ABSTRACT 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. Objective Thymidinedependent Thymidine dependent smallcolony small colony TDSCVs TD SCVs (TD-SCVs CF. . (CF) TDSCV SCV trimethoprimsulfamethoxazole trimethoprim sulfamethoxazole TMPSMX TMP SMX (TMP-SMX microorganisms Methods casecontrol case control Paraná Curitiba Brazil 201 2022 Demographic clinical data 32 64. 64 64) Results p 0.009, 0009 0.009 , 0 009 0.009) 0.001, 0001 0.001 001 0.001) 0.04. 004 0.04 04 0.04) Conclusions TDSCVs, SCVs, sample (CF 20 202 3 6 000 0.00 00 0.0 2 0.

Resumo Objetivo Verificar a eficácia da profilaxia de curto prazo para o parto vaginal ou cesáreo com inibidor de C1 derivado de plasma concentrado em mulheres grávidas. Eles devem ter angioedema hereditário e inibidor normal de C1 no plasma. Métodos Relato de caso de gestantes diagnosticadas com angioedema hereditário com inibidor de C1 normal que foram tratadas com inibidor intravenoso de concentrado de C1 para profilaxia de ataques de angioedema quando hospitalizadas para o parto. O exon 9 do gene de genotipagem do fator 12 (F12) foi realizado por sequenciamento automático em todos os pacientes. Resultados Três casos de gestantes com angioedema hereditário com nível sérico normal de inibidor de C1 são relatados. O teste genético detectou a presença de uma mutação patogênica no gene F12. Os partos ocorreram sem intercorrências e as pacientes não apresentaram sintomas hereditários de angioedema nas 72 horas seguintes. Conclusão O concentrado de inibidor de C1 pode ser útil para prevenir ataques de angioedema durante e após o parto.

Abstract Objective To verify the efficacy of short-term prophylaxis for vaginal or cesarean section childbirth with plasma-derived C1-inhibitor concentrate in pregnant women. They should have hereditary angioedema (HAE) and normal plasma C1-inhibitor. Methods Case report of pregnant women diagnosed with HAE with normal C1- inhibitor who had been treated with intravenous C1-inhibitor concentrate for prophylaxis of angioedema attacks when hospitalized for delivery. The exon 9 of the Factor 12 (F12) genotyping gene was performed by automatic sequencing in all patients. Results Three cases of pregnant women with HAE with normal serum level of C1- inhibitor are reported. The genetic test detected the presence of a pathogenic mutation in the F12 gene. Deliveries occurred uneventfully and patients had no HAE symptoms in the following 72 hours. Conclusion C1-inhibitor concentrate could be useful to prevent angioedema attacks during and after delivery.

RESUMO Anafilaxia é uma reação de hipersensibilidade generalizada ou sistêmica grave, com risco de morte, que exige atendimento rápido e correto. Este estudo teve como objetivo obter uma visão integrada do nível de conhecimento dos médicos no atendimento da anafilaxia à luz dos estudos publicados internacionalmente nos últimos 5 anos. Foram encontrados 16 estudos, com quatro pontos identificados como de maior interesse dos autores: (1) tratamento farmacológico de emergência, (2) prescrição de autoinjetores de adrenalina, (3) conhecimento dos principais indícios da anafilaxia e (4) observação do paciente para verificar reações bifásicas. A preocupação com o uso da adrenalina intramuscular como primeira escolha frente à anafilaxia foi evidente na maioria dos estudos, mas o conhecimento sobre seu uso se mostrou desigual e especialmente baixo em estudo que incluiu dados do Brasil, onde a frequência de seu uso foi de 23,8%. A adrenalina autoinjetável é altamente recomendada entre especialistas para pacientes em risco de anafilaxia, mas seu uso ainda é pouco frequente entre não especialistas e em países que não dispõem dela em seus mercados internos. Estudos de intervenção comprovaram a melhora no entendimento dos médicos sobre anafilaxia após a divulgação das informações contidas nas diretrizes internacionais. A análise dos estudos reforça a necessidade de disseminar as diretrizes internacionais no manejo da anafilaxia, bem como de disponibilizar a adrenalina autoinjetável, a fim de melhorar o atendimento e evitar um desfecho fatal.

ABSTRACT Anaphylaxis is a severe, life-threatening generalized or systemic hypersensitivity reaction that requires rapid and adequate care. This study aimed to obtain an integrated view of the level of physicians' knowledge related with treatment of anaphylaxis in studies published within the last 5 years. Sixteen studies were found and four points were identified as of the great interest to the authors: (1) emergency pharmacological treatment, (2) epinephrine auto-injectors prescription, (3) knowledge of the main signs of anaphylaxis, and (4) admission of the patient to verify biphasic reactions. Concern about the use of intramuscular adrenaline as the first choice in relation with anaphylaxis was evident in most studies, rather than its use in the comparison dial, and especially low in a study that included data from Brazil, in which the frequency of its use was 23.8%. An adrenaline autoinjector is highly recommended among specialists for patients at risk of anaphylaxis, however, its use is still infrequent among non-specialists and in countries that this agent is not available. Intervention studies have shown improved medical knowledge of anaphylaxis following disclosure of the information contained in the international guidelines. The analysis of these studies reinforces the need to disseminate international guidelines for diagnosis and treatment of anaphylaxis, as well as providing an adrenaline autoinjector, to improve management and to prevent a fatal outcome.

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, recAsequencing, 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 recAsequencing to identify Bcc species. The recAsequencing 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.

OBJETIVO: Avaliar a hiperresponsividade brônquica à solução salina hipertônica a 4,5% como método alternativo a outros agentes broncoconstritores e sua relação com a sensibilização alérgica do paciente. MÉTODOS: Estudo transversal, experimental, com 85 indivíduos assim distribuídos: 45 no grupo de asmáticos e 17 no grupo controle não asmáticos e não alérgicos, que completaram o teste. Para nebulizar a solução salina hipertônica foi utilizado um nebulizador ultra-sônico de grande volume, sucessivamente durante 0,5, 1, 2, 4 e 8 minutos até haver queda > 15% em relação ao volume expiratório forçado no primeiro segundo basal. A dosagem de imunoglobulina E específica ao Dermatophagoides pteronyssinus por ImmunoCap foi considerada positiva quando > 0,35 kU/L. RESULTADOS: No grupo de asmáticos, 36 apresentaram queda média do volume expiratório forçado no primeiro segundo de 27,4% após nebulização de solução salina hipertônica. Nenhum do grupo controle (imunoglobulina E < 0,35 kU/L) apresentou resposta à solução salina hipertônica e a queda média do volume expiratório forçado no primeiro segundo foi de 9%. Nove asmáticos tiveram provocação brônquica negativa. A freqüência de provocação brônquica positiva foi maior nos indivíduos com imunoglobulina E específica elevada, o que indica uma relação entre hiperresponsividade brônquica e o nível sérico de imunoglobulina E específica. A sensibilidade do teste foi de 80% e a especificidade de 92%. CONCLUSÃO: A inalação de solução salina hipertônica é um método de provocação útil para avaliar hiperresponsividade brônquica em crianças e adolescentes, com adequadas sensibilidade e especificidade, além do baixo custo e necessidade de poucos equipamentos.

OBJECTIVE: To assess airway hyperresponsiveness to 4.5% hypertonic saline solution in comparison to that obtained through challenge with other bronchoconstriction agents and in relation to patient allergic sensitization. METHODS: A cross-sectional, experimental study was conducted, initially involving 85 subjects. After exclusions, the final sample consisted of 62 patients, divided into two groups: a study group of those with asthma (n = 45) and a control group of those with no asthma or allergies (n = 17). Hypertonic saline was nebulized using an ultrasonic nebulizer and administered successively for 0.5, 1, 2, 4 and 8 minutes until a drop in forced expiratory volume in one second of = 15% was achieved in relation to the baseline value. The level of specific immunoglobulin E to Dermatophagoides pteronyssinus level was determined by ImmunoCAP assay and was considered positive when > 0.35 kU/L. RESULTS: In the 36 asthma group subjects presenting a response, the mean drop in forced expiratory volume in one second after hypertonic saline nebulization was 27.4%. None of control group subjects (immunoglobulin E < 0.35 kU/L) presented a positive response to hypertonic saline. The mean forced expiratory volume in one second for control group subjects was 9%. The results of a bronchial provocation test were negative in 9 of the asthma group subjects. The frequency of bronchial provocation test positivity was higher in the subjects presenting elevated levels of specific immunoglobulin E, indicating that there is a relationship between bronchial hyperresponsiveness and the level of specific immunoglobulin E. The sensitivity and specificity of the test were 80% and 92%, respectively. CONCLUSION: Bronchial provocation with hypertonic saline presents satisfactory sensitivity and specificity. Therefore, in addition to being a low cost procedure that requires very little equipment, it is a useful means of assessing hyperresponsiveness in children and adolescents.

São apresentados dois casos de paracoccidioidomicose óssea, cujas diferenças eram em relação ao número das lesões, ao aspecto radiológico e à presença do fungo em biópsia das lesões. O relato se deve à raridade desta doença em crianças.

The rarity of paracoccidioidomycosis in childhood prompted us to report two cases with different clinical and radiological skeletal involvement. The number of osteolytic lesions, the presence of periosteal reaction and the finding of Paracoccidioides brasiliensis in biopsy specimens, were distinctive features in both cases.