THE INFLUENCE OF HLA-DQ2 HETERODIMERS ON THE CLINICAL FEATURES AND LABORATORY OF PATIENTS WITH CELIAC DISEASE.

BACKGROUND AND AIM: the essential genetic marker related with celiac disease (CD) is the HLA-DQ2 molecule encoded by the DQA1*0501 and DQB1*0201 genes. The aim of this study is to evaluate effect of these alleles on the clinical, serological and histological features of Turkish children with celiac disease. MATERIAL AND METHODS: we divided 36 celiac patients to 4 groups according to their HLA-DQ2 genotype based on the presence or absence of DQA1*0501 and DQB1*0201 alleles. Group 1: 4 patients had no HLA-DQ2A1*0501 and DQ2B1*0201 alleles, Group 2: 12 patients had at least one of these alleles with heterozygous status, Group 3: 12 patients had both alleles with heterozygous status, Group 4: 8 patients had both alleles with homozygous status. We compared groups according to the clinical, serological, histological, and biochemical features. RESULTS: there was no statistical significance among the groups for age, body mass index (BMI), weight for height, and onset of symptoms. However, both in groups 3 and 4 compared with groups 1 and 2, minor differences were observed for BMI and anti-gliadin antibody (AGA) without statistical significance. According to the anti-endomysial antibody (EMA), Marsh scores, clinical presentations, hematological and biochemical values, there was no statistical significance among groups without constipation that observed higher rate in the 4th group without statistically significance. Hypothyroidism was detected in one patient (25%) in the lowest genetic load group (Group 1) with statistically significance (p < 0.046). CONCLUSION: in this study, small differences found among groups were not elucidated the impact of HLADQ2 A1*0501 and DQ2B1*0201 alleles on the clinical, serological and laboratory manifestations of celiac patients. Further studies are needed to assess the effect of reported HLA alleles and other genetic polymorphisms on CD outcomes in children.

Antecedentes y objetivo: el marcador genético esencial relacionado con la enfermedad celíaca (CD) es la molécula HLA-DQ2 codificada por los genes DQA1*0501 y DQB1*0201. El objetivo de este estudio es evaluar el efecto de estos alelos en las características clínicas, serológicas e histológicas de los niños turcos que tienen la enfermedad celíaca. Material y métodos: nosotros hemos dividido los 36 pacientes celíacos en 4 grupos de acuerdo con su genotipo HLA-DQ2 basado en la presencia o la ausencia de los alelos DQA1*0501 y DQB1*0201. Grupo 1: 4 pacientes que no tenían los alelos HLA-DQ2A1*0501 y DQ2B1*0201; Grupo 2: 12 pacientes que tenían por lo menos uno de estos alelos con un estado heterocigoto; Grupo 3: 12 pacientes que tenián ambos alelos con un estado heterocigoto; Grupo 4: 8 pacientes que tenían ambos alelos con un estado heterocigoto. Nosotros hemos comparado los grupos de acuerdo con las características clínicas, serológicas, histológicas y bioquímicas. Resultados: no había significación estadística entre los grupos por edad, índice de masa corporal, (IMC), peso por altura y aparición de síntomas. Sin embargo, en los grupos 3 y 4 comparados con los grupos 1 y 2 se observaron unas diferencias menores en IMC y anticuerpos antigliadina (AGA), sin una significación estadística. De acuerdo con los antiendomisios (EMA), la puntuación Marsh, las presentaciones clínicas y los valores hematológicos y bioquímicos, no había una significación estadística entre los grupos sin estreñimiento respecto a los valores más altos observados en el grupo 4 sin significación estadística. Se detectó hipertiroidismo en un paciente (25%) del grupo de carga genética más baja (grupo 1) con significación estadística (p < 0,046). El resultado: en este estudio, las pequeñas diferencias que se encontraton entre los grupos no dilucidaron el impacto de los alelos HLA-DQ2 A1*0501 y DQ2B1*0201 en las manifectaciones clínicas, serológicas y de laboratorio de los pacientes celíacos. Se necesitan nuevos estudios para evaluar el efecto de los alelos HLA y otros polimorfismos genéticos en los resultados sobre la enfermedad celíaca en los niños.

The influence of HLA-DQ2 heterodimers on the clinical features and laboratory of patients with celiac disease / La influencia de los heterodímeros HLA-DQ2 en las características clínicas y de laboratorio de los pacientes con enfermedad celíaca

Background and aim: the essential genetic marker related with celiac disease (CD) is the HLA-DQ2 molecule encoded by the DQA1*0501 and DQB1*0201 genes. The aim of this study is to evaluate effect of these alleles on the clinical, serological and histological features of Turkish children with celiac disease. Material and methods: we divided 36 celiac patients to 4 groups according to their HLA-DQ2 genotype based on the presence or absence of DQA1*0501 and DQB1*0201 alleles. Group 1: 4 patients had no HLA-DQ2A1*0501 and DQ2B1*0201 alleles, Group 2: 12 patients had at least one of these alleles with heterozygous status, Group 3: 12 patients had both alleles with heterozygous status, Group 4: 8 patients had both alleles with homozygous status. We compared groups according to the clinical, serological, histological, and biochemical features. Results: there was no statistical significance among the groups for age, body mass index (BMI), weight for height, and onset of symptoms. However, both in groups 3 and 4 compared with groups 1 and 2, minor differences were observed for BMI and anti-gliadin antibody (AGA) without statistical significance. According to the anti-endomysial antibody (EMA), Marsh scores, clinical presentations, hematological and biochemical values, there was no statistical significance among groups without constipation that observed higher rate in the 4th group without statistically significance. Hypothyroidism was detected in one patient (25%) in the lowest genetic load group (Group 1) with statistically significance (p<0.046). Conclusion: in this study, small differences found among groups were not elucidated the impact of HLADQ2 A1*0501 and DQ2B1*0201 alleles on the clinical, serological and laboratory manifestations of celiac patients. Further studies are needed to assess the effect of reported HLA alleles and other genetic polymorphisms on CD outcomes in children (AU)

Antecedentes y objetivo: el marcador genético esencial relacionado con la enfermedad celíaca (CD) es la molé- cula HLA-DQ2 codificada por los genes DQA1*0501 y DQB1*0201. El objetivo de este estudio es evaluar el efecto de estos alelos en las características clínicas, serológicas e histológicas de los niños turcos que tienen la enfermedad celíaca. Material y métodos: nosotros hemos dividido los 36 pacientes celíacos en 4 grupos de acuerdo con su genotipo HLA-DQ2 basado en la presencia o la ausencia de los alelos DQA1*0501 y DQB1*0201. Grupo 1: 4 pacientes que no tenían los alelos HLA-DQ2A1*0501 y DQ2B1*0201; Grupo 2: 12 pacientes que tenían por lo menos uno de estos alelos con un estado heterocigoto; Grupo 3: 12 pacientes que tenián ambos alelos con un estado heterocigoto; Grupo 4: 8 pacientes que tenían ambos alelos con un estado heterocigoto. Nosotros hemos comparado los grupos de acuerdo con las características clínicas, serológicas, histológicas y bioquímicas. Resultados: no había significación estadística entre los grupos por edad, índice de masa corporal, (IMC), peso por altura y aparición de síntomas. Sin embargo, en los grupos 3 y 4 comparados con los grupos 1 y 2 se observaron unas diferencias menores en IMC y anticuerpos antigliadina (AGA), sin una significación estadística. De acuerdo con los antiendomisios (EMA), la puntuación Marsh, las presentaciones clínicas y los valores hematológicos y bioquímicos, no había una significación estadística entre los grupos sin estreñimiento respecto a los valores más altos observados en el grupo 4 sin significación estadística. Se detectó hipertiroidismo en un paciente (25%) del grupo de carga genética más baja (grupo 1) con significación estadística (p<0,046). El resultado: en este estudio, las pequeñas diferencias que se encontraton entre los grupos no dilucidaron el impacto de los alelos HLA-DQ2 A1*0501 y DQ2B1*0201 en las manifectaciones clínicas, serológicas y de laboratorio de los pacientes celíacos. Se necesitan nuevos estudios para evaluar el efecto de los alelos HLA y otros polimorfismos genéticos en los resultados sobre la enfermedad celíaca en los niños (AU)

Investigation of cardiomyopathy in children with cirrhotic and noncirrhotic portal hypertension.

BACKGROUND: Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiological disturbances, and diastolic and/or systolic dysfunction in patients with liver disease. This disorder is a well-defined entity in adults, but pediatric data are limited. The aim of the study was to determine the incidence, features, and risk factors of CCMP in children with portal hypertension (PHT). METHODS: This study included 50 children with cirrhotic PHT (40/50) and noncirrhotic PHT (10/50). Fifty healthy children were also selected for the control group. Electrocardiography and echocardiography were used to evaluate cardiac functions. Corrected QT (QTc) ≥ 0.45 was accepted as prolonged on electrocardiography. The study group was divided into 3 groups: cirrhotic, noncirrhotic, and control. Then, the CCMP group was created according to the diagnostic criteria. Latent CCMP was diagnosed in the presence of prolonged-QTc along with a minor criterion (tachycardia). Manifest CCMP was diagnosed in the presence of at least 2 major criteria (prolonged-QTc along with abnormal echocardiographic findings). Moreover, in this study, the risk factors for CCMP were investigated. RESULTS: The CCMP group included 10 cases (20%). Nine of these cases had latent CCMP (18%), and the remaining one (2%) had manifest CCMP. All of the cases with CCMP had cirrhosis and ascites. None of the patients with CCMP had severe cardiac symptoms, but they were already using some cardioprotective drugs such as propanolol and spironolactone. As risk factors for CCMP, pediatric end-stage liver disease scores, Child-Pugh scores, and ascites grades were found to be significant for the determination of CCMP. The most important risk factor was ascites severity (P = 0.001, odds ratio 9.4). CONCLUSIONS: Approximately 20% of children with PHT have CCMP. A detailed cardiac examination should be carried out periodically in children with cirrhotic PHT, especially in the presence of ascites and high Child-Pugh score.

Serum and hair zinc levels of infants and their mothers.

OBJECTIVES: This cross-sectional study aimed to investigate the zinc status of mothers and their infants attending a well-child clinic. METHODS: Blood and hair samples were collected from infants and their mothers at 2nd, 6th,12th month after delivery. Information on infant and their mothers' dietary habits was gathered. RESULTS: Of all infants and their mothers, 54.6% and 12.6% had low hair zinc levels; 17% and 4.6% low serum zinc levels respectively. There was a positive relationship between mother's hair zinc level and her meat consumption at 2 and 6 months after delivery. CONCLUSION: A significant number of infants and mothers had low hair zinc levels. Hair zinc concentrations of infants decreased significantly towards the end of first year. This may be due to low zinc intake of mothers. The main contribution of our study to the literature was the positive relationship between the red meat intake and maternal hair zinc levels.

Assessment of living donors with respect to pre- and posttransplant psychosocial properties and posttransplant family functioning in pediatric liver transplantation.

BACKGROUND/AIMS: We aimed in this study to investigate pre- and posttransplant clinical and psychosocial features of the donors and the effects of living-related liver transplantation and possible relevant factors on psychosocial outcome and family functioning. METHODS: Thirty-two living donors (19 females, age 31.84 ± 7.10 years) were evaluated. Medical records of donors regarding pre- and posttransplant clinical and psychological features and family life were evaluated. RESULTS: The donors were parents (n=28, 87.6%) in most. In the pretransplant evaluation, 5 donors (19.3%) had anxiety regarding postoperative complications and quality of life. Donors were discharged from the hospital in a median of 7 days (range, 5-30 days). Return to work and feeling of complete well-being were accomplished in a median of 4 weeks (range, 1-32 weeks) and 10 weeks (range, 4-48 weeks), respectively. Sixteen recipients (50.0%) suffered from major complications, and 3 (9.4%) required invasive intervention. Fourteen donors (43.4%) reported pain around the surgical incision and nonspecific gastrointestinal problems postoperatively. Psychological problems were observed in 8 donors (25.0%); 2 (6.3%) had depression requiring drug and psychotherapeutic intervention. Psychological disruption was found to be correlated with the presence of problems in the recipient (p<0.01, r=0.487). The donors' relationship with the recipient was negatively affected in 1 (3.1%), but improved in 15 (46.9%) cases. Nine donors (34.6%) displayed nervous behavior toward their spouses, and 2 (7.7%) later divorced. Life of the other family members was negatively affected in 8 (30.7%). Two donors' spouses (7.7%) failed to carry out domestic responsibilities. CONCLUSIONS: Psychological disturbance and abnormal family functioning are frequently observed during the posttransplant period. Therefore, psychologic assessment and evaluation of family functioning should be regularly repeated during the posttransplant period.

Human herpes virus type 8-associated Kaposi sarcoma in a pediatric liver transplant recipient.

Development of KS in pediatric liver transplant recipients is a rare entity and has dismal prognosis. Latent HHV-8 infection, immunosuppression, and genetic predisposition are possible etiological factors. Decreasing the dose or cessation of immunosuppressive drugs, switching to sirolimus with antiproliferative and antitumor properties, and different chemotherapeutic regimens are the current therapeutic strategies. We herein report a pediatric liver transplant recipient who developed generalized KS at post-transplant fifth month. The disease had an aggressive course despite the highly toxic chemotherapy. On the other hand, a prompt and durable response was provided by paclitaxel with tolerable side effects. The patient is now free of disease for at least 24 months and healthy with good graft function under sirolimus therapy as maintenance immunosuppression. Instead of highly toxic chemotherapy, paclitaxel can be used as therapeutic option in cases with generalized disease and in those who are unresponsive to conventional chemotherapy. However, new studies are needed to assess the efficacy of the paclitaxel therapy in KS in the liver transplant recipients.

The role of the non-invasive serum marker FibroTest-ActiTest in the prediction of histological stage of fibrosis and activity in children with naïve chronic hepatitis B infection.

The aim of this study was to investigate whether the non-invasive serum marker FibroTest-ActiTest (FT-AT) reliably predicts the histological stage of fibrosis and/or activity, and decreases the need for a liver biopsy. Twenty-five children with naïve chronic hepatitis B were analyzed for haptoglobin, alpha2-macroglobulin, apolipoprotein A1, bilirubin, gamma-glutamyl transferase, and alanine aminotransferase activity, and the FT-AT scores were computed. FT-AT scores were compared with histological data. FT predicted insignificant fibrosis in 14/23 (61%) patients at a cut-off level of 0.31. Nine patients (36%) had significant histological fibrosis, but none were predicted by FT. There was no correlation between FT scores and histological stage of fibrosis (r: -0.221, p = 0.228). All 4 patients with significant histological activity had corresponding significant activity in AT (100%). Fifteen out of the 19 patients (78.9%) with significant activity in AT had insignificant histological activity. At the cut-off level of 0.36, AT predicted insignificant activity in all 6 patients (100%). There was no correlation between AT scores and histological activity (r: 0.245, p = 0.237). According to histological data, 12 patients were candidates for treatment, but FT-AT did not predict 3 of them (25%). FT-AT does not appear ready for use in detecting either the stage of fibrosis or activity in children with chronic hepatitis B.

Valproic acid-associated vanishing bile duct syndrome.

Hepatotoxicity as a result of valproic acid therapy is well documented. Elevation in aminotransferase activities is rarely associated with symptoms. It sometimes manifests as acute liver failure. Here, we report a 8-year-old girl who was referred for unresolving jaundice and itching for 3 months. Past history revealed afebrile convulsion 5 months previously and beginning of valproic acid treatment. Valproic acid was discontinued after the development of jaundice. Physical examination revealed ichterus, xanthomas on extensor surfaces of extremities, and hepatomegaly without any sign of chronic liver disease. Total and direct bilirubin levels were 20.2 and 12.9 mg/dL, respectively. Enzyme activities indicating cholestasis were increased together with blood cholesterol. Tests for infectious and autoimmune, metabolic, and genetic disorders were not informative. Liver biopsy revealed portal inflammation, severe bile duct loss, and cholestasis. The patient was considered to have valproic acid-associated vanishing bile duct syndrome, which has not been reported previously.

Is there a relationship between Helicobacter pylori infection and erosive reflux disease in children?

AIM: The aim of this study was to investigate the relationship between Helicobacter pylori infection and erosive reflux disease in children. METHODS: A total of 206 children [mean age 8.4 +/- 4.9 (0.16-18) years] who underwent diagnostic upper endoscopy were tested for H. pylori infection between 2002 and 2005 and the relationship between H. pylori infection and gastro-oesophageal reflux disease was investigated retrospectively. Endoscopic and histopathological findings were examined retrospectively. When reflux-related oesophageal damage was identified as a result of the histological examination of endoscopic biopsy samples collected from distal oesophagus, the patients were diagnosed with gastro-oesophageal reflux disease and divided into two groups: those with macroscopic erosions or ulceration constituted the erosive oesophagitis group; those without constituted the non-erosive reflux disease group. RESULTS: Prevalence of H. pylori infection was 31.3% in the patients with gastro-oesophageal reflux disease and 36.7% in the control group (p > 0.05). Prevalence of erosive oesophagitis was found to be 23.8% in the patients with H. pylori infection and 41.3% in those without (p > 0.05). CONCLUSION: No negative significant association was found between the prevalence of H. pylori infection and erosive oesophagitis. Presence of H. pylori infection did not influence the severity of oesophagitis either.

Investigation of impaired carbohydrate metabolism in pediatric liver transplant recipients.

OGTT was performed in 28 liver transplants maintained with tacrolimus to investigate carbohydrate metabolism and assess risk factors for development of PTDM. None had PTDM that was detected by OGTT. Early PTDM in four cases (14.3%) resolved in follow-up. Five new cases (17.9%) demonstrated DCM (DCM = IGT +/- hyperinsulinemia). Fasting measurements were normal in two hyperinsulinemic cases. With one (20%, p > 0.05) exception none of the children with DCM were overweight or had a family history of diabetes. All five (100%) children with DCM had been given high cumulative dosage of steroids 18 (78.3%)--without DCM (p > 0.05). The median age of children with DCM was greater [4.3 (12.7-18.0) vs. 7.0 (2.3-18.0) yr, p < 0.01] and duration of follow-up longer [5.3 (2.3-7.0) vs. 2.5 (0.7-7.3) yr, p < 0.05]. Four children (80%) with DCM were pubertal (p < 0.05). However, neither age nor duration of follow-up or pubertal stage had significant effect on DCM development. Early PTDM is a transient phenomenon and is not predictive for future development of diabetes. DCM is frequently observed in liver transplanted children. Albeit the children with DCM were given high cumulative dose of steroids, were older, mostly were pubertal, and had longer duration of follow-up, we cannot draw firm conclusions on effects of the risk factors on carbohydrate metabolism because of the small sample size and relatively short duration of follow-up. Unlike fasting measurements, OGTT can detect all children with DCM.

Association of Helicobacter pylori infection with gastroduodenal disease, epidemiologic factors and iron-deficiency anemia in Turkish children undergoing endoscopy, and impact on growth.

BACKGROUND: The purpose of the present paper was to investigate the relationship between Helicobacter pylori infection and clinical symptomatology, breast-feeding and socioeconomic level. The relationship between H. pylori and iron-deficiency anemia (IDA) and the effect of H. pylori infection on growth were also investigated. METHODS: The subjects consisted of 70 patients aged 4-16 years who underwent upper gastrointestinal endoscopy for recurrent abdominal pain, nausea, vomiting, and dyspeptic complaints during a 2 year period. Patients were divided into two groups according to presence of histological evidence of H. pylori infection (group 1, H. pylori positive; group 2, H. pylori negative) and groups were compared with respect to epidemiologic characteristics, gastrointestinal complaints, height and weight SD scores and IDA. RESULTS: Thirty-five (50%) of the 70 patients participating in the study were H. pylori positive. The mean age of group 1 was significantly higher than that of group 2. There were similar characteristics and symptomatology between groups. The majority of the patients in group 1 belonged to low socioeconomic class (class I and II; P < 0.05). The number of the patients exclusively breast-fed for > or =4 months was significantly higher in group 2 than in group 1. Gastritis was significantly more frequent in group 1. Mean hemoglobin, serum Fe and ferritin levels were 11.6 +/- 1.7 g/dL, 45.0 +/- 23.2 microg/dL and 11.9 +/- 8.4 microg/dL, respectively, for group 1 and 12.2 +/- 0.7 g/dL, 79.3 +/- 26.4 microg/dL and 42.1 +/- 31.8 microg/dL, respectively, for group 2. The mean serum Fe and ferritin levels of group 2 were significantly higher than those of group 1. IDA was observed in 20 (57.1%) and six (17.1%) patients in groups 1 and 2, respectively. IDA was significantly more frequent in group 1. Helicobacter pylori infection was found to be the only variable that had significant effect on IDA. Mean SD height and weight for group 1 were lower than those of the group 2. When the patients were evaluated in four groups according to H. pylori and IDA status, mean height SD score of patients with both H. pylori infection and IDA was significantly lower than that of the patients negative for H. pylori and IDA concomitantly. CONCLUSION: Low socioeconomic status seems to be an important risk factor for H. pylori infection. Exclusive breast-feeding at least for 4 months can have a protective role against H. pylori infection. Increased frequency of growth retardation and IDA in H. pylori-infected patients in the present study supports similar findings in the literature, although there is still need for detailed studies to clarify the causative mechanisms.

Analbuminemia produced by a novel splicing mutation.

Analbuminemia is a rare autosomal recessive disorder manifested by the absence or severe reduction of circulating human serum albumin in homozygous or compound heterozygous individuals. It is an allelic heterogeneous defect, caused by a variety of mutations within the albumin gene. The analbuminemic condition was diagnosed in a Turkish female infant on the basis of low albumin concentration ( approximately 9.0 g/L). The albumin gene was screened by single-strand conformation polymorphism and heteroduplex analysis and submitted to direct sequencing. The proband was found to be homozygous for a T-->C transition at nucleotide 13381, the 2nd base of intron 11. The effect of this previously unreported mutation, which inactivates the strongly conserved GT dinucleotide at the 5' splice site consensus sequence of intron 11, was evaluated by examining the cDNA obtained by reverse transcription-PCR from the albumin mRNA extracted from the proband leukocytes. This analysis revealed that the mutation, named Bartin for the geographical origin of the patient's family, results in the skipping of exon 11. The subsequent frameshift within exon 12 originates a premature stop codon located 5 codons downstream at position 411. The predicted translation product would consist of 410 amino acids. This novel extensive cDNA alteration is responsible for the analbuminemic trait.

Elevated hair levels of cadmium and lead in school children exposed to smoking and in highways near schools.

OBJECTIVES: Heavy metal pollution has become a serious health concern in recent years. Cadmium (Cd) and Lead (Pb) are toxic heavy metals. This study was aimed to determine the risk factors for high cadmium and lead levels in school children. DESIGN AND METHODS: The scalp hair samples were obtained from 760 children at 13 schools in Istanbul. A questionnaire was prepared to gather information about demographic and socioeconomic characteristics of the children. Hair cadmium and lead concentrations were determined by atomic absorption spectrophotometer. RESULTS: Household exposure to smoking and attending a school near to Main Streets were found to be the most important risk factors for the high hair cadmium and lead levels in our study. CONCLUSION: These findings support the public health recommendations that children should not have household exposure to smoking, schools should not be near to the main streets and unleaded gasoline use should be promoted.

Comparison of interferon monotherapy with interferon-lamivudine combination treatment in children with chronic hepatitis B.

AIM: To compare interferon monotherapy with combination treatment using interferon and lamivudine in children with chronic hepatitis B. METHODS: Data from 65 children who had received either interferon-alpha (5 MU/m2 subcutaneous thrice a week for 6 months; n=35; Group 1) or this dose of interferon-alpha for 6 months with oral lamivudine for one year (4 mg/Kg/day, maximum 100 mg/day; n=30; Group 2) were analyzed retrospectively. Complete response was defined as ALT normalization, HBeAg/anti-HBe seroconversion and HBV DNA clearance. RESULTS: ALT normalization rates were similar in Groups 1 and 2 at the end of interferon treatment (13 [38%] and 16 [52%], respectively), at 12 months (19 [56%] and 18 [58%]) and at 24 months (24 [71%] and 23 [74%]). HBV DNA clearance was more frequently observed at 6 months in Group 2 than in Group 1 (19 [63%] versus 7 [20%]; p=0.01), but not at 12 months (19 [63%] versus 17 [49%]) or at 24 months (20 [67%] versus 21 [60%]). Rate of HBeAg/anti-HBe seroconversion was higher in Group 2 at 12 months (18 [60%] versus 11 [31%]; p< 0.05). Rate of complete response was similar in Groups 1 and 2 at 6 months (5 [14%] and 10 [33%], respectively), 12 months (14 [40%] and 17 [57%]) and 24 months (20 [57%] and 19 [63%]). CONCLUSION: Although lamivudine and interferon combination achieved higher initial rates of HBV DNA loss and HBeAg/anti-HBe seroconversion than interferon alone, the final response rates were similar with the two treatments. The combination treatment is therefore not indicated for chronic hepatitis B in children.

CagA positivity and its association with gastroduodenal disease in Turkish children undergoing endoscopic investigation.

BACKGROUND: Cytotoxin-associated gene A (CagA) product is a bacterial virulence factor contributing to the pathogenicity of Helicobacter pylori (HP) infection in humans. Host factors, which vary in different countries, interact with bacterial factors to determine the disease state. Our objective was to investigate the frequency of CagA-positive HP strains and evaluate the contribution of CagA positivity to symptoms and development of mucosal lesions in HP-infected Turkish children. METHODS: We conducted a prospective clinical trial in 240 consecutive Turkish children undergoing endoscopy (110 girls, 130 boys; mean age, 8.7 +/- 4.3 years). HP infection was diagnosed on the basis of a positive rapid urease test and histology of the mucosal specimens. HP IgG and CagA IgG antibodies were measured by enzyme-linked immunosorbent assay in HP-positive children. RESULTS: The HP positivity rate was 50.4% in our study group (51 girls, 70 boys; mean age, 9.9 +/- 3.9 years). CagA was positive in 74.4%. HP infection was less common in children with vomiting (25.9%, P < 0.05). CagA positivity was not associated with any clinical symptom. HP positivity was higher in children with duodenal ulcer (80% vs. 49.1%, P = 0.05); while CagA positivity was similar. Antral nodularity was strongly associated with HP positivity and CagA positivity (30.6% vs. 3.4% and 36.7% vs. 12.9%, respectively, P < 0.05). A negative association between CagA positivity and esophagitis was observed (20% vs. 76.7%, P < 0.05). CONCLUSIONS: CagA positivity is common in HP-infected Turkish children. Esophageal lesions are less common in children infected with CagA-positive strains. Although HP is associated with duodenal ulcer disease, CagA positivity does not seem to contribute to development of ulcers in children in our series.

Successful living-donor liver transplantation and retransplantation with cavoportal hemitransposition: a case report.

An 11-month-old female infant underwent living-donor liver transplantation for secondary biliary cirrhosis 8 months after Kasai operation. The portal vein was hypoplastic, and its diameter was only 4 mm at the level of the splenomesenteric confluence. End-to-end anastomosis of the recipient suprarenal vena cava to the graft portal vein (a left lateral section from the patient's mother) was performed. An end-to-side portocaval shunt with the recipient portal vein was constructed to mitigate portal hypertension. The early postoperative course was relatively uneventful. However, persistent hepatitis caused by infection with Cytomegalovirus and chronic rejection resulted in progressive hepatic dysfunction. Nine months after the initial operation, a living-donor retransplantation (a left lateral section from the patient's grandmother) was performed. One month after retransplantation, severe acute rejection that eventually required OKT3 treatment developed. The patient was in excellent health until 4 months after retransplantation, when another acute rejection episode (for which she was successfully treated) developed. Cavoportal hemitransposition should be included in the armamentarium of the transplant surgeon for the management of extensive portal system thrombosis and portal vein hypoplasia. An additional shunt may be useful in mitigating portal hypertension.

Long-term outcome after sclerotherapy with or without a beta-blocker for variceal bleeding in children.

BACKGROUND: Esophageal variceal bleeding is a life-threatening complication of portal hypertension. Optimal treatment for the prophylaxis of variceal rebleeding in children has not yet been determined. In the present study, we aimed to compare the long-term efficacy of endoscopic sclerotherapy with or without oral beta-blocker therapy in the secondary prophylaxis of variceal bleeding. METHODS: Thirty-eight children who had undergone endoscopic sclerotherapy (EST) sessions for variceal bleeding in the Department of Pediatric Gastroenterology, Istanbul University Istanbul School of Medicine, were entered into this retrospective cohort study. Twenty patients (mean +/- SD age 7.0 +/- 2.7 years) had undergone only sclerotherapy sessions (SG), whereas 18 patients (mean age 6.8 +/- 3.4 years) had received oral propranolol (1-2 mg/kg per day) additionally for 2 years (SPG). The number of patients with successful obliteration, the time required for obliteration and variceal recurrence rate were analyzed as primary indicators of the effectiveness of therapy. RESULTS: Variceal obliteration was achieved in 16 of 20 patients (80%) in the SG group and in 16 of 18 patients (88%) in the SPG group. Time required for variceal obliteration was significantly shorter in the SPG group compared with the SG group (4.1 +/- 1.4 vs 3.2 +/- 0.9 months; P < 0.05). The variceal recurrence rate was 65 and 38.8% in the SG and SPG groups, respectively. Compared with the SG group, less variceal rebleeding was observed during EST in the SPG group (25 vs 16.6%, respectively).However, these differences were not statistically significant. CONCLUSIONS: Endoscopic sclerotherapy combined with oral propranolol treatment shortens the time required for variceal obliteration. However, the other indicators of treatment effectiveness are not influenced statistically by the addition of propranolol to the treatment regimen. Randomized prospective clinical studies in larger pediatric series are needed before offering a combination of EST with oral propranolol as the most rational approach in the secondary treatment of esophageal variceal bleeding in children.