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HMD can be diagnosed with an MRI, using a tracing dye in the subject's blood, and observing the flow of blood through the subject's liver and surrounding areas (stomach, intestine) for anomalies. It can also be diagnosed using a bile-acid level test; or more accurately, a "fasting-blood ammonia levels" test.
If the common bile duct is plugged, the surgeon may have to open the duodenum opposite of where the common bile duct enters the small intestine and flush it with a catheter. [ 13 ] Some complications that may occur due to this procedure includes vomiting, bile peritonitis if bile leaks into the abdominal cavity, pancreatitis and in some cases ...
Measurement of 7α-Hydroxy-4-cholesten-3-one, (C4), a bile acid precursor, in serum, shows the increased bile acid synthesis found in bile acid malabsorption. [20] This test is an alternative diagnostic means when available. Fasting blood FGF19 values may have value in the recognition of the disease and prediction of response. [21]
Bile salt-dependent lipase (or BSDL), also known as carboxyl ester lipase (or CEL) is an enzyme produced by the adult pancreas and aids in the digestion of fats. Bile salt-stimulated lipase (or BSSL) is an equivalent enzyme found within breast milk. BSDL has been found in the pancreatic secretions of all species in which it has been looked for.
Bile acids also have hormonal actions throughout the body, particularly through the farnesoid X receptor and GPBAR1 (also known as TGR5). [7] Bile acid synthesis is the only manner in which humans or other mammals may excrete excess cholesterol, as the parent compound of all bile acids is cholesterol. [citation needed]
In addition, unabsorbed fatty acids, converted to hydroxy-fatty acids by colonic flora, as well as unabsorbed bile acids both impair absorption and induce secretion of water and electrolytes by the colon adding to stool mass. Weight loss is common among patients with significant intestinal malabsorption but must be evaluated in the context of ...