Standardized clinical trials evaluating anaplerotic therapy are lacking [21], but out of different treatment strategies in 48 patients with TMEM70 deficiency some general recommendations have been deduced by Magner et al. consisting basically of moderate glucose supplementation, high dose lipid infusions, and acidosis correction via bicarbonate infusions [18]. This evidence concerns the gene TMEM70 and hyperinsulinemic hypoglycemia, familial, 4.