A 22-year-old man is evaluated for repeated episodes of pulmonary infections

A6-year-old child has a long history of a hereditary bleeding disorder characterized by spontaneous nontraumatic hemorrhages into joint spaces, skeletal muscle, and mucous membranes. Laboratory studies reveal a normal prothrombin time, elevated partial thromboplastin time, very low factor VIII, normal factor X, normal factor XI, and normal platelet aggregation studies with ristocetin. Which of the following is the most likely diagnosis?

  • (A) Christmas disease
  • (B) hemophilia A
  • © hemophilia B
  • (D) Rosenthal syndrome
  • (E) von Willebrand disease

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A 22-year-old man is evaluated for repeated episodes of pulmonary infections, including a recent one that is productive of foul, purulent sputum, accompanied by shortness of breath. Past medical history is also significant for chronic sinus infections with mucopurulent discharge. Physical examination is remarkable for scattered rhonchi and rales throughout all lung fields. Imaging studies show abnormally dilated and thickened airways extending close to the pleural surface and a cardiac silhouette in the right thorax. Bronchoscopy demonstrates numerous mucopurulent plugs. Which of the following is most likely responsible for these changes?

  • (A) abnormal neutrophilic function resulting in impaired degranulation
  • (B) alpha-1 antiprotease deficiency (PiZZ phenotype)
  • © cell membrane defect resulting in abnormally viscous glandular secretions
  • (D) defect in ciliary motility due to the absence of dynein arms
  • (E) hypersensitivity reaction associated with c-ANCAand granuloma formation

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A67-year-old retiree was employed for many years in the plastics industry where he was exposed to vinyl chloride. This industrial exposure has increased his likelihood of developing which of the following?

  • (A) focal nodular hyperplasia
  • (B) hepatic adenoma
  • © hepatic angiosarcoma
  • (D) hepatic fibroma
  • (E) hepatocellular carcinoma

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