title: Pyruvate Improved Insulin Secretion Status in a Mitochondrial Diabetes Mellitus Patient.
abstract: Mitochondrial diabetes is a rare form of diabetes mellitus accounting for up to 1% of all diabetes. Pyruvate therapy has been reported to be a potential therapeutic choice for patients with mitochondrial diseases.
  
  
  
  Water-based sodium pyruvate solutions (0.5 g/kg, thrice daily) were administrated orally to a 32-year-old Japanese male with mitochondrial diabetes and myopathy caused by m.14709T>C mutation. At the age of 20 years, he was diagnosed with diabetes mellitus and started insulin therapy. He tested negative for islet cell and glutamic decarboxylase antibodies. To evaluate favorable therapeutic improvements, we measured the lactate and pyruvate levels in plasma and cerebrospinal fluid; urinary C-peptide, glycated hemoglobin, and glycoalbumin levels; and total daily insulin dose (TDD). The patient experienced no side effects such as diarrhea because of pyruvate therapy. His urinary C-peptide level improved from 4.3 to 17.2 μg/d after 1 day and to 30.2 μg/d after 6 months of pyruvate therapy. TDD decreased from 33 to 20 U/d after 6 months of pyruvate therapy, but the lactate levels of plasma and cerebrospinal fluid and the lactate/pyruvate ratio did not change.
  
  
  
  Sodium pyruvate improved insulin secretion and resulted in decreased TDD in a patient with mitochondrial diabetes. Pyruvate therapy may be a potential therapeutic choice for patients with mitochondrial diabetes. Clinical trials involving a larger number of patients and long-term evaluation of the therapy are necessary to clarify the efficacy of pyruvate therapy.
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Inoue
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Murakami
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Tadayuki
authors.initials: T
authors.lastname: Ayabe
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Oto
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Ichizo
authors.initials: I
authors.lastname: Nishino
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Yu-Ichi
authors.initials: Y
authors.lastname: Goto
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Yasutoshi
authors.initials: Y
authors.lastname: Koga
authors.affiliation: Department of Pediatrics (T.I., N.M., T.A., Y.O.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan; Department of Neuromuscular Research (I.N.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Mental Retardation and Birth Defect Research (Y.G.), National Center of Neurology and Psychiatry, 4-1-1 Ogawa-Higashi, Kodaira, Tokyo, Japan; Department of Pediatrics and Child Health (Y.K.), Kurume University Hospital, 67 Asahi-machi, Kurume, Fukuoka, Japan; and Center for Child Development and Psychosomatic Medicine (R.S.), Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minamikoshigaya, Koshigaya, Saitama, Japan.
authors.forename: Ryoichi
authors.initials: R
authors.lastname: Sakuta
mesh_terms: D000328:Adult; D002096:C-Peptide / Q000652:urine; D003920:Diabetes Mellitus / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D007328:Insulin / Q000627:therapeutic use*; D008297:Male; D028361:Mitochondrial Diseases / Q000188:drug therapy* / Q000235:genetics; D009154:Mutation; D019289:Pyruvic Acid / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D002096:C-Peptide; D007328:Insulin; D019289:Pyruvic Acid