Oshi Amira, Alfaifi Abdullah, Seidahmed Mohammed Z, Al Hussein Khalid, Miqdad Abeer, Samadi Abdelmohsin, Abdelbasit Omar
Department of Pediatrics Security Forces Hospital Riyadh Saudi Arabia.
Clin Case Rep. 2021 Jan 9;9(3):1295-1298. doi: 10.1002/ccr3.3753. eCollection 2021 Mar.
GABA transaminase deficiency should be considered in the differential diagnosis of early onset epileptic encephalopathies. This case was diagnosed post-mortem, but increased vigilance to this will allow for earlier diagnoses in other infants and families. This is a case study which involved diagnosis of a rare neurometabolic disorder in one of the babies in the family and eventual genetic counselling of the family. The family has been offered pre-implantation genetic diagnosis for future pregnancies. This case reporting has been approved by the hospital research and ethical committee.
γ-氨基丁酸转氨酶缺乏症应在早发性癫痫性脑病的鉴别诊断中予以考虑。该病例是在尸检后确诊的,但提高对此病的警惕性将有助于其他婴儿及其家庭更早得到诊断。这是一项病例研究,涉及对该家庭中一名婴儿的罕见神经代谢紊乱进行诊断,并最终为该家庭提供遗传咨询。已为该家庭提供了针对未来妊娠的植入前基因诊断。本病例报告已获医院研究与伦理委员会批准。