3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency is an autosomal recessive disorder, caused by mutations in the HMGCL gene. This enzyme is important in the ketogenic pathway and in the last step of leucine catabolism, leading to inadequate ketone body synthesis and accumulation of toxic metabolites of leucine catabolism.1 It occurs in 1 in 100,000 live neonates and is more common in Saudi Arabia, Portugal, Spain and Pakistan, with a high incidence of parental consanguineous marriage.2 Although there is a considerable heterogeneity, it usually presents with vomiting, hypotonia and lethargy in the first year of life. Hypoketotic hypoglycemia, metabolic acidosis and hepatomegaly are common.3 The primary aim of management is rigorous emergency management with a high carbohydrate intake during infections, avoiding extended fasting, with dietary protein, and possibly, fat restriction. Carnitine supplementation is usually prescribed.4

Type 1 diabetes (T1D) is a chronic disease wherein there is an absence of insulin production, resulting in hyperglycemia and, in severe cases, ketoacidosis. T1D requires painstaking efforts in order to reduce hyperglycemia while minimizing the risk of hypoglycemia. Most patients with T1D are treated with intensive insulin regimens, either via multiple day injections or continuous subcutaneous insulin infusion.5, 6 Good dietary adherence is necessary and is associated with better glycemic control in children with T1D, resulting in a reduction of the micro and macrovascular complications and the premature mortality associated with T1D.7, 8