Israëls T, Hoovers J, Turpijn H M, Wijburg F A, Hennekam R C
University of Amsterdam, Department of Pediatrics, Amsterdamic, The Netherlands.
Clin Genet. 1996 Dec;50(6):520-4. doi: 10.1111/j.1399-0004.1996.tb02726.x.
Here we report on a boy with both a partial deletion of chromosome 18p and a partial duplication of chromosome 18q, caused by a paternal pericentric inversion (46,XY,rec(18),dup q,inv(18)(p11.2q21.1)pat). The findings in the patient are compared to those in the literature. The symptoms in the described patient can be explained for the major part by the 18p- and 18q+ syndromes separately. A specific 18p-/18q+ syndrome cannot be clearly delineated yet. Inspiratory stridor is a symptom that has not been described before in either 18p- or 18q+, but has been found twice before in patients with the combined 18p-/18q+ syndrome.
在此,我们报告一名患有18号染色体短臂部分缺失和18号染色体长臂部分重复的男孩,其病因是父源性臂间倒位(46,XY,rec(18),dup q,inv(18)(p11.2q21.1)pat)。将该患者的检查结果与文献中的结果进行了比较。所描述患者的症状在很大程度上可分别由18p-综合征和18q+综合征来解释。目前尚不能明确界定一种特定的18p-/18q+综合征。吸气性喘鸣是一种在18p-或18q+综合征中均未被描述过的症状,但之前在18p-/18q+综合征患者中曾发现过两例。