We are analyzing https://link.springer.com/article/10.1007/s00467-007-0650-8.

Title:
Nephropathic cystinosis: late complications of a multisystemic disease | Pediatric Nephrology
Description:
Cystinosis is a rare autosomal recessive disorder due to impaired transport of cystine out of cellular lysosomes. Its estimated incidence is 1 in 100,000 live births. End-stage renal disease (ESRD) is the most prominent feature of cystinosis and, along with dehydration and electrolyte imbalance due to renal tubular Fanconi syndrome, has accounted for the bulk of deaths from this disorder. Prior to renal transplantation and cystine-depleting therapy with cysteamine for children with nephropathic cystinosis, their lifespan was approximately 10 years. Now, cystinotic patients have survived through their fifth decade, but the unremitting accumulation of cystine has created significant non-renal morbidity and mortality. In this article we review the classic presentation of nephropathic cystinosis and the natural history, diagnosis, and treatment of the disorder’s systemic involvement. We also emphasize the role of oral cysteamine therapy in preventing the late complications of cystinosis.
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Keywords {🔍}

cystinosis, pubmed, google, scholar, cas, nephropathic, gahl, pediatr, nephrol, cysteamine, renal, patients, infantile, cystine, med, thoene, disease, bernardini, schneider, central, article, children, treatment, genet, broyer, kaiserkupfer, metab, clin, kleta, dis, therapy, longterm, complications, engl, schulman, neurol, transplantation, child, trauner, research, access, mol, vant, hoff, antignac, human, privacy, cookies, function, content,

Topics {✒️}

end-stage renal disease month download article/chapter randomized placebo-controlled trial insulin-dependent diabetes mellitus lysosomal cystine counter-transport de graaf-hess ac chronic renal failure long-term cysteamine therapy kaiser-kupfer mi veno-occlusive disease inflammatory bowel disease lysosomal cystine transport long-standing nephropathic cystinosis intramural research program privacy choices/manage cookies kaiser-kupper mi kidney dis 39 cystinotic human fibroblasts related subjects kidney transplantation full article pdf sonies bc long-term evolution european collaborative study decreased sweat production kidney int 48 cystine counter-transport electrolyte imbalance due american-based population article nesterova late-treated nephropathic cystinosis european study group age-related prevalence long-term follow human genetics branch african american patients william gahl von bredow ma renal fanconi syndrome long-term treatment long-standing cystinosis intracellular cystine depletion cultured human fibroblasts van den heuvel improved renal function european economic area probable irish origin guay-woodford lm idiopathic intracranial hypertension dissecting aortic aneurysm

Questions {❓}

Olgar S, Nisli K, Dindar A, Omeroglu RE, Ertugrul T (2006) Can cystinosis cause coronary artery dilatation?

Schema {🗺️}

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      headline:Nephropathic cystinosis: late complications of a multisystemic disease
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