Person: YÜCELTEN, AYŞE DENİZ
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YÜCELTEN
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AYŞE DENİZ
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Publication Metadata only Infantile Bullous Pemphigoid Treated Using Intravenous Immunoglobulin: Case Report and Review of the Literature(WILEY-BLACKWELL, 2015) YÜCELTEN, AYŞE DENİZ; Tekin, Burak; Yucelten, Ayse DenizWe report a 5-month-old girl diagnosed with bullous pemphigoid who initially did not respond to systemic corticosteroids and dapsone but rapidly improved after the addition of intravenous immunoglobulin (IVIG) infusions. A literature search revealed anecdotal cases of infantile bullous pemphigoid treated with IVIG, although variable treatment regimens were used, and some resistant cases required additional medications such as rituximab for clinical remission.Publication Metadata only Acquired progressive lymphangioma: Case report with partial response to imiquimod 5% cream(WILEY, 2017) SALMAN, ANDAÇ; Salman, Andac; Sarac, Gonca; Kuru, Burce Can; Cinel, Leyla; Yucelten, Ayse Deniz; Ergun, TulinAcquired progressive lymphangioma (APL), or benign lymphangioendothelioma, is an unusual entity derived from vascular structures. Clinically and histopathologically it may resemble Kaposi's sarcoma and well-differentiated angiosarcoma, causing a diagnostic problem. We report an individual with APL initially diagnosed with Kaposi's sarcoma who underwent unnecessary laboratory testing. Imiquimod 5% cream stopped the progression of the lesion. Awareness of this rare entity may prevent patients from undergoing excessive testing. Imiquimod may be used as a safe, effective treatment option.Publication Metadata only Potentially Beneficial Effect of Hydroxychloroquine in a Patient with a Novel Mutation in Protein Kinase C delta Deficiency(SPRINGER/PLENUM PUBLISHERS, 2015) ÖZEN, AHMET OĞUZHAN; Kiykim, Ayca; Ogulur, Ismail; Baris, Safa; Salzer, Elisabeth; Karakoc-Aydiner, Elif; Ozen, Ahmet Oguzhan; Garncarz, Wojciech; Hirschmugl, Tatjana; Krolo, Ana; Yucelten, Ayse Deniz; Boztug, Kaan; Barlan, Isil B.Protein kinase C delta (PRKCD) has essential functions in controlling B-cell proliferation and apoptosis, development of B-cell tolerance and NK-cell cytolitic activity. Human PRKCD deficiency was recently identified to be causative for an autoimmune lymphoproliferative syndrome like disorder with significant B-cell proliferation particularly of immature B cells. Here we report a child with a novel mutation in PRKCD gene who presented with CMV infection and an early onset SLE-like disorder which was successfully treated with hydroxychloroquine.Publication Metadata only Low Density Granulocytes and Dysregulated Neutrophils Driving Autoinflammatory Manifestations in NEMO Deficiency(SPRINGER/PLENUM PUBLISHERS, 2022) ÖZEN, AHMET OĞUZHAN; Yilmaz, Naz Surucu; Eltan, Sevgi Bilgic; Kayaoglu, Basak; Geckin, Busranur; Heredia, Raul Jimenez; Sefer, Asena Pinar; Kiykim, Ayca; Nain, Ercan; Kasap, Nurhan; Dogru, Omer; Yucelten, Ayse Deniz; Cinel, Leyla; Karasu, Gulsun; Yesilipek, Akif; Sozeri, Betul; Kaya, Goksu Gokberk; Yilmaz, Ismail Cem; Baydemir, Ilayda; Aydin, Yagmur; Kahraman, Deniz Cansen; Haimel, Matthias; Boztug, Kaan; Karakoc-Aydiner, Elif; Gursel, Ihsan; Ozen, Ahmet; Baris, Safa; Gursel, MaydaNF-kappa B essential modulator (NEMO, IKK-gamma) deficiency is a rare combined immunodeficiency caused by mutations in the IKBKG gene. Conventionally, patients are afflicted with life threatening recurrent microbial infections. Paradoxically, the spectrum of clinical manifestations includes severe inflammatory disorders. The mechanisms leading to autoinflammation in NEMO deficiency are currently unknown. Herein, we sought to investigate the underlying mechanisms of clinical autoinflammatory manifestations in a 12-years old male NEMO deficiency (EDA-ID, OMIM #300,291) patient by comparing the immune profile of the patient before and after hematopoietic stem cell transplantation (HSCT). Response to NF-kB activators were measured by cytokine ELISA. Neutrophil and low-density granulocyte (LDG) populations were analyzed by flow cytometry. Peripheral blood mononuclear cells (PBMC) transcriptome before and after HSCT and transcriptome of sorted normal-density neutrophils and LDGs were determined using the NanoString nCounter gene expression panels. ISG15 expression and protein ISGylation was based on Immunoblotting. Consistent with the immune deficiency, PBMCs of the patient were unresponsive to toll-like and T cell receptor-activators. Paradoxically, LDGs comprised 35% of patient PBMCs and elevated expression of genes such as MMP9, LTF, and LCN2 in the granulocytic lineage, high levels of IP-10 in the patient's plasma, spontaneous ISG15 expression and protein ISGylation indicative of a spontaneous type I interferon (IFN) signature were observed, all of which normalized after HSCT. Collectively, our results suggest that type I IFN signature observed in the patient, dysregulated LDGs and spontaneously activated neutrophils, potentially contribute to tissue damage in NEMO deficiency.Publication Metadata only Coexistence of Aplasia Cutis and Nevus Psiloliparus-Report of a Novel Case(WILEY-BLACKWELL, 2014) YÜCELTEN, AYŞE DENİZ; Tekin, Burak; Yucelten, Ayse Deniz; Akpinar, Ihsan Nuri; Ekinci, GazanferNevus psiloliparus is a type of mesodermal nevus of the scalp classically seen with encephalocraniocutaneous lipomatosis. The close association between nevus psiloliparus and aplasia cutis congenita is called didymosis aplasticopsilolipara. Although typically associated with neurologic, ocular, and skeletal findings, didymosis aplasticopsilolipara can be seen without the context of encephalocraniocutaneous lipomatosis.Publication Metadata only Development of a Quality of Life Scale for Vitiligo(KARGER, 2013) YÜCELTEN, AYŞE DENİZ; Senol, Asli; Yucelten, Ayse Deniz; Ay, PinarBackground: No vitiligo-specific quality of life scale exists. Objective:To develop a reliable and valid quality of life scale for vitiligo patients. Methods: The content was derived from in-depth interviews with vitiligo patients. The internal consistency, test-retest reliability and validity of the scale, VLQI (Vitiligo Life Quality Index), were evaluated. Results: Internal consistency was high with 30 patients and then with 183 patients (Cronbach's a 0.92 and 0.91). Test-retest scores were correlated (r = 0.86, p < 0.001). There was no difference between the test and retest scores (p > 0.05). VLQI was correlated with DLQI (Dermatology Life Quality Index) (r = 0.77, p < 0.001) and with the perceived severity by the patients (r = 0.57, p < 0.001). There was a significant relationship between the VLQI and the extent of the disease (p = 0.015). Factor analysis revealed six subscales. Conclusion: The VLQI is a valid and reliable instrument assessing quality of life of vitiligo patients. Copyright (C) 2013 S. Karger AG, BaselPublication Metadata only Pediatrician's role in the management of giant congenital melanocytic nevus: Case report [Dev konjenital melanositik nevus yönetiminde pediatri hekiminin rolü](Turkiye Klinikleri, 2015) YÜCELTEN, AYŞE DENİZ; Boran P., Yücelten D., Subaşi B., Berenjian A.Although, congenital giant melanocytic nevi (CGMN) are uncommon in pediatric patients, they carry an increased risk of malignancy, neurocutaneous melanocytosis, and malformations. The management requires a multidisciplinary approach and the pediatrican plays an important role. We aimed to report a case with CGMN, and discuss the management with special emphasis on the pediatrican's role. During a well child visit, physical examination of a 3 months old girl revealed a circumferential large melanocytic nevus over her right leg. There were satellite lesions on her left leg, and back. Melanocytic nevus presents a challenge to the pediatricans because they are lacking the necessary knowledge. Close follow up with with documentation of the size, and location of the nevi, palpation of the lesion for nodule, measurement of head circumference and neurodevelopmental assessment, taking an appropriate medical history with emphasis on neurological symptoms associated with neurocutaneous melanocytosis is important. Copyright © 2015 by Türkiye Klinikleri.