Divertículo da Uretra no Serviço de Cirurgia Pediátrica do Hospital D. Estefânia

Os autores apresentam uma revisão de casos clínicos de crianças, do Serviço de Cirurgia Pediátrica do Hospital D. Estefânia (HDE), com o diagnóstico de divertículo da uretra (DU)num período compreendido entre 1999 e 2005. Foram encontradas 5 crianças com este diagnóstico, 4 do sexo masculino e uma do sexo feminino, com idades (na altura do diagnóstico) compreendidas entre 6º dia de vida e os 11 anos. As primeiras manifestações clínicas incluíam infecções urinárias de repetição, hidronefrose bilateral com alterações da função renal, diminuição da força e calibre do jacto urinário e hematúria total. O diagnóstico foi feito por uretrocistografia permiccional (UCGPM) em três casos e uretroscopiaemdois. Emtrês casos optou-se por ressecção da porção distal do divertículo. Noutra criança optou-se por espera vigilante, tendo-se efectuado uretrografias periódicas para verificar a variação de volume do díverticulo. A uretroplastia com retalho perineal foi opção terapêutica para uma criança com DU congénito volumoso e obstrução ao esvaziamento vesical muito grave. Quer nas crianças em que se optou pela espera vigilante, quer nas que foram submetidas a tratamento cirúrgico, os resultados foram, globalmente bons: todas as crianças foram capazes de urinar com jacto normal no pós-operatório imediato, a função renal retomou a normalidade nas crianças em que se verificava elevação pré-operatória dos níveis séricos de creatinina e todas as crianças se encontram assintomáticas desde há nove meses.

Analgesia e Anestesia Obstétricas na Maternidade do Hospital D. Estefânia: Estudo Retrospectivo

Resumo: Por intermédio deste estudo, pretendeu-se verificar qual a realidade em termos de analgesia e anestesia obstétricas na Maternidade do Hospital D. Estefânia, ao longo de 4 anos. Para isso, foi realizado um estudo retrospectivo no período entre Janeiro de 2005 e Dezembro de 2008. Foram avaliados a classificação ASA das parturientes, o número total de partos, o número de parturientes submetidas a técnicas locorregionais e suas complicações, número de cesarianas e a necessidade de conversão de técnica regional para anestesia geral. No período do estudo, houve um total de 8291 partos, maioritariamente em mulheres ASA I, dos quais 2643 foram cesarianas. A maioria das parturientes (77%) foi submetida a uma técnica locorregional, para analgesia de trabalho de parto ou anestesia para cesariana, com baixa taxa de complicações (2,9%) e com rara necessidade de conversão para anestesia geral (3,5%). As autoras concluiram que, na Maternidade do Hospital D. Estefânia, a epidural continua a ser a técnica gold standard para analgesia do trabalho de parto, com raras complicações e permitindo a fácil conversão para anestesia para cesariana.

Intravenous Immunoglobulin for the Treatment of Quetiapin-Induced Bullous Pemphigoid in an HIV-Infected Patient

The authors present the case of a 48-year-old woman with HIV-­associated dementia treated with antiretroviral therapy and psychoactive drugs, to whom bullous pemphigoid(BP) was diagnosed. Given incomplete response to corticotherapy, and azathioprine-­induced bicytopenia,intravenous immunoglobulin(IVIG) was initiated. Despite transient disease control, recurrent flares suggested a persistent triggering factor. Specifically, quetiapin was implicated and discontinued with an immediate clinical response. Inadvertent re-­challenge with olanzapine(a related drug)led to a new eruption, confirming drug-­induced BP (DIBP). A total of six IVIG cycles were completed, without severe side effects to report, namely HIV disease progression. HIV-­related autoimmune bullous diseases are rare. Treatment of severe drug eruptions is primarily based on immunossupressive drugs, raising concerns regarding additional immunossupression. This case suggests IVIG as a valuable option for the treatment of BP in HIV patients. In addition, quetiapin should be added to the list of neuroleptics previously linked to DIBP.

Ferramentas de comunicação interna como estratégia na melhoria do ambiente de trabalho do D'Oliva Lisboa

Trabalho de Projecto apresentado para cumprimento dos requisitos necessários à obtenção do grau de Mestre em Ciências da Comunicação – Área de Especialização em Comunicação Estratégica

IgG4-Related Hashimoto's Thyroiditis - A New Variant of a Well Known Disease

Hashimoto's thyroiditis (HT) has been characterized for many years as a well-defined clinicopathologic entity, but is now considered a heterogeneous disease. IgG4-related HT is a new subtype characterized by thyroid inflammation rich in IgG4-positive plasma cells and marked fibrosis. It may be part of the systemic IgG4-related disease. We report a case of a 56-year-old Portuguese man who presented with a one-month history of progressive neck swelling and dysphagia. Laboratory testing revealed increased inflammatory parameters, subclinical hypothyroidism and very high levels of thyroid autoantibodies. Cervical ultrasound (US) demonstrated an enlarged and heterogeneous thyroid gland and two hypoechoic nodules. US-guided fine needle aspiration cytology was consistent with lymphocytic thyroiditis. The patient was submitted to total thyroidectomy and microscopic examination identified typical findings of HT, marked fibrosis limited within the thyroid capsule and lymphoplasmacytic infiltration, with >50 IgG4-positive plasma cells per high-power field and an IgG4/IgG ratio of >40%. After surgery, serum IgG4 concentration was high-normal. Symptoms relief and reduction in laboratory inflammatory parameters were noticed. Thyroid function is controlled with levothyroxine. To our knowledge we report the first case of IgG4-related HT in a non-Asian patient. We also perform a review of the literature regarding IgG4-related disease and IgG4-related HT. Our case highlights this new variant of the well known HT, and helps physicians in recognizing its main clinical features, allowing for proper diagnosis and treatment.

Meningite Assética Associada à Terapêutica com Imunoglobulina Endovenosa

Aseptic meningitis can be an adverse drug reaction to intravenous immunoglobulin. We describe a previously healthy 4-yearold boy, admitted for idiopathic thrombocytopaenic purpura. He received two infusions of intravenous immunoglobulin. Four hours after the last administration the patient developed a meningeal syndrome. Analysis of cerebrospinal fluid revealed 500 cells/μl (predominantly neutrophils) and normal biochemistry. Bacteriological and virological tests were negative. After 48h he was asymptomatic. Given the absence of other aetiological factors and the temporal relationship between the administration of immunoglobulin and the development of symptoms, we believe the patient had an aseptic meningitis related to intravenous immunoglobulin. This therapy may cause headache, fever and vomiting; however, lumbar puncture is not usually performed, so this complication may be underdiagnosed.

Leg Ulcers in Antiphospholipid Syndrome Secondary to Systemic Lupus Erythematosus Treated with Intravenous Immunoglobulin

BACKGROUND: Despite encouraging reports on the efficacy of intravenous immunoglobulin (IVIg) in antiphospholipid syndrome, the clinical value of this treatment is not well established, and most of the data are based on case reports and small series of patients. OBSERVATION: We describe the significant improvement of leg ulcers with IVIg in a 61-year-old female, with diabetes mellitus, venous peripherical insufficiency and secondary antiphospholipid syndrome to systemic lupus erythematosus. CONCLUSIONS: This case illustrates a rare cause of leg ulcers and documents that IVIg may be an effective adjuvant treatment in the management of selected patients with antiphospholipid syndrome when conventional strategies using subcutaneous heparin and low-dose aspirin are insufficient.

De infanta de Portugal a duquesa de Borgonha D. Isabel de Lencastre e Avis (1397-1429)

Dissertação apresentada para cumprimento dos requisitos necessários à obtenção do grau de Mestre em História, área de especialização em História Medieval

O Santuário de Santa Bárbara de Padrões – uma Perspectiva Religiosa e Artística na Lusitânia dos Séculos I a III d. C.

Dissertação apresentada para cumprimento dos requisitos necessários à obtenção do grau de Mestre em História da Arte – Área de especialização em História da Arte da Antiguidade

Triple Regimen with Rituximab, Plasmapheresis and Intravenous Immunoglobulin in the Treatment of Dialysis Dependent Acute Humoral-Mediated Rejection in Kidney Grafts

Introduction: The clinical importance of humoral-mediated acute rejection has been progressively recognised. Early recognition and treatment with plasmapheresis and intravenous immunoglobulin have recently improved short term prognosis. Case report: In this report we describe the clinical features of three 2nd transplant patients developing severe acute humoral rejection during the first week post-transplant while on anti-thymocyte globulin therapy. Treatment with plasmapheresis/ intravenous immunoglobulin/rituximab resulted in rapid reversal of oliguria,and recovery of renal function within the 1st week of treatment in 2/3 patients. Diagnosis was confirmed by graft biopsies revealing peritubular neutrophiles and C4d deposits. Sequential graft biopsies in all three patients revealed complete histological recovery within two weeks. One patient never recovered renal function, and one patient lost his graft at three months following hemorrhagic shock. After 2 years follow up, the remaining patient maintains a serum creatinine of 1.1mg/dl. Conclusion: The regimen using plasmapheresis plus intravenous immunoglobulin and rituximab was effective in rapidly reversing severe acute humoral rejection.

Testagem do 1 - beta - D - Ribofuranosil, 1.2.4 - triazole - 3 - carboxamide em pacientes com hepatite

Realizou-se estudo do tipo duplo anonimato em 18 pacientes com hepatite aguda benigna. O gruoo experimental foi testado com uma provável droga de ação antiviral: 1-BETA-D-RIBOFURANOSIL, 1,2,4-TRIAZOLE-3- CARBOXAMIDE. O grupo controle ingeriu um placebo de lactose. Teve-se especial cuidado na seleção de pacientes, incluindo apenas pacientes que preenchessem critérios bem estabelecidos. Os pacientes foram seguidos semanalmente, avaliando-os clínica e laboratorialmente. Os resultados não evidenciaram diferenças significativas entre os dois grupos, sugerindo-se estudos com casuística mais numerosa e em regime de internação hospitalar.

Comparação da capacidade ovipositora de Biomphalaria glabrata (Say, 1818), Biomphalaria tenagophila (D'Orbigni, 1835) e Biomphalaria straminea (Dunker, 1848), mantidas em laboratório

O autor comparou a capacidade ovipositora de 3 espécies de Planorbidae, Biomphalaria glabrata, B. tenagophila e B. straminea, durante um período de 30 dias, compreendidos entre os meses de Março e Abril de 1978. Registrou-se diariamente o número de ovos e desovas, referentes a 20 exemplares de cada espécie em questão. A análise estatística dos dados obtidos revelou que os exemplares de B. glabrata apresentaram maior capacidade ovipositora que as outras duas espécies em questão. A menor capacidade ovipositora foi atribuída à espécie B. tenagophila.

Henoch-Schönlein Purpura Associated with Pulmonary Adenocarcinoma

Introduction: The Henoch-Schönlein purpura (HSP) is an immunoglobulin A (IgA)-mediated smallvessel systemic vasculitis, rare in adults. The association with solid tumours has been described, especially with lung cancer. Case Report: We present the case of a 60-year-old Caucasian male, diagnosed with lung adenocarcinoma that underwent surgical resection without (neo)adjuvant theraphy. Two months latter he was admitted for abdominal pain, purpuric rash on his lower extremities and acute kidney injury, with serum creatinine (Scr) of 2 mg/dl. Urinalysis revealed haematuria and 24h proteinuria (P24h) of 1.5 g. The serum protein electrophoresis, complement components C3 and C4, circulating immune complexes, cryoglobulins, ANCA, ANA, anti-dsDNA and the remaining immunologic study as screening for viral infections (HCV, HBV and HIV) were negative. Renal ultrasound was normal and kidney biopsy revealed mild mesangial proliferation; 2 cellular glomerular crescents and 1 fibrinoid necrosis lesion; large amounts of red blood cell casts; lymphocytic infiltration in the intertubular interstitial capillaries; moderate arteriolar hyalinosis. Immunofluorescence demonstrated mesangial and parietal deposits of IgA. The diagnosis of HSP was assumed, and the patient started prednisolone 1 mg/kg/day. Ten months after diagnosis the patient’s baseline Scr is 1.4 mg/dl with P24h of 0.18g, without haematuria. Conclusion: Although this is a rare association and the exact mechanism behind the disease is yet unknown, physicians should be aware of it. The early recognition and treatment may prevent renal disease progression.