[Clinical manifestations of familial hypercholesterolemia]. / Klinicke manifestacije porodicne hiperholesterolemije.

Familial hypercholesterolaemia (FH) is an autosomal-dominant inherited disorder clinically characterized by high serum cholesterol (low-density lipoprotein--LDL-fraction) concentrations, xanthomas and premature atherosclerosis. Homozygous individuals suffer from vascular disease in childhood or young adolescence since heterozygous persons are usually at ligh risk of premature cardiovascular death. We present a 42-year old female with coronary heart disease and tuber and tendinous xanthomas, which appeared as a consequence of delayed diagnosis of familial hypercholesterolaemia. She was admitted to the hospital due to unstable angina pectoris. On admission the patient was haemodinamically compensated. Cardiac rhythm was regular and heart sounds were of normal intensity. She also presented two systolic murmurs. The first, the ejection murmur had maximal intensity in the second right intercostal space and radiated to the appex. The second murmur was regurgitant, generated at the appex and propagated to the anterior aixllary line. On inspection, we observed xanthomas in Achillis tendons and palmar extensors as well as tuberous xanthomas in the knees and fingers of both hands and feet. We also observed bilateral xantholasms. Arcus corneae was detected by ophthalmological examination. On admittance, the cholesterol serum level was 13.2 mmol/L, and LDL fraction was 7.6 mmol/L. Echocardiography revealed sclerosis of the aortic valve and mitral annulus. Coronarography documented the three-vessel disease. An aggressive medical treatment, which consisted of bile salts and HMGCoA reductase inhibitors, resulted in significant lowering of serum cholesterol--more than 30%. However, due to refractory angina pectorts, she had to be operated on and aorto-coronary by-pass was performed.

[Isolated systolic arterial hypertension in the elderly]. / Izolovana sistolna arterijska hipertenzija kod starnih osoba.

Isolated systolic hypertension is a common disorder in the elderly carrying a high risk of stroke and cardiovascular disease. Isolated systolic hypertension is usually defined as a systolic blood pressure greater than or equal to 160 mmHg and diastolic blood pressure less than 95 mmHg. The arterial stiffening is the principal cause of increasing systolic pressure in advanced age. It is due to degeneration of the arterial wall and is associated with progressive arterial dilatation. Hypertension in elderly patients is also characterized by increase of peripheral vascular resistance. Due to the wide variability of blood pressure usually seen in old persons, the isolated systolic hypertension is not easy to recognize and final diagnosis requires a long period of observation. The ambulatory blood pressure monitoring proved to be helpful in distinguishing patients with true isolated systolic hypertension from subjects with exaggerated alarm reaction to the pressure measurement. Although the increased risk of cardiovascular and cerebrovascular mortality is well established for isolated systolic hypertension, there has been much debate whether available antihypertensive treatment can prevent or delay cardiovascular and cerebrovascular complications in this condition. During the last year several large new trials have been published, the so-called STOP-Hypertension, SHEP and MRC trials. All studies have demonstrated that the treatment of isolated systolic hypertension with diuretics or/and beta blockers (frequently used in combination) resulted in a significant reduction in the incidence of stroke and major cardiovascular events. New antihypertensive agents such as calcium channel blockers and angiotensin-converting enzyme inhibitors have also been shown to effectively lower systolic blood pressure in the elderly but the effects on long-term morbidity and mortality are still unknown.