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    A case of gliosarcoma in a child with neurofibromatosis type 1
    (2020) Doğan, Güleç Mert; Sığırcı, Ahmet; Cengiz , Aslınur; Erbay, Mehmet Fatih; Gökçe, Hasan
    Abstract: Gliosarcoma (GS) is a rarely seen form of glioblastoma. These tumors are mostly seen in males older than 60 years of age. It is extremely rare in pediatric central nervous system (CNS) tumors. In this case report, we present a 3-year-old boy with a giant gliosarcoma. Magnetic resonance (MR) imaging and histopathologic findings are discussed. A 3 year-old boy with a clinically diagnosed NF-1 was admitted to the emergency department with a complaint of intractable vomiting. Magnetic resonance imaging (MRI) of the brain was suggestive of a large lobulated mass lesion in the left parietal lobe extending to the vertex and slightly compressing the left lateral ventricle. The final histopathologic diagnosis of the tumor was considered as gliosarcoma. To our knowledge, this case constitutes the first youngest case with neurofibromatosis type 1 reported in the literature in all pediatric cases of GS.
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    Classical Maple Syrup Urine Disease successfully treatedwith living donor liver transplantation
    (2021) Doğan, Güleç Mert; Doğan, Sait Murat; Okut, Gokalp; Sığırcı, Ahmet; Yılmaz, Sezai
    Maple syrup urine disease (MSUD) is a disease that causes ketoacid accumulation in body. Diffusion-weighted imaging (DWI) is animportant imaging modality for the diagnosis. Two children were diagnosed with MSUD at the neonatal period. They had uncontrolledketosis and epileptic seizures although they were in compliance with their medical nutrition. Their DWIs were similar and showedhigh signal intensity localized within the myelinated white matter areas. Both of the patients were treated with living donor livertransplantation. The patients with classic form of MSUD are normal at birth. If the disease is not diagnosed and treated early, it canlead to serious neurological complications. Most researchers conclude that, the best choice for detecting MSUD encephalopathyin newborns is DWI. The traditional treatment of MSUD had been a protein-restricted diet until the liver transplantation became analternative and better option for the cure of the patients..
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    Evaluation of the relationship of the time of vesicoureteral reflux between renal scar development and endoscopic treatment success in early childhood
    (2022) Taşolar, Sevgi; Sığırcı, Ahmet; Doğan, Güleç Mert; Cengiz, Aslınur; Yıldız, Turan; Subaşı, Vedat; Tabel, Yılmaz
    Aim: It is aimed to evaluate the effect of reflux time in primary VUR in terms of predicting endoscopic treatment success and treatment timing in VUR management. Vesicoureteral reflux (VUR) is an important cause of urinary tract infection and chronic renal disease. Voiding cystourethrography (VCUG) is the gold standard diagnostic test in the diagnosis of VUR. In recent years, new parameters related to VCUG have been more objectively studied to evaluate the clinical prognosis. Materials and Methods: In our study, the records and imaging findings of children with primary VUR who underwent VCUG examination between 2012 and 2019, who were treated with endoscopic injections, were retrospectively reviewed. Forty-one children (67 renal units) were included in our study. Patients with preoperative VCUG, urinary ultrasonography, dimercaptosuccinic acid (DMSA) renal scan, and post-operative control VCUG were included in this study. Results: Patients with preoperative VUR grade 1-2 were divided into two groups as “low grade” and patients with stage 3-4-5 as “high grade”. In the general patient pop- ulation, endoscopic success rates in terms of renal unit deficit were found to be 46.3% (31/67). When the subgroup was analyzed, the endoscopic treatment success rate was 72.2% (13/18) in the low-grade group, versus 36.7% (18/49) in the high-grade group (p = 0.010). In the multivariate logistic regression analysis performed to identify indepen- dent predictors of scarring, reflux degree and reflux time were found to be independent predictors of scarring. Conclusion: In our study, we found that in VCUG evaluated preoperatively, VUR time as well as VUR grade were effective on endoscopic success rate and scar development. Based on these results, it could be thought that VUR time may be effective in selecting the best candidates for surgery and in the management and timing of treatment of VUR patients.
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    Neurofibromas of the bladder in a child with neurofibromatosis type 1
    (Internatıonal braz j urol, 2018) Doğan, Güleç Mert; Sigirci, Ahmet; Karaca, Leyla