Asphyxiating Thoracic Dystrophy, or Jeune's Syndrome as we will refer to it from here on out, is an autosomal recessive form of dwarfism. This means that both parents must carry this gene and both must pass it on to the child in order for the child to have the syndrome. Until recently this disorder was considered lethal, with death usually resulting from respiratory problems or from renal failure early on, often within the first year. While there are different degrees to which a child may have this disease, most are characterized by a long, narrow thorax with a reduced thoracic (rib) cage capacity causing the lungs to not have enough room to breath. The child is short in stature, with the long bones in the arms and legs being shorter than average. Abnormalities of the pelvic area are usually present as well. Shortened fingers are characteristic in many cases, though not all. Varying degrees of respiratory difficulty exist, as well as renal (kidney) and hepatic (liver) dysfunction in different cases.
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While children disgnosed with Jeune's Syndome are often given almost hopeless predictions of their future health, much has been done to give these children a good chance in life. Several types of chest expansion surgeries have been developed to give their lungs more room to grow and expand in. Kidney transplants have been successful in treating renal problems, and the general growth of available medical treatments alone has brought about new opportunities to give these children every chance at a healthy life.
To many families one of the hardest things about this syndrome is isolation and lack of information due to its rarity. The Jeune's Family Support group is a group of twenty-seven children ranging in age from one year to twenty-two. We have also gathered many articles, so hat we can send copies to any families needing informationIf you would like any further information please feel free to write to:
Links to other sites on the Web
NORD information file on ATD
Article on chest expansion surgery for ATD.
Medical article describing ATD.
Article on a specific case of chest expansion on a child with ATD.
Scientific session report of using a CT spiral with ATD reconstruction.
The most comprehensive site on ATD that I have found to date, not alot of updated info, but contains many many references.
Family Village Medical site on ATD.
I will attempt to add more information pages on ATD as I come across them on the web; if you know of any not listed, I would appreciate if you would email me the site address. Thank you. Listing of above web sights does not indicate my agreement or the validity of these pages, I am merely listing information that I have found available on the web.
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