Search results for: Sevgi Keles

Items from 1 to 20 out of 43 results

article

Successful treatment of postinfectious bronchiolitis obliterans with gamma globulin in a tertiary center: 10 years of experience

Aslı İmran Yilmaz, Yahya Gül, Hasan Kapaklı, Gökçen Ünal, more

Pediatric Pulmonology > 58 > 10 > 2769 - 2776

Introduction Bronchiolitis obliterans is characterized by partial or total occlusion of the bronchioles due to inflammation and fibrosis, and the most common form is postinfectious bronchiolitis obliterans (PIBO). This study aimed to retrospectively present our intravenous immunoglobulin (IVIG) treatment experience in PIBO patients with a clinically severe course despite receiving commonly used treatment...

article

Retrospective analysis of patients with severe combined immunodeficiency and alternative diagnostic criteria: A 20‐year single centre experience

Sevim Busra Korkmaz, Mehmet Ali Karaselek, Selma Erol Aytekin, Huseyin Tokgoz, more

International Journal of Immunogenetics > 50 > 4 > 177 - 184

Severe combined immunodeficiency (SCID) is an inborn errors of immunity (IEI) disorder characterized by impairment in the development and function of lymphocytes and could be fatal if not treated with hematopoietic stem cell transplant in the first 2 years of life. There are various diagnostic criteria for SCID among different primary immunodeficiency societies. We retrospectively evaluated clinical...

article

Hematopoietic stem cell transplantation in serine/threonine kinase 4 (STK4) deficiency: Report of two cases and literature review

Vedat Uygun, Sevgi Keleş, Hayriye Daloğlu, Seda Öztürkmen, more

Pediatric Transplantation > 27 > 2 > n/a - n/a

Background Serine/threonine kinase 4 (STK4) deficiency is a combined immunodeficiency (CID) characterized by early onset recurrent bacterial, viral, and fungal infections. Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative therapy for CID; however, little is known about the necessity and benefits of HSCT in patients with STK4 deficiency. Methods We report two siblings with...

article

Comparing the levels of CTLA‐4‐dependent biological defects in patients with LRBA deficiency and CTLA‐4 insufficiency

Mehmet C. Catak, Bengu Akcam, Sevgi Bilgic Eltan, Royala Babayeva, more

Allergy > 77 > 10 > 3108 - 3123

Background Lipopolysaccharide‐responsive beige‐like anchor protein (LRBA) deficiency and cytotoxic T‐lymphocyte protein‐4 (CTLA‐4) insufficiency are recently described disorders that present with susceptibility to infections, autoimmunity, and lymphoproliferation. Clinical and immunological comparisons of the diseases with long‐term follow‐up have not been previously reported. We sought to compare...

article

A family screening of CD19 gene mutation by PCR-RFLP

Mehmet Ali Karaselek, Hasan Kapaklı, Şükrü Nail Güner, Ercan Kurar, more

European Journal of Clinical and Experimental Medicine > 2022 > 2 > 141-145

Introduction and aim. Mutation(s) in the gene encoding the CD19 molecule affect CD19 protein expression and primary immunodeficiency (PID) occurs. The PCR-RFLP method, which is faster and cheaper than other mutation detection methods, is rarely used in the diagnosis of PID. The study aimed to genetically identify CD19 deficiency, which is a PID, using the PCR-RFLP method. Material and methods. A total...

article

An association between immune status and chest CT scores in COVID‐19 patients

Fatih Çölkesen, Necdet Poyraz, Şevket Arslan, Pınar Diydem Yılmaz, more

International Journal of Clinical Practice > 75 > 11 > n/a - n/a

Background The characteristic features of the immune responses of COVID‐19 patients and how they reflect lung involvement have not been clearly elucidated. Aim The aim of this study was to examine the immune status and the correlations thereof with chest CT scores and lung involvement of patients with COVID‐19. Methods In this retrospective and single‐center study, 72 patients with laboratory‐confirmed...

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Clinical, immunological features and follow up of 20 patients with dedicator of cytokinesis 8 (DOCK8) deficiency

Sule Haskologlu, Sevgi Kostel Bal, Candan Islamoglu, Caner Aytekin, more

Pediatric Allergy and Immunology > 31 > 5 > 515 - 527

Biallelic mutations in the dedicator of cytokinesis 8 gene (DOCK8) cause a progressive combined immunodeficiency (CID) characterized by susceptibility to severe viral skin infections, atopic diseases, recurrent respiratory infections, and malignancy. Hematopoietic stem cell transplantation (HSCT) is only curative treatment for the disease. However, there is limited information about long‐term outcome...

article

ILC3 deficiency and generalized ILC abnormalities in DOCK8‐deficient patients

Ahmet Eken, Murat Cansever, Fatma Zehra Okus, Serife Erdem, more

Allergy > 75 > 4 > 921 - 932

Background Dedicator of cytokinesis 8 (DOCK8) deficiency is the main cause of the autosomal recessive hyper‐IgE syndrome (HIES). We previously reported the selective loss of group 3 innate lymphoid cell (ILC) number and function in a Dock8‐deficient mouse model. In this study, we sought to test whether DOCK8 is required for the function and maintenance of ILC subsets in humans. Methods Peripheral...

article

Hypomorphic DOCK8 deletion causes hypereosinophilic syndrome

Zahra Aryan, Mohammad Nabavi, Mahsima Shabani, Sevgi Keles, more

Pediatric Blood & Cancer > 67 > 2 > n/a - n/a

article

Intrauterine detection of DCLRE1C (Artemis) mutation by restriction fragment length polymorphism

Mehmet Ali Karaselek, Hasan Kapaklı, Sevgi Keleş, Şükrü Nail Güner, more

Pediatric Allergy and Immunology > 30 > 6 > 668 - 671

article

Type I IFN–related NETosis in ataxia telangiectasia and Artemis deficiency

Ersin Gul, Esra Hazar Sayar, Bilgi Gungor, Fehime Kara Eroglu, more

Journal of Allergy and Clinical Immunology > 2018 > 142 > 1 > 246-257

Pathological inflammatory syndromes of unknown etiology are commonly observed in ataxia telangiectasia (AT) and Artemis deficiency. Similar inflammatory manifestations also exist in patients with STING-associated vasculopathy in infancy (SAVI).We sought to test the hypothesis that the inflammation-associated manifestations observed in patients with AT and Artemis deficiency stem from increased type...

article

Clinical, immunologic, and genetic spectrum of 696 patients with combined immunodeficiency

Hassan Abolhassani, Janet Chou, Wayne Bainter, Craig D. Platt, more

Journal of Allergy and Clinical Immunology > 2018 > 141 > 4 > 1450-1458

Combined immunodeficiencies (CIDs) are diseases of defective adaptive immunity with diverse clinical phenotypes. Although CIDs are more prevalent in the Middle East than Western countries, the resources for genetic diagnosis are limited.This study aims to characterize the categories of patients with CIDs in Iran clinically and genetically.Clinical and laboratory data were obtained from 696 patients...

article

Exaggerated follicular helper T-cell responses in patients with LRBA deficiency caused by failure of CTLA4-mediated regulation

Fayhan J. Alroqi, Louis-Marie Charbonnier, Safa Baris, Ayca Kiykim, more

Journal of Allergy and Clinical Immunology > 2018 > 141 > 3 > 1050-1059.e10

LPS-responsive beige-like anchor protein (LRBA) and cytotoxic T lymphocyte–associated antigen 4 (CTLA4) deficiencies give rise to overlapping phenotypes of immune dysregulation and autoimmunity, with dramatically increased frequencies of circulating follicular helper T (cT_FH) cells.We sought to determine the mechanisms of cT_FH cell dysregulation in patients with LRBA deficiency and the utility of...

article

DOCK8 Deficiency Presenting as an IPEX-Like Disorder

Fayhan J. Alroqi, Louis-Marie Charbonnier, Sevgi Keles, Fatima Ghandour, more

Journal of Clinical Immunology > 2017 > 37 > 8 > 811-819

Purpose The dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive-combined immunodeficiency whose clinical spectra include recurrent infections, autoimmunity, malignancies, elevated serum IgE, eczema, and food allergies. Here, we report on patients with loss of function DOCK8 mutations with profound immune dysregulation suggestive of an immune dysregulation, polyendocrinopathy, enteropathy,...

article

Hematopoietic stem cell transplantation from unrelated donors in children with DOCK8 deficiency

Dilara Fatma K. Uygun, Vedat Uygun, İsmail Reisli, Sevgi Keleş, more

Pediatric Transplantation > 21 > 7 > n/a - n/a

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, and cancer. HSCT is considered the standard of care for this deadly disease. We have retrospectively analyzed the outcome of allogeneic HSCT from unrelated donors in patients with DIDS. Data...

article

DOCK8 deficiency: Insights into pathophysiology, clinical features and management

Catherine M. Biggs, Sevgi Keles, Talal A. Chatila

Clinical Immunology > 2017 > 181 > C > 75-82

Dedicator of cytokinesis 8 (DOCK8) deficiency is a combined immunodeficiency that exemplifies the broad clinical features of primary immunodeficiencies (PIDs), extending beyond recurrent infections to include atopy, autoimmunity and cancer. It is caused by loss of function mutations in DOCK8, encoding a guanine nucleotide exchange factor highly expressed in lymphocytes that regulates the actin cytoskeleton...

article

Dedicator of cytokinesis 8 regulates signal transducer and activator of transcription 3 activation and promotes TH17 cell differentiation

Sevgi Keles, Louis Marie Charbonnier, Venkataraman Kabaleeswaran, Ismail Reisli, more

Journal of Allergy and Clinical Immunology > 2016 > 138 > 5 > 1384-1394.e2

The autosomal recessive hyper-IgE syndrome (HIES) caused by dedicator of cytokinesis 8 (DOCK8) deficiency shares clinical features with autosomal dominant HIES because of signal transducer and activator of transcription 3 (STAT3) mutations, including recurrent infections and mucocutaneous candidiasis, which are suggestive of T_H17 cell dysfunction. The mechanisms underlying this phenotypic overlap...

article

Hematopoietic stem cell transplantation outcomes for 11 patients with dedicator of cytokinesis 8 deficiency

Waleed Al-Herz, Julia I. Chu, Jet van der Spek, Raj Raghupathy, more

Journal of Allergy and Clinical Immunology > 2016 > 138 > 3 > 852-859.e3

Dedicator of cytokinesis 8 (DOCK8) deficiency can be cured by allogeneic hematopoietic stem cell transplantation (HSCT). Reports of outcomes are still limited.We sought to analyze the results of HSCT in patients with DOCK8 deficiency and report whether approaches resulting in mixed chimerism result in clinically relevant immune reconstitution.We performed a retrospective chart review of 11 patients...

article

Efficacy of intravenous immunoglobulin treatment in immunocompromised children with H1N1 influenza: a clinical observation

Bahar Gokturk, Sevgi Pekcan, Sukru Nail Guner, Hasibe Artac, more

The Clinical Respiratory Journal > 10 > 2 > 223 - 230

Background and AimsThe appropriate treatment of pandemic H1N1 influenza which was first identified in April 2009 in Mexico is insufficient especially for immunocompromised patients. We aimed to evaluate the features and prognostic factors of the children with H1N1, especially immunocompromised ones, and whether intravenous immunoglobulin G (IVIG) replacement could aid for a better outcome. MethodsTwenty‐one...

article

HSCT for DOCK8 Deficiency - an International Study on 74 Patients

Susanne Aydin, Alexandra F. Freeman, Helen Su, Dennis Hickstein, more

Biology of Blood and Marrow Transplantation > 2016 > 22 > 3(S) > S103-S104

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INFONA - science communication portal

Search results for: Sevgi Keles

Successful treatment of postinfectious bronchiolitis obliterans with gamma globulin in a tertiary center: 10 years of experience

Retrospective analysis of patients with severe combined immunodeficiency and alternative diagnostic criteria: A 20‐year single centre experience

Hematopoietic stem cell transplantation in serine/threonine kinase 4 (STK4) deficiency: Report of two cases and literature review

Comparing the levels of CTLA‐4‐dependent biological defects in patients with LRBA deficiency and CTLA‐4 insufficiency

A family screening of CD19 gene mutation by PCR-RFLP

An association between immune status and chest CT scores in COVID‐19 patients

Clinical, immunological features and follow up of 20 patients with dedicator of cytokinesis 8 (DOCK8) deficiency

ILC3 deficiency and generalized ILC abnormalities in DOCK8‐deficient patients

Hypomorphic DOCK8 deletion causes hypereosinophilic syndrome

Intrauterine detection of DCLRE1C (Artemis) mutation by restriction fragment length polymorphism

Type I IFN–related NETosis in ataxia telangiectasia and Artemis deficiency

Clinical, immunologic, and genetic spectrum of 696 patients with combined immunodeficiency

Exaggerated follicular helper T-cell responses in patients with LRBA deficiency caused by failure of CTLA4-mediated regulation

DOCK8 Deficiency Presenting as an IPEX-Like Disorder

Hematopoietic stem cell transplantation from unrelated donors in children with DOCK8 deficiency

DOCK8 deficiency: Insights into pathophysiology, clinical features and management

Dedicator of cytokinesis 8 regulates signal transducer and activator of transcription 3 activation and promotes TH17 cell differentiation

Hematopoietic stem cell transplantation outcomes for 11 patients with dedicator of cytokinesis 8 deficiency

Efficacy of intravenous immunoglobulin treatment in immunocompromised children with H1N1 influenza: a clinical observation

HSCT for DOCK8 Deficiency - an International Study on 74 Patients

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