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Re: Ivemark syndromeFrom: Joshua Copel (joshua.copel@yale.edu)Sun Jan 10 19:49:14 1999
RE>>Ivemark syndrome 1/10/99 Terry, The address should be at the top of my message, it came out on the full text of yesterday's digest, but in any event it is Dr. Peter Bowers at <PedCard.Fellows@quickmail.yale.edu>. He is certainly interested in any samples. The lab he has been in has been working on mapping the situs inversus gene, and he is looking at left heart hypoplasia/aortic stenosis I believe, but they are interested in banking DNA from any families with affected children who are willing to contribute. The clinical consequences of situs inversus depend on the extent of structural heart disease, the presence of normal splenic function, and less importantly on other lesions. Intestinal malrotation is the rule, and many pediatric surgeons believe that a Ladd procedure to "tack down" the bowel is indicated to prevent formation of volvulus. Since nothing is 100% in medicine, I am sure that there can be other forms of situs inversus. As mentioned in my original post, for example, Kartagener syndrome includes a mirror image situation with the thoracic and abdominal organs, as well as bronchiectasis due to immotile cilia on the bronchial epithelium and abnormalities wherever else cilia are important (for example affected men are infertile due to sperm abnormalities). Josh Copel -------------------------------------- Date: 1/10/99 12:10 PM -------------------------------------- To: Joshua Copel -- -------------------------------------- From: Terry J. DuBose Dr. Copel, thanks for this discussion. Since you mentioned Ivemark syndrome can involve interrupted inferior vena cave, it make me think of a color Doppler image that one of my students brought from clinic. It is from an adult with a duplicated IVC, one on the right anterior of the spine and one two the left. This is not the "mirror artifact" of the IVC that is occasionally seen in sonography, but a true duplication.
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