Literature DB >> 4053393

Bile acid therapies applied to patients suffering from cerebrotendinous xanthomatosis.

B J Koopman, B G Wolthers, J C van der Molen, R J Waterreus.   

Abstract

Patients suffering from cerebrotendinous xanthomatosis, an inborn error of metabolism in bile acid synthesis, were given oral treatment with chenodeoxycholic acid, ursodeoxycholic acid, cholic acid and taurocholic acid. The effectiveness of the different therapies was evaluated by measuring the urinary excretion of 5 beta-cholestane-3 alpha,7 alpha,12 alpha,23,25-pentol, which should decrease, when the administered bile acid is able to suppress endogenous bile acid synthesis. From the results it is concluded that chenodeoxycholic acid and cholic acid activate the bile acid negative feedback mechanism, contrary to ursodeoxycholic acid and taurine conjugated cholic acid. Either cholic acid or chenodeoxycholic acid are the therapies of choice for the treatment of cerebrotendinous xanthomatosis. For various reasons the use of cholic acid is especially recommended.

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Year:  1985        PMID： 4053393     DOI： 10.1016/0009-8981(85)90182-2

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN： 0009-8981            Impact factor:   3.786


  11 in total

1.  Case of cerebrotendinous xanthomatosis. I: Unusual ophthalmic features.

Authors:  S J Morgan; P McKenna; R C Bosanquet
Journal:  Br J Ophthalmol       Date:  1989-12       Impact factor: 4.638

2.  A suspicion index for early diagnosis and treatment of cerebrotendinous xanthomatosis.

Authors:  Andrea Mignarri; Gian Nicola Gallus; Maria Teresa Dotti; Antonio Federico
Journal:  J Inherit Metab Dis       Date:  2014-01-18       Impact factor: 4.982

3.  Case report 427: Cerebrotendinous xanthomatosis.

Authors:  M Burnstein; K A Buckwalter; W Martel; K D McClatchey; D Quint
Journal:  Skeletal Radiol       Date:  1987       Impact factor: 2.199

4.  Delta 4-3-oxosteroid 5 beta-reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis.

Authors:  K D Setchell; F J Suchy; M B Welsh; L Zimmer-Nechemias; J Heubi; W F Balistreri
Journal:  J Clin Invest       Date:  1988-12       Impact factor: 14.808

5.  Nationwide survey on cerebrotendinous xanthomatosis in Japan.

Authors:  Yoshiki Sekijima; Shingo Koyama; Tsuneaki Yoshinaga; Masayoshi Koinuma; Yuji Inaba
Journal:  J Hum Genet       Date:  2018-01-10       Impact factor: 3.172

Review 6.  Mechanisms of disease: Inborn errors of bile acid synthesis.

Authors:  Shikha S Sundaram; Kevin E Bove; Mark A Lovell; Ronald J Sokol
Journal:  Nat Clin Pract Gastroenterol Hepatol       Date:  2008-06-24

7.  Prospective treatment of cerebrotendinous xanthomatosis with cholic acid therapy.

Authors:  Germaine Pierre; Kenneth Setchell; Jacqueline Blyth; Mary Anne Preece; Anupam Chakrapani; Patrick McKiernan
Journal:  J Inherit Metab Dis       Date:  2008-12-27       Impact factor: 4.982

Review 8.  Epidemiology, diagnosis, and treatment of cerebrotendinous xanthomatosis (CTX).

Authors:  Gerald Salen; Robert D Steiner
Journal:  J Inherit Metab Dis       Date:  2017-10-04       Impact factor: 4.982

9.  Cerebrotendinous xanthomatosis: a review of biochemical findings of the patient population in The Netherlands.

Authors:  B J Koopman; B G Wolthers; J C van der Molen; W van der Slik; R J Waterreus; A van Spreeken
Journal:  J Inherit Metab Dis       Date:  1988       Impact factor: 4.982

10.  Development of Photodynamic Antimicrobial Chemotherapy (PACT) for Clostridium difficile.

Authors:  Luisa De Sordi; M Adil Butt; Hayley Pye; Darina Kohoutova; Charles A Mosse; Gokhan Yahioglu; Ioanna Stamati; Mahendra Deonarain; Sinan Battah; Derren Ready; Elaine Allan; Peter Mullany; Laurence B Lovat
Journal:  PLoS One       Date:  2015-08-27       Impact factor: 3.240
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