Literature DB >> 3148071

The effect of oral betaine on vertebral body bone density in pyridoxine-non-responsive homocystinuria.

W A Gahl1, I Bernardini, S Chen, D Kurtz, K Horvath.   

Abstract

Five pyridoxine-non-responsive homocystinuric patients aged 5 to 32 years were treated with oral betaine, 3 g b.i.d, in a double-blind, placebo-controlled, two-year crossover study of its effect on bone mineralization. Betaine therapy significantly reduced mean plasma homocystine (36 +/- 9 (SEM) mumol L-1 to 9 +/- 4 mumol L-1), with variable increases in plasma methionine and no adverse effects. Bone density, measured by computerized tomographic scanning of vertebral bodies, was below normal in all patients at the start of the study, and was not significantly altered by betaine therapy administered according to this protocol.

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Year:  1988        PMID: 3148071     DOI: 10.1007/bf01800372

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN: 0141-8955            Impact factor:   4.982


  12 in total

1.  The use of betaine for the treatment of homocystinuria.

Authors:  L A Smolin; N J Benevenga; S Berlow
Journal:  J Pediatr       Date:  1981-09       Impact factor: 4.406

2.  Homocystinuria. Clinical and dietary studies.

Authors:  D P Brenton; D C Cusworth; C E Dent; E E Jones
Journal:  Q J Med       Date:  1966-07

3.  Betaine in the treatment of homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency.

Authors:  U Wendel; H J Bremer
Journal:  Eur J Pediatr       Date:  1984-06       Impact factor: 3.183

4.  Quantitative computed tomography of vertebral spongiosa: a sensitive method for detecting early bone loss after oophorectomy.

Authors:  H K Genant; C E Cann; B Ettinger; G S Gordan
Journal:  Ann Intern Med       Date:  1982-11       Impact factor: 25.391

5.  Homocystinuria--the effects of betaine in the treatment of patients not responsive to pyridoxine.

Authors:  D E Wilcken; B Wilcken; N P Dudman; P A Tyrrell
Journal:  N Engl J Med       Date:  1983-08-25       Impact factor: 91.245

6.  Decreased spinal mineral content in amenorrheic women.

Authors:  C E Cann; M C Martin; H K Genant; R B Jaffe
Journal:  JAMA       Date:  1984-02-03       Impact factor: 56.272

7.  Hepatic methionine adenosyltransferase deficiency in a 31-year-old man.

Authors:  W A Gahl; J D Finkelstein; K D Mullen; I Bernardini; J J Martin; P Backlund; K G Ishak; J H Hoofnagle; S H Mudd
Journal:  Am J Hum Genet       Date:  1987-01       Impact factor: 11.025

8.  Crossover and self-controlled designs in clinical research.

Authors:  T A Louis; P W Lavori; J C Bailar; M Polansky
Journal:  N Engl J Med       Date:  1984-01-05       Impact factor: 91.245

9.  The natural history of homocystinuria due to cystathionine beta-synthase deficiency.

Authors:  S H Mudd; F Skovby; H L Levy; K D Pettigrew; B Wilcken; R E Pyeritz; G Andria; G H Boers; I L Bromberg; R Cerone
Journal:  Am J Hum Genet       Date:  1985-01       Impact factor: 11.025

10.  Homocystinuria due to cystathionine beta-synthase deficiency--the effects of betaine treatment in pyridoxine-responsive patients.

Authors:  D E Wilcken; N P Dudman; P A Tyrrell
Journal:  Metabolism       Date:  1985-12       Impact factor: 8.694

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  2 in total

1.  Homocystinuria.

Authors:  D M Isherwood
Journal:  BMJ       Date:  1996-10-26

2.  An indirect response model of homocysteine suppression by betaine: optimising the dosage regimen of betaine in homocystinuria.

Authors:  Angela Matthews; Trevor N Johnson; Amin Rostami-Hodjegan; Anupam Chakrapani; J Edward Wraith; Stuart J Moat; James R Bonham; Geoffrey T Tucker
Journal:  Br J Clin Pharmacol       Date:  2002-08       Impact factor: 4.335

  2 in total

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