A 14-year-old male patient was diagnosed with cooccurring type 1 diabetes mellitus (DM1) and very long chain acyl-CoA dehydrogenase deficiency (VLCADD), a type of long chain fatty acid oxidation disorder (LCFAOD), as reported in a case study published in Clinical Biochemistry.

The co-occurrence of both disorders requires careful balancing to avoid hyperglycemia and hypoglycemia. Hyperglycemia due to too little insulin could cause intracellular glucose depletion and lead to major metabolic decompensation due to the ineffective reliance on fatty acid oxidation. Too much insulin, on the other hand, could lead to hypoglycemia due to the already higher risk of hypoglycemia due to VLCADD.

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The patient had been diagnosed with VLCADD since the neonatal period after 2 previous siblings had apparently died from the disease. His disease was managed through prevention of fasting, restricting long chain fatty acid intake, and supplementing with medium chain triglycerides (MCTs) and walnut oil. He presented to the emergency room at age 14 after 2 days of vomiting and epigastric pain.

Rounds of testing revealed hyperglycemia and high anion gap metabolic acidosis. Further testing showed he had elevated HbA1c of 12.8%, and a strongly positive anti-glutamic acid decarboxylase antibody test confirmed a diagnosis of DM1.

Following the DM1 diagnosis, the patient was treated for diabetic ketoacidosis with weight-based IV fluid replacement and insulin infusions. After 24 hours, the diabetic ketoacidosis resolved, and the patient was switched to subcutaneous insulin for management.

The patient was advised to continue to limit their nutritional intake of long chain fatty acids (10% of calories) and MCTs (20% of calories) while maintaining their intake of carbohydrates (55%-60% of calorie requirements). Avoidance of simple sugars for complex carbohydrates was advised. The patient was also educated on how to maintain blood sugar levels through correct insulin dosing.

“We reported a case of a 14-year-old male with VLCADD who was recently diagnosed with DM1,” the authors wrote.

“The patient-centered approach supported by a multidisciplinary team has led to a favorable outcome and prevented complications related to hypoglycemia or hyperglycemia,” the authors continued.


Al-Busaidi SA, Al Nou’mani JA, Al-Falahi Z, et al. Very long-chain acyl-CoA dehydrogenase deficiency and type I diabetes mellitus: case report and management challenges. Clin Biochem. Published online March 12, 2023. doi:10.1016/j.clinbiochem.2023.03.005