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Medline ® Abstract for Reference 109

of 'Renal manifestations of tuberous sclerosis complex'

109
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[Tuberous sclerosis with chronic renal failure treated by hemodialysis and transplantation (author's transl)].
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Meyrier A, Rainfray M, Roland J, Merlier J
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Nephrologie. 1980;1(2):85-8.
 
We report on a case of tuberous sclerosis in a 36-year-old female with chronic renal failure, who was treated by hemodialysis and transplantation. The diagnosis of tuberous sclerosis was ascertained by the association of typical cutaneous retinal and osseous lesions. On the contrary, the renal localisations were unusual. Early hypertension was exacerbated in the course of 2 pregnancies but was never severe. It preceded the late onset of renal failure which progressed slowly. The kidneys were small, with a peculiar angiographic aspect: the renal tissue was not invaded by hamartomas but the whole arterial bed was diffusely modified, with a cortical perfusion defect. Both kidneys were studied after bilateral nephrectomy. The lesions were far more complex than a simple replacement of the renal tissue by angiomyolipomas: they involved the vessels, the interstitium (with lipids inclusions surrounded by a macrophagic reaction) and the glomeruli (with focal and segmental sclerosis). This observation (which is the 11th case in the literature of tuberous sclerosis with chronic renal failure and the 3rd treated by transplantation) documents a particular variety of congenital and familial nephropathy with delayed revelation.
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