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Association of mannose-binding lectin genotype with cardiovascular abnormalities in Kawasaki disease.

Kawasaki disease is an acute vasculitis of possible infectious cause, which in particular affects the coronary arteries. Young children rely mostly on their innate immune system for protection against invading microorganisms, of which mannose-binding lectin is an important component. We aimed to investigate the possible role of the gene for this molecule (MBL) in white Dutch patients with Kawasaki disease. In 90 patients, frequency of mutations in the MBL gene was higher than in healthy children. In children younger than 1 year, those with mutations were at higher risk of development of coronary artery lesions than were those without (odds ratio 15.7, 95% CI 1.4-176.5, p=0.026). Our findings suggest that the innate immune system contributes differently to pathophysiology of Kawasaki disease at various ages.

Case-Control Studies↗

Legal consequences of standard setting for competitive athletes with cardiovascular abnormalities.

This paper addresses the issue of whether establishing consensus standards for the treatment of particular medical conditions increases a physician's exposure to legal liability. The conclusion reached is that the legal effects of standard setting, rather than representing a significant threat of liability, should be seen as beneficial to the medical profession. A fundamental point is that the legal test for liability is entirely dependent on the medical profession's definition of what constitutes adequate care. The law incorporates the standard of care defined by the medical profession and does not impose an external norm. In the absence of formally stated standards, the process of defining relevant medical criteria will involve a great deal of uncertainty. Outcomes of legal contests will be affected by such extraneous factors as the relative experience of the lawyers involved, their access to knowledgeable expert witnesses, and their strategic decisions made with respect to tactics and procedures. Establishment of formal standards has the salutory effect of limiting the influence of these factors and thus reducing the randomness of the results reached. Formal standards also have the advantage of being easily replicated in unrelated proceedings and thereby contribute to the development of a consistent, evenly applied rule of liability. Finally, even if formal standards are either more, or less, progressive than the actual state of medical practice, there is relatively little risk that they will produce untoward results.

Heart Diseases↗

Contributions of color Doppler to the evaluation of cardiovascular abnormalities in the fetus.

The applications of color Doppler sonography are reviewed in this article. Color Doppler is an efficient and time-saving means of assessing normal and abnormal flow in the fetus, making it an essential part of the echocardiographic examination for fetal congenital heart disease. In survey mode, color Doppler can be used to quickly evaluate whether flow channels are normal and to detect abnormal flow patterns. These abnormal flow patterns can then be examined further by pulsed or continuous wave Doppler, if needed. Color Doppler, which is subject to less rib shadowing than other Doppler techniques, also can help in assessing the fetal heart when there is less-than-adequate gray scale imaging.

Blood Flow Velocity↗

Cardiovascular abnormalities in the relatives of patients operated upon for annulo-aortic ectasia. A clinical and echocardiographic study of 40 families.

This study is based on 40 consecutive patients operated on for annuloaortic ectasia at the Turku University Central Hospital between March 1970 and April 1983. Supracoronary graft insertion was used in the first nine patients, while the remaining patients were treated by radical repair using a composite graft and reimplantation of the coronary ostia. The mean age of the patients was 40.3 years (10 to 66 years). The patients were examined postoperatively for their physical, radiological, and ophthalmological parameters. Eight had the classic Marfan syndrome. The patients had 230 first-degree relatives, and 200 of them were alive. Three of the 30 deceased relatives had died due to rupture of an ascending aortic aneurysm; 187 of the living relatives were examined as above. In addition, echocardiography was performed. Twenty-six of the surviving relatives had dilatation of the aortic root; 24 were siblings, and two were children of the operated patients. Echocardiography of the relatives revealed an increased incidence of mitral valve prolapse and enlargement of the left ventricle and left atrium. None of the relatives examined had the classic Marfan syndrome, although the incidence of some Marfan features occurred more frequently in the Marfan families. It seems clear that annuloaortic ectasia, even without the Marfan syndrome, is a hereditary disease. To detect annuloaortic ectasia at an early stage, examination of the first-degree relatives of patients operated upon is recommended.

Adolescent↗

Abnormal cardiovascular responses induced by localized high power microwave exposure.

A hypothesis of microwave-induced circulatory under perfusion was tested in ketamine anesthetized rats whose heart rate, mean arterial pressure, pulse pressure, respiration rate, and body temperatures were monitored continuously. Fifty-eight ventral head and neck exposures in a waveguide consisted of sham-exposure and exposure to continuous wave (CW) and pulsed 1.25 GHz microwaves for 5 min. The 0.5 Hz (10 microseconds, 2 W average) and 16 Hz (1 microsecond, 6.4 W average) pulse-modulated microwaves were delivered at 400 kW peak power. The CW microwaves were 2 and 6.4 W. The average specific absorption rate was 4.75 W/kg per watt transmitted in the brain and 17.15 W/kg per watt transmitted in the neck. Respiration rate and mean arterial pressure were not altered. Changes in heart rate and pulse pressure were observed in rats exposed to higher power (16 Hz pulses and 6.4 W CW) but not to the lower average power microwaves (0.5 Hz pulses and 2 W CW). Depression of pulse pressure, an indication of a decrease in stroke volume, and increased (tachycardia) or decreased (bradycardia) heart rate were noted in presence of whole-body hyperthermia. The cardiac output of those animals exposed to higher average power microwaves was considered to be below normal as hypothesized. Decreased cardiac output and normal mean arterial pressure resulted in an increase in the total peripheral resistance which was contrary to the anticipated thermal response of animals.

Animals↗

Abnormal cardiovascular and catecholamine responses to supine exercise in human subjects with sympathetic dysfunction.

1. The cardiovascular and catecholamine responses to supine leg exercise were measured in fifteen normal subjects (controls) and in three groups with sympathetic dysfunction: fifteen with central failure (Shy-Drager syndrome; SDS), fifteen with peripheral failure (pure autonomic failure; PAF) and two with isolated dopamine beta-hydroxylase deficiency (DBH deficiency). 2. With exercise, blood pressure increased in controls, fell markedly in SDS and PAF and was unchanged in DBH deficiency. After exercise, blood pressure rapidly returned to baseline in controls, but remained low in SDS and PAF. With exercise, heart rate increased more in controls than SDS or PAF; the response varied in DBH deficiency. 3. With exercise, cardiac output increased similarly in controls, SDS and PAF, with a larger increase in DBH deficiency. Vascular resistance fell less in controls than SDS, PAF and DBH deficiency. 4. With exercise, plasma noradrenaline increased in controls only; plasma adrenaline remained unchanged in all groups. In DBH deficiency, plasma noradrenaline and adrenaline were undetectable, but plasma dopamine was elevated and rose further with exercise. 5. Supine exercise substantially lowered blood pressure in sympathetic failure due to SDS and PAF. In DBH deficiency blood pressure was unchanged; this lack of fall may have been due to vasoconstriction induced by dopamine and other substances released from otherwise intact sympathetic terminals, or to preserved cardiac vagal function.

Adult↗