V��lvula A��rtica Quadric��spide. Casu��stica de 10 Anos e Revis��o da Literatura

Introdu����o: A v��lvula a��rtica quadric��spide �� uma malforma����o rara, com uma incid��ncia estimada de 0,003 a 0,043% de todas as cardiopatias cong��nitas. Surge habitualmente como uma anomalia cong��nita isolada, podendo igualmente estar associada a outras malforma����es, sendo as mais frequentes as anomalias das art��rias coron��rias. A tecnologia actual permite o diagn��stico n��o invasivo na grande maioria das situa����es. A sua hist��ria natural �� a evolu����o para a insufici��ncia, rara antes da idade adulta. Objectivos: Revis��o dos casos de v��lvula a��rtica quadric��spide diagnosticados nos ��ltimos 10 anos num centro terci��rio de Cardiologia Pedi��trica. Material e M��todos: Revis��o retrospectiva do processo cl��nico dos doentes aos quais foi detectada uma v��lvula a��rtica quadric��spide, entre Janeiro de 2000 e Dezembro de 2009. Resultados: Nos ��ltimos 10 anos, foram diagnosticados quatro casos de v��lvula a��rtica quadric��spide, em crian��as com idades compreendidas entre os 6 meses e os 8 anos, duas do sexo masculino. Em tr��s casos, os quatro folhetos eram de dimens��es semelhantes, que �� o achado mais frequente. Duas das v��lvulas eram normofuncionantes e duas apresentavam insufici��ncia m��nima. Todos os doentes apresentavam outras malforma����es card��acas associadas (uma comunica����o interauricular, duas comunica����es interventriculares, uma estenose��upravalvular a��rtica e uma v��lvula pulmonar quadric��spide). Um doente tinha tamb��m o diagn��stico de S��ndrome de Williams. Com um tempo de seguimento mediano de 2 anos [0 --- 9], todos os doentes se mantiveram assintom��ticos e n��o requereram tratamento m��dico ou cir��rgico para a v��lvula a��rtica. Conclus��o: O diagn��stico de v��lvula a��rtica quadric��spide �� raro, sobretudo em idade pedi��trica, quando a maioria dos doentes s��o assintom��ticos e apresentam v��lvulas normofuncionantes. Nesta casu��stica, metade apresentava insufici��ncia a��rtica m��nima. Ao contr��rio do que est�� descrito na literatura, todos os doentes apresentavam malforma����es card��acas concomitantes. Descrevemos pela primeira vez a associa����o com a S��ndrome de Williams. Estes doentes dever��o manter seguimento em ambulat��rio, de forma a detectar atempadamente o aparecimento ou agravamento de altera����es funcionais e permitir uma interven����o terap��utica oportuna.

Fusobacterium necrophorum causing infective endocarditis and liver and splenic abscesses

A 25-year-old male without prior co-morbidities was admitted to hospital with Fusobacterium necrophorum bacteremia, where he was found to have liver and splenic abscesses. Further evaluation with echocardiography revealed a bicuspid aortic valve with severe insufficiency and a 1.68 x 0.86 cm vegetation. The patient required abscess drainage, intravenous antimicrobial therapy and aortic valve replacement. Complete resolution of the infection was achieved after valve replacement and a prolonged course of intravenous antimicrobial therapy. A brief analysis of the patient's clinical course and review of the literature is presented.

Transoesophageal Echocardiography for Monitoring Liver Surgery: Data from a Pilot Study

A pilot study aimed to introduce intraoperative monitoring of liver surgery using transoesophageal echocardiography (TEE) is described. A set of TEE measurements was established as a protocol, consisting of left atrial (LA) dimension at the aortic valve plane; mitral velocity flow integral, calculation of stroke volume and cardiac output (CO); mitral annular plane systolic excursion; finally, right atrial area. A total of 165 measurements (on 21 patients) were performed, 31 occurring during hypotension. The conclusions reached were during acute blood loss LA dimension changed earlier than CVP, and, in one patient, a dynamic left ventricular (LV) obstruction was observed; in 3 patients a transient LV systolic dysfunction was documented. The comparison between 39 CO paired measurements obtained by TEE and PiCCO2 revealed a statistically significant correlation (P < 0.001, r = 0.83). In this pilot study TEE successfully answered the questions raised by the anesthesiologists. Larger cohort studies are needed to address this issue.

Implanta����o Percut��nea de V��lvulas Pulmonares: Experi��ncia Inicial

Os doentes com cardiopatias submetidos a interven����o cir��rgica com implanta����o de condutos entre o ventr��culo direito e a art��ria pulmonar (VD-AP) podem desenvolver estenose e/ou insufici��ncia pulmonares ao longo do tempo. Nestas situa����es, associadas a arritmias card��acas, disfun����o ventricular direita e morte s��bita, a re-interven����o cir��rgica permanece um desafio pela complexidade, mortalidade e morbilidade significativas. A implanta����o percut��nea de v��lvulas pulmonares (IPVP), recentemente desenvolvida, representa uma abordagem alternativa para estes doentes. Objectivo: Reportar a experi��ncia inicial com a IPVP, analisando o seu impacto no manejo destes doentes. Material e M��todos: Avalia����o prospectiva dos dados cl��nicos, ecocardiogr��ficos, da resson��ncia magn��tica, hemodin��micos e angiogr��ficos dos doentes submetidos a IPVP. Resultados: Seis doentes em classe funcional igual ou superior a II com disfun����o do conduto VD-AP foram submetidos a IPVP. Tinham todos evid��ncia de insufici��ncia e 5/6 de estenose. O procedimento consistiu na implanta����o de stents n��o cobertos no conduto para refor��ar a sua rigidez e prevenir fracturas dos stents valvulados pulmonares Melody �� (Medtronic), que foram em seguida implantados com sucesso em todos. Obteve-se uma redu����o da press��o ventricular direita (94��27 para 44��7mmHg), da rela����o entre a press��o ventricular direita e esquerda (94��27 para 44��7%), do gradiente do conduto (65��28 para 11��4mmHg) e aus��ncia de regurgita����o pulmonar. O procedimento teve uma dura����o mediana de 180 minutos e decorreu sem complica����es major. Os doentes tiveram alta em mediana dois dias ap��s o procedimento. Na ��ltima avalia����o, em mediana 7,8 meses ap��s o procedimento, os doentes encontramse em classe funcional I (5) ou II (1), sem evid��ncia n��o invasiva de disfun����o do conduto. Conclus��es: Os nossos resultados acompanham a experi��ncia actual com esta modalidade terap��utica, com excelentes resultados nestes grupo de doentes complexos. A implementa����o da IPVP em doentes com cardiopatia cong��nita que requerem re-interven����o no tracto de sa��da do ventr��culo direito tem-se revelado uma t��cnica promissora, embora complexa, alternativa a mais uma interven����o cir��rgica.

Mosaic Trisomy 18 in a Five-Month-Old Infant - Case Report

Individuals with mosaic trisomy 18, only approximately 5% of all trisomy 18 cases, carry both a trisomy 18 and an euploid cell line. Their clinical findings are highly variable, from the absence of dysmorphic features to the complete trisomy 18 syndrome. A five month old daughter of a 38-year-old mother, with vomiting and feeding problems, was referred to our department. She was undernourished and had axial hypotony and developmental delay, an irregular pattern of hypopigmentation on the right side of the abdomen, and moderate sagittal body asymmetry with left-side muscular hemihypotrophy.Mild craniofacial dysmorphy included dolichocephaly, frontal bossing, prominent occiput, long downslanting palpebral fissures, hypertelorism, and retrognathia. A complex heart defect with atrial and ventricular septal defects, pulmonary artery stenosis, and bicuspid aortic valve was identified. Cytogenetic analysis revealedmosaic trisomy 18with trisomy in 90%of peripheral lymphocytes and 17%of skin fibroblasts.This case adds to our knowledge of the phenotypic spectrum and the natural history of mosaic trisomy 18 by adding a dysmorphic feature and a cardiac abnormality that, to the best of our knowledge, had not been previously described.

Uso Sustent��vel de Vapor

Em 2006, a IEA (Ag��ncia Internacional de Energia), publicou alguns estudos de consumos mundiais de energia. Naquela altura, apontava na fabrica����o de produtos, um consumo mundial de energia el��trica, de origem f��ssil de cerca 86,16 EJ/ano (86,16��018 J) e um consumo de energia nos sistemas de vapor de 32,75 EJ/ano. Evidenciou tamb��m nesses estudos que o potencial de poupan��a de energia nos sistemas de vapor era de 3,27 EJ/ano. Ou seja, quase tanto como a energia consumida nos sistemas de vapor da U.E. N��o se encontraram n��meros relativamente a Portugal, mas comparativamente com outros Pa��ses publicitados com alguma similaridade, o consumo de energia em vapor rondar�� 0,2 EJ/ano e por conseguinte um potencial de poupan��a de cerca 0,02 EJ/ano, ou 5,6 �� 106 MWh/ano ou uma pot��ncia de 646 MW, mais do que a pot��ncia de cinco barragens Crestuma/Lever! Trata-se efetivamente de muita energia; interessa por isso perceber o onde e o porqu�� deste desperd��cio. De um modo muito modesto, pretende-se com este trabalho dar algum contributo neste sentido. Procurou-se evidenciar as possibilidades reais de os utilizadores de vapor de ��gua na ind��stria reduzirem os consumos de energia associados �� sua produ����o. N��o est��o em causa as diferentes formas de energia para a gera����o de vapor, sejam de origem f��ssil ou renov��vel; interessou neste trabalho estudar o modo de como �� manuseado o vapor na sua fun����o de transporte de energia t��rmica, e de como este poder�� ser melhorado na sua efici��ncia de ced��ncia de calor, idealmente com menor consumo de energia. Com efeito, de que servir�� se se optou por substituir o tipo de queima para uma mais sustent��vel se a jusante se continuarem a verificarem desperd��cios, descarga exagerada nas purgas das caldeiras com perda de calor associada, emiss��es permanentes de vapor para a atmosfera em tanques de condensado, perdas por v��lvulas nos vedantes, purgadores avariados abertos, press��o de vapor exageradamente alta atendendo ��s temperaturas necess��rias, ���layouts��� do sistema de distribui����o mal desenhados, inexist��ncia de registos de produ����o e consumos de vapor, etc. A base de organiza����o deste estudo foi o ciclo de vapor: produ����o, distribui����o, consumo e recupera����o de condensado. Pareceu importante incluir tamb��m o tratamento de ��gua, atendendo ��s implica����es na transfer��ncia de calor das superf��cies com incrusta����es. Na produ����o de vapor, verifica-se que os maiores problemas de perda de energia t��m a ver com a falta de controlo, no excesso de ar e purgas das caldeiras em exagero. Na distribui����o de vapor aborda-se o dimensionamento das tubagens, necessidade de purgas a v montante das v��lvulas de controlo, a redu����o de press��o com v��lvulas redutoras tradicionais; ser�� de destacar a experi��ncia americana no uso de micro turbinas para a redu����o de press��o com produ����o simult��nea de eletricidade. Em Portugal n��o se conhecem instala����es com esta op����o. Fabricantes da Rep��blica Checa e ��ustria, t��m tido sucesso em algumas dezenas de instala����es de redu����o de press��o em diversos pa��ses europeus (UK, Alemanha, R. Checa, Fran��a, etc.). Para determina����o de consumos de vapor, para projeto ou mesmo para estimativa em m��quinas existentes, disponibiliza-se uma s��rie de equa����es para os casos mais comuns. D��-se especial relevo ao problema que se verifica numa grande percentagem de permutadores de calor, que �� a estagna����o de condensado - ���stalled conditions���. Tenta-se tamb��m evidenciar as vantagens da recupera����o de vapor de flash (infelizmente de pouca tradi����o em Portugal), e a aplica����o de termocompressores. Finalmente aborda-se o benchmarking e monitoriza����o, quer dos custos de vapor quer dos consumos espec��ficos dos produtos. Esta abordagem �� algo ligeira, por manifesta falta de estudos publicados. Como trabalhos pr��ticos, foram efetuados levantamentos a instala����es de vapor em diversos sectores de atividades; 1. ISEP - Laborat��rio de Qu��mica. Porto, 2. Prio Energy - F��brica de Biocombust��veis. Porto de Aveiro. 3. Inapal Pl��sticos. Componentes de Autom��vel. Le��a do Balio, 4. Malhas Sonix. Tinturaria T��xtil. Barcelos, 5. Uma instala����o de cart��o canelado e uma instala����o de alimentos derivados de soja. Tamb��m se inclui um estudo comparativo de custos de vapor usado nos hospitais: quando produzido por geradores de vapor com queima de combust��vel e quando �� produzido por pequenos geradores el��tricos. Os resultados est��o resumidos em tabelas e conclui-se que se o potencial de poupan��a se aproxima do referido no in��cio deste trabalho.

Iatrogenic Aortic Pseudoaneurysm: a Forgotten Complication

Pseudoaneurysms of the ascending aorta are a rare complication of cardiac surgery. However, the poor prognosis associated with this condition if untreated makes early diagnosis and treatment important. We present the case of a 66-year-old woman who had undergone mitral valvuloplasty 12 days previously, who was admitted with a diagnosis of new-onset atrial fibrillation. The transthoracic echocardiogram showed a clot in the right atrium and anticoagulation was initiated, followed by antibiotic therapy. After further investigation, the patient was diagnosed with a pseudoaneurysm of the ascending aorta and underwent surgical repair, followed by six weeks of antibiotic therapy. She was readmitted six months later for an abscess of the lower sternum and mediastinum. After a conservative approach with antibiotics and local drainage failed, recurrence of a large pseudoaneurysm compressing the superior vena cava was documented. A third operation was performed to debride the infected tissue and to place an aortic allograft. There were no postoperative complications.

Insufici��ncia Pulmonar Valvular Cong��nita

Descrevem-se 4 casos de Insufici��ncia Pulmonar Valvular Cong��nita com septo interventricular intacto em crian��as de sexo masculino com idades compreendidas entre os 20 meses e os 10 anos, na 1a observa����o na Consulta de Cardiologia Pedi��trica. 0 diagn��stico cl��nico de regurgita����o pulmonar foi confirmado por electrocardiograma, fonocardiograma e radiografia de t��rax em todos os doentes e tamb��m por ecocardiograma, exame hemodin��mico e angiocardiogr��fico em 3 deles. Ap��s um follow-up que variou entre 1 e 9 anos, m��dia 5 anos e 2 meses, concluiu-se que a Insufici��ncia Pulmonar Valvular Cong��nita, como cardiopatia isolada, �� bem tolerada em idades pedi��tricas e compat��vel com desenvolvimento f��sico e actividade normais.

Hepatic Actinomycosis

Actinomycosis is a rare disorder caused by an anaerobic gram-positive bacillus (Actinomyces), predominantly by the Actinomyces israelii species. Only 20% of cases show an abdominal manifestation, the appendix and ileocecal valve being the most frequent locations. Definitive diagnosis is based on microbiological cultures, microscopy or macroscopy examination. Nevertheless, histological examination of the percutaneous biopsy and blood microbiological cultures are rarely positives. Preoperative diagnosis is hampered by the lack of specific clinical and imaging manifestations, which often mimic malignancy. The rate of preoperative diagnosis is less than 10%, however, the outcome is excellent, with a low mortality rate. The authors describe the case of a patient who was diagnosed with primary hepatic actinomycosis only by a histological examination of the surgical specimen of left hepatectomy extended to segments V and VIII, for suspected malignant lesion. This case demonstrates the difficulties in diagnosing hepatic actinomycosis.

Aortic and Tricuspid Endocarditis in Hemodialysis Patient with Systemic and Pulmonary Embolism

This is a case report of a 43-year-old Caucasian male with end-stage renal disease being treated with hemodialysis and infective endocarditis in the aortic and tricuspid valves. The clinical presentation was dominated by neurologic impairment with cerebral embolism and hemorrhagic components. A thoracoabdominal computerized tomography scan revealed septic pulmonary embolus. The patient underwent empirical antibiotherapy with ceftriaxone, gentamicin and vancomycin, and the therapy was changed to flucloxacilin and gentamicin after the isolation of S. aureus in blood cultures. The multidisciplinary team determined that the patient should undergo valve replacement after the stabilization of the intracranial hemorrhage; however, on the 8th day of hospitalization, the patient entered cardiac arrest due to a massive septic pulmonary embolism and died. Despite the risk of aggravation of the hemorrhagic cerebral lesion, early surgical intervention should be considered in high-risk patients.

Towards cryogenic liquid ���vapor energy storage units for space applications

Dissertation to obtain the Doctoral degree in Physics Engineering

Validation of alternative additives for paste production

Dissertation presented to confer Master Degree in Chemical and Biochemical Engineering

Atherogenesis and atherosclerosis in primary antiphospholipid syndrome

ABSTRACT: In the late seventies the term ���Haematological Stress Syndrome��� defined some haematological abnormalities appearing in the course of acute and chronic disorders, such as raised plasma levels of fibrinogen (FNG) and factor VIII, reduced fibrinolytic activity and hyperviscosity. In the early nineties the ���Membrane stress syndrome hypothesis��� proposed the unification of the concepts of haematological stress syndrome with those of oxidation, inflammation and immune activation to explain the pathogenesis of the antiphospholipid syndrome (APS) Antiphospholipid antibodies, coagulation, fibrinolysis and thrombosis. This chapter investigated the occurrence of the ���Haematological Stress Syndrome��� and thrombosis in 144 participants positive for aPL detected by clotting and immune tests. Among the clotting assays for the detection of lupus anticoagulant, dilute Russell's viper venom time better correlated with a history of venous thrombosis than activated partial thromboplastin time (p<0.0002 vs p<0.009) and was the only test correlated with a history of arterial thrombosis (p<0.01). By regression analysis, serum levels of IgG anticardiolipin antibodies (aCL) associated with the number of venous occlusions (p<0.001). With regards to FNG and von Willebrand factor (vWF), the former rose by 36% (95% CI; 21%, 53%) and the latter by 50% (95% CI; 29%, 75%) at the first venous occlusion and remained unchanged after subsequent occlusions. At variance FNG rose by 45% (95% CI; 31%, 60%) per arterial occlusion and vWF by 27% (95% CI; 10%, 47%) per arterial occlusion throughout. The coagulation/fibrinolytic balance was cross-sectionally evaluated on 18 thrombotic PAPS patients, 18 subjects with persistence of idiopathic aPL and in healthy controls. Markers of thrombin generation prothrombin fragment 1+2 (F1+2), thrombin-antithrombin complex (TAT) and of fibrin turnover D-Dimer (D-D) were higher in thrombotic (p=0.006)and non-thrombotic subjects (p=0.0001) than in controls as were those of D-D (p<0.0001 and p=0.003 respectively). TAT levels did not differ. Gender analysed data revealed blunted tPA release (hence a negative venous occlusion test) in thrombotic females but neither in thrombotic males (p=0.01) nor in asymptomatic subjects of either sex. Also, in both patient groups females had higher mean PAI than males (p<0.0002) and control females (p<0.02). The activity of factor XIII (FXIIIa) was evaluated was evaluated in 29 patients with PAPS, 14 persistent carriers of aPL without thrombosis, 24 thrombotic patients with inherited thrombophilia, 28 healthy controls and 32 patients with mitral and aortic valve prosthesis as controls for FXIII only. FXIIIa was highest in PAPS (p=0.001), particularly in patients with multiple (n=12) than single occlusion (p=0.02) and in correlation with PAI (p=0.003) and FNG (p=0.005). Moreover FXIIIa was strongly associated with IgG aCL and IgG anti-���2GPI (p=0.005 for both) in the PAPS group and to a lesser degree in the aPL group (FXIIIa with IgG aCL, p=0.02, with IgG anti-���2GPI, p=0.04). Altogether these results indicate: 1) a differential relationship of aPL, vWF and FNG with venous and arterial thrombosis; 2) heightened thrombin generation, accelerated fibrin turnover and fibrinolysis abnormalities also in asymptomatic carriers of aPLs; 3) enhanced FXIIIa that may contribute to atherothrombosis via increased fibrin/fibrinogen cross-linking. Lipid profile, lipid peroxidation and anti-lipoprotein antibodies in thrombotic primary antiphospholipid syndrome. Given the atherogenic lipid profile of SLE, the same possibility was explored in PAPS by comparing high-density lipoprotein (HDL), low-density lipoprotein (LDL), total cholesterol (CHO), apolipoprotein AI (ApoAI), apolipoprotein B (ApoB), triglycerides (TG), anti-lipoprotein antibodies, beta-2-glycoprotein I complexed to oxidized low-density lipoprotein (oxLDL-���2GPI) and C-reactive protein (CRP) in 34 thrombotic PAPS patients compared to 36 thrombotic patients with inherited thrombophilia (IT), to 18 subjects persistently positive for antiphospholipid antibodies (aPL) with no underlying autoimmune or non-autoimmune disorders and to 28 healthy controls. Average concentrations of HDL (p<0.0001), LDL (p<0.0001), CHO (p=0.0002), ApoAI (p=0.002) were lower in PAPS whereas average TRY was higher (p=0.01) than other groups. Moreover PAPS showed higher IgG anti-HDL (p=0.01) and IgG anti-ApoAI (p<0.0001) as well as greater average oxLDL-���2GPI (p=0.001) and CRP (p=0.003). Within PAPS, IgG anti-HDL correlated negatively to HDL (p=0.004) and was an independent predictor of oxLDL-���2GPI (p=0.009). HDL and ApoAI correlated negatively with CRP (p=0.001 and p=0.007, respectively). IgG anti-HDL may hamper the antioxidant and anti-inflammatory effect of HDL favouring low-grade inflammation and enhanced oxidation in thrombotic PAPS. Indeed plasma 8-epi-prostaglandin F2�� (a very specific marker of lipid peroxidation) was significantly higher in 10 patients with PAPS than 10 age and sex matched healthy subjects (p=0.0002) and strongly related to the titre of plasma IgG aCL (r=0.89, p=0.0004). Hence oxidative stress, a major player in atherogenesis, also characterises PAPS. Nitric oxide and nitrative stress in thrombotic primary antiphosholipid syndrome. Oxidative stress goes hand in hand with nitrative stress and to address the latter plasma nitrotyrosine (NT, marker of nitrative stress), nitrite (NO2-) and nitrate (NO3-) were measured in 46 thrombotic PAPS patients, 21 asymptomatic but persistent carriers of antiphospholipid antibodies (PCaPL), 38 patients with inherited thrombophilia (IT), 33 patients with systemic lupus erythematosus (SLE) and 29 healthy controls (CTR). Average crude NT was higher in PAPS and SLE (p=0.01) whereas average plasma NO2- was lower in PAPS and average NO3- highest in SLE (p<0.0001). In PAPS, IgG aCL titer and number of vascular occlusions negatively predicted NO2-, (p=0.03 and p=0.001, respectively) whereas arterial occlusions and smoking positively predicted NO3- (p=0.05 and p=0.005). Moreover CRP (an inflammatory marker) positively predicted NT (p=0.004). Nitric oxide metabolites relates to type and number of vascular occlusions and to aPL titers, whereas nitrative stress relates to low grade marker) positively predicted NT (p=0.004). Nitric oxide metabolites relates to type and number of vascular occlusions and to aPL titers, whereas nitrative stress relates to low grade inflammation and both phenomena may have implications for thrombosis and atherosclerosis in PAPS Inflammation and immune activation in thrombotic primary antiphospholipid syndrome. To investigate inflammation and immune activation in thrombotic PAPS high-sensitivity CRP (hs-CRP), serum amyloid A (SAA), oxLDL-���2GPI, CRP bound to oxLDL-���2GPI (CRP-oxLDL-���2GPI) (as inflammatory markers) neopterin (NPT) and soluble CD14 (sCD14) (as immune activation markers) were measured by ELISA in 41 PAPS patients, in 44 patients with inherited thrombophilia (IT) and 39 controls (CTR). Compared to other groups, PAPS presented with higher plasma concentrations of inflammatory, hs-CRP (p=0.0004), SAA (p<0.01), CRP-oxLDL-���2GPI (p=0.0004) and immune activation markers, NPT (p<0.0001) and sCD14 (p=0.007). By regression analysis SAA independently predicted thrombosis number (p=0.003) and NPT independently predicted thrombosis type (arterial, p=0.03) and number (p=0.04). These data confirm that low-grade inflammation and immune activation occur and relate to vascular features of PAPS. Antiphosholipid antibodies, haemostatic variables and atherosclerosis in thrombotic primary antiphospholipid syndrome To evaluate whether IgG aCL titre, haemostatic variables and the lipid profile bore any relationship to the intima media thickness (IMT) of carotid arteries high-resolution sonography was applied to the common carotid (CC), carotid bifurcation (CB) and internal carotid (IC) of 42 aPL subjects, 29 with primary thrombotic antiphospholipid syndrome and 13 with persistence of aPL in the absence of any underlying disorder. The following were measured: plasma FNG, vWF, PAI, homocysteine (HC), CHO, TG, HDL, LDL, platelet numbers and aCL of IgG and IgM isotype. By multiple regression analysis, IgG aCL titre independently predicted IMT at all carotid segments examined (p always <0.005). Plasma FNG and HC independently predicted IMT at the CB (p=0.001 and p<0.0001, respectively) and IC (p=0.03 and p<0.0001, respectively). These data strongly support an atherogenic role for IgG aCL in patients with aPL in addition to traditional risk factors. The atherosclerosis hypothesis was investigated in an age and sex-matched case-double-control study including 49 thrombotic PAPS patients (18 M, 31 F, mean age 37 �� 11), 49 thrombotic patients for IT and 49 healthy subjects. Average IMT was always greater in PAPS than control patients (CC: p=0.004, CB: p=0.013, IC: p=0.001). By dividing participants into age tertiles the IMT was greater in the second (CC: p=0.003, CB: p=0.023, IC: p=0.003) and third tertiles (CC: p=0.03, CB: p=0.004, IC: p=0.007). Conclusion: Coagulation activation, fibrinolysis depression, hightened fibrin turnover, oxidative and nitrative stress in parallel with low grade inflammation and immune activation characterise thrombotic PAPS: all these are early atherogenic processes and contribute to the demonstrated premature atherosclerosis that should be considered a clinical feature of PAPS.

Efeitos da laquea����o proximal dos vasos curtos e da fundoplicatura total sobre a microcircula����o do fundo g��strico : estudo microvascular em cobaias

RESUMO: A opera����o de Nissen, por laparoscopia, �� considerada a cirurgia antirefluxo mais adequada por ser a que melhor replica a fisiologia normal da v��lvula gastresof��gica na maioria dos doentes com sintomas t��picos de doen��a do refluxo gastresof��gico (DRGE). S��o crit��rios t��cnicos o encerramento seguro dos pilares do diafragma e a cria����o de fundoplicatura completa (360 graus), curta (inferior a dois cent��metros), lassa e sem tens��o ��� desiderando para o qual a laquea����o proximal dos vasos curtos g��stricos �� crucial. Realizei a opera����o de Nissen, por laparoscopia, em sessenta mulheres e quarenta homens com DRGE, sem mortalidade operat��ria, no Servi��o de Cirurgia 6 do Hospital dos Capuchos, CHLC, EPE. Os cem doentes apresentavam m��dia et��ria de 46 anos e queixas, com tempo de evolu����o entre 1 e 43 anos, de pirose (90%), regurgita����o (80%), azia (73%), epigastralgias (54%). A endoscopia alta revelou esofagite de grau Savary-Miller 0-I (62%), II (23%), III (8%), IV (7%); h��rnia de deslizamento (71%), h��rnia paraesof��gica (8%), sem h��rnia (21%); a pHmetria de 24h diagnosticou padr��o misto (38%), levantado (20%), deitado (20%), inconclusiva (22%) e a manometria diagnosticou EEI hipot��nico (35%), perist��lise esof��gica normal (88%), hipomotilidade ligeira (5%) e foi omissa (7%). H��rnia hiatal, esofagite grave, inefic��cia do controlo sintom��tico com inibidor da bomba de prot��es e desejo de descontinuidade terap��utica constitu��ram as indica����es para tratamento cir��rgico. Por celioscopia, efetuei laquea����o dos vasos curtos g��stricos (70%), cruroplastia e fundoplicatura total (seda 2/0), curta (dimens��o m��dia 1,5-2 cm), lassa, sem tens��o e sem calibra����o intraoperat��ria do es��fago. A fundoplicatura de Nissen laparosc��pica mostrou-se segura e eficaz no tratamento da DRGE. A sua idoneidade foi ainda comprovada pela normaliza����o da pHmetria de 24 horas e da manometria p��s-operat��rias, com significado estat��stico, num grupo de catorze volunt��rios assintom��ticos. Em catamnese com recuo m��dio 30,7 meses 94% dos indiv��duos persistem assintom��ticos. Interrogando-me acerca das repercuss��es desta opera����o sobre a microcircula����o do fundo g��strico coloquei, como premissa, a possibilidade de na opera����o de Nissen a laquea����o dos vasos curtos poder induzir modifica����o no di��metro arteriolar da parede do fundo g��strico. Para pesquisar a influ��ncia da laquea����o dos vasos curtos g��stricos e da fundoplicatura total sobre o calibre arteriolar da parede do est��mago no c��rdia, no fundo e na regi��o dos vasos curtos g��stricos, idealizei um Projeto de investiga����o experimental em cobaias. O Projeto foi desenvolvido no Centro de Investiga����o do Departamento de Anatomia da FCM-UNL. Para a sua realiza����o obtive autoriza����o da Comiss��o Cient��fica e Pedag��gica da FCM-UNL, requeri a acredita����o como investigador �� Dire����o Geral de Veterin��ria e, por recorrer �� utiliza����o de animais, submeti-o �� Comiss��o de ��tica da FCM-UNL, que o aprovou por unanimidade. Para limitar o n��mero de animais utilizados ao m��nimo necess��rio, calculei, por m��todo estat��stico, a quantidade de cobaias necess��rias. Subdividindo-as num grupo de ensaio (GE), onde realizei a opera����o de Nissen, e num grupo de controlo (GC), onde apenas procedi a tra����o g��strica, defini e apliquei protocolos de anestesia, de cirurgia e de eutan��sia, segundo os princ��pios dos 3R ��� Replacement, Reduction, Refinement da t��cnica de experimenta����o humana de Russell e Burch (1959) ��� uma estrutura ��tica amplamente aceite para a realiza����o de experimenta����o cient��fica humanizada com animais. A utiliza����o das t��cnicas de estudo angiomorfol��gico permitiu-me analisar e descrever a anatomia normal, a vasculariza����o arterial macrosc��pica, a microangioarquitetura, por microscopia eletr��nica de varrimento de moldes de corros��o vascular, e a histologia da parede do est��mago da cobaia. Procedi, tamb��m, �� defini����o dos crit��rios morfol��gicos que considerei suscet��veis de valida����o deste modelo animal para o estudo proposto. Por raz��es acad��micas, foi necess��rio abreviar o Projeto encurtando, em cerca de dois anos, o prazo dispon��vel para conclus��o do estudo. Apreciando-o com o Gabinete de An��lise Epidemiol��gica e Estat��stica do Centro de Investiga����o do CHLC, EPE, optou-se, perante a escassez de elementos ap��s j�� terem sido recrutados 46 animais, por uma amostra, suplementar, de dimens��o de conveni��ncia de oito cobaias (quatro em cada grupo), condicionada pelo limite temporal universit��rio e pelo respeito pela dignidade dos animais. Neste subgrupo procedi, por microscopia eletr��nica de varrimento, �� medi����o dos calibres arteriolares nos moldes vasculares do c��rdia, do fundo e da zona dos vasos curtos g��stricos tanto no GC como no GE efetuando 469 medi����es no primeiro e 461 no ��ltimo. Os dados foram enviados ao Centro de Investiga����o do CHLC, EPE que procedeu �� sua an��lise estat��stica (ANOVA). A referida an��lise revelou que as arter��olas do plexo mucoso e as do plexo submucoso do c��rdia, do fundo e da regi��o dos vasos curtos g��stricos, mostraram aumento de calibre no GE. O aumento foi, estatisticamente, significativo por ser superior a 50% do calibre do GC. Nos vasos curtos, a diferen��a foi mais pequena, mas persistiu sendo, estatisticamente, significativa. Os vasos retos dilataram na base, na sua emerg��ncia do plexo seroso, apenas no fundo g��strico. Na cobaia a opera����o de Nissen ��� fundoplicatura total com laquea����o dos vasos curtos g��stricos ���, provocou vasodilata����o arteriolar do fundo g��strico. Considero que essa vasodilata����o constituiu acomoda����o �� modifica����o introduzida e infiro que o mesmo possa acontecer no ser humano. Admito, assim, que tamb��m ocorra vasodilata����o no ser humano, na sequ��ncia da laquea����o dos vasos curtos g��stricos, pela analogia microvascular entre as duas esp��cies e que essa vasodilata����o corresponda, igualmente, a um mecanismo de adapta����o arteriolar visando, por exemplo, suprir a perda incorrida pela laquea����o. A associa����o experimental entre laquea����o dos vasos curtos g��stricos e realiza����o de fundoplicatura total, que exerce aumento inerente de press��o sobre a JEG, n��o s�� n��o provocou d��fice da microcircula����o do es��fago distal ou do est��mago proximal como desencadeou um mecanismo de vasodilata����o f��ndica que refor��a o conceito de seguran��a da opera����o de Nissen para tratamento da DRGE. -------------- ABSTRACT: The laparoscopic Nissen operation is considered to be the most appropriate antirefluxsurgery because it suitably replicates the standard physiology of the gastroesophageal valve in most patients with typical symptoms of gastroesophageal reflux disease (GERD). The technical criteria includes the safe shutdown of the diafragmatic crura(cruroplasty) and the creation of a complete fundoplication (360 degrees), short (lesser than two inches), floppy and without tension ��� a goal for which the proximal ligation of the gastric short vessels is crucial. The laparoscopic Nissen operation was performed in sixty women and forty men with GERD, without any operative mortality, at the Surgical Department of the Hospital dos Capuchos, CHLC, EPE. The one hundred patients, averaged 46 years old, complained of heartburn (90%), regurgitation (80%) and upper abdominal pain (54 %). The endoscopy process revealed Savary-Miller esophagitis of grade 0-I (62%), II (23%), III (8%), IV (7%), sliding hernia (71%), paraesophageal hernia (8%) or no herniation (21%). The pHmetry/24h diagnosed mixed pattern (38%), raised (20%), lying (20%) or inconclusive (22%). The manometry diagnosed hypotensive LES (35%), normal esophageal peristalsis (88%), mild hypomotility (5%) and was absent (7%). Hiatal hernia, severe esophagitis, ineffective symptomatic control with proton pump inhibitor and request for treatment discontinuation were the signs for surgical action. A laparoscopic ligation of short gastric vessels (70%), cruroplasty and fundoplication (silk 2/0), short (average size 1.5���2 cm) and floppy, without tension and without intraoperative calibration of the esophagus were thus performed. The laparoscopic Nissen fundoplication behaved safe and effective in treating GERD. In a group of 14 asymptomatic volunteers its reputation was confirmed with statistical significance by normalization of postoperative pHmetry/24h and manometry. 94% of the individuals remained asymptomatic up to 30.7 months (average) in the follow-up. Interrogating myself about the impact of this operation on the microcirculation of the gastric fundus I put premised on the possibility of the ligation of the short gastric vessels in the Nissen procedure can induce changes in the arteriolar diameter in the Wall of the gastric fundus. To explore the influence of ligation of the short gastric vessels and the fundoplication at the arteriolar caliber of the cardia, the fundus and the region of the short vessels of the gastric wall, I designed a project of experimental research in guinea pigs with two interdependent components: one veterinary and another technical where I applied angiomorphological studies. The project was developed at the Research Centre of the Department of Anatomy FCMUNL. For its accomplishment I got permission from the Scientific and Pedagogical Committee of the FCM-UNL, I requested for accreditation as a researcher at the General Directorate of Veterinary and, by resorting to the use of animals I submitted it to the Ethics Committee of the FCM-UNL, which approved it unanimously. The guinea pigs were divided into two experimental groups: an experimental group (EG), in which the Nissen procedure was performed and a control group (CG) in which only a gastric traction was done. Protocols of anesthesia, surgery and euthanasia were applied according to the 3Rs ��� Replacement, Reduction, Refinement of the technique of human experimentation of Burch and Russell (1959) ��� a widely accepted ethical framework for conducting scientific experiments using animals humanely. Using histological and angiomorphological techniques, I performed the analysis and the description of the normal, macro and microvascular, anatomy of the guinea pig stomach and I defined the morphological criteria that I considered susceptible for validation of this animal model for the proposed study. By means of scanning electron microscopy I measured the arteriolar calibers of the vascular casts of the cardia, of the fundus and of the short gastric vessels in both CG and EG, making 469 measurements in the former and 461 in the latter. The data were sent to the Research Center of the CHLC which conducted the statistical analysis (ANOVA). The data were sent to the Centre for Research of the CHLC, EPE which proceeded to statistical analysis (ANOVA). This analysis revealed that the arterioles plexus of the mucosal and submucosal plexus of the cardia, fundus and region of the short gastric vessels, showed increased caliber in EG. The increase was statistically significant for being greater than 50% CG gauge. In the short gastric vessels, the difference was smaller, but persisted and statistically significant. Straight vessels were dilated at the base, on its emergence of the plexus serous only in the fundus. In the guinea pig, the Nissen procedure - complete fundoplication with ligation of the short gastric vessels - caused arteriolar vasodilation on the gastric fundus. I believe that this vasodilation constituted some accommodation to the modification introduced and infer that the same might happen in humans. I admit therefore that vasodilation also occurs in humans following the ligation of the short gastric vessels by microvascular analogy between the two species and that this vasodilation corresponds also to na adaptation mechanism arteriolar, for example, to compensate the loss incurred by ligation. The association of experimental ligation of the short gastric vessels with conducting complete fundoplication, which exerts increased pressure on the EGJ, not only did not cause a microcirculation deficit of the distal esophagus or proximal stomach as triggered a mechanism of fundic vasodilation which reinforces the security concept of the Nissen procedure for treatment of GERD.

Upgrade of an experimental volumetric unit for gas adsorption equilibrium studies

In this work, a volumetric unit previously assembled by the research group was upgraded. This unit revamping was necessary due to the malfunction of the solenoid valves employed in the original experimental setup, which were not sealing the gas properly leading to erroneous adsorption equilibrium measurements. Therefore, the solenoid valves were substituted by manual ball valves. After the volumetric unit improvement its operation was validated. For this purpose, the adsorption equilibrium of carbon dioxide (CO2) at 323K and 0 - 20 bar was measured on two different activated carbon samples, in the of extrudates (ANG6) and of a honeycomb monolith (ACHM). The adsorption equilibrium results were compared with data previously measured by the research group, using a high-pressure microbalance from Rubotherm GmbH (Germany) ��� gravimetric. The results obtained using both apparatuses are coincident thus validating the good operation of the volumetric unit upgraded in this work. Furthermore, the adsorption equilibrium of CO2 at 303K and 0 - 10 bar on Metal-Organic Frameworks (MOFs) Cu-BTC and Fe-BTC was also studied. The CO2 adsorption equilibrium results for both MOFs were compared with the literature results showing good agreement, which confirms the good quality of the experimental results obtained in the new volumetric unit. Cu-BTC sample showed significantly higher CO2 adsorption capacity when compared with the Fe-BTC sample. The revamping of the volumetric unit included a new valve configuration in order to allow testing an alternative method for the measurement of adsorption equilibrium. This new method was employed to measure the adsorption equilibrium of CO2 on ANG6 and ACHM at 303, 323 and 353K within 0-10 bar. The good quality of the obtained experimental data was testified by comparison with data previously obtained by the research group in a gravimetric apparatus.