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Are some multiple congenital anomalies with mental retardation (MCA/MR) the clinical expression of rare autosomal fragile sites?
Stoll, C; Bolender, C; Geraudel, A; Finck, S; Alembik, Y; Dott, B.
Afiliación
  • Stoll C; Institut de Puériculture, CHU Hôpital Civil, Strasbourg, France.
Genet Couns ; 2(4): 211-5, 1991.
Article en En | MEDLINE | ID: mdl-1799418
A boy with MCA/MR and a fragile site (FS) at 8q22 opens the discussion of a possible association between a rare autosomal FS and an abnormal phenotype. The child was born after prenatal diagnosis of ureterohydronephrosis. He had facial dysmorphia and mental retardation (IQ = 40). The karyotype showed 8q22 FS in 12% of the cells obtained after addition of FUdR to the culture medium. No other etiologic factor was shown to be responsible for the MCA/MR syndrome. Several authors have reported a variety of neurodevelopmental abnormalities and mental retardation in individuals with rare FS expressed on chromosomes 2, 9, 10, 16 and 19. If rare FS predispose to phenotypic abnormalities what are the mechanisms?
Asunto(s)
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Anomalías Congénitas / Aberraciones Cromosómicas / Fragilidad Cromosómica / Enfermedades Fetales / Discapacidad Intelectual Tipo de estudio: Qualitative_research Límite: Female / Humans / Newborn / Pregnancy Idioma: En Revista: Genet Couns Asunto de la revista: ETICA / GENETICA MEDICA Año: 1991 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Suiza
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Anomalías Congénitas / Aberraciones Cromosómicas / Fragilidad Cromosómica / Enfermedades Fetales / Discapacidad Intelectual Tipo de estudio: Qualitative_research Límite: Female / Humans / Newborn / Pregnancy Idioma: En Revista: Genet Couns Asunto de la revista: ETICA / GENETICA MEDICA Año: 1991 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Suiza