Bile acid loss syndrome K90.8

The bile acid loss syndrome is a disease that results in a functionally relevant loss of bile acids (Camilleri M 2014).

A bile acid loss syndrome can be divided into the stages of compensated and decompensated GSVS.
In the compensated bile acid loss syndrome stage, a still sufficient amount of bile acid is produced in the liver, diarrhoea occurs due to a laxative effect of the bile acid in the colon.
During the decompensated stage, there is a severe loss of bile acids without a compensatory production by the liver; fatty stools therefore occur.

A bile acid loss syndrome can occur in the absence of absorption of the bile acids in the ileum(Crohn's disease, ileum resection) and in the context of bacterial deconjugation of the bile acids in the small intestine (blind sack syndrome).

If the reabsorption of the bile acids in the ileum fails, they enter the colon instead. If the ileum fails to reabsorb the bile acids in the ileum by > 40 cm, there is a risk of chologic diarrhoea.

In the presence of a partial resection of the ileum, the loss of bile acids is compensated by an increased synthesis of the liver. With an ileum resection of > 100 cm, there is a risk of steatorrhea.
An increased loss of bile acids can then lead to the formation of gallstones and cholesterol stones.
An intestinal binding of calcium to fatty acids leads to an increased absorption of oxalic acid and thus to oxal kidney stones.

Chologic diarrhea or steatorrhea. The consequences: weight loss, toxic anal eczema, hypovitaminosis.

Medical history and clinic.

A specific test for the detection of bile acid loss syndrome is the 14C-glycocholate breath test (not a routine test): after oral administration of radioactively labelled bile acid (14c-glycocholate), normally 95% is absorbed in the terminal ileum. The remainder reaches the colon where it is bacterially deconjugated. Radioactive CO2 is released, absorbed and exhaled via the lungs or can be determined there. A bile acid loss syndrome leads to increased CO2 exhalation.

SeHCAT scintigraphy: This nuclear medicine test involves repeated imaging at certain time intervals and is therefore able to map several cycles of the bile acid cycle. However, this examination is associated with a high level of radiation exposure.

Quantitative detection of bile acids in stool using enzymatic, chromatographic or mass spectrometric methods: This detection method is only carried out as part of scientific investigations.

Maldigestion syndrome or cholesterol gallstones as well as oxal kidney stones may occur.

Causal therapy:

If present: treatment of Crohn's disease.

In blindsack syndrome (the blindsack syndrome is characterized by a bag-like protrusion of the small intestine. The main cause of this syndrome is surgery on the small intestine). Trials with ibuprofen (Basturk A et al. 2016) or cephalosporins, metronidazole or clotrimazole may be successful in individual cases (Zhao Z et al. 2017).

Symptomatic accompanying therapy:

• Treatment of the malabsorption syndrome
• Oxalate stones: Calcium 1 g oral/day (binds oxalate); MCT fats (medium-chain trigylcerides), increase drinking quantity
• Treatment of chologic diarrhoea: in compensated GSVS: use of exchange resins such as colestyramine. In severe chologic diarrhoea, the use of colestyramine can further aggravate steatorrhoea. Hypovitaminosis due to malabsorption should be compensated. Restriction of fat intake, intake of medium-chain fatty acids (MCT products).

1. Basturk A et al (2016) Acute vanishing bile duct syndrome after the use of ibuprofen. Arab J Gastroenterol 17:137-139.
2. Camilleri M (2014) Advances in understanding of bile acid diarrhea. Expert Rev Gastroenterol Hepatol 8:49-61.
3. Pereira-Fantini PM et al (2106) Short bowel syndrome (SBS)-associated alterations within the gut-liver axis evolve early and persist long-term in the piglet model of short bowel syndrome. J Gastroenterol Hepatol 31:1946-1955.
4. Zhao Z et al (2017) Acute vanishing bile duct syndrome after therapy with cephalosporin, metronidazole, and clotrimazole: A case report. Medicine (Baltimore) 96:e8009.