Liver pathology (hepatitis, cirrhosis) — Flashcards

Flashcard 1: Wilson disease is also known as _____

Answer: hepatolenticular degeneration

Flashcard 2: What pathologic RBC is seen in liver disease and abetalipoproteinemia (states of cholesterol dysregulation)?_____

Answer: Acanthocytes

Flashcard 3: Patients with sarcoidosis may have scattered granulomas in their liver; most commonly affecting the _____

Answer: portal triads

Flashcard 4: Poliovirus causes a(n) _____ paralysis (symmetric/asymmetric)

Answer: asymmetric

Flashcard 5: Herpes encephalitis is associated with _____ protein in the CSF

Answer: increased

Flashcard 6: What pathology is characterized by a defective type IV collagen? _____

Answer: Alport syndrome

Flashcard 7: What is the enzyme deficiency and accumulated substrate in Niemann-Pick disease?

Answer: Lysosomal storage disease caused by a deficiency in sphingomyelinase, leading to accumulation of sphingomyelin.

Extra: • Clinical Features: Progressive neurodegeneration, hepatosplenomegaly, and cherry-red spot on macula. • Pathology: Foam cells (lipid-laden macrophages) are characteristic. • Genetics: Autosomal recessive (AR). • Population: Increased frequency in Ashkenazi Jews. *Distinction: Unlike Tay-Sachs, Niemann-Pick presents with hepatosplenomegaly.*

Flashcard 8: Gaucher's disease

Answer:

Extra: accumulation of glucocerebrosidehepatosplenomegaly, aseptic necrosis of femur, bone crisescrumpled-paper cells Ashkenazi JewARglucocerebrosidase

Flashcard 9: Patau's syndrome

Answer: Trisomy 13 Key features: Microphthalmia, Microcephaly, Cleft lip/palate, Polydactyly, Holoprosencephaly, and Rocker-bottom feet.

Extra: First trimester pregnancy screen: - Decreased β-hCG - Decreased PAPP-A - Ultrasound: Increased nuchal translucency Note: Most cases result in death within the first year of life.

Flashcard 10: In metachromatic leukodystrophy, sulfatides accumulate in the lysosomes of _____

Answer: oligodendrocytes

Extra:   https://onlinemeded.org/spa/metabolism?ref=anki Atlas: