Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.23/378
Título: Síndrome de Russel-Silver
Outros títulos: Russel-Silver syndrome
Autor: Nogueiras, AM
Costeiras, MT
Moreira, HS
Antunes, AH
Palavras-chave: Malformações Múltiplas
Malformações Craniofaciais.
traso no Crescimento Fetal
Data: 2001
Citação: An Esp Pediatr. 2001;54(6):591-4.
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.
Peer review: yes
URI: http://comum.rcaap.pt/handle/123456789/3583
http://hdl.handle.net/10400.23/378
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