Maximilian C, Ioan D M, Fryns J P
Department of Clinical Genetics, Institute of Endocrinology, C.I. Parhon, Bucarest, Roumania.
Genet Couns. 1992;3(2):115-8.
We present a family with four children in which three, a girl and two boys, present a similar MR/MCA syndrome with slight to moderate mental retardation, short stature, peculiar facies with palpebral ptosis, pectus excavatum and pulmonary stenosis. As both parents are mentally and physically normal, autosomal recessive inheritance of this Noonan-like phenotype is most likely. The findings in the present family confirm that the Noonan phenotype may be caused by different etiologies with different types of genetic transmission.
我们报告一个有四个孩子的家庭,其中三个孩子,一个女孩和两个男孩,患有类似的MR/MCA综合征,伴有轻度至中度智力发育迟缓、身材矮小、特殊面容(伴有眼睑下垂、漏斗胸和肺动脉狭窄)。由于父母双方智力和身体均正常,这种类似努南综合征的表型很可能是常染色体隐性遗传。本家庭的研究结果证实,努南综合征的表型可能由不同病因和不同类型的基因传递引起。