This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This protocol is designed to assess the metabolism of lipids in patients with hypoketotic hypoglycemia. Levels of acetoacetate, 3-hydroxybutyric and carnitine are assessed as are the effects of treatment with carnitine.