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    The frequency of lysosomal acid lipase deficiency in children with unexplained liver disease
    (LIPPINCOTT WILLIAMS & WILKINS, 2019) Kuloglu, Zarife; Kansu, Aydan; Selbuz, Suna; Kalayci, Ayhan G.; Sahin, Gulseren; Kirsaclioglu, Ceyda Tuna; Demiroren, Kaan; Dalgıç, Buket; Kasırga, Erhun; Önal, Zerrin; İşlek, Ali; National LAL-D Study Group
    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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    Sydenham's chorea: A clinical follow-up of 65 patients
    (SAGE PUBLICATIONS INC, 2007) Demiroren, Kaan; Yavuz, Haluk; Cam, Lokman; Oran, Bulent; Karaaslan, Sevim; Demiroren, Saadet
    Sydenham's chorea, the neurological manifestation of rheumatic fever, is the most common acquired chorea of childhood. In this retrospective study, the authors aim to present the clinical and laboratory findings of 65 Sydenham's chorea patients, followed up in a clinic over less than 7 years. The mean age at the onset of the symptoms was 11.7 +/- 2.6 years (range, 6-17 years). Of the patients, 63% were female and 37% were male (male/female: 1.7/1). Chorea was generalized in 78.5% of the patients, right hemichorea in 12.3%, and left hemichorea 9.2%. There was a history of rheumatic fever in 30.8% of the patients. Echocardiographic study showed cardiac valve involvement in 70.5% of 61 patients. Brain magnetic resonance imaging, which was performed on only 18 patients, was evaluated as normal in all. Electroencephalography was also performed on only 18 patients and showed abnormal waves in 50% of them. Pimozide was mostly the first choice of drug therapy. Nevertheless, drug therapy was not needed in 18.5% of the patients. The recovery period of the first attack of the chorea was I to 6 months in 51.7% of the patients. The recurrence rate was 37.9%. In conclusion, Sydenham's chorea is still an important health problem in Turkey with respect to its morbidity.
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    Unexpected Result in the Etiological Approaching to an Anemic Case: a Leech Infestation
    (TAYLOR & FRANCIS INC, 2003) Demiroren, Kaan; Caliskan, Ümran
    One of the causes of the childhood anemia is gastrointestinal system bleeding, which rarely results from parasites. The authors report on a 3-year-old boy with severe anemia and a history of hematemesis. While they were investigating the cause of the anemia and the hematemesis, a leech showing itself in the nasal passage led to a quick and exact treatment. Leeches should be considered for differential diagnosis of anemia and gastrointestinal system bleeding in the children.