ABSTRACT
Urbach-Wiethe disease is a rare disorder, inherited in an autosomal recessive fashion. In this work we have studied the clinical data in six patients with well-established UWD. The histopathological and histochemical findings are discussed. Unless the otolaryngologist has a knowledge of the disease, the diagnosis can be missed.
Subject(s)
Laryngeal Mucosa/pathology , Larynx/pathology , Lipidoses/pathology , Lipoid Proteinosis of Urbach and Wiethe/pathology , Adolescent , Adult , Child, Preschool , Esterases/metabolism , Female , Histocytochemistry , Humans , Laryngeal Mucosa/enzymology , Lipoid Proteinosis of Urbach and Wiethe/enzymology , Male , Succinate Dehydrogenase/metabolismABSTRACT
Fifty-eight male and non-pregnant female guinea pigs with an average weight of 400 gm were used in this work, ten animals as a control group, the remaineder receiving oestrogen in the form of ethanyl oestradiol in a dose of 10 microgram/animal/day. Histopathological studies were carried out after periods of one, two, three, four and eight weeks, and three, six and twelve months respectively. The respiratory mucosa, at the different levels mentioned, showed definite changes. The surface epithelium showed metaplastic changes with mitotic figures, until complete and definite stratifications were reached. The cilia decreased and then disappeared completely, while the mucus-secreting goblet cells showed an initial augmentation in number and increased activity, followed by a gradual decrease in their number, until total disappearance occurred at the stage of stratification in the surface epithelium.
Subject(s)
Bronchi/drug effects , Ethinyl Estradiol/pharmacology , Lung/drug effects , Trachea/drug effects , Animals , Bronchi/pathology , Epithelium/drug effects , Epithelium/pathology , Female , Guinea Pigs , Lung/pathology , Male , Mucous Membrane/drug effects , Mucous Membrane/pathology , Trachea/pathologyABSTRACT
An experimental histopathological and histochemical work carried out in thirty guinea-pigs of an average weight of 475 gm. receiving oestrogen in the form of ethynyl oestradiol in a dose of 10 microgram/animal/day and aiming at a study of the effects of oestrogen on the respiratory nasal mucosa. The histopathalogical lesions of the respiratory nasal mucosa were in the form of squamous metaplasia and spongiosis of the lining epithelium, with oedema of the underlying corium, glandular hyperplasia submucosal cellular infiltration, increased vascularity and some vascular changes in the form of endothelial proliferation with intimal thickening. Histochemical enzymatic alterations were in the form of increased succinic dehydrogenase activity in the epithelium as well as in the hyperplastic submucous glands, intensified reaction of the acid phosphatase in the cells of the corium, and the appearance of alkaline phosphatase activity in the apical parts of the cells lining the glands, indicating increased secretory activity. All the changes obtained in the histopathological and histochemical studies can be attributed to hormonal stimulation of the nasal mucosa.
PIP: The histopathological and histochemical effects of 10 mcg/day of eth inyl estradiol (EE) on the nasal respiratory mucosa were studied in 30 female guinea pigs. Histopathological changes observed after treatment were: 1) squamous metaplasia, 2) spongcosis and edema of the corium, 3) glandular hyperplasia, 4) cellular infiltrations, 5) increased vascularity, and 6) vascular changes in the form of endothelial proliferation with intimal thickening. The observed histochemical changes were: 1) increased succinic dehydrogenase activity in the epithelium and hyperplastic submucous glands, 2) an intensified reaction of acid phosphatase activity in the cells of the corium, and 3) alkaline phosphatase activity in the apical areas of the cells lining the glands. All of the observed changes are attributed to the stimulation of the nasal mucosa by EE.
