Which hereditary disease is most closely linked to an aneurysm?
A. Cystic fibrosis
B. Lupus erythematosus
C. Marfan’s syndrome
D. Myocardial infarction
Correct Answer: C. Marfan’s syndrome
Marfan’s syndrome results in the degeneration of the elastic fibers of the aortic media. Therefore, clients with the syndrome are more likely to develop an aortic aneurysm. Marfan syndrome (MFS) is a spectrum of disorders caused by a heritable genetic defect of connective tissue that has an autosomal dominant mode of transmission. The defect itself has been isolated to the FBN1 gene on chromosome 15, which codes for the connective tissue protein fibrillin. Abnormalities in this protein cause a myriad of distinct clinical problems, of which the musculoskeletal, cardiac, and ocular system problems predominate.
Option A: Although cystic fibrosis is hereditary, it hasn’t been linked to aneurysms. Cystic fibrosis (CF) is the most common potentially lethal genetic disease in the white population. Improvements in life expectancy have led to increasing recognition of hepatobiliary complications from CF. Splenic artery aneurysms are a rare complication of portal hypertension with high mortality due to their significant potential for rupture, resulting in life-threatening i.p. hemorrhage.
Option B: Lupus erythematosus isn’t hereditary. Systemic lupus erythematosus (SLE), is the most common type of lupus. SLE is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels.
Option D: Myocardial infarction is neither hereditary nor a disease. Myocardial infarction (MI) (ie, heart attack) is the irreversible death (necrosis) of heart muscle secondary to prolonged lack of oxygen supply (ischemia). Approximately 1.5 million cases of MI occur annually in the United States.