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Biomedical subjects

M Kaiser-Kupfer

Publications and source records attributed to M Kaiser-Kupfer.

25 records · Page 2Linked to original sources
PubMed1980

Gyrate atrophy of the choroid and retina: amino acid metabolism and correction of hyperornithinemia with an arginine-deficient diet.

Four patients with gyrate atrophy of the choroid and retina were studied, all of whom exhibited the hyperornithinemia characteristic of this disorder. Elevated plasma histidine and diminished plasma lysine and branched-chain amino acids were also noted. The renal clearances of these four amino acids were not sufficiently elevated to explain their low plasma levels. In one subject, an arginine-deficient diet led to progressive reduction in plasma ornithine from 13 times normal to the upper limits of normal, along with the disappearance of ornithinuria and lysinuria. Orally administered alpha-aminoisobutyric acid facilitated the fall in plasma ornithine by increasing renal losses of ornithine. It also increased the clearances of most other amino acids. When plasma ornithine approached normal (less than 200 microM), plasma lysine became normal, plasma arginine became subnormal, and renal clearances of basic amino acids decreased. Long-term (1.5 yr) maintenance with a diet containing 10-20 g of protein plus essential amino acids served to keep plasma ornithine at between 55-355 microM; chorioretinal degeneration did not progress and vision apparently improved.

Adult↗
PubMed1977

Progressive bilateral essential iris atrophy.

Bilateral progressive essential iris atrophy, more advanced in one eye than in the other, occurred in a 24-year-old white man. Histopathologic examination of the iris from the eye with the more advanced iris atrophy disclosed no vascular abnormalities in that the vessels were patent and appeared normal except for accumulated perivascular connective tissue. Fluorescein angiographic examination of the fellow eye that was undergoing the earliest changes of iris atrophy failed to reveal any vascular abnormalities. However, tonographic examination indicated a markedly decreased outflow facility. There does not appear to be any vascular abnormality involved in the pathogenesis.

Adult↗
PubMed1976

Progressive bilateral essential iris atrophy.

A case of bilateral progressive essential iris atrophy, more advanced in one eye than the other, has been studied. Histopathologic examination of the iris from the eye with the more advanced iris atrophy failed to find any vascular abnormalities in that the vessels were patent and appeared normal except for accumulated perivascular connective tissue. Fluorescein angiographic examination of the right eye which was undergoing the earliest changes of iris atrophy failed to show any vascular abnormalities. However, tonographic examination indicated a markedly decreased outflow facility. There does not appear to be any vascular abnormality involved in the pathogenesis of this case.

Adult↗
PubMed1975

The histopathology of pigmentary dispersion syndrome with glaucoma.

Iris tissue and trabecular meshwork, obtained at time of trabeculectomy, was studied using the light and electron microscope in a 54-year-old woman with pigmentary dispersion syndrome with glaucoma. The specific defect was a loss of the outer epithelial cells of the iris with marked thinning of the remaining outer layers so that the two-cell architecture of the iris epithelium was maintained. In addition, the radial muscle layer was increased in both number and size of muscle fiber. It would appear that the pigmentary dispersion syndrome may represent a congenital or developmental abnormality of the iris epithelium, or both, and that the glaucoma which occasionally occurs in conjunction with this syndrome is of the usual open-angle type.

Atrophy↗