Wos Açık Erişimli Yayınlar

Permanent URI for this collectionhttps://hdl.handle.net/11727/10754

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Author: search.filters.author.Canan, OguzSubject: search.filters.subject.children

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    The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease
    (2019) Ozcay, Figen; Canan, Oguz; 0000-0002-5214-516X; 30540705; ABG-5684-2020
    Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study. Methods: Patients (aged 3 months-18 years) who had unexplained transaminase elevation, unexplained hepatomegaly or hepatosplenomegaly, obesity-unrelated liver steatosis, biopsy-proven cryptogenic fibrosis and cirrhosis, or liver transplantation for cryptogenic cirrhosis were enrolled. A Web-based electronic data collection system was used. LAL activity (nmol/punch/h) was measured using the dried blood spot method and classified as LAL-D(<0.02), intermediate (0.02-0.37) or normal (>0.37). Asecond dried blood spot sample was obtained from patients with intermediate LAL activity for confirmation of the result. Results: A total of 810 children (median age 5.6 years) from 795 families were enrolled. The reasons for enrollment were unexplained transaminase elevation (62%), unexplained organomegaly (45%), obesity-unrelated liver steatosis (26%), cryptogenic fibrosis and cirrhosis (6%), and liver transplantation for cryptogenic cirrhosis (<1%). LAL activity was normal in 634 (78%) and intermediate in 174 (21%) patients. LAL-D was identified in 2 siblings aged 15 and 6 years born to unrelated parents. Dyslipidemia, liver steatosis, and mild increase in aminotransferases were common features in these patients. Moreover, the 15-year-old patient showed growth failure and microvesicular steatosis, portal inflammation, and bridging fibrosis in the liver biopsy. Based on 795 families, 2 siblings in the same family were identified as LAL-D cases, making the prevalence of LAL-D in this study population, 0.1% (0.125%-0.606%). In the repeated measurement (76/174), LAL activity remained at the intermediate level in 38 patients. Conclusions: Overall, the frequency of LAL-D patients in this study (0.1%) suggests that LAL-D seems to be rare even in the selected high-risk population.
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    Percutaneous endoscopic gastrostomy experience in children and family satisfaction
    (2019) Canan, Oguz
    Purpose: The aim of this study was to evaluate the demographic data, complication rates of children who underwent percutaneous endoscopic gastrostomy (PEG) and to question family satisfaction. Materials and Methods: Demographic information, underlying diseases, anthropometric measurements before and after PEG and z scores advanced complications due to processing and follow-up periods were obtained from patient files. Results: In three years, 21 patients underwent PEG procedures. The median age of the patients was 74 months. The majority of the cases consisted of children with neurological, oncological and metabolic diseases (71.4%, 14.3% and 14.3%, respectively). There was a statistically significant increase between baseline and 6th month and between baseline and 12th month of the all antropometric measures z scores. But no significant difference was observed between 6th month and 12th month of the median weight and height z scores. Parents' opinions about PEG were positive. After the procedure, one patient had ostomy leakage and three patients had local stoma infection. Conclusion: Percutaneous endoscopic gastrostomy is a very successful and reliable method in children and adolescents as well as in infants. Families' opinions on PEG after the procedure were positive.