[LETTER-SIEVE DISEASE (A NEOPLASTIC FORM WITH LIPOIDOSIS)].
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The aortas of 22 practically healthy persons who died accidentally at the age of 20 to 60 years and 112 patients with atherosclerosis dying from the complications of the ischaemic heart disease (IHD) at the age of 30 to 80 years were studied by macroluminescent analysis. The incidence and distribution of prelipid changes (PC) in the intima were compared to those of lipidosis. PC of the type of a simple oedema, gelatinous plaque or muscular-elastic hyperplasia extinguish the primary fluorescence and macroscopically look as areas having the shades of a blue colour. The spread of PC in the aorta depends on the age and the degree of atherosclerosis. A significant increase of the PC surface in the aorta of persons dying from IHD as compared to the healthy persons indicate the exacerbation of the atherosclerotic process. The type and the gravity of the atherosclerosis in the human aorta are determined by the qualitative and quantitative correlations of PC and lipidosis.
A new hereditary disease, transmitted autosomal-dominant, which affects the kidneys has been observed in four members of the same family, three males and a woman. No specific clinical manifestations of the disease have been established. A slight proteinuria was first revealed at the age of 17 years. After about twenty years of moderate proteinuria and microscopic hematuria terminal renal failure and severe arterial hypertension occur. Specific for the disease is the storage of lipids, mainly cholesterol and phospholipids in the glomerular mesangium. No lipid storage in other renal structures or other organs could be established. As no changes of cholesterol and triglycerides in the serum have been recorded a general disturbance in their turnover may be excluded. The storage of cholesterol in the mesangial matrix in form of crystals in vitro is specific for the reported hereditary disease and distinguishes it from other diseases in which a glomerular storage of cholesterol is well known.
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