Literature DB >> 1471895

Intercurrent illness in inborn errors of intermediary metabolism.

M A Dixon1, J V Leonard.   

Abstract

Metabolic decompensation may occur in patients with disorders of intermediary metabolism during intercurrent illness. To prevent complications it is normal practice to change the diet to an 'emergency regimen'. The mainstay of this is a high carbohydrate intake, using soluble glucose polymer, given as frequent drinks by day and during the night. Additional therapy is given for some disorders. Practical details of the treatment are outlined.

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Year:  1992        PMID： 1471895      PMCID： PMC1793747          DOI： 10.1136/adc.67.11.1387

Source DB:  PubMed          Journal:  Arch Dis Child        ISSN： 0003-9888            Impact factor:   3.791


  4 in total

1.  Sources of propionate in inborn errors of propionate metabolism.

Authors:  G N Thompson; J H Walter; J L Bresson; G C Ford; S L Lyonnet; R A Chalmers; J M Saudubray; J V Leonard; D Halliday
Journal:  Metabolism       Date:  1990-11       Impact factor: 8.694

2.  Muscle protein synthesis measured by stable isotope techniques in man: the effects of feeding and fasting.

Authors:  M J Rennie; R H Edwards; D Halliday; D E Matthews; S L Wolman; D J Millward
Journal:  Clin Sci (Lond)       Date:  1982-12       Impact factor: 6.124

3.  Acute illness in maple syrup urine disease: dynamics of protein metabolism and implications for management.

Authors:  G N Thompson; D E Francis; D Halliday
Journal:  J Pediatr       Date:  1991-07       Impact factor: 4.406

4.  Renal tubular dysfunction in methylmalonic acidaemia.

Authors:  C T D'Angio; M J Dillon; J V Leonard
Journal:  Eur J Pediatr       Date:  1991-02       Impact factor: 3.183
  4 in total
  20 in total

1.  Screening for inherited metabolic disease in newborn infants using tandem mass spectrometry.

Authors:  James V Leonard; Carol Dezateux
Journal:  BMJ       Date:  2002-01-05

Review 2.  Newborn screening for medium chain acyl-CoA dehydrogenase deficiency: evaluating the effects on outcome.

Authors:  Carol Dezateux
Journal:  Eur J Pediatr       Date:  2003-11-20       Impact factor: 3.183

Review 3.  Acute management of propionic acidemia.

Authors:  Kimberly A Chapman; Andrea Gropman; Erin MacLeod; Kathy Stagni; Marshall L Summar; Keiko Ueda; Nicholas Ah Mew; Jill Franks; Eddie Island; Dietrich Matern; Loren Pena; Brittany Smith; V Reid Sutton; Tiina Urv; Charles Venditti; Anupam Chakrapani
Journal:  Mol Genet Metab       Date:  2011-09-24       Impact factor: 4.797

4.  Guideline for the diagnosis and management of glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I).

Authors:  S Kölker; E Christensen; J V Leonard; C R Greenberg; A B Burlina; A P Burlina; M Dixon; M Duran; S I Goodman; D M Koeller; E Müller; E R Naughten; E Neumaier-Probst; J G Okun; M Kyllerman; R A Surtees; B Wilcken; G F Hoffmann; P Burgard
Journal:  J Inherit Metab Dis       Date:  2007-01-03       Impact factor: 4.982

Review 5.  Early recognition of metabolic decompensation.

Authors:  A A Morris; J V Leonard
Journal:  Arch Dis Child       Date:  1997-06       Impact factor: 3.791

Review 6.  The liver is a metabolic and immunologic organ: A reconsideration of metabolic decompensation due to infection in inborn errors of metabolism (IEM).

Authors:  Tatyana N Tarasenko; Peter J McGuire
Journal:  Mol Genet Metab       Date:  2017-06-24       Impact factor: 4.797

Review 7.  Proposed recommendations for diagnosing and managing individuals with glutaric aciduria type I: second revision.

Authors:  Nikolas Boy; Chris Mühlhausen; Esther M Maier; Jana Heringer; Birgit Assmann; Peter Burgard; Marjorie Dixon; Sandra Fleissner; Cheryl R Greenberg; Inga Harting; Georg F Hoffmann; Daniela Karall; David M Koeller; Michael B Krawinkel; Jürgen G Okun; Thomas Opladen; Roland Posset; Katja Sahm; Johannes Zschocke; Stefan Kölker
Journal:  J Inherit Metab Dis       Date:  2016-11-16       Impact factor: 4.982

8.  Isolated (biotin-resistant) 3-methylcrotonyl-CoA carboxylase deficiency: four sibs devoid of pathology.

Authors:  J Mourmans; J Bakkeren; J de Jong; R Wevers; O P van Diggelen; T Suormala; R Baumgartner; U Wendel
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

9.  Screening for medium chain acyl-CoA dehydrogenase deficiency using electrospray ionisation tandem mass spectrometry.

Authors:  P T Clayton; M Doig; S Ghafari; C Meaney; C Taylor; J V Leonard; M Morris; A W Johnson
Journal:  Arch Dis Child       Date:  1998-08       Impact factor: 3.791

Review 10.  Diagnosis and management of glutaric aciduria type I.

Authors:  I Barić; J Zschocke; E Christensen; M Duran; S I Goodman; J V Leonard; E Müller; D H Morton; A Superti-Furga; G F Hoffmann
Journal:  J Inherit Metab Dis       Date:  1998-06       Impact factor: 4.982
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