Progression of nonimmune hydrops in a fetus with Noonan syndrome.
Identifieur interne : 004F84 ( PubMed/Checkpoint ); précédent : 004F83; suivant : 004F85Progression of nonimmune hydrops in a fetus with Noonan syndrome.
Auteurs : V L Katz ; B. Kort ; W J WatsonSource :
- American journal of perinatology [ 0735-1631 ] ; 1993.
Descripteurs français
- KwdFr :
- MESH :
- étiologie : Anasarque foeto-placentaire.
- Adulte, Femelle, Grossesse, Humains, Hydramnios, Issue fatale, Maladies foetales, Mâle, Nouveau-né, Syndrome de Noonan.
English descriptors
- KwdEn :
- MESH :
- complications : Noonan Syndrome.
- etiology : Hydrops Fetalis.
- Adult, Fatal Outcome, Female, Fetal Diseases, Humans, Infant, Newborn, Male, Polyhydramnios, Pregnancy.
Abstract
Presented is a case of nonimmune hydrops caused by Noonan syndrome. This autosomal dominant disorder, with an incidence of 1 in 1000 births, is manifested by trunk and limb edema, lymphatic abnormalities, webbed neck, short stature, and cardiac and genital anomalies. In this case the initial presentation was polyhydramnios at 31 weeks' gestation, followed in 1 week by pleural effusions and a week later by skin edema. An 18-week ultrasound had been normal. Because of variable expressivity, only the most severe cases of the disease are usually diagnosed. Thus, the incidence may be higher than 1 in 1000, and Noonan syndrome may be underdiagnosed as a cause of nonimmune hydrops. Since lymphedema in this syndrome may regress spontaneously, conservative obstetric management is preferable unless the fetus develops distress.
DOI: 10.1055/s-2007-994620
PubMed: 8267802
Affiliations:
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pubmed:8267802Le document en format XML
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<front><div type="abstract" xml:lang="en">Presented is a case of nonimmune hydrops caused by Noonan syndrome. This autosomal dominant disorder, with an incidence of 1 in 1000 births, is manifested by trunk and limb edema, lymphatic abnormalities, webbed neck, short stature, and cardiac and genital anomalies. In this case the initial presentation was polyhydramnios at 31 weeks' gestation, followed in 1 week by pleural effusions and a week later by skin edema. An 18-week ultrasound had been normal. Because of variable expressivity, only the most severe cases of the disease are usually diagnosed. Thus, the incidence may be higher than 1 in 1000, and Noonan syndrome may be underdiagnosed as a cause of nonimmune hydrops. Since lymphedema in this syndrome may regress spontaneously, conservative obstetric management is preferable unless the fetus develops distress.</div>
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<Abstract><AbstractText>Presented is a case of nonimmune hydrops caused by Noonan syndrome. This autosomal dominant disorder, with an incidence of 1 in 1000 births, is manifested by trunk and limb edema, lymphatic abnormalities, webbed neck, short stature, and cardiac and genital anomalies. In this case the initial presentation was polyhydramnios at 31 weeks' gestation, followed in 1 week by pleural effusions and a week later by skin edema. An 18-week ultrasound had been normal. Because of variable expressivity, only the most severe cases of the disease are usually diagnosed. Thus, the incidence may be higher than 1 in 1000, and Noonan syndrome may be underdiagnosed as a cause of nonimmune hydrops. Since lymphedema in this syndrome may regress spontaneously, conservative obstetric management is preferable unless the fetus develops distress.</AbstractText>
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