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Question: 1 / 200

What is a primary characteristic of exocrine pancreatic insufficiency in Schwachman-Diamond Syndrome?

Defective development of acinar cells

A primary characteristic of exocrine pancreatic insufficiency in Schwachman-Diamond Syndrome is the defective development of acinar cells. Acinar cells are responsible for producing digestive enzymes essential for the digestion and absorption of nutrients in the small intestine. In individuals with Schwachman-Diamond Syndrome, these cells do not develop properly, leading to a deficiency in enzyme production. As a result, this insufficient enzymatic action results in malabsorption of nutrients and a spectrum of gastrointestinal symptoms.

The other options do not align with the understanding of this syndrome. Excessive production of digestive enzymes is the opposite of what occurs in this condition, where enzyme levels are inadequate, leading to malabsorption. Normal absorption of nutrients would not be a characteristic of pancreatic insufficiency, as that very condition leads to nutrient deficiencies due to poor absorption. Overproduction of fatty tissues in the pancreas does not characterize Schwachman-Diamond Syndrome either, as the problem lies in the insufficient development of acinar cells and their secretory function.

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Excessive production of digestive enzymes

Normal absorption of nutrients

Overproduction of fatty tissues in the pancreas

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