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Life-long hepatitis B immunoglobulin (HBIG) administration is a main component of prophylactic strategy to prevent hepatitis B virus (HBV) reinfection after liver transplantation (LT

Background Hereditary angioedema (HAE) is rare and still underdiagnosed in some countries. We aimed to describe HAE diagnosis and sociodemographic and clinical features in patients with HAE due to C1 inhibitor deficiency (HAE-C1-INH) followed up at a tertiary-level center in Rio de Janeiro, Brazil.Methods A descriptive, cross-sectional study with prospective data collection of 138Brazilian patients with HAE was performed. From the total, 107 patients with HAE-C1-INH were selected. Data were assessed based on a specific questionnaire. ResultsOne hundred and five patients had HAE type I (76.1%), and two had HAE type II (1.4%). Seventy-two were female (67.3%), and 35 were male (32.7%). Mean age was 38.0 AE 15.0 years (range: 12-73 years). A long delay (17.7 AE 12.6 years) until diagnosis was observed. About 86.9% had a familial history. Cutaneous edema (95.8%), abdominal pain (88.5%), and laryngeal edema (65.6%) were the most frequent symptoms. Triggering factors (95.8%) and prodromal symptoms (47.9%) were referred. Attacks were severe in 55.1% and moderate in 24.3%. Eleven (10.3%) were asymptomatic. HAE attacks were more frequent and severe (P = 0.021) in females. ConclusionsWe observed a considerable delay in diagnosis, even with familial history.The severity of HAE attacks, especially in females, highlights the need for an awareness of disease by gynecologists and obstetricians. Screening of familial members, including asymptomatic individuals, is critical for earlier diagnosis. Regional evaluation of patient profiles can be helpful to draw more attention about HAE and to improve quality of life.

bSome reports have demonstrated an inadequate response to hepatitis B vaccination in patients affected by celiac disease. The aim of our study was to evaluate hepatitis B vaccination response in relation to gluten exposure status in patients with celiac disease. To measure the gluten exposure status at the time of vaccination, we considered three groups: group A (exposed to gluten), including patients vaccinated as 12-year-old adolescents (the celiac disease diagnosis was established after vaccination); group B (not exposed to gluten), including patients vaccinated as 12-year-old adolescents on a gluten-free diet at the time of vaccination; and group C (infants), including patients vaccinated at birth. The response of celiac patients to hepatitis B vaccination was compared to that of healthy subjects, i.e., those in the control group (group D). This study included 163 celiac patients (group A, 57 patients; group B, 46 patients; and group C, 60 patients) and 48 controls (group D). An inadequate response to hepatitis B immunization was present in 43.9% of patients in group A, 34.8% of patients in group B, 58.3% of patients in group C, and 8.3% of patients in group D (group A versus group D, P < 0.001; group B versus group D, P ‫؍‬ 0.002; group C versus group D, P ‫؍‬ 0.001) (no significant difference for group A versus group B and group A versus group C was evident). Our data suggest that gluten exposure does not influence the response to hepatitis B immunization and that the human leukocyte antigen probably plays the main immunological role in poor responses to hepatitis B-vaccinated celiac patients. Several studies have reported an inadequate response to hepatitis B virus (HBV) vaccination in patients affected by celiac disease (CD). Unfortunately, the causes of this impaired response are unknown (1-8).In the general population, it is recognized that several factors influence the production of protective levels of antibodies against HBV after the standard immunization. Well-known modifiers include age, obesity, smoking, drug abuse, alcoholism, infections, immune suppression, and the route of vaccination (9, 10). Additionally, hepatitis B vaccine nonresponsiveness, due to the presence of specific human leukocyte antigen (HLA) genotypes, has been described (11-13).Celiac disease (CD) is an HLA-associated immunological disease, and for this reason, a genetic predisposition as a possible cause of a lower grade of immunization to recombinant hepatitis B vaccines has been considered (4, 5). In fact, HLA-DQ2 status may predispose CD patients to fail to develop immunity after hepatitis B vaccination through a Th2 response that is inadequate for B-cell differentiation and the formation of memory B cells (5). In contrast, several studies have hypothesized gluten intake as a cause of failed immunity at the time of vaccination. Gluten might be implicated because both hepatitis B surface antigen (HBsAg) protein fragments and gliadin peptides bind to HLA-DQ2 molecules and induce proliferation of T lymphocytes. Competition bet...

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To evaluate the impact of pregnancy on bone, we studied bone turnover at the first (T1) and third (T3) trimester of gestation in 58 adolescents and 28 healthy adolescents who had never been pregnant. Total body (TB) and lumbar spine (LS) bone mineral density (BMD) and body composition were evaluated by dual-energy X-ray absorptiometry in all control patients (C) and after parturition in 28 pregnant patients (G). Paired and unpaired t tests, Mann-Whitney and Pearson correlation tests were used. Bone turnover markers were above the reference range for adult women in more than 80% of the adolescents, with no difference between C and G patients at T1. Increase in urinary N-telopeptide crosslinks of type I collagen and serum bone-specific alkaline phosphatase, markers of bone turnover, was seen during pregnancy ( p < 0.0001). Body composition did not differ between groups, but LS BMD, percentage of expected LS BMD, LS Z-score, percentage of expected TB BMD and TB Z-score were lower in G than C patients ( p < 0.05). TB BMD was positively correlated with LS BMD (r2 = 0.52). The inverse correlations between bone markers and LS BMD suggest that the increased bone turnover during pregnancy probably explains the low bone density after parturition. The impact on future peak bone mass must be studied.