Marfan's syndrome: early and severe form in siblings

Marfan's syndrome is an inherited disorder of the connective tissue. Cardiologic manifestations, especially aortic dilation, are important causes of morbidity and mortality in the clinical course of the disease in adults and teenagers. In children, the presence of aortic aneurysm and its dissection or rupture is rare, occurring in patients with genetic mutation of the fibrillin gene but not in those who have the familial form of the disease. We describe here 2 patients, from the same family (siblings), diagnosed with gigantic aortic aneurysm early in infancy, one of them successfully undergoing surgery.

Tratamento endovascular de dissecção crônica toracoabdominal complicada com aneurisma torácico, mediante implante de endoprótese vascular

Our objective is to report a case of a patient with a descending thoracic aortic aneurysm and chronic aortic dissection, who was submitted to an endovascular treatment. A 68-year-old male with coronary artery disease and hypertension, with no history of trauma, diabetes or smoking. He had myocardial infarction ten years ago. Under general anesthesia, the left femoral artery was surgically exposed and the left braquial artery was catheterized with a "pigtail" catheter, under Seldinger technique. The proximal 46mm/Æ and distal 34mm/Æ stent-graft was placed just distal to the origen of the left subclavian artery. Control arteriography showed that the lesion was completely excluded. The patient was discharged seven days after the surgery, when a computed tomographic control, was performed showing a sustained aneurysm exclusion and a satisfactory endovascular position.

Hemoptise como manifestação de aneurisma de aorta torácica descendente

Aortotracheal fistula is a rare condition that is invariably fatal if not diagnosed and surgically treated. Patients usually present with small intermittent hemoptysis. The findings using computerized tomography (CT) are usually diagnostic. CT should be considered in the initial investigation of patients suspected to have such a disease. A 62-year-old woman with a aneurysm of the descending thoracic aorta presented with new-onset back pain and hemoptysis. The hemoptysis was thought to be the result of invasion of the bronchial tree by the aneurysm.

Inhibition of PKC-dependent extracellular Ca2+ entry contributes to the depression of contractile activity in long-term pressure-overloaded endothelium-denuded rat aortas

We examined the contractile responsiveness of rat thoracic aortas under pressure overload after long-term suprarenal abdominal aortic coarctation (lt-Srac). Endothelium-dependent angiotensin II (ANG II) type 2 receptor (AT2R)-mediated depression of contractions to ANG II has been reported in short-term (1 week) pressure-overloaded rat aortas. Contractility was evaluated in the aortic rings of rats subjected to lt-Srac or sham surgery (Sham) for 8 weeks. ANG I and II levels and AT2R protein expression in the aortas of lt-Srac and Sham rats were also evaluated. lt-Srac attenuated the contractions of ANG II and phenylephrine in the aortas in an endothelium-independent manner. However, lt-Srac did not influence the transient contractions induced in endothelium-denuded aortic rings by ANG II, phenylephrine, or caffeine in Ca2+-free medium or the subsequent tonic constrictions induced by the addition of Ca2+ in the absence of agonists. Thus, the contractions induced by Ca2+ release from intracellular stores and Ca2+ influx through stored-operated channels were not inhibited in the aortas of lt-Srac rats. Potassium-elicited contractions in endothelium-denuded aortic rings of lt-Srac rats remained unaltered compared with control tissues. Consequently, the contractile depression observed in aortic tissues of lt-Srac rats cannot be explained by direct inhibition of voltage-operated Ca2+ channels. Interestingly, 12-O-tetradecanoylphorbol-13-acetate-induced contractions in endothelium-denuded aortic rings of lt-Srac rats were depressed in the presence but not in the absence of extracellular Ca2+. Neither levels of angiotensins nor of AT2R were modified in the aortas after lt-Srac. The results suggest that, in rat thoracic aortas, lt-Srac selectively inhibited protein kinase C-mediated activation of contraction that is dependent on extracellular Ca2+ entry.

Hemodynamic responses to acute aortic coarctation in conscious sinoaortic denervated rats

The hemodynamic responses to acute (45 min) partial aortic constriction were studied in conscious intact (N = 7) or sinoaortic denervated (SAD) adult male Wistar rats (280-350 g, N = 7) implanted with carotid and femoral arterial catheters, a pneumatic cuff around the abdominal aorta and a pulsed Doppler flow probe to measure changes in aortic resistance. In addition, the hypertensive response and the reflex bradycardia elicited by total (N = 8) vs partial (N = 7) aortic constriction (monitored by maintenance of the pressure distal to the cuff at 50 mmHg) were compared in two other groups of intact rats. Intact rats presented a smaller hypertensive response (26 to 40% above basal level) to partial aortic constriction than SAD rats (38 to 58%). The calculated change in aortic resistance imposed by constriction of the aorta increased progressively only in intact rats, but was significantly smaller (193 to 306%) than that observed (501 to 591%) in SAD rats. Intact rats showed a significant bradycardia (23 to 26% change in basal heart rate) throughout coarctation, whereas the SAD rats did not (1 to 3%). Partial or total occlusion of the aorta induced similar hypertensive responses (37-38% vs 24-30% for total constriction) as well as reflex bradycardia (-15 to -17% vs -22 to -33%) despite a greater gradient in pressure (97-98 vs 129-140 mmHg) caused by total constriction. The present data indicate that the integrity of the baroreflex in intact rats can cause the hypertensive response to level off at a lower value than in SAD rats despite a progressive increase in aortic resistance. In addition, they also indicate that the degree of partial aortic constriction by maintenance of the pressure distal to the cuff at 50 mmHg already elicits a maximal stimulation of the arterial baroreflex

Fatores associados à obesidade abdominal em mulheres em idade reprodutiva

OBJETIVO: Investigar variáveis potencialmente associadas à obesidade abdominal em mulheres em idade reprodutiva. MÉTODOS: Foram investigadas 781 mulheres a partir de informações coletadas pela Pesquisa Nutrição e Saúde realizada em 1996 no Município do Rio de Janeiro. A obesidade abdominal foi definida como circunferência da cintura (CC) > 80 cm ou como Razão Cintura Quadril (RCQ) > 0,85. A análise estatística envolveu o cálculo de medidas de tendência central. A associação entre obesidade abdominal e Índice de Massa Corporal, idade, paridade e uso de tabaco foi testada por meio do cálculo do "Odds Ratio" (OR), usando a técnica de regressão logística multivariada. RESULTADOS: As maiores freqüências de obesidade abdominal foram observadas em mulheres acima de 35 anos e com dois ou mais filhos (50,7%). Os valores de OR demonstram o efeito da interação entre paridade e idade para CC>80 cm quando controlado apenas o efeito dessas duas variáveis. A partir dos modelos de regressão logística, verificou-se que quando a população foi estratificada em mulheres com e sem sobrepeso, apenas a escolaridade esteve associada à RCQ, enquanto a associação com idade e paridade desapareceu para a CC>80 cm. CONCLUSÕES: A obesidade abdominal nesse grupo populacional independe da idade e da paridade quando ajustado pelo peso relativo, sendo suas maiores determinantes a adiposidade geral e a escolaridade. Ter maior escolaridade significou possuir uma RCQ menor. É fundamental implementar estratégias de prevenção para o desenvolvimento da obesidade, cujo enfoque sejam mulheres em idade reprodutiva.

Excesso de peso e gordura abdominal para a síndrome metabólica em nipo-brasileiros

OBJETIVO: A obesidade, especialmente de distribuição abdominal, associa-se a fatores de risco cardiovasculares como a dislipidemia, a hipertensão arterial (HA) e o diabetes mellitus (DM). A importância desses fatores em nipo-brasileiros foi previamente demonstrada, apesar de a obesidade não ser característica marcante dos migrantes japoneses. Realizou-se estudo com o objetivo de avaliar a prevalência de excesso de peso e a adiposidade central (AC) em nipo-brasileiros e suas relações com distúrbios metabólicos. MÉTODOS: A amostra incluiu 530 nipo-brasileiros (40-79 anos) de primeira e segunda gerações, submetidos a medidas antropométricas de pressão arterial, perfil lipídico e teste oral de tolerância à glicose. A prevalência (por ponto e intervalo de confiança) de excesso de peso foi calculada pelo valor de corte >26,4 kg/m². O diagnóstico de AC foi baseado na razão entre as circunferências da cintura e do quadril (RCQ), sendo que valores > ou = 0,85 e > ou = 0,95, para mulheres e homens, respectivamente, firmavam esse diagnóstico. RESULTADOS: A prevalência de excesso de peso foi de 22,4% (IC95%-- 20,6-28,1), e a de AC, de 67,0% (IC95% -- 63,1-70,9). Além de maiores prevalências de DM, HA e dislipidemia, estratificando-se pelo índice de massa corporal (IMC) e RCQ, indivíduos com excesso de peso e adiposidade central apresentaram pior perfil metabólico: a pressão arterial foi significantemente maior naqueles com excesso de peso, sem e com AC; indivíduos com AC apresentaram maiores índices de glicemia, triglicerídeos, colesterol total e LDL e menor HDL quando comparados aos sem excesso de peso e sem AC; a insulinemia de jejum foi significantemente maior em indivíduos com excesso de peso (sem e com AC) do que naqueles sem excesso de peso e sem AC. CONCLUSÃO: A comparação de subgrupos com e sem adiposidade central foi compatível com a hipótese de que a deposição abdominal de gordura representa fator de risco para doenças interligadas pela resistência à insulina, inclusive em população de origem oriental. A alta prevalência de síndrome metabólica nos migrantes japoneses pode ser decorrente da deposição visceral de gordura, implicada na gênese da resistência à insulina.

Importância relativa do Índice de Massa Corporal e da circunferência abdominal na predição da hipertensão arterial

OBJETIVO: Avaliar a importância relativa do Índice de Massa Corporal (IMC) e da circunferência abdominal na determinação da hipertensão arterial em adultos. MÉTODOS: Estudo transversal com amostra de funcionários (N=1.584), entre 18 e 64 anos de idade, de hospital geral privado do município de São Paulo. A coleta de dados envolveu questionário estruturado, medida da pressão arterial, peso, altura e circunferência abdominal. A hipertensão foi diagnosticada com pressão arterial > 140/90 mmHg ou uso de medicação anti-hipertensiva. A importância relativa do IMC e da circunferência abdominal foi calculada pela fração atribuível de hipertensão correspondente a cada indicador antropométrico, empregando-se níveis de cortes usuais e baseados na distribuição observada na população estudada. Adicionalmente, foi desenvolvido um indicador que combinou simultaneamente valores de IMC e circunferência abdominal. RESULTADOS: A prevalência de hipertensão foi de 18,9% (26,9% em homens e 12,5% em mulheres). Em homens, a fração de hipertensão atribuível ao IMC superou aquela atribuível à circunferência abdominal segundo níveis de corte usuais (56% x 48%, respectivamente) e quartis da distribuição observada (73% x 69%, respectivamente). Para mulheres, a fração de hipertensão atribuível à circunferência abdominal superou ligeiramente aquela atribuível ao IMC nos níveis de corte usuais (44% x 41%, respectivamente); mas se observou situação inversa empregando a classificação em quartis (41% x 57%, respectivamente). Somente em mulheres a fração de hipertensão atribuível ao indicador que combinou IMC e circunferência abdominal (64%) superou a fração atribuível a cada medida isolada. CONCLUSÕES: Tanto o IMC quanto a circunferência abdominal se associaram positiva e independentemente com a ocorrência de hipertensão arterial, sendo superior a influência exercida pelo IMC em homens.

Consumo de bebida alcoólica e adiposidade abdominal em doadores de sangue

OBJETIVO: Analisar a associação entre o consumo de bebidas alcoólicas e adiposidade abdominal. MÉTODOS: Estudo transversal com uma amostra de homens doadores de sangue (N=1.235), de 20 a 59 anos, em Cuiabá (MT), realizado de agosto/1999 a janeiro/2000. Os indicadores de adiposidade abdominal foram circunferência da cintura e relação cintura/quadril, ajustados pela adiposidade total. As medidas aferidas foram: peso, estatura, circunferências da cintura e do quadril. O consumo de álcool foi avaliado utilizando-se um questionário sobre tipo, freqüência e quantidade da bebida consumida. A associação entre o consumo de álcool e adiposidade abdominal foi analisada por regressão linear múltipla, com os modelos ajustados para idade, atividade física, tabagismo e adiposidade total. RESULTADOS: Após ajuste, a circunferência da cintura e a relação cintura quadril mantiveram-se associadas positivamente ao consumo de cerveja (p=0,02) e ao total de álcool consumido (p=0,01 e 0,03, respectivamente). O consumo de aguardente mostrou associação somente com a circunferência da cintura (p=0,04). CONCLUSÕES: O consumo de álcool, particularmente de cerveja, associou-se com a localização abdominal de gordura.

Educational inequality in the occurrence of abdominal obesity:Pró-Saúde Study

OBJECTIVE To estimate the degree of educational inequality in the occurrence of abdominal obesity in a population of non-faculty civil servants at university campi.METHODS In this cross-sectional study, we used data from 3,117 subjects of both genders aged 24 to 65-years old, regarding the baseline ofPró-Saúde Study, 1999-2001. Abdominal obesity was defined according to abdominal circumference thresholds of 88 cm for women and 102 cm for men. A multi-dimensional, self-administered questionnaire was used to evaluate education levels and demographic variables. Slope and relative indices of inequality, and Chi-squared test for linear trend were used in the data analysis. All analyses were stratified by genders, and the indices of inequality were standardized by age.RESULTS Abdominal obesity was the most prevalent among women (43.5%; 95%CI 41.2;45.9), as compared to men (24.3%; 95%CI 22.1;26.7), in all educational strata and age ranges. The association between education levels and abdominal obesity was an inverse one among women (p < 0.001); it was not statistically significant among men (p = 0.436). The educational inequality regarding abdominal obesity in the female population, in absolute terms (slope index of inequality), was 24.0% (95%CI 15.5;32.6). In relative terms (relative index of inequality), it was 2.8 (95%CI 1.9;4.1), after the age adjustment.CONCLUSIONS Gender inequality in the prevalence of abdominal obesity increases with older age and lower education. The slope and relative indices of inequality summarize the strictly monotonous trend between education levels and abdominal obesity, and it described educational inequality regarding abdominal obesity among women. Such indices provide relevant quantitative estimates for monitoring abdominal obesity and dealing with health inequalities.

Clinical and epidemiological aspects of abdominal angiostrongyliasis in Southern Brazil

Most of the cases of abdominal angiostrongyliasis in Brazil were reported from the southern States of São Paulo, Paraná, Santa Catarina and Rio Grande do Sul (RS). A study in 27 cases from RS revealed a distinct local epidemiology. Peasants were usually affected, either adults or children, from the mountainous areas in the north of the Suite. There was a seasonal increase in the number of cases, from late spring to autumn, that does not coincide with the rainy season. Besides the most common clinical features of abdominal pain, fever and cosinophilia in the leucogram, painful relapsing episodes were detected in some patients. The abdominal pain could be either localized or diffuse during the rapid evolution to a surgical abdominal condition, with a letality of 7.4%. The use of a serological test and the greater awareness of physicians working in endemic areas is expected to improve the recognition of uncomplicated and benign courses of the disease. This study confirms the known clinical manifestations of abdominal angiostrongyliasis and demonstrates the diversity of its epidemiology.

HEART ANEURYSM IN CHAGAS' DISEASE

This prospective study on 41 autopsy collected human hearts concerns the "apical" lesion in Chagas' disease. Previous report did not show a correlation between lesion frequency and heart weight then discarding a vascular factor in its pathogenesis. The present paper involves other variables besides the heart weight to evaluate the relative coronary insufficiency. Distinct colored gel (green and red) injected through the capillary beds of both coronary arteries defined the extent of both vessels before separating the atria and removing the sub-epicardium fat. The Right Ventricle (RV) and Left Ventricle (LV) free walls furnished the RV/LV mass ratio. The myocardium mass colored green (right coronary artery - RC) and the whole Ventricular Weight (VW) determined the RC/VW mass ratio. The heart weight plus these mass ratios, graded and added, composed a score inversely proportional to the myocardium irrigation condition. It intended to be a more sensitive morphologic evaluation of the relative ischaemia to correlate to the apical lesion. This study showed a right deviation for the relative accumulated frequency of lesions plotted as a score function and a significant difference for higher scores in hearts with aneurysm. It suggests a ischaemic factor intervening in the apical lesion pathogenesis in Chagas' cardiopathy.

Jejunal perforation caused by abdominal angiostrongyliasis

The authors describe a case of abdominal angiostrongyliasis in an adult patient presenting acute abdominal pain caused by jejunal perforation. The case was unusual, as this affliction habitually involves the terminal ileum, appendix, cecum or ascending colon. The disease is caused by the nematode Angiostrongylus costaricensis, whose definitive hosts are forest rodents while snails and slugs are its intermediate hosts. Infection in humans is accidental and occurs via the ingestion of snail or slug mucoid secretions found on vegetables, or by direct contact with the mucus. Abdominal angiostrongyliasis is clinically characterized by prolonged fever, anorexia, abdominal pain in the right-lower quadrant, and peripheral blood eosinophilia. Although usually of a benign nature, its course may evolve to more complicated forms such as intestinal obstruction or perforation likely to require a surgical approach. Currently, no efficient medication for the treatment of abdominal angiostrongyliasis is known to be available. In this study, the authors provide a review on the subject, considering its etiopathogeny, clinical picture, diagnosis and treatment.

Natural infection of Deroceras laeve (Mollusca: gastropoda) with metastrongylid larvae in a transmission focus of abdominal angiostrongyliasis

Angiostrongylus costaricensis is a nematode parasitic of rodents. Man may become infected by ingestion of the third stage larvae produced within the intermediate hosts, usually slugs from the family Veronicellidae. An epidemiological study carried out in a locality in southern Brazil (western Santa Catarina State) where these slugs are a crop pest and an important vector for A. costaricensis has documented for the first time the natural infection of Deroceras laeve with metastrongylid larvae. This small limacid slug is frequently found amid the folds of vegetable leaves and may be inadvertently ingested. Therefore D. laeve may have an important role in transmission of A. costaricensis to man.