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.

Analysis of kinetic and operational parameters in a structured model for acrylic acid production through experimental design.

In biotechnological processes, a great number of factors can influence the income productivity and conversion. Normally, it is not evident which of these factors are the most important and how they interact. In this work, multivariate analysis techniques are used as experimental design coupled to a detailed deterministic model to identify the parameters with the most significant impact on the model to represent well the acrylic acid production process. It is proposed as an alternative process, having sugarcane as feedstock, to the petrochemical-based ones that have significant environmental impacts for their production. To increase the competitiveness of renewable acrylic-acid-based process, it is necessary to find out working conditions near the optimal region, which is not an easy task, as the process is multivariable and non-linear. The mapping of the dynamics of the developed process is made using techniques of factorial design together with the methodology of Plackett-Burman. It is shown that it is possible to increase the process performance by choosing optimized conditions for the reactor operation.

Risk factors of childhood asthma in Sao Jose do Rio Preto, Sao Paulo, Brazil.

BACKGROUND: Epidemiological studies have shown increased prevalence of asthma over the last decades and a considerable variation in these rates between different countries, mainly developed. Changes in lifestyle of different populations could explain the increasing prevalence of asthma and its different rates in different communities. OBJECTIVES: A case-control study was conducted to investigate some risks factors associated to asthma in schoolchildren in São José do Rio Preto, São Paulo, Brazil. CASUISTIC AND METHODS: A total of 3793 self-administered written questionnaire (WQ) was filled in by parents of 6-7-year-old schoolchildren, applying the International Study of Asthma and Allergies in Childhood (ISAAC) protocol. Additionally, was added a supplementary WQ evaluating risk factors: gender, personal history of rhinitis and atopic eczema, familial history of atopy, older siblings, day care, breastfeeding, and exposure to pets and to cigarette smoking. Children whose parents responded affirmatively to question 'Has your child had wheezing in the past 12 monthsquest;' were identified as having current asthma. RESULTS: The prevalence of current asthma was 18.3%. In multivariate analysis the following risk factors were associated with asthma: rhinitis (OR = 10.6; 95% CI = 8.27-13.56), atopic eczema (OR = 1.54; 95% CI = 1.00-2.35), father with asthma (OR = 3.49; 95% CI = 1.83-6.67), mother with asthma (OR = 1.84; 95% CI = 1.11-3.05), current exposure to pets (OR = 1.83; 95% CI = 1.45-2.32) and passive tobacco smoking exposure (OR = 1.43; 95% CI = 1.12-1.83). CONCLUSIONS: Our results show that genetic background, environmental factors and the presence of co-morbidities such as rhinitis and atopic eczema are associated to the genesis of asthma in Brazilian schoolchildren.

A new process for acrylic acid synthesis by fermentative process.

With the synthesis of chemical products through biotechnological processes, it is possible to discover and to explore innumerable routes that can be used to obtain products of high added value. Each route may have particular advantages in obtaining a desired product, compared with others, especially in terms of yield, productivity, easiness to separate the product, economy, and environmental impact. The purpose of this work is the development of a deterministic model for the biochemical synthesis of acrylic acid in order to explore an alternative process. The model is built-up with the tubular reactor equations together with the kinetic representation based on the structured model. The proposed process makes possible to obtain acrylic acid continuously from the sugar cane fermentation.

Reduction in the oral doses of theophylline in asthmatic children during concomitant treatment with ketotifen.

Sixteen children with moderate or severe bronchial asthma underwent a double-blind study receiving either ketotifen (K) or placebo (P) in order to verify the sparing effect of K on theophylline. The study lasted 18 weeks. Slow release theophylline was administered to all patients at a dose of 300 mg every 12 hours for the first six weeks of the study ("washout"). From the seventh week on, eight patients received K (1 mg, 2x/day) and the others P. The theophylline dose was maintained and reduced every 4 weeks to 200 mg and 100 mg. The initial clinical characteristics were identical in both groups. The serum levels of theophylline decreased below the ideal range (less than 10 mcg/ml) in groups K and P with a 100 mg dose of theophylline. However, in group K patients, fewer reported worsening of symptoms (number of days with cough and/or wheeze, altered daytime or nighttime peak expiratory flow rate and consumption of beta agonist) when compared to group P. Increased PC20 of methacholine only occurred in group K patients.

[Familial Bloom's syndrome associated with neuroblastoma]. / Síndrome de Bloom familiar associada a neuroblastoma.

Bloom's syndrome (BS) is an autosomal recessive disease characterized by short stature, sensitivity to sunlight, and telangiectasic malar erythema. It is associated to chromosomal breakage, to primary combined immunodeficiency, and to a high incidence of neoplasias. The authors report the case of two siblings with BS and associated immunodeficiency. Both patients were male and 5 (A) and 4 (B) years old at the time of diagnosis. Chronic diarrhea, recurrent otitis media, purulent rhinitis, conjunctivitis and pyodermatitis were reported by patient A. Patient B was admitted with diagnosis of bilateral neuroblastoma and had the tumor resected. Later on, he presented with oral moniliasis, herpetic stomatitis, and skin abscesses. This patient did not have recurrent infections. Immunological evaluation showed normal serum levels of CH50, C3, and C4 for both patients. Serum IgG, IgA, IgM, and salivary IgA levels were: 455 mg/dl, 15mg/dl, 20mg/dl, 0.6mg/dl for A, and 400mg/dl, 15mg/dl, 20mg/dl, and 0.2mg/dl for B, respectively. Serum antipolio antibodies (1, 2, and 3) were normal, and low levels of isohemagglutinins were observed in both patients. T cells subset determination showed: patient A--OKT3 = 66%, OKT4 = 33%, OKT8 = 32%, and 4/8 ratio = 1.0; patient B--OKT3 = 70%, OKT4 = 32%, OKT8 = 34%, and 4/8 ratio = 1.0. In vitro cellular immune response to PHA was depressed only in patient B. Patients karyotype showed chromosomal breaks with sister chromatid exchanges. Neither patient had abnormal alphafetoprotein and carcinoembryonic antigen serum levels. The rarity of such associations justifies the presentation of the cases.

Unilateral exophthalmos in orbital echinococciasis.

A case of hydatic cyst located in the right orbit and diagnosed by computed tomography is reported.