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  1. Home
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Browsing by Author "Yildirim, AT"

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    Successful Treatment of a Child with Hemoglobin Hammersmith with Hematopoietic Stem Cell Transplantation
    Yildirim, AT; Gülen, H; Türkmen, H; Özek, G; Oymak, Y; Durmaz, B; Karaca, E
    Hemoglobin (Hb) Hammersmith, formed by serine substitution for phenylalanine at residue 42 in the beta-globin chain, is a very rare variant of unstable hemoglobin with low oxygen affinity. For patients with hemoglobinopathies, it is well-established that hematopoietic stem cell transplantation provides a complete cure, but the literature on its role for those with Hb Hammersmith is limited. A seven-month-old girl who was examined for anemia and splenomegaly was followed up for congenital hemolytic anemia. The patient with visible cyanosis of the lips and whose p50 was low in blood gas was diagnosed with Hb Hammersmith through the DNA sequence analysis. During the follow-up, frequent blood transfusions had to be given due to anemia aggravated by infections. Following a successful hematopoietic stem cell transplant from an HLA-matched sibling, the patient completely recovered from Hb Hammersmith. The case is presented because of its rarity.
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    Typical hematological findings facilitating the diagnosis of sitosterolemia
    Gülen, H; Yildirim, AT; Yigit, Y; Yorulmaz, A
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    Transfusion-related Acute Lung Injury: A Case Report
    Polat, AÇ; Yigit, Y; Önder, ENA; Yildirim, AT; Ertan, P; Gülen, H
    Transfusion-related acute lung injury (TRALI) is a non-cardiac pulmonary edema presenting with severe dyspnea, hypoxia and bilateral diffuse infiltrates on chest X-ray. It usually occurs within the first 6 hours following the administration of plasma and plasma rich blood products. Herein, we present a patient who developed TRALI shortly after plasmapheresis due to Hemolytic Uremic syndrome and exhibited a dramatic response with early systemic steroid treatment.
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    Neutropenic Fever in a Two-and-a-Half Month Old Girl: Severe Congenital Neutropenia
    Gülen, H; Öçalan, M; Yildirim, AT; Çam, FS; Çelebi, HBG
    Severe congenital neutropenia is a hereditary disease characterized by a low number of neutrophils occurring from the first months of life leading to severe infections. It is rare but threatens life because of severe infections. For this reason, early diagnosis and treatment of this disease is important. Here, we present a case of a two and a half month old girl who was admitted to our clinic with fever and absolute neutropenia was determined in her clinical course.
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    EPISODE OF ACUTE HEMOLYSIS DUE TO UNDIAGNOSED GLUCOSE-6-PHOSPHATE DEHIDROGENASE DEFICIENCY IN AN ADOLESCENT WITH NEWLY DIAGNOSED TYPE 1 DIABETES MELLITUS: CASE REPORT AND REVIEW OF LITERATURE
    Goren, TA; Kilimci, DD; Yigit, Y; Yildirim, AT; Gulen, H; Ersoy, B
    Glucose-6-phosphate dehydrogenase (G6PD) enzyme deficiency is common in the community. The most important clinical manifestation of G6PD deficiency is acute hemolytic anemia due to oxidative stressors. Diabetes Mellitus (DM) can precipitate hemolysis in patients with G6PD deficiency. Here, we described a 15-year-old male with newly diagnosed type 1 DM (T1DM) and unknown G6PD deficiency who suffered from hemolytic anemia during normalization of blood glucose. On admission, the patient did not have ketoacidosis. After the patient's blood sugars were regulated with insulin therapy, he presented five days later with hemolytic anemia. The cause of hemolytic anemia was G6PD deficiency. The patient had no previous episodes of hemolysis and had no relevant family history. Hypoglycemia did not occur during blood glucose regulation. The return of blood sugar to normal after a long period of hyperglycemia was thought to be the possible cause of hemolysis. In conclusion, G6PD deficiency should be considered when there is an episode of hemolysis in newly diagnosed children and adolescents with T1DM, especially in the absence of ketoacidosis and hypoglycemia.
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    Wernicke's encephalopathy in a child with Down syndrome, undergoing treatment for acute lymphoblastic leukemia
    Yildirim, AT; Bilgili, G; Akman, B; Ovali, GY; Özgüven, AA; Gülen, H
    Wernicke's encephalopathy (WE), the early diagnosis of which is crucial to prevent permanent sequelae, is an acute neurologic disorder that develops due to thiamine deficiency, and which is associated with considerable morbidity and mortality. A 10-year-old boy diagnosed with Down syndrome and acute lymphoblastic leukemia had severe vomiting and impaired consciousness following methotrexate treatment at a dose of 1 g/m(2). Biochemistry was consistent with the syndrome of inappropriate anti-diuretic hormone secretion. Level of consciousness did not improve following meticulous correction of hyponatremia, therefore cranial magnetic resonance imaging was done, which indicated specific findings consistent with WE. It should be kept in mind that Down syndrome patients are more likely to incur toxic side-effects of chemotherapeutic agents, and thus neurological complications such as WE may occur.
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    A Rare Complication in a Patient with Acute Promyelocytic Leukemia; ATRA and Posaconazole Associated Hypercalcemia
    Yigit, Y; Demiral, M; Yildirim, AT; Gülen, H
    All-trans retinoic acid (ATRA), a derivative of vitamin A, has dramatically altered the treatment landscape for acute promyelocytic leukemia (APL). APL is characterized by the abnormal maturation of myeloid cells, which become arrested at the promyelocyte stage. ATRA effectively induces these cells to differentiate and undergo apoptosis. While generally well-tolerated, ATRA has been associated with rare adverse effects, including hypercalcemia. This case report underscores the importance of vigilant monitoring for ATRA-related side effects, especially when combined with medications which inhibit cytochrome P450 enzymes. Antifungal prophylaxis is common during leukemia treatment. Here, we present a rare instance of hypercalcemia in a pediatric patient attributed to the concurrent use of posaconazole and ATRA. A 15-year-old girl presented with widespread bruising, abnormal uterine bleeding, and pancytopenia. Subsequent investigations led to an APL diagnosis. Classified as standard-risk APL, she received chemotherapy according to the acute myelogenous leukemia-Berlin-Frankfurt-M & uuml;nster 2004 protocol. After an ATRA course was started in the third month of maintenance treatment, she applied to the hospital with constitutional symptoms of weakness and fatigue on the third day of treatment. In the biochemical tests of the patient, serum Ca concentration was determined to be 16.5 mg/dL. Parathormone was 64.3 pg/mL and the 25-OH D vitamin level was 22 ng/mL and so were within the normal limits. Complete blood count was within the normal range. Although hypercalcemia is a side effect seen in the combined use of ATRA and azole antifungals, to the best of our knowledge, this is the first report in the literature that it was observed in the pediatric age group due to the simultaneous use of posaconazole and ATRA.
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    The Wolf in Sheep's Clothing Leishmania tropica: : Two Pediatric Visceral Cases
    Gülen, H; Yildirim, AT; Çavus, I; Türkmen, H; Özgüven, E; Özbilgin, A
    According to the World Health Organization (WHO), leishmaniasis is a zoonotic/anthroponotic parasitic disease endemic in 99 countries. It is estimated that approximately 12 million people are infected with Leishmania spp. and 350 million people live at risk. Every year, two million new cases are added to these figures. One and a half million cases of zoonotic/anthroponotic cutaneous leishmaniasis and 500 000 cases of visceral leishmaniasis are reported annually. One person is estimated to to be infected with cutaneous leishmaniasis in every 20 seconds and visceral leishmaniasis causes 60 000 deaths. In this report, two pediatric cases diagnosed with visceral leishmaniasis were presented. In the study, bone marrow aspirations were performed to determine the etiology of the disease in an eight-month-old male patient with fever and hepatosplenomegaly who had been followed up in Manisa Celal Bayar University, Department of Pediatrics, Division of Pediatric Hematology with the diagnosis of severe glucose-6-phosphate dehydrogenase (G-6PD) deficiency since the neonatal period and in a nine-month-old female patient who had had a high fever and bicytopenia for two weeks. Bone marrow aspirations were cultured in NNN medium and their smears were stained and examined with Giemsa. rk-39 and Leishmania IFAT tests were performed by using patients' sera. Quantitative reverse transcriptase polymerase chain reaction (RT-qPCR) analysis was also performed for Leishmania spp. Leishmania spp. amastigotes were observed in Giemsa-stained smear preparations, Leishmania spp. promastigotes were grown in NNN medium, rk39 rapid diagnostic kit was weakly positive, Leishmania IFAT was positive at a titer of 1/1024 and Leishmania tropica was identified as the causative agent by RT-qPCR analysis for both cases. These two cases suggested that fatal cases of visceral leishmaniasis may increase with the spread of visceralized isolates of L.tropica, , the most common causative agent of cutaneous leishmaniasis in T & uuml;rkiye, and this issue may create a significant public health problem.
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    Sweet Syndrome due to G-CSF Use in Children with Acute Lymphoblastic Leukemia: Case Report
    Yildirim, AT; Gülen, H; Yigit, Y; Temiz, P
    Sweet syndrome is a rarely seen disease characterized with fever, arthrolgia, neutrophilic leukocytosis and painful erythematous plaques, and dense neutrophilic inifltration in dermis at lesion sites which occur mostly on the face and upper extremities. A nine-year-old girl with a diagnosis of acute lymphoblastic leukemia was admitted to the hospital with high fever. Empirical broad spectrum antibiotic and granulocyte-colony stimulating factor (G-CSF) treatments were initiated for febrile neutropenia. Although antifungal therapy was added to the treatment during follow-up, hyperfebrile state could not be resolved. On the 14th day of G-CSF treatment, the result of the skin biopsy performed from red, painful eruptions seen on the legs was compatible with Sweet syndrome. G-CSF treatment was discontinued and intravenous steroids were started wit resultant control of fever, and resolution of the rash Sweet syndrome should be kept in mind when erythematous rashes develop in patients with febrile neutropenia who have been using G-CSF for a long time and who have not been able to control their fever despite appropriate antibiotics and antifungals.
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    Candida Infection During Successful Treatment of Mucor Infection Through Antifungal and Minimal Invasive Surgery in a Child with Acute Lymphoblastic Leukemia
    Yildirim, AT; Gülen, H; Günhan, K; Nese, N; Özkol, M; Degerli, K
    Invasive mucormycosis is a fungal infection that is rare but has a high mortality rate. It is often seen in immune supressed patients with diabetic ketoacidosis, hematologic malignancy, or those that have undergone stem cell transplantation. Febrile neutropenia, swelling of the right cheek, and a necrotic area in the palate developed during the induction chemotherapy of a three year-old male patient with acute lymphoblastic leukemia. Minor debridements and antifungal treatment was applied, and in the sixth month of the treatment, softening of the right maxillary bone was detected in the same area. From the biopsy sample, Candida tropicalis grew in the culture, and was treated with combined antifungal medicines. This case is presented to emphasize the feasibility of mucor infection treatment via minor debridements and antifungal medicines without any need for major surgery, and also to emphasize that breaktrough fungal infections should not be ignored, in spite of the antifungal medicines that are highly effective and have broad-spectrum.
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    Sirolimus is effective and safe in childhood relapsed-refractory autoimmune cytopenias: A multicentre study
    Acar, SO; Tahta, N; Al, IO; Erdem, M; Gözmen, S; Karapinar, TH; Kilinç, B; Celkan, T; Kirkiz, S; Koçak,Ü; Ören, H; Yildirim, AT; Arslantas, E; Ayhan, AC; Oymak, Y
    Autoimmune cytopenias are a heterogeneous group of disorders characterized by immune-mediated destruction of haematopoietic cell lines. Effective and well-tolerated treatment options for relapsed-refractory immune cytopenias are limited. In this study, the aim was to evaluate the efficacy and safety of sirolimus in this disease group within the paediatric age group. The study enrolled patients in the paediatric age group who used sirolimus with a diagnosis of immune cytopenia between December 2010 and December 2020, followed at six centres in Turkey. Of the 17 patients, five (29.4%) were treated for autoimmune haemolytic anaemia (AIHA), six (35.2%) for immune thrombocytopenic purpura (ITP) and six (35.2%) for Evans syndrome (ES). The mean response time was 2.7 months (range, 0-9 months). Complete response (CR) and partial response (PR) were obtained in 13 of 17 patients (76.4%) and nonresponse (NR) in four patients (23.5%). Among the 13 patients who achieved CR, three of them were NR in the follow-up and two of them had remission with low-dose steroid and sirolimus. Thus, overall response rate (ORR) was achieved in 12 of 17 patients (70.5%). In conclusion, sirolimus may be an effective and safe option in paediatric patients with relapsed-refractory immune cytopenia. In a retrospective multicentre study of 17 cases (11 boys and 6 girls) who were receiving sirolimus for immune cytopenia were analysed. We found that complete response (CR) and partial response (PR) were obtained in 13 of 17 patients (76.4%) and nonresponse (NR) in 4 patients (23.5%). Overall response rate (ORR) was achieved in 12 of 17 patients (70.5%).image
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    Isolated Optic Nerve Relapse in Acute Lymphoblastic Leukemia: Two Case Reports
    Yildirim, AT; Oymak, Y; Yaman, Y; Saatçi, O; Malatyali, R; Kamer, S; Alper, H; Vergin, C
    Notwithstanding extensive chemotherapy, intrathecal methotrexate treatments and prophylactic central nervous system radiotherapy, extramedullary relapses can still be seen in leukemia patients. Isolated optic nerve relapses, one of its symptoms being a sudden vision loss that occurs prior to a bone marrow relapse, are rarely seen in acute lymphoblastic leukemia patients. Sudden vision loss can be cured by an early diagnosis and rapid treatment. In this study, we investigate two male patients (10 and 12 years old) with optic nerve relapses, sudden vision loss complaints and pre-T acute lymphoblastic leukemia in the light of the literature.
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    Successful management of delayed-onset adenosine deaminase deficiency with novel mutation
    Çelik, FÇ; Soyöz, Ö; Bölük, SÖ; Taskirdi, I; Haci, IA; Kaya, MS; Demir, A; Uzunoglu, B; Yildirim, AT; Onay, H; Gözmen, S; Gülez, N; Genel, F
    A 4-year-old boy presented with acute-onset autoimmune cytopenia with severe, persistent lymphopenia, autoimmune thyroiditis, elevated IgE and glucose 6-phosphate dehydrogenase enzyme deficiency. In immunologic evaluation, lower T, B and natural killer cells and higher levels of adenosine deaminase (ADA) metabolites were observed. The compound heterozygous novel ADA gene mutations causing ADA deficiency were detected. Successful immunologic and metabolic cure was achieved with enzyme replacement therapy, followed by reduced intensity conditioning hematopoietic stem cell transplantation from a matched unrelated donor. An interesting aspect of this patient is the detection of novel compound heterozygous mutations without consanguinity and a secondary outcome is the recovery of glucose 6-phosphate dehydrogenase deficiency after hematopoietic stem cell transplantation.
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    Congenital amegakaryocytic thrombocytopenia: three case reports from patients with different clinical diagnoses and somatic abnormalities
    Yildirim, AT; Günes, BT; Oymak, Y; Yaman, Y; Özek, G; Carti, Ö; Yesilipek, A; Vergin, C
    The congenital amegakaryocytic thrombocytopenia (CAMT) is a syndrome characterized by preservation of granulocytic and erythroid cells during genesis, with a gradual or progressive decrease in the number of megakaryocytic series of cells in the bone marrow. At later times, most patients develop aplastic anemia. It is important to rule out specific causes of thrombocytopenia that develop in the early stages of CAMT. Typically, there are no specific somatic abnormalities that accompany this deadly disease. Here we present three CAMT cases that presented with different clinical diagnoses, with various physical anomalies in two of those cases. The first patient was examined because of a cytomegalovirus infection. The second patient had been referred with a suspected neonatal alloimmune thrombocytopenia, whereas the third patient presented with chronic immune thrombocytopenic purpura. Subsequently, all three patients were diagnosed with CAMT. Two of the patients had physical anomalies. In particular, the first patient had a duplex urinary system. To our knowledge, this is the first patient with CAMT to have a duplicated collecting sysem. The second patient had a secundum atrial septal defect, an atypical facial appearance, and growth retardation. Since CAMT could also be observed outside the neonatal period, the differential diagnosis for thrombocytopenia should be considered for all age groups. Moreover, it should be considered that CAMT may also be accompanied with somatic abnormalities. Blood Coagul Fibrinolysis 26: 337-341 Copyright (C) 2015 Wolters Kluwer Health, Inc. All rights reserved.
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    Common viral respiratory infections in children with cancer during the COVID-19 pandemic: a multicenter study from Türkiye
    Kaçar, D; Kebudi, R; Özyörük, D; Tugcu, D; Bahadir, A; Özdemir, ZC; Özgüven, AA; Orhan, MF; Yildirim, AT; Albayrak, C; Kartal, I; Sari, N; Tokgöz, H; Albayrak, M; Ayhan, AC; Eroglu, N; Aydin, S; Üzel, VH; Zülfikar, B; Yildirim, ÜM; Büyükavci, M; Gülen, H; Töret, E; Bör,Ö; Özbek, NY; Ilhan, IE; Yarali, N
    Background. Microbiologic confirmation of respiratory tract infections gained importance during the coronavirus disease 2019 (COVID-19) pandemic. This study retrospectively evaluated seasonal distribution, clinical presentation, and complications of respiratory viral infections (RVIs) other than COVID-19 in children with cancer during and after the pandemic lockdown. Methods. Two hundred and sixty-five inpatient and outpatient RVI episodes in 219 pediatric cancer patients confirmed by multiplex reverse transcriptase polymerase chain reaction (RT-PCR) panels from 13 centers were enrolled. Results. Eighty-six (32.5%) of the total 265 episodes occurred in 16 months corresponding to the lockdowns in T & uuml;rkiye, and the remaining 67.5% in 10 months thereafter. Human rhinovirus/enterovirus (hRE) (48.3%) was the most common agent detected during and after lockdown. Parainfluenza virus (PIV) (23.0%), influenza virus (9.8%), and respiratory syncytial virus (RSV) (9.1%) were the other common agents. The 28.7% of episodes were lower respiratory tract infections (LRTIs), and complications and mortality were higher than upper respiratory tract infections (URTIs) (25.0% vs 5.3%). Bacteremia was identified in 11.5% of culture-drawn episodes. Treatment delay in one-third and death within four weeks after RVI in 4.9% of episodes were observed. Conclusion. During the pandemic, fewer episodes of RVIs occurred during the lockdown period. Respiratory viruses may cause complications, delays in treatment, and even death in children with cancer. Therefore, increased awareness of RVIs and rapid detection of respiratory viruses will benefit the prevention and, in some cases, abrupt supportive and some antiviral treatment of RVI in children with cancer.
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    Thrombolysis with Systemic Recombinant Tissue Plasminogen Activator in Children: A Multicenter Retrospective Study
    Zengin, E; Sarper, N; Erdem, AY; Al, IO; Evim, MS; Yarali, N; Belen, B; Akçay, A; Yildirim, AT; Karapinar, TH; Günes, AM; Gelen, SA; Ören, H; Olcay, L; Baytan, B; Gülen, H; Öztürk, G; Orhan, MF; Oymak, Y; Akpinar, S; Tüfekçi, Ö; Albayrak, M; Günen, BT; Canpolat, A; Özbek, N
    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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    NONCOVID-19 VIRAL RESPIRATORY INFECTIONS IN CHILDREN WITH CANCER DURING THE COVID-19 PANDEMIC: A MULTICENTER OBSERVATIONAL STUDY
    Kebudi, R; Kacar, D; Ozyoruk, D; Tugcu, D; Yarali, N; Ilhan, I; Bahadir, A; Ozdemir, Z; Ozguven, A; Orhan, MF; Yildirim, AT; Albayrak, C; Kartal, I; Ozbek, NY; Sari, N; Tokgoz, H; Albayrak, M; Aydin, AC; Eroglu, N; Aydin, S; Uzel, H; Zulfikar, B; Buyukavci, M; Gulen, H; Toret, E; Bor, O
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    Genetic basis and hematologic manifestations of sitosterolemia in a group of Turkish patients
    Kaya, Z; Sal, E; Yorulmaz, A; Hsieh, YP; Gülen, H; Yildirim, AT; Niu, DM; Tekin, A
    BACKGROUND: Sitosterolemia is a rare lipid disorder caused by mutations in adenosine triphosphatebinding cassette genes ( ABCG ) 5 and 8. OBJECTIVE: To evaluate the phenotypic/genotypic features of sitosterolemia in a group of Turkish patients. METHODS: Seven probands with unexplained hematologic abnormalities and their 13 relatives were enrolled. Sterol levels were measured by gas chromatography and genetic studies were performed using Sanger sequencing. Individuals were diagnosed with sitosterolemia if they were found to have frankly elevated sitosterol level > 15 mu g/mL and/or pathogenic variants of the ABCG 5/ ABCG 8. RESULTS: The seven probands and their six relatives were diagnosed with frank sitosterolemia, and all these patients had hematologic abnormalities. The remaining seven relatives were asymptomatic heterozygous carriers. Three novel variants in the ABCG 5 gene (c.161G > A , c.1375C > T , IVS10-1G > T ), one novel variant in the ABCG 8 gene (c.1762G > C ) and one known variant in the ABCG 5 gene (c.1336 C > T ) were identified. No variant was identified in one case. The mean sitosterol level was significantly higher and mean platelet count was significantly lower in patients with homozygous variants compared to heterozygous variants ( p < 0.05, for all). Diets low in plant sterols were recommended for 13 symptomatic cases. Four homozygotes received ezetimibe, and their splenomegaly, anemia, and thrombocytopenia completely resolved except one. CONCLUSION: The five pathogenic variants identified in this study indicate the genetic heterogeneity of sitosterolemia in Turkish population. Patients with unexplained hematologic abnormalities (specifically macrothrombocytopenia) should have their sterol level measured as initial testing. Ezetimibe can be a good choice for sitosterolemia. (c) 2021 National Lipid Association. Published by Elsevier Inc. All rights reserved.
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    The role of prothrombotic factors in children with hemiplegic cerebral palsy
    Yildirim, AT; Sütçü, R; Köroglu, M; Delibas, N; Kisioglu, N; Akar, N; Ilhan, IE
    Aim. Hemiplegic cerebral palsy (HCP) is a condition occurring as a consequence of a non-progressive damage of the brain with incomplete anatomical and physical development during the early period of life. Its etiology is multifactorial, with the cause remaining unexplained in the majority of cases. This study aims to investigate whether thrombophilic factors correlates with the etiology in children with HCP. Methods. We included 36 children with HCP in the patient group, and 41 healthy children with no neurologic disorders in the control group. No significant difference was found between the two groups in terms of factor V leiden, methylenetetrahydrofolate reductase and prothrombin 20210A mutation frequency and protein C, protein S and antithrombin III levels. Results. Homocysteine levels were significantly higher in the group of patients with HCP as compared to the control group (P=0.012). Because we could not identify the origin of hyperhomocysteinemia as congenital or acquired, the impact of hyperhomocysteinemia on HCP was considered insignificant. Each thrombophilic disorder was assessed in terms of relatedness to atrophy, periventricular leukomalacia, infarct, congenital anomaly and porencephalic cyst, respectively. No significant correlation was detected between thrombophilic disorders and cranial imaging findings. Conclusion. Our study has shown that thrombophilic factors are not involved in the etiology of HCP.
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    Central nervous system thrombosis in pediatric acute lymphoblastic leukemia in Turkey: A multicenter study
    Guzelkucuk, Z; Karapinar, DY; Gelen, SA; Tokgoz, H; Ozcan, A; Ay, Y; Bahadir, A; Ozbek, NY; Oren, AC; Ayhan, AC; Akyay, A; Akinci, B; Karadas, N; Unuvar, A; Oren, H; Fettah, A; Kaya, Z; Isik, B; Eker, I; Karaman, S; Yildirim, AT; Orhan, MF; Oymak, Y; Timur, C; Yazici, N; Simsek, A; Karakurt, N; Toret, E; Evim, MS
    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 Turkiye. 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 were determined. ResultsData from 70 patients with CNS thrombosis during treatment, out of 3968 pediatric patients with ALL, were reviewed. The incidence of CNS thrombosis was 1.8% (venous: 1.5 %; arterial: 0.03%). Among patients with CNS thrombosis, 47 had the event in the first 2 months. Low molecular weight heparin (LMWH) was the most commonly used treatment with a median of 6 months (min-max: 3-28 months). No treatment-related complications occurred. Chronic thrombosis findings occurred in four patients (6%). In five (7%) patients who developed cerebral vein thrombosis, neurological sequelae (epilepsy and neurological deficit) remained. One patient died related to thrombosis, and the mortality rate was 1.4%. ConclusionCerebral venous thrombosis and, less frequently, cerebral arterial thrombosis may develop in patients with ALL. The incidence of CNS thrombosis is higher during induction therapy than during other courses of treatment. Therefore, patients receiving induction therapy should be monitored carefully for clinical findings suggestive of CNS thrombosis.
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