Progression of nonimmune hydrops in a fetus with Noonan syndrome.
Identifieur interne : 005666 ( PubMed/Corpus ); précédent : 005665; suivant : 005667Progression 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.
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
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Kort</name></author><author><name sortKey="Watson, W J" sort="Watson, W J" uniqKey="Watson W" first="W J" last="Watson">W J Watson</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1993">1993</date><idno type="RBID">pubmed:8267802</idno><idno type="pmid">8267802</idno><idno type="doi">10.1055/s-2007-994620</idno><idno type="wicri:Area/PubMed/Corpus">005666</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">005666</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Progression of nonimmune hydrops in a fetus with Noonan syndrome.</title><author><name sortKey="Katz, V L" sort="Katz, V L" uniqKey="Katz V" first="V L" last="Katz">V L Katz</name><affiliation><nlm:affiliation>Department of OB/GYN, UNC School of Medicine, Chapel Hill 27599-7570.</nlm:affiliation></affiliation></author><author><name sortKey="Kort, B" sort="Kort, B" uniqKey="Kort B" first="B" last="Kort">B. Kort</name></author><author><name sortKey="Watson, W J" sort="Watson, W J" uniqKey="Watson W" first="W J" last="Watson">W J Watson</name></author></analytic><series><title level="j">American journal of perinatology</title><idno type="ISSN">0735-1631</idno><imprint><date when="1993" type="published">1993</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Fatal Outcome</term><term>Female</term><term>Fetal Diseases</term><term>Humans</term><term>Hydrops Fetalis (etiology)</term><term>Infant, Newborn</term><term>Male</term><term>Noonan Syndrome (complications)</term><term>Polyhydramnios</term><term>Pregnancy</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Noonan Syndrome</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Hydrops Fetalis</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Fatal Outcome</term><term>Female</term><term>Fetal Diseases</term><term>Humans</term><term>Infant, Newborn</term><term>Male</term><term>Polyhydramnios</term><term>Pregnancy</term></keywords></textClass></profileDesc></teiHeader><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></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">8267802</PMID><DateCreated><Year>1994</Year><Month>01</Month><Day>31</Day></DateCreated><DateCompleted><Year>1994</Year><Month>01</Month><Day>31</Day></DateCompleted><DateRevised><Year>2004</Year><Month>11</Month><Day>17</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0735-1631</ISSN><JournalIssue CitedMedium="Print"><Volume>10</Volume><Issue>6</Issue><PubDate><Year>1993</Year><Month>Nov</Month></PubDate></JournalIssue><Title>American journal of perinatology</Title><ISOAbbreviation>Am J Perinatol</ISOAbbreviation></Journal><ArticleTitle>Progression of nonimmune hydrops in a fetus with Noonan syndrome.</ArticleTitle><Pagination><MedlinePgn>417-8</MedlinePgn></Pagination><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></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Katz</LastName><ForeName>V L</ForeName><Initials>VL</Initials><AffiliationInfo><Affiliation>Department of OB/GYN, UNC School of Medicine, Chapel Hill 27599-7570.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Kort</LastName><ForeName>B</ForeName><Initials>B</Initials></Author><Author ValidYN="Y"><LastName>Watson</LastName><ForeName>W J</ForeName><Initials>WJ</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Am J Perinatol</MedlineTA><NlmUniqueID>8405212</NlmUniqueID><ISSNLinking>0735-1631</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D017809" MajorTopicYN="N">Fatal Outcome</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005315" MajorTopicYN="Y">Fetal Diseases</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D015160" MajorTopicYN="N">Hydrops Fetalis</DescriptorName><QualifierName UI="Q000209" MajorTopicYN="Y">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007231" MajorTopicYN="N">Infant, Newborn</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D009634" MajorTopicYN="N">Noonan Syndrome</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006831" MajorTopicYN="N">Polyhydramnios</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011247" MajorTopicYN="N">Pregnancy</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1993</Year><Month>11</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1993</Year><Month>11</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1993</Year><Month>11</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">8267802</ArticleId><ArticleId IdType="doi">10.1055/s-2007-994620</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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