Primary Familial Amyloidosis

Secondary Glaucoma Accompanied with Primary Familial Amyloidosis

“…The secondary glaucoma accompanied with primary familial amyloidosis has been re ported by Kaufman [4], Duke and Tat on [20], Talon and Duke [5], Legrand el al. [6] Meretoja [7], Limon et al [8] and Meretoja et al [9], All of these descrip tions have been done about one or two cases at the most and their main purpose was not to report about the secondary glaucoma induced by amyloidosis.…”

“…15 out of 22 patients underwent ophthalmologieal examination, and secondary glaucoma, due to primary familial amyloidosis, was found in 4 patients. The clinical characteristic features of the secondary glaucoma were described as follows: (I) having the systemic symptoms of primary fa milial amyloidosis; (2) belonging to relatively older age groups and having a longer duration of the course of the disease; (3) having vitreous opacities; (4) having the wide open angle with pigment de position in the gonioscopical examination; (5) hav ing white flocculent materials in the pupillary margin, and (6) having flaky substances on the surface of the lens.…”

“…Until now, Kaufman [4], Paton and Duke [5], Legrand et al [6], Meretoja [7], Limon et al [8] and Meretoja and Tarkannen [9], described the secondary glaucoma accompanied with primary famil ial amyloidosis. However, the pathogenesis of this glaucoma is unknown and histo logical examination of the trabecular meshwork has not been carried out in these reports by electron microscope.…”

Genetic factors in amyloidosis.

“…Kantarjian and Dejong (1953) described a family from the United States in which a man and his two daughters were affected. The features again in general resembled the Portuguese type, with an insidious onset after the second decade, as did those in a further family from the United States (Shulman and Bartter, 1956;von Sallmann et al, 1960), in which particular attention was paid to a description of vitreous opacification due to amyloid (Kaufman, 1958;Kaufman and Thomas, 1959;Paton and Duke, 1966).…”

Unusual Cause of Vitreous Opacities

“…Andersson and Kassman [4] described a number of cases in Scandinavian families and there seems to be no doubt that our cases belong to the same type. According to Kaufman [5] the vitreous opacities are the most distinctive ocular feature and may be looked upon as pathognomonic; Brownstein et al [6], however, have some doubts about this. …”