Matches in SemOpenAlex for { <https://semopenalex.org/work/W2073599719> ?p ?o ?g. }
- W2073599719 endingPage "562" @default.
- W2073599719 startingPage "555" @default.
- W2073599719 abstract "Cystinosis, an autosomal recessive disorder of lysosomal cystine accumulation, results from mutations in the CTNS gene that encodes the lysosomal cystine transporter, cystinosin. Renal tubular Fanconi syndrome occurs in infancy, followed by rickets, growth retardation, photophobia, and renal failure, which requires renal transplantation at approximately 10 yr of age. Treatment with cysteamine decreases cellular cystine levels, retards renal deterioration, and allows for normal growth. Patients with a history of inadequate cystine depletion therapy may survive, after renal transplantation, into the third to fifth decades but will experience other, extrarenal complications of the disease. Routine chest and head computed tomography scans of 41 posttransplantation patients with cystinosis were reviewed for vascular calcification. The radiologic procedures had been performed to examine lung and brain parenchyma, so there was little ascertainment bias. Thirteen of the 41 patients had vascular calcification, including 11 with coronary artery calcification. One 25-yr-old man required three-vessel coronary artery bypass graft surgery. There were no significant differences between the 13 patients with calcification and the 28 without calcification in the following parameters: Time on dialysis, frequency of transplantation, hypertension, hypercholesterolemia, homozygosity for the 57-kb deletion in CTNS, serum creatinine, and calcium-phosphate product. However, the finding of vascular calcification correlated directly with duration of life without cysteamine therapy and inversely with duration of life under good cystine-depleting therapy. The accumulation of intracellular cystine itself maybe a risk factor for vascular calcifications, and older patients with cystinosis should be screened for this complication." @default.
- W2073599719 created "2016-06-24" @default.
- W2073599719 creator A5012741329 @default.
- W2073599719 creator A5024215317 @default.
- W2073599719 creator A5025176644 @default.
- W2073599719 creator A5033894193 @default.
- W2073599719 creator A5043234105 @default.
- W2073599719 creator A5078931814 @default.
- W2073599719 creator A5082442398 @default.
- W2073599719 creator A5090769479 @default.
- W2073599719 date "2006-05-01" @default.
- W2073599719 modified "2023-09-25" @default.
- W2073599719 title "Coronary Artery and Other Vascular Calcifications in Patients with Cystinosis after Kidney Transplantation" @default.
- W2073599719 cites W19129488 @default.
- W2073599719 cites W1971853757 @default.
- W2073599719 cites W1975360166 @default.
- W2073599719 cites W1980180365 @default.
- W2073599719 cites W1985381301 @default.
- W2073599719 cites W1990560333 @default.
- W2073599719 cites W1996130473 @default.
- W2073599719 cites W2001137143 @default.
- W2073599719 cites W2003106879 @default.
- W2073599719 cites W2011786812 @default.
- W2073599719 cites W2012412619 @default.
- W2073599719 cites W2016945265 @default.
- W2073599719 cites W2029870086 @default.
- W2073599719 cites W2032200785 @default.
- W2073599719 cites W2051852204 @default.
- W2073599719 cites W2061542287 @default.
- W2073599719 cites W2071484860 @default.
- W2073599719 cites W2071818248 @default.
- W2073599719 cites W2072047406 @default.
- W2073599719 cites W2084818126 @default.
- W2073599719 cites W2085905503 @default.
- W2073599719 cites W2090309128 @default.
- W2073599719 cites W2090735785 @default.
- W2073599719 cites W2090824142 @default.
- W2073599719 cites W2093471474 @default.
- W2073599719 cites W2095558456 @default.
- W2073599719 cites W2106652794 @default.
- W2073599719 cites W2110140698 @default.
- W2073599719 cites W2143600593 @default.
- W2073599719 cites W2156215371 @default.
- W2073599719 cites W2168938422 @default.
- W2073599719 cites W2315717074 @default.
- W2073599719 cites W2327035781 @default.
- W2073599719 cites W2328615536 @default.
- W2073599719 cites W2341322678 @default.
- W2073599719 cites W2465739619 @default.
- W2073599719 cites W2474023059 @default.
- W2073599719 cites W4233141843 @default.
- W2073599719 doi "https://doi.org/10.2215/cjn.01431005" @default.
- W2073599719 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/17699259" @default.
- W2073599719 hasPublicationYear "2006" @default.
- W2073599719 type Work @default.
- W2073599719 sameAs 2073599719 @default.
- W2073599719 citedByCount "41" @default.
- W2073599719 countsByYear W20735997192012 @default.
- W2073599719 countsByYear W20735997192013 @default.
- W2073599719 countsByYear W20735997192014 @default.
- W2073599719 countsByYear W20735997192015 @default.
- W2073599719 countsByYear W20735997192016 @default.
- W2073599719 countsByYear W20735997192017 @default.
- W2073599719 countsByYear W20735997192018 @default.
- W2073599719 countsByYear W20735997192019 @default.
- W2073599719 countsByYear W20735997192020 @default.
- W2073599719 countsByYear W20735997192021 @default.
- W2073599719 countsByYear W20735997192022 @default.
- W2073599719 countsByYear W20735997192023 @default.
- W2073599719 crossrefType "journal-article" @default.
- W2073599719 hasAuthorship W2073599719A5012741329 @default.
- W2073599719 hasAuthorship W2073599719A5024215317 @default.
- W2073599719 hasAuthorship W2073599719A5025176644 @default.
- W2073599719 hasAuthorship W2073599719A5033894193 @default.
- W2073599719 hasAuthorship W2073599719A5043234105 @default.
- W2073599719 hasAuthorship W2073599719A5078931814 @default.
- W2073599719 hasAuthorship W2073599719A5082442398 @default.
- W2073599719 hasAuthorship W2073599719A5090769479 @default.
- W2073599719 hasBestOaLocation W20735997191 @default.
- W2073599719 hasConcept C126322002 @default.
- W2073599719 hasConcept C126894567 @default.
- W2073599719 hasConcept C134018914 @default.
- W2073599719 hasConcept C141071460 @default.
- W2073599719 hasConcept C142724271 @default.
- W2073599719 hasConcept C164705383 @default.
- W2073599719 hasConcept C181199279 @default.
- W2073599719 hasConcept C185592680 @default.
- W2073599719 hasConcept C2776609658 @default.
- W2073599719 hasConcept C2777608289 @default.
- W2073599719 hasConcept C2778518273 @default.
- W2073599719 hasConcept C2778653478 @default.
- W2073599719 hasConcept C2779026464 @default.
- W2073599719 hasConcept C2779201268 @default.
- W2073599719 hasConcept C2780091579 @default.
- W2073599719 hasConcept C2780303639 @default.
- W2073599719 hasConcept C2780309369 @default.
- W2073599719 hasConcept C2911091166 @default.
- W2073599719 hasConcept C55493867 @default.