| Literature DB >> 27682613 |
Shinichiro Yoshida1,2, Jun Kido1, Shirou Matsumoto1, Ken Momosaki1, Hiroshi Mitsubuchi1, Tomoyuki Shimazu3, Keishin Sugawara2, Fumio Endo1, Kimitoshi Nakamura4.
Abstract
In the prenatal diagnosis of Gaucher disease (GD), glucocerebrosidase (GBA) activity is measured with fetal cells, and gene analysis is performed when pathogenic mutations in GBA are identified in advance. Herein is described prenatal diagnosis in a family in which two children had GD. Although prior genetic information for this GD family was not obtained, next-generation sequencing (NGS) was carried out for this family because immediate prenatal diagnosis was necessary. Three mutations were identified in this GD family. The father had one mutation in intron 3 (IVS2 + 1), the mother had two mutations in exons 3 (I[-20]V) and 5 (M85T), and child 1 had all three of these mutations; child 3 had none of these mutations. On NGS the present fetus (child 3) was not a carrier of GD-related mutations. NGS may facilitate early detection and treatment before disease onset.Entities:
Keywords: Gaucher disease; long-range polymerase chain reaction; lysosomal storage disease; next-generation sequencing; prenatal diagnosis
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Year: 2016 PMID: 27682613 DOI: 10.1111/ped.13069
Source DB: PubMed Journal: Pediatr Int ISSN: 1328-8067 Impact factor: 1.524