Autochtonal case of chronic, unifocal, pulmonary paracoccidioidomycosis with methotrexate use, in Salvador ‒ Brazil.

We report an autochthonous case of mild unifocal chronic pulmonary paracoccidioidomycosis in a 48-year-old previously healthy woman with no history of possible environmental exposures in endemic rural areas, supposedly resulting from reactivation of a latent pulmonary focus secondary to the use of methotrexate for the control of Chikungunya arthropathy. Laboratory investigation ruled out other immunosuppression. Her only symptoms were a dry cough and chest pain. Diagnosis confirmed by needle lung biopsy. There were no abnormalities on physical examination nor evidence of central nervous system involvement. MRI of the total abdomen showed no involvement of other organs. Computed chest tomography showed a favorable evolution under the use of itraconazole (200 mg/day). Different tomographic presentations findings are highlighted when performed before and after treatment. CONCLUSIONS: PCM should be considered even in a woman without a history of consistent environmental exposure and in a non-endemic geographic area.

Autochtonal case of chronic, unifocal, pulmonary paracoccidioidomycosis with methotrexate use, in Salvador ‒ Brazil

Abstract Summary We report an autochthonous case of mild unifocal chronic pulmonary paracoccidioidomycosis in a 48-year-old previously healthy woman with no history of possible environmental exposures in endemic rural areas, supposedly resulting from reactivation of a latent pulmonary focus secondary to the use of methotrexate for the control of Chikungunya arthropathy. Laboratory investigation ruled out other immunosuppression. Her only symptoms were a dry cough and chest pain. Diagnosis confirmed by needle lung biopsy. There were no abnormalities on physical examination nor evidence of central nervous system involvement. MRI of the total abdomen showed no involvement of other organs. Computed chest tomography showed a favorable evolution under the use of itraconazole (200 mg/day). Different tomographic presentations findings are highlighted when performed before and after treatment. Conclusions PCM should be considered even in a woman without a history of consistent environmental exposure and in a non-endemic geographic area.

Novel CACNA1S mutation in hypokalaemic periodic paralysis.

A 15-year-old girl was admitted to emergency department with an acute flaccid tetraparesis with no other symptoms. A history of recurrent similar episodes with spontaneous recovery was reported and no family history was known. Laboratory tests revealed severe hypokalaemia and hypokaluria. Symptoms resolution occurred after potassium replacement. The diagnosis of hypokalaemic periodic paralysis (HPP) was confirmed by genetic testing, which revealed a not previously described mutation in CACNA1S gene (c.3715C>G p.Arg1239Gly). HPP is a rare neuromuscular disorder that causes episodic attacks of flaccid paralysis with concomitant hypokalaemia. Primary forms of the disease are skeletal muscle ion channelopathies. HPP occurs due to a problem in potassium distribution rather than a total body potassium deficiency. Therefore potassium replacement should be carefully performed because of the risk of rebound hyperkalaemia. Knowing this rare entity is important in order to avoid diagnostic delays and so that proper treatment can be initiated to reduce morbidity and mortality.

Serum leptin levels correlate negatively with the capacity of vitamin D to modulate the in vitro cytokines production by CD4+ T cells in asthmatic patients.

Both obesity and low vitamin D levels have been associated with allergic asthma (AA) severity. In the present study, severity of AA was associated with obesity but to the in vitro IgE production. In those patients, higher levels of IL-5, IL-6 and IL-17 were quantified in CD4+ T-cell cultures as compared with patients with mild and moderate AA. In addition, the lowest IL-10 levels were detected in the cell cultures from patients with a worse prognosis. Interestingly, the occurrence of AA elevates the plasma levels of leptin, and this adipokine was positively correlated with the release of IL-5, IL-6 and IL-17, but inversely correlated with IL-10 production, by CD4+ T-cells from patients. In AA-derived CD4+ T-cell cultures, 1,25(OH)2D3 was less efficient at inhibiting IL-5, IL-6 and IL-17 production, and up regulating IL-10 release, as those from healthy subjects. Interestingly, the in vitro immunomodulatory effects of vitamin D were inversely correlated with serum leptin levels. In summary, our findings suggested that obesity, probably due to the overproduction of leptin, negatively impacts AA as it favors imbalance between Th2/Th17 and regulatory phenotypes. The deleterious effects of leptin may also be due to its ability to counter-regulate the immunosuppressive effects of vitamin D.

A novel genetic mutation in a Portuguese family with GCK-MODY.

INTRODUCTION: Maturity-onset diabetes of the young (MODY) is a genetically heterogeneous form of diabetes mellitus, with autosomal dominant inheritance. It accounts for 2%-5% of all diabetes cases. Glucokinase-MODY is the second most frequent form, which has been shown to be the result of mutations in the glucokinase (GCK) gene. It mostly presents with mild hyperglycemia, and, usually, no diabetes-related complications occur. CASE REPORT: A 9-year-old female was admitted to the Endocrine Clinic to study her fasting hyperglycemia. Despite her obesity (body mass index 28 kg/m2), her physical examination had no other abnormalities. Blood tests showed a 6.3% hemoglobin A1c, with normal standard oral glucose tolerance test result, normal insulin value and normal C-peptide level. Insulin autoantibodies and antibodies against glutamate decarboxylase were negative. She began metformin and adequate diet. She had a strongly positive family history for diabetes. The patient's mother, uncle, grandfather, great-aunt and great-grandfather on her mother's side were diagnosed with diabetes. Complete sequencing of the GCK gene, carried out in the patient, identified a novel mutation c.1268T>A (p.Phe423Tyr) in exon 10 of the gene GCK in heterozygosity. Further studies revealed the same mutation in her mother and maternal grandfather. CONCLUSION: Finding the same mutation in three different generations of diabetic patients, in the same family, is highly suggestive of its pathogenicity. To the authors' knowledge, this is the first time it is described in the literature. Correct molecular diagnosis of MODY predicts better the clinical course of diabetes and facilitates individualised management.

Endocervical component in conventional cervical smears: influence on detection of squamous cytologic abnormalities.

The objective of this study was to correlate the number of endocervical cells and the number of atypical cells in cervical smears with cytological abnormalities to verify if a higher number of endocervical cells would implicate in a higher number of squamous atypical cells. In this cross sectional study, it was made a blinded review of 294 conventional cervical smears. The consensus diagnoses reached by the blinded review by three observers included 213 low-grade squamous intraepithelial lesions (LSILs) and 81 high-grade squamous intraepithelial lesions (HSILs). In each cervical smear were computed the number of endocervical cells and the number of squamous atypical cells, and it was established the following score: one (0-5 cells); two (6-10 cells); three (11-25 cells); four (26-50 cells); and five (more than 50 cells). Compared to the smears with an account of endocervical cells classified in score 1 (0-5 cells), an account of more than 50 endocervical cells was significantly associated to the presence of at least 10 squamous atypical cells in the cervical smears (OR 2.87 95% CI 1.54-5.35). Data from this study suggest that there is a positive association between the number of endocervical cells and the number of squamous atypical cells suggestive of SIL.

Planejamento estratégico para o desenvolvimento de padrões profissionais de enfermagem: nota prévia / Strategic plan for developing nursing professional standards: preview note

CONTEXTO: Diante da função primordial de um Hospital Universitário e após a análise do contexto e dos envolvidos, avaliou-se que o desvio do foco assistencial do cliente (usuário externos) e do aluno (usuário interno) é o problema prioritário e central. OBJETIVOS: Avaliar o impacto da implantação do cuidado centrado no cliente, integral e hierarquizado, baseado em evidências científicas, desenvolvendo uma base de dados científicos sobre a relação entre os resultados do cliente sensíveis aos cuidados de enfermagem. MÉTODO: O monitoramento e a avaliação do plano estratégico elaborado pela Coordenação de Enfermagem do HUPE/UERJ. Os dados serão coletados do prontuário do cliente e dos documentos da administração hospitalar (escalas, etc). Os dados qualitativos serão colhidos por meio de entrevistas com os clientes e funcionários. Os dados serão submetidos a análise estatística (regressão múltipla) e qualitativa para analisar a relação entre os resultados e a implantação dos padrões profissionais de enfermagem. RESULTADOS ESPERADOS: o perfil do atendimento ao cliente com base nos indicadores sensíveis aos cuidados de enfermagem.

PROBLEM: At the University Hospital of the University of Rio de Janeiro State (HUPE/UERJ),the Nursing Coordination analysed the social context and the stakeholders of health assistence, and identified a problem to address: a change shift in the focus from the client and student to the institution itself. OBJECTIVE: To achieve the vision of a 24/7 nursing care, centered in the client and based on scientifc evidence, a strategic plan was developed to establish nursing professional standards. METHOD: As an instrument of social management, an Strategic Project represents the process of assessment, diagnosis, planning, and evaluation of the nursing managers to improve the quality of care at HUPE/UERJ. EXPECTED OUTCOME: a hospital database related to client outcomes related to nursing care.

Cultivation of Bacillus thuringiensis subsp. Kurstaki S93 in a fed-Batch process: effect of the ratio glucose to yeast extract concentrations in the feeding culture media

The feeding media was studied in a fed-batch process for production of the complex spore-d-endotoxin by B. thuringiensis S93. The microorganism was first cultvated in a initial batch followed by an exponential feeding (m=0,25h(-1)) with concentrated culture media (160 g glucose/L) containing different ratios of glucose and autolysed yeast: 8/7, 8/4 and 8/2 (g/g). The batch culture medium was composed of glucose (8 g/L), autolysed yeast (7g/L) and mineral salts. Sporulation and d-endotoxin production were observed only after the end of feeding. To compare the experiments, batch cultivations were also performed with an initial concentration of 8 g/L of glucose and the same ratio of glucose and autolized yeast. Batch cultivations reached lower concentrations of total biomass and spores than the fed-batch ones and percentagens of sporulation higher than 80 per cente. The 8/7 ratio fed-batch cultivations reached the highest biomass concentration, producing however a very low level of sporulation (27per cent) and virtually no d-endotoxin. Cultivations with 8/4 and 8/2 ratios reached the highest concentrations of spores. In those assays, the maximum spores concentration and the maximum sporulation percentage were 8,3x10(9) spores/mL and 90per cent for the 8/4 ratio and 5,6x10(9) spores/mL and 89per cent for the 8/2 ratio.