Severe combined immunodeficiency in Brazil: management, prognosis, and BCG-associated complications.

BACKGROUND: Severe combined immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency. The objectives of this study were to analyze the diagnosis, treatment, and prognosis of SCID in Brazil and to document the impact of BCG vaccine. METHODS: We actively searched for cases by contacting all Brazilian referral centers. RESULTS: We contacted 23 centers and 70 patients from 65 families. Patients were born between 1996 and 2011, and 49 (70%) were male. More than half (39) of the diagnoses were made after 2006. Mean age at diagnosis declined from 9.7 to 6.1 months (P = .058) before and after 2000, respectively, and mean delay in diagnosis decreased from 7.9 to 4.2 months (P = .009). Most patients (60/70) were vaccinated with BCG before the diagnosis, 39 of 60 (65%) had complications related to BCG vaccine, and the complication was disseminated in 29 of 39 (74.3%). Less than half of the patients (30, 42.9%) underwent hematopoietic stem cell transplantation (HSCT). Half of the patients died (35, 50%), and 23 of these patients had not undergone HSCT. Disseminated BCG was the cause of death, either alone or in association with other causes, in 9 of 31 cases (29%, no data for 4 cases). CONCLUSIONS: In Brazil, diagnosis of SCID has improved over the last decade, both in terms of the number of cases and age at diagnosis, although a much higher number of cases had been expected. Mortality is higher than in developed countries. Complications of BCG vaccine are an important warning sign for the presence of SCID and account for significant morbidity during disease progression.

Proficiency testing for the determination of pesticides in mango pulp: a view of the employed chromatographic techniques and the evaluation of laboratories' performance.

The proficiency testing for determination of pesticides residues in mango pulp was the third work of the partnership established by INMETRO and INCQS/Fiocruz. Three mango pulp samples were sent to each participant laboratory, two being spiked with the pesticides and one exempt of pesticides. The added pesticides were: deltamethrin, ethion, fenitrothion, malathion, and permethrin. The evaluation of the results of the homogeneity and the stability tests, as well as the determination of the assigned value was made in agreement with ISO GUIDE 35 and ISO 13528, assuming the samples were considered homogeneous and stable for the studied period. The assigned values and the standard deviation for proficiency evaluation was calculated using the robust algorithm, according to ISO 13528, and the evaluation of the results was carried through in accordance with ABNT ISO/IEC Guide 43-1. The z-score graphs and confidence ellipse was also used in the evaluation of the results. In the evaluation carried through from the values of the z-scores, 71% of the reported results were considered satisfactory based on the results found for this index. The evaluation of the analytical viability for the determination of each pesticide and of the analytical capacity of the participant laboratories was carried through. A summarized view of the chromatographic techniques and of preparation of sample used by the participant laboratories was also carried through in this work.