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.

The prevalence of celiac disease in children with iron-deficiency anemia.

BACKGROUND/AIMS: Celiac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals. Iron-deficiency anemia is the most commonly encountered anemia in humans. Iron-deficiency anemia also is a common extraintestinal manifestation of celiac disease. To determine the celiac disease prevalence in children with iron-deficiency anemia and to compare the hematologic parameters in iron-deficiency anemia patients with and without celiac disease. MATERIALS AND METHODS: A total of 61 patients aged 2-16 years who presented with iron-deficiency anemia were included in this study. Hemoglobin, red cell indices (mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, red cell distribution width), serum iron, and serum ferritin were determined. Venous blood samples for anti-tissue transglutaminase antibody immunoglobuline A were obtained from these patients. Upper gastrointestinal endoscopy was recommended to patients who had positive serology. RESULTS: Of 61 patients with iron-deficiency anemia, 13 (21,3%) had positive serology for celiac disease. The small intestine biopsy of all patients with positive serology showed villous atrophy (Marsh 3). The mean hemoglobin level was significantly lower in iron-deficiency anemia patients with celiac disease when compared to those without celiac disease (7,8±2,6 vs. 11,3±0,9 g/dL, p>0,05). There was a statistically significant negative correlation of tissue transglutaminase titers with hemoglobin, red cell indices, serum iron, and serum ferritin levels. CONCLUSIONS: Screening of celiac disease by anti-tissue transglutaminase antibody should be done as a routine investigation in children with iron-deficiency anemia. Biopsy should be recommended in patients with iron-deficiency anemia who have positive celiac disease serology.

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.

Do children with Wilson's disease have distinct craniofacial morphology? A cephalometric study.

OBJECTIVES: Patients with Wilson's disease (WD) develop osseous changes such as osteoporosis, spontaneous fractures, areas of sclerosis and demineralization of maxillary and mandibular bones, and neurologic symptoms including swallowing dysfunctions, which may affect dento-facial growth. However, dento-maxillo-facial structures of these patients have never been investigated. The present study aimed to discover if subjects with WD have different dentofacial structures. METHODS: Lateral cephalometric films of 13 children (5 males and 8 females) with WD and of 15 normal subjects (6 males and 9 females) were evaluated. Mean ages of the patients and controls were 12.62 ± 3.09 years and 12.01 ± 1.38 years, respectively. Lateral cephalometric cranial films of all subjects were taken in the same cephalostat in a habitual and unstrained body posture. Thirteen linear and 11 angular parameters were measured to describe the craniofacial characteristics of the subjects. RESULTS: Statistical analysis showed that there is no statistically significant difference between parameters of normal children and children with WD, with the exception of palatal plane inclination. The inclination of palatal plane was higher in children with WD than in normal subjects. CONCLUSIONS: Children with WD and healthy children have approximately the same dento-maxillo-facial structures. However, increased palatal plane inclination may be a finding of WD.

Craniofacial features of children with celiac disease.

BACKGROUND AND GOALS: Growth retardation is one of the most important signs of childhood celiac disease (CD); however, it is not very well known whether craniofacial growth is also affected. We aimed to carry out a detailed craniofacial morphological study to derive a conclusion on the craniofacial features of children with CD. PARTICIPANTS AND METHODS: Eighty-four 2-16-year-old children with biopsy-proven CD and 84 age-matched and sex-matched healthy children were included. Of these, 37 children (44.0%) had been newly diagnosed and 47 (56.0%) were on a gluten-free diet. Anteroposterior and lateral photographs were evaluated using the Scion Image software program for the measurements of the distances between reference points on the face. RESULTS: Except for nasofrontal angle (nfa), nasolabial angle (nla), pronasale height (prnh), nasal dorsum height (ndh), and nasal radix height (nrh), all measurements were significantly greater in patients compared with controls. In celiac children, all facial proportions except forehead/face height (t-gl/t-gn) and nose length/face height (n-ns/t-gn) were significantly different from those of controls. Except for nla, prnh, ndh, nrh, t-gl/t-gn, face height to total face height ratio (sn-gn/t-gn), n-sn/t-gn, ear length to face height ratio (s-sba/t-gn), and face width to face height ratio (z-z/t-gn), all measurements were statistically different in those on a gluten-free diet and newly diagnosed children. CONCLUSION: Most of the facial measurements and proportions of celiac children were different from those of controls. Our data confirm those of a previous study reporting that the forehead proportion is not altered in childhood CD. Pathophysiological mechanisms underlying these alterations are not clear but disruptions of growth during certain critical periods may be responsible.

Is there need for a new hepatitis B vaccine schedule for children with celiac disease?

BACKGROUND: Celiac disease (CD) is an autoimmune disease characterized by immunemediated inflammatory damage of the small intestinal mucosa, precipitated by the ingestion of gluten-containing foods. Since human leucocyte antigen DQ2 (HLA-DQ2) is a marker of nonresponsiveness to hepatits B virus (HBV) vaccine, CD may also be associated with this nonresponsiveness. OBJECTIVES: The aim of this study was to compare the responses to HBV vaccine between children with CD and healthy children. We also investigated the relationship between the patients' responses to hepatitis B vaccine, the clinical presentation of CD, and dietary compliance in the patients. PATIENTS AND METHODS: We recruited 52 children with CD and 20 age- and sex-matched healthy children who received HBV vaccination according to the standard immunization schedule. The production of specific antihepatitis B surface antigen (HBsAg) antibodies was evaluated in all patients and control participants. Subjects with less than 10 IU/L anti-HBs were consided nonresponders to the vaccination. RESULTS: 31 of the 52 patients (59.6%) were female and 21 (40.4%) were male. The mean age of the CD patients was 10.7 ± 4 years (range, 4-18 years). Anti-HBs titers were positive in 32 (61.5%) patients and negative in 20 (38.5%) patients, while they were positive in 18 (90%) of the children in the control group (P < 0.05). We found statistically significant differences between negative anti-HBs titers, clinical presentation of CD, and dietary compliance in patients with CD (P < 0.05). CONCLUSIONS: Nonresponsiveness to hepatitis B vaccination was more frequently found in children with CD than in the control group. Therefore, the response to HBV vaccination should be investigated in children with CD, and a different immunization schedule may need to be developed. Further, compliance to the prescribed gluten-free diet (GFD) may improve the immune response to HBV vaccination in children with CD.

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.

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.

Opsoclonus-myoclonus syndrome attributable to hepatitis C infection.

Opsoclonus-myoclonus syndrome is a rare neurologic disorder characterized by multidirectional chaotic eye movements, myoclonus in the limbs, and ataxia. We present a 9-year-old boy with opsoclonus-myoclonus syndrome associated with hepatitis C infection. To the best of our knowledge, this is the first report of opsoclonus-myoclonus syndrome associated with hepatitis C infection.

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.