A5002390465- Hematopoietic Stem Cell Transplantation
- Immunodeficiency and Autoimmune Disorders
- Hemoglobinopathies and Related Disorders
- Acute Lymphoblastic Leukemia research
- Acute Myeloid Leukemia Research
- Blood disorders and treatments
- Childhood Cancer Survivors' Quality of Life
- Immune Cell Function and Interaction
- Cytomegalovirus and herpesvirus research
- Polyomavirus and related diseases
- Neutropenia and Cancer Infections
- Pneumocystis jirovecii pneumonia detection and treatment
- Renal Transplantation Outcomes and Treatments
- Viral-associated cancers and disorders
- Erythrocyte Function and Pathophysiology
- Lysosomal Storage Disorders Research
- Blood groups and transfusion
- Parvovirus B19 Infection Studies
- Iron Metabolism and Disorders
- Lymphoma Diagnosis and Treatment
- IL-33, ST2, and ILC Pathways
- CNS Lymphoma Diagnosis and Treatment
- Fungal Infections and Studies
- Chronic Lymphocytic Leukemia Research
- Neurogenetic and Muscular Disorders Research
Antalya IVF
2014-2025
Istinye University
2020-2025
Antalya Eğitim ve Araştırma Hastanesi
2013-2024
Marmara University
2024
Ark Medical Center
2023
Dokuz Eylül University
2015-2021
Bahçeşehir University
2014-2019
Necmettin Erbakan University
2019
In-Q-Tel
2015
Akdeniz University
2005-2014
BackgroundRecent findings strongly support hematopoietic stem cell transplantation (HSCT) in patients with severe presentation of LPS-responsive beige-like anchor protein (LRBA) deficiency, but long-term follow-up and survival data beyond previous patient reports or meta-reviews are scarce for those who do not receive a transplant.ObjectiveThis international retrospective study was conducted to elucidate the longitudinal clinical course LRBA deficiency transplant.MethodWe assessed disease...
Higher mortality and morbidity are well established in children with malignancies whom intensive care admissions required. A retrospective cohort study was conducted to assess the risk factors for cancer pediatric unit (PICU) short-term outcome (survival vs. nonsurvival when leaving PICU). The records of 36 a median age 5 years (range: 0.5 21) between August 2004 2007 were reviewed. Mortality rate 55%, higher than yearly overall PICU 12% (P<0.0001). mean Pediatric Risk Score (PRISM) III...
Despite the increasing performance of allogeneic hematopoietic cell transplantation over last decades, graft-versus-host disease (GVHD) remains main cause morbidity and mortality. The efficacy ruxolitinib against GVHD has been demonstrated in adult studies; however, very few studies have conducted children.This study aimed to evaluate 29 children with steroid-refractory acute or chronic GVHD. Twenty-five (87%) patients received at least three different immune modulator agents, including...
Background/Aims: Preimplantation genetic diagnosis (PGD) with human leukocyte antigen (HLA) typing represents a significant advancement in treating inherited hematological disorders, particularly thalassemia major.This technology enables the birth of healthy children who can serve as compatible stem cell donors for their affected siblings.Turkey is world leader both PGD+HLA and hematopoietic transplantation from savior siblings born through typing.Aims: This study investigated experiences...
It is uncertain whether hematopoietic stem cell transplantation (HSCT), versus standard enzyme replacement therapy (ERT), effective for type VI mucopolysaccharidosis (MPS VI). New related advances in HSCT prompted an examination of the transplant procedures performed a recent cohort. This single-center retrospective study reviewed medical records 17 pediatric patients with MPS who underwent allogeneic 2021-2023. All conditioning regimens were myeloablative. Engraftment days, complications,...
Abstract Primary hemophagocytic lymphohistiocytosis (p-HLH) can be cured with allogeneic haematopoietic stem cell transplantation (allo-HSCT). It remains unclear whether HSCT outcomes are affected by the presence of different genetic mutations. We used data obtained from children who underwent allo-HSCT for HLH to examine effects mutations on outcomes. Data 153 paediatric patients in 18 centres were retrospectively evaluated. Patients divided into four groups: 1) PRF1 mutation ( n = 46), 2)...
Background ARPC1B deficiency leads to a combined immunodeficiency characterized by early clinical onset, recurrent infections, and platelet abnormalities with bleeding tendency. Although most patients mutations tolerate transplant conditioning, high rate of resolution, there is lack studies comparing the outcome quality life undergoing transplantation or treated conservatively. The aim study compare managed conservatively HSCT assessing life. Methods was approved ESID, EBMT, CIS inborn error...
This is a prospective, randomized, and open-label clinical trial that examines the efficiency safety of PIP/TAZO monotherapy in comparison to cefepime (CEF), for empirical treatment pediatric cancer patients with neutropenia fever.One hundred thirty-one consecutive febrile episodes 70 neutropenic received randomized either piperacillin/tazobactam (PIP/TAZO) 80 mg/kg piperacillin/10 tazobactam every 6 hr or CEF 50 8 hr. Clinical response was determined at completion therapy. Duration fever,...
SEC23B gene encodes an essential component of the coat protein complex II (COPII)-coated vesicles. Mutations in this cause vast majority congenital dyserythropoietic anemia Type (CDA II), a rare disorder resulting from impaired erythropoiesis. Here, we investigated 28 CDA patients 21 unrelated families enrolled International Registry. Overall, found 19 novel variants [c.2270 A>C p.H757P; c.2149-2 A>G; c.1109+1 G>A; c.387(delG) p.L129LfsX26; c.1858 A>G p.M620V; c.1832 G>C p.R611P; c.1735 T>A...
Although successful hematopoietic stem cell transplantation (HSCT) can offer a cure in thalassemia major, there are only few and noncomprehensive studies of its effect on the quality life (QoL), as it is expected to increase QoL by ending transfusion-related issues. Our objective was compare health-related (HRQoL) transplanted nontransplanted major patients developing country. We have studied HSCT consecutively invited 50 49 who had received transplants from HLA matched related donors at...
Severe congenital neutropenia is a rare disease, and autosomal dominantly inherited ELANE mutation the most frequently observed genetic defect in registries from North America Western Europe. However, eastern countries where consanguineous marriages are common, recessive forms might be more frequent.Two hundred sixteen patients with severe 28 different pediatric centers Turkey were registered.The was HAX1 (n = 78, 36.1%). A heterozygous detected 29 (13.4%) our cohort. Biallelic mutations of...
There is limited data about the long-term treatment outcome and prognosis of childhood acute lymphoblastic leukemia (ALL) in developing countries. Our study was designed to assess survival identify risk factors. Data 142 children with ALL who were treated a modified BFM 95 protocol between 1997 2007 evaluated. The median age 4.3 years. Complete remission (CR) rate after induction phase 93.5%; 2.1% induction-related mortality 0.7% having resistance disease. Of complete responders, 67.1% are...
Abstract Background Dedicator of cytokinesis 8 (DOCK8) deficiency is the main cause autosomal recessive hyper‐IgE syndrome (HIES). We previously reported selective loss group 3 innate lymphoid cell (ILC) number and function in a Dock8 ‐deficient mouse model. In this study, we sought to test whether DOCK8 required for maintenance ILC subsets humans. Methods Peripheral blood ILC1‐3 16 DOCK8‐deficient patients recruited at pretransplant stage, seven with dominant (AD) HIES due STAT3 mutations,...
In thalassemia major (TM), without iron chelation therapy, iron-mediated free radical damage causes liver, endocrine, and myocardial toxicities. Deferoxamine has universally been the standard therapeutic option for therapy; however, its usage is troublesome, leading to suboptimal patient compliance. order maximize effectiveness of oral chelators deferiprone deferasirox constitute an important development, offering a potential improve Although both drugs are effective, they have differences...
Abstract Severe combined immunodeficiency (SCID) has a diverse genetic aetiology, where clinical phenotype, caused by single and/or multiple gene variants, can give rise to presentations. The advent of next‐generation sequencing (NGS) recently enabled rapid identification the molecular aetiology SCID, which is crucial for prognosis and treatment strategies. We sought identify various phenotypes SCIDs assessed both immunologic characteristics associated with variants. An amplicon‐based...
Abstract Background The aim was to evaluate the feasibility of donor lymphocyte infusion (DLI) in transplanted patients with thalassemia who were at imminent risk graft rejection (GR). Procedure We retrospectively evaluated outcomes a cohort 19 received DLI following 21 transplantations. Patients divided into three groups depending on indication and time DLI: group I, mixed chimerism‐level‐3 (MC‐level‐3) within 2 months subsequently receiving DLI; II, MC‐level‐3 deferred beyond...
Highlights•Deferasirox up to 20 mg/kg/day significantly reduced serum ferritin ceoncentrations in children with β-thalassemia major post-hematopoietic stem cell transplantation.•Deferasirox also decreased liver iron concentration and increased cardiac T2* levels.•Deferasirox was well tolerated, only 6 adverse events necessitating dosage adjustment or interruption.AbstractWe conducted a prospective, phase II, multicenter, single-arm study evaluate the efficacy safety of deferasirox patients...
Extracorporeal photopheresis (ECP) is a difficult procedure to perform in the pediatric population. This retrospective review of 12 patients who underwent with Therakos Cellex system for graft-versus-host disease (GVHD). Acute GVHD (aGVHD) occurred 6 patients, and overlap syndrome chronic (cGVHD) 4 2 respectively. The ECP regimen was same all aGVHD cGVHD patients: initially, every week (2 sessions/wk) months; next, weeks finally, month at least 1 year. Improvement observed 7 10 (70%) (66%)....
Allogeneic hematopoetic stem cell transplantation (HSCT) is the only radical cure of β-thalassemia. However, iron overload remains a cause morbidity and mortality in posttransplant period. The authors present 7 patients as preliminary report who underwent bone marrow transplant (BMT) received oral chelating therapy (deferasirox) because poor compliance to phlebotomy desferrioxamine. investigated mainly for possible side effects deferasirox. No negative effect was seen aspartate...
Abstract DIDS is a unique form of combined immune deficiency characterized by an unusual susceptibility to cutaneous viral infections, severe allergies with eosinophilia and elevated immunoglobulin E titers, autoimmunity, cancer. HSCT considered the standard care for this deadly disease. We have retrospectively analyzed outcome allogeneic from unrelated donors in patients . Data four patients, five transplants, are presented. All received transplants donors' BM, except one patient who cord...