Hepatoportal sclerosis in childhood: descriptive analysis of 12 patients.

Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.

Fecal calprotectin concentration in celiac disease.

GOALS: We aimed to determine fecal calprotectin (FC) concentration and its relation with histopathologic findings of children with celiac disease (CD) and to observe the probable alterations under gluten-free diet (GFD). BACKGROUND: As FC is regarded as a marker of inflammation in the gastrointestinal tract, we hypothesized that it might be increased in untreated CD. STUDY: The study included 29 newly diagnosed patients with CD (mean age: 6.6+/-0.6 y) and sex and age-matched 10 healthy children. All of the children with CD admitted to the hospital were classical form who has chronic diarrhea and failure to thrive. The degree of mucosal damage was graded according to the modified Marsh criteria. FC concentration was determined by enzyme-linked immunosorbent assay method on admission and after 1 year of GFD. RESULTS: Mean FC concentration of children with CD on admission and of healthy children were 13.40+/-8.5 and 4.3+/-3.3 mg/L, respectively (P=0.004). FC concentration under GFD was 4.6+/-2.7 mg/L and there was a significant statistical difference between untreated patients and those under GFD for 1 year (P=0.001). There was no statistical difference between FC concentration of those under GFD and healthy children (P=0.8). Mean FC concentrations of children with total-villous atrophy and partial-villous atrophy were significantly different (13.8+/-9.3 mg/L vs. 3.7+/-1.8 mg/L, P=0.005). CONCLUSIONS: It was found that FC concentration is increased in childhood CD, related to the severity of histopathologic findings and responsive to GFD. The pathogenetic mechanism by which FC is increased in CD should be investigated in further studies.

Prevalence of orthorexia among medical students in Erzurum, Turkey.

Orthorexia is a new term about eating behavior disorder and consists of pathologic obsession for biologically pure foods, free of herbicides, pesticides, and other artificial substances. It is not an independent diagnostic category, but it has some similarities with other eating disorders. This study was conducted to examine the orthorexia among 878 medical students. Of 878 students, 464 (52.8%) were male and 359 (40.9%) were female. The mean age, height, weight, and body mass index were 21.3 +/- 2.1 years, 171.0 +/- 8.5 cm, 65.6 +/- 12.3 kg, 22.4 +/- 2.99, respectively. The rates of the ORTO-11 scores between 0 and 15 was 1.9%; between 16 and 30, 57.5%; and between 31 and higher, 21.1%. There were 17 students with a score of 0 to 15. The mean score for the ORTO-11 test was 27. There were statistically significant differences between age, sex, and smoking habit of the students. In the male students, there was a statistically significantly higher tendency for orthorexia (P = .001), and there was a statistically significant difference between the age groups for tendency for orthorexia (P = .025). In logistic regression analysis, age, sex, Eating Attitude Test-40 (EAT-40), and height affected the ORTO-11 scores.

Hartnup disease masked by kwashiorkor.

This report describes an 11-month old girl with Hartnup disease presenting with kwashiorkor and acrodermatitis enteropathica-like skin lesions but free of other clinical findings. This case with kwashiorkor had acrodermatitis enteropathica-like desquamative skin eruption. Since zinc level was in the normal range, investigation for a metabolic disorder was considered, and Hartnup disease was diagnosed.

Treatment results of chronic hepatitis B in children: a retrospective study.

In this retrospective study, we aimed to share our experience with different treatment modalities for chronic hepatitis B in a series of children. The study included 126 children (mean: 9.5 +/- 3.8 years). Normalization of alanine aminotransferase (ALT), loss of hepatitis B virus (HBV)-DNA and hepatitis B e antigen (HBeAg), and development of antibody to HBeAg (anti-HBe) altogether at the end of the treatment was considered as end of therapy response (ETR). Seroconversion ongoing one year after the cessation of therapy was considered as sustained response. Of the total children, 90 (71.4%) were treated, whereas the remaining were just followed-up. High-dose interferon (IFN)-alpha (10 MU/m2) alone, standard-dose IFN-alpha (6 MU/m2) plus lamivudine (4 mg/kg/d), high-dose IFN-alpha plus lamivudine, or lamivudine alone was used, IFN-alpha thrice weekly for six months, and lamivudine daily for one year. Of children who had completed their treatment, 34 (37.8%) achieved ETR. Sustained response rate was 36.7%. Response rates were different in the different treatment groups (p: 0.01). The highest response rate was observed in those who received standard-dose IFN-alpha plus lamivudine treatment (61.5%). Of children without treatment, one (2.8%) had anti-HBe seroconversion. Standard-dose IFN-alpha plus lamivudine treatment was found superior to the other treatment modalities. Predictors of ETR were similar to those found in previous studies.

Dermatological signs in Wilson's disease.

BACKGROUND: Because no data on skin and mucosal findings of patients with Wilson's disease have been published so far, the aim of the present study was to investigate the prevalence of mucosal and skin findings in childhood Wilson's disease and to determine its specific dermatological findings, if any exist. METHODS: Thirty-seven 4-17-year-old children with Wilson's disease were included. A complete skin, scalp skin, mucous membrane and nail examination was performed. RESULTS: Of the children, 26 (70.3%) had at least one dermatological finding. Twenty-five (67.6%), five (13.5%), nine (24.3%) had at least one skin, mucosal and nail finding, respectively. The most prevalent dermatological diagnosis of the Wilson's disease patients was xerosis (45.7%). The presence of dermatological findings was not related to drug usage, severity of the disease, or malnutrition. The duration of the disease was not different in patients with or without dermatological findings. The frequency of skin findings alone, however, was high in relatively newly diagnosed patients (<2 years). CONCLUSION: Dermatologist should be aware of the various dermatological manifestations of Wilson's disease, because a careful and objective skin, mucosa, nail and hair examination may be indicative of a diagnosis of Wilson's disease, particularly in early cases.

Serum ghrelin levels in children with primary protein-energy malnutrition.

BACKGROUND: Ghrelin, an appetite-stimulating peptide, increases in cachectic conditions. It probably reflects peripheral nutritional status and influences nutrient intake and growth. The aim of the present study was to determine serum ghrelin levels in children with primary protein-energy malnutrition (PEM) and to find if any correlation exists between serum ghrelin levels and the clinical presentation of those patients. METHODS: Twenty-eight children with primary PEM and 10 healthy children were included. Serum fasting ghrelin levels were measured using radioimmunoassay. RESULTS: Mean serum ghrelin level of healthy children and those with PEM were 107.7 +/- 40.1 pg/mL and 141.6 +/- 123.8 pg/mL, respectively (P < 0.001). Ghrelin levels were independent of age and sex (P > 0.05). Ghrelin was negatively correlated with body mass index in healthy children (P < 0.01), but not in those with PEM (P > 0.05). Mean serum ghrelin level of children with moderate malnutrition was higher than that of children with severe malnutrition (199.2 +/- 154.1 pg/mL vs 98.4 +/- 74.3 pg/mL, P < 0.05). Mean serum ghrelin levels of patients with kwashiorkor, marasmic kwashiorkor, and marasmus were 127.9 +/- 97.8 pg/mL, 138.7 +/- 95.8 pg/mL, and 162.3 +/- 185.0 pg/mL, respectively (P > 0.05). CONCLUSION: Serum ghrelin level is higher in patients with PEM, especially in those with marasmus, compared to healthy children. Although this observation suggests that ghrelin helps to fight malnutrition in children, it is obvious that further studies are needed to clarify the exact pathogenetic mechanism regarding this condition.

Comparison of two different regimens of combined interferon-alpha2a and lamivudine therapy in children with chronic hepatitis B infection.

AIM: To evaluate the efficacy of two regimens of combined interferon-alpha2a (IFN-alpha2a) and lamivudine (3TC) therapy in childhood chronic hepatitis B. METHODS: A total of 177 patients received IFN-alpha2a, 9 million units (MU)/m2 for 6 months. In group I (112 patients, 8.7 +/- 3.5 years), 3TC (4 mg/kg/day, max 100 mg) was started simultaneously with IFN-alpha2a, in group II (65 patients, 9.6 +/- 3.8 years) 3TC was started 2 months prior to IFN-alpha2a. 3TC was continued for 6 months after antiHBe seroconversion or stopped at 24 months in nonresponders. 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 II, respectively (P > 0.005). Complete response was 55.3% and 27.6% in groups I and II, 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 II, 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 II, 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-alpha2a 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.

Breast-feeding duration and childhood acute leukemia and lymphomas in a sample of Turkish children.

OBJECTIVES: Whether breast-feeding is associated with decreased incidence of the lymphoid malignancies in children is uncertain. We evaluated childhood acute leukemia and lymphoma in relation to duration of breast-feeding. METHODS: We investigated this issue in a case-control study comprising 137 patients, aged 1 to 16 years, with acute lymphocytic leukemia (ALL), acute myeloid leukemia (AML), Hodgkin or non-Hodgkin lymphoma, in addition to 146 controls matched for age and sex. RESULTS: The median duration of breast-feeding among patients was shorter than that of controls (10 vs 12 months). Patients with ALL and AML had shorter mean breast-feeding duration compared with healthy children (P = 0.001 and P < 0.001, respectively). The shortest mean breast-feeding duration was noted in the children with AML. Breast-feeding for a duration of 0 to 6 months, when compared with feeding of longer than 6 months, was associated with increased odds ratios (ORs) for ALL [OR = 2.44, 95% confidence interval (CI) = 1.17-5.10], AML (OR = 6.67, 95% CI = 1.32-33.69), Hodgkin lymphoma (OR = 3.33, 95% CI = 0.60-18.54), non-Hodgkin lymphoma (OR = 1.90, 95% CI = 0.68-5.34) and overall (OR = 2.54, 95% CI = 1.51-4.26). CONCLUSIONS: Our findings suggest that breast-feeding of more than 6 months is protective against childhood lymphoid malignancies, especially for AML and ALL.

Effects of lamivudine therapy on the glucose metabolism in children with chronic hepatitis B: first year follow-up results.

BACKGROUND AND AIM: Although the most common major toxicity of lamivudine has been pancreatitis, there is no report investigating possible impaired pancreatic functions, including glucose intolerance due to lamivudine therapy. The aim of this study was to evaluate the effects of lamivudine on the glucose metabolism in children. METHOD: Twenty-three children were included: eight patients were treated with lamivudine, others with both lamivudine and interferon-alpha. An oral glucose tolerance test (OGTT) was performed before the treatment, and after 6 and 12 months. RESULTS: After 6 and 12 months of the treatment four (18.4%) and eight (34.8%) patients had impaired OGTT, respectively. We did not find any relationship between impaired OGTT and age, gender, elevated amylase, abdominal pain and the mode of therapy (P>0.05). While mean glucose value after 2 h was higher than that of baseline, mean insulin concentrations and area under the curve values were not different (P<0.0001, P>0.05, and P>0.05, respectively). CONCLUSION: This is the first report demonstrating that lamivudine may impair the OGTT. Since at least 8.7% of our patients had persistently impaired OGTTs during the first year of the therapy, it may be reasonable to screen children before lamivudine therapy is started.

Seroepidemiology of varicella-zoster virus infection in a cosmopolitan city (Erzurum) in the eastern Turkey.

OBJECTIVE: The aim of the study was to determine VZV seroprevalence under age 30 and to identify the relationship of VZV seroprevalence and several sociodemographic characteristics of the study subjects. The results were presented in order to design a strategy for vaccination against varicella-zoster virus (VZV). MATERIAL AND METHOD: It was planned to include a total of 568 subjects. The sampling method of 30 clusters recommended for field studies was used for selecting subjects of a predetermined number in the rural and urban areas in eastern Turkey. ELISA method was used to examine the blood samples for VZV seropositivity. Age, gender, place of living, educational level, family size and socioeconomic status was investigated in the study subjects. RESULTS: Positive VZV seroprevalence was detected in 78% of 559 subjects. Seroprevalence increased with age. Seroprevalence was 16.67% at the age of 1 year, subsequently increased to 57.58% at the age of 4 years, 70% at the age of 7 years, 92.31% at the age of 10 years and then remained 86.78-96.36% in subjects over the age of 10 years. No association was found between sociodemographic variables studied and prevalence levels of antibodies except for educational level in the 0-14 year group. CONCLUSION: These results suggest that the majority of VZV infections occur during the early childhood; the best option to reduce the circulation of wild type VZV in the population would be the immunization of young children. VZV vaccine should be introduced into the routine childhood vaccination programme in Turkey.

Celiac disease, phylloid hypomelanosis and autoimmune thyroiditis: a case report.

Pigmentary mosaicism is a term used to encompass all of these different types of pigmentary patterns. Among these mosaic patterns, there have been only a few reports of the phylloid presentation in the literature. On the other hand, autoimmune disorders can be associated with neurocutaneous markers and syndromes. A fifteen-year-old girl was presented for chronic diarrhea and abdominal pain. Her physical examination had determined multiple hypopigmented patches. Finally, she was diagnosed phylloid hypomelanosis together with partial trisomy 13 accompanying celiac disease and autoimmune thyroiditis.

Fasting and postprandial glucose, insulin, leptin, and ghrelin values in preterm babies and their mothers: relationships among their levels, fetal growth, and neonatal anthropometry.

OBJECTIVE: To investigate relationship between anthropometric values of premature babies with their's glucose, insulin, leptin, and ghrelin at birth and on day 15. METHODS: We analyzed fasting and postprandial glucose, insulin, leptin, and ghrelin levels at birth and on day 15 in babies born prematurely between 24 and 37 weeks, and who did not have serious problems aside from prematurity at birth. RESULTS: Fasting glucose, insulin, leptin and ghrelin values of babies at birth and on day 15 were significantly lower than postprandial values (all p values p < 0.001). There were positive correlations between the mean insulin, leptin, and ghrelin levels with the gestational age, birth weight, body mass index, head circumference of babies at birth, and anthropometric values on day 15 (all r values > 0.400, all p values < 0.05). Fasting glucose, leptin, and ghrelin values of mothers birth were significantly lower than post-prandial values (all p values p < 0.05). CONCLUSIONS: The positive correlations between the insulin, leptin, and ghrelin values of babies at birth with gestational age and anthropometric values suggest that both hormones play important roles in fetal and neonatal growth and development.

Association of Bartter's syndrome and empty sella.

Bartter's syndrome is characterized by hypochloremia, hypokalemia, metabolic alkalosis associated with renal potassium leakage, and normal blood pressure despite increased plasma renin activity. Although association of empty sella with Gitelman syndrome has been reported, no association has been reported with Bartter's syndrome. Here we report a patient with Bartter's syndrome and empty sella. A 12 month-old male patient presented with a history of nausea, vomiting, abdominal distension, constipation, and edema in the lower extremities that had begun in the early postnatal period. The patient was born at 32 weeks gestation by operative delivery for polyhydramnios. Blood pressure was normal. Serum sodium, potassium, calcium, phosphate, chloride, albumin and alkaline phosphatase levels were 129 mEq/l, 2.5 mEq/l, 9 mg/dl, 3.8 mg/dl, 72 mg/dl, 4.2 g/dl and 1285 IU/l, respectively. Serum magnesium level was normal. Arterial blood gas levels revealed pH 7.55 (normal, 7.35-7.45), PCO2 33.6 mm/Hg (36-46), base excess +7.1 (+/- 2.3), and total CO2 33.6 mmol/l (23-27). Renin and aldosterone levels were elevated. Urine had pH 8.0 and specific gravity 1.010. Urinary calcium excretion was 22.8 kg/day (urine calcium/creatinine ratio 0.46). Urinary potassium and chloride levels were elevated. MRI of the brain was normal except for partially empty sella. We present the first pediatric patient with the association of Bartter's syndrome and empty sella.

Serum leptin levels in neonatal bacterial septicemia.

The principal aim of this study was to evaluate serum leptin concentrations and to analyze the interaction between serum leptin levels and C-reactive protein (CRP) levels, hematological parameters before and after antimicrobial therapy in neonates with bacterial septicemia. We studied 16 neonates with bacterial septicemia and 15 controls. Blood samples in neonates with septicemia were collected just before antimicrobial therapy and 2 weeks after treatment. The mean concentration of serum leptin, CRP levels, and immature/total neutrophil (IT) ratio in newborns with septicemia were significantly higher than those of controls at the start. Two weeks after treatment, serum leptin levels in newborns with septicemia had decreased and were similar to those of controls. Although there were positive correlations between serum leptin levels and serum CRP levels and IT ratio in the septicemic group at the start, there were no correlations between serum leptin levels and other hematological parameters. These results suggest that leptin is not only an adipostatic hormone but also a stress-related hormone.