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Mid-dermal elastolysis (MDE) is a particular elastic tissue disorder in which selective loss of elastic fibres occurs in the mid-dermis. It is clinically characterized by the appearence of fine wrinkling of the epidermis and perifollicular protrusion which gives the skin an aged appearence. It is sometimes associated with an inflammatory event such as(More)
The etiopathogenesis of thoracic aortic aneurysms is currently an issue of debate. The present study investigated ultrastructural, morphometric, and immunohistochemical aspects of smooth muscle cells (SMCs) in chronic aneurysm of the thoracic aorta (aneurysm group), aortic dilatation associated with valvular disease (valvular group), and dissection of the(More)
A case of giant axonal neuropathy (GAN) in a boy of 4 years and 6 months, is reported. Nerve conduction velocity (NCV), EEG and CT scan indicated both peripheral and central nervous system involvement. Intestinal absorption tests did not reveal vitamin B12 malabsorption; the endocrine situation was found to be substantially normal. The clinical picture was(More)
The localization of dopamine receptors within rabbit renal artery was studied using 3H-spiroperidol as a label for dopamine receptors and a histoautoradiographic technique. Preliminary radioreceptor binding studies showed that 3H-spiroperidol was bound to sections of renal artery in a manner consistent with the existence of dopamine receptors. In fact the(More)
Increased amounts of free sialic acid were found in cultured fibroblasts and urine of a 4-year-7-month-old Italian boy with mental retardation, hypotonia, failure to thrive, coarse facial features, convergent strabismus, pale skin and fair hair. Ultramicroscopic examination of conjunctival and skin tissues showed a number of membrane-bound vacuoles(More)
Iron is released in a free [desferrioxamine (DFO)-chelatable] form in mouse erythrocytes incubated with the oxidizing agents acrolein and phenylhydrazine or in erythrocytes drawn from allyl alcohol-intoxicated mice. The release is accompanied by peroxidation of membrane lipids when the cells are depleted of glutathione. Lipid peroxidation is always followed(More)
The authors report their experience using carbon dioxide (CO2) therapy for the treatment of 48 female patients presenting adipose accumulations, located on the thighs, knees, and/or abdomen; a Carbomed Programmable Automatic Carbon Dioxide Therapy apparatus was used. In light of the effects of CO2 on the microcirculation recently described in the(More)
Little has been published on the histochemical and cytochemical properties of anchoring filaments of initial lymph vessels. Previous research suggests that the microfibrils of the anchoring filaments have ultrastructural, histochemical and cytochemical characteristics similar to those of the microfibrils associated with elastic fibers. With the aim of(More)
Three sisters (ages 27, 24, and 17 years) presented with slowly progressing dystonic dementia and spastic tetraparesis with infantile onset. CSF, bone marrow, and conjunctival cells showed storage vacuoles. Biochemical analysis revealed increased urinary oligosaccharide excretion and decreased activity of acid beta-D-galactosidase and beta-D-fucosidase in(More)