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Re: Ivemark SyndromeFrom: Terry J. DuBose (tjdubose@juno.com)Sat Jan 9 18:40:19 1999
Thanks Mary, as I have said earlier today, I have learned a lot from our discussions on the Internet. Your information encouraged me to take the time to do a MedLine search and I did find 7 articles concerning Ivemark and Ultrasound (I probably should have done the search first, would not have shown my ignorance ;-). Two of the articles discuss with prenatal diagnosis. The following are the references and abstracts. Gaudenz F. Bon of Switzerland hope this helps. Peace, Terry J. DuBose, M.S., RDMS, FAIUM University of Arkansas for Medical Sciences, USA <1> Unique Identifier 98109853 Authors Hajdu J. Marton T. Papp C. Cesko I. Oroszne NJ. Papp Z. Institution Semmelweis Orvostudomanyi Egyetem 1. sz. Szuleszeti es Nogyogyaszati Klinikaja, Budapest. Title [Prenatal diagnosis of atrioventricular septal defect and its prognostic significance]. [Review] [30 refs] [Hungarian] Source Orvosi Hetilap. 139(1):23-6, 1998 Jan 4. Abstract Atrioventricular septal defect also known as endocardial cushion defect is severe congenital heart disease which is often associated with chromosomal abnormalities (30-50%) or is a part of a malformation syndrome (30%). Between 1, october 1992, and 30, september 1996. 21 cases were diagnosed prenatally. The chromosomal analysis found in 1 case trisomy 18, in 1 case trisomy 22 and in 7 cases trisomy 21. In 7 cases atrioventricular septal defect was part of Ivemark syndrome, none of them had chromosomal abnormality, except 1, who had trisomy 18. Out of 21 cases there was only one survivor. None of cases associated with Ivemark syndrome survived. In 4 cases sibs or parents had congenital heart disease. Four mother were above 40 years. The report summarises the most important abnormalities in the prenatal diagnosis of endocardial cushion defect and echocardiographic and embryopathologic foundings. [References: 30] <2> Unique Identifier 96131536 Authors Larson RS. Rudloff MA. Liapis H. Manes JL. Davila R. Kissane J. Institution Department of Pathology, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA. Title The Ivemark syndrome: prenatal diagnosis of an uncommon cystic renal lesion with heterogeneous associations. [Review] [16 refs] Source Pediatric Nephrology. 9(5):594-8, 1995 Oct. Abstract Renal dysplasia has been reported in association with a number of anatomical abnormalities, including pancreatic dysgenesis and hepatic anomalies. The combination of renal, hepatic, and pancreatic dysplasia (RHPD), also known as Ivemark syndrome, is rare and uniformly fatal. We report here the gross and histological findings in 4 cases of combined RHPD, 2 of which were detected by prenatal ultrasonography. Evaluation of these 4 and the other 20 reported cases shows that combined RHPD has considerable variability in the histological findings and in organ involvement. In addition, nearly half were associated with anomalies in other organ systems, and 11 of the 24 were familial. In this study, ultrasonographic and histological abnormalities were seen as early as 18.5 weeks gestation in 1 case. [References: 16]
On Sat, 9 Jan 1999 18:12:57 -0600 case@mediaone.net (M.C. Scarboro RNC
RDMS) writes:
>Terry
or call Juno at (800) 654-JUNO [654-5866]
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