Dietary Management in Coexistence of Hereditary Tyrosinemia Type-1 and Type-1 Diabetes Mellitus: A Case Report

Abstract

Hereditary tyrosinemia type-1 (HT1) is a rare hepatorenal inherited metabolic disease due to fumarilacetoacetate hydrolase enzyme deficiency. In the management of hereditary tyrosinemia type-1, it is recommended to use nitisinone therapy together with a tyrosine and phenylalanine restricted diet. Type-1 Diabetes Mellitus (T1DM), also known as autoimmune diabetes, is a chronic endocrinological disease characterized by absolute insulin deficiency due to pancreatic β-cell dysfunction and leading to hyperglycemia. In order to provide glycemic control in patients, nutritional therapy with insulin and appropriate physical activity have an important place. Coexistence of HT1 and T1DM has been described in a limited number of cases in the literature. In this paper, the diet management and follow-up process of a case who developed T1DM while being followed up due to HT1, one of the tyrosine metabolism disorders, was shared. Intensive insulin therapy and phenylalanine and tyrosine-restricted diet therapy have been successfully managed in the comorbidity of the two diseases.