In I-cell disease, which organ enlargement is commonly observed?

Unlock all questions

This demo includes only 20 questions. Upgrade to access hundreds of questions, flashcards, exam simulations, and disable ads.

Full question bankExam simulationsFlashcards
From $9.99Unlock all

Improve your NBME Form 16 Test performance with interactive questions. Access detailed explanations and hints for each question to maximize your test readiness and success!

Multiple Choice

In I-cell disease, which organ enlargement is commonly observed?

Explanation:
I-cell disease shows a problem with delivering lysosomal enzymes to their proper destinations because mannose-6-phosphate tagging is defective. As a result, storage materials accumulate inside lysosomes throughout many tissues. The liver and spleen, rich in macrophages, are especially affected; these cells take up the excess storage material and enlarge. Since both organs tend to be involved, the typical finding is hepatosplenomegaly.

I-cell disease shows a problem with delivering lysosomal enzymes to their proper destinations because mannose-6-phosphate tagging is defective. As a result, storage materials accumulate inside lysosomes throughout many tissues. The liver and spleen, rich in macrophages, are especially affected; these cells take up the excess storage material and enlarge. Since both organs tend to be involved, the typical finding is hepatosplenomegaly.

More Multiple Choice Questions
Subscribe

Get the latest from Examzify

You can unsubscribe at any time. Read our privacy policy