Doctors' awareness concerning primary immunodeficiencies in Brazil.

BACKGROUND: PIDs are a heterogeneous group of genetic illnesses, and delay in their diagnosis is thought to be caused by a lack of awareness among physicians concerning PIDs. The latter is what we aimed to evaluate in Brazil. METHODS: Physicians working at general hospitals all over the country were asked to complete a 14-item questionnaire. One of the questions described 25 clinical situations that could be associated with PIDs and a score was created based on percentages of appropriate answers. RESULTS: A total of 4026 physicians participated in the study: 1628 paediatricians (40.4%), 1436 clinicians (35.7%), and 962 surgeons (23.9%). About 67% of the physicians had learned about PIDs in medical school or residency training, 84.6% evaluated patients who frequently took antibiotics, but only 40.3% of them participated in the immunological evaluation of these patients. Seventy-seven percent of the participating physicians were not familiar with the warning signs for PIDs. The mean score of correct answers for the 25 clinical situations was 48.08% (±16.06). Only 18.3% of the paediatricians, 7.4% of the clinicians, and 5.8% of the surgeons answered at least 2/3 of these situations appropriately. CONCLUSIONS: There is a lack of medical awareness concerning PIDs, even among paediatricians, who have been targeted with PID educational programmes in recent years in Brazil. An increase in awareness with regard to these disorders within the medical community is an important step towards improving recognition and treatment of PIDs.

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.

[Risk factors, clinical and laboratory aspects of asthma in children] / Fatores de risco, aspectos clínicos e laboratoriais da asma em crianças.

OBJECTIVE: To evaluate the clinical and laboratory aspects and the risk factors associated with asthma in children treated at the Pediatric Outpatient Clinic of Hospital Universitário Júlio Müller. METHODS: A case-control study including 59 asthmatic children (cases) and 104 nonasthmatic children (controls). The following factors were considered for risk analysis: parents level of education, domestic exposure to allergens, passive smoking, breast feeding, low income, and family history of allergy. Samples of blood were collected for hemogram and to determine the total serum IgE as well as the one specific to allergens. Immediate hypersensitivity skin tests were performed with puncture for the detection of the following allergens: house dust mite, animals, molds, and cockroaches with positive (histamine) and negative controls (physiologic solution). A logistic regression model was used to calculate the odds ratio (OR) and 95% confidence intervals (95%CI) adjusted for risk factors and for confounding factors. RESULTS: Among the risk factors studied, sex, parents low level of education, low income, length of the breast feeding period, and passive smoking were not associated with the presence of asthma. The domestic exposure to allergens was similar in both groups except for the higher frequency of pets at the homes of control patients (chi-square=16.9; P<0.05). Paternal history of rhinitis was the only association with asthma (OR=3.33; 95%CI: 1.03-11.17; P<0.05). The asthmatic children presented higher frequency of positive reactions to skin tests than the controls, mainly to house dust mites: Dermatophagoides pteronyssinus (69.5%), Dermatophagoides farinae (59.3%) and Blomia tropicalis (59.3%); cockroaches: Periplaneta americana (59.3%), and cat: Felis domesticus (37.3%), with OR between 11.2-21.0; P<0.05. Eosinophilia and serum levels of total IgE were more elevated in the group of asthmatic children (P<0.05). The positivity of the specific IgE test for Dermatophagoides pteronyssinus and Blomia tropicalis was higher in the cases than in the controls (P<0.05). The multivariate analysis showed that sensitization to the allergens produced by cockroaches (OR=9.26; 95%CI: 2.59-33.4), animals (OR=3.93; 95%CI: 1.05-14.67) and house dust mites (OR=3.74; 95%CI: 1.18-11.8) were the most important risk factors for asthma. CONCLUSIONS: The sensitization to indoor allergens, mainly to house dust mites, cockroaches, and cats showed a strong association with asthma in this study.

Placental transfer of IgG and IgG subclass antibodies anti-purified Escherichia coli LPS O16, O6 and O111.

We evaluated 22 paired maternal and cord sera regarding the presence of IgG and IgG subclasses against purified Escherichia coli LPS O6, O16 and O111 employing ELISA for titre and avidity analysis, isoelectric focusing associated with affinity-blotting for spectrotypic analysis, and the Western-blotting technique for recognition of the various bands in lipopolysaccharide (LPS). Levels of anti-LPS IgG antibodies in cord sera were equivalent to their respective maternal sera, showing a significant correlation (P < 0.0001). IgG1 antibody levels were higher in cord sera than in maternal sera (P < 0.005 for anti-O111, P < 0.05 for anti-O16 and P < 0.02 for anti-O6). Cord IgG2 antibody levels were not different from the maternal levels (P > 0.1). The levels of IgG3 and IgG4 were undetectable. The avidity of anti-O6 and anti-O111 IgG in 10 cord sera showed an extremely significant correlation with maternal antibody avidity (P < 0.0001). Identical patterns of recognition were found in the paired samples analysed by Western blotting. Most of the serum samples recognized the O-repetitive chains and also the region corresponding to core and lipid A. Although the antibody spectrotypes varied among individuals, paired cord and maternal serum samples showed identical patterns. Our findings suggest the occurrence of placental transfer of IgG antibodies against LPS O6, O16 and O111, mainly involving the IgG1 or IgG2 subclasses.

[Isolation of Haemophilus aegyptius associated with Brazilian purpuric fever, of Chloropidae (Diptera) of the genera Hippelates and Liohippelates]. / Isolamento de Haemophilus aegyptius associado à febre purpúrica brasileira, de cloropídeos (Diptera) dos gêneros Hippelates e Liohippelates.

The recognition of the Brazilian purpuric fever (BPF) in 1984 led to a number of studies which showed a relation between this disease and conjunctivitis caused by Haemophilus aegyptius. The increase in cases of conjunctivitis in children associated with higher population density of eye gnats (Chloropidae: Hippelates) has been reported since last century. This phenomenon is related to the attraction that those flies show for the eyes, secretions and wounds, from where they feed on. Although there are evidences on the role of these flies in the mechanical transmission of seasonal bacterial conjunctivitis, the isolation of Haemophilus aegyptius from them in their natural habitat had not been demonstrated yet. In this study Haemophilus aegyptius associated to BPF was isolated from two pools of chloropids collected around the eyes of children with conjunctivitis which were identified as Liohippelates peruanus (Becker) and a new species Hippelates neoproboscideus.

Isolamento de Haemophilus aegyptius associado a febre purpurica brasileira de cloropideos (diptera) dos generos Hippelates e Lioheppelates / Isolation of Haemophilus aegyptius associated to brazilian purpuric fever from Hippelates and Liohippelates flies (Diptera: Chloropidae)

O reconhecimento da Febre Purpurica Brasileira (FPB), em 1984, originou uma serie de estudos que revelaram uma correlacao desta doenca com conjuntivites causadas por Haemophilus aegyptius. A associacao do aumento de conjuntivites em criancas e a maior densidade populacional de cloropideos do genero Hippelates ja havia sido verificada desde o seculo passado. Este fenomeno esta relacionado ao tropismo que estes insetos apresentam pelos olhos, secrecoes e feridas de onde se alimentam....

Comparative efficacy of oral rifampin and topical chloramphenicol in eradicating conjunctival carriage of Haemophilus influenzae biogroup aegyptius. Brazilian Purpuric Fever Study Group.

Persistent conjunctival carriage of the Haemophilus influenzae biogroup aegyptius (Hae) strain (BPF clone) responsible for Brazilian purpuric fever (BPF) has been documented. Topical chloramphenicol is routinely used to treat conjunctivitis in areas affected by BPF in Brazil. Although the BPF clone is susceptible to chloramphenicol, we observed a number of children treated with topical chloramphenicol for conjunctivitis who still developed BPF. During an investigation of an outbreak of BPF in Mato Grosso State, Brazil, we compared oral rifampin (20 mg/kg/day for 4 days) with topical chloramphenicol for eradication of conjunctival carriage of H. influenzae biogroup aegyptius among children with presumed BPF clone conjunctivitis. Conjunctival samples were taken for culture on the day treatment was initiated and a mean of 8 and 21 days later. At 8 days the eradication rates for oral rifampin and topical chloramphenicol were 100 and 44%, respectively (P = 0.003); at 21 days they were 100 and 50% (P = 0.01). Oral rifampin was more effective than topical chloramphenicol for eradication of the BPF clone and may be useful in prevention of BPF.