title: Successful Glycemic Control Decreases the Elevated Serum FGF21 Level without Affecting Normal Serum GDF15 Levels in a Patient with Mitochondrial Diabetes.
abstract: Mitochondrial diabetes mellitus is a subtype of diabetes linked to mutations in mitochondrial DNA. In patients with mitochondrial diabetes mellitus, the effect of glycemic control on the serum concentrations of fibroblast growth factor 21 (FGF21) and growth differentiation factor 15 (GDF15) has not been evaluated. FGF21 and GDF15 have been reported to be useful biomarkers for the diagnosis and severity assessment of mitochondrial diseases like mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Recent studies have shown FGF21 acts in an endocrine fashion to regulate glucose and lipid metabolism in type 2 diabetes mellitus, while the exact biological functions of GDF15 remain unknown. Although mitochondrial diabetes mellitus is commonly found in cases with mitochondrial diseases, the comparison of FGF21 and GDF15 levels between those with and without diabetes has not been performed. Here, we report a 24-year-old woman with mitochondrial diabetes mellitus, who showed a high level of serum FGF21, but not serum GDF15, at diagnosis. In our case, liraglutide, a glucagon-like peptide-1 receptor agonist, added to insulin glargine was effective for her glycemic control and showed no adverse effects, including gastrointestinal symptoms and hypoglycemia, during a 14-week observation. The successful glycemic control caused a decrease in the FGF21 level, without affecting the GDF15 level. Thus, we should consider patients' glycemic control levels in using FGF21 values for the diagnosis of mitochondrial diseases. In addition, sustained GDF15 levels during glycemic treatment in our case suggest the usefulness of GDF15 as a marker for clinical severity of muscle-manifested mitochondrial diseases.
authors.affiliation: Department of Diabetes and Endocrinology, Osaka Red Cross Hospital.
authors.forename: Takaaki
authors.initials: T
authors.lastname: Murakami
authors.forename: Yoko
authors.initials: Y
authors.lastname: Ueba
authors.forename: Yuya
authors.initials: Y
authors.lastname: Shinoto
authors.forename: Yasutoshi
authors.initials: Y
authors.lastname: Koga
authors.forename: Daita
authors.initials: D
authors.lastname: Kaneda
authors.forename: Tomonobu
authors.initials: T
authors.lastname: Hatoko
authors.forename: Tomoko
authors.initials: T
authors.lastname: Kato
authors.forename: Shin
authors.initials: S
authors.lastname: Yonemitsu
authors.forename: Seiji
authors.initials: S
authors.lastname: Muro
authors.forename: Shogo
authors.initials: S
authors.lastname: Oki
mesh_terms: D003924:Diabetes Mellitus, Type 2 / Q000097:blood* / Q000150:complications; D005260:Female; D005346:Fibroblast Growth Factors / Q000097:blood*; D055436:Growth Differentiation Factor 15 / Q000097:blood*; D006801:Humans; D006943:Hyperglycemia / Q000097:blood* / Q000150:complications; D028361:Mitochondrial Diseases / Q000097:blood* / Q000150:complications; D010343:Patient Admission; D010351:Patient Discharge; D055815:Young Adult
chemical_list: C526140:GDF15 protein, human; D055436:Growth Differentiation Factor 15; C414620:fibroblast growth factor 21; D005346:Fibroblast Growth Factors