Mastocytosis: a Rare Case of Anaphylaxis in Paediatric Age and Literature Review

The term “mastocytosis” denotes a heterogeneous group of disorders characterised by abnormal growth and accumulation of mast cells (MC) in one or more organ systems. Symptoms result from MC chemical mediator’s release, pathologic infiltration of neoplastic MC in tissues or both. Multiple molecular, genetic and chromosomal defects seem to contribute to an autonomous growth, but somatic c-kit D816V mutation is more frequently encountered, especially in systemic disease. We present a literature review of mastocytosis and a rare case report of an 18 month-old-girl with a bullous dermatosis, respiratory distress and anaphylaxis, as clinical manifestations of mastocytosis. The developments of accepted classification systems and novel useful markers allowed a re-evaluation and updating of the classification of mastocytosis. In paediatric age cutaneous forms of disease prevail and may regress spontaneously. SM is more frequently diagnosed in adults and is a persistent(clonal) disease of bone marrow. The clinical course in these patients is variable.Today diagnostic criteria for each disease variant are reasonably well defined. There are, however, peculiarities, namely in paediatric age, that makes the diagnostic approach difficult. Systemic disease may pose differential diagnostic problems resulting from multiple organ systems involvement. Coversly, the “unexplained” appearance of those symptoms with no skin lesions should raise the suspicion of MC disease. This case is reported in order to stress the clinical severity and difficult diagnostic approach that paediatric mastocytosis may assume.

Adaptation and Localization of Open Source ERPs - The case of Parties in OFBiz

O software tem vindo a tornar-se uma parte importante de qualquer empresa, cobrindo várias áreas funcionais, tais como manufaturação, vendas ou recursos humanos. O facto de uma empresa possuir um software capaz de ligar todas ou a maior parte das suas áreas funcionais e de acomodar as suas regras de negócio permite que estas tenham acesso a dados em tempo real nos quais se podem basear para tomar decisões. Estes tipos de software podem ser categorizados como Enterprise resource planning (ERP). Tendo em conta que estes tipos de software têm um papel importante dentro de uma empresa, a aquisição dos mesmos é algo que deve ser bem estudado. As grandes empresas normalmente optam pela aquisição de soluções comerciais uma vez que estas tendem a ter mais funcionalidades, maior suporte e certificações. Os ERPs comerciais representam, no entanto, um esforço elevado para que a sua compra possa ser feita, o que limita a possibilidade de aquisição dos mesmos por parte de pequenas ou médias empresas. No entanto, tal como acontece com a maior parte dos tipos de software, existem alternativas open-source. Se nos colocássemos na posição de uma pequena empresa, a tentar iniciar o seu negócio em Portugal, que tipo de ERP seria suficiente para os nossos requisitos? Teríamos que optar por comprar uma solução comercial, ou uma solução open-source seria suficiente? E se optássemos por desenvolver uma solução à medida? Esta tese irá responder a estas questões focando-se apenas num dos componentes base de qualquer ERP, a gestão de entidades. O componente de gestão de entidades é responsável por gerir todas as entidades com as quais a empresa interage abrangindo colaboradores, clientes, fornecedores, etc. A nível de funcionalidades será feita uma comparação entre um ERP comercial e um ERP open-source. Como os ERPs tendem a ser soluções muito genéricas é comum que estes não implementem todos os requisitos de um negócio em particular, como tal os ERPs precisam de ser extensíveis e adaptáveis. Para perceber até que ponto a solução open-source é extensível será feita uma análise técnica ao seu código fonte e será feita uma implementação parcial de um gerador de ficheiros de auditoria requerido pela lei Portuguesa, o SAF-T (PT). Ao estudar e adaptar a solução open-source podemos especificar o que teria que ser desenvolvido para podermos criar uma solução à medida de raiz.

FIRST CASE OF HUMAN INFECTION BY Bertiella studeri (Blanchard, 1891) Stunkard,1940 (Cestoda; Anoplocephalidae) IN BRAZIL

SUMMARY Cestodes of the Bertiella genus are parasites of non-human primates found in Africa, Asia, Oceania and the Americas. Species Bertiella studeri and Bertiella mucronatacould, accidentally, infect human beings. The infection occurs from ingestion of mites from the Oribatida order containing cysticercoid larvae of the parasite. The objective of this report is to register the first case of human infection by Bertiella studeri in Brazil. Proglottids of the parasite, found in the stool sample of a two-and-a-half-year-old child, were fixed, stained and microscopically observed to evaluate its morphological characteristics. Eggs obtained from the proglottids were also studied. The gravid proglottids examined matched the description of the genus Bertiella. The eggs presented a round shape, with the average diameter of 43.7 µm, clearly showing the typical pyriform apparatus of B. studeri. The authors concluded that the child was infected with Bertiella studeri,based on Stunkard's (1940) description of the species. This is the fifth case of human Bertiellosis described in Brazil through morphometric analysis of the parasite, the third in Minas Gerais State and the first diagnosed case of Bertiella studeriin Brazil.

Atypical Case of Mediterranean Spotted Fever

The authors present a case of atypical severe (malignant) Mediterranean spotted fever, with a brief review on the subject. Although not previously described in Brazil, the possibility of imported cases, especially from Portuguese tourists, is real. This case report highlights the severe form of the disease and the possibility of atypical presentation with confounding differential diagnosis. A brief review of classical presentation is also done. The authors believe it is a valid paper and a good contribution to your Journal of Infectious Diseases. The content of the manuscript represents the views of the coauthors, and neither the corresponding author nor the coauthors have submitted duplicate or overlapping manuscripts elsewhere.

A Case of Kawasaki Disease in Portugal

This case report is believed to be the first case of Kawasaki disease in Portugal. An otherwise healthy 20 years old female was carefully examined and diagnosis of mucocutaneous lyrnphnode syndrome estab lished, based on: typical clinical picture, exclusion of other mimicking situations and middle term evolution of this patient. The A. A. wish to emphasize their diagnosis complied on C. D. C. criteria for Kawasaki disease. A short up dated briefing on this peculiar entity and geographycal pathology are included in this article.

Is This Septic Shock? A Rare Case of Distributive Shock

The authors report a rare case of shock in a patient without significant clinical history, admitted to the intensive care unit for suspected septic shock. The patient was initially treated with fluid therapy without improvement. A hypothesis of systemic capillary leak syndrome was postulated following the confirmation of severe hypoalbuminemia, hypotension, and hemoconcentration - a combination of three symptoms typical of the disease. The authors discussed the differential diagnosis and also conducted a review of the diagnosis and treatment of the disease.

A Case of Post-Radiotherapy Gastritis: Radiation Does Not Explain Everything

Hemorrhagic gastritis is a possible late toxicity outcome after radical radiotherapy but it is nowadays a very rare condition and most likely depends on other clinical factors. We report the case of a 77-year-old woman with a symptomatic solitary extramedullary intra-abdominal plasmacytoma and multiple gastric comorbidities, treated with external beam radiotherapy. Despite the good response to radiotherapy, the patient experienced multiple gastric bleeding a few months later, with the need of multiple treatments for its control. In this paper we will discuss in detail all aspects related to the different causes of hemorrhagic gastritis.

Studying the impact of co-authorship with external researchers:the case of research community in Portugal using social network analysis.

Project work presented as a partial requirement to obtain a Master Degree in Information Management

A Case of Late-Diagnosed Ovotesticular Disorder of Sex Development

We report acase of!ovotesticular disorder of sex development!(DSD) with ambiguous genitalia, 46XX presenting the clinical, laboratory, imaging and operative findings and highlighting the pertinent features of this case. Results of hormonal, genetic testing and histopathology findings are reviewed. Diagnosis of true hermaphroditism is well defined and the condition can be recognized even prenatally. Conservative gonadal surgery is the procedure of choice after the diagnosis of true hermaphroditism, if the risk of a gonadal malignancy is low. Continued follow-up is necessary because of the multiple psychological, gynecological and urological problems encountered postpubertally by these patients.

The use of technology in Portuguese hospitals: The case of MRI

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Economics from the NOVA – School of Business and Economics

Dimensions of source credibility in the case of user-generated advertisements

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA – School of Business and Economics

How to engage low category users through social media: The case of the make-up sector in Portugal

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA – School of Business and Economics

Corporate social responsibility reporting: The case of Portuguese hospitals

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA – School of Business and Economics

Measuring the impact with the social return on investment: the case of the social innovation HUB

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Economics from the NOVA – School of Business and Economics

Corporate social responsibility and its effect on image and reputation: The case of L’Oréal and its acquisition of The Body Shop

A Work Project, presented as part of the requirements for the Award of a Masters Degree in Management from the NOVA – School of Business and Economics