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    Go to the top of the page   ID: 20030122112757 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    | The Authors | Discussion | Write a Comment to this Case |
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    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


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

    J. Blöcker, G. Hahn (Dresden)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    <1 Years  

     
     Pediatric Radiology CasesGender:

    N/A  

     
     Pediatric Radiology CasesRegion-Organ:

    Syndromes  

     
     Pediatric Radiology CasesMost likely etiology:

    congenital  

     
     Pediatric Radiology CasesHistory:

    Prenatally, an obvious dysplastic thorax and polyhydramy was seen in ultrasound. At the time of birth, eutrophic newborn 37+1, 45 cm, 2590 grams, 45 cm, 35 cm head circumference. Clinically noticeable thorax dysplasia (long, narrow thorax) with congenital lung hypoplasia and respiratory insufficiency (long-term respiration).

    Initally, patent ductus arteriosus and a small patent foramen ovale, which was no longer seen after 14 days.
    Oligury (during treatment with furosemide, spironolactone, etacrynic acid, diuresis was normal), obvious flank edema.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    -Autosomal-recessive illness.
    -Pathogenesis unknown.

    Typical findings:
    -short, horizontally running rib with widening of the costochondral connections; distally emphasized shortened and thickened long bones; three point configuration of the lower edge of the pelvis. Horizontal acetabula; early ossification of the femur head epiphyses
    -cystic tubular dysplasia; renal insufficiency with proteinuria and arterial hypertension; Nephronophtisis, polycystic kidney, usually starting in grade school.

     

     
     Pediatric Radiology CasesRadiological findings:


    X-Ray 1 <- view X-Ray 1

    X-ray 1: Thorax a. p. : short, horizontally running rib with widening of the costochondral connections.







    X-Ray 2 <- view X-Ray 2

    X-ray 2: pelvis a. p. : typical three point configuration of the lower edge




    X-Ray 3 <- view X-Ray 3

    X-ray 3: baby image: long bones (Femur/Humerus) appear shortened in comparison to the long thorax







     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    Expert's opinion  

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

    Forms of the short-rib-polydactaly-syndrome.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Frequency:
    - Over 50 published cases

    Course/Prognosis:
    - Early infancy is often not outlived
    - Later, the kidney insufficiency determines the prognosis
    - The dyspnea lessens, the thoracic dysplasia remains the same.
    - Usually, normal intelligence  

     
     Pediatric Radiology CasesComments of the author about the case:

    Sonographic: Skull and abdomen unnoticeable.  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    1. Medline: Medline
    Wiedemann,Kunze
    Atlas der klinischen Syndrome 5. Auflage
    Schattauer GmbH 2001  

     
     Pediatric Radiology CasesKeywords:

    Asphyxiating thoracic dysplasia, Asphyxial thorax dysplasia, Jeune, Thorax, Asphyxiating, Dysplasia, child, childhood, pediatric radiology  

     
     Pediatric Radiology Cases Cite this article:

    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757  

     
     Pediatric Radiology Cases Read similar articles: with corresponding keywords
    in the same field: Syndromes
    or in the region: Syndromes
    or in the tissue/organ:
    or with the etiology: congenital
     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] View all modalities [ All ]   
     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     

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    Asphyxiating thoracic dysplasia (Jeune syndrome)
    Other cases by these authors:

    Search J. Blöcker in Medline J. Blöcker (2)   
    Search G. Hahn in Medline G. Hahn (22)   

    Asphyxiating thoracic dysplasia (Jeune syndrome)  
     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     

    Which diagnosis have other collegues guessed?


    • Short rib-polydactyly syndrome
      Votes: 4 (28 %)


    • Asphyxiating thoracic dysplasia (Jeune Syndrome)
      Votes: 6 (42 %)


    • Tantaphoric dysplasia
      Votes: 2 (14 %)


    • Chondrodysplasia punktata
      Votes: 2 (14 %)



        Total answers: 14

     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     

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    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     




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