Transient elastography of the liver in children with Wilson's disease: Preliminary results.

PURPOSE: In chronic liver diseases, liver stiffness (LS) is increased, primarily because of liver fibrosis, but other factors, such as intrahepatic deposits, may also be involved. We hypothesized that intrahepatic copper accumulation occurring in Wilson's disease (WD) also leads to an increase in LS. The aim of this study was thus to investigate the changes in LS during treatment in pediatric patients with WD. METHODS: Consecutive patients younger than 18 years old, whose WD was diagnosed between 2008 and 2013, were enrolled. All patients underwent testing for liver function and urinary copper excretion, and LS was measured on transient elastography at baseline and at 6- to 12-month intervals during specific therapy. RESULTS: Nine patients were included in the analysis; only two were diagnosed with cirrhosis. The median LS decreased during treatment from 15.1 kPa (range, 5.1-66 kPa) at baseline to 10 kPa (4-16.1 kPa) at follow-up interval 1, and to 6.1 kPa (3.9-11.6 kPa) at follow-up interval 2 (p < 0.0001). In parallel, the differences in urinary copper excretion over the follow-up period were not statistically significant, although the decrease in LS correlated with the increase in urinary copper excretion (r = 0.6). CONCLUSIONS: In pediatric patients with WD, LS is high at the time of diagnosis and decreases during specific treatment, in parallel with an increase in the urinary copper concentration.

Evaluation of anti-cyclic citrullinated peptide antibodies may be beneficial in RF-negative juvenile idiopathic arthritis patients.

Despite the high diagnostic and prognostic performance in adult rheumatoid arthritis, the role of antibodies to cyclic citrullinated peptide (anti-CCP) in juvenile idiopathic arthritis (JIA) is controversial. Occurrence of anti-CCP was mainly seen in rheumatoid factor (RF)-positive polyarthritis patients. In the present study, our aim was to investigate the prevalence and significance of anti-CCP for subjects with JIA in our population. We evaluated anti-CCP reactivity in the sera of 70 patients with various subtypes of JIA in a prospective cohort study. Anti-CCP titres were correlated with the evolution of joint involvement and the presence of joint damage. Nine JIA patients were seropositive for anti-CCP with respect to the cut-off value of the test. In our cohort, 34 patients had a polyarticular joint disease, most of them being RF-negative (30/34, 88 %). All four RF-positive polyarthritis patients had high anti-CCP concentrations and an aggressive erosive disease. In the RF-negative JIA patients, anti-CCP reactivity was in lower titres but significantly associated with polyarticular joint involvement (p = 0.016) and also with the presence of joint damage (p < 0.001). Presence of anti-CCP, at both low and high concentration, was significantly associated with a more severe articular disease in our JIA patients. Investigating anti-CCP should clearly be taken into consideration even among patients with JIA subtypes other than RF-positive polyarthritis.

Clinical significance of the antinuclear antibodies in chronic viral hepatitis B in children.

BACKGROUND: The presence of autoantibodies is frequent in chronic viral hepatitis evolution. Defining the role of autoimmunity in disease evolution or response to interferon treatment is still in debate, both in children and in adults. There are few studies about the influence of autoimmunity in children with chronic viral hepatitis. The aim of our study was to establish the clinical significance of antinuclear autoantibodies (ANA) in chronic hepatitis B in children. METHODS: We have studied 80 children with chronic hepatitis B (30 female; mean age 12.31 +/- 4.13 years). For each patient we performed haematological and biochemical tests and in 41 patients we analyzed the liver histology. The immunological parameters analyzed were: circulating immune complexes (CIC), complement, and the serum levels of immunoglobulin G, A, and M. We analysed by indirect fluorescence the presence of ANA and other autoantibodies. The response to treatment (hepatoprotectors, interferon and Lamivudine) was established depending on seroconversion to HBeAb and HBsAb. RESULTS: Positive ANA were found at baseline in 15% of the patients and during follow-up in 21.25%. In ANA-positive patients compared to those without autoantibodies we found lower levels of haemoglobin and platelet numbers (p = 0.0245, p = 0.0236, respectively), higher gamma-glutamyl-transferase level (p = 0.040) and higher CIC level (p = 0.0155). During interferon treatment 13.79% of those who initially tested ANA-negative presented ANA, compared to 2.56% in those without interferon (p = 0.042). There was no difference in response to interferon treatment according to the presence of ANA (p = 0.4201). CONCLUSIONS: Searching for ANA in children with chronic hepatitis B is a key element in describing the evolution of these patients, especially when considering interferon therapy. Interferon treatment stimulates the development of ANA, but their presence does not influence the response to this particular treatment. The role of autoimmune processes in the evolution and response to treatment of chronic hepatitis B patients remains controversial.

New HLA associations identified in Romanian juvenile idiopathic arthritis patients.

BACKGROUND: Several associations between HLA polymorphisms and JIA subtypes have been reported in multiple populations, but with significant variations across different ethnic groups. The aim of our study was to search specific HLA-DRB1, -DQA1 and -DQB1 polymorphisms that are associated with JIA and different disease phenotypes in Romanian patients compared with other ethnic groups. METHODS: HLA genotyping was performed in 61 JIA patients and 30 ethnicity-matched controls using molecular biology methods, PCR-SSOP. RESULTS: A protective effect across the whole cohort was observed for HLA-DRB1*15 (OR = 0.179, 95% CI: 0.054 -0.58) and HLA-DQB1*06 (OR = 0.285, 95% CI: 0.11-0.72). Each ILAR subgroup had a characteristic pattern of HLA associations. Unlike other populations, we found a positive association of HLA-DQA1*03 with chronic uveitis (OR = 14.67, 95% CI: 1.5-143) and a negative association of HLA-DQA1*01 with early disease onset (OR = 0.175, 95% CI: 0.036- 0.862), suggesting that the HLA-DR/DQ profile can affect the clinical expression of the disease. CONCLUSIONS: The study reveals multiple HLA-DR/DQ associations with JIA, not only similar with those found in other populations, but also some distinctive associations. However, some of the previously established associations were not replicated in Romanian patients. These results might be due to the small size of our cohort or to true population differences.

The activity of 8-iso-prostaglandin F2alpha as an oxidative stress marker in vivo in paediatric patients with type 1 diabetes mellitus and associated autoimmunities.

BACKGROUND: Oxidative stress and inflammatory reactions are known to hold an important role in the etiopathogeny and persistence of acute or chronic clinical entities. Isoprostanes--a group of prostaglandin-like compounds, active products of arachidonic acid--have proved to be representative biomarkers of lipid peroxidation. The aim of this study was to determine the activity of serum 8-iso-prostaglandin F2alpha, (8iPGF2alpha), as an in vivo oxidative stress marker, in paediatric patients with diabetes mellitus type 1 (DM1) and in a control group. The main goals of this study were the following: establishing a possible correlation between the activity of 8iPGF2alpha and the presence of an autoimmune disease associated with DM1 and identifying a possible correlation between 8iPGF2alpha, the value of glycosylated hemoglobin (HbA1c) and the pancreatic autoimmune markers GAD65, IA2, IA in the group of patients with DM1 and other associated autoimmune diseases. METHODS: Fifty-one children and adolescents (31 males) aged 11.65 +/- 4.1 years with DM1 were enrolled in the study. Twenty-seven healthy children, age- and gender-matched, were enrolled as controls. Patients and controls underwent the 8iPGFzalpha assessment through an ELISA serum method. RESULTS: The mean 8iPGF2alpha value was 2090.6 +/- 3536.5 in the DM1 patient group and 509.9 +/- 493.5 in controls (p = 0.03). The mean 8iPGF2alpha value was 2178.19 +/- 4017.05 in patients with DM1 who did not suffer from other associated autoimmune diseases (n = 38) vs. 1834.95 +/- 1504.73 in patients with DM1 and other associated autoimmune diseases (n = 13) (p = 0.76). The correlation between the 8iPGF2alpha and the HbA1c values was determined by obtaining a correlation coefficient r = 0.38 and p = 0.0057. No correlation was observed between GAD65 and 8iPGF2alpha (r = 0.3; p = 0.29), IA2 and 8iPGF2alpha (r = -0.02; p = 0.92), IAA and 8iPGF2alpha (r = 0.4; p = 0.12). CONCLUSIONS: Oxidative stress reactions are more intense in patients with diabetes mellitus type 1 than in healthy patients. Similar results were obtained in patients associating other autoimmune diseases. 8iPGF2alpha can be an ideal marker for determining oxidative reactions in vivo.

Antinuclear antibody screening by ELISA and IF techniques: discrepant results in juvenile idiopathic arthritis but consistency in childhood systemic lupus erythematous.

Lately, many hospital laboratories are using for antinuclear antibody (ANA) screening the solid-phase immunoassays (ELISA-ANA) instead of the traditional immunofluorescence ANA test (IF-ANA). Results of previous studies that compare the two technologies show poor correlation between them in both juvenile idiopathic arthritis (JIA) and childhood lupus erythematous (SLE) patients. In this study, we investigated whether ELISA-ANA and traditional IF-ANA results are comparable in pediatric patients with different rheumatic diseases. A total of 156 consecutive patients were included in the study-90 children with JIA, 33 with reactive arthritis, 19 with SLE, 4 with idiopathic chronic uveitis, and 10 with other systemic rheumatic diseases. ANA determination was performed using both assays. The higher rate of discrepancies between the two methods of ANA screening appeared in the JIA population (19/90, p < 0.001). All JIA patients with false-negative results by ELISA had significant or high IF-ANA titres. The prevalence of JIA-associated uveitis was higher in the group of false-negative ELISA-ANA children than in the ELISA-positive patients but without statistical significance (p = 0.62). On the contrary, in SLE group, the consistency rate of the two assays was 100 %, and the reactivity levels of ELISA-ANA were significantly higher than in ELISA-positive JIA patients (p < 0.001). ELISA seems to be a reliable method for screening ANA in childhood SLE, but not in JIA. Limited by the few SLE patients, our findings need further consideration.

Pill -Induced Erosive Esophagitis in Children.

AIMS: Pill-induced esophagitis has been recognized in adults, but rarely in children. The aim of this article is to discuss endoscopic features, drugs implicated, prevention and treatment in pill-induced esophagitis in children. PATIENTS AND METHODS: Over a period of 4 years, 26 patients presented at our clinic with drug-induced esophageal ulcerations. All patients were diagnosed by means of endoscopy and treated with proton-pump inhibitors and prokinetics. The mean age of the children was 10.76 years. RESULTS: The ulcers were frequently located at the mid-esophagus. Odynophagea, retrosternal pain and dysphagia were the most common presenting symptoms. All children took pills (non-steroidal anti-inflammatory drugs, antibiotics - Doxycycline and ferrous sulfate) with little water and at bed time. The mean elapse between the drug intake and endoscopy was 4.96 days. The symptoms resolved within a maximum of one week of antireflux therapy. CONCLUSIONS: In pediatric cases treated by tablets or capsules, the possibility of medication-induced esophagitis should always be considered. The drug-induced esophagitis should be suspected in all patients presenting with chest pain and dysphagia. Physicians must warn the patients to take the pills and capsules with enough water and in the upright position.

Correlations Among Parental and Neonatal Anthropometric Parameters, Feeding Practices and Infant Obesity.

BACKGROUND AND AIMS: Infant and adult obesity is becoming a real public health concern in Romania, similar to other countries of the European Union. Maternal obesity and excessive weight gain during pregnancy are proven risk factors for the obesity of the child. The protective role of the breastfeeding against obesity has also been demonstrated. The most important issue is whether the choice of a milk formula with the right protein composition could or not protect the newborn from becoming a future obese infant and child. Our study aims to describe the characteristics of a group of macrosomic newborns, in relation to the mothers' weight gain during pregnancy, mode of delivery, birth weight, complications at birth, time of first feeding and type of feeding during maternity stay. PATIENTS AND METHODS: We conducted a retrospective study on 179 newborns with birth weights >4000 grams, born over a period of three months (March-May) in 6 large maternity hospitals in Romania. RESULTS: the newborns had a mean gestational age of 39.5 weeks and a mean birth weight of 4195 grams. Male newborns were prevalent (74%). More than half were born by Cesarian section and had Apgar scores with a median of 9. Macrosomes are prone to complications at birth and in our study those were mainly hypoglycemia and birth trauma. Time at first feeding was 95 minutes (mean), with a high percentage of formula/mixed feeding (68%). CONCLUSION: Macrosomia itself attracts the risk of birth by cesarean section (54% of study group), birth trauma and a low rate of exclusive breast milk feeding (32% of study group) at discharge.

Atrophic gastritis: Helicobacter pylori versus duodenogastric reflux.

OBJECTIVES: The objective of this study was to asses the prevalence of atrophic gastritis in children. We also wanted to compare the clinical manifestation, endoscopic appearance and the degree of the gastric atrophy in children and to identify the possible causes which determine gastric atrophy. METHODS: We evaluated 247 children with chronic gastritis (153 female/94 male, mean age 12.32 years). Atrophy was defined as the loss of normal glandular components, including replacement with fibrosis and/or intestinal metaplasia. RESULTS: The prevalence of the atrophic gastritis was 16.6% (41 cases), mean age 11.59+/-1.75 years, male-to-female ratio 16:25. The clinical manifestations were correlated with the patient age (infants and toddlers were evaluated mostly for weight loss - 4 cases, and older children for abdominal pain - 22 cases). The endoscopic appearance was described as either nodular (15 cases), or erythematous gastritis (10 cases), or normal (10 cases). According to the Sydney System, the degree of atrophy was found to be mild in 3 patients, moderate in 25, and severe in 13 patients; 14 cases were associated with duodenogastric reflux, 5 with Helicobacter pylori and 2 with Helicobacter heilmannii infection, but in 17 cases the etiology was unknown. CONCLUSIONS: Atrophic gastritis is present in childhood, even at very young ages (infants, toddlers). The endoscopic appearance is not characteristic for the presence of atrophy. The degree of the atrophy is not correlated with the age of the children. Because of the relatively high number of duodenogastric reflux associated with gastric atrophy, further studies need to evaluate the potential causes and clinical course.

Maternal obesity - a risk factor for metabolic syndrome in children.

OBJECTIVE: To determine the association between the metabolic syndrome in children (MS) and the pre-pregnancy nutritional status of the mother. DESIGN AND METHODS: A total number of 180 children aged between 6-19 years were examined. Self reported data about parents and their children were collected. The children underwent physical examination; weight, height, waist circumference, blood pressure (BP) were measured. The nutritional status of the children was assessed by body mass index (BMI) and laboratory tests needed to diagnose MS were performed. IDF criteria for MS were used in children 10 years and older, and age and gender specific cut-off points in children younger than 10 years. The mothers were classified in the normal weight, overweight and obese categories according to the pre-pregnancy BMI. The statistical analysis of the data was descriptive and inferential analysis. In the bivariate analysis of the association between qualitative variables, we used the Chi-Square test and the exact Fisher test. The statistical analysis was performed with SPSS v 13.0. RESULTS: 73 (40.55%) children were normal weight, 54 (30%) were overweight and 53 (29.44%) were obese. None of the normal weight children, 16 (29.60%) of the overweight and 23 (43.40%) of the obese ones had MS; 125 (69.44%) of the mothers were normal weight, 44 (24.44%) were overweight and 11 (6.11%) were obese. Pre-pregnancy maternal BMI was significantly associated with offspring MS in both genders, obese children and in the 10-16 age group. CONCLUSIONS: Pre-pregnancy maternal overweight/obesity represents a risk factor for offspring MS. The results are very difficult to compare between studies because of different cut-off values and definition of MS in children. If prevention is the goal rather than treatment, the perinatal period may be an important focus for future research.

Immunological manifestations in type I diabetic children.

In the past decade a number of studies suggested that type 1 diabetes mellitus is an oxidative stress influenced disease. Paraoxonase 1 enzyme plays a crucial role in antiatherogenic-antioxidant circle. The aim of our study was to examine the possible differences in paraoxonase 1 enzymatic activities in diabetic children associated other autoimmune diseases versus a control group. Another objective of the study was to determine if there is any difference according to the gender in paraoxonase 1 activities (arylesterase and paraoxonase activities). Paraoxonase 1 activities were determined in 51 diabetic children and 36 healthy controls. In diabetic children we determined also the C-peptide level. The paraoxonase 1 arylesterase activity was lower in diabetic females compared with diabetic males. The level of C-peptide is in an inverse correlation with the years of the disease. The paraoxonase activities have a correlation with the level of insulin antibodies in type I diabetic children. Our data suggest that paraoxonase enzymatic pattern may be different in these two activities. PON1 arylesterase activity may exhibit a tendency to low levels in women in comparison to men. The C-peptide level is a valuable tool in assessing the restant beta cell function.

Relation of interleukin-6, TNF-alpha and interleukin-1alpha with disease activity and severity in juvenile idiopathic arthritis patients.

BACKGROUND: Evaluation of disease activity in children with Juvenile Idiopathic Arthritis (JIA) is primarily based on clinical examination and conventional parameters of inflammation. But, in daily clinical practice, these two findings often fail to be in accord, making therapeutic decisions difficult. The aim of our research was to evaluate the potential usefulness of IL-6, IL-1alpha and TNF-alpha in monitoring disease activity and severity in JIA. METHODS: In a 2-year prospective study, IL-6, IL-1alpha, and TNF-alpha levels were measured using ELISA in 63 serum samples for 40 JIA patients. The control population consisted of 18 healthy children. The data were correlated with disease activity and severity (quantified with JADAS-27 composite score). RESULTS: The patients with active disease had greater IL-6 levels than did the patients with inactive disease [47.2 pg/mL (2 to 578.7) vs. 2.6 pg/mL (2 to 8.3); p = 0.002] and controls [47.2 pg/mL (2 to 578.7) vs. 2.25 pg/mL (2 to 4); p = 0.006]. Differences between active disease and remission were also significant for every JIA subgroup. The cutoff value for IL-6 in the diagnosis of active disease obtained from the ROC curve analysis was 8.33 pg/mL. Levels of circulating IL-6 were elevated in patients with severe and moderate disease activity (JADAS-27 score > 10) compared with those of low disease activity (JADAS-27 score < or = 10) [80.1 pg/mL (2 to 578.7) vs. 7.41 (2 to 69); p = 0.010]. We found no correlation of serum TNF-alpha and IL-1alpha levels with disease activity in our patients. The most elevated levels of serum TNF-alpha were found in patients during clinical remission with Etanercept. CONCLUSIONS: Serum IL-6 concentrations may serve as a biomarker of disease activity and severity in JIA, providing additional information in certain clinical situations with great discrepancy between clinical assessment and conventional laboratory tests. Upon treatment with Etanercept, although many JIA patients reached remission on medication, they developed increased circulating TNF-alpha levels.

IgA anti-tissue transglutaminase antibodies, first line in the diagnosis of celiac disease.

BACKGROUND: According to the 2008 celiac disease working group run by Dr. A. Fassano under the auspices of the Federation of International Societies of Pediatric Gastroenterology, Hepatology and Nutrition, celiac disease is a chronic immune-mediated enteropathy characterized by gluten sensitivity, which can affect any organ or system, having a wide range of clinical manifestations of variable severity. The serological diagnosis of celiac disease is based on high sensitivity and specificity tests. The measurement of IgA anti-tissue transglutaminase antibodies by ELISA is universally accepted in the screening of celiac disease. METHODS: Using the gold standard represented by IgA anti-endomysium antibodies in a group of 890 children investigated during 2008-2009, we aimed to evaluate IgA anti-tissue transglutaminase antibodies (tTG IgA), as well as to establish their prevalence in associated diseases. RESULTS: Following the measurement of tTG IgA in the entire group, we obtained: sensitivity 773%, positive predictive value 55.2%, specificity 93.1%, negative predictive value 973%, p = 0.000, and in tTG IgA associations we obtained the value 0.51 for the ROC curve area. We found associations of tTG IgA with type 1 diabetes mellitus (235% prevalence), protein-calorie malnutrition (0.89% prevalence), and intestinal malabsorption (0.56% prevalence). CONCLUSIONS: Our results have a high specificity and sensitivity in the screening of celiac disease, while requiring a second method of confirmation.

Ectodermal Dysplasia Showing the Clinical Overlap between Hay Wells Syndrome and Bowen Armstrong Syndrome.

BACKGROUND: Several clinical entities combine ectodermal dysplasia (ED) and cleft lip and/or palate (CL/P). These disorders have been recognized with a narrow phenotypic spectrum and very similar clinical features. CASE PRESENTATION: We report a case with a clinical diagnosis of Hay Wells syndrome (ankyloblepharon, ED and CL/P), who is a descendent of a mother with Bowen Armstrong syndrome (ED, CL/P, mental retardation). CONCLUSION: Due to the clinical similarities, we suggest that Hay Wells syndrome and Bowen Armstrong syndrome may be the same clinical entity with variable manifestations. This case highlights the difficulties in trying to classify the ED syndromes on clinical features.

Romanian experience in child celiac disease diagnosis.

Last consensus in celiac disease in 2008 conducted under the aegis of the European Society of Pediatric Gastroenterology, Hepatology and Nutrition jointly with North American Society of Pediatric Gastroenterology, Hepatology and Nutrition reveals the following: "celiac disease is a chronic immune-mediated enteropathy characterized by sensitization to gluten. That can affect any organ or system, with a wide range of clinical manifestations of variable severity". Thus, in recent years, clinical picture of celiac disease has changed the old paradigm--bowel disease with villous atrophy and malnutrition, being replaced with the new paradigm--multi-organ autoimmune disease, affecting many organs and systems throughout but with more less specific symptoms, which undiagnosed leads to delayed diagnosis, at a late-onset disease and long-term major complications as the risk of cancer. According to this consensus "the serological diagnosis of celiac disease is based on high sensitivity and specificity tests", but in line with changing clinical features of celiac disease, its diagnosis has undergone significant changes in recent years. These changes in the diagnosis of celiac disease, we have decided to analyze them.

The role of Power Doppler ultrasonography in comparison with biological markers in the evaluation of disease activity in Juvenile Idiopathic Arthritis.

OBJECTIVE: To evaluate the performance of Power Doppler Ultrasonography (PDUS) compared with biological markers, in the assessment of disease activity in children with Juvenile Idiopathic Arthritis (JIA). METHODS: Forty hospital visits were studied comprising 32 patients with JIA, during one year of follow-up. Each patient underwent clinical, laboratory and ultrasound (PDUS) evaluation. The physician global assessment score on the visual analog scale (PhGA) was used as a standard for assessing disease activity, based on previous studies. The PDUS signal was scored according to a semiquantitative four grade scale (0-3). RESULTS: PDUS assessment of synovial vascularisation was more sensitive than erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) in identification of the active disease: 90.4% vs. 57% and 28.5% respectively. CRP had a higher specificity (94%) in comparison with PDUS (89.5%). A significant association between clinical examination (PhGA) and PDUS score or ESR was found. Kappa statistics revealed a high level of agreement between PhGA and PDUS score (k=0.799) and a low level of agreement between PhGA and biological markers (k=0.356 and k=0.225 respectively). Patients with higher PDUS score (>or=2), ESR>or=30 mm/h or CRP>or=2 mg/dl were more likely to have active disease. CONCLUSION: Laboratory tests used today are not sufficiently sensitive for the prediction of active disease. PDUS assessment of synovial vascularisation is a technique with good sensitivity and specificity, thus it may be a beneficial criteria for evaluating disease activity in JIA, completing conventional clinical examination.

Controversies in the laboratory diagnosis of celiac disease in children; new haplotypes discovered.

The latest consensus on celiac disease in 2008, under the auspices of the International Societies of Pediatric Gastroenterology, Hepatology and Nutrition, shows that HLA DQ2/DQ8 typing indicates the highest negative predictive value for celiac disease, which would exclude the diagnosis of celiac disease. In Romania, there are no studies on the implication of HLA-DQ2/DQ8 in celiac disease in children. The aim of our study was to analyze the significance of genetic tests, with a focus on negative HLA-DQ2/DQ8 cases, as well as to determine the main haplotypes involved in celiac disease in children. We tested in 37 children with old celiac disease, confirmed based on the presence of intestinal villi changes on duodenal biopsy, the IgA anti-tissue transglutaminase antibodies (TgA-IgA) by ELISA and the IgA anti-endomysium antibodies (EmA-IgA) by indirect immunofluorescence, compared to HLA-DQ2/DQ8 typing by polymerase chain reaction (PCR). In 25 children, the determined HLA haplotypes predominantly belonged to DQ2, and in 3 children we report the presence of a new haplotype, DR3-DQ2/DR4-DQ8, formed by pattern 1, DR3-DQ2-the DQA1*0501 and DQB1*0201 alleles, and pattern 5, DR4-DQ8-the DQA1*0301 and DQB1*0302 alleles. In 9 children, genetic tests were negative for celiac disease. The identification of HLA-DQ2/DQ8 provides additional data in the diagnosis of celiac disease, but a rigid algorithm in the diagnosis of celiac disease has no practical applicability.

IgG-F-actin antibodies in celiac disease and dermatitis herpetiformis.

Anti-actin antibodies are found in 52-85% of patients with autoimmune hepatitis or chronic active hepatitis and in 22% of patients with primary biliary cirrhosis. In patients with celiac disease, anti-actin antibodies correlate with the degree of villous atrophy. Studies on their involvement in celiac disease and dermatitis herpetiformis in Romania have not been done. The purpose of this study was to evaluate of the quality of IgG anti-F-actin antibodies (IgG-AAA) tests compared with IgA tissue transglutaminase antibodies (IgA-TgA) having IgA endomysial antibody (IgA-EmA) as gold standard in celiac disease and dermatitis herpetiformis and to see if there is any relationship between them. The study included 70 pediatric patients with celiac disease under gluten-free diets and 10 adult patients with dermatitis herpetiformis, during 2010. The IgG-AAA antibodies levels were determined by ELISA. Assessing the qualities of IgG-AAA compared to IgA-TgA, we obtained the following values sensitivity (Se) 27.8%, specificity (Sp) 79.4%, respectively Se 88.9%, Sp 79.4% in celiac disease and Se 33.3%, Sp 100%, respectively Se 100%, Sp 100% in dermatitis herpetiformis. Also, there was a prevalence of 24.3% and 30% of IgG-AAA in the two groups of patients, but no statistically significant associations were found. Therefore, we concluded that IgG-AAA can not replace IgA-TgA in children patients with celiac disease under gluten-free diets and in adult patients with dermatitis herpetiformis. AAA-IgG serum activity in both diseases exist, but without a relationship of association with them.

The long-term evolution of chronic hepatitis B acquired in childhood.

UNLABELLED: The AIM of this study was to assess the long-term evolution of chronic hepatitis B acquired in childhood. METHODS: The study was carried out in 2007 - 2008 on a group of 77 adult patients who were diagnosed with chronic hepatitis B in childhood. The actual assessment included epidemiological, clinical, biological and virological data, ultrasound examination in all patients and liver histology in 3 patients. RESULTS: From the 77 patients, 69 were HBeAg positive and the other 8 patients were anti-HBe positive when the diagnosis was made in their childhood. Thirty-seven patients from the HBeAg positive group and 2 patients from the anti-HBe group had been treated in childhood with IFN-alpha and the other 38 patients remained untreated (32 patients with HBeAg positive and 6 patients anti-HBe positive). Overall, 78.26% seroconverted to anti-HBe (87.50% untreated and 70.27% of patients treated with IFN). After a median follow-up period of 13 years, 36 patients from the HBeAg positive group (48.65% of treated patients and 56.25% of untreated ones) became inactive carriers. Seroconversion to anti-HBs, in the HBeAg positive group, occurred in 10.14% of cases (8.1% in treated patients) without statistical significance. Three patients from the whole group developed cirrhosis but none developed hepatocellular carcinoma. CONCLUSION: The long-term outcome in our patients with CHB acquired in childhood did not differ between treated and untreated patients.

Inferior mesentericocaval shunt--an efficient therapeutical alternative in Budd Chiari syndrome associated with portal and splenic vein thrombosis in a teenager.

The authors present the case of a 17 year old girl admitted to hospital for poor general state, mild scleral jaundice, deficient nutritional state, oliguria and massive ascites. She was diagnosed with Budd-Chiari syndrome: thrombosis of the left suprahepatic vein and nonocclusive thrombosis of the inferior vena cava at the level of the 12th thoracal and the lumbar vertebrae. The specific feature of the case was the association of portal and splenic vein thrombosis. A mesentericocaval shunt with external jugular grefon was performed. The evolution at 20 months after surgery has been favorable. She has no ascites, the nutritional state has normalized and hepatic laboratory findings have returned to normal values. There still persists a high consistency splenomegaly, but without hematological hypersplenism. Even though the mesentericocaval shunt is not without complications, it represents an efficient alternative for the treatment of Budd-Chiari syndrome, when endovascular techniques are not available.