Nurten Girgin

Current therapeutic approaches in childhood chronic hepatitis B infection: A multicenter study

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.

The value of the levels of acute phase reactants for the prediction of familial Mediterranean fever associated amyloidosis: a case control study

In order to determine the role of levels of acute phase proteins (APPs) for the development of amyloidosis in familial Mediterranean fever (FMF) patients, the levels of serum amyloid A (SAA), C reactive protein (CRP), fibrinogen and erythrocyte sedimentation rate were measured in paired sera of 36 FMF patients during and in between acute attacks, 39 of their healthy parents (obligate heterozgotes), and 15 patients with FMF associated amyloidosis. To compare the levels of APPs, 39 patients with chronic infections or inflammatory diseases who may develop secondary amyloidosis, 20 patients with acute infections who are known to have elevated acute phase response but will never develop amyloidosis and 19 healthy controls were included. The median levels of all APPs are increased in the patients with FMF during attacks and a significant decrease was observed after the attack was over. The level of SAA was above reference range in all FMF patients during the attack free period and the level of at least one other APP was also above normal in 64% of the patients. Both CRP and SAA levels were found to be higher in obligate heterozygotes compared to controls. The levels of SAA in patients with FMF during the attack-free period, obligate heterozygotes and patients with FMF-amyloidosis were found to be similar. The levels in each group were found to be higher than SAA levels found in healthy controls yet lower than the levels measured in the patients with acute infections and patients with chronic inflammation or chronic infections. In conclusion, our results show that SAA level reflects subclinical inflammation with high sensitivity but its value for the prediction of amyloid formation process seems to be low.

Celiac disease: presentation of 109 children.

Purpose The clinical features of patients with celiac disease (CD) are variable. In the present study, clinical and laboratory features of 109 patients with CD were retrospectively evaluated. Materials and Methods In all cases, diagnosis of CD was made by European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) criteria and clinical and laboratory findings, including hematological and biochemical analyses, immunoglobulin levels, autoantibodies [antinucler antibody (ANA), antidouble stranded DNA (dsDNA), antimitochondrial antibody (AMA), anti-smooth muscle antibody (ASMA), liver kidney antibody (LKM-1), anti thyroid peroxidase (TPO), anti thyroglobulin (Tg)], bone mineral density (BMD), and electroencephalogram were evaluated. The type of CD was recorded. Results Of 109 patients with CD, 66 (60.6%) were classical type, 41 (37.6%) were atypical type and 2 (1.8%) were silent type. The mean age was 8.81 ± 4.63 years and the most common symptom was diarrhea (53.2%) followed by failure to thrive, short stature, and abdominal pain. Paleness (40.4%), underweight (34.8%), and short stature (31.2%) were the most common findings. Iron deficinecy anemia (81.6%), zinc deficiency (64.1%), prolonged prothrombin time (35.8%), and elevated transaminase levels (24.7%) were the most common laboratory findings. Eight percent of patients had at least 1 autoantibody, and 28 of 52 patients had low BMD. Four of 38 patients had abnormalty in electroencephalograms. The prevalance of selective immunoglobulin (Ig) A deficiency was 9.1%. Histocompatibility antigen HLA-DQ and/or DQ8 genotypes were found in 91% of patients. Abdominal distention, iron deficiency, prolonged prothrombine time, hypoalbuminemia, and elevated transaminase levels were more significantly frequent in the classical type than atypical type (p < 0.005). Conclusion Although classical CD was seen in most patients in the present study, clinical variability of the condition should be kept in mind.

Celiac disease in Turkish short‐statured children and the value of antigliadin antibody in diagnosis

Abstract Background. It is generally accepted that celiac disease (CD) must always be taken into consideration when dealing with children manifesting growth failure. It is, therefore, important to have laboratory tests capable of detecting patients who should undergo intestinal biopsy. In this study, we have prospectively evaluated clinical characteristics, gliadin antibody measurements and duodenal biopsies in 47 children with short stature and without gastrointestinal symptoms, in order to determine the incidence of CD and the diagnostic value of immunoglobulin (Ig)A and IgG antigliadin antibodies (AGA) for CD.

A rapid lateral flow stool antigen immunoassay and 14C-urea breath test for the diagnosis and eradication of Helicobacter pylori infection in children

Our aim was to evaluate diagnostic accuracy of rapid immunochromatographic stool antigen test (Rapid HpSA; LINEAR Chemical, Barcelona, Spain) and a practical low-dose (14)C urea breath test (UBT) (Heliprobetrade mark) test before and after eradication therapy. One hundred nine children with abdominal symptoms (age range, 5-17 years; mean, 12.1) underwent endoscopy, (14)C-UBT, and Rapid HpSA. Patients were defined as Hp infected when histology was positive for Hp. Forty children (36.6%) were Hp infected. The sensitivity of Rapid HpSA and (14)C-UBT was 65% and 92.5% (P = 0.0003), respectively; the specificity of Rapid HpSA and (14)C-UBT was 92.3% and 85.5% (P = 0.180), respectively. After eradication therapy endoscopy, (14)C-UBT and Rapid HpSA were repeated. The eradication rate was 70.5%. After eradication, the sensitivity of Rapid HpSA and (14)C-UBT was 60% and 100%, respectively; the specificity of Rapid HpSA and (14)C-UBT was 100%. (14)C-UBT was more reliable than the Rapid HpSA test for the diagnosis and for confirming eradication of Hp infection.

A very rare cause of intestinal pseudoobstruction: familial visceral myopathy type IV

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HEPATIC DAMAGE IN GLUTEN SENSITIVE ENTEROPATHY

Background: Gluten sensitive enteropathy has been reported to occur concomitantly with liver abnormalities, such as primary biliary cirrhosis, chronic active hepatitis and primary sclerosing cholangitis.

Hypertrophic osteoarthropathy in a child with biliary atresia.

Hypertrophic osteoarthropathy is a syndrome characterized by clubbing of the digits of the hand/foot, periosteal reaction and arthralgia or arthritis which is usually secondary to cyanotic congenital heart disease and chronic pulmonary infections. This syndrome rarely occurs in association with chronic liver disease in childhood. Here, we report on a child with biliary atresia who developed arthralgia and arthritis during follow‐up and which was diagnosed as hepatic hypertrophic osteoarthropathy. It is emphasized that hypertrophic osteoarthropathy should be considered in the differential diagnosis of arthralgia and arthritis in children with long‐standing chronic liver diseases, especially if finger clubbing is also present.

Effects of cisapride on ventricular repolarization in children.

Life-threatening ventricular dysrhythmias mainly attributed to QTc prolongation have been reported in adults and children who were using cisapride, a prokinetic agent that facilitates gastrointestinal motility. Recent adult and paediatric case reports have suggested an association of malignant ventricular dysrhythmias with administration of cisapride in conjunction with drugs that inhibit its cytochrome P-450 metabolism. Therefore, to analyse the time- and dose-related effects of cisapride on ventricular repolarization, we prospectively studied infants and children receiving cisapride with no concomitant medications. Standard 12-lead resting ECGs were obtained from 38 patients (mean age: 6.6 +/- 4.4 y) before the first dose of cisapride (0.8-1.2 mg/kg/d) therapy, and 3 d, 7 d and 1 mo after the first dose of continuing cisapride therapy. The corrected QT interval (QTc), dispersion of QT and QTc (QTD, QTcD) were calculated. Patients were divided into two groups according to dose of cisapride: Group 1 (n = 22) (0.8 mg/kg/d), Group 2 (n = 16) (1.2 mg/ kg/d). Data obtained from these patients were compared with a control group consisting of 372 normal children. No clinical adverse effects such as palpitations, presyncope or syncope were noted during the study. Baseline QTc, QTD and QTcD measurements of the study group were not different from those of the control group. Mean QTc values of the study group on days 7 and 30 of cisapride therapy were found to be significantly higher than those of the control group (p < 0.001 and <0.0001, respectively). Mean QTc values of the study group on days 7 and 30 of therapy were also significantly higher than those of baseline value (p < 0.01 and <0.001, respectively). Mean QTD and mean QTcD values that were recorded throughout the cisapride treatment in the study group were not found to be different from the baseline values and the values of the controls. Mean QTD and QTcD were also not found to be different between Groups 1 and 2. However, mean QTc was found to be more significantly increased from baseline at the first month of therapy in Group 2 (p < 0.05). The results of this study suggest that cisapride treatment cause prolongation of ventricular repolarization without causing increased heterogeneity of repolarization (QT dispersion). However, the clinical significance of this effect is unclear, because all the patients in this study group remained asymptomatic, without signs of dysrhythmia.

Liver Histology of Children With Chronic Hepatitis Treated With Interferon-α Alone or in Combination With Lamivudine

Aim: To evaluate histological changes with interferon monotherapy or interferon plus lamivudine combination therapy in children with hepatitis B e antigen (HBeAg)–positive chronic hepatitis B. Patients and Methods: 31 children aged 2–13 years were randomly treated with interferon (IFN) (group 1, n = 16) or IFN plus simultaneously started lamivudine (group 2, n = 15). IFN-α2a was given 9 MU/m2 3 times per week for 6 months in each group; lamivudine was given 4 mg · kg−1 · day−1 for 24 months. Liver biopsy specimens were evaluated according to the Knodell score before therapy and after 24 months of therapy. Histological response was defined as a decrease in the histological activity index (HAI) score by at least 2 points. Efficacy of therapy was evaluated at 24 months of therapy in all children. Results: Alanine aminotransferase normalization, HbeAg, and hepatitis B virus DNA clearance were not different. Complete response and histological response were 37.5%/62.5% and 40%/46.7% in groups 1 and 2, respectively (P = NS). At baseline and at 24 months of therapy, total HAI and components of HAI were not different in the 2 groups. In comparison with baseline, a significant decrease in scores of periportal ± bridging necrosis was observed in group 1 (P = 0.01); periportal ± bridging necrosis, intralobular degeneration, focal necrosis, and necroinflammation scores significantly decreased in group 2 (P = 0.04 and P = 0.02) at 24 months of therapy. Conclusions: The addition of lamivudine to IFN-α did not increase the effectiveness of the treatment in terms of complete and histological responses. Both therapies seemed to be effective in the regression of periportal ± bridging necrosis. In addition, combination therapy was also effective in the regression of intralobular degeneration, focal necrosis, and necroinflammatory activity index.