Full Text Journal Articles by
Author Tekin Aksu

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A Patient With AIRE Mutation Who Presented With Severe Diarrhea and Lung Abscess.

Elif Soyak Aytekin, Oguzhan Serin, Deniz Cagdas, Cagman Tan, Tekin Aksu, Yagmur Unsal, Selma Yeni, Diclehan Orhan, Zeynep Alev Ozon, Ilhan Tezcan,

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) (polyglandular endocrinopathy type 1) is a rare autosomal recessive disorder caused by mutations in the autoimmune regulator gene (AIRE). The major clinical features of APECED are hypoparathyroidism, adrenal insufficiency (Addison disease), and chronic mucocutaneous candidiasis. This disease is also associated with multiple other and uncommon autoimmune ... Read more >>

Pediatr Infect Dis J (The Pediatric infectious disease journal)
[2021, 40(1):66-69]

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Allogeneic hematopoietic stem cell transplantation in leukocyte adhesion deficiency type I and III

Shahrzad Bakhtiar, Emilia Salzmann-Manrique, Henric-Jan Blok, Dirk-Jan Eikema, Sheree Hazelaar, Mouhab Ayas, Amos Toren, Gal Goldstein, Despina Moshous, Franco Locatelli, Pietro Merli, Gerard Michel, Gülyüz Öztürk, Ansgar Schulz, Carsten Heilmann, Marianne Ifversen, Rob F. Wynn, Olga Aleinikova, Yves Bertrand, Abdelghani Tbakhi, Paul Veys, Musa Karakukcu, Alphan Kupesiz, Ardeshir Ghavamzadeh, Rupert Handgretinger, Emel Unal, Antonio Perez-Martinez, Muge Gokce, Fulvio Porta, Tekin Aksu, Gülsün Karasu, Isabel Badell, Per Ljungman, Elena Skorobogatova, Akif Yesilipek, Tsila Zuckerman, Robbert R. G. Bredius, Polina Stepensky, Bella Shadur, Mary Slatter, Andrew R. Gennery, Michael H. Albert, Peter Bader, Arjan Lankester,

Key Points We observed excellent survival in LAD-I and -III after allo-HSCT from 10/10 HLA-matched sibling, nonsibling family, or unrelated donor. Acute GVHD remains a relevant complication, affecting patient’s outcome. Type I and III leukocyte adhesion deficiencies (LADs) are primary immunodeficiency disorders resulting in early death due to infections and ... Read more >>

Blood Adv (Blood advances)
[2021, 5(1):262-273]

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Prognostic factors for survival in children who relapsed after allogeneic hematopoietic stem cell transplantation for acute leukemia.

Volkan Hazar, Gülsün Tezcan Karasu, Gülyüz Öztürk, Alphan Küpesiz, Serap Aksoylar, Namık Özbek, Vedat Uygun, Talia İleri, Fatma Visal Okur, Ülker Koçak, Suar Çakı Kılıç, Arzu Akçay, Elif Güler, Savaş Kansoy, Musa Karakükcü, İbrahim Bayram, Tekin Aksu, Akif Yeşilipek, Barbaros Şahin Karagün, Şebnem Yılmaz, Mehmet Ertem, Duygu Uçkan, Tunç Fışgın, Orhan Gürsel, Yöntem Yaman, Ceyhun Bozkurt, Müge Gökçe, ,

<h4>Background</h4>Post-transplant relapse has a dismal prognosis in children with acute leukemia undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Data on risk factors, treatment options, and outcomes are limited.<h4>Procedure</h4>In this retrospective multicenter study in which a questionnaire was sent to all pediatric transplant centers reporting relapse after allo-HSCT for a cohort ... Read more >>

Pediatr Transplant (Pediatric transplantation)
[2020, :e13942]

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Central nervous system lesions in Fanconi anemia: Experience from a research center for Fanconi anemia patients.

Tekin Aksu, Fatma Gümrük, Turan Bayhan, Çağrı Coşkun, Kader K Oğuz, Sule Unal,

<h4>Background</h4>Brain atrophy, abnormal pituitary morphology, corpus callosum, and posterior fossa abnormalities have been described in patients with Fanconi anemia (FA). We aimed to provide an overview of cranial neuroimaging findings and to evaluate the clinical implications in FA patients.<h4>Procedure</h4>Cranial magnetic resonance imaging (MRI) studies of 34 patients with FA were ... Read more >>

Pediatr Blood Cancer (Pediatric blood & cancer)
[2020, 67(12):e28722]

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Comment on: Clinical, cytogenetic, and molecular analyses of 17 neonates with transient abnormal myelopoiesis and nonconstitutional trisomy 21.

Tekin Aksu, Fatma Gümrük, Şule Ünal,

Pediatr Blood Cancer (Pediatric blood & cancer)
[2020, 67(11):e28289]

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Role of a second transplantation for children with acute leukemia following posttransplantation relapse: a study by the Turkish Bone Marrow Transplantation Study Group.

Volkan Hazar, Gülsün Tezcan Karasu, Vedat Uygun, Namık Özbek, Musa Karakükçü, Gülyüz Öztürk, Hayriye Daloğlu, Suar Çakı Kılıç, Tekin Aksu, Ekrem Ünal, Ülker Koçak, Akif Yeşilipek, Arzu Akçay, Orhan Gürsel, Alphan Küpesiz, Fatma Visal Okur, Talia İleri, Savaş Kansoy, İbrahim Bayram, Barbaros Şahin Karagün, Müge Gökçe, Zühre Kaya, İkbal Ok Bozkaya, Türkan Patıroğlu, Serap Aksoylar,

We examined outcomes of 51 pediatric patients with relapsed acute leukemia (AL) who underwent a second allogeneic hematopoietic stem cell transplantation (alloHSCT). After a median follow-up of 941 days (range, 69-2842 days), leukemia-free survival (LFS) and overall survival (OS) at 3 years were 26.6% and 25.6%, respectively. The nonrelapse mortality rate (NMR) and ... Read more >>

Leuk Lymphoma (Leukemia & lymphoma)
[2020, 61(6):1465-1474]

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Hb H Disease Diagnosed During Adolescent Pregnancy.

Tekin Aksu, Çağrı Coşkun, Barış Kuşkonmaz, Şule Ünal, Selin Aytaç, Fatma Gümrük,

Hb H disease is a moderate to severe form of α-thalassemia (α-thal). Patients with Hb H disease may become symptomatic, especially during infections and pregnancy, and may require transfusions. Herein, we present a 16-year-old female with Hb H disease who was initially diagnosed during adolescent pregnancy and was found to ... Read more >>

Hemoglobin (Hemoglobin)
[2020, 44(2):137-138]

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The remarkable response to ponatinib therapy in a child with blastic phase of chronic myeloid leukemia.

Tekin Aksu, Şule Ünal, Fatma Gümrük,

<h4>Background</h4>Chronic myeloid leukemia (CML) rarely occurs in children and adolescents, which shows more aggressive features like high risk of more advanced disease at the time of diagnosis. Suboptimal response to tyrosine kinase inhibitors (TKIs), adverse events, or advanced disease may impede the treatment.<h4>Case</h4>Herein we present a nine-year-old chronic phase CML ... Read more >>

Turk J Pediatr (The Turkish journal of pediatrics)
[2020, 62(3):479-481]

Cited: 1 time

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Children with lymphoma presenting with hemophagocytic lymphohistiocytosis.

Tekin Aksu, Turan Bayhan, Belgin Gülhan, Selma Çakmakcı, Ayşe Selcen Oğuz, Neşe Yaralı, Namık Yaşar Özbek, İnci İlhan,

BACKGROUND:< 7b > Hemophagocytic lymphohistiocytosis (HLH) may precede malignancy, in particular lymphomas and leukemias. However, the causative factors, appropriate treatment and the prognosis of this association is not established. CASE:Herein, we present two patients, one with nodular sclerosing Hodgkin lymphoma (HL) and concomitant Epstein-Barr virus (EBV) infection, and the other ... Read more >>

Turk J Pediatr (The Turkish journal of pediatrics)
[2020, 62(2):284-288]

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A Case With Pyruvate Kinase Deficiency Remarkably Sensitive to Heat.

Tekin Aksu, Neşe Yarali, Elisa Fermo, Anna Marcello, Şadan Hacisalihoğlu, Paola Bianchi, Namik Y Özbek,

Pyruvate kinase (PK) deficiency is the most common defect of the glycolytic pathway leading to congenital hemolytic anemia. We present the case of an 18-year-old boy with chronic nonspherocytic hemolytic anemia, who had remarkable sensitivity to heat. Moreover, the patient showed clinical impairment in the last year. For this reason, ... Read more >>

J Pediatr Hematol Oncol (Journal of pediatric hematology/oncology)
[2018, 40(7):e458-e460]

Cited: 0 times

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Homozygous OB-fold variants in telomere protein TPP1 are associated with dyskeratosis congenita-like phenotypes.

Hemanth Tummala, Laura C Collopy, Amanda J Walne, Alicia Ellison, Shirleny Cardoso, Tekin Aksu, Nese Yarali, Deniz Aslan, Rüştü Fikret Akata, Juliana Teo, Zhou Songyang, Nikolas Pontikos, Jude Fitzgibbon, Kazunori Tomita, Tom Vulliamy, Inderjeet Dokal,

Blood (Blood)
[2018, 132(12):1349-1353]

Cited: 1 time

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Genome instability is a consequence of transcription deficiency in patients with bone marrow failure harboring biallelic ERCC6L2 variants.

Hemanth Tummala, Arran D Dokal, Amanda Walne, Alicia Ellison, Shirleny Cardoso, Saranha Amirthasigamanipillai, Michael Kirwan, Isobel Browne, Jasmin K Sidhu, Vinothini Rajeeve, Ana Rio-Machin, Ahad Al Seraihi, Andrew S Duncombe, Matthew Jenner, Owen P Smith, Helen Enright, Alice Norton, Tekin Aksu, Namık Yaşar Özbek, Nikolas Pontikos, Pedro Cutillas, Inderjeet Dokal, Tom Vulliamy,

Biallelic variants in the ERCC excision repair 6 like 2 gene (<i>ERCC6L2</i>) are known to cause bone marrow failure (BMF) due to defects in DNA repair and mitochondrial function. Here, we report on eight cases of BMF from five families harboring biallelic variants in <i>ERCC6L2</i>, two of whom present with ... Read more >>

Proc Natl Acad Sci U S A (Proceedings of the National Academy of Sciences of the United States of America)
[2018, 115(30):7777-7782]

Cited: 2 times

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Acute Promyelocytic Leukemia in Children: A Single Centre Experience from Turkey.

Tekin Aksu, Ali Fettah, İkbal Ok Bozkaya, Mehmet Baştemur, Abdurrahman Kara, Vildan Koşan Çulha, Namık Yaşar Özbek, Neşe Yaralı,

<h4>Background and objectives</h4>Acute promyelocytic leukemia (APL), is a distinct subtype of acute myeloid leukemia (AML) characterized by a tendency to hemorrhage and excellent response to all-trans retinoic acid (ATRA). In this retrospective study, we aimed to determine the incidence, clinical symptoms, toxicities, and outcome of children with APL in our ... Read more >>

Mediterr J Hematol Infect Dis (Mediterranean journal of hematology and infectious diseases)
[2018, 10(1):e2018045]

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Two versus three day upfront use of granulocyte-colony stimulating factor in healthy bone marrow donors for pediatric bone marrow transplantation.

Tekin Aksu, İkbal Ok Bozkaya, Sibel Akpınar Tekgündüz, Mehtap Olcar Kanbur, Yasin Köksal, Meltem Özgüner, Namık Yaşar Özbek,

In order to decrease donors' exposure to granulocyte-colony stimulating factor (G-CSF), we compared the effect of two versus three days of G-CSF priming on CD34+ yield in bone marrow (BM) harvest. Although the number of BM-CD34+ cells was higher in 3day G-CSF priming, we achieved the same number of CD34+ ... Read more >>

Transfus Apher Sci (Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis)
[2017, 56(6):829-831]

Cited: 0 times

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Angiopoietins in the bone marrow microenvironment of acute lymphoblastic leukemia.

Neslihan Karakurt, Tekin Aksu, Yasin Koksal, Nese Yarali, Bahattin Tunc, Duygu Uckan-Cetinkaya, Meltem Ozguner,

<h4>Objective</h4>Angiogenesis have implications in leukemia biology. Angiopoietin 1 (Ang 1) is an angiogenic cytokine which is essential in survival and proliferation of endothelial cells. Angiopoietin 2 (Ang 2) promotes dissociation of pericytes and increases vascular permeability and stromal derived factor 1 alpha (SDF 1α) which is a key player in ... Read more >>

Hematology (Hematology (Amsterdam, Netherlands))
[2016, 21(6):325-331]

Cited: 3 times

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An Unusual Congenital Anomaly in Fanconi Aplastic Anemia: Congenital Lobar Emphysema.

Ali Fettah, Gökçe Pınar Reis, Soner Sertan Kara, Tekin Aksu, Afak Durur Karakaya, Mahmut Subaşı, Atilla Çayır,

Turk J Haematol (Turkish journal of haematology : official journal of Turkish Society of Haematology)
[2016, 33(3):263-264]

Cited: 1 time

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Massive splenic infarction and portal vein thrombosis in children with chronic myeloid leukemia.

Tekin Aksu, Arzu Y Erdem, Ali Fettah, Dilek Kaçar, Zekai Avci, Nese Yarali, Bahattin Tunc,

Massive splenic infarction and portal vein thrombosis (PVT) due to chronic myeloid leukemia (CML) is extremely rare. We describe 2 children who were presented with massive splenic infarction and PVT in the course of CML. Massive splenic infarction and PVT treated with splenectomy in one and with medical treatment in ... Read more >>

J Pediatr Hematol Oncol (Journal of pediatric hematology/oncology)
[2014, 36(7):e471-2]

Cited: 1 time

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Secondary antifungal prophylaxis in pediatric hematopoietic stem cell transplants.

Fatih M Azik, Hasan Tezer, Aslinur Ozkaya-Parlakay, Tekin Aksu, Cengiz Bayram, Ali Fettah, Betül Tavil, Bahattin Tunç,

Invasive fungal infections (IFIs) constitute a leading cause of morbidity and infection-related mortality among hematopoietic stem cell transplant (HSCT) recipients. With the use of secondary prophylaxis, a history of IFI is not an absolute contraindication to allo-HSCT. However, still, IFI recurrence remains a risk factor for transplant-related mortality. In this ... Read more >>

J Pediatr Hematol Oncol (Journal of pediatric hematology/oncology)
[2015, 37(1):e19-22]

Cited: 3 times

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The effects of breast milk storage and freezing procedure on interleukine-10 levels and total antioxidant activity.

Tekin Aksu, Yıldız Atalay, Canan Türkyılmaz, Özlem Gülbahar, Ibrahim M Hirfanoğlu, Nihal Demirel, Esra Önal, Ebru Ergenekon, Esin Koç,

<h4>Objective</h4>To compare interleukine-10 (IL-10) and total antioxidant capacity (TAC) levels after breast milk storage by studying premature and term mothers’ colostrum and mature milk and by analyzing those levels relative to gestational week.<h4>Methods</h4>Fifty-four colostrum and mature breast milk samples were collected from both premature and term mothers. The samples were ... Read more >>

J Matern Fetal Neonatal Med (The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians)
[2015, 28(15):1799-1802]

Cited: 8 times

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Homozygosity for HBA1: c.179G > A: Hb Adana in an infant.

Tekin Aksu, Neşe Yarali, Cengiz Bayram, Ali Fettah, Zekai Avci, Bahattin Tunç,

Hb Adana (HBA1: c.179G > A) is a very rare, unstable form of α-globin variant that results from deficient synthesis of functional α chains. We present a 2-month-old boy with hypochromic microcytic anemia, and remarkable anisocytosis, target cells and basophilic stippling on his peripheral blood smear. α-Globin gene analysis of ... Read more >>

Hemoglobin (Hemoglobin)
[2014, 38(6):449-450]

Cited: 2 times

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Use of plerixafor for peripheral blood stem cell mobilization failure in children.

Suna Emir, Hacı Ahmet Demir, Tekin Aksu, Abdurrahman Kara, Meltem Özgüner, Bahattin Tunç,

<h4>Background</h4>Peripheral blood stem cell mobilization is usually performed following chemotherapy plus G-CSF in children. This standard approach may not be successful in some heavily pretreated patients undergoing mobilization. Plerixafor (AMD3100) has been used in adults as a second line mobilizing agent. Our aim is to analyze our experiences with plerixafor ... Read more >>

Transfus Apher Sci (Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis)
[2014, 50(2):214-218]

Cited: 5 times

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Staphylococcal pyomyositis in a child with Fanconi aplastic anemia.

Betul Tavil, Tekin Aksu, Vildan Çulha, Nese Yarali, Bahattin Tunç,

Pediatr Hematol Oncol (Pediatric hematology and oncology)
[2011, 28(5):447-450]

Cited: 1 time

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Hypopituitarism masquerading as Prasad's syndrome: a case report.

Fatma Demirel, Tekin Aksu, Ihsan Esen, Neşe Yarali, Gülhan Karakaya, Bahattin Tunç,

Prasad's syndrome is characterized by geophagia, growth retardation, hypogonadism, and zinc deficiency. We report a 15-year-old boy whose medical history and clinical and laboratory findings were fully compatible with Prasad's syndrome. In addition to severe growth retardation and pubertal delay, iron deficiency anemia and zinc deficiency were determined. His gliadin ... Read more >>

Turk J Pediatr (The Turkish journal of pediatrics)
[2011, 53(6):702-704]

Cited: 1 time

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Sulphasalazine treatment in protracted familial Mediterranean fever arthritis.

Sevcan A Bakkaloglu, Tekin Aksu, Berna Goker, Aysel Unlusoy, Harun Peru, Kibriya Fidan, Oguz Soylemezoglu, Enver Hasanoglu, Necla Buyan,

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited attacks of fever and polyserositis. Articular involvement in early-onset FMF is a common finding characterized by non-erosive, generally asymmetric monoarthritis in large joints. Protracted FMF arthritis was reported in 2.6% of Turkish patients. An 8-year-old female who has ... Read more >>

Eur J Pediatr (European journal of pediatrics)
[2009, 168(8):1017-1019]

Cited: 7 times

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