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Biomedical subjects

K Khun

Publications and source records attributed to K Khun.

13 recordsLinked to original sources

[Oxalosis in patients on regular dialysis therapy].

The authors draw attention to the case of a 46-year-old man who developed after three years of regular dialyzation treatment three times a week the first manifestations of acquired generalized oxalosis. The oxalosis was most marked on the skin, skeleton, heart, and kidneys. Oxalosis was confirmed after eight years of dialysis after death by post-mortem and histological examination and microscopic detection of oxalate crystals. The authors recommend that as part of regular dialyzation treatment patients should not be given more than 100 mg ascorbic acid per day. Oxalates are also the end product of degradation of ascorbic acid and high doses may lead also to hyperoxalosis. Moreover deposits in some visceral organs, in particular the heart may cause clinical manifestations. In the authors patients they caused attacks of auricular flutter. Although these clinical manifestations develop only rarely, the latent form of acquired oxalosis is probably more frequent.

Bone Diseases

Membranocystic lesion in the brain in cerebrotendinous xanthomatosis. Histochemical and ultrastructural study with evidence of its ceroid nature.

A case is described of cerebrotendinous xanthomatosis with purely neurological manifestations. Cholestanol deposition in both affected and unaffected brain regions was markedly increased, reaching 18.5-20.8% of the sterol fraction. The unilateral lesions localized in the basal ganglia and cerebellar white matter featured perivascular accumulation of foam cells containing apolar lipid and ceroid. Necrosis with lipid-rich debris was a frequent finding often accompanied by prominent collagen deposition. Within these lesions there were numerous refractile thick membranes which, according to lipid histochemical techniques, could be qualified as ceroid-type lipopigment. It is suggested that the ceroid membranes arise extracellularly directly from the lipid-rich debris. Ultrastructurally, they were composed of convolutes of highly organized trilaminar membranes about 15 nm thick similar to those seen in intracellular ceroid granules. The membranes were embedded in an amorphous substance of low or medium density and were identical in their general appearance, stainability and fine structure to the membranocystic lesion in Nasu-Hakola disease and to the extracellular ceroid in atherosclerotic plaques.

Adult