Anti-myelin antibodies modulate clinical expression of childhood multiple sclerosis.

Anti-myelin basic protein (MBP) antibodies in pediatric-onset MS and controls were characterized. Serum samples were obtained from 94 children with MS and 106 controls. Paired CSF and serum were obtained from 25 children with MS at time of their initial episode of acute demyelinating syndrome (ADS). Complementary assays were applied across samples to evaluate the presence, and the physical binding properties, of anti-MBP antibodies. While the prevalence and titers of serum anti-MBP antibodies against both immature and mature forms of MBP were similar in children with MS and in controls, binding characteristics and formal Surface Plasmon Resonance (SPR) studies indicated surprisingly high binding affinities of all pediatric anti-MBP antibodies. Serum levels of anti-MBP antibodies correlated significantly with their CSF levels, and their presence in children with MS was associated with significantly increased risk of an acute disseminated encephalomyelitis-like initial clinical presentation. While antibodies to both immature and mature forms of MBP can be present as part of the normal pediatric humoral repertoire, these anti-myelin antibodies are of surprisingly high affinity, can access the CNS during inflammation, and have the capacity to modulate disease expression. Our findings identify an immune mechanism that could contribute to the observed heterogeneity in spectrum of clinical presentations in early-onset MS.

Arterial distensibility as a possible compensatory mechanism in chronic aortic insufficiency.

OBJECTIVE: To evaluate elastic properties of conduit arteries in asymptomatic patients who have severe chronic aortic regurgitation. METHODS: Twelve healthy volunteers aged 30+/-1 years (control group) and 14 asymptomatic patients with severe aortic regurgitation aged 29+/-2 years and left ventricular ejection fraction of 0.61+/-0.02 (radioisotope ventriculography) were studied. High-resolution ultrasonography was performed to measure the systolic and diastolic diameters of the common carotid artery. Simultaneous measurement of blood pressure enabled the calculation of arterial compliance and distensibility. RESULTS: No differences were observed between patients with aortic regurgitation and the control group concerning age, sex, body surface, and mean blood pressure. Pulse pressure was significantly higher in the aortic regurgitation group compared with that in the control group (78+/-3 versus 48+/-1mmHg, P<0.01). Arterial compliance and distensibility were significantly greater in the aortic regurgitation group compared with that in the control group (11.0+/-0.8 versus 8.1+/-0.7 10(-10) N-1 m4, P=0.01 e and 39.3+/-2.6 versus 31.1+/-2.0 10(-6) N-1 m2, P=0.02, respectively). CONCLUSION: Patients with chronic aortic regurgitation have increased arterial distensibility. Greater vascular compliance, to lessen the impact of systolic volume ejected into conduit arteries, represents a compensatory mechanism in left ventricular and arterial system coupling.

Ressonância nuclear magnética no diagnóstico de pseudoaneurisma de ventrículo esquerdo: antigo problema, nova opção / Nuclear magnetic resonance imaging in the diagnosis of left ventricular pseudoaneurysm: an old problem, a new option

Paciente feminina, 67 anos, internada por infarto agudo do miocárdio de parede lateral, com edema agudo de pulmão e evolução para choque cardiogênico nas primeiras horas. Ecocardiograma transesofágico e ressonância magnética confirmaram o diagnóstico de pseudoaneurisma de ventrículo esquedo. A paciente foi submentida a tratamento cirúrgico com sucesso.

Eight years experience using the inferior epigastric artery for myocardial revascularization.

We present the initial experience with the use of inferior epigastric artery as a free graft for myocardial revascularization in a series of 41 patients operated on between 1987 and 1989 to show the clinical and angiographic results. Of the 41 patients, 29 were male, 12 female, with ages ranging from 33 to 72 years, and weights from 50 to 86 kg. The inferior epigastric artery grafts were predominantly employed to the anterior interventricular branch and diagonal branch. The proximal anastomosis into the aorta was done directly or with a patch of vein or pericardium. The 32 patients who survived had mean follow-up of 82 months and 22 are in functional class I, 8 in class II and 2 in class III of angina. The early patency rate was: inferior epigastric artery-anterior interventricular branch, 85.7% and inferior epigastric artery-diagonal branch. 85.7%. Sixteen patients underwent angiographic study at a mean follow-up of 81.2 months and the patency rate to the anterior interventricular branch was 77.7% and to the diagonal branch was 100%. Three patients with early occluded inferior epigastric artery were reoperated on 3, 3 and 11 months after the operation. A filiform lumen of the graft and a small ostium in the aorta was found and explained the imperfection of the direct anastomosis due to unbalance thickness of the graft and the aortic wall. Therefore it is occluded that the inferior epigastric artery is an alternative arterial graft for myocardial revascularization and that the use of a vein or pericardium patch can help the proximal anastomosis and improve potency of the graft.

[Nuclear magnetic resonance imaging in the diagnosis of left ventricular pseudoaneurysm. An old problem, a new option]. / Ressonância nuclear magnética no diagnóstico de pseudoaneurisma de ventrículo esquerdo. Antigo problema, nova opção.

We describe the case of a 61 year-old female patient admitted to the hospital with acute lateral myocardial infarction, in pulmonary edema, who evolved to cardiogenic shock in the first hours. Transesophageal echodoppler-cardiogram and nuclear magnetic resonance imaging showed the diagnosis of a left ventricular pseudoaneurysm. Surgical repair was successfully undertaken.

[Mitral subvalvular aneurysm of the left ventricle]. / Aneurisma subvalvar mitral de ventrículo esquerdo.

A 21-year-old white man presented with cardiogenic shock and refractory pulmonary congestion. At the transthoracic echocardiogram a subvalvar left ventricular aneurysm of the inferior wall with severe mitral regurgitation was observed. The outcome was favorable after surgical correction of the mitral regurgitation and of the subvalvar aneurysm. We emphasize that, whenever possible, valvar repair is better than mitral replacement, since annulus tissue fragility causes suturing of the mitral prosthesis to be difficult.

Carotid arterial compliance in patients with congestive heart failure secondary to idiopathic dilated cardiomyopathy.

Conduit artery distensibility affects the pulsatile component of afterload and may contribute to impaired left ventricular function in patients with congestive heart failure (CHF). The objectives of this study were to (1) determine whether arterial distensibility is reduced in patients with CHF, and (2) determine whether decreased arterial compliance is related to an abnormality in vascular wall structure (i.e., wall thickness or excessive levels of circulating neurohumoral vasoconstrictors, or both). The study participants included 40 patients with CHF secondary to idiopathic dilated cardiomyopathy and 33 age-matched healthy volunteers. High-resolution ultrasonography was performed to directly visualize the common carotid artery and measure its diameter and wall thickness. Its elastic properties were determined by relating changes in arterial diameter to changes in pressure generated with each heart beat. Carotid artery distensibility was less (14.1 +/- 1.1 vs 25.3 +/- 1.6 10(-6).N-1.m2, p < 0.001) and Young's modulus of elasticity was greater (3.99 +/- 0.51 vs 2.29 +/- 0.23 10(5).N.m-2, p < 0.005) in patients with CHF than in normal subjects. Also, carotid artery wall thickness was increased in patients with CHF. When the entire population was considered, age, wall thickness, and plasma norepinephrine and aldosterone concentrations correlated inversely with distensibility, whereas age and plasma norepinephrine concentration correlated directly with elasticity. Among normal subjects, only age correlated inversely with distensibility; among patients with CHF, only plasma norepinephrine concentration correlated with elasticity. It is concluded that carotid artery distensibility is reduced in patients with CHF.(ABSTRACT TRUNCATED AT 250 WORDS)

[Acute liver failure secondary to cardiogenic shock in patients with congestive heart failure]. / Falência hepática aguda secundária a choque cardiogênico em portadores de insuficiência cardíaca congestiva.

Fulminant hepatic failure is a complication of severe cardiocirculatory failure, with high morbidity and mortality, and is frequently misdiagnosed as fulminant viral hepatitis. We report three cases of patients with chronic severe heart failure who developed cardiogenic shock complicated by elevation of aminotransferase levels above 1,000 soon after the most severe episode of hypotension. All the three patients presented regression of hepatic enzymes 72h after admission. Two patients developed hepatic encephalopathy and renal failure. One underwent the implantation of an artificial left ventricle, followed by orthotopic heart transplantation. One died of systemic multiple organ failure, after he had showed improvement on his hepatic profile, and one was sent to the ward, after 15 days with marked improvement on his clinical status and no signs of hepatic disease.

[Thiocyanate toxicity arising from prolonged use of sodium nitroprusside in severe heart failure]. / Toxicidade pelo tiocianato decorrente do uso prolongado de nitroprussiato de sódio na insuficiência cardíaca grave.

PURPOSE: To evaluate the toxicity of thiocyanate induced by the infusion of sodium nitroprusside in patients with severe congestive failure. METHODS: We studied 23 patients with congestive heart failure, in class IV (NYHA) under continuous infusion of sodium nitroprusside with doses varying between 0.5 and 5.5 micrograms/kg/min. Clinical evaluation, thiocyanate serum dosage and laboratorial evaluation of renal, hepatic and pulmonary functions were done. RESULTS: Seventeen patients (74%) presented toxic levels of thiocyanate (over 10 micrograms/ml), with the average of 29.9 +/- 4.4 micrograms/ml. Only renal function was related to the presence of intoxication. Clinical evaluation was not accurate to diagnose the thiocyanate toxicity in the patients. CONCLUSION: Sodium nitroprusside is potentially toxic, especially when the renal function is abnormal. Thiocyanate dosage is useful in diagnosing nitroprusside induced toxicity and then it contributes to an adequate treatment and prevention of clinical toxicity.

Choque cardiogênico / Shock cardiogenic

O choque cardiogênico caracteriza-se quando uma severa disfunçäo miocárdica provoca hipoperfusäo tecidual e falência orgânica. Os critérios exatos para a presença de choque cardiogênico variam muito na literatura. Além disso é extremamente importante comparar o grau da disfunçäo miocárdica quando se analisam os resultados de diferentes procedimentos. Em 1973, importante estudo multicêntrico estabeleceu os seguintes critérios para a definiçäo de choque cardiogênico: - pressäo arterial sistólica < 80 mmHg (intra-arterial); - débito urinário < 20 ml/h ou confusäo mental; - pressäo de enchimento ventricular > 12 mmHg; - pressäo venosa central > 10 cm H20. Esta definiçäo tem grande utilidade clínica pois inclui a classificaçäo de Kilip para o infarto agudo do miocárdio. Além disso, incorpora os três principais componentes do choque cardiogênico: 1) comprometimento da funçäo ventricular; 2) evidência de falência orgânica como resultado do decréscimo da perfusäo tecidual; e 3) exclusäo de hipovolemia e outras causas do choque. Uma vez que esta definiçäo já foi largamente aplicada com sucesso em estudos prospectivos, pode ser considerada padräo para estudos clínicos futuros.

[Rupture of the left sinus of Valsalva forming a subepicardial pseudoaneurysm]. / Rotura do seio de Valsalva esquerdo com formação de pseudoaneurisma subepicárdico.

A healthy 44 year-old male patient presented a calcified mass in the middle mediastinum on a chest film. During diagnostic investigation cineangiograms showed a rupture of the left sinus of Valsalva forming a large pseudoaneurysm that produced important distortion of the left coronary artery and it's main branches. The patient was submitted to surgical repair through the closure of the orifice of the ruptured left sinus of Valsalva with a bovine pericardium patch. The subepicardium was filled with organized thrombi which were removed. He was discharged from the hospital after uncomplicated postoperative course. Pathological examination of the aortic fragment did not yield the etiology of the rupture.

Inferior epigastric artery as a free graft for myocardial revascularization.

The inferior epigastric artery was used as a free graft for direct myocardial revascularization in 22 patients from October 1987 to July 1988. The artery was used either alone or along with internal mammary artery or saphenous vein grafts. The inferior epigastric artery was dissected through an infraumbilical incision without entrance into the peritoneal cavity. The results depend on the technique used for the aortoepigastric anastomosis. When a segment of saphenous vein or a patch of bovine pericardium was sutured to a large aortic orifice with the inferior epigastric artery previously anastomosed to these patches, the patency rate of the free grafts in the early postoperative period was 100%. Histologic examination showed identical structure of the inferior epigastric artery and the internal mammary artery. Application of the inferior epigastric artery is an attempt to increase the use of arterial grafts for myocardial revascularization.

Esophageal echocardiography.

Esophageal echocardiography has been developed for use in patients with chronic obstructive pulmonary disease and is a safe diagnostic procedure which provides high resolution mirror image echoes of many cardiac structures. Conventional anterior and esophageal echocardiograms were performed in 38 subjects. Esophageal echoes were of diagnostic quality in all 38 subjects, anterior echoes were of diagnostic quality in only 18. Measurements from anterior and esophageal echocardiograms correlated well for aortic valve diameter (r = 0.87), left atrium diameter (r = 0.96), mitral valve EF slope (r = 0.97) and less well for aortic root diameter (r = 0.69).