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Öğe A rare trigger for photosensitive seizure: fireworks(Springer Heidelberg, 2022) Gürbüz, Gürkan; Gürbüz, Özge Berfu[No Abstract Available]Öğe An unexpected clinical presentation in periodontal Ehler-Danlos syndrome: preterm birth, profound intellectual disability and self-injurious behavior(Lippincott Williams & Wilkins, 2023) Baykal, Saliha; Yıldız, Tuğçe; Gürbüz, Gürkan; Tozkır, Hilmi[Abstract Not Available]Öğe Evaluation of immunization status in patients with cerebral palsy: a multicenter CP-VACC study(Springer, 2022) Bozkaya-Yılmaz, Sema; Karadağ Öncel, Eda; Olgaç-Dündar, Nihal; Gencpinar, Pınar; Sarıoğlu, Berrak; Arican, Pınar; Çavuşoğlu, Dilek; Gürbüz, GürkanChildren with chronic neurological diseases, including cerebral palsy (CP), are especially susceptible to vaccine-preventable infections and face an increased risk of severe respiratory infections and decompensation of their disease. This study aims to examine age-appropriate immunization status and related factors in the CP population of our country. This cross-sectional prospective multicentered survey study included 18 pediatric neurology clinics around Turkey, wherein outpatient children with CP were included in the study. Data on patient and CP characteristics, concomitant disorders, vaccination status included in the National Immunization Program (NIP), administration, and influenza vaccine recommendation were collected at a single visit. A total of 1194 patients were enrolled. Regarding immunization records, the most frequently administrated and schedule completed vaccines were BCG (90.8%), hepatitis B (88.9%), and oral poliovirus vaccine (88.5%). MMR was administered to 77.3%, and DTaP-IPV-HiB was administered to 60.5% of patients. For the pneumococcal vaccines, 54.1% of children received PCV in the scope of the NIP, and 15.2% of children were not fully vaccinated for their age. The influenza vaccine was administered only to 3.4% of the patients at any time and was never recommended to 1122 parents (93.9%). In the patients with severe (grades 4 and 5) motor dysfunction, the frequency of incomplete/none vaccination of hepatitis B, BCG, DTaP-IPV-HiB, OPV, and MMR was statistically more common than mild to moderate (grades 1-3) motor dysfunction (p = 0.003, p < 0.001, p < 0.001, p < 0.00, and p < 0.001, respectively). Physicians' influenza vaccine recommendation was higher in the severe motor dysfunction group, and the difference was statistically significant (p = 0.029). Conclusion: Children with CP had lower immunization rates and incomplete immunization programs. Clinicians must ensure children with CP receive the same preventative health measures as healthy children, including vaccines.Öğe Investigation of Etiology, Treatment Outcomes and Risk Factors of Epilepsy in Down Syndrome(2022) Gürbüz, Gürkan; Samancı, NedimObjective: Although epilepsy does not appear in the classic definitions of Down syndrome (DS), the prevalence of epilepsy is higher in these cases than in the general population. The purpose of this retrospective study was to evaluate the demographic, neuroradiological, and electrophysiological characteristics, and responses to treatment of patients with DS undergoing epileptic seizure. Methods: Karyotype analysis, time of onset of seizures, types of seizure, electroencephalography (EEG) characteristic, antiepileptic drug used, and comorbidity were considered during evaluation. EEG and magnetic resonance imaging at the time of first admission were assessed during patient evaluation. Results: Patients with DS (n=43) were enrolled in this study. Twenty-three of them were subjects with epilepsy. Seventeen (73.9%) of the 23 patients were boys and six (26.1%) were girls. The mean age of the patients was 21.7 months (standard deviation ± 4.8), and mean age at onset of seizures was 12.6 months. Comorbidity other than epilepsy was present in 13 (56.5%) patients. The most common seizure type, in 14 cases (60.9%), was focal seizures, four of which involved epilepsy developing following stroke secondary to cardiac surgery. Hypothyroidism was observed in all six patients with epileptic spasm. Only four of 20 patients without epilepsy have non-neurologic comorbidities. Conclusion: This study may support the knowledge regarding the relationship between hypothyroidism and epilepsy in DS. Non-neurologic comorbidities are a significant risk factor for epilepsy in DS.Öğe The effectiveness of the ketogenic diet in drug-resistant childhood epilepsy(2022) Yılmaz, Ünsal; Edizer, Selvinaz; Akışın, Zeynep; Köse, Melis; Güzin, Yiğithan; Gürbüz, Gürkan; Baysal, BaharBackground. We aimed to investigate the effectiveness of ketogenic diet (KD) in children with various types of refractory epilepsy. Methods. A total of 91 children (49 females) aged 3 to 193 months (median, 52 months) with drug resistant epilepsy who received KD treatment for at least 12 months were enrolled in the study. Seizure frequency, adherence to diet, reason for discontinuation of KD, and adverse effects were recorded. Response was defined as ?50% improvement in seizure frequency compared to baseline. We also searched for influences of different variables on the outcome. Results. Intent-to-treat analysis revealed an improvement in seizure frequency for ?50% in 73.6%, 80.2%, 75.8%, 73.6%, and 70.3% of patients at month-1, -3, -6, -9, and month-12, respectively. Overall, 32 (35.2%) patients remained seizure-free at month-12. There was no significant differences between responders and nonresponders in terms of age at onset of epilepsy, age at onset of KD, gender, or etiology. Mild hyperlipidemia was associated with a higher response rate. At the last follow-up (median: 20 months), 38 (41.8%) patients were still maintained on KD. While 15.4% of patients completed the diet with a success in seizure control, remainder discontinued KD due to lack of efficacy (23.1%), non-adharence to diet (11%), intercurrent infection (4.4%), adverse effects (3.3%), and death (1.1%). Conclusion. Ketogenic diet treatment appears to be effective in about two-thirds of children with various types of drug-resistant epilepsy, including one-third remaining seizure free. Mild hyperlipidemia seems to be associated with a higher response rate. Discontinuation of KD is mostly due to lack of efficacy or nonadherence, and rarely side effects.Öğe The effectiveness of the ketogenic diet in drug-resistant childhood epilepsy(Turkish J Pediatrics, 2022) Yılmaz, Ünsal; Edizer, Selvinaz; Akışın, Zeynep; Köse, Melis; Güzin, Yiğithan; Gürbüz, Gürkan; Ünalp, AycanBackground. We aimed to investigate the effectiveness of ketogenic diet (KD) in children with various types of refractory epilepsy. Methods. A total of 91 children (49 females) aged 3 to 193 months (median, 52 months) with drug resistant epilepsy who received KD treatment for at least 12 months were enrolled in the study. Seizure frequency, adherence to diet, reason for discontinuation of KD, and adverse effects were recorded. Response was defined as >= 50% improvement in seizure frequency compared to baseline. We also searched for influences of different variables on the outcome. Results. Intent-to-treat analysis revealed an improvement in seizure frequency for >= 50% in 73.6%, 80.2%, 75.8%, 73.6%, and 70.3% of patients at month-1,-3,-6,-9, and month-12, respectively. Overall, 32 (35.2%) patients remained seizure-free at month-12. There was no significant differences between responders and non responders in terms of age at onset of epilepsy, age at onset of KD, gender, or etiology. Mild hyperlipidemia was associated with a higher response rate. At the last follow-up (median: 20 months), 38 (41.8%) patients were still maintained on KD. While 15.4% of patients completed the diet with a success in seizure control, remainder discontinued KD due to lack of efficacy (23.1%), non-adharence to diet (11%), intercurrent infection (4.4%), adverse effects (3.3%), and death (1.1%). Conclusion. Ketogenic diet treatment appears to be effective in about two-thirds of children with various types of drug-resistant epilepsy, including one-third remaining seizure free. Mild hyperlipidemia seems to be associated with a higher response rate. Discontinuation of KD is mostly due to lack of efficacy or nonadherence, and rarely side effects.