Kaan Demiroren

Sydenham's chorea: a clinical follow-up of 65 patients.

Sydenhams 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 Sydenhams 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 1 to 6 months in 51.7% of the patients. The recurrence rate was 37.9%. In conclusion, Sydenhams chorea is still an important health problem in Turkey with respect to its morbidity.

Protective effects of L-carnitine, N-acetylcysteine and genistein in an experimental model of liver fibrosis

AIM Liver fibrosis is a reversible wound-healing response that occurs following liver injury. In this study, we aimed to investigate the possible protective effects of L-carnitine, N-acetylcysteine and genistein in liver fibrosis induced by carbon tetrachloride (CCl4). In addition, the effects of these agents were compared in the same study. METHODS In this study, rats were randomly allocated into 8 groups, consisting of 10 rats each, as follows: a control group, CCl4, L-carnitine, N-acetylcysteine, genistein, CCl4 and L-carnitine, CCl4 and N-acetylcysteine, and CCl4 and genistein. At the end of 6 weeks, blood and liver tissue specimens were collected. Alanine aminotransferase (ALT); aspartate aminotransferase (AST); complete blood count, tumor necrosis factor-α (TNF-α); platelet-derived growth factor-BB (PDGF-BB); interleukin-6 (IL-6); liver glutathione level; oxidant/antioxidant status; scores of hepatic steatosis, necrosis, inflammation, and fibrosis; and the expression of α-smooth muscle actin were studied. RESULTS Although the ALT and AST values in the group administered CCl4 were significantly higher than in all the other groups (P<0.05), there was no significant difference between the control group and the groups administered CCl4 combined with L-carnitine, N-acetylcysteine and genistein (P>0.05). There were significant differences in the levels of TNF-α, PDGF-BB and IL-6 (P<0.05) between the CCl4 group and the groups with L-carnitine, N-acetylcysteine and genistein added to CCl4. N-acetylcysteine and genistein had positive effects on the oxidant/antioxidant status and on liver necrosis and fibrosis scores. CONCLUSIONS In our study, L-carnitine, N-acetylcysteine and genistein showed significant protective effects in liver fibrosis induced by CCl4.

Prevalence of Helicobacter pylori virulence genotypes among children in Eastern Turkey

AIM To identify the virulence genotypes of Helicobacter pylori (H. pylori) if present in children in Eastern Turkey and if those genotypes are mostly associated with severe clinical presentations. METHODS A total of 49 H. pylori positive Turkish children (42 with antral nodularity and 7 with peptic ulcer) who underwent upper gastrointestinal endoscopy with abdominal symptoms during the period from March 2011 to September 2012 were enrolled in this study. Antral nodularity was diagnosed endoscopically by two of the authors. We determined for the presence of cagA, vacA, cagE, iceA and babA2 genotypes of H. pylori isolates in DNA obtained directly from frozen gastric biopsy samples by polymerase chain reaction test using specific primers. RESULTS Of the 49 H. pylori isolates studied, 61.2%, 91.8%, 22.4%, 28.6%, 57.1% and 40.8% were positive for the cagA, vacA s1, cagE, iceA1, iceA2 and babA2 genes, respectively. We showed that the most common vacA subtype was s1a (79.6%). However, the s2 gene was found less frequently with an isolation rate of 8.2% of the H. pylori isolates. The genotypes iceA2 and vacA s1m2 were the most frequently found types in children with antral nodularity. In addition, the genotypes iceA1, babA2 and vacA s1m1 were found in similar ratios in all the H. pylori isolates obtained from children with peptic ulcer. The genotypes vacA s2m1 and s1c were not observed in any of isolates studied. CONCLUSION This study showed that vacA s1m2, cagA and iceA2 were the most common genotypes, and no association between antral nodularity and genotypes was observed.

Prevalence of Helicobacter pylori in children in eastern Turkey and molecular typing of isolates.

The objectives of the present study were to determine Helicobacter pylori via culture, polymerase chain reaction and histopathological diagnosis in 101 children ranging in age from 4 to 18 years, to identify the association among restriction fragment length polymorphism types and clinical disease and to investigate the relationships among different isolates of H. pylori in different age groups. We observed a high prevalence of H. pylori infections in children between the ages of 13 and 18 (75.8%), while children aged 4 to 6 years had the lowest prevalence of infection (40%). H. pylori was detected in 30.7% (31 of 101), 66.3% (67 of 101) and 63.2% (60 of 95) of children as determined by culture methods, PCR and histological examination, respectively. H. pylori isolates with RFLP types I and III were the most common among children with antral nodularity, whereas RFLP types II and IV were the least detected types. Interestingly, all isolates from peptic ulcer patients were type III. Although our results show a high prevalence of H. pylori infections in the pediatric population in eastern Turkey, no association was identified between H. pylori infection with antral nodularity and recurring abdominal pain. In addition, we found low genetic variation among H. pylori isolates from children and no association between RFLP types and antral nodularity (p > 0.05). Additionally, we found that H. pylori isolates with specific RFLP types were predominant in different age groups.

The importance of the serum quantitative levels of hepatitis B surface antigen and hepatitis B e antigen in children with chronic hepatitis B

BACKGROUND/AIMS We aimed to investigate the clinical importance of quantitative levels of serum hepatitis B surface antigen (HBsAg) and hepatitis B e antigen (HBeAg), and to detect their correlation with hepatitis B virus (HBV) DNA load, alanine aminotransferase (ALT) levels, hepatic activity index (HAI) and fibrosis scores. MATERIALS AND METHODS A total of 56 HBeAg-positive children with chronic hepatitis B (CHB) were included in the study. Quantification of HBsAg and HBeAg was performed using an automated chemiluminescent microparticle immunoassay. Comparisons were performed using the paired t-test, Mann-Whitney U test or t-test for independent samples. Correlations were tested using the Pearson correlation analysis. RESULTS Significant differences were found between groups of pre- and post treatment quantitative levels of HBsAg, HBeAg, HBV DNA, and ALT. Comparison of HBsAg, HBeAg, HBV DNA, and ALT levels before the treatment and decrease ratios of these levels after treatment according to HAI and fibrosis scores did not show any statistically significant differences. There was a positive correlation between pretreatment HBV DNA load and HBeAg levels, and a negative correlation between pretreatment HBV DNA and ALT levels. There was a negative correlation between decrease ratios of HBsAg and ALT levels after treatment. Patients with post treatment HBeAg seroconversion had a lower post treatment HBV DNA load and a higher decrease ratio of HBsAg than patients who did not have HBeAg seroconversion. CONCLUSION The present study indicated that HBsAg and HBeAg levels significantly decreased during treatment and that HBeAg correlated with HBV DNA load. Quantitative HBeAg and HBsAg assays could therefore have an important role in treatment of CHB.

Prevalence of Celiac Disease in Children With Iron Deficiency Anemia in Van Lake Region of Turkey.

We aimed to determine the prevalence of celiac disease (CD) in children with iron deficiency anemia (IDA) and compare the hematological variables of these children with those with IDA, but not with CD. A total of 250 patients aged 1 to 16 years with IDA were included. Upper gastrointestinal system endoscopy was recommended to the patients who were serologically positive for CD. According to the endoscopic biopsy results of 12 patients who have positive screening tests (57.1%), Marsh grade II histopathologic findings were detected in 1 (4.8%) and Marsh grade III histopathologic findings were detected in 11 (52%). The hemoglobin, hematocrit, iron, and ferritin levels were significantly lower in patients with CD with IDA, compared with those with IDA, but not with CD. According to Marsh classification, the percentage of Marsh grade IIIc was found to be higher than the other Marsh grades. As the Marsh grading increased, hemoglobin, hematocrit, serum iron, and ferritin levels decreased and tissue transglutaminase Ig A levels increased. Our results show that CD should be kept in mind as the underlying etiologic factor in patients with IDA.

Assessment of Cardiac Functions in Infants with Cow’s Milk Allergy

Background Cow’s milk allergy is the most common food allergy in children, with rates estimated at 1.9% to 4.9%. Clinical phenotypes of cow’s milk allergy are varied and involve 1 or more target organs, with the main targets being the skin, respiratory system, and gastrointestinal tract. To date, no studies have investigated detailed cardiac function in children with cow’s milk allergy. The current study aimed to investigate cardiac function in infants with cow’s milk allergy. Material/Methods We studied 42 infants with cow’s milk allergy and 30 age- and sex-matched healthy subjects. Cardiac functions were evaluated by M-mode, pulsed-wave, and tissue Doppler echocardiography. Results There were no significant differences in ejection fraction or mitral and tricuspid annular plane systolic excursion between the 2 groups. Pulsed-wave Doppler-derived E/A ratios in mitral and tricuspid valves were similar in both groups. Ea/Aa ratios in the left ventricle posterior wall and right ventricle free wall were lower in patients with cow’s milk allergy than in the control group. The E/Ea ratio in the left ventricle, isovolumic relaxation time, deceleration time, and right and left ventricular myocardial performance indices were higher in patients in the study group. Conclusions Our study identified reduced early diastolic tissue Doppler velocities in infants with cow’s milk allergy.

Is Kawasaki Disease a Side Effect of Vaccination as Well

Different subtypes of vasculitis have been reported after vaccination in adults. However, only one case of Kawasaki disease in an infant after hepatitis B vaccination has been described. We report a case of Kawasaki disease in a 2 month-old infant who developed the Kawasaki disease 1 day after receiving his first dose of diphtheria tetanus-acellular pertussis, Haemophilus b and pneumococcal conjugate vaccine.

A comparison of the effects of thymoquinone, silymarin and N-acetylcysteine in an experimental hepatotoxicity

This study investigated the effects of thymoquinone, silymarin, and N-acetylcysteine in a rat model with carbon tetrachloride (CCl4)-induced hepatotoxicity. Although numerous similar studies are available, we aimed to compare the efficacy of these agents by considering N-acetylcysteine as a reference compound. A total of 50 male Wistar albino rats were randomly designated as 5 groups: Group I, CCl4; group II, thymoquinone and CCl4; group III, silymarin and CCl4; group IV, N-acetylcysteine and CCl4; group V, control group. CCl4 was administered intraperitoneally at a dose of 1.5 mL/kg (a mixture of CCl4: olive oil, 1:2) twice a week. Thymoquinone was administered at a dose of 10 mg/kg, silymarin was administered at a dose of 100 mg/kg, and N-acetylcysteine was administered at a dose of 100 mg/kg by daily intraperitoneal injection. At the end of four weeks, blood and liver tests were analyzed. The results were evaluated statistically via the one-way ANOVA test. A p-value <0.05 was considered statistically significant. Thymoquinone, silymarin, and N-acetylcysteine improved the levels of alanine aminotransferase, tumor necrosis factor-α, platelet-derived growth factor-BB, and interleukin-6, which were increased by CCl4. Thymoquinone and silymarin showed the positive increase in liver glutathione levels. Thymoquinone, silymarin, and N-acetylcysteine improved blood total oxidant status. In the histological examinations of liver tissue, thymoquinone decreased necrosis, and inflammation. The most positive decrease in the α-smooth muscle actin-stained hepatic stellate cell count was only observed with thymoquinone. These findings suggest that thymoquinone, silymarin, and N-acetylcysteine have potential for the treatment of diseases causing liver injury. Among these agents, thymoquinone showed the best results on most of the parameters. Since TQ appears to be at least as effective as SM and NAC in our in-vitro study, we propose that it is time for clinical studies with thymoquinone on hepatotoxicity.

MORTAL BIR EPIDERMOLIZIS BÜLLOZA OLGUSU

Epidermolizis bulloza (EB), travmayi takiben olusan bullerle karakterize, kalitsal gecisli bir hastaliktir. EB’nin farkli tiplerinin olusumunda cildin bazal membran tabakasini olusturan proteinleri kodlayan genlerde cesitli mutasyonlar rol oynar. Doku ayrismasinin duzeyine gore, EB simpleks (EBS), “junctional” (JEB), distrofik (DEB) ve Kindler tip olmak uzere 4 tipe ayrilir. Nadir gorulen JEB tipi yenidogan doneminde yuksek mortaliteye sahiptir. 28 yasindaki saglikli annenin 2 gebeliginden, miadinda erkek bebek olarak spontan vajinal yol ile dogan olgumuza cilt biyopsisi ve klinik bulgular isiginda JEB tanisi kondu. Olgumuzun fizik muayenesinde, extremite, tirnak yapilarinin hipoplastik ya da dismorfik oldugu, ayak bileklerinden baslayan ve tum ayak ve ayak parmaklarini icine alan atrofik yapi, tum vucutta ve agiz mukozasinda yaygin bulloz lezyonlar gozlenmistir. Tedavide kismi duzelme gozlenmis olmasina ragmen, klinik tablosunda ani bozulma ve kardiyak arrest sonucu hasta kaybedildi. Epidermolizis bullozanin “junctional” tipinin prognozunun oldukca mortal oldugunu ve araya enfeksiyon girmeksizin ani kayiplarin olabilecegi akilda tutulmalidir.