Chronic diarrhea caused by idiopathic bile acid malabsorption: An explanation at last

Expert Review of Gastroenterology and Hepatology

Volumn 3, Issue 5, 2009, Pages 461-464

  Hofmann, Alan F ^a  

^a UNIVERSITY OF CALIFORNIA SAN DIEGO   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

7ALPHA HYDROXYCHOLEST 4 ENE 3 ONE; BILE ACID; BILE ACID CONJUGATE; BILE ACID SEQUESTRANT; BIOLOGICAL MARKER; FIBROBLAST GROWTH FACTOR; FIBROBLAST GROWTH FACTOR 19; HYDROXYL GROUP; ORGANIC ANION TRANSPORTER; UNCLASSIFIED DRUG;

ABSORPTION; ACTIVE TRANSPORT; BACTERIAL METABOLISM; BILE ACID MALABSORPTION; BILE ACID METABOLISM; BILE ACID SYNTHESIS; CELL MEMBRANE TRANSPORT; CHRONIC DIARRHEA; COLON ABSORPTION; CONJUGATION; DIARRHEA; DRUG RESPONSE; EDITORIAL; ENTEROHEPATIC CIRCULATION; HUMAN; IDIOPATHIC DISEASE; ILEUM; INTESTINE CELL; MICELLE;

ANIMALS; BILE ACIDS AND SALTS; CHRONIC DISEASE; DIARRHEA; ENTEROCYTES; ENTEROHEPATIC CIRCULATION; FIBROBLAST GROWTH FACTORS; HEPATOCYTES; HUMANS; INTESTINAL ABSORPTION; MALABSORPTION SYNDROMES;

EID: 70449729508     PISSN: 17474124     EISSN: None     Source Type: Journal    
DOI: 10.1586/egh.09.49     Document Type: Editorial

References (44)

1. Hofmann AF, Mysels KJ. Bile salts as biological surfactants. Coll. Surf. 30, 145-173 (1988).
2. Gass J, Vora H, Hofmann AF, Gray GM, Khosla C. Enhancement of dietary protein digestion by conjugated bile acids. Gastroenterology 133, 16-23 (2007). (Pubitemid 47031127)
3. Hofmann AF, Hagey LR. Bile acids: chemistry, pathochemistry, biology, pathobiology, and therapeutics. Cell. Mol. Life Sci. 65, 2461-2483 (2008). • Concise overview of bile acid biology and chemistry in health and disease.
4. Dawson PA, Lan T, Rao A. Bile acid transporters. J. Lipid Res. DOI: 10.1194/ jlr.R900012-JLR200 (2009) (Epub ahead of print). •• Review of bile acid transporters from the laboratory that cloned the apical transporter and elucidated the functions of both apical and basolateral transporters of the ileal enterocyte.
5. Hofmann AF, Cravetto C, Molino G, Belforte G, Bona B. Simulation of the metabolism and enterohepatic circulation of endogenous deoxycholic acid in man using a physiological pharmacokinetic model for bile acid metabolism. Gastroenterology 93, 693-709 (1987). (Pubitemid 17155993)
6. Keating N, Scharl MM, Marsh C, Ferguson G, Hofmann AF, Keely SJ. Physiological concentrations of bile acids downregulate agonist induced secretion in colonic epithelial cells. J. Cell. Mol. Med. DOI: 10.1111/j.1582-4934.2009.00838.x (2009) (Epub ahead of print). • Experiments using a polarized colonic cell line present evidence that low concentrations of bile acids have an antisecretory effect on colonic epithelial cells.
7. Chadwick VS, Gaginella TS, Carlson GL, Debongnie JC, Phillips SF, Hofmann AF. Effect of molecular structure on bile acid-induced alterations in absorptive function, permeability, and morphology in the perfused rabbit colon. J. Lab. Clin. Med. 94(5), 661-674 (1979).
8. Keely S, Scharl M, Bertelsen LS, Hagey LR, Barrett KE, Hofmann AF. Bile acid induced secretion in polarized monolayers of T84 colonic epithelial cells: structureactivity relationships. Am. J. Physiol. 292, G292-G297 (2007).
9. Shefer S, Hauser S, Bekersky I, Mosbach EH. Feedback regulation of bile acid biosynthesis in the rat. J. Lipid Res. 10, 646-655 (1969).
10. Vlahcevic ZR, Pandak WM, Stravitz RT. Regulation of bile acid biosynthesis. Gastroenterol. Clin. North Am. 28, 1-25 (1999).
11. Parks DJ, Blanchard SG, Bledsoe RK et al. Bile acids: natural ligands for an orphan nuclear receptor. Science 284, 1365-1368 (1999).
12. Makashima M, Okamoto AY, Repa JJ et al. Identification of a nuclear receptor for bile acids. Science 284, 1362-1365 (1999).
13. Wang H, Chen J, Holister K, Sowers LC, Forman BM. Endogenous bile acids are ligands for the nuclear receptor FXR/BAR. Mol. Cell 3, 543-553 (1999). (Pubitemid 29268438)
14. Lu TT, Makashima M, Repa JJ et al. Molecular basis for feedback regulation of bile acid synthesis by nuclear receptors. Mol. Cell 6, 507-515 (2000).
15. Chiang JYL. Bile acids: regulation of synthesis. J. Lipid Res. DOI: 10.1194/jlr. R900010-JLR200 (2009) (Epub ahead of print). • Summarizes current knowledge of the regulation of bile acid synthesis by an investigator who has contributed greatly to our understanding of this complex feedback pathway.
16. Pandak WM, Heuman DM, Hylemon PB, Chiang JYL, Vlahcevic ZR. Failure of intravenous infusion of taurocholate to down-regulate cholesterol 7 a-hydroxylase in rats with biliary fistulas. Gastroenterology 108, 533-544 (1995).
17. Nagano M, Kuroki S, Mizuta A et al. Regulation of bile acid synthesis under reconstructed enterohepatic circulation in rats. Steroids 69, 701-709 (2004). (Pubitemid 39297711)
18. Inagaki T, Choi M, Moschetta A et al. Fibroblast growth factor 15 functions as an enterohepatic signal to regulate bile acid homeostasis. Cell Metab. 2, 217-225 (2005). •• Key paper identifying FGF19 as a signaling molecule secreted by the ileal enterocyte that is required for the downregulation of bile acid synthesis in the liver.
19. Goetz R, Beenken A, Ibrahimi OA et al. Molecular insights into the klotho-dependent, endocrine mode of action of fibroblast growth factor 19 subfamily members. Mol. Cell Biol. 27, 3417-3428 (2007). (Pubitemid 46685217)
20. Lin BC, Wang M, Blackmore C, Desnovers LR. Liver-specific activities of FGF19 require Klotho b. J. Biol. Chem. 282, 27277-27284 (2007). (Pubitemid 47501977)
21. Wu X, Ge H, Gupte J et al. Co-receptor requirements for fibroblast growth factor-19 signaling. J. Biol. Chem. 282, 29069-29072 (2007). (Pubitemid 350043330)
22. Yu C, Wang F, Kan M, Satoh J, Nabeshima Y, Nabeshima Y. Elevated cholesterol metabolism and bile acid synthesis in mice lacking membrane tyrosine kinase receptor FGFR4. J. Biol. Chem. 275, 15482-15489 (2000). (Pubitemid 30337280)
23. Ito S, Fujimori T, Furuya A et al. Impaired negative feedback suppression of bile acid synthesis in mice lacking bklotho. J. Clin. Invest. 115, 2202-2208 (2005). (Pubitemid 41134162)
24. Grundy SM, Ahrens EH Jr, Salen G. Interruption of the enterohepatic circulation of bile acids in man: comparative effects of cholestyramine and ileal exclusion on cholesterol metabolism. J. Lab. Clin. Med. 78, 94-121 (1971).
25. 12 and serum lipids in patients with ileal resections. Eur. J. Clin. Invest. 1, 452-460 (1971).
26. Hofmann AF, Poley JR. Role of bile acid malabsorption in pathogenesis of diarrhea and steatorrhea in patients with ileal resection. I. Response to cholestyramine or replacement of dietary long chain triglyceride by medium chain triglyceride. Gastroenterology 62, 918-934 (1972).
27. Aldini R, Roda A, Festi D et al. Bile acid malabsorption and bile acid diarrhea in intestinal resection. Dig. Dis. Sci. 27, 495-502 (1982).
28. McJunkin B, Fromm H, Sarva RP, Amin P. Factors in the mechanism of diarrhea in bile acid malabsorption: fecal pH - a key determinant. Gastroenterology 80, 1454-1464 (1981). (Pubitemid 11113561)
29. Hofmann AF, Poley JR. Cholestyramine treatment of diarrhea associated with ileal resection. N. Engl. J. Med. 281, 397-402 (1969).
30. Thaysen EH, Pedersen L. Idiopathic bile acid catharsis. Gut 17, 965-970 (1975). • Original description of the syndrome of idiopathic bile acid malabsorption by a master clinician.
31. Williams AJ, Merrick MV, Eastwood MA. Idiopathic bile acid malabsorption - a review of clinical presentation, diagnosis, and response to treatment. Gut 32, 1004-1006 (1991).
32. Smith MJ, Cherian P, Raju GS, Dawson BF, Mahon S, Bardhan KD. Bile salt malabsorption in persistent diarrhoea. J. R. Coll. Physicians Lond. 34, 448-451 (2000).
33. Galatola G. The prevalence of bile acid malabsorption in irritable bowel syndrome and the effect of cholestyramine: an uncontrolled open multicentre study. Eur. J. Gastroenterol. Hepatol. 4, 533-537 (1992).
34. Wildt S, Norby Rasmussen S, Lysgard Madsen J et al. Bile acid malabsorption in patients with chronic diarrhoea: clinical value of SeHCAT test. Scand. J. Gastroenterol. 38, 826-830 (2003). (Pubitemid 37011574)
35. Tilburg AJ, de Rooij FW, van den Berg JW, van Blankenstein M. Primary bile acid diarrhoea without an ileal carrier defect: quantification of active bile acid transport across the ileal brush border membrane. Gut 32, 500-503 (1991).
36. Bajor A, Kilander A, Fae A et al. Normal or increased bile acid uptake in isolated mucosa from patients with bile acid malabsorption. Eur. J. Gastroenterol. Hepatol. 18, 397-403 (2006).
37. Walters JRF, Tasleem AM, Omer OS, Brydon WG, Dew T, Le Roux CW. A new mechanism for bile acid diarrhea: excessive bile acid biosynthesis caused by defective fibroblast growth factor 19 release from the ileum. Clin. Gastro. Hepatol. DOI:10.1016/j.cgh.2009.04.024 (2009) (Epub ahead of print). •• Clinical study showing that patients with idiopathic bile acid malabsorption have abnormally low plasma levels of FGF19 and provides an explanation for the condition.
38. Song KH, Li T, Owsley E, Strom S, Chiang JY. Bile acids activate fibroblast growth factor 19 signaling in human hepatocytes to inhibit cholesterol 7α-hydroxylase gene expression. Hepatology 49(1), 297-305 (2009).
39. Gälman C, Arvidsson T, Angelin B, Rudling M. Monitoring hepatic cholesterol 7 α-hydroxylase activity by assay of the stable bile acid intermediate 7 α-hydroxy-4-cholesten-3-one in peripheral blood. J. Lipid Res. 44, 859-866 (2003). • Presents evidence that plasma levels of an intermediate in bile acid synthesis were directly proportional to the rate of bile acid biosynthesis and suggests a simple test for bile acid malabsorption. (Pubitemid 37279689)
40. Eusufzai S, Axelson M, Angelin B, Einarsson K. Serum 7α-hydroxy-4- cholesten-3-one concentrations in the evaluation of bile acid malabsorption in patients with diarrhoea: correlation to SeHCAT test. Gut 34, 698-701 (1993). (Pubitemid 23146900)
41. Sauter GH, Munzing W, von Ritter C, Paumgartner G. Bile acid malabsorption a cause of chronic diarrhea: diagnostic value of 7α-hydroxy-4-cholest-3-one in serum. Dig. Dis. Sci. 44, 14-19 (1999). (Pubitemid 29053072)
42. Brydon WG, Nyhlin H, Eastwood MA, Merrick MV. Serum 7 α-hydroxy-4-cholesten-3-one and selenohomocholyltaurine (SeHCAT) whole body retention in the assessment of bile acid induced diarrhoea. Eur. J. Gastroenterol. Hepatol. 8, 117-123 (1996). (Pubitemid 26130232)
43. Puleston J, Morgan H, Andreyev J. New treatment for bile salt malabsorption. Gut 54, 441-442 (2005). • Letter to the Editor describing the successful use of colesevelam to treat diarrhea caused by bile acid malabsorption. (Pubitemid 40299109)
44. Hofmann AF. The syndrome of ileal disease and the broken enterohepatic circulation: cholerheic enteropathy. Gastroenterology 52, 752-757 (1967). • Editorial proposing that bile acid malabsorption played a major role in the diarrhea of patients with ileal dysfunction and suggesting that bile acid sequestrants might provide symptomatic benefit.