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    Go to the top of the page   ID: 20060322175555 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] There are Ultrasound-images available for this case. [ Ultrasound ] There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    A. Nordwig, N. Lorenz, B. Biereder, T. Haufe, M. Kabus (Städtisches Krankenhaus Dresden-Neustadt-Deutschland)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    15 Years  

     
     Pediatric Radiology CasesGender:

    Male  

     
     Pediatric Radiology CasesRegion-Organ:

    Abdomen-Liver  

     
     Pediatric Radiology CasesMost likely etiology:

    inflammatory or infectious  

     
     Pediatric Radiology CasesHistory:

    - 15 year old boy, clinically and neurologically unremarkable
    - 03/2005 Diagnosis of rohn's disease, therapy: Azathioprin, Mesalazin
    - 11/2005 generalised pruritus, mild scleric icterus
    - Labs: Leukocytes 4,1 Gpt/l, Thrombocytes 152 Gpt/l, ASAT 2,28 µmol/s*l, ALAT 3,11 µmol/s*l, Gamma-GT 4,11 µmol/s*l, Bilirubin gesamt 40 µmol/l, Bilirubin direct 17 µmol/l, Bile acides 418 µmol/l , BSG, Alpha1-Glycoprotein, AP, Coagulation, Albumin, Protein electrophoresis, Cholinesterase, Ammonia, Alpha1-Antitrypsin, Iron, Ferritin, Copper, Coeruloplasmin within normal limits.
    - Autoantibody screening: pANCA positive, ANA, AMA, SMA, LKM, PBCNA, LC1 negative.
     

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    PSC is associated in 87% with Ulcerative colitis and in 13% with Crohn's disease and precedes the cIBD (chronic inflammatory bowel disease) symptoms in 50% (Wilschanski M et al, 1995). The pathogenesis of the chronic inflammation of the bile ducts is not clear. Discussed are chronische portal bacterimia, toxic bile metabolites from the intestinal flora, ischemic vascular factors and autoimmun processes which lead to an inflammation and scarring with stenosis of the bile ducts. Affected are initially in most cases small intrahepatic and later on also extrahepatic bile ducts.  

     
     Pediatric Radiology CasesRadiological findings:


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



    MRI 1 <- view MRI 1

    MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





    X-Ray 1 <- view X-Ray 1

    X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    Total constellation (Consens)  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Drug induced hepatopathy (Azathioprin), autoimmunehepatitis, primary biliary cirrhosis.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    PSC leads often to liver transplantation. Feared is PSC within the transplanted organ. Course and grade of PSC are independent of the activity of cIBD. Therapy of choice is ursodesoxycholic acid with a positive, but only transient effect on the symptoms and lab results (Feldstein AE et al, 2003). There is no proven influence on mortality or liver transplant rate by ursodesoxycholic acid (Breuers U et al, 1992). Very high doses of ursodesoxycholic acid improve possible the log time survival in a PSC patient (40 mg/kg KG). The early endoscopic dilatation of stenotic bile ducts and eventual stent implant is effective in addition to drug therapy (Stiehl A et al, 2002).  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    N/A  

     
     Pediatric Radiology CasesKeywords:

    Primary sclerosing cholangitis, PSC, Crohn, liver disease, Crohn's disease, generalised pruritus, icterus, scleric icterus, child, childhood, pediatric radiology  

     
     Pediatric Radiology Cases Cite this article:

    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555  

     
     Pediatric Radiology Cases Read similar articles: with corresponding keywords
    in the same field: Abdomen-Liver
    or in the region: Abdomen
    or in the tissue/organ: Liver
    or with the etiology: inflammatory or infectious
     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] There are Ultrasound-images available for this case. [ Ultrasound ] There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     

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    Primary sclerosing cholangitis (PSC)
    Other cases by these authors:

    Search A. Nordwig in Medline A. Nordwig (4)   
    Search N. Lorenz in Medline N. Lorenz (1)   
    Search B. Biereder in Medline B. Biereder (1)   
    Search T. Haufe in Medline T. Haufe (4)   
    Search M. Kabus in Medline M. Kabus (1)   

    Primary sclerosing cholangitis (PSC)  
     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     

    Which diagnosis have other collegues guessed?


    • Primary sclerosing cholangitis
      Votes: 16 (88 %)


    • Caroli syndrome
      Votes: 1 (5 %)


    • Obstructing concrement in the ductus choledochus
      Votes: 0 (0 %)


    • Hepatitis
      Votes: 0 (0 %)


    • Liver cirrhosis
      Votes: 1 (5 %)



        Total answers: 18

     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     

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    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     




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