Erythroderma: analysis of 247 cases

The profile of 247 patients with erythroderma during a 23 year period from January, 1962 through March, 1985, with a follow-up period ranging from 1 to 26 years were analysed. The patients presented with diffuse erythema, scaling and pruritus of more than 2 months' duration, and the age ranged from 16 to 60 years. Psoriasis was the most frequent underlying disease with an estimated frequency of 44.9%, the reaction to the use of drugs appeared in 7.3% of total cases and association with reticulosis showed a frequency of 4.1%. The cause of the erythroderma could not be determined in 29.2% of the cases. Sex differences in terms of underlying diseases were not observed. One or more skin biopsies along with the clinical findings were diagnostic or suggestive of the underlying disease in 63.6% of the cases. Repeated skin biopsies are recommended as the best method for etiologic diagnosis of erythroderma. At P=0.05 significance level, masculine/feminine ratio of 2 : 1 was found. The question arises wether causal agent of erythroderma may not be somehow related to different exposure by sex to environmental antigens.

Maternal neurotic symptoms and infants' risk of developing persistent diarrhoea

A previously calculated predictive model for health risk selects infants who suffer 4-5 times more morbidity than their unselected peers. Preliminary results suggested that this risk is related to maternal neurotic symptomatology. To evaluate this hypothesis, 52 consecutive mothers whose infants had a positive predictive score (Group 1) and 52 in whom this was negative (Group 2) were evaluated by means of Goldberg's General Health Questionnaire (GHQ - 30). A total of 41.9% and 20.5% of the mothers in Groups 1 and 2, respectively, scored above 11 points in GHQ-30, established as the cut off point. It is concluded that among poor urban families in Santiago mothers of infants with high risk of persistent diarrhoea have increased frequency of detectable neurotic symptoms. New programs aimed at this type of infant should include psychological support for their mothers.

Doenças lisossomais de sobrecarga em Portugal: 10 anos de experiência em estudos moleculares no INSA (2006-2016)

As Doenças Lisososomais de Sobrecarga (DLS) são um grupo de mais de 50 doenças hereditárias do metabolismo, sendo a maioria causada por defeitos em enzimas lisossomais específicas. A característica distintiva das DLS é a acumulação lisossomal do(s) substrato(s) não degradado(s), bem como a acumulação de outro material secundariamente à disfunção lisossomal. A apresentação clínica destas patologias é bastante heterogénea, variando desde formas pré-natais, até apresentações infantis ou na idade adulta, sendo frequente a presença de atraso psicomotor e neurodegeneração progressiva. Neste artigo são apresentados os resultados de vários estudos de caracterização molecular efetuados ao longo da última década (2006-2016) em doentes portugueses com as seguintes DLS: Mucopolissacaridose II, Mucopolissacaridose IIIA, Mucopolissacaridose IIIB, Mucopolissacaridose IIIC, Sialidose, Galactosialidose, Gangliosidose GM1, Mucolipidose II alfa/beta, Mucolipidose III alfa/beta, Mucolipidose III gama e Doença de Unverricht-Lundborg. De um modo geral, estes trabalhos permitiram conhecer as variações genéticas associadas a estas DLS, analisar a sua distribuição na população portuguesa e compreender o seu papel na forma de apresentação clínica destas patologias.

Comportamentos interactivos das educadoras de infância em salas de creche em função do tipo de actividades e das características estruturais do contexto

O presente artigo pretende examinar a quantidade, qualidade e adequação dos comportamentos interactivos das educadoras em salas de creche, em actividades livres e estruturadas, investigando a sua relação com aspectos de estrutura destes contextos. Participaram neste estudo 30 responsáveis por salas de creche da Área Metropolitana do Porto (incluindo 5 auxiliares de acção educativa). Os comportamentos interactivos das educadoras foram avaliados com a Escala de Interacção Educador-Criança (Farran, & Collins 1995). Os resultados obtidos sugerem que as educadoras exibem maior quantidade de comportamentos interactivos no decurso de actividades estruturadas (r = .65) e que a adequação destes comportamentos é superior quando estes profissionais têm bacharelato ou licenciatura (rь=.57), quando auferem salários superiores (r = .42) e quando despendem menos horas de trabalho directo com as crianças (r = -.37). A qualidade dos comportamentos interactivos está relacionada com o salário auferido (r = .43). As implicações práticas dos resultados obtidos são discutidas com o objectivo de analisar formas possíveis de alcançar níveis de excelência em contextos de educação de infância.

O educador de Infância também “brinca”?

Relatório da Prática Profissional Supervisionada Mestrado em Educação Pré-Escolar

Psoríase: o impacto da doença no dia-a-dia dos portadores

Mestrado em Fisioterapia

Detection of classic and invasive E. coli and Shigella serotypes in stools by indirect immunofluorescence

IIF in the detection of invasive and classic enteropathogenic E. coli and Shigella serotypes was compared with traditional coproculture methods. IIP results agreed with the coproculture findings in 128 out of 140 cases tested for enteropathogenic E. coli (91%) and in 108 out of 112 for Shigella (96%). All cases with positive reactions by coproculture were confirmed by IIP. In the control group it were obtained by IIF 12 cases with positive reactions for enteropathogenic E. coli and 4 cases for Shigella, including two cases of mixed infection by E. coli 026/Sh. dysenteriae and E. coli 0124/Sh. dysenteriae. It was discussed the high sensitivity and specificity of the IIF when compared with the traditional methods, being suggested that IIF is a valuable tool in epidemiological studies involving these organisms and an important aid in the stablishment of an early presumptive diagnosis of the acute infantile diarrhea.

Contributos para a avaliação económica de medicamentos em Portu

RESUMO: A importância da avaliação económica na área da saúde tem sido crescente ao longo das últimas três décadas. É um campo de análise multi-disciplinar pelo qual se têm vindo a interessar investigadores de todo o mundo e onde, naturalmente, a economia da saúde tem contributos significativos para dar. Contudo, é uma área ainda relativamente inexplorada em termos de investigação metodológica em Portugal. Espera-se com este trabalho colmatar algumas das lacunas existentes e lançar pontes para investigação futura. Neste estudo pretendem-se desenvolver contributos autónomos, numa óptica de resolução de problemas concretos, para a avaliação económica de medicamentos em Portugal. Em primeiro lugar abordou-se a importância da estimação dos custos nos estudos de avaliação económica em saúde. Em segundo lugar determinou-se o custo de um doente com esclerose múltipla, por nível de severidade em Portugal com recurso à metodologia de estudos custo da doença. Em terceiro lugar, aplicando a mesma metodologia, determinou-se o custo de um doente com psoríase, por nível de severidade, em Portugal. Em quarto lugar mediu-se o impacto da psoríase na qualidade de vida relacionada com a saúde dos indivíduos afectados através de dois instrumentos genéricos de medição da qualidade de vida relacionada com a saúde (SF-36 e EQ-5D) e de dois instrumentos específicos (DLQI e PDI). Em quinto lugar, foi feita a adaptação de um modelo sobre a prevenção do tromboembolismo venoso em cirurgia ortopédica ao contexto nacional com recurso a dados recolhidos numa amostra de hospitais portugueses. Foi ainda apresentada uma estimativa do impacto orçamental decorrente da inclusão, ou não, de um novo medicamento na prática nacional. ABSTRACT: The importance of economic evaluation in the health care sector has been growing in the last three decades. It is a multidisciplinary field of research that attracts attention from researchers working all over the world. It is should be noted that health economics can contribute significantly to the development of this area. However, in Portugal is difficult to find methodological contributes in this field of research. With this work one hope to be able to overcome some of the existing shortcomings. This study seeks to develop independent contributions for the economic evaluation of pharmaceuticals in Portugal, aiming to help to solve existing problems. Firstly, one approached the importance of the accurate estimation of costs in economic evaluations of pharmaceuticals. Secondly, the cost of patient with multiple sclerosis was estimated, by severity level, in Portugal, using a cost of illness methodology. In third place, employing the same methodology, the cost of patient with psoriasis by severity level was estimated for Portugal. In fourth place, the impact of psoriasis in the quality of life related to health was ascertained by two generic measures (SF-36 and EQ-5D) and two specific measures (DLQI and PDI). Lastly, a model on the prevention of venous thromboembolism in orthopedic surgery was adapted for Portugal through the inclusion of data collected in a sample of Portuguese hospita

EVALUATION OF A RAPID SCREENING ASSAY FOR BACTERIAL IDENTIFICATION (DOT-ELISA) IN FECAL SAMPLES FROM CHILDREN

With the objective of standardizing a Dot Enzyme-Linked Immunosorbent Assay (Dot-ELISA) to detect antigens of fecal bacterial enteropathogens, 250 children, aged under 36 months and of both sexes, were studied; of which 162 had acute gastroenteritis. The efficacy of a rapid screening assay for bacterial enteropathogens (enteropathogenic Escherichia coli "EPEC", enteroinvasive Escherichia coli "EIEC", Salmonella spp. and Shigella spp.) was evaluated. The fecal samples were also submitted to a traditional method of stool culture for comparison. The concordance index between the two techniques, calculated using the Kappa (k) index for the above mentioned bacterial strains was 0.8859, 0.9055, 0.7932 and 0.7829 respectively. These values express an almost perfect degree of concordance for the first two and substantial concordance for the latter two, thus enabling this technique to be applied in the early diagnosis of diarrhea in infants. With a view to increasing the sensitivity and specificity of this immunological test, a study was made of the antigenic preparations obtained from two types of treatment: 1) deproteinization by heating; 2) precipitation and concentration of the lipopolysaccharide antigen (LPS) using an ethanol-acetone solution, which was then heated in the presence of sodium EDTA

Toxocara seroprevalence in children from a subtropical city in Argentina

Most studies from Argentina have focused on toxocariasis as an environmental problem of big cities, and there are no available data about children infection from small or middle-sized cities. In order to assess the prevalence of anti-Toxocara antibodies in infantile population, 206 children from Resistencia, of both sexes, aged 1-14 years old were studied by Elisa testing with E/S T. canis L2 antigens. Hematological parameters and immunoglobulin levels were determined; five days' stool samples were studied and epidemiological data were obtained by means of a questionnaire to parents. Results showed that 73% of the children had one or more dogs living at home, 57% reported geophagia and 37.9% were positive for Toxocara serology, but there was no significant difference in prevalence neither for boys and girls, nor concerning age. An increased risk of infection was observed in age groups 5-6 and 7-8 for boys, and in age groups 3-4 and 5-6 for girls.

Electrophoretic protein patterns and numerical analysis of Candida albicans from the oral cavities of healthy children

The aim of this research was to evaluate the protein polymorphism degree among seventy-five C. albicans strains from healthy children oral cavities of five socioeconomic categories from eight schools (private and public) in Piracicaba city, São Paulo State, in order to identify C. albicans subspecies and their similarities in infantile population groups and to establish their possible dissemination route. Cell cultures were grown in YEPD medium, collected by centrifugation, and washed with cold saline solution. The whole-cell proteins were extracted by cell disruption, using glass beads and submitted to SDS-PAGE technique. After electrophoresis, the protein bands were stained with Coomassie-blue and analyzed by statistics package NTSYS-pc version 1.70 software. Similarity matrix and dendrogram were generated by using the Dice similarity coefficient and UPGMA algorithm, respectively, which made it possible to evaluate the similarity or intra-specific polymorphism degrees, based on whole-cell protein fingerprinting of C. albicans oral isolates. A total of 13 major phenons (clusters) were analyzed, according to their homogeneous (socioeconomic category and/or same school) and heterogeneous (distinct socioeconomic categories and/or schools) characteristics. Regarding to the social epidemiological aspect, the cluster composition showed higher similarities (0.788 < S D < 1.0) among C. albicans strains isolated from healthy children independent of their socioeconomic bases (high, medium, or low). Isolates of high similarity were not found in oral cavities from healthy children of social stratum A and D, B and D, or C and E. This may be explained by an absence of a dissemination route among these children. Geographically, some healthy children among identical and different schools (private and public) also are carriers of similar strains but such similarity was not found among other isolates from children from certain schools. These data may reflect a restricted dissemination route of these microorganisms in some groups of healthy scholars, which may be dependent of either socioeconomic categories or geographic site of each child. In contrast to the higher similarity, the lower similarity or higher polymorphism degree (0.499 < S D < 0.788) of protein profiles was shown in 23 (30.6%) C. albicans oral isolates. Considering the social epidemiological aspect, 42.1%, 41.7%, 26.6%, 23.5%, and 16.7% were isolates from children concerning to socioeconomic categories A, D, C, B, and E, respectively, and geographically, 63.6%, 50%, 33.3%, 33.3%, 30%, 25%, and 14.3% were isolates from children from schools LAE (Liceu Colégio Albert Einstein), MA (E.E.P.S.G. "Prof. Elias de Melo Ayres"), CS (E.E.P.G. "Prof. Carlos Sodero"), AV (Alphaville), HF (E.E.P.S.G. "Honorato Faustino), FMC (E.E.P.G. "Prof. Francisco Mariano da Costa"), and MEP (E.E.P.S.G. "Prof. Manasses Ephraim Pereira), respectively. Such results suggest a higher protein polymorphism degree among some strains isolated from healthy children independent of their socioeconomic strata or geographic sites. Complementary studies, involving healthy students and their families, teachers, servants, hygiene and nutritional habits must be done in order to establish the sources of such colonization patterns in population groups of healthy children. The whole-cell protein profile obtained by SDS-PAGE associated with computer-assisted numerical analysis may provide additional criteria for the taxonomic and epidemiological studies of C. albicans.

Acute generalized exanthematous pustulosis (AGEP). Case report

Acute Generalized Exanthematous Pustulosis (AGEP) is a drug-induced dermatosis characterized by an acute episode of sterile pustules over erythematous-edematous skin. It is accompanied by an episode of fever, which regresses a few days after discontinuation of the drug that caused the condition or as a result of corticosteroid treatment. The main triggering drugs are antibiotics, mainly beta-lactam ones. Other medications, such as antifungal agents, non steroid anti-inflammatory drugs, analgesics, antiarrhythmic, anticonvulsant and antidepressant drugs, may also be responsible. Histologically, it is characterized by the existence of vasculitis, associated with non-follicular subcorneal pustules. A case of a Caucasian female outpatient unit of Dermatology with AGEP, who presented with generalized pustulosis lesions after the use of cephalosporin for urinary infection is related. The diagnosis was confirmed by the clinical and pathological correlations, the resolution of the dermatosis after discontinuation of the drug and use of systemic corticosteroid treatment, and the recurrence of the disorder after the introduction of a similar drug. The importance of the recognition of this drug-induced dermatosis is given by its main differential clinical and histological diagnoses: generalized pustular psoriasis and subcorneal pustulosis.

Rotavirus serotypes and electropherotypes identified among hospitalised children in São Luís, Maranhão, Brazil

During June 1997-June 1999 rotavirus infection was screened in infants aged up to 2 years and hospitalised with acute diarrhoea in São Luís, Northeastern Brazil. Altogether, 128 stool samples were collected from diarrhoeic patients and additional 122 faecal specimens from age- and- temporal matched inpatients without diarrhoea were obtained; rotavirus positivity rates for these groups were 32.0% (41/128) and 9.8% (12/122), respectively (p < 0.001). Both electropherotyping and serotyping could be performed in 42 (79.2%) of the 53 rotavirus-positive stool samples. Long and short electropherotypes were detected at similar rates - 38.1% and 40.5% of specimens, respectively. Overall, a G serotype could be assigned for 35 (83.3%) of specimens, the majority of them (66.7%) bearing G1-serotype specificity. Taking both electropherotypes and serotypes together, G1 rotavirus strains displaying long and short RNA patterns accounted for 30.9% and 19.0% of tested specimens, respectively; all G2 strains had short electropherotype. Rotavirus gastroenteritis was detected year-round and, in 1998, the incidence rates tended to be higher during the second semester than in the first semester: 45.2% and 26.1% (p = 0.13), respectively. Rotavirus infections peaked at the second semester of life with frequencies of 30.1% and 13.5% for diarrhoeic children and controls, respectively. While the six rotavirus strains bearing G2-type specificity were circulating throughout the whole study period, G1 serotypes (n = 27) emerged as from June 1998 onwards, 20 (74.1%) of which clustering in 1998. These data underscore the importance of rotaviruses in the aetiology of severe infantile gastroenteritis in Northeastern Brazil and sustain the concept that a future vaccine should confer protection against more than one serotype.

Origem Anómala da Artéria Coronária Esquerda a Partir da Artéria Pulmonar

OBJECTIVE: To assess the frequency and severity of the anomalous origin of the left coronary artery (ALCA) from the pulmonary artery (PA). DESIGN OF THE STUDY: Prospective study of case series between March 1991 and December 1994. SETTING: Referral-based Paediatric Cardiology Department of a Tertiary Care Center. PATIENTS AND METHODS: Five consecutive patients (pts) with anomalous origin of the LCA from the PA; there were three infants aged 4 months and two children one 8 year and one 9 year old. There were three girls and two boys. All pts had clinical and 2D-echo and Doppler investigation prior to cardiac catheterization (CC). Indication for CC was based in the association of symptoms and signs of myocarditis or dilated cardiomyopathy of acute or subacute onset and electrocardiographic (ECG) signs of ischemia in infants. In older patients (pts) diagnosis was suspected mainly from ECG. During CC in all pts, aortograms and when necessary selective coronary angiograms were performed. Surgical correction was performed in all children. In two pts stress exercise ECG and stress Thallium studies before and after surgery were performed. RESULTS: two pts had "adult" an three had "infantile" type of ALCA from the PA. CC was performed and diagnosis was confirmed at surgery in all cases. In one child, correct diagnosis was made by ECO prior to CC and in one case LCA to PA fistula was suspected on Colour-Doppler study. No complications were attributed to CC. Several types of surgery were performed: reimplantation of the ALCA from the PA to the aorta (three pts); tunnel connection of the aorta to the ALCA via the PA (one pt) and left internal mammary to LCA anastomosis (one pt). Two infants died intraoperatively due to extensive myocardial infarction and poor left ventricular function. All the three survivors are asymptomatic after a mean follow up of 34 months. Two oldest pts are currently in New York Heart Association functional class I with normal ECG and improved myocardial perfusion on Thallium scan despite almost total occlusion of LCA at the site of implantation in the aorta as diagnosed on coronary angiogram. CONCLUSIONS: ALCA from PA is associated with major morbidity and mortality. Diagnosis should be suspected in pts with unexplained myocardial ischemia on ECG and even more if it is associated to clinical signs of dilated cardiomyopathy or myocarditis. Careful assessment on ECO and pulsed Doppler and colour flow mapping should make the diagnosis in most cases. Although surgery can be performed based only on ECO diagnosis, we strongly advise for angiography in all cases as in our experience there are false negative diagnosis by ECO. Preoperative Thallium studies can be useful for the selection of the type of surgery as pts with very little viable myocardium will not survive the establishment of a direct systemic to coronary blood flow and may be candidates for heart transplantation.

Psoríase

Pretende-se nestas Recomendações Terapêuticas dar uma visão global dos conhecimentos actuais sobre a patogenia, clínica e tratamento da psoríase. A educação dos doentes deve incidir na evicção dos factores desencadeantes (medicamentos, trauma, álcool, infecções, stress) e enfa tizar a acção benéfica da exposição solar controlada na psoríase. A terapêutica tópica emolientes, queratolíticos, derivados do alcatrão, antralina, corticosteróides tópicos, calcipotriol — é essencial no controlo da psoríase vulgar em placas e é importante como adjuvante nos casos mais graves; chama-se a atenção para os graus de potência e para os potenciais efeitos indesejáveis da corticoterapia tópica. A radiação UV isolada (fototerapia) ou em associação a fotos sensibilizantes (fotoquimioterapia) está indicada nalgumas formas de psoríase (gutata, em placas, palmo-plantar) refractárias aos tópicos. A terapêutica sistémica — retinóides, metotrexato, ciclosporina está reservada para as formas mais graves, extensas e refractárias de psoríase, sendo indispensável na psoríase eritrodérmica, pustulosa ou artropática. A estratégia actual visa obter o melhor índice terapêutico e dá particular importância às terapêuticas combinadas e/ou rotativas. Para finalizar, incluem-se alguns fluxogramas, com o objectivo de ordenar os passos a dar na abordagem do doente com psorfase e salientar a validade científica das diferentes terapêuticas disponíveis.