Lugli Licia, Bertucci Emma, Mazza Vincenzo, Elmakky Amira, Ferrari Fabrizio, Neuhaus Christine, Percesepe Antonio
Neonatal Intensive Care, Department of Mother and Child, University Hospital of Modena, Italy.
Prenatal Diagnosis Unit, Department of Mother and Child, University Hospital of Modena, Italy.
Eur J Med Genet. 2016 Apr;59(4):232-6. doi: 10.1016/j.ejmg.2016.01.009. Epub 2016 Feb 2.
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.
3-M综合征(OMIM #273750)是一种罕见的常染色体隐性遗传性生长障碍,其特征为严重的产前和产后生长受限,并伴有轻微的骨骼异常和畸形。尽管3-M综合征作为一种原始侏儒症广为人知,但关于其产前生长情况的描述却缺失。我们报告了一个具有3-M综合征可变表型特征的家系,并描述了两名受影响姐妹的产前和产后生长模式,她们携带一种新的纯合CUL7突变(c.3173-1G>C),表现出产前和产后生长不足以及正常的头围。