Eliglustat (Genz 112638) as a glucosylceramide synthase inhibitor for use in a method of treating fabry or gaucher disease, the method comprising adjusting the individual therapeutic dose to the patient's P-450 metabolism
A compound represented by the following structural formula ** Formula ** or a pharmaceutically acceptable salt thereof, for use in a method of treating a subject with Fabry disease, the method comprising: evaluating the subject to determine if the subject is a poor, intermediate or broad / ultrafast P450 metabolizer, where the subject is assessed as a poor, intermediate or broad / ultrafast P450 metabolizer by genotyping, where a poor P450 metabolizer carries two mutant alleles of the CYP2D6 gene resulting in loss Complete with enzymatic activity, an intermediate P450 metabolizer has one allele with reduced activity and one null allele of the CYP2D6 gene, a broad P450 metabolizer has at least one and no more than two normal functional alleles of the CYP2D6 gene, and a P450 metabolizer. Ultrafast carries multiple copies of functional alleles of the CYP2D6 gene and produces excess enzyme activity； and administering to the subject an adjusted effective amount of the compound if the subject is an intermediate or broad / ultrafast P450 metabolizer and administering to the subject an effective amount of the compound if the subject is a poor P450 metabolizer, wherein said effective amount is a daily dose from 25 milligrams to 300 milligrams, and where said adjusted effective amount is greater than said effective amount.Un compuesto representado por la siguiente fórmula estructural**Fórmula** o una sal farmacéuticamente aceptable del mismo, para su uso en un método de tratamiento de un sujeto con enfermedad de Fabry, comprendiendo el método: evaluar al sujeto para determinar si el sujeto es un metabolizador de P450 pobre, intermedio o amplio/ultrarrápido, donde el sujeto se evalúa como metabolizador pobre, intermedio o amplio/ultrarrápido de P450 mediante genotipado, donde un metabolizador de P450 pobre porta dos alelos mutantes del gen CYP2D6 que dan como resultado una pérdida completa de actividad enzimática, un metabolizador de P450 intermedio posee un alelo con activida
