cystinosis/albümin

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Urinary protein excretion pattern and renal expression of megalin and cubilin in nephropathic cystinosis.

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BACKGROUND Nephropathic cystinosis is the most common cause of inherited renal Fanconi syndrome, caused by mutations in lysosomal cystine carrier cystinosin that result in lysosomal cystine accumulation throughout the body. How defects in cystinosin cause proximal tubular dysfunction is not known.

ACE inhibitorenalapril diminishes albuminuria in patients with cystinosis.

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OBJECTIVE Cystinosis, a rare autosomal recessive disease, manifests with renal Fanconi syndrome during the first year of life. Interstitial damage is a major cause of renal failure in patients with cystinosis. We presume that albuminuria contributes to the development of renal failure in these

[Continuous enteral feeding in pediatric nephrology. Long-term results in children with congenital nephrotic syndrome, severe cystinosis and renal failure (author's transl)].

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This is the report of the results obtained with continuous enteral feeding in congenital nephrotic syndrome (3 cases), cystinosis (3 cases) and renal failure (3 cases). Ages of patients at the beginning of treatment ranged from 10 days to 3 years. Enteral nutrition was given for periods ranging from

Native nephrectomy prior to pediatric kidney transplantation: biological and clinical aspects.

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BACKGROUND Pre-transplant nephrectomy is performed to reduce risks to graft and recipient. The aims of this study were to evaluate (1) indications, surgical approach, and morbidity of native nephrectomy and (2) the effects of kidney removal on clinical and biological parameters. METHODS This study

Role of thiols in degradation of proteins by cathepsins.

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The effects of thiols on the breakdown of 125I-labelled insulin, albumin and formaldehyde-treated albumin by highly purified rat liver cathepsins B, D, H and L at pH 4.0 and 5.5 were studied. At both pH values degradation was strongly activated by the thiols cysteamine, cysteine, dithiothreitol,

Cysteamine restores glutathione redox status in cultured cystinotic proximal tubular epithelial cells.

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Recent evidence implies that impaired metabolism of glutathione has a role in the pathogenesis of nephropathic cystinosis. This recessive inherited disorder is characterized by lysosomal cystine accumulation and results in renal Fanconi syndrome progressing to end stage renal disease in the majority

Urine retinol-binding protein 4: a functional biomarker of the proximal renal tubule.

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Measurement of retinol-binding protein 4 in urine (uRBP4) is arguably the most sensitive biomarker for loss of function of the human proximal renal tubule. Megalin- and cubilin-receptor-mediated endocytosis normally absorbs > 99% of the approximately 1.5 g/24 h of protein filtered by the renal

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Herbal & Natural Medicine

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