EBR 2023

Question 1

primary adrenal insufficiency in a young man should always raise the suspicion of —–, particularly when concurrent neurologic symptoms are present.

Answer 1

adrenoleukodystrophy (ALD)

Question 2

—- is an X-linked inherited metabolic storage disease associated with a defect in an ABC transporter that results in defective peroxisomal β-oxidation and accumulation of very long-chain fatty acids (C24, C26) in tissues, especially in the brain and the adrenal glands

Answer 2

adrenoleukodystrophy (ALD)

Question 3

adrenoleukodystrophy (ALD) is an X-linked inherited metabolic storage disease associated with a defect in an ABC transporter that results in defective peroxisomal β-oxidation and accumulation of very long-chain fatty acids (C24, C26) in tissues, especially in the brain and the adrenal glands

Question 4

adrenoleukodystrophy (ALD) is an X-linked inherited metabolic storage disease associated with a defect in an —- that results in defective peroxisomal β-oxidation and accumulation of very long-chain fatty acids (C24, C26) in tissues, especially in the brain and the adrenal glands

Answer 4

ABC transporter

Question 5

adrenoleukodystrophy (ALD) is an X-linked inherited metabolic storage disease associated with a defect in an ABC transporter that results in defective peroxisomal β-oxidation and accumulation of —– in tissues, especially in the brain and the adrenal glands

Answer 5

very long-chain fatty acids (C24, C26)

Question 6

adrenoleukodystrophy (ALD) is an X-linked inherited metabolic storage disease associated with a defect in an ABC transporter that results in defective peroxisomal β-oxidation and accumulation of very long-chain fatty acids (C24, C26) in tissues, especially in the —- and —-

Answer 6

brain and the adrenal glands