Number of the records: 1  

xantomatóza cerebrotendinózna

  1. SYSd019294
    LBL
      
    00000nz--a2200000o--4500
    005
      
    20250606220354.2
    008
      
    960610|||anznnbabn-----------|-a|a------
    040
      
    $b slo $a DNLM $d BA006
    065
      
    $a C16.320.565.398.925
    065
      
    $a C18.452.584.563.925
    065
      
    $a C18.452.584.750.975
    065
      
    $a C18.452.648.398.925
    066
      
    $a 01 $c 03
    150
      
    $a xantomatóza cerebrotendinózna $x BL $x CF $x CI $x CL $x CO $x DG $x DH $x DI $x DT $x EC $x EH $x EM $x EN $x EP $x ET $x GE $x HI $x IM $x ME $x MI $x MO $x NU $x PA $x PC $x PP $x PS $x PX $x RH $x RT $x SU $x TH $x UR $x VE $x VI $2 slo
    450
      
    $w v $a Van Bogaert-Scherer-Epstein Disease $2 eng
    450
      
    $w v $a van Bogaertov-Schererov-Epsteinov syndróm $2 slo
    450
      
    $w v $a cholesterinosis cerebrotendinosa $2 slo
    450
      
    $w v $a xanthomatosis normolipaemica $2 slo
    450
      
    $w v $a CTX $2 slo
    450
      
    $w v $a van Bogaertova-Schererova-Epsteinova choroba $2 slo
    550
      
    $7 sllk_us_auth*d053493 $Y Cholestanetriol 26-Monooxygenase $w b $a cholestántriol 26-monooxygenáza
    665
      
    $a 97 $2 eng
    665
      
    $a Xanthomatosis (1968-1996) $2 eng
    680
    9-
    $i An autosomal recessive lipid storage disorder due to mutation of the gene CYP27A1 encoding a CHOLESTANETRIOL 26-MONOOXYGENASE. It is characterized by large deposits of CHOLESTEROL and CHOLESTANOL in various tissues resulting in xanthomatous swelling of tendons, early CATARACT, and progressive neurological symptoms. $2 eng
    750
    -2
    $a Xanthomatosis, Cerebrotendinous $2 eng
    980
      
    $x M
Number of the records: 1  

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