Browsing by Author "Kansu, A"

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    The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease
    Kuloglu, Z; Kansu, A; Selbuz, S; Kalayci, AG; Sahin, G; Kirsaclioglu, CT; Demirören, K; Dalgiç, B; Kasirga, E; Önal, Z; Islek, A; Eren, E; Hosnut, FÖ; Urganci, N; Yaman, A; Özkan, T; Bozbulut, E; Dogan, G; Eksi Bozbulut, N; Dogan, G; Durmaz Ugurcan, Ö; Usta, AM; Arslan, D; Akçam, M; Isik, IA; Ecevit, ÇÖ; Usta, Y; Özgür, T; Özçay, F; Balamtekin, N; Öztürk, Y; Balamtekin, N; Öztürk, Y; Cantez, S; Gülerman, F; Üstündag, GH; Emiroglu, HH; Karacabey, N; Comba, A; Erdemir, G; Aydogan, AU; Gökçe, S; Kuyum, P; Gülsan, M; Tosun, MS; Tokgöz, Y; Güven, B; Yüksekkaya, H; Tümgör, G; Eren, M; Baran, M; Gümüs, M; Canan, O; Kocamaz, H; Gerenli, N; Çakir, M; Agin, M; Hizli, S; Dogan, Y; Çeltik, Ç; Deveci, U; Balci Sezer, O
    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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    Comparison of two different regimens of combined interferon-α2a and lamivudine therapy in children with chronic hepatitis B infection
    Kansu, A; Doganci, T; Akman, SA; Artan, R; Kuyucu, N; Kalayci, AG; Dikici, B; Dalgiç, B; Selimoglu, A; Kosirga, E; Özkan, TB; Kuloglu, Z; Aydogdu, S; Bosnak, M; Ertekin, V; Tanir, G; Haspolat, K; Girgin, N; Yagci, RV
    Aim: To evaluate the efficacy of two regimens of combined interferon-alpha 2a (IFN-alpha 2a) and lamivudine (3TC) therapy in childhood chronic hepatitis B. Methods: A total of 177 patients received IFN-alpha 2a, 9 million units (MU)/m(2) for 6 months. In group 1 (112 patients, 8.7 +/- 3.5 years), 3TC (4 mg/kg/day, max 100 mg) was started simultaneously with IFN-alpha 2a, in group 11 (65 patients, 9.6 +/- 3.8 years) 3TC was started 2 months prior to IFN-alpha 2a. 3TC was continued for 6 months after antiHBe seroconversion or stopped at 24 months in non-responders. Results: Baseline alanine aminotransferase (ALT) was 134.2 +/- 34.1 and 147.0 +/- 45.3; histological activity index (HAI) was 7.4 +/- 2.7 and 7.1 +/- 2.3; and HBV DNA levels were above 2,000 pg/ml in 76% and 66% of patients in groups I and 11, respectively (P > 0.005). Complete response was 55.3% and 27.6% in groups I and 11, respectively (P < 0.01). AntiHBe seroconversion was higher and earlier, and HBV DNA clearance was earlier in group I (P < 0.05). HBsAg clearance was 12.5% and 4.6% and antiHBs seroconversion was 9.8% and 6.2% in groups I and 11, respectively (P > 0.05). Breakthrough occurred in 17.9% and 24.6%; breakthrough times were 15.9 +/- 4.6 and 14.1 +/- 5.1 months; and relapse rates were 6.8% and none in groups I and 11, respectively (P > 0.05, P > 0.05, P > 0.05). Responders had higher HAI (HAI > 6) and higher pre-treatment ALT than non-responders. Conclusion: Simultaneous 3TC+IFN-alpha 2a yields a higher response and earlier antiHBe seroconversion and viral clearance than consecutive combined therapy. Relapse rate is low. Predictors of response are high basal ALT and high HAI scores. 3TC can be administered for 24 months without any side effect and breakthrough rate is comparable with previous studies.
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    Current therapeutic approaches in childhood chronic hepatitis B infection: A multicenter study
    Dikici, B; Ozgenc, F; Kalayci, AG; Targan, S; Ozkan, T; Selimoglu, A; Doganci, T; Kansu, A; Tosun, S; Arslan, N; Kasirga, E; Bosnak, M; Haspolat, K; Buyukgebiz, B; Aydogdu, S; Girgin, N; Yagci, RV
    Background and Aim: The aim of the present study was to compare the therapeutic efficacy of three different regimens in childhood chronic hepatitis B (CHB) infection. Methods: A total of 182 children with CHB infection were prospectively allocated to three random groups. Sixty-two patients in the first group received high-dose interferon (IFN)-alpha 2b (10 MU/m(2)) thrice/weekly alone for 6 months. In the second (n = 60) and third groups (n = 60), IFN-alpha was used for 6 months (5 MU/m(2)) thrice/weekly in combination with lamivudine (LAM) (4 mg/kg, maximum 100 mg/day) for 12 months. Lamivudine was started simultaneously with IFN in the second group, while it was started 2 months prior to IFN injections in the third group. Results: The initial mean alanine aminotransferase (ALT) values for the first, second and third groups were 109 +/- 93 IU/L, 101 +/- 64 IU/L and 92 +/- 42 IU/L, respectively (P > 0.05). At the end of the therapy, ALT values decreased to 82 +/- 111 IU/L, 38 +/- 41 IU/L and 29 +/- 16 IU/L in groups 1, 2 and 3, respectively. The mean ALT value of the first group was significantly different to the second and third groups (P = 0.046 and P = 0.002, respectively) at the end of the therapy and these differences were found to be sustained after 18 months. However, results in the second and third groups were similar (P > 0.05). There were no significant differences in HBeAg clearance and anti-HBe seroconversion at the initial stage, 12 months and 18 months between the three groups (P > 0.05). Hepatitis B virus (HBV) DNA clearance in the first group was different from the second and third groups, while the second and third groups had similar HBV DNA clearance ratios at 12 and 18 months. No significant difference was found in the complete response (normalization of ALT, clearance of HBV DNA and seroconversion of anti HBe) ratios of all groups (at 12 months: 28.8, 45.5, 35.8% and at 18 months 33.3, 49 and 34% in groups 1, 2 and 3, respectively, P > 0.05). Conclusions: Although the ALT normalization and HBV DNA clearance ratios of IFN plus LAM combination groups were better than the high-dose IFN-alpha monotherapy group, no significant difference was found in the complete response ratios of all three groups. (C) 2004 Blackwell Publishing Asia Pty Ltd.
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    Familial Mediterranean Fever Mutation Analysis in Pediatric Patients With Inflammatory Bowel Disease: A Multicenter Study
    Urganci, N; Ozgenc, F; Kuloglu, Z; Yüksekkaya, H; Sari, S; Erkan, T; Önal, Z; Çaltepe, G; Akçam, M; Arslan, D; Arslan, N; Artan, R; Aydogan, A; Balamtekin, N; Baran, M; Baysoy, G; Çakir, M; Dalgiç, B; Dogan, Y; Durmaz, Ö; Ecevit, Ç; Eren, M; Gökçe, S; Gülerman, F; Gürakan, F; Hizli, S; Isik, I; Kalayci, AG; Kansu, A; Kutlu, T; Karabiber, H; Kasirga, E; Kutluk, G; Hosnut, FÖ; Özen, H; Özkan, T; Öztürk, Y; Soylu, ÖB; Tutar, E; Tümgör, G; Ünal, F; Ugras, M; Ustundag, G; Yaman, A
    Background: the aim of the study was to evaluate familial Mediterranean fever (FMF) mutation analysis in pediatric patients with inflammatory bowel disease (IBD). The relation between MEFV mutations and chronic inflammatory diseases hos been reported previously. Methods: Children with IBD (334 ulcerative colitis (UC), 224 Crohn's disease (CD), 39 indeterminate colitis (IC)) were tested for FMF mutations in this multicenter study. The distribution of mutations according to disease type, histopathological findings, and disease activity indexes was determined. Results: A total of 597 children (mean age: 10.8 +/- 4.6 years, M/F: 1.05) with IBD were included in the study. In this study, 41.9% of the patients had FMF mutations. E148Q was the most common mutation in UC and CD, and M694V in IC (30.5%, 34.5%, 47.1%, respectively). There was a significant difference in terms of endoscopic and histopathological findings according to mutation types (homozygous/heterozygous) in patients with UC (P <.05). There was a statistically significant difference between colonoscopy findings in patients with or without mutations (P=.031, P=.045, respectively). The patients with UC who had mutations had lower Pediatric Ulcerative Colitis Activity Index (PUCAI) scores than the patients without mutations (P=.007). Conclusion: Although FMF mutations are unrelated to CD patients, but observed in UC patients with low PUCAI scores, it was established that mutations do not hove a high impact on inflammatory response and clinical outcome of the disease.