Lupus erythematosus tumidus induced by sex reassignment surgery

OBJECTIVE: We describe a case of sex reassignment surgery and the subsequent development of lupus erythematosus tumidus (LET). LET is characterized clinically by erythematous, succulent, edematous, nonscarring plaques in sun-exposed areas. Results of histological examination show perivascular and periadnexal lymphocytic infiltration and interstitial mucin deposition. This report emphasizes that environmental triggers, including prolonged exposure to significant doses of estrogens as part of sex reassignment surgery, may lead to the development of lupus in a nonpredisposed individual.     

Antibody kinetics among 8–10 years old respondents to hepatitis B vaccination in a low endemic country and the effect of a booster dose given 5 or 10 years later

Few data are available concerning the persistence of anti-HBs and the effect of booster doses given several years post-vaccination against hepatitis B during preadolescence. The objective of this open-labelled clinical trial was to evaluate the persistence of antibodies after vaccination with three paediatric doses of Engerix-B at the age of 8–10 years and the effect of a booster dose given 5 (Group Y5) or 10 (Group Y10) years later. Anti-HBs were measured before and one month post-primary vaccination, then 5 and 10 years later, before the booster dose, as well as one month and 1 year post-booster. The anamnestic response was defined as a ≥fourfold increase of anti-HBs post-booster (≥10 IU/L) when compared to pre-booster. Ten years post-primary vaccination, 559 of the 652 initially randomized subjects (86%) were eligible for analysis. Group Y5, 5 years post-booster results: 99% of subjects had detectable levels of antibodies and 96% a titer ≥10 IU/L. The anti-HBs GMTs decreased from 114,489 IU/L one month post-booster to 3354 IU/L 5 years later. Group Y10 results: 10 years post-primary vaccination 96% of subjects had a detectable level of anti-HBs and 85% were above the threshold of 10 IU/L. The GMTs one month post-booster were 31,030 IU/L. The challenge with a booster demonstrated an anamnestic response in 99% of subjects in group Y5 and 100% of subjects in group Y10. All subjects were anti-HBc negative. The booster doses were well tolerated. The excellent anamnestic response observed after the booster dose demonstrates the persistence of immunity in virtually all young adults vaccinated at the age of 8–10 with three paediatric doses of Engerix-B.     

Association between Helicobacter pylori concentration and the combining frequency of histopathological findings in gastric biopsies specimens

BACKGROUND: Helicobacter pylori is the most prevalent infectious agent worldwide. About 90% of patients with chronic gastritis are infected with this bacterium. Some studies have shown a association between the H. pylori concentration and the scores of gastritis activity and severity. AIM: To evaluate the association between H. pylori concentration and the combining frequency of findings on histopathological examination. METHODS: Two hundred consecutive gastric endoscopic biopsies diagnosed as chronic gastritis were retrospectively investigated. The Warthin-Starry silver stain was used to study H. pylori and the following parameters were assessed (according to the Sydney system): 1. infiltration by polymorphonuclear cells in chorio and epithelium (activity) graded as mild, moderate and marked; 2. presence of lymphoid follicles; 3. presence of intestinal metaplasia; 4. presence of regenerative cell atypias, graded as mild, moderate and marked; and 5. H. pylori concentration on the mucous covering the foveolar epithelium. RESULTS: The most frequent association was chronic gastritis and activity, regardless of H. pylori concentration. The association of five histopathological findings in the same biopsy has not occurred in the cases of higher concentration of H. pylori. CONCLUSION: Our study has not revealed any association between H. pylori concentration and an increasing in the number of histopathological findings found in the gastric mucosa. Since referring to its presence is much more important than to its concentration.     

Performance comparison between the mycobacteria growth indicator tube system and L?wenstein-Jensen medium in the routine detection of Mycobacterium tuberculosis at public health care facilities in Rio de Janeiro, Brazil: preliminary results of a pragmatic clinical trial

In view of the fact that the World Health Organization has recommended the use of the mycobacteria growth indicator tube (MGIT) 960 system for the diagnosis of tuberculosis and that there is as yet no evidence regarding the clinical impact of its use in health care systems, we conducted a pragmatic clinical trial to evaluate the clinical performance and cost-effectiveness of the use of MGIT 960 at two health care facilities in the city of Rio de Janeiro, Brazil, where the incidence of tuberculosis is high. Here, we summarize the methodology and preliminary results of the trial. (ISRCTN.org Identifier: ISRCTN79888843 [http://isrctn.org/])     

Development and multi-site validation of a new condition-specific quality of life measure for eating disorders

Background

In eating disorders (EDs) treatment, outcome measurement has traditionally focused on symptom reduction rather than functioning or quality of life (QoL). Generic QoL measures lack sensitivity for some diagnoses and many not be responsive in eating disorder patients. This article describes the development and validation of a condition-specific QoL measure for adolescents and adults with eating disorders – the Eating Disorders Quality of Life Scale (EDQLS).

Methods

Multi-source and multi-stage methods were used to develop the EDQLS, with participation of patients with EDs, their family members and ED treatment providers. Sources for domain and item development included 39 articles, 12 patient and 10 treatment provider interviews, and 31 first person narratives from the internet. Four stages of validation and pre-testing involving 17 patients, 10 family members and 18 providers reduced 233 items to 40 items in 12 domains. These items were pilot tested in 41 ED patients.

Results

The final instrument was then validated in a 12 site sample of 171 individuals aged 14–60 with EDs. All items showed good dispersion. The total raw mean score was 110 out of 200 (SD 27.6) with higher scores indicating better QoL. Internal consistency was excellent (Cronbach's alpha = .96) and subscale internal consistency ranged from alpha .36 to .79 providing evidence for a strong overall construct and some multi-dimensionality. Validity was supported by significant differences in mean EDQLS according to severity levels on the EDI-2 (F = 95.3, p <.001) and the BSI (F = 86.9, p <.001). EDQLS scores were positively associated with time in treatment (F = 4.65, p = .01) suggesting responsiveness. A strong positive association was also found between EDQLS scores and stage of change (F = 15.1 p <.001). Pearson's correlations between the EDQLS and criterion instrument scores were .71 for the SF-12 mental subscale, .61 for the QoLI and .78 for the 16D, supporting construct validity. Exploratory principal components and item response theory analyses identified only a few poor fitting items.

Conclusion

The EDQLS has promising psychometric characteristics and may be useful for evaluating ED treatment effectiveness.     

Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries

The results of hematopoietic stem cell transplant (HSCT) for primary immunodeficiency diseases (PID) have been improving over time. Unfortunately, developing countries do not experience the same results. This first report of Brazilian experience of HSCT for PID describes the development and results in the field. We included data from transplants in 221 patients, performed at 11 centers which participated in the Brazilian collaborative group, from July 1990 to December 2015. The majority of transplants were concentrated in one center (n?=?123). The median age at HSCT was 22 months, and the most common diseases were severe combined immunodeficiency (SCID) (n?=?67) and Wiskott-Aldrich syndrome (WAS) (n?=?67). Only 15 patients received unconditioned transplants. Cumulative incidence of GVHD grades II to IV was 23%, and GVHD grades III to IV was 10%. The 5-year overall survival was 71.6%. WAS patients had better survival compared to other diseases. Most deaths (n?=?53) occurred in the first year after transplantation mainly due to infection (55%) and GVHD (13%). Although transplant for PID patients in Brazil has evolved since its beginning, we still face some challenges like delayed diagnosis and referral, severe infections before transplant, a limited number of transplant centers with expertise, and resources for more advanced techniques. Measures like newborn screening for SCID may hasten the diagnosis and ameliorate patients’ conditions at the moment of transplant.     

Expression of matrix metalloproteinase-2 and metalloproteinase-9 in the skin of dogs with visceral leishmaniasis

Parasitology Research - The skin is the first organ to be infected by the parasite in canine visceral leishmaniasis. The enzyme matrix metalloproteinase (MMP) acts towards degradation of the...