Correlação entre as formas clínicas da hanseníase e o grau de incapacidade neurológica / Correlation between the clinical forms of leprosy and the degree of neurological incapacity

Objetivo: Correlacionar as formas clínicas e o grau de incapacidade neurológica de pacientes com hanseníase, além de obter informações sobre características epidemiológicas e verificação do grau da correlação pesquisada. Método: foram estudados 200 pacientes diagnosticados com hanseníase, no período de <1950 a > 2000, independente da forma clínica apresentada e do sexo, provenientes da "Colônia do Prata". Utilizou-se protocolo de pesquisa que continha variáveis epidemiológicas, como idade, sexo, procedência, data de nascimento e informações clínicas referentes à hanseníase, como data do diagnóstico, forma clínica e o grau de incapacidade neurológica no momento do diagnóstico até a alta. Resultados: verificou-se que a maioria dos pacientes era do sexo masculino(63%), com idade entre 15 a 60 anos e que manifestaram a forma multibacilar(62,5%). Quanto aos números de casos, houve maior incidência a partir da década de 70. Observou-se que pacientes com grau de incapacidade 0 apresentavam predominantemente a forma indeterminada, enquanto os que apresentavam alguma incapacidade tinham essencialmente a forma virchowiana. Conclusão: o estudo constatou que pacientes do sexo masculino, que adoecem depois dos 15 anos de idade e que desenvolvem a forma multibacilar possuem maior chance de evoluírem com algum grau de incapacidade neurológica.

Objective: to correlate the clinical forms and the degree of neurological incapacity of patients from with Leprosy, beyond getting information on epidemiological characteristics and verification of the degree of the searched correlation. Method: 200 patients diagnosed with Leprosy were studied, in the period of <1950 > 2000, independent of the clinical form and the sex, proceeding from "Colônia do Prata". A research protocol was used, and it contained epidemiological variables, as age, sex, origin, date of birth and referring clinical information to the Leprosy, as date of the diagnosis, clinical form and the degree of neurological incapacity at the moment of the diagnosis until the successful treatment. Results: it was verified that the majority of the patients were male (63%), with age between 15 and 60 years and that they had revealed the form Multibacillary (62,5%). The highest incidence of cases was on seventy's decade. It was observed that patients with degree of incapacity 0 presented the indetermined form predominantly, while the ones that presented some incapacity essentially had the Virchowian form. Conclusion: it was evidenced that male patients that got sick after 15 years old and that developed the Multibacillary form possess higher possibility to evolve with some disability.

Treatment of Pediatric Takayasu arteritis with infliximab and cyclophosphamide: experience from an American-Brazilian cohort study.

BACKGROUND: Pediatric Takayasu arteritis (pTA) is difficult to treat and can lead to significant morbidity and mortality. OBJECTIVES: The objective of this study was to describe clinical characteristics for pTA and determine the safety and efficacy of cyclophosphamide (CYC) and infliximab (IFX) in pTA. METHODS: This was a retrospective analysis of 23 pTA patients seen at Children's Hospital Los Angeles and Universidade Federal de São Paulo-Brazil from 1990 to 2011. All patients fulfilled the 1990 American College of Rheumatology criteria for Takayasu arteritis. Disease activity was assessed using a modified National Institutes of Health score. RESULTS: Twenty-three patients (14 female and 9 male patients), mean age of 15.7 ± 6.0 years, were included. Cyclophosphamide was used before IFX treatment in 17 of 23 and IFX before CYC in 2 of 23 patients. The average time from disease onset to treatment initiation was 2.6 ± 2.4 years for CYC and 4.1 ± 2.4 years for IFX. Nine (60%) of 15 patients failed CYC, and of these 6 were changed to IFX with subsequent clinical stabilization in 5 (83%) of 6. Two patients initially treated with IFX were switched to CYC because of lack of appropriate response: 1 patient later worsened, and the other was lost to follow-up. Five opportunistic infections requiring hospitalization occurred in the CYC group, whereas none were observed in the IFX group. Patients in the IFX group were more likely to decrease or stop their corticosteroids when compared with the CYC patients. CONCLUSIONS: Cyclophosphamide is often used as initial treatment but has many adverse effects. In this retrospective case series, IFX was equivalent to CYC with fewer adverse effects, making IFX an alternative therapeutic option for pTA.

First seizure as late presentation of velo-cardio-facial syndrome.

Congenital hypoparathyroidism caused by velo-cardio-facial syndrome (VCFS) typically presents with hypocalcemia in the neonatal period associated with other characteristic findings. We report the case of an 8-year-old boy presenting a hypocalcemic seizure (ionized calcium, 0.65 mM); he had a history of velopharyngeal insufficiency and late development with language impairment; at presentation, minor dysmorphic features were noticed. Laboratory evaluation revealed a parathormone level of <1.0 pg/mL. VCFS was confirmed by fluorescence in situ hybridization study revealing the 22q11.2 deletion. High levels of calcium administration were needed to normalize serum calcium. In this case, signs of hypoparathyroidism only developed in late childhood, and the patient, despite the minor clinical signs of VCFS, remained symptom-free. He presented no cardiac malformations, immunologic abnormalities, or renal dysfunction often associated with this diagnosis. In a child with hypocalcemia, considering the hypothesis of VCFS, even if there are no other manifestations, is important both for clinical diagnosis and management and for genetic counseling.

Juvenile systemic lupus erythematosus in Portugal: clinical and immunological patterns of disease expression in a cohort of 56 patients.

OBJECTIVE: To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. METHODS: The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed. RESULTS: A cohort of 56 patients with a mean age at disease onset of 12.6 ± 4.04 years (mean ± 1SD) (range, 1.0-17.0 years) and a mean period of follow-up of 5.5 ± 5.4 years. Forty six (82.1%) patients were female. The most common disease manifestations were musculoskeletal (87.5%), mucocutaneous (80.3%) and haematological abnormalities (75%). Lupus nephritis was diagnosed in 46.4% of patients and consisted of glomerular nephritis in all cases. Neuropsychiatric manifestations occurred in 21.4% but severe central nervous system complications were uncommon, as brain infarcts and organic brain syndrome in 4 (7.1%) patients. Antinuclear antibodies and anti-double stranded DNA were positive in most patients in (98.2% and 71.4% respectively), as well as low C3 and/or C4 were observed frequently (85.7%). Generally, most patients had a good response to therapy as demonstrated by a significant decreasing of SLEDAI score from disease presentation to the last evaluation. The SLEDAI at diagnosis, the maximum SLEDAI and the incidence of complications were significantly higher in patients with neurolupus and/or lupus nephritis. Therapy included oral steroids (87.5%), hydroxychloroquine (85.7%), azathioprine (55.4%), IV cyclophosphamide (28.6%) along with other drugs. Six (10.7%) patients were treated with rituximab. Long-term remission was achieved in 32%, disease was active in 68%, adverse reactions to therapy occurred in 53.6% and complications/severe manifestations in 23.2%. Two patients died, being active disease and severe infection the causes of death. CONCLUSIONS: This study suggests that in our patients the clinical and laboratory features observed were similar to juvenile systemic lupus erythematosus patients from other series. Clinical outcome was favourable in the present study. Complications from therapy were frequent.

Portuguese recommendations for the use of biological therapies in children and adolescents with juvenile idiopathic arthritis--December 2011 update.

OBJECTIVE: To update the Portuguese recommendations in order to assist the rational and safe prescribing of biological therapies in children and adolescents with Juvenile Idiopathic Arthritis (JIA) as more evidence and experience with these drugs are available. METHODS: The recommendations were formulated by Rheumatologists and Pediatricians, with experience in Pediatric Rheumatology, based on literature evidence and consensus opinion. The evidence was sought through a MEDLINE search. The retrieved results were discussed and a set of recommendations proposed. All propositions were extensively debated and the final recommendations formulated. RESULTS: A consensus was achieved regarding the eligibility, response criteria, maintenance of biologic thera py, and procedures in case of non-response. Also, specific recommendations concerning safety procedures before and while on biologic therapies were formulated. CONCLUSIONS: Thirteen recommendations for guidance biological therapy in children and adolescents with JIA were developed using both evidence-based and expert consensus. These recommendations will be updated as more evidence becomes available and more biological therapies are licensed.

Avaliação da função cognitiva em pacientes dom Doença de Parkinson / Assessment of cognitive function in patients with Parkinson Disease

Objetivos: analisar presença de déficits cognitivos em pacientes portadores de Doença de Parkinson. Método: foiobtida uma casuística de 58 indivíduos, sendo distribuídos em dois grupos: Grupo Parkinson (GP), composto por 25pacientes e Grupo Controle (GC), constituído por 33 indivíduos. O GP foi subdividido em outros dois subgrupos: 1-Parkinson com depressão; 2- Parkinson sem depressão. Os grupos foram submetidos a testes de avaliação cognitivacom posterior comparação dos resultados utilizando-se o teste t student por meio do BioEstat 5.0. Resultados: o GPcomo um todo apresentou déficits cognitivos mais evidentes quando comparados ao GC na avaliação do MEEM,Fluência verbal, Desenho do relógio e Bateria Breve de Rastreio Cognitivo, porém, quando se comparou, dentro doGP, os indivíduos com e sem depressão, não foram encontrados dados, estatisticamente, significativos, apesar de umpior desempenho ser observado nos testes aplicados ao GP com depressão. Conclusão: o estudo conclui que ospacientes do GP apresentaram maior comprometimento cognitivo quando comparado ao GC. Faz-se relevantedestacar que a complexidade da DP exige que trabalhos futuros, com amostras maiores, sejam conduzidos a fim dese esclarecer a complicada relação entre a doença e as alterações neurológicas observadas

Objective: evaluating the presence of cognitive deficits in patients with Parkinson's disease. Method: It wasobtained a sample of 58 subjects, divided into two groups: Parkinson Group (GP), composed of 25 patients andControl Group (CG), consisting in 33 individuals. The GP was subdivided into two subgroups: 1 - Parkinson withdepression, 2 - Parkinson without depression. The groups were submitted the cognitive testing with subsequentcomparison of results using the Student t test with the BioEstat 5.0. Results: The GP as a whole showed moreevident cognitive deficits when compared to GC in evaluating the MMSE, Verbal Fluency, Clock Drawing Test andthe Brief Cognitive Screening Battery , however, when compared, inside the GP, the individuals with and withoutdepression, there wasn?t statistically significant data, despite of a worse performance being noticed in the testsapplied to the GP with depression. Conclusion: The study concludes that patients in the GP showed higher cognitiveimpairment when compared to GC. It is relevant to detach that the complexity of PD requires that future work, withlarger samples, are conducted to clarify the complicated relationship between the disease and the neurologicaldisorders observed.

Prevalência de fatores de risco cardiovascular em profissionais de saúde no ambiente de trabalho / Prevalence of cardiovascular risk factors in health professionals in the work environment

Objetivo: Determinar a prevalência de fatores de risco para doença cardiovascular em profissionais de saúde no ambiente de trabalho. Métodos: Foi realizado um estudo descrito transversal, envolvendo os profissionais do Hospital Municipal Doutor Munir Rafful, que responderam a um questionário, e foram submetidos à avaliação clínica de peso, altura, pressão arterial e avaliação bioquímica do colesterol total. Foi calculada a porcentagem e feita a análise dos resultados. Resultados: Do total de 210 profissionais habilitados, 188 foram avaliados, sendo 57 homens e 131 mulheres. As prevalências dos fatores de risco cardiovascular foram: sedentarismo (83,5 por cento), etilismo (35,6 por cento), hipertensão arterial (22,9 por cento), obesidade (20,2 por cento), tabagismo (19,7 por cento), colesterol maior que 200mg/dl(10,6 por cento). Conclusão: A detecção de índices elevados dos fatores de risco para doenças cardiovasculares em profissionais de saúde é bastante preocupante, visto que servem de exemplo à sociedade, e apontam a necessidade de medidas para promoção da saúde e prevenção de doenças cardiovasculares.