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Caspase-4 may play a role in loss of proximal tubules and renal injury in nephropathic cystinosis
Pediatric Nephrology, 08/28/09
Sansanwal P et al. – Nephropathic cystinosis is characterized clinically by generalized proximal renal tubular dysfunction, renal Fanconi Syndrome and progressive renal failure. Glomerular–proximal tubule disconnection has been noted in renal biopsies from patients with nephropathic cystinosis.
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