Literature DB >> 9584259

Pregnancy in a woman with maple syrup urine disease.

S Grünewald1, F Hinrichs, U Wendel.   

Abstract

We present the favourable outcome of a pregnancy in a woman with maple syrup urine disease. Keeping the maternal plasma levels of the branched-chain amino acids between 100 and 300 mumol/L is compatible with delivery of a normal infant. Leucine tolerance increased progressively from the 22nd week of gestation from 350 to 2100 mg/day. The risk of metabolic decompensation in the postpartum period can be minimized by careful monitoring of the mother after delivery.

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Year:  1998        PMID： 9584259     DOI： 10.1023/a:1005396823030

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


  13 in total

1.  Hyperammonemia in women with a mutation at the ornithine carbamoyltransferase locus. A cause of postpartum coma.

Authors:  P H Arn; E R Hauser; G H Thomas; G Herman; D Hess; S W Brusilow
Journal:  N Engl J Med       Date:  1990-06-07       Impact factor: 91.245

2.  Pregnancy and argininosuccinic aciduria.

Authors:  S Worthington; J Christodoulou; B Wilcken; B Peat
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982

Review 3.  Nutrition support of maternal phenylketonuria.

Authors:  P B Acosta
Journal:  Semin Perinatol       Date:  1995-06       Impact factor: 3.300

4.  Postoperative metabolic decompensation in maple syrup urine disease is completely prevented by insulin.

Authors:  B Biggemann; R Zass; U Wendel
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

5.  Postpartum coma and death due to carbamoyl-phosphate synthetase I deficiency.

Authors:  L J Wong; W J Craigen; W E O'Brien
Journal:  Ann Intern Med       Date:  1994-02-01       Impact factor: 25.391

6.  Maternal isovaleric acidemia.

Authors:  V E Shih; R H Aubry; G DeGrande; S F Gursky; K Tanaka
Journal:  J Pediatr       Date:  1984-07       Impact factor: 4.406

7.  Outcome implications of the International Maternal Phenylketonuria Collaborative Study (MPKUCS): 1994.

Authors:  R Koch; H Levy; W Hanley; R Matalon; B Rouse; F Trefz; F de la Cruz
Journal:  Eur J Pediatr       Date:  1996-07       Impact factor: 3.183

8.  German Maternal Phenylketonuria Study.

Authors:  S Cipcic-Schmidt; F K Trefz; B Fünders; G Seidlitz; K Ullrich
Journal:  Eur J Pediatr       Date:  1996-07       Impact factor: 3.183

9.  Methylmalonic aciduria in pregnancy: a case report.

Authors:  E Diss; J Iams; N Reed; D S Roe; C Roe
Journal:  Am J Obstet Gynecol       Date:  1995-03       Impact factor: 8.661

10.  Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia.

Authors:  S C Van Calcar; C O Harding; S R Davidson; L A Barness; J A Wolff
Journal:  Am J Med Genet       Date:  1992-11-15
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  13 in total

1.  Successful pregnancy outcome in a woman with argininosuccinate lyase deficiency.

Authors:  M R Mardach; K Roe; S D Cederbaum
Journal:  J Inherit Metab Dis       Date:  1999-04       Impact factor: 4.982

2.  Successful Pregnancy in a Woman with Maple Syrup Urine Disease: Case Report.

Authors:  Stefanie Heiber; Henryk Zulewski; Marianne Zaugg; Caroline Kiss; Matthias Baumgartner
Journal:  JIMD Rep       Date:  2015-02-27

3.  The management of pregnancy in maple syrup urine disease: experience with two patients.

Authors:  Michel Tchan; M Westbrook; G Wilcox; R Cutler; N Smith; R Penman; B J G Strauss; B Wilcken
Journal:  JIMD Rep       Date:  2013-02-14

4.  Variant maple syrup urine disease (MSUD)--the entire spectrum.

Authors:  E Simon; N Flaschker; P Schadewaldt; U Langenbeck; U Wendel
Journal:  J Inherit Metab Dis       Date:  2006-10-25       Impact factor: 4.982

5.  Pregnancy issues in inherited metabolic disorders.

Authors:  Philip J Lee
Journal:  J Inherit Metab Dis       Date:  2006 Apr-Jun       Impact factor: 4.982

Review 6.  Inborn errors of metabolism and pregnancy.

Authors:  J H Walter
Journal:  J Inherit Metab Dis       Date:  2000-05       Impact factor: 4.982

7.  Successful pregnancy in severe methylmalonic acidaemia.

Authors:  M P Wasserstein; S Gaddipati; S E Snyderman; K Eddleman; R J Desnick; C Sansaricq
Journal:  J Inherit Metab Dis       Date:  1999-10       Impact factor: 4.982

Review 8.  Pregnancy in women with inherited metabolic disease.

Authors:  Elaine Murphy
Journal:  Obstet Med       Date:  2015-03-29

9.  Successful pregnancy and delivery in a woman with propionic acidemia from the Amish community.

Authors:  Jessica Scott Schwoerer; Sandra van Calcar; Gregory M Rice; James Deline
Journal:  Mol Genet Metab Rep       Date:  2016-06-02

Review 10.  Successful pregnancy in maple syrup urine disease: a case report and review of the literature.

Authors:  Sarah Catharina Grünert; Stefanie Rosenbaum-Fabian; Anke Schumann; Karl Otfried Schwab; Nadja Mingirulli; Ute Spiekerkoetter
Journal:  Nutr J       Date:  2018-05-12       Impact factor: 3.271
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