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Öğe A CASE WITH ABSENCE OF B LYMPHOCYTES(SPRINGER/PLENUM PUBLISHERS, 2014) Ozdemir, Hulya; Artac, Hasibe; Ural, Onur; Karabagli, Hakan; Caliskaner, A. Zafer[Abstract not Available]Öğe CD19 deficiency: A village screening study(AVES, 2009) Reisli, Ismail; Artac, Hasibe; Pekcan, Sevgi; Kara, Reyhan; Yumlu, Kadir; Karagol, Cuneyt; Cimen, OmerAim: We identified a new CD19 deficient case who was a relative of the first CD19 deficient patient and lived in the same village. These cases suggested that other patients with CD19 deficiency or carriers could be found in this village. The aim of this study was to assess the warning signs of primary immunodeficiency and the CD19 molecule deficiency in this population. Material and Method: The relatives of CD19 deficient patients and controls were examined for the warning signs of primary immunodeficiency using a questionnaire form Blood samples were taken from these subjects CD19 and CD21 expressions (median fluorescent intensity, MFI) were analyzed by flowcytometry in these samples. Results: A total of 208 subjects (59 families, 126 females, 82 males) including 129 children 9.5 +/- 3 7 years and 79 adults (37 13 years) were evaluated. A new case with CD19 deficiency was not detected There was at least one warning sign for immunodeficiency in 67 subjects (57 children and 10 adults) CD19 median expression value was lower than 60 MFI value in 20 subjects, which was known as in the carriers who were detected to have heterozygous mutation Conclusions: Our findings suggested that CD19 median expression might be a diagnostic tool for the subjects who have CD19 heterozygous mutations. Mutation analyses are planned for the confirmation of CD19 heterozygous mutation in these subjects who have low CD19 median expression. (Turk Arch Ped 2009; 44. 127-30)Öğe Chilaiditi Syndrome as a Cause of Respiratory Distress(Sprınger, 2006) Keleş, Sevgi; Artac, Hasibe; Reisli, İsmail; Alp, Hayrullah; Koç, OsmanChilaiditi syndrome is the interposition of the colon between the diaphragma and the liver. In general, patients are asymptomatic, but some patients have been associated with gastrointestinal and respiratory symptoms. A 5-month-old infant boy was admitted to the hospital with a history of cough, cyanosis and recurrent respiratory distress that had persisted during the preceding 2 months. The chest X-ray revealed an elevation of the right hemidiaphragma caused by the presence of a dilated colonic loop below. Computed tomography showed a hepatodiaphragmatic interposition of the colon, leading to the diagnosis of Chilaiditi syndrome. The patient was conservatively treated with oxygen, fluid supplementation and stool softeners. We conclude that this rare syndrome should be kept in mind when young patients present with recurrent respiratory distress.Öğe Clinical heterogeneity can hamper the diagnosis of patients with ZAP70 deficiency(SPRINGER, 2009) Turul, Tuba; Tezcan, Ilhan; Artac, Hasibe; de Bruin-Versteeg, Sandra; Barendregt, Barbara H.; Reisli, Ismail; Sanal, OzdenOne of the severe combined immunodeficiencies (SCIDs), which is caused by a genetic defect in the signal transduction pathways involved in T-cell activation, is the ZAP70 deficiency. Mutations in ZAP70 lead to both abnormal thymic development and defective T-cell receptor (TCR) signaling of peripheral T-cells. In contrast to the lymphopenia in most SCID patients, ZAP70-deficient patients have lymphocytosis, despite the selective absence of CD8(+)T-cells. The clinical presentation is usually before 2 years of age with typical findings of SCID. Here, we present three new ZAP70-deficient patients who vary in their clinical presentation. One of the ZAP70- deficient patients presented as a classical SCID, the second patient presented as a healthy looking wheezy infant, whereas the third patient came to clinical attention for the eczematous skin lesions simulating atopic dermatitis with eosinophilia and elevated immunoglobulin E (IgE), similar to the Omenn syndrome. This study illustrates that awareness of the clinical heterogeneity of ZAP70 deficiency is of utmost importance for making a fast and accurate diagnosis, which will contribute to the improvement of the adequate treatment of this severe immunodeficiency.Öğe The Correlation between the Level of Doxorubicin-Induced Cardiac Damage and Serum Soluble Fas in an Experimental Rat Model(WOLTERS KLUWER MEDKNOW PUBLICATIONS, 2018) Kose, Dogan; Ozdemir, Hulya; Celik, Zeliha Esin; Unlu, Ali; Artac, Hasibe; Koksal, YavuzAim: This study was planned to research the relationship between doxorubicin cardiomyopathy and the soluble Fas (sFas) level. Materials and Methods: Two groups of rats were included in the study. The control group was given physiological saline, while the study group was given doxorubicin. The rats, whose blood samples were taken weekly, were sacrificed and their myocardial tissues were removed. The tissues were examined in terms of morphological changes and surface Fas expression, while the blood samples were examined in terms of sFas level. Results: In the study group, the sFas levels at 2nd-9th weeks were higher than those found at 1st week before administrating the drug, and the increase at 2nd-7th weeks was meaningful. In addition, sFas levels were gradually increased each week during 1st-5th weeks when compared to the values of a previous week, and the increase during the first 4 weeks was meaningful. After the 5th week, the values gradually decreased each week. The mean values of the study group at 1st-8th weeks were higher than those of the control group, and the increases at 2nd-8th weeks were meaningful. The severe forms of interfibrillar hemorrhage, vascular dilatation, myocardial necrosis, inflammatory infiltration, and splitting of muscle fibers occurred with 15, 15, 17.5, 20, and 22.5 mg/kg dose of medicine, respectively. Conclusions: As the tissue injury increased, the increasing cell-surface Fas expression and sFas plasma level at the acute phase of doxorubicin-related cardiotoxicity decreased. The sFas level determined at acute phase may be helpful in predicting the existing injuries and possible late-term problems.Öğe Defects along the T(H)17 differentiation pathway underlie genetically distinct forms of the hyper IgE syndrome(MOSBY-ELSEVIER, 2009) Al Khatib, Shadi; Keles, Sevgi; Garcia-Lioret, Maria; Koc-Aydiner, Elif Kara; Reisli, Ismail; Artac, Hasibe; Camcioglu, YildizBackground: The hyper IgE syndrome (HIES) is characterized by abscesses, eczema, recurrent infections, skeletal and connective tissue abnormalities, elevated serum IgE, and diminished inflammatory responses. It exists as autosomal-dominant and autosomal-recessive forms that manifest common and distinguishing clinical features. A majority of those with autosomal-dominant HIES have heterozygous mutations in signal transducer and activator of transcription (STAT)-3 and impaired T(H)17 differentiation. Objective: To elucidate mechanisms underlying different forms of HIES. Methods: A cohort of 25 Turkish children diagnosed with HIES were examined for STAT3 mutations by DNA sequencing. Activation of STAT3 by IL-6 and IL-21 and STAT1 by IFN-alpha was assessed by intracellular staining with anti-phospho (p)STAT3 and -pSTAT1 antibodies. T(H)17 and T(H)1 cell differentiation was assessed by measuring the production of IL-17 and IFN-gamma, respectively. Results: Six subjects had STAT3 mutations affecting the DNA binding, Src homology 2, and transactivation domains, including 3 novel ones. Mutation-positive but not mutation-negative subjects with HIES exhibited reduced phosphorylation of STAT3 in response to cytokine stimulation, whereas pSTAT1 activation was unaffected. Both patient groups exhibited impaired TH17 responses, but whereas STAT3 mutations abrogated early steps in TH17 differentiation, the defects in patients with HIES with normal STAT3 affected more distal steps. Conclusion: In this cohort of Turkish children with HIES, a majority had normal STAT3, implicating other targets in disease pathogenesis. Impaired TH17 responses were evident irrespective of the STAT3 mutation status, indicating that different genetic forms of HIES share a common functional outcome. (J Allergy Clin Immunol 2009;124:342-8.)Öğe Dysgerminoma in a child with ataxia-telangiectasia(TAYLOR & FRANCIS INC, 2007) Koksal, Yavuz; Caliskan, Umran; Ucar, Canan; Yurtcu, Muslim; Artac, Hasibe; Ilerisoy-Yakut, Zeynep; Reisli, IsmailAlaxia - telangiectasia is an autosomal recessive disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, high incidence of cancer; and increased sensitivity to ionizing radiation. The authors report a case of dysgerminoma in a child with high alpha-fetoprotein, CA125 and beta-human chorionic gonadotropin, who has been followed-up for ataxia-letangiectasia for 2 years.Öğe The effect of allergen immunotherapy on serum periostin levels in children with allergic rhinitis(BILIMSEL TIP YAYINEVI, 2019) Ucaryilmaz, Hulya; Emsen, Ayca; Dikener, Ahmet Hakan; Akdam, Neriman; Unlu, Ali; Artac, HasibeObjective: Periostin, an extracellular matrix protein, is related to the eosinophilic airway inflammation. There is no specific marker in allergen immunotherapy to evaluate clinical response. We aimed to investigate the serum periostin levels in the children who receive allergen immunotherapy. Materials and Methods: Sixteen patients between 8-18 years (12.7 +/- 2.8 years) with allergic rhinitis and/or asthma due to grass pollen hypersensitivity and 30 healthy subjects (11.7 +/- 2.6 years) were included. Demographic data, eosinophil counts, skin prick tests and the specific IgE levels of the patients are recorded. Symptom scores, visual analog scales, medication scores were determined and the serum periostin levels were measured in the beginning, 4th and 12th months of the allergen immunotherapy. Results: The symptom scores for rhinitis in the 4th month showed significant improvements in all of the patients (p< 0.05). Nine patients with allergic rhinitis accompanied by asthma, showed significant improvements in 12th month symptom score for asthma (p=0.018). A significant correlation was detected between the initial serum periostin levels and the symptom scores for the eye (r=0.668, p=0.005). No significant difference was found in serum periostin levels between the patient and the control groups. There were no significant differences in serum periostin levels in 4th and 12th months compared to the ones in the beginning. Conclusion: In this study, there were no significant differences in serum periostin levels of children during the allergen immunotherapy. The association of serum periostin levels with symptom scores for the eye needs to be confirmed in more children with allergic rhinoconjunctivitis.Öğe The effects of trastuzumab, paclitaxel, and carboplatin on HER2-positive cancer stem cells that are isolated from primary breast cancer cultures: a preliminary report(AMER ASSOC CANCER RESEARCH, 2015) Artac, Mehmet; Kayadibi, Gozde; Ceylan, Ayca; Kars, Meltem Demirel; Artac, Hasibe; Cakir, Murat; Boruban, Cem[Abstract not Available]Öğe Efficacy of intravenous immunoglobulin treatment in immunocompromised children with H1N1 influenza: a clinical observation(WILEY-BLACKWELL, 2016) Gokturk, Bahar; Pekcan, Sevgi; Guner, Sukru Nail; Artac, Hasibe; Keles, Sevgi; Kirac, Mine; Reisli, IsmailBackground 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 hospitalized children with laboratory-confirmed H1N1 were evaluated retrospectively. Data were extracted from files and electronic medical records. ResultsThe median age was 37 (1-216) months; 62% of them were under 5years of age and 71.4% had one or more underlying disorders. Main symptoms were high fever, cough, fatigue and vomiting. Lower respiratory tract manifestations were seen in 66.6% of children. Mortality rate was 4.7%. The patient who died had the lowest lymphocyte (100/mm(3)), thrombocyte (21000/mm(3)) and highest blood urea nitrogen (87mg/dL) levels. Fifty-eight percent of evaluated patients had one of the primary immunodeficiency disorders. Surprisingly, none of the six patients with primary immunodeficiency who are on regular IVIG replacement needed intensive care unit and died. Although median durations of cough, fever and hospitalization were lower, they did not change statistically according to get IVIG replacement regularly (P=0.47, 0.97, 0.09, respectively). ConclusionOur study is important while it is the first one that shows the course of primary immunodeficient children with H1N1 infection who were on regular IVIG replacement. A trial of high-dose IVIG may be a useful adjunctive therapy in severe H1N1 influenza, particularly in the immunocompromised patients.Öğe Hypogammaglobulinemia and Silver-Russell Phenotype Associated With Partial Trisomy 7q and Partial Monosomy 21q(WILEY-LISS, 2009) Artac, Hasibe; Reisli, Ismail; Yildirim, Mahmut Selman; Bagci, Gulseren; Luleci, Guven; Hosgor, Orhan; Karaaslan, Sevim[Abstract not Available]Öğe Infliximab therapy for inflammatory colitis in an infant with NEMO deficiency(HUMANA PRESS INC, 2019) Artac, Hasibe; Emsen, Ayca; Ucaryilmaz, Hulya; Emiroglu, Halil Haldun; Uygun, Vedat; Stray-Pedersen, Asbjorg[Abstract not Available]Öğe Iris atrophy in a patient with X-linked agammaglobulinemia(CANADIAN OPHTHAL SOC, 2007) Reisli, Ismail; Artac, Hasibe; van der Burk, Midam; Ozturk, Bann Turgut[Abstract not Available]Öğe N-terminal-pro brain natriuretic peptide levels in children with allergic rhinitis(TUBITAK SCIENTIFIC & TECHNICAL RESEARCH COUNCIL TURKEY, 2016) Artac, Hasibe; Vatansev, Husamettin; Cimen, Derya; Arslan, Derya; Kaya, Fatma; Kayis, Seyit Ali; Oran, BulentBackground/aim: Persistent upper airway obstruction may lead to increased pulmonary arterial pressure (PAP). The aim of this study was to evaluate N-terminal pro brain natriuretic peptide (NT-proBNP) concentrations and PAP values in children with allergic rhinitis. Materials and methods: Sixty-six patients with allergic rhinitis and 22 healthy children were prospectively enrolled in this study. Plasma NT-proBNP levels were measured at first admission and after treatment. Simultaneously, echocardiography was done to assess pulmonary arterial hypertension, and rhinitis symptom scores were recorded. Results: The median age of the study group was 9.0 (5.0. 17.0) years; 26 were female. PAP was found to be normal in all the patients. There was a negative correlation between age and NT-proBNP levels (r = -0.452, P < 0.01). Nasal blockage levels affected NT-proBNP levels mildly (P = 0.067). No significant difference between before and after nasal steroid treatment was observed in NT-proBNP levels. Conclusion: These results suggest that NT-proBNP level and PAP may not be affected in children with allergic rhinitis, and primarily this influence may be associated with the severity of nasal obstruction.Öğe A Novel Mutation in the Complement Component 3 Gene in a Patient with Selective IgA Deficiency(SPRINGER/PLENUM PUBLISHERS, 2013) Santos-Valente, Elisangela; Reisli, Ismail; Artac, Hasibe; Ott, Raphael; Sanal, Ozden; Boztug, KaanPurpose Immunological and molecular evaluation of a patient presenting with recurrent infections caused by Streptococcus pneumoniae and low complement component 3 (C3) levels. Methods Immunological evaluation included complement components and immunoglobulin level quantification as well as number and function of T cells, B cells and neutrophils. Serotype-specific immunoglobulin G antibodies against S. pneumoniae capsular polysaccharides were quantified by ELISA in serum samples before and after vaccination with unconjugated polysaccharide vaccine. For the molecular analysis, genomic DNA from the patient and parents were isolated and all exons as well as exon-intron boundaries of the C3 gene were sequenced by Sanger sequencing. Results A 16-year-old male, born to consanguineous parents, presented with recurrent episodes of pneumonia caused by S. pneumoniae and bronchiectasis. The patient showed severely reduced C3 and immunoglobulin A levels, while the parents showed moderately reduced levels of C3. Mutational analysis revealed a novel, homozygous missense mutation in the C3 gene (c. C4554G, p. Cys1518Trp), substituting a highly conserved amino acid in the C345C domain of C3 and interrupting one of its disulfide bonds. Both parents were found to be carriers of the affected allele. Vaccination against S. pneumoniae resulted in considerable clinical improvement. Conclusions We report a novel homozygous mutation in the C3 gene in a patient with concomitant selective IgA deficiency who presented with a marked clinical improvement after vaccination against S. pneumoniae. This observation underlines the notion that vaccination against this microorganism is an important strategy for treatment of PID patients, particularly those presenting with increased susceptibility to infections caused by this agent.Öğe Pollen grains in the atmosphere of Konya (Turkey) and their relationship with meteorological factors, in 2008(TUBITAK SCIENTIFIC & TECHNICAL RESEARCH COUNCIL TURKEY, 2012) Kizilpinar, Ilginc; Dogan, Cahit; Artac, Hasibe; Reisli, Ismail; Pekcan, SevgiAtmospheric pollen was collected by a Burkard Volumetric 7-Day spore trap in Konya, Turkey, in 2008. In the present study, the pollen concentration is presented in relation to meteorological parameters (mean temperature, relative humidity, rainfall, and wind speed). Pollen grains of 35 taxa were identified. Of these, 18 taxa were arboreal plants, while the others were non-arboreal plants. The distribution of the total pollen grains was as follows: arboreal plants, 61.29%; Poaceae, 16.09%; non-arboreal plants, 20.25%; and undetermined, 2.37%. Arboreal taxa pollen were represented by Pinaceae, Cupressaceae/Taxaceae, Fabaceae, Betulaceae, Quercus L., Juglandaceae, and Aesculus L., while non-arboreal taxa pollen were represented by Poaceae, Asteraceae, Chenopodiaceae/Amaranthaceae, Brassicaceae, Boraginaceae, Plantago L., and Urticaceae. These were the dominant pollen types found in the atmosphere in Konya. In addition, there are generally significant positive effects of temperature and wind speed and a negative influence of relative humidity on the daily pollen levels belonging to those taxa which contribute more than 1% of the total pollen in the Konya atmosphere. The findings of the present study may be helpful for designing allergen panels as well as for allergy doctors and patients suffering from pollen allergies.Öğe A rare cause of preseptal cellulitis: Anthrax(BLACKWELL PUBLISHING, 2007) Artac, Hasibe; Silahli, Musa; Keles, Sevgi; Ozdemir, Mustafa; Reisli, IsmailInfection of the eyelids confined to the preseptal space is relatively common but potentially serious. We report a child with cutaneous anthrax to remind that the interesting contagious cause be included in the differential diagnosis of the preseptal cellulitis.Öğe Reduced CD19 expression and decreased memory B cell numbers in transient hypogammaglobulinemia of infancy(SPRINGER-VERLAG ITALIA SRL, 2013) Artac, Hasibe; Kara, Reyhan; Gokturk, Bahar; Reisli, IsmailWe aimed to evaluate the role of the CD19 complex in the pathogenesis of transient hypogammaglobulinemia of infancy (THI) and to better characterize the subsets of memory B cells. The study population consisted of 22 male and 14 female patients with a mean age at presentation of 20 +/- A 9.9 months. The CD19 complex and B cell subsets were evaluated by flow cytometry. While the CD19 median fluorescence index (MFI) in patients with THI was significantly lower than controls (122.9 +/- A 66.7 in patients; 184.2 +/- A 39 in controls, p < 0.01), expression of CD21 and CD81 was increased (94.4 +/- A 3, 96.8 +/- A 2.5 % in patients; 91 +/- A 3.9; 94.7 +/- A 3.5 % in controls, p < 0.01 vs. p < 0.05, respectively). The expressions of switched memory B cells and IgM memory B cells were found to be reduced in THI. Considering that the CD19 complex regulates the events following antigen stimulation, the change in CD19 complex detected in THI may be related to insufficiency of antibody production.Öğe Reference ranges for serum immunoglobulin (IgG, IgA, and IgM) and IgG subclass levels in healthy children(TUBITAK SCIENTIFIC & TECHNICAL RESEARCH COUNCIL TURKEY, 2019) Bayram, Rumeysa Olcay; Ozdemir, Hulya; Emsen, Ayca; Dagi, Hatice Turk; Artac, HasibeBackground/aim: The serum immunoglobulin levels are used routinely in clinical practice because they provide key information on the humoral immune status. This study aimed to determine the age-related reference values of serum immunoglobulin levels in healthy children. Materials and methods: A total of 330 healthy children, aged between 0 and 18 years, were included in this study. The serum immunoglobulin levels were measured using a nephelometric method in a total of 11 groups, each group consisting of 30 individuals, and IgG subclasses in 6 groups of children aged more than 2 years. Results: The serum IgG levels were high during the newborn period, decreased until the sixth month, and again increased to a maximum level at the age of 18 years. The level of IgA was found to be extremely low in the newborn period and then increased with age. While the lowest value was in the newborn period for serum IgM level, the highest value was in the 16- to 18-year-old period. The IgG subclasses varied depending on the age groups. Conclusion: The updated reference intervals of immunoglobulin levels in children may be used for the accurate diagnosis of immune deficiencies.Öğe Sarcoid-like granulomas in common variable immunodeficiency(SPRINGER HEIDELBERG, 2009) Artac, Hasibe; Bozkurt, Banu; Talim, Beril; Reisli, IsmailCommon variable immunodeficiency (CVID) is a disorder characterized by hypogammaglobulinemia, poor antibody responses and recurrent bacterial infections. CVID patients have a higher prevalence of autoimmune disease and some of them develop noncaseating granulomas of the lungs, spleen, liver, skin, lymph nodes and eye. We report herein a 5-year-old girl with CVID presented with cutaneous nodules, granulomatous uveitis and oligoarthritis. The lesions, arthritis and uveitis responded well to treatment with the systemic administration of steroid. Different autoimmune diseases could be seen together in children with CVID. These patients require therapeutic cooperation of the immunologists with different specialists, including dermatologists, rheumatologists and ophthalmologists.
