YIN-HSIU CHIENHsu C.-C.Huang A.Chou S.-P.FRANK LEIGH LUWANG-TSO LEEWUH-LIANG HWU2020-12-082020-12-0820040883-0738https://www.scopus.com/inward/record.uri?eid=2-s2.0-1542603300&doi=10.1177%2f08830738040190010702&partnerID=40&md5=38c3dc11d91336fdf4809f33aff0bd8ehttps://scholars.lib.ntu.edu.tw/handle/123456789/524958Neonatal-type nonketotic hyperglycinemia treatment remains unsatisfactory, even if started early. A review of six patients who underwent treatment for neonatal-type nonketotic hyperglycinemia in our hospital is presented. All patients were treated with a standardized protocol. Medical histories were retrieved from case notes. All six patients had elevated cerebrospinal fluid plasma glycine levels initially. All but one had received sodium benzoate and dextromethorphan from 1 month of age. All suffered from intractable seizures and severe mental retardation, and only two patients remain alive. One patient died at 5 days of age. No resuscitation was attempted in accordance with the family's wish after genetic counseling. The prognosis of neonatal nonketotic hyperglycinemia remains poor with current treatment. Genetic counseling helps parents cope with this devastating genetic disease.[SDGs]SDG3benzoic acid; dextromethorphan; glycine; ketamine; amino acid blood level; anamnesis; article; cerebrospinal fluid analysis; clinical article; clinical protocol; decision making; disease classification; disease severity; dose response; drug megadose; family counseling; gastrointestinal symptom; genetic counseling; human; hyperglycinemia; infant; intractable epilepsy; mental deficiency; mortality; priority journal; prognosis; resuscitation; sedation; side effect; standardization; treatment outcomejournal article10.1177/08830738040190010702150323822-s2.0-1542603300