SSIEM 2023

Acute renal failure due to severe rhabdomyolysis provoked by a mild covid-19 infection in a patient with LCHAD deficiency

Dunja Leskovar Drazen Perica Nediljko Sucur
Department of Internal Medicine, Division for Metabolic Diseases, University Hospital Centre Zagreb, Croatia

BACKGROUND: LCHAD (long-chain 3-hydroxy-acyl-CoA dehydrogenase) deficiency is an inherited fatty acid oxidation disorder in which the body is unable to break down certain fats resulting in hypoketotic hypoglycemia, myopathy, episodic rhabdomyolysis, and neuropathy. Metabolic decompensation is often precipitated by infection or fasting.

CASE REPORT: A 26-year-old patient was admitted to the emergency department because of generalized myalgias. This is a patient with a congenital deficiency of long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD), who was diagnosed at the age of 3 years and regularly undergoes check-ups in a specialized metabolic department. 10 days prior to the symptoms he was diagnosed with a milder form of covid-19 infection with a persistent dry cough. Previously, he was vaccinated with two doses of the mRNA SARS-COV 2 vaccine. Laboratory findings showed elevated creatine kinase levels (46000 U/L) initially with normal renal function (egfr: 105 ml/min/1.73m2). Chest X-ray excluded pneumonia. Abundant hydration with intravenous infusions (0.9% NaCl, 5% glucose) was started, but during the observation, the patient developed oliguria with urine output <10 ml/hour. Further laboratory findings showed acute kidney injury with worsening rhabdomyolysis (CK>80,000 U/L, egfr: 19 ml/min/1.73m2, creatinine: 369 umol/L). Due to the need for hemodialysis, he was hospitalized in the intensive care unit where dialysis procedures (CVVHD, CVVHDF) was continuously performed for 7 days until a gradual decrease in creatinine and CK levels. In continuation, he was carefully hydrated with infusions of 10% glucose and received a specially adapted diet to ensure sufficient caloric intake and to prevent catabolism. In total, he was 12 days on continuous hemodialysis and the renal function completely recovered after 3 weeks with the normalization of creatinine and CK values. Beside SARS-CoV-2 infection, we haven’t found any other cause of patient’s metabolic decompensation.

CONCLUSION: Patients with LCHAD should be educated and controlled more often during the covid-19 pandemic, as even the mild form of SARS-CoV-2 infection can lead to a rapid metabolic decompensation and a possible fatal outcome