    (Wiley, 2021-08-16) Yalçınbayır, Özgür; Sezgin Evim, Melike; Uçan Gündüz, Gamze; Güler, Salih; Meral Güneş, Adalet; YALÇINBAYIR, ÖZGÜR; SEZGİN EVİM, MELİKE; UÇAN GÜNDÜZ, GAMZE; GÜLER, SALİH; MERAL GÜNEŞ, ADALET; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Göz Hastalıkları Anabilim Dalı; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Hematoloji Anabilim Dalı; 0000-0002-7311-5277; 0000-0002-1219-8304; 0000-0002-5458-1686; 0000-0002-4792-269X; 0000-0002-0686-7129; IYJ-9408-2023; AAH-1452-2021; AAH-6661-2021; DWH-8632-2022; EXD-8400-2022
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    Thrombolysis with systemic recombinant tissue plasminogen activator in children: A multicenter retrospective study
    (Galenos Yayıncılık, 2021-08-18) Zengin, Emine; Sarper, Nazan; Erdem, Arzu Yazal; Al, Işık Odaman; Evim, Melike Sezgin; Yaralı, Neşe; Belen, Burcu; Akçay, Arzu; Yıldırım, Aysen Türedi; Karapınar, Tuba Hilkay; Güneş, Adalet Meral; Gelen, Sema Aylan; Oren, Hale; Olcay, Lale; Baytan, Birol; Gülen, Hüseyin; Öztürk, Gülyüz; Orhan, Mehmet Fatih; Oymak, Yeşim; Akpınar, Sibel; Tüfekci, Özlem; Albayrak, Meryem; Günen, Burçak Tatlı; Canpolat, Aylin; Özbek, Namık; SEZGİN EVİM, MELİKE; MERAL GÜNEŞ, ADALET; Baytan, Birol; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk Hematoloji Bilim Dalı.; 0000-0002-4792-269X; 0000-0002-0686-7129; 0000-0002-9375-2855; JGX-6145-2023 ; AAH-1452-2021 ; DVW-8108-2022
    Objective: This study aimed to evaluate systemic thrombolysis experiences with recombinant tissue plasminogen activator (rtPA). Materials and Methods: Retrospective data were collected from 13 Turkish pediatric hematology centers. The dose and duration of rtPA treatment, concomitant anticoagulant treatment, complete clot resolution (CCR), partial clot resolution (PCR), and bleeding complications were evaluated. Low-dose (LD) rtPA treatment was defined as 0.01-0.06 mg/kg/h and high-dose (HD) rtPA as 0.1-0.5 mg/kg/h. Results: Between 2005 and 2019, 55 thrombotic episodes of 54 pediatric patients with a median age of 5 years (range: 1 day to 17.75 years) were evaluated. These patients had intracardiac thrombosis (n=16), deep vein thrombosis (DVT) (n=15), non-stroke arterial thrombosis (n=14), pulmonary thromboembolism (PE) (n=6), and stroke (n=4). The duration from thrombus detection to rtPA initiation was a median of 12 h (range: 2-504 h) and it was significantly longer in cases of DVT and PE compared to stroke, non-stroke arterial thrombosis, and intracardiac thrombosis (p=0.024). In 63.6% of the episodes, heparin was initiated before rtPA treatment. LD and HD rtPA were administered in 22 and 33 of the episodes, respectively. Concomitant anticoagulation was used in 90% and 36% of the episodes with LD and HD rtPA, respectively (p=0.0001). Median total duration of LD and HD rtPA infusions was 30 h (range: 2-120 h) and 18 h (2-120 h), respectively (p=0.044). Non-fatal major and minor bleeding rates were 12.5% and 16.7% for LD and 3.2% and 25.8% for HD rtPA, respectively. At the end of the rtPA infusions, CCR and PCR were achieved in 32.7% and 49.0% of the episodes, respectively. The most successful site for thrombolysis was intracardiac thrombosis. HD versus LD rtPA administration was not correlated with CCR/PCR or bleeding (p>0.05). Conclusion: Systemic thrombolytic therapy may save lives and organs effectively if it is used at the right indications and the right times in children with high-risk thrombosis by experienced hematologists with close monitoring of recanalization and bleeding.
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    Retrospective evaluation of hemophagocytic lymphohistiocytosis cases treated
    (Galenos Yayıncılık, 2020-04-01) Temuroğlu, Aytül; Evim, Melike Sezgin; Sevinir, Betül; Baytan, Birol; Güler, Salih; Güneş, Adalet Meral; TEMUROĞLU, AYTÜL; SEZGİN EVİM, MELİKE; SEVİNİR, BETÜL BERRİN; Baytan, Birol; GÜLER, SALİH; MERAL GÜNEŞ, ADALET; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Hematoloji ve Onkoloji Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Hematoloji Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Onkoloji Anabilim Dalı.; 0000-0002-8943-6585; 0000-0002-3232-7652; 0000-0002-9375-2855; AAH-1570-2021; AAH-1452-2021; GES-3112-2022; DVW-8108-2022; JHO-2788-2023; JGX-6145-2023
    INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare inflammatory syndrome resulting from uncontrolled proliferation in the monocyte / macrophage system. It is divided into two classes as familial (primary) and secondary. Since the rate of consanguineous marriage in our country is high with 29.2%, the frequency of HLH is also increasing. Secondary HLH can be seen at any age, but its true incidence is unknown. It can accompany malignancies, infections and inflammatory processes. Major clinical findings are persistent high fever, cytopenia, splenomegaly and / or hepatomegaly. The first finding is usually fever. The goal of treatment is to stop abnormal inflammation and treat the underlying cause.MATERIALS and METHODS: We retrospectively reviewed 15 patients diagnosed and treated in our clinic between 2010 and 2019. Statistical analysis was performed with kruskal-wallis test.RESULTS: The median age of diagnosis of 15 patients diagnosed and treated in our clinic was 18 months (1 month-17 years). The female to male ratio was 6/9. Fever was seen in all patients, hepatosplenomegaly was 80%. The mean ferritin value was 33.927 +/- 51.461 mu / 1 (964-201.074). In our patient group, consanguineous marriage rate was 53% (n = 8) and it was high and the factor was EBV in all patients who developed secondary to infection. 33% (n 5) of the cases were primary and 67% (n = 10) were secondary. 40% (n = 4) of secondary cases had malignancy in etiology, 50% (n = 5) infection and 10% (n = 1) metabolic disease.CONCLUSIONS: HLH is important for early diagnosis in cases with persistent high fever, hepatosplenomegaly and high ferritin conditions in our country. In cases presenting with HLH, malignancy should not be forgotten in etiology besides primary causes.
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    Acute endocrine and metabolic complications during treatment in childhood acute lymphoblastic leukemia patients: Retrospective study
    (Güncel Pediatri, 2023-04-01) Evim, Melike Sezgin; Tobçu, Zeynep; Güler, Salih; Öngen, Yasemin Denkboy; Güneş, Adalet Meral; SEZGİN EVİM, MELİKE; TOBCU, ZEYNEP; GÜLER, SALİH; DENKBOY ÖNGEN, YASEMİN; MERAL GÜNEŞ, ADALET; Uludağ Üniversitesi Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk Hematoloji Bilim Dalı; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dal; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk Endokrinoloji Bilim Dal; IRI-7023-2023; JJX-6307-2023; JHO-2788-2023; JJP-0399-2023; JGX-6145-2023
    Introduction: Acute leukemias are the most common malignancy of childhood, and acute lymphoblastic leukemia (ALL) is the most common subtype. In this study; we aimed to asses to acute endocrine and metabolic complications which occurs during treatment. Newly diagnosed ALL patients were included in the study.Materials and Methods: The endocrine and metabolic complication of 293 patients aged 1-18 years old who were newly diagnosed ALL between January 2007 and December 2017 in Uludag University Faculty, Department of Pediatric Hematology were analyzed. Patients' age, gender, risk groups, leukemia subtypes, and chemotherapy phases at the time of endocrine complications were noted.Results: In total, 250 patients were follow-up with B-ALL and 43 patients with T-ALL. 64% (n=188) of patients were male and 36% (n=105) were female. In our study, 36.4% of patients were in the high risk group but ten of the patients died before the risk group could be determined. During the two-year treatment, We found that 83% of the patients developed at least one endocrine complication. Hyperglycemia, osteoporosis and avascular necrosis were observed more frequently in patients older than 10 years at the time of diagnosis. The difference between the sexes was found only in vitamin D and was lower in girls. In regression analysis, only to be high risk group were found to be effective for the development of endocrine complications.Conclusion: Although the chemotherapeutics used today have prolonged the survival time, they have caused an increase in the incidence of complications. We think that these complications can be reduced by determining the risk factors in advance.
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    Identification and in silico characterization of a novel PKLR genotype in a Turkish newborn
    (Springer, 2020-09-09) Canu, Giulia; De Paolis, Elisa; Righino, Benedetta; Mazzuccato, Giorgia; De Paolis, Giulio; Capoluongo, Ettore; De Rosa, Maria Cristina; Urbani, Andrea; Güneş, Adalet Meral; Minucci, Angelo; MERAL GÜNEŞ, ADALET; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Hematoloji Anabilim Dalı.; EXD-8400-2022
    Pyruvate kinase deficiency (PKD) is the most common glycolytic defect leading to chronic nonspherocytic hemolytic anemia (CNSHA). Clinical manifestations of PKD reflect the symptoms and complications of the chronic hemolysis, including anemia, jaundice, bilirubin gallstones due to hyperbilirubinemia, splenomegaly and iron overload. In this study, we report the finding of a 5-months-old Turkish male newborn with moderate CNSHA and PKD. Mutation screening ofPyruvate Kinase Liver/Red(PKLR)gene revealed that the patient carried the known pathogenic variant (PV)c.1456C > T(p.Arg486Trp) and an unreported variantc.1067T > G(p.Met356Arg). Computational variant analysis (CVA) highlighted the deleterious structural effects on the mutant PK enzyme, suggesting its pathogenic role. In this patient, the molecular evaluation of PKD, that allowed the identification of thenovel PKLRgenotype, coupled with CVA led to the definitive and correct diagnosis of CNSHA.
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    Healthcare-associated infections in the department of pediatric hematology-oncology; a single center evaluation
    (Galenos Publishing House, 2024-04) Özdel, Zeynep Gizem Ergün; Çelebi, Solmaz; Güneş, Adalet Meral; Evim, Melike Sezgin; Sevinir, Betül Berrin; Baytan, Birol; Demirkaya, Metin; Köse, Serdal Kenan; Çetin, Benhur; Çelik, Taylan; Salı, Enes; Hacımustafaoğlu, Mustafa; ERGÜN ÖZDEL, ZEYNEP GİZEM; ÇELEBİ, SOLMAZ; MERAL GÜNEŞ, ADALET; SEZGİN EVİM, MELİKE; SEVİNİR, BETÜL BERRİN; HACIMUSTAFAOĞLU, MUSTAFA KEMAL; Bursa Uludağ Üniversitesi/Tıp Fakültesi; H-2691-2017
    Introduction: With advances in the treatment of Pediatric Hematology -Oncology (PHO) patients, the survival of patients are increasing day by day. However, Healthcare -Associated Infections (HAI) is still a significant cause of mortality and morbidity in this group of patients. This study aimed to evaluate the rates of HAI in patients who were hospitalized in Uludag University Medical Faculty PHO Clinic during the 4 -year study period. Materials and Methods: HAI was diagnosed according to the CDC (Center for Disease Control and Prevention) 2008 criteria. We recorded the number of patients admitted, the number of patients with HAI, the total number of HAI episodes, and the length of stay in hospital. We calculated HAI rate and HAI density (per 1000 patient -days). Results: During the four years, 3069 hospitalizations were recorded in 607 PHO patients (5.05 admissions per patient). 38.6% of the patients were female, and 61.4% male. The mean age was 100.8 +/- 63.6 months. The mean duration of hospitalization was 14 +/- 17.16 days. A total of 232 HAI episodes were recorded in 141 hospitalizations. HAI rate was 7.5%, and HAI density was 5.36/1000 patient -days. Conclusion: The HAI rates in our PHO unit were comparable to those of developed countries and lower than those of developing countries and prior results from our country.
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    Congenital malformation in children with acute leukemia: Single center report
    (Bursa Uludağ Üniversitesi, 2021-04-01) Güler, Salih; Temuroglu, Aytul; Evim, Melike Sezgin; Baytan, Birol; Güneş, Adalet Meral; GÜLER, SALİH; TEMUROĞLU, AYTÜL; SEZGİN EVİM, MELİKE; Baytan, Birol; MERAL GÜNEŞ, ADALET; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Hematoloji Anabilim Dalı; 0000-0002-8943-6585; 0000-0002-2398-0959; 0000-0002-4792-269X; 0000-0002-0686-7129; JHO-2788-2023; GES-3112-2022; AAH-1452-2021; DVW-8108-2022; JGX-6145-2023
    Introduction:Leukemia is a multifactorial disease. Some genetic syndromes is well known related to leukemia. We evaluated non-syndromic malformation and leukemia relation.Materials and Methods: 288 patients diagnosed with acute leukemia are included the study. 201 patients with non-malign hematologic disease are accepted as a control. Syndromic children were excluded both group. All children were examined according to ICD-10th, Chapter XVII for congenital malformation. The type and number 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<0.001). The most common malformation in the control group was on the skin. Whereas the most common malformation in leukemic children was seen in the circulatory system, second region was skin. Having circulatory system malformation explained 12.53 high of the leukemia risk.Conclusions: Malformations were more common in leukemic children. We found that having a circulatory system malformation significantly increased the risk of leukemia. But the risk was very high previous study. This related to we evaluated echocardiography result which is common use for basal test.
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    Deferasirox in children with transfusion-dependent thalassemia or sickle cell anemia: A large cohort real-life experience from turkey (reach-them)
    (Wiley, 2019-02-01) Antmen, Bülent; Karakaş, Zeynep; Yeşilipek, Mehmet Akif; Küpesiz, Osman Alphan; Şaşmaz, Ilgen; Uygun, Vedat; Kurtoğlu, Erdal; Oktay, Gönül; Aydogan, Gönül; Akın, Mehmet; Salcıoğlu, Zafer; Vergin, Canan; Kazancı, Elif Güler; Ünal, Selma; Çalışkan, Ümran; Aral, Yusuf Ziya; Türkkan, Emine; Güneş, Adalet Meral; Tunc, Bahattin; Gümrük, Fatma; Ayhan, Aylin Canbolat; Söker, Murat; Koç, Ahmet; Oymak, Yeşim; Ertem, Mehmet; Timur, Çetin; Yıldırmak, Yıldız; İrken, Gülersu; Apak, Hilmi; Biner, Betül; Eren, Tuğba Gürleyen; Balcı, Yasemin Işik; Koçak, Ulker; Karasu, Gulsun; Akkaynak, Diyar; Patıroğlu, Türkan; MERAL GÜNEŞ, ADALET; Uludağ Üniversitesi/Tıp Fakültesi; JGX-6145-2023
    Objectives To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (>= 100 mL/kg of pRBC or a serum ferritin [SF] level >1000 mu g/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 mu g/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 mu g/L), SCA (1655.5 to 1260 mu g/L), and across age groups of 2-6 years (1971.5 to 1499 mu g/L), 7-12 years (1688.5 to 1159.8 mu g/L), and 13-18 years (1496.5 to 1107 mu g/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses >= 30 mg/kg/d (n = 120, -579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. Conclusions Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (>= 30 mg/kg/d) may be required to achieve iron balance.