Aydan İkincioğulları
- Immunodeficiency and Autoimmune Disorders
- Blood disorders and treatments
- Immune Cell Function and Interaction
- T-cell and B-cell Immunology
- Asthma and respiratory diseases
- Hematopoietic Stem Cell Transplantation
- Diabetes and associated disorders
- Pediatric health and respiratory diseases
- Cytomegalovirus and herpesvirus research
- Antifungal resistance and susceptibility
- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Neurogenetic and Muscular Disorders Research
- Fungal Infections and Studies
- Platelet Disorders and Treatments
- Tuberculosis Research and Epidemiology
- Pneumocystis jirovecii pneumonia detection and treatment
- Drug-Induced Adverse Reactions
- Immune Response and Inflammation
- Eosinophilic Disorders and Syndromes
- Dermatology and Skin Diseases
- Respiratory and Cough-Related Research
- Inflammasome and immune disorders
- Lymphoma Diagnosis and Treatment
- Viral-associated cancers and disorders
Ankara University
2016-2025
Ministry of Health
2010-2022
Sağlık Bilimleri Üniversitesi
2022
Dr Sami Ulus Çocuk Sağlığı ve Hastalıkları Eğitim ve Araştırma Hastanesi
2020
Ankara Onkoloji Eğitim ve Araştırma Hastanesi
2020
Martel
2020
Diyarbakır Askeri Hastanesi
2016
Children's Clinical University Hospital
2013
Hacettepe University Hospital
2013
Uniwersytecki Szpital Dziecięcy
2013
Interleukin-12 receptor β1 (IL-12Rβ1) deficiency is the most common form of Mendelian susceptibility to mycobacterial disease (MSMD). We undertook an international survey 141 patients from 102 kindreds in 30 countries. Among probands, first infection occurred at a mean age 2.4 years. In 78 patients, this was caused by Bacille Calmette-Guérin (BCG; n = 65), environmental mycobacteria (EM; also known as atypical or nontuberculous mycobacteria) (n 9) Mycobacterium tuberculosis 4). Twenty-two...
Background Combined immunodeficiencies are marked by inborn errors of T-cell immunity in which the T cells that present quantitatively or functionally deficient. Impaired humoral is also common. Patients have severe infections, autoimmunity, both. The specific molecular, cellular, and clinical features many types combined remain unknown. Methods We performed genetic cellular immunologic studies involving five unrelated children with early-onset invasive bacterial viral lymphopenia, defective...
Chronic mucocutaneous candidiasis (CMC) is characterized by recurrent or persistent infections of the skin, nail, oral, and genital mucosae with Candida species, mainly C. albicans. Autosomal-recessive (AR) IL-17RA ACT1 deficiencies autosomal-dominant IL-17F deficiency, each reported in a single kindred, underlie CMC otherwise healthy patients. We report three patients from unrelated kindreds, aged 8, 12, 37 yr isolated CMC, who display AR IL-17RC deficiency. The are homozygous for different...
Hundreds of patients with autosomal recessive, complete IL-12p40 or IL-12Rβ1 deficiency have been diagnosed over the last 20 years. They typically suffer from invasive mycobacteriosis and, occasionally, mucocutaneous candidiasis. Susceptibility to these infections is thought be due impairments IL-12-dependent IFN-γ immunity and IL-23-dependent IL-17A/IL-17F immunity, respectively. We report here IL-12Rβ2 IL-23R deficiency, lacking responses IL-12 IL-23 only, all whom, unexpectedly, display...
Significance Chronic mucocutaneous candidiasis (CMC) is defined as persistent or recurrent infections of the skin and/or mucosae by commensal fungi Candida genus. It often seen in patients with T-cell deficiencies, whether inherited acquired, who typically suffer from multiple infectious diseases. Rare are otherwise healthy and display isolated CMC, which segregates a Mendelian trait. In 2011, we described first genetic cause autosomal recessive (AR), complete IL-17 receptor A (IL-17RA)...
In this study, we describe four patients from two unrelated families of different ethnicities with a primary immunodeficiency, predominantly manifesting as susceptibility to Epstein-Barr virus (EBV)–related diseases. Three presented EBV-associated Hodgkin’s lymphoma and hypogammaglobulinemia; one also had severe varicella infection. The fourth viral encephalitis during infancy. Homozygous frameshift or in-frame deletions in CD70 these abolished either surface expression binding its cognate...
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...
<b>Objective</b> Lactoferrin (LF) is effective in the prevention of sepsis very low birth weight (VLBW) neonates. T-regulatory cells (Tregs) are important subsets T lymphocytes that control pathogen-specific immune responses and essential for intestinal homoeostasis. The aim present study to determine whether oral LF at a dosage 200 mg/d reduces nosocomial episodes necrotizing enterocolitis (NEC) premature infants evaluate possible effects on Treg levels. <b>Study Design</b> In this...
Autosomal dominant hyper-IgE syndrome (AD-HIES) is typically caused by dominant-negative (DN) STAT3 mutations. Patients suffer from cold staphylococcal lesions and mucocutaneous candidiasis, severe allergy, skeletal abnormalities. We report 12 patients 8 unrelated kindreds with AD-HIES due to DN IL6ST identified seven different truncating mutations, one of which was recurrent. The mutant alleles encode GP130 receptors bearing the transmembrane domain but lacking both recycling motif all four...
Naive CD4+ T cells differentiate into specific effector subsets—Th1, Th2, Th17, and follicular helper (Tfh)—that provide immunity against pathogen infection. The signaling pathways involved in generating these are partially known. However, the effects of mutations underlying human primary immunodeficiencies on processes, how they compromise immune responses, remain unresolved. By studying individuals with key pathways, we identified nonredundant regulating cell differentiation vitro....
Hematopoietic stem cell transplantation (HSCT) represents a curative treatment for patients with severe combined immunodeficiency (SCID), group of monogenic immune disorders an otherwise fatal outcome.We performed comprehensive multicenter analysis genotype-specific HSCT outcome, including detailed reconstitution (IR) and the predictive value clinical outcome.HSCT outcome was studied in 338 genetically confirmed SCID who underwent 2006-2014 were registered SCETIDE registry. In representative...
Interleukin 12Rβ1 (IL-12Rβ1)-deficient patients are prone to clinical disease caused by mycobacteria, Salmonella, and other intramacrophagic pathogens, probably because of impaired interleukin 12-dependent interferon γ production. About 25% also display mucocutaneous candidiasis, owing 23-dependent 17 immunity. The features outcome candidiasis in these have not been described before, our knowledge. We report here the signs 35 with IL-12Rβ1 deficiency.Most (n = 71) 76 episodes were...
CD40 ligand (CD40L) deficiency, an X-linked primary immunodeficiency, causes recurrent sinopulmonary, Pneumocystis and Cryptosporidium species infections. Long-term survival with supportive therapy is poor. Currently, the only curative treatment hematopoietic stem cell transplantation (HSCT).We performed international collaborative study to improve patients' management, aiming individualize risk factors determine optimal HSCT characteristics.We retrospectively collected data on 130 patients...