| Literature DB >> 26850509 |
Licia Lugli1, Emma Bertucci2, Vincenzo Mazza2, Amira Elmakky3, Fabrizio Ferrari1, Christine Neuhaus4, Antonio Percesepe5.
Abstract
3-M syndrome (OMIM #273750) is a rare autosomal recessive growth disorder characterized by severe pre- and post-natal growth restriction, associated with minor skeletal abnormalities and dysmorphisms. Although the 3-M syndrome is well known as a primordial dwarfism, descriptions of the prenatal growth are missing. We report a family with variable phenotypic features of 3-M syndrome and we describe the prenatal and postnatal growth pattern of two affected sisters with a novel homozygous CUL7 mutation (c.3173-1G>C), showing a pre- and post-natal growth deficiency and a normal cranial circumference.Entities:
Keywords: 3-M syndrome; CUL7; Dwarfism; Knee dislocations
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Year: 2016 PMID: 26850509 DOI: 10.1016/j.ejmg.2016.01.009
Source DB: PubMed Journal: Eur J Med Genet ISSN: 1769-7212 Impact factor: 2.708