A 4-Month-Old Boy Is Being Evaluated for a Hereditary Metabolic

question 56

Multiple Choice

A 4-month-old boy is being evaluated for a hereditary metabolic disorder after presenting with failure to thrive and developmental delay.  He was born outside the United States and had several seizures during the neonatal period.  Physical examination reveals an abnormal head shape with a large anterior fontanelle and widely splayed cranial sutures.  Profound hypotonia and hepatomegaly are also noted.  Biochemical studies show elevated levels of very long chain fatty acids (VLCFAs) and phytanic acid.  Cultured skin fibroblasts show impaired ability to oxidize VLCFAs.  This patient's condition is best explained by dysfunction of which of the following cellular structures?

• A)Golgi apparatus
• B)Lysosomes
• C)Mitochondria
• D)Peroxisomes
• E)Proteasomes
• F)Rough endoplasmic reticulum

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Definitions:

Charismatic Leadership

A leadership style characterized by an individual's ability to inspire and motivate followers through personal charm and persuasion.

Merits

The qualities of being particularly good or worthy, especially so as to deserve praise or reward.

Demerits

The negative points or marks received for poor performance or misconduct.

Leader-Member Exchange Theory

A theory that focuses on the relationship between leaders and their subordinates, proposing that the quality of these relationships influences many workplace outcomes.