ABSTRACT
A 4-year-old girl with pyruvate dehydrogenase deficiency, static encephalopathy, and seizure disorder treated with the ketogenic diet presented in severe diabetic ketoacidosis. Pyruvate dehydrogenase deficiency is a rare genetic defect of mitochondrial energy metabolism that leads to inefficient glucose use and lactic acidosis. The ketogenic diet provides the brain with an alternate fuel source, but its implementation opposes traditional diabetes management. Faced with this therapeutic dilemma, we aimed to maintain ketosis without compromising safety to optimize neurologic function and quality of life. This is the first report, to our knowledge, of a child simultaneously treated with the ketogenic diet and exogenous insulin. A 28-month follow-up revealed excellent glycemic control, improved activity level, significant developmental achievements, and, perhaps most striking, catch-up linear growth from < 5th percentile to the 50th percentile. Her progress to date indicates that diabetes does not preclude use of the ketogenic diet.
Subject(s)
Diabetes Mellitus, Type 1/drug therapy , Diabetic Ketoacidosis/prevention & control , Hypoglycemic Agents/administration & dosage , Insulin/administration & dosage , Pyruvate Dehydrogenase Complex Deficiency Disease/diet therapy , Child, Preschool , Diabetes Mellitus, Type 1/complications , Diabetic Ketoacidosis/etiology , Female , Humans , Pyruvate Dehydrogenase Complex Deficiency Disease/complicationsABSTRACT
The purpose of the study was to compare three nursing interventions and their impact on glycemic control among children with type I diabetes. The 75 subjects' mean +/- SD age was 12.5 +/- 3.4 years, 55% were boys, and 55% were White. Subjects were randomly assigned to a standard care (SC), an education (ED), or an education and telephone case management (ED + TCM) group. The primary outcome measure was glycemic control (hemoglobin A1c, or HbA1c). Secondary outcome measures were diabetes knowledge (KNOW), parent-child teamwork (TEAM), and adherence (ADH). After 6 months of follow-up, results demonstrated no significant differences among groups in HbA1c. KNOW and TEAM scores improved slightly in the ED and ED + TCM groups, but no statistically significant differences were found among the three groups. Significant improvement in ADH scores among ED + TCM groups was reported when compared with the ED and SC groups. This change may represent a move toward improved adherence to diabetes care and subsequent improvement in diabetes control. The challenges of recruitment and retention of subjects in this study will also be discussed.
