Schwachman-Diamond Syndrome is caused from a genetic mutation that affects many parts of the body particularly the bone marrow, pancreas, and bones. This syndrome decreases the exocrine function (synthesis and secretion of digestive enzymes) of the pancreas while its endocrine function remains fully functional.
A. Less trypsinogen, chymotrypsinogen, and procarboxypeptidase will be secreted from acinar cells in the pancreas into the duodenum of the small intestine. Therefore, less trypsin, chymotrypsin, and carboxypeptidase will be activated. Fewer di/tri peptides will be created from the digestion of proteins using typsin and chymotrypsin.
B. Glucagon will be synthesized and secreted from the beta cells of the pancreas during post-absorptive states.
C. This disease would decrease the absorption of nutrients (malabsorption) in the GI tract. Therefore, people with this disease will undergo lipolysis of stored triglycerides to try to compensate for the malabsorption. The lipolysis of triglycerides will result in fatty acids and glycerol. The glycerol will undergo beta-oxidation in the liver to form acetyl CoA.
D. Insulin, which is chemically classified as a steroid, will be secreted normally, and bind to receptors on the target cells to lower the blood glucose levels.