ABSTRACT
OBJECTIVE: CD36 deficiency is characterized by limited cellular long chain fatty acid uptake in the skeletal and cardiac muscles and often causes energy crisis in these muscles. However, suitable treatment for CD36 deficiency remains to be established. The aim of this study was to evaluate the clinical and metabolic effects of medium chain triacylglycerols (MCTs) in two CD36-deficient preschool children who often developed fasting hypoglycemia and exercise-induced myalgia. METHODS: Fasting blood glucose, total ketone bodies, and free fatty acids were examined and compared for usual supper diets and for diets with replacement of one component with 2 g/kg of 9% MCT-containing milk (MCT milk). Changes in serum creatine kinase and alanine aminotransferase levels, resulting from replacement of glucose water intake with 1 g/kg of MCT milk and determined by using bicycle pedaling tasks, were examined and compared. Hypoglycemic and/or myalgia episodes in daily life were also investigated. RESULTS: Biochemically, participants' blood glucose and total ketone bodies levels after overnight fasting substantially increased after dietary suppers containing MCT milk. Increases in serum creatine kinase and alanine aminotransferase levels resulting from the bicycle pedaling task were suppressed by MCT milk. Hypoglycemia leading to unconsciousness and tachycardia before breakfast decreased after introduction of dietary suppers containing MCT milk. Occurrence of myalgia in the lower limbs also decreased after intakes of MCT milk before long and/or strenuous exercising. CONCLUSION: Our results suggest that MCTs can prevent fasting hypoglycemia and exercise-induced myalgia in CD36-deficient young children.
Subject(s)
Blood Platelet Disorders/diet therapy , Dietary Fats/administration & dosage , Food, Fortified/analysis , Genetic Diseases, Inborn/diet therapy , Milk/chemistry , Triglycerides/administration & dosage , Alanine Transaminase/blood , Animals , Blood Glucose/analysis , Blood Platelet Disorders/blood , Blood Platelet Disorders/complications , Child, Preschool , Creatine Kinase/blood , Exercise/physiology , Fasting/blood , Fatty Acids, Nonesterified/blood , Genetic Diseases, Inborn/blood , Genetic Diseases, Inborn/complications , Humans , Hypoglycemia/etiology , Hypoglycemia/prevention & control , Ketones/blood , Male , Myalgia/etiology , Myalgia/prevention & control , Treatment OutcomeABSTRACT
The vascular component of hemostatis is determined by the bleeding time as designed by Duke or by the Ivy modification, which is a combined bleeding time-tourniquet test. A prolonged bleeding time is observed: (1) in thrombopathic thrombocytopenia, which may be (a) hereditary or (b) acquired, as in the immune types or from depression of bone marrow, and (2) athrombocytopenically, as in von Willebrand's disease, Glanzmann's thrombasthenia, or from aspirin intolerance. The role of a specific bleeding time determinant in the plasma (labile bleeding time factor of Perkins) is discussed and its possible relation to platelets suggested.
