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Objective: To provide cutting-edge information on the impact and risks of using Electronic Nicotine Delivery Systems (ENDS) by children and adolescents, based on the latest evidence published in the literature., Data Source: A comprehensive search was carried out on PubMed, using the expressions ''electronic cigarettes'' OR ''electronic nicotine delivery systems" OR "vaping" AND ''adolescent'' AND "risks" AND ''acute lung injury'. All retrieved articles had their titles and abstracts read to identify and fully read the papers reporting the most recent evidence on each subject., Summary of Findings: The use of ENDS has alarmingly increased in Brazil and around the world. The possibility of customizing use, the choice of flavors and nicotine content, and the general notion that these devices are harmless when compared to conventional cigarettes are some of the factors responsible for this increase. Numerous scientific studies have proven that electronic cigarettes have serious consequences for the respiratory system, such as EVALI (E-cigarette or Vaping-Associated Lung Injury) and difficult-to-control asthma, as well as harmful effects on the neurological, cardiovascular, gastrointestinal, and immunological systems. High concentrations of nicotine make many young people addicted to this substance. In Brazil, commercialization, import, and advertising are prohibited. The viable interventions to address the use of these devices in children and adolescents are prevention and behavioral counseling., Conclusion: There is clear scientific evidence that these devices pose a risk to the physical and mental health of children and adolescents., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2024 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objective: Thymidine-dependent small-colony variants (TD-SCVs) of Staphylococcus aureus are being isolated with increasing frequency from patients with cystic fibrosis (CF). The aim of this study was to evaluate the relationship between TD-SCV isolation and pulmonary function in patients with CF, as well as to determine whether the emergence of TD-SCVs was associated with trimethoprim-sulfamethoxazole (TMP-SMX) use and with coinfection with other microorganisms., Methods: This was a retrospective case-control study including patients with CF who visited the Clinical Hospital Complex of the Federal University of Paraná, in Curitiba, Brazil, between 2013 and 2022. Demographic, clinical, and spirometric data, as well as information on TD-SCVs and other isolated microorganisms, were collected from the medical records of patients with CF and TD-SCVs (TD-SCV group; n = 32) and compared with those of a matched group of patients with CF without TD-SCVs (control group; n = 64)., Results: Isolation of TD-SCVs was positively associated with TMP-SMX use (p = 0.009), hospitalization (p < 0.001), and impaired pulmonary function (p = 0.04)., Conclusions: The use of TMP-SMX seems to contribute to the emergence of TD-SCVs, the isolation of which was directly associated with worse pulmonary function in our sample.

Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.

Objectives: Fecal calprotectin is an inflammatory marker used for monitoring intestinal diseases. It has been studied as a marker of intestinal inflammation in cystic fibrosis (CF), a multi-systemic genetic disease caused by alterations to the CFTR gene. Manifestations of the disease favor a systemic inflammation not limited to the respiratory tract, therefore, calprotectin is a non-invasive and effective diagnostic method. The aim of the study was to perform a systematic review of the literature with a qualitative synthesis of studies., Sources: The articles were selected from PubMed, Web of Science, Scielo and Lilacs., Summary of the Findings: Nine studies were selected for that qualitative synthesis, one was a randomized clinical trial, and eight were case-control or cohort designs. Most studies have indicated that calprotectin is a marker of systemic inflammation in CF and not just intestinal inflammation. Calprotectin is an aid in monitoring inflammatory bowel conditions in patients with cystic fibrosis., Conclusion: Further studies should be conducted to investigate the role of this marker in the systemic inflammation of CF., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (Copyright © 2022 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

We characterized Staphylococcus aureus small-colony variant (SCV) strains isolated from cystic fibrosis (CF) patients in southern Brazil. Smaller colonies of S. aureus were isolated from respiratory samples collected consecutively from 225 CF patients from July 2013 to November 2016. Two phenotypic methods-the auxotrophic classification and a modified method of antimicrobial susceptibility testing-were employed. PCR was conducted to detect the mecA , ermA , ermB , ermC , msrA , and msrB resistance genes. Furthermore, DNA sequencing was performed to determine the mutations in the thyA gene, and multilocus sequence typing was used to identify the genetic relatedness. S. aureus strains were isolated from 186 patients (82%); suggestive colonies of SCVs were obtained in 16 patients (8.6%). The clones CC1 (ST1, ST188, and ST2383), CC5 (ST5 and ST221), and ST398 were identified. Among SCVs, antimicrobial susceptibility testing showed that 77.7% of the isolates were resistant to multiple drugs, and all of them were susceptible to vancomycin. mecA (2), ermA (1), ermB (1), ermC (3), and msrB (18) were distributed among the isolates. Phenotypically thymidine-dependent isolates had different mutations in the thyA gene, and frameshift mutations were frequently observed. Of note, revertants showed nonconservative or conservative missense mutations. SCVs are rarely identified in routine laboratory tests. IMPORTANCE Similar findings have not yet been reported in Brazil, emphasizing the importance of monitoring small-colony variants (SCVs). Altogether, our results highlight the need to improve detection methods and review antimicrobial therapy protocols in cystic fibrosis (CF) patients.

Background: Small-colony variants (SCVs) are a morphologic subtype of Staphylococcus aureus that may occur through several mechanisms including auxotrophism for thymidine, hemin, or menadione. Auxotrophic SCV for thymidine fail to synthesize DNA specifically because of mutations in the thymidylate synthase gene. We isolated S. aureus thymidine-dependent SCVs (TD-SCV) from blood and respiratory samples of a pediatric patient with cystic fibrosis and pulmonary exacerbation., Methods: Nutritional dependence of SCVs on hemin, menadione, and thymidine was evaluated. Antimicrobial susceptibility testing was performed through broth microdilution. Polymerase chain reaction was carried out for mecA, ermA, ermB, ermC, msrA, and msrB resistance genes. DNA sequencing was used to determine mutations in thyA and the multilocus sequence typing to identify genetic relatedness., Results: Methicillin-sensitive S. aureus with normal and TD-SCV phenotypes were isolated from respiratory samples and a TD-SCV phenotype was isolated from blood culture. Macrolides resistance was attributed to ermC and msrB genes. All isolates belonged to ST398. The thyA gene in S. aureus is 957 nucleotides in length and encodes a protein of 318 amino acids. The TD-SCV isolates carried a -2 nt frameshift mutation (delta 667GC668) in thyA, creating a stop codon at residue 222 close to the predicted binding site for deoxyuridine monophosphate., Conclusions: The pathogenesis of SCVs is complex and not fully elucidated. Factors inherent to the patient such as physiological conditions, recurrent infections, or coinfection should be considered. Although SCVs are considered less virulent, they showed the ability to invade and cause bacteremia in the patient., (© 2020 Wiley Periodicals, Inc.)

Objective: To verify the relationship between polymorphisms of the vitamin D receptor gene (VDR), clinical findings, and serum vitamin D (VD) levels in asthmatics., Methods: A cross sectional study of 77 children aged 7 to 14 years old, who were attended at a specialized clinic. The children were divided into 3 groups: asthmatics who had been using inhaled corticosteroids (ICS) for more than one year; asthmatics who had not been using ICS; non-asthmatics, and children without allergies (according to the International Study of Asthma and Allergies in Childhood -- ISAAC). Spirometry, skin prick tests, the presence of a VDR promoter CDX2 polymorphism from an allele-specific polimerase chain reaction (PCR), exons 2 and 3 polymorphisms genotyping by PCR-SSCA (single-strand conformational analysis), total immunoglobulin E (IgE) and specific IgE to mites and grass were evaluated in these three groups. Levels of 25-hydroxyvitamin D were determined in asthmatics only., Results: The mean age of the children was 10.8±2.0 years old, 57% were male, 38 were asthmatic and using ICS, 22 were asthmatic and not using ICS, and 17 were non-asthmatic. Allergic rhinitis was present in 90% of asthmatics. Homozygous CDX2 was detected in 23% of the patients and absent in the control group (p=0.03). Lower forced expiratory volume in 1 second (FEV1%) values were observed in CDX2 homozygous asthmatics (p=0.001). Variations in the exon 2 and 3 sequences were not related to asthma or the other tests. VD deficiency or insufficiency was detected in 98% of asthmatics. There was no association between VD levels and genetic polymorphisms from exons 2 and 3., Conclusions: There was a positive association between homozygous CDX2 polymorphism, asthma and lower FEV1% values. CDX2 is capable of modifying cell interaction between VDR and VD, and it could be associated with the prevalence of asthma, and the difficulty in controlling the disease.

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions. RESUMO A fibrose cística (FC) é uma doença genética autossômica recessiva caracterizada pela disfunção do gene CFTR. Trata-se de uma doença multissistêmica que ocorre mais frequentemente em populações descendentes de caucasianos. Nas últimas décadas, diversos avanços no diagnóstico e tratamento da FC mudaram drasticamente o cenário dessa doença, com aumento expressivo da sobrevida e qualidade de vida. Atualmente, o Brasil dispõe de um programa de ampla cobertura para a triagem neonatal de FC e centros de referência distribuídos na maior parte desses estados para seguimento dos indivíduos. Antigamente confinada à faixa etária pediátrica, tem-se observado um aumento de pacientes adultos com FC tanto pelo maior número de diagnósticos de formas atípicas, de expressão fenotípica mais leve, assim como pelo aumento da expectativa de vida com os novos tratamentos. Entretanto, ainda se observa uma grande heterogeneidade no acesso aos métodos diagnósticos e terapêuticos para FC entre as diferentes regiões brasileiras. O objetivo dessas diretrizes foi reunir as principais evidências científicas que norteiam o manejo desses pacientes. Um grupo de 18 especialistas em FC elaborou 82 perguntas clínicas relevantes que foram divididas em cinco categorias: características de um centro de referência; diagnóstico; tratamento da doença respiratória; tratamento gastrointestinal e nutricional; e outros aspectos. Diversos profissionais brasileiros atuantes na área da FC foram convidados a responder as perguntas formuladas pelos coordenadores. A literatura disponível foi pesquisada na base de dados PubMed com palavras-chave, buscando-se as melhores respostas às perguntas dos autores.

Serpentine and amphibole asbestos occur naturally in certain geologic settings worldwide, most commonly in association with ultramafic rocks, along associated faults. Ultramafic rocks have been used in Piên County, Southern Brazil for decades for the purpose of road paving in rural and urban areas, but without the awareness of their adverse environmental and health impact. The aim of this study was the chemical characterization of aerosols re-suspended in two rural roads of Piên, paved with ultramafic rocks and to estimate the pulmonary deposition of asbestos aerosols. Bulk aerosol samples were analyzed by means of X-ray fluorescence spectrometry and X-ray diffraction analysis, in order to characterize elemental composition and crystallinity. Single-particle compositions of aerosols were analyzed by computer-controlled electron-probe microanalysis, indicating the presence of a few percentages of serpentine and amphibole. Given the chemical composition and size distribution of aerosol particles, the deposition efficiency of chrysotile, a sub-group of serpentine, in two principal segments of the human respiratory system was estimated using a lung deposition model. As an important finding, almost half of the inhaled particles were calculated to be deposited in the respiratory system. Asbestos depositions were significant (∼25 %) in the lower airways, even though the selected breathing conditions (rest situation, nose breathing) implied the lowest rate of respiratory deposition. Considering the fraction of inhalable suspended chrysotile near local roads, and the long-term exposure of humans to these aerosols, chrysotile may represent a hazard, regarding more frequent development of lung cancer in the population of the exposed region.

Cystic fibrosis (CF) patients with Burkholderia cepacia complex (Bcc) pulmonary infections have high morbidity and mortality. The aim of this study was to compare different methods for identification of Bcc species isolated from paediatric CF patients. Oropharyngeal swabs from children with CF were used to obtain isolates of Bcc samples to evaluate six different tests for strain identification. Conventional (CPT) and automatised (APT) phenotypic tests, polymerase chain reaction (PCR)-recA, restriction fragment length polymorphism-recA, recA sequencing, and matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) were applied. Bacterial isolates were also tested for antimicrobial susceptibility. PCR-recA analysis showed that 36 out of the 54 isolates were Bcc. Kappa index data indicated almost perfect agreement between CPT and APT, CPT and PCR-recA, and APT and PCR-recA to identify Bcc, and MALDI-TOF and recA sequencing to identify Bcc species. The recA sequencing data and the MALDI-TOF data agreed in 97.2% of the isolates. Based on recA sequencing, the most common species identified were Burkholderia cenocepacia IIIA (33.4%),Burkholderia vietnamiensis (30.6%), B. cenocepaciaIIIB (27.8%), Burkholderia multivorans (5.5%), and B. cepacia (2.7%). MALDI-TOF proved to be a useful tool for identification of Bcc species obtained from CF patients, although it was not able to identify B. cenocepacia subtypes.

The predisposition of patients with cystic fibrosis (CF) for recurrent pulmonary infections can result in poor prognosis of the disease. Although the clinical significance in CF of micro-organisms, such as Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa, is well established, the implication of uncommon glucose non-fermenting Gram-negative bacilli (UGNF-GNB) in respiratory samples from CF patients is still unclear. Because of limitations of traditional methods used in most clinical laboratories, the accurate identification of these microbes is a challenge. Matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) is an alternative tool for efficient identification of bacteria. This was a retrospective study to evaluate different identification methods in a collection of UGNF-GNB isolated from children with CF during a period of three years. The performance of MALDI-TOF was compared to that of 16S rDNA gene sequencing and to a conventional and automated phenotypic identification. The discriminatory power of MALDI-TOF (75.0 % agreement) was superior to automated techniques (67.1 % agreement) and to conventional phenotypical identification (50.0 % agreement). MALDI-TOF also demonstrated high accuracy in identifying Stenotrophomonas maltophilia, Achromobacter xylosoxidans and Chryseobacterium indologenes, but had limited utility in identifying Pandoraea spp. and some species of Acinetobacter and Chryseobacterium (other than C. indologenes). Although MALDI-TOF identified only 75 % of the isolates in comparison with 16S rDNA gene sequencing, the prompt identification and high discriminatory power exhibited by MALDI-TOF make it a useful tool for the characterization of micro-organisms that are difficult to identify using routine methods., (© 2014 The Authors.)

Objective: this study aimed to prepare a silkworm moth (Bombyx mori) antigenic extract and to perform skin prick tests with this extract in patients with allergic respiratory diseases; to evaluate serum specific immunoglobulin E (IgE) to Bombyx mori using ImmunoCAP® system and to report the frequency of positivity between the two methods and with clinical data., Methods: this was a cross-sectional study with 99 children and adolescents diagnosed with asthma and/or allergic rhinitis, who had skin reactivity to at least one of the six aeroallergens tested. Clinical data were evaluated: skin prick tests with Bombyx mori in-house extract, and total and specific IgE analysis using ImmunoCAP® were performed., Results: the frequency of Bombyx mori specific IgE was found to be 52.5% and 60% using the skin prick test and ImmunoCAP®, respectively. An association between a positive skin test for Bombyx mori and the presence of allergic rhinitis, atopic dermatitis, and urticaria was observed, but the same was not true for asthma or allergic conjunctivitis. There was no relation with the severity of asthma or rhinitis symptoms., Conclusions: a high frequency of sensitization to Bombyx mori was observed in a selected population of patients with respiratory allergic diseases in the city of Curitiba, state of Paraná, Brazil. The extract prepared from the wings of this moth species is effective in demonstrating this sensitivity., (Copyright © 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Our objective was to evaluate the reproducibility of Asthma Control Questionnaire 7 (ACQ-7) in asthma patients, comparing our results against those obtained with the Global Initiative for Asthma (GINA) criteria. We evaluated 52 patients. Patients completed the ACQ-7, underwent spirometry, and were clinically assessed to determine the level of asthma control according to the GINA criteria, in two visits, 15 days apart. The ACQ-7 cutoff for uncontrolled asthma was a score of 1.5. The ACQ-7 showed good reproducibility, with a correlation coefficient of 0.73. The ACQ-7 identified a greater number of patients with uncontrolled asthma than did the GINA criteria; according to the GINA criteria, 47 patients (90.4%) presented with partially controlled asthma.

Objective: The prevalence of allergic conjunctivitis (AC) has not been established. Estimates suggest that ocular allergies affect 15% to 20% of the worldwide population, yet most epidemiological studies encompass nasal and ocular allergy symptoms and have not been specific to AC. The aim of this study was to verify the prevalence of ocular allergy symptoms, co-morbidities, and their impact on adolescents., Methods: Adolescents were selected from a sample of schools, and completed in classrooms a previously validated questionnaire on symptoms of AC. AC diagnosis was considered when more than three episodes of ocular itching were reported in the past 12 months. Related symptoms such as tearing, photophobia, foreign body sensation, impact on daily activities, and diagnosis of AC were analyzed., Results: Questionnaires were obtained from 3,120 adolescents (mean age 13.3±1.1 years). Ocular itching in the past 12 months occurred in 1,592 (51%). The most frequently associated symptom was tearing (74%), followed by photophobia (50.1%) and foreign body sensation (37.1%). The prevalence of AC was 20.7%, affecting more females than males (56.1% versus 45.9%; p= 0.01). The risks of an adolescent with ocular allergy to present asthma, rhinitis, and atopic eczema were (OR= 5.7; 95% CI: 4.5 to 7.1), (OR=3.6; 95% CI: 3.0 to 4.3), and (OR=2.6; 95% CI: 2.0 to 3.5), respectively. Severe interference in daily activities was reported by 30.5%., Conclusions: Symptoms of ocular allergy are common, frequently associated to other allergic diseases, and impact the daily activities of adolescents., (Copyright © 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objective: To determine whether nebulizers are a source of microbial contamination in patients with cystic fibrosis, as well as whether the technique and frequency of disinfection of these devices is appropriate., Methods: This was a cross-sectional, uncontrolled observational study. Samples were collected from 28 patients with cystic fibrosis. Samples were collected at the homes of the patients, who were not previously informed of the purpose of the visit. Three samples were collected from each patient: one from the nebulizer chamber, one from the mask/mouthpiece, and one from the patient (oropharyngeal swab /sputum). The samples were properly stored and taken for analyses. The patients, their parents, or their legal guardians completed a questionnaire regarding nebulizer cleaning and disinfecting methods., Results: We collected 84 samples from the 28 patients. Of those 28 patients, 15 (53.5%) were male. The median age of the patients was 11 years (range, 1-27 years). Of the 28 patients, 15 presented with positive oropharyngeal swab /sputum sample cultures. The most common bacterial isolates were Staphylococcus aureus (in 8 patients) and Pseudomonas aeruginosa (in 4 patients). Although the samples obtained from the nebulizers presented with various pathogens in culture, no specific species predominated. In 27 cases (96.7%), there were no associations between the samples obtained from the nebulizers and those obtained from the patients in terms of the results of the cultures. Cleaning and disinfection of nebulizers were inappropriate in 22 cases (78.6%)., Conclusions: In this sample of patients, despite the inappropriate disinfection techniques, nebulizers were not found to be a source of microbial contamination.

Objectives: To assess bacterial colonization prospectively in patients with cystic fibrosis identified by neonatal screening. To assess susceptibility to antimicrobials and to perform the molecular typing of Staphylococcus aureus strains isolated from the oropharynx of patients during the study., Methods: Twenty-five cystic fibrosis patients receiving regular treatment at the Cystic Fibrosis Outpatient Clinic of Hospital de Clínicas of Universidade Federal do Paraná, Brazil, were included in the study. All patients were identified by trypsin-like immunoreactivity and their diagnosis was confirmed by two or more sweat tests. Oropharyngeal swabs were collected and cultured according to routine methods; bacterial colonies were phenotypically identified and their susceptibility to antimicrobials was tested. S. aureus isolates were submitted to molecular typing using pulsed-field gel electrophoresis., Results: Out of 234 oropharyngeal swabs, S. aureus was the most frequently isolated strain (76% of patients, 42% of swabs), followed by Pseudomonas aeruginosa (36% of patients, 16% of swabs) and Haemophilus spp. (76% of patients; 19% of swabs). Seventy-three isolates were obtained from 19 patients colonized with S. aureus, of which 18 were oxacillin-resistant (24.6%), isolated from two patients, with the same electrophoretic profiles as that of the Brazilian clone. The remaining oxacillin-sensitive isolates were distributed into 18 electrophoretic profiles., Conclusion: There was higher prevalence of S. aureus, with earlier isolation than other pathogens. Multi-sensitive isolates were distributed into different clones, characterizing non-transmissibility among community-acquired strains. The isolated oxacillin-resistant S. aureus showed identical electrophoretic profiles, probably acquired in hospital. P. aeruginosa was not so frequent in the studied population.

Objectives: To present and analyze the results of the National Neonatal Cystic Fibrosis Screening Program in Paraná, 30 months after its implementation., Methods: This is a descriptive study, with an analysis of the data from the screening of around 98% of all neonates in the period from September 2001 to April 2004, undertaken at the Neonatal Screening Program laboratory of the Fundação Ecumênica de Proteção ao Excepcional do Paraná. Blood samples for the Guthrie test were collected on hospital discharge, ideally between the second and sixth days postpartum, and filter papers were sent for immunoreactive trypsin assay by the immunofluorometric method. Children whose immunoreactive trypsin assay results were > or = 70 ng/ml for two distinct samples during the first 30 days of life, were referred for sweat conductivity testing by the Wescor method. In cases when the result was greater than 50 mMol/l quantitative chlorine and/or sodium in sweat was assayed (iontophoresis with pilocarpine)., Results: From a total of 456,982 tests, 4,028 (0.9%) children presented a first immunoreactive trypsin assay above the cutoff point set. Four hundred and seventy-eight of these (12.5%) also had a second blood sample assayed with immunoreactive trypsin above 70 ng/ml and 56 (11.7%) of these were referred to specialized clinics after their sweat conductivity test results were above 50 mMol/l and 48 (0.01% of the total number of children screened) had a diagnosis of cystic fibrosis confirmed. The incidence for the state of Paraná was 1:9,520, although some children have not yet been fully investigated., Conclusions: Neonatal screening for cystic fibrosis in the State of Paraná, in accordance with Health Ministry directives, was a pioneering initiative for Brazil. Many patients were diagnosed early, even asymptomatic ones, which is a challenge to improving prognosis with this fatal disease.

Objective: We sought to assess changes in the prevalence of asthma and other allergic diseases in schoolchildren of Curitiba between 1995 and 2001., Methods: Schoolchildren (aged 13-14) were evaluated by a self-reported written questionnaire of ISAAC protocol for symptoms of asthma and allergic diseases. The diagnoses of asthma, rhinoconjunctivitis and atopic eczema were considered according to the ISAAC protocol. Probable asthma was considered: more than 4 attacks of wheezing in the last 12 months; 1 to 3 attacks of wheezing in the last 12 months with dry cough at night, and/or with sleep disturbance due to wheezing, and/or with wheezing after exercise. The data obtained in 2001 were compared with the data obtained in 1995 for the same age group., Results: We evaluated 2,946 schoolchildren in 1995 and 3.628 in 2001. The prevalence of symptoms of asthma, probable asthma, rhinoconjunctivitis and eczema in 1995 and 2001 was 18.4% and 18.7%, 11.6% and 12.4%, 14.2% and 17.2%, 3.8% and 3.7%, respectively. There was a 23.7% increase in the prevalence of rhinoconjunctivitis symptoms (p < 0.05)., Conclusions: The prevalence of symptoms of asthma, probable asthma and eczema did not change over the past 6 years but a significant increase of the rhinoconjunctivitis symptoms was detected.

Objective: To investigate the efficacy of a single dose of inhaled budesonide as compared to oral prednisone in patients with acute asthma., Methods: Randomized double-blind, double-dummy and placebo-controlled clinical trial. Forty-nine children aged 2 to 7 years with acute asthma were randomized in three groups after receiving nebulized salbutamol (0.15 mg/kg). Group I received placebo both as tablets and nebulization, group II was treated with a single dose of oral prednisone (1 mg/kg) and inhaled placebo, and group III received a single dose of placebo tablet and nebulized budesonide (2 mg). Patients were evaluated in terms of symptom score and transcutaneous hemoglobin saturation. Nebulized salbutamol was repeated in case of increasing symptom score or lower saturation., Results: Progressive clinical improvement was observed in all three groups. However, a significant increase in hemoglobin saturation was observed after 2 hours with prednisone, 4 hours with budesonide, and 24 hours with placebo., Conclusion: A combination of single-dose nebulized budesonide and salbutamol may be as effective as oral prednisone to improve symptom severity, but the latter increases hemoglobin saturation in exacerbation of asthma.

OBJECTIVE: To correlate sweat conductivity by Wescor with quantitative sodium determination in the same sample. METHODS: We analyzed 206 sweat samples, being 31 obtained from patients with cystic fibrosis (CF). Sweat was collected by quantitative pilocarpine iontophoresis in the Macroduct system for 30 minutes. Samples were immediately determined by Sweat-Chek analyzer (Wescor) and sodium quantification with a flame photometer. RESULTS: In non-CF subjects sweat (n=175), mean conductivity and quantitative sodium were 41mmol/L (16-75mmol/l) and 36mEq/ l (12-75mEq/l) respectively. In CF subjects (n=31), mean values of conductivity and sodium were 119mmol/L (84-155mmol/l) and 113mEq/l (80 - 146mEq/l) respectively. None of CF patients showed values lower than 80mmol/L. There was a correlation between sweat conductivity and sodium determination (r= + 0.99; p<0.0001). CONCLUSION: Sweat conductivity by Wescor simplifies the analysis with lower volumes and is well correlated to sodium concentration.

OBJECTIVE: To report clinical presentation, diagnostic procedures and treatment of patients with anomalous subclavian artery. SUBJECTS: Fifteen patients with anomalous subclavian artery followed up at the Department of Pediatrics, Hospital de Clínicas da Universidade Federal do Paraná. RESULTS: Age at diagnosis varied from birth to eight years, and 10/15 were female. Nine presented with respiratory symptoms (cough, pneumonia, stridor, wheezing). Eleven had gastrointestinal symptoms (vomiting and choking). Six patients have both respiratory and digestive symptoms. Diagnosis was suggested by barium swallow in 14/15; confirmed in 10 by arteriography or helicoidal tomography with vascular reconstruction. In fourteen cases, the anomalous subclavian was at the right side and in one at the left side. Frequent associations were gastroesophageal reflux (6/15), cardiac septum defect and Down syndrome (3/15). Surgical repair was undertaken in eight, two of whom had chylothorax in the immediate post operative period. CONCLUSION: Although anomalous subclavian artery is mostly asymptomatic, it should be looked for in children with respiratory symptoms, mainly cough and choking. The chief investigation tool is barium swallow, and surgical repair is needed when symptoms are present.