Browsing by Author "Erbay, Ayse"
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Item A Case of Idiopathic Pulmonary Hemosiderosis Presenting with Signs and Symptoms Mimicking Hemolytic Anemia(2017) Dogruel, Dilek; Erbay, Ayse; Yazici, Nalan; Arslan, Alev; Bicen, Bermal Hasbay; https://orcid.org/0000-0003-4444-0027; 27918350; F-6136-2016; AAM-5138-2021; V-1112-2019Idiopathic pulmonary hemosiderosis is primarily a disorder of childhood, which is characterized by hemoptysis, iron deficiency anemia, and diffuse parenchymal infiltrates on chest x-ray secondary to recurrent attacks of alveolar hemorrhage. It can be diagnosed by showing hemosiderin laden macrophages in bronchoalveolar lavage fluid after other specific causes of diffuse alveolar hemorrhage are definitely excluded. A 5-year-old male patient was admitted to our clinic with sudden-onset pallor during iron therapy given for anemia. While he was being investigated for clinical and laboratory signs mimicking hemolytic anemia, he developed cough and dyspnea. He had infiltrates on chest x-ray and scattered patchy infiltrates in both lungs on high-resolution computed tomography. Hemosiderin laden macrophages were identified in fasting gastric juice and bronchoalveolar lavage fluid. The patient was diagnosed with idiopathic pulmonary hemosiderosis and started corticosteroid therapy.Item A Different Cause for Respiratory Disorder in Children: Cases with Pulmonary Langerhans Cell Histiocytosis(2017) Asilsoy, Suna; Yazici, Nalan; Demir, Senay; Erbay, Ayse; Kocer, Emrah; Sarialioglu, Faik; https://orcid.org/0000-0003-4465-8229; https://orcid.org/0000-0002-4209-9075; https://orcid.org/0000-0002-8257-810X; 26083968; AAM-5138-2021; AAK-9310-2021; AAL-7766-2021Background and AimsIn children, complaints of a respiratory disorder are very frequent. Etiology of respiratory illness is a broad spectrum that varies from a simple viral infection to a malignant disorder. Pulmonary Langerhans cell histiocytosis (PLCH) is one of these entities and it is truly rare in children. The aim of this study is to evaluate our patients with PLCH. MethodsPatients who had been diagnosed with PLCH were retrospectively evaluated. Features of medical history, onset of the complaints, date of the diagnosis, chest X-Ray and computed tomography (CT) findings, histopathology and other laboratory investigations were considered. ResultsThere were four cases with PLCH. All of them were male, ages were between 5 months and 16 years. In three cases, major complaints were chronic respiratory problems whereas in one of them there was acute respiratory distress beginning with cough and leading to pneumothorax. In all of the cases, multisystemic involvement was prominent. The diagnosis was proven by histopathology in all of the cases. In two children with smaller age, skin involvement was detected. Time from complaint to diagnosis was minimum 3 months and maximum 3 years. ConclusionPLCH is a rare disorder in children. Pulmonary involvement is generally a component of systemic involvement but in many cases it might have been detected with early respiratory complaints. So, children with chronic respiratory problems should be carefully evaluated and should be followed up for rare entities like PLCH.Item Intracranial Hemorrhage, Cerebral Venous Thrombosis, and Hypereosinophilia(2023) Aktekin, Elif Habibe; Erbay, Ayse; Saygi, Semra; Yazici, Nalan; 37879137Item Measurements of Retinal Nerve Fiber Thickness and Ganglion Cell Complex in Neurofibromatosis Type 1, with and Without Optic Pathway Gliomas: A Case Series(2018) Sahinoglu-Keskek, Nedime; Altan-Yaycioglu, Rana; Canan, Handan; Coban-Karatas, Muge; Erbay, Ayse; Yazici, Nalan; Alkan, Ozlem; https://orcid.org/0000-0001-8544-103X; https://orcid.org/0000-0002-9139-8848; https://orcid.org/0000-0002-5877-6536; https://orcid.org/0000-0001-7526-3460; 29185816; T-4258-2017; AAG-3306-2019; AAB-6394-2021; AAM-5138-2021; AAM-4169-2021Purpose: The aim of this study was to investigate differences in retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thicknesses in neurofibromatosis 1 (NF1) cases, with and without optic pathway gliomas (OPGs). Materials and Methods: In total, 33 eyes of 33 subjects were evaluated in this prospective observational case series. Twenty-one patients with a diagnosis of NF1 were enrolled. Patients with NF1 and OPGs were included in Group 1 (n=9), and patients with NF1 without OPGs were included in Group 2 (n=12). The control group (Group 3) was comprised of 12 age- and sex-matched subjects with no history of ophthalmic or systemic diseases. All of the subjects underwent complete ophthalmic examinations, including best-corrected visual acuity (BCVA), slit lamp microscopy, and indirect ophthalmoscopy. Additionally, optical coherence tomography (OCT) measurements were obtained. Results: There were no statistically significant between-group differences in age and sex (p=0.227 and 0.986, respectively). The average RNFL thickness in Group 1 (NF1 patients with OPGs) was significantly lower than in Groups 2 and 3 (86.6 +/- 22.5, 107.4 +/- 6.65, and 108.4 +/- 5.05 mu m, respectively; p=0.001). Furthermore, the average GCC thickness in Group 1 was significantly lower than in Groups 2 and 3 (78.6 +/- 16.3, 94.8 +/- 3.55, and 94.9 +/- 3.82 mu m, respectively; p<0.001). Conclusions: Both RNFL and GCC thicknesses were significantly lower in NF1 patients with OPGs. The use of OCT to quantify damage to the visual pathway may enable earlier detection of OPGs in NF1 patients.Item MicroRNA-17, MicroRNA-19b, MicroRNA-146a, MicroRNA-302d Expressions in Hepatoblastoma and Clinical Importance(2019) Ecevit, Cigdem O.; Aktas, Safiye; Yildirim, Hulya Tosun; Demirag, Bengu; Erbay, Ayse; Karaca, Irfan; Celik, Ahmet; Demir, Ayse Banu; Ercetin, Ayse Pinar; Olgun, Nur; 29889802Hepatoblastoma (HB) is the most common liver malignancy in children. The prognosis changes according to the histologic subtypes of HB. In the present study, we aimed to characterize the expression level of selected microRNAs (miRNAs) in HB as well as in histologic subtypes, and to consider the association with the prognosis. A total of 22 HB tumor samples, subtyped as fetal (n= 16) and embryonal (n= 6), and 10 nontumorous surrounding liver samples were evaluated in this study. Expressions of miR-17, miR-146a, miR-302d, and miR-19b were analyzed in 22 HB tumor samples and 10 nontumorous surrounding liver samples by quantitative real-time polymerase chain reaction. Lower miRNA-17 expression levels were obtained in tumor samples in comparison with nontumorous surrounding liver samples (P= 0.028). Lower miRNA-17 expression was significant for predicting prognosis in HB patients (area under receiver-operator characteristic curve= 0.875, P= 0.044). A higher-level of miR-19b was found in embryonal samples (P= 0.008). Overall and event-free survival was not found to correlate with miRNA expression levels (P> 0.05). This research finds miRNA-17 and miRNA-19b expression levels can provide important data on diagnosis and prognosis in HB showing different clinical behaviors.Item Neuromyelitis Optica Mimics the Morphology of Spinal Cord Tumors(2016) Erol, Ilknur; Ozkale, Murat; Savas, Tulin; Alkan, Ozlem; Cekinmez, Melih; Erbay, Ayse; 0000-0002-3530-0463; 0000-0003-0625-1057; 0000-0001-9658-9005; 0000-0001-7526-3460; 28266199; AAK-4825-2021; A-7806-2016; AAM-4169-2021Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system, that predominantly affects the spinal cord and the optic nerve. Its key features include transverse myelitis, commonly associated with extensive inflammation spanning three or more consecutive vertebral segments. Longitudinal extensive spinal cord lesions can also occur in systemic autoimmune diseases, infections, vascular and metabolic disorders, subsequent to irradiation, intramedullary tumors and paraneoplastic myelopathies. We present a case study of an 8-year-old girl seropositive for antibodies against the aquaporin 4 who displayed longitudinal extensive spinal cord lesions, that was initially misdiagnosed as an intramedullary tumor.Item Pathology, Classification, Clinical Manifestations and Prognosis of Langerhan's Cell Histiocytosis: A Single Center Experience(2022) Hasbay, Bermal; Kocer, Nazim Emrah; Kayaselcuk, Fazilet; Canpolat, Emine Tuba; Buyukkurt, Nurhilal; Erbay, AyseObjective: The aim of the study is to raise awareness about clinical features, histopathological and radiological analyzes and treatment details of this rare disease. Methods: A total of 55 Langerhans cell histiocytosis patients, diagnosed between the year 2006 and October 2020 in our department were included in the study. The patients were evaluated in terms of age, gender, tumor localization, risk groups, treatment modalities, recurrence, and outcome of the disease. Results: Twenty-three out of 55 patients were children and 32 were adults. The ages of the patients were between 7 months and 72 years. Thirty-seven of the cases were male and 18 were female. The most common clinical complaint in both groups was pain and swelling. The duration between the onset of the patient complaints and admission to the hospital varies between 7 days-12 months in children, and 10 days-23 years in adults. Forty-three of the cases had single organ involvement and 12 had multiorgan involvement. The most frequently affected organ in both groups was bone. Forty of the 55 patients had follow-up data and the treatment modalities are as follows: Nine patients radiotherapy, 8 patients chemotherapy+steroid, 7 patients chemotherapy, 2 patients chemotherapy+radiotherapy+steroid, 1 patient steroid, 2 patients chemotherapy+radiotherapy. Eleven patients were followed up without additional treatment after surgery. Median follow-up from the time of biopsy was 45.9 months in children and 41.9 months in adults. Conclusions: As a result, diagnosis requires a high degree of suspicion and final diagnosis is based on the histological examination of the lesions and biopsies.Item Pediatric Langerhans Cell Histiocytosis: Single Center Experience Over A 17-Year Period(2016) Ince, Dilek; Demirag, Bengu; Ozek, Gulcihan; Erbay, Ayse; Ortac, Ragip; Oymak, Yesim; Kamer, Serra; Yaman, Yontem; Kundak, Selcen; Vergin, Canan; 28276205This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.Item Secondary Hemophagocytic Lymphohistiocytosis: Do We Really Need Chemotherapeutics for All Patients?(2017) Haytoglu, Zeliha; Yazici, Nalan; Erbay, Ayse; https://orcid.org/0000-0002-8371-5137; 28060133; AAM-5138-2021Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio= 1.02, P = 0.006), pediatric logistic organ dysfunction scores (hazard ratio = 1.01, P = 0.001) were the predictors of the survival. The hospital survival was 83% for patients with sHLH who were treated with less immunosuppressive therapy. In conclusion initiation of HLH-specific therapy for the patients with hyperferritinemia-associated sHLH should be delayed while awaiting resolution of systemic inflammation with less immunosuppressive therapy.Item Serum Neuron-specific Enolase Levels in Preterm and Term Newborns and in Infants 1-3 Months of Age(2015) Abbasoglu, Aslihan; Sarialioglu, Faik; Yazici, Nalan; Bayraktar, Nilufer; Haberal, Aysegul; Erbay, Ayse; 25315754Background: Elevated serum levels of neuron-specific enolase (NSE) was initially assumed to be specific to neuronal tumors (particularly neuroblastoma), but is now known to accompany nontumoral conditions and tumors other than neuroblastomas. There is a need to establish normal ranges for NSE, especially in early infancy. The aims of this study were to determine reference values for NSE in newborns and young infants and to assess whether NSE levels in early infancy (i.e., preterm infants and term infants) differ from the adult reference range for this enzyme. Methods: We enrolled 140 healthy babies, which included 40 preterm newborns (3-15 days old and born at 28-42 weeks gestation), 40 term newborns (< 1 month old and born at term), and 60 young infants 1-3 months old (n = 20 per subgroup of 1-, 2-, and 3-month-old infants). The determination of NSE levels was performed by the electrochemiluminescence immunoassay (ECLIA) method using the Elecysys 2010 device (Roche Diagnostics, Mannheim, Germany). The mean serum NSE levels for the preterm newborns was 21.83 +/- 15.06 ng/mL [95% confidence interval (95%Cl), 16.95-26.71 ng/mL]; term newborns, 18.06 +/- 12.83 ng/mL (95%Cl, 13.94-22.19 ng/mL); and young infants, 9.09 +/- 4.38 ng/mL (95%Cl, 7.96 -10.23 ng/mL). The mean serum NSE level for infants 1-3 months old was within the ECLIA kit's normal range (4.7-18 ng/mL for adults), whereas the corresponding means for the preterm and term newborns were higher (p < 0.001, for both). Conclusion: Our findings suggest that adult reference values should not be applied to the pre-term and term age groups. Copyright (C) 2014, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved.Item Tuberous sclerosis complex; a single center experience(2015) Erol, Ilknur; Savas, Tulin; Sekerci, Sevda; Yazici, Nalan; Erbay, Ayse; Demir, Senay; Saygi, Semra; Alkan, Ozlem; 26078697Aim: This study was planned with the aim of retrospectively reviewing the clinical and laboratory findings and therapies of our patients diagnosed with tuberous sclerosis and redefining the patients according to the diagnostic criteria revised by the 2012 International Tuberous Sclerosis Complex Consensus Group and comparing them with the literature. Material and Methods: 20 patients diagnosed with tuberous sclerosis complex in the Pediatric Neurology Clinic were examined retrospectively in terms of clinical findings and therapies. The diagnoses were compared again according to 1998 and 2012 criteria. Results: It was observed that the complaint at presentation was seizure in 17 of 20 patients and hypopigmented spots on the skin in 3 of 20 patients. On the initial physical examination, imaging findings related with the disease were found in the skin in 17 of the patients, in the eye in 5, in the kidneys in 7 and in the brain in 17. No cardiac involvement was observed in the patients. Infantile spasm was observed in 7 of the patients who presented because of seizure (n=17), partial seizure was observed in 7 and multiple seizure types were observed in 3. It was found that sirolimus treatment was given to 9 of 20 patients because of different reasons, 7 of these 9 patients had epileptic seizures and sirolimus treatment had no effect on epileptic seizures. According to 2012 diagnostic criteria, no marked change occured in the diagnoses of our patients. Conclusions: It was observed that the signs and symptoms of our patients were compatible with the literature. Molecular genetic examination was planned for the patients who were being followed up because of possible tuberous sclerosis complex. It was observed that sirolimus treatment had no marked effect on the seizure frequency of our patients.Item Use of Proporanolol Compounding from Tablet in Infantile Hemangiomas(2017) Yazici, Nalan; Sarialioglu, Faik; Erbay, Ayse; 0000-0002-8257-810X; 0000-0003-4465-8229; 28612583; AAL-7766-2021; AAM-5138-2021Item Variability of Dose and Level of Sirolimus in a Patient With Tuberous Sclerosis Complex and Subependymal Giant Cell Astrocytoma(2016) Yazici, Nalan; Sarialioglu, Faik; Alkan, Ozlem; Erol, Ilknur; Erbay, Ayse; 0000-0003-4465-8229; 0000-0002-3530-0463; 0000-0001-7526-3460; 0000-0002-8257-810X; 27082208; AAM-5138-2021; AAK-4825-2021; AAM-4169-2021; AAL-7766-2021Item Variations in apparent diffusion coefficient values following chemotherapy in pediatric neuroblastoma(2015) Demir, Senay; Altinkaya, Naime; Kocer, Nazim Emrah; Erbay, Ayse; Oguzkurt, Pelin; 25519453PURPOSE In children the assessment of solid tumors' response to chemotherapy is based primarily on size reduction, which can be unreliable and a late marker, in the presence of necrosis. We aimed to establish whether apparent diffusion coefficient (ADC) values of childhood neuroblastomas show proportional changes in relation to chemotherapy response. METHODS We evaluated 15 pediatric patients with abdominopelvic neuroblastomas, who had undergone MRI before and after chemotherapy. Two radiologists retrospectively analyzed all images by drawing a round uniform region-of-interest in the solid/contrast-enhancing portion of the lesions in consensus. The ADC values from pre- and postchemotherapy images were compared. RESULTS Postchemotherapy ADC values were significantly higher than those obtained before treatment (P < 0.05, for minimum, maximum, and median ADC values). CONCLUSION Our results support diffusion-weighted MRI as a promising noninvasive biomarker of therapeutic responses. To the best of our knowledge, this is the first report to compare diffusion-weighted imaging findings before and after chemotherapy in childhood neuroblastic tumors.