Germain Dominique P, Bruneval Patrick, Tran Thi-Chien, Balouet Pierre, Richalet Bernard, Benistan Karelle
Division of Medical Genetics, Hôpital Raymond Poincaré (AP-HP), 92380 Garches, France.
Eur J Med Genet. 2010 Mar-Apr;53(2):111-2. doi: 10.1016/j.ejmg.2009.12.004. Epub 2010 Jan 1.
No reproductive studies have been performed with enzyme replacement therapy (ERT) for Fabry disease (FD, OMIM 301500), a lysosomal storage disorder. Therefore, use during pregnancy is theoretically contraindicated. We report the first case of agalsidase beta treatment throughout pregnancy. High-range proteinuria remained stable and the patient gave birth to a healthy boy after an uncomplicated pregnancy. The decision to administer ERT during pregnancy should be made on an individual basis, considering the FD status and possible risks.
对于法布里病(FD,OMIM 301500)这种溶酶体贮积症,尚未进行过酶替代疗法(ERT)的生殖研究。因此,理论上孕期禁用。我们报告了首例在整个孕期使用阿加糖酶β治疗的病例。高范围蛋白尿保持稳定,患者在孕期未出现并发症,之后生下一名健康男婴。孕期给予ERT的决定应根据个体情况,考虑法布里病病情及可能的风险来做出。