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Item Neurologic Complications After Pediatric Heart Transplant: A Single-Center Experience(2022) Orgun, Ali; Erdogan, Ilkay; Varan, Birgul; Sezer, Taner; Tokel, N. Kursad; Ozkan, Murat; Sezgin, Atilla; 33797352Objectives: Neurologic complications that can lead to serious mortality and morbidity in pediatric heart transplant recipients have been reported to range from 23.6% to 45%. In this study, the frequency, time, cause, and characteristics of neurologic complications in pediatric heart transplant recipients were evaluated. Materials and Methods: We retrospectively reviewed data of 37 pediatric heart transplant recipients aged <18 years who were seen at our hospital between 2007 and 2017. Medical records were reviewed to identify neurologic complications. Clinical features were compared between pediatric heart transplant patients with and without neurologic complications. Results: The rate of posttransplant neurologic complications in pediatric heart transplant was 27% (10/37). Median age of patients with neurologic complications was 12 years (range, 11-18 years). Median time for neurologic complications was 3 days (range, 2-46 days). Primary diagnoses of these 10 recipients were dilated cardiomyopathy (n = 7) and restrictive cardiomyopathy (n = 3). There were no significant differences between recipients with and without neurologic complications (P>.05). The etiologies of neurologic complications were posterior reversible encephalopathy syndrome in 3 patients (8.1%), stroke in 2 patients (5.4%), peripheral neuropathy in 2 patients (5.4%), hypertensive encephalopathy in 1 patient (2.7%), and drug encephalopathy in 1 patient (2.7%). Conclusions: Neurologic complications may lead to serious mortality and morbidity in pediatric heart transplant patients. Seizures, posterior reversible encephalopathy syndrome, stroke, peripheral neuropathy, transient ischemic attack, and cerebral infections are the most common neurologic complications, which are seen in the perioperative period in particular. Careful follow-up of pediatric heart transplant patients, with detection and early treatment of neurologic findings, will contribute to lower rates of sequelae. To our knowledge, this is the largest study to show a detailed experience of neurologic complications in pediatric heart transplant patients from a single center in Turkey.Item The Prevalence and Clinical Characteristics of Primary Stabbing Headache(2022) Saygi, Semra; https://orcid.org/0000-0002-8522-5078; 36245416; AAB-1203-2021Objective This study aimed to determine the prevalence and characteristics of primary stabbing headache in children and adolescents that presented because of headache. Materials and Methods: The medical files of 772 children and adolescents who presented with headache to the Pediatric Neurology Outpatient Clinic at Baskent University between 2012 and 2020 were retrospectively reviewed. In total, 77 patients (9.97%) with primary stabbing headache and those thought to have primary stabbing headache were included in the study. Patient data, including demographic features, headache characteristics, family history of primary headache, electroencephalographic (EEG) findings, and cranial magnetic resonane imaging (MRI) findings, were noted. Results: Age at presentation was <6 years in 16.9% of the patients and onset time of headache was below 3 months in 55.8%. Daily headache attacks occurred in 46.8% of the patients. Headache localization was frontal in 54.5% of patients and bilateral in 68.8%, whereas the quality of headache was undefined in 40.3%. Headache attack duration was seconds long in 37.7% of the patients, attacks occurred at any time of the day in 83.1%, and 80.5% did not have accompanying symptoms. In all, 54.5% of the patients had a negative family history of primary headache. In 95.8% of the patients, EEG findings were normal and cranial MRI findings were normal in 100% of the patients. Conclusion: The prevalence of primary stabbing headache is not rare in children and adolescents. Clinician awareness of the diagnosis and underlying causes of primary stabbing headache should be increased.Item Case Report First pediatric case with primary familial brain calcification due to a novel variant on the MYORG gene and review of the literature(2021) Orgun, Leman Tekin; Besen, Seyda; Sangun, Ozlem; Bisgin, Atil; Alkan, Ozlem; Erol, IlknurVariants in the myogenesis-regulating glycosidase (MYORG) gene which is known as the first autosomal recessive gene that has been associated with primary familial brain calcification (AR-PFBC). Although adult patients have been reported, no pediatric case has been reported until now. Herein, we review the clinical and radiological features of all AR- PFBC patients with biallelic variants in the MYORG gene who were reported until now, and we report the youngest patient who has a novel homozygous variant. Since the first identification of the MYORG gene in 2018, 74cases of MYORG variants related to AR-PFBC were evaluated. The ages of symptom onset of the patients ranged between 7.5 and 87 years. The most frequent clinical courses were speech impairment, movement disorder and cerebellar signs. All patients showed basal ganglia calcification usually bilaterally with different severities. Conclusion; herein, we reported the first pediatric patient in the literature who had a novel homozygous variant in the MYORG gene with mild clinic findings. (c) 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.Item The safety and efficacy of ERCP in the pediatric population with standard scopes: Does size really matter?(2016) Yildirim, Abdullah Emre; Altun, Reskan; Ocal, Serkan; Kormaz, Murat; Ozcay, Figen; Selcuk, Haldun; 26933627Experience with endoscopic retrograde cholangiopancreatography in the pediatric population is limited. The aim of this study was to evaluate the outcomes of ERCP in the pediatric population performed by adult gastroenterologists with standard duodenoscopes. This study is a structured retrospective review of endoscopic reports, computerized and paper medical records, and radiographic images of patients under the age of 18 who underwent ERCP for any indication at a tertiary referral centre. Data regarding demographic characteristics and medical history of patients, indications, technical success rate, final clinical diagnosis, and complications were analyzed. Forty-eight children with a mean age of 13 years (range 2-17) underwent a total of 65 ERCPs. The indications of ERCP were as follows; suspected choledocholithiasis (55 %), post-liver transplantation anastomotic biliary strictures (21 %), post-surgical bile duct injury (10 %), choledochal cyst (2 %), recurrent or chronic pancreatitis (10 %), and trauma (2 %). The cannulation success rate in the overall procedure was 93.8 %. Therapeutic interventions were performed in 70.7 % of patients. Post ERCP pancreatitis was the most common complication occurring in 9.2 % of patients, and no procedure related mortality occurred. When performed by well-trained adult gastroenterologists, the use of endoscopic retrograde cholangiopancreatography with standard duodenoscopes is safe in pediatric population.Item A Rare Diagnosis in the Neck During Childhood: Congenital Chondrolipoma(2017) Ozer, Fulya; Bal, Nebil; 0000-0001-5381-6861; 24715556; ABC-1809-2020Chondrolipomas are mesenchymal tumors that found as mature cartilage tissue in a fat tissue. A 2-year-old boy was seen with a complaint of a mass of the neck. On physical examination of the child, there was a one-centimeter mass above the sternocleidomastoid muscle on the lateral neck. Pathological examination of this mass after excision was reported as chondrolipoma. Chondrolipomas are seen mostly in the breast and in the adult. They are rare tumors of the head and neck area and seen mostly the in oral cavity here. This is the first case of chondrolipoma with this age and localization combination in the literature. Chondrolipoma should therefore also be considered in the differential diagnosis of congenital masses located at lateral cervical area.