Abstract
Summary Two autopsy cases of children with cystinosis (Abderhalden-Lignac-Fanconi syndrome) were examined with regard to bone changes. The nature of bone changes and the effect of a vitamin D therapy upon them (one of the cases was treated with high doses of vitamin D) were especially studied. The trabecular bone was morphometrically analysed in undecalcified sections of the iliac crest. The quantitative data were compared with those obtained in a control collective of the same age group with normal skeletons. In both cases, we observed an interstitial nephritis with atrophy of the renal parenchyme caused by cystinosis. The renal failure induced the charactaristic bone alterations which were measured morphometrically. The bone changes were charactarized by an increase of the total bone mass in both cases. All parameters of bone resorption were increased. Only in one case was bone formation activated and osteoid volume enlarged, even though this patient was treated with high doses of vitamin D. Renal insufficiency in cystinosis induces a typical renal osteopathy charactarized by secondary hyperparathyroidism and severe osteomalacia. Vitamin D therapy does not influence the development of this metabolic bone disease.
Published Version
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