Wrong about X-linked adrenoleukodystrophy:
Correct Answer: Is a mitochondrial disorder
Description: 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.
Category:
Pediatrics
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now