Síndrome de Russel-Silver
Autor(a) principal: | |
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Data de Publicação: | 2001 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | spa |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10400.23/378 |
Resumo: | Russel-Silver syndrome is a pattern of malformations whose most characteristic features are intrauterine and postnatal growth retardation, a characteristic facial appearance and limb asymmetry. We report a 9-month-old male born at 37 weeks' gestation. Family history, pregnancy and delivery revealed no significant anomalies. The infant had no intrauterine growth retardation (birth weight and height ranging between the 25 th and 50 th percentile). The infant's head circumference was disproportionately large for the small facial mass. Clinical findings included a small triangular face with frontal bossing, blue sclerae, thin lips with down-turned corners, retromicrognathia, clinodactyly of the fifth finger in both hands and limb asymmetry with undergrowth of the right leg and the left arm. The patient presented postnatal growth deficiency with weight and height 5th percentile; his head circumference grew normally along the 90 th percentile. Psychomotor development was appropriate for his age (9 months) according to the Sheridan Scale. On the basis of this clinical evidence we diagnosed Russel-Silver syndrome, attributing particular relevance to the normal intrauterine growth and the crossed asymmetry of the limbs. |
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Síndrome de Russel-SilverRussel-Silver syndromeMalformações MúltiplasMalformações Craniofaciais.traso no Crescimento FetalRussel-Silver syndrome is a pattern of malformations whose most characteristic features are intrauterine and postnatal growth retardation, a characteristic facial appearance and limb asymmetry. We report a 9-month-old male born at 37 weeks' gestation. Family history, pregnancy and delivery revealed no significant anomalies. The infant had no intrauterine growth retardation (birth weight and height ranging between the 25 th and 50 th percentile). The infant's head circumference was disproportionately large for the small facial mass. Clinical findings included a small triangular face with frontal bossing, blue sclerae, thin lips with down-turned corners, retromicrognathia, clinodactyly of the fifth finger in both hands and limb asymmetry with undergrowth of the right leg and the left arm. The patient presented postnatal growth deficiency with weight and height 5th percentile; his head circumference grew normally along the 90 th percentile. Psychomotor development was appropriate for his age (9 months) according to the Sheridan Scale. On the basis of this clinical evidence we diagnosed Russel-Silver syndrome, attributing particular relevance to the normal intrauterine growth and the crossed asymmetry of the limbs.Repositório Científico do Hospital de BragaNogueiras, AMCosteiras, MTMoreira, HSAntunes, AH2013-01-10T12:43:48Z2001-01-01T00:00:00Z2001-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.23/378spaAn Esp Pediatr. 2001;54(6):591-4.info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2022-09-21T09:01:59Zoai:repositorio.hospitaldebraga.pt:10400.23/378Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T15:54:48.609739Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Síndrome de Russel-Silver Russel-Silver syndrome |
title |
Síndrome de Russel-Silver |
spellingShingle |
Síndrome de Russel-Silver Nogueiras, AM Malformações Múltiplas Malformações Craniofaciais. traso no Crescimento Fetal |
title_short |
Síndrome de Russel-Silver |
title_full |
Síndrome de Russel-Silver |
title_fullStr |
Síndrome de Russel-Silver |
title_full_unstemmed |
Síndrome de Russel-Silver |
title_sort |
Síndrome de Russel-Silver |
author |
Nogueiras, AM |
author_facet |
Nogueiras, AM Costeiras, MT Moreira, HS Antunes, AH |
author_role |
author |
author2 |
Costeiras, MT Moreira, HS Antunes, AH |
author2_role |
author author author |
dc.contributor.none.fl_str_mv |
Repositório Científico do Hospital de Braga |
dc.contributor.author.fl_str_mv |
Nogueiras, AM Costeiras, MT Moreira, HS Antunes, AH |
dc.subject.por.fl_str_mv |
Malformações Múltiplas Malformações Craniofaciais. traso no Crescimento Fetal |
topic |
Malformações Múltiplas Malformações Craniofaciais. traso no Crescimento Fetal |
description |
Russel-Silver syndrome is a pattern of malformations whose most characteristic features are intrauterine and postnatal growth retardation, a characteristic facial appearance and limb asymmetry. We report a 9-month-old male born at 37 weeks' gestation. Family history, pregnancy and delivery revealed no significant anomalies. The infant had no intrauterine growth retardation (birth weight and height ranging between the 25 th and 50 th percentile). The infant's head circumference was disproportionately large for the small facial mass. Clinical findings included a small triangular face with frontal bossing, blue sclerae, thin lips with down-turned corners, retromicrognathia, clinodactyly of the fifth finger in both hands and limb asymmetry with undergrowth of the right leg and the left arm. The patient presented postnatal growth deficiency with weight and height 5th percentile; his head circumference grew normally along the 90 th percentile. Psychomotor development was appropriate for his age (9 months) according to the Sheridan Scale. On the basis of this clinical evidence we diagnosed Russel-Silver syndrome, attributing particular relevance to the normal intrauterine growth and the crossed asymmetry of the limbs. |
publishDate |
2001 |
dc.date.none.fl_str_mv |
2001-01-01T00:00:00Z 2001-01-01T00:00:00Z 2013-01-10T12:43:48Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10400.23/378 |
url |
http://hdl.handle.net/10400.23/378 |
dc.language.iso.fl_str_mv |
spa |
language |
spa |
dc.relation.none.fl_str_mv |
An Esp Pediatr. 2001;54(6):591-4. |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
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reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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