This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Inborn Errors of Metabolism, Subject name: Pediatrics
[{'content': "Wrong about X-linked adrenoleukodystrophy: \nSelect and explain. \nA. Is a mitochondrial disorder, B. May present as Addison's disease, C. Involves accumulation of short chain fatty acids, D. Involves progressive neuronal grey matter degeneration", 'role': 'user'}, {'content': "Answer: A \nExplanation: Ans. A. Is a mitochondrial disorderX-linked adrenoleukodystrophy is a peroxisomal disorder of very long chain fatty acid (VLCFA) oxidation. It result in progressive adrenal cortex and neuronal white matter degeneration, and the phenotype is variable. It may present with Addison's disease, or it may present with neurological features (behaviour disturbance, developmental regression, ataxia, seizures, and spasticity). Diagnostic investigations include VLCFAs, adrenal cortical function tests, MRI brain scan and neuropsychiatric assessment. Management is supportive with adrenal steroid replacement, anticonvulsants, NG or gastrostomy feeding. Supplementation with monounsaturated amino acids (Lorenzo's oil) may be beneficial but is not curative.", 'role': 'assistant'}]