Which of the following syndromes is commonly characterised by acute encephalopathy and liver failure?

DSSSB 13 August 2024

Medical Surgical Nursing

Easy

Appeared in: DSSSB 13 August 2024

Explanation

Reye's syndrome is a rapidly progressive condition characterized by two primary features: acute non-inflammatory encephalopathy and fatty degenerative liver failure.
It typically develops in children and teenagers following a viral illness, such as influenza or chickenpox.
A significant risk factor is the use of salicylates, like aspirin, to manage fever or pain during the viral infection.
The syndrome involves mitochondrial dysfunction, leading to the characteristic brain and liver damage.

Option A: Angelman syndrome is a genetic neurodevelopmental disorder. Its main features are severe intellectual disability, ataxia, and a characteristic happy demeanor with frequent laughter. It does not involve acute encephalopathy or liver failure.
Option B: Edward syndrome, or Trisomy 18, is a severe chromosomal disorder. It is characterized by major physical abnormalities, such as heart defects, clenched hands with overlapping fingers, and severe developmental delays. It does not cause acute encephalopathy and liver failure.
Option C: Cri du chat syndrome is a genetic condition caused by a chromosomal deletion. Its most distinctive feature is a high-pitched, cat-like cry in infants. It also involves intellectual disability and developmental delays, but not acute liver and brain failure.

Clinical Relevance

Nursing practice connection: This is primarily an exam-oriented knowledge point with limited direct bedside application, so retain Clinical presentation and etiology of Reye's syndrome versus other genetic syndromes as background academic context rather than a clinical decision trigger.
The primary clinical relevance is the prevention of Reye's syndrome. Nurses play a crucial role in educating parents and caregivers to avoid giving aspirin or salicylate-containing products to children and teenagers, especially during viral illnesses.
Nurses must be able to recognize the early signs of Reye's syndrome, such as persistent vomiting, lethargy, and confusion following a viral illness, to ensure prompt medical intervention, which is critical for survival and reducing long-term neurological damage.
What if? If the patient was an adult with a history of chronic alcohol use presenting with encephalopathy and liver failure, the primary diagnosis would shift from Reye's syndrome to hepatic encephalopathy secondary to alcoholic cirrhosis.

How to Approach the Question

First, analyze the question to identify the core clinical signs: 'acute encephalopathy' and 'liver failure'. These are severe, acute symptoms.
Next, review the options. Notice that three of the options (Angelman, Edward, Cri du chat) are well-known genetic or chromosomal syndromes typically diagnosed in infancy or early childhood and are associated with chronic developmental issues.
Contrast these with the acute presentation described in the question. Recall or deduce that Reye's syndrome is an acquired condition, not a congenital one, that specifically targets the brain and liver.
The key differentiator is the acute onset of brain and liver dysfunction, which points directly to Reye's syndrome and away from the chronic, developmental genetic disorders.

Concept Tested & Keywords

Concept Tested: Clinical presentation and etiology of Reye's syndrome versus other genetic syndromes.
Stem keywords: syndromes, acute encephalopathy, liver failure
Lead-in keywords: characterised by

QBSKBpTJ5Xo0sLEtS4asRR

E6 Kaplan Sadock's Synopsis of Psychiatry-2022 (pp 1-3768 of 3768) p. 277-279