Match each clinical presentation to the most likely hepatobiliary or metabolic liver diagnosis.
- A middle-aged female with insidious onset of pruritus, fatigue, xanthelasma, and elevated alkaline phosphatase. Serology shows anti-mitochondrial antibodies.Primary biliary cholangitis
- A young adult male with a history of bloody diarrhea presents with fatigue and elevated alkaline phosphatase. MRCP reveals alternating strictures and dilations of the bile ducts.Primary sclerosing cholangitis
- A young female with amenorrhea, fatigue, and jaundice. Labs reveal AST 850 U/L, ALT 920 U/L, elevated IgG levels, and positive anti-smooth muscle antibodies.Autoimmune hepatitis
- A middle-aged man with bronze skin pigmentation, recent-onset diabetes mellitus, hepatomegaly, and arthropathy involving the second and third metacarpophalangeal joints.Hereditary hemochromatosis
- A young adult male with new-onset resting tremor, dysarthria, mildly elevated liver enzymes, and decreased serum ceruloplasmin levels.Wilson disease
Cevap
The correct pairings are: the middle-aged female with pruritus and AMA matches with Primary biliary cholangitis; the young male with bloody diarrhea and beaded bile ducts matches with Primary sclerosing cholangitis; the young female with transaminitis and anti-smooth muscle antibodies matches with Autoimmune hepatitis; the middle-aged man with bronze diabetes matches with Hereditary hemochromatosis; and the young male with tremor and low ceruloplasmin matches with Wilson disease.
Each specific clinical vignette provides pathognomonic features for a distinct liver pathology. Recognizing demographic risk factors (e.g., female gender in PBC, young males in PSC), characteristic serologies (AMA, ASMA), and unique systemic findings (neurologic deficits in Wilson disease, bronze diabetes in hemochromatosis) allows for accurate pairing with the underlying hepatobiliary or metabolic disease.
Adım Adım Çözüm
Anahtar Kavram
Clinical and serological diagnosis of autoimmune and metabolic liver diseases