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Literature DB >> 6761134

Maple syrup urine disease--therapeutic use of insulin in catabolic states.

U Wendel, U Langenbeck, I Lombeck, H J Bremer.

Abstract

High and neurotoxic blood levels of leucine and its ketoanalogue develop in catabolic patients with maple syrup urine disease. The use of relatively high doses of insulin and additional glucose had a more pronounced effect on lowering leucine (and alpha-ketoisocaproate) blood levels than dietary elimination of leucine alone. This is demonstrated in 2 neonates after blood exchange transfusion and in one 4-months old patient suffering from febrile diarrhea.

Entities: Chemical Disease Gene Species

Mesh：

Substances：
Insulin
Leucine
Glucose

Year: 1982 PMID： 6761134 DOI： 10.1007/bf01377350

Source DB: PubMed Journal: Eur J Pediatr ISSN： 0340-6199 Impact factor: 3.183

17 in total

1. Blood amine acid levels in patients with insulin excess (functioning insulinoma) and insulin deficiency (diabetic ketosis).

Authors: M Berger; H Zimmermann-Telschow; P Berchtold; H Drost; W A Müller; F A Gries; H Zimmermann
Journal: Metabolism Date: 1978-07 Impact factor: 8.694

2. Treatment of branched chain ketoaciduria (BCKA) during acute illness: approaches to the correction of the acidosis.

Authors: H A Waisman; B A Smith; E S Brown; T Gerritsen
Journal: Clin Pediatr (Phila) Date: 1972-06 Impact factor: 1.168

3. Enzyme activity in classical and variant forms of maple syrup urine disease.

Authors: J Dancis; J Hutzler; S E Snyderman; R P Cox
Journal: J Pediatr Date: 1972-08 Impact factor: 4.406

4. Key role of various individual amino acids in host response to infection.

Authors: R W Wannemacher
Journal: Am J Clin Nutr Date: 1977-08 Impact factor: 7.045

5. Peritoneal dialysis in maple-syrup-urine disease: studies on branched-chain amino and keto acids.

Authors: U Wendel; K Becker; H Przyrembel; M Bulla; C Manegold; A Mench-Hoinowski; U Langenbeck
Journal: Eur J Pediatr Date: 1980-06 Impact factor: 3.183

Review 6. The Banting Memorial Lecture 1971. Physiology of insulin in man.

Authors: G F Cahill
Journal: Diabetes Date: 1971-12 Impact factor: 9.461

7. Maple syrup urine disease: treatment of the acutely ill newborn.

Authors: G Hammersen; L Wille; H Schmidt; P Lutz; H Bickel
Journal: Eur J Pediatr Date: 1978-10-12 Impact factor: 3.183

8. Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease.

Authors: U Langenbeck; U Wendel; A Mench-Hoinowski; D Kuschel; K Becker; H Przyrembel; H J Bremer
Journal: Clin Chim Acta Date: 1978-09-01 Impact factor: 3.786

9. Insulin to inhibit protein catabolism after injury.

Authors: A M Woolfson; R V Heatley; S P Allison
Journal: N Engl J Med Date: 1979-01-04 Impact factor: 91.245

10. Outcome of early and long-term management of classical maple syrup urine disease.

Authors: C L Clow; T M Reade; C R Scriver
Journal: Pediatrics Date: 1981-12 Impact factor: 7.124

7 in total

Review 1. 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

Maple syrup urine disease--therapeutic use of insulin in catabolic states.

1. Blood amine acid levels in patients with insulin excess (functioning insulinoma) and insulin deficiency (diabetic ketosis).

2. Treatment of branched chain ketoaciduria (BCKA) during acute illness: approaches to the correction of the acidosis.

3. Enzyme activity in classical and variant forms of maple syrup urine disease.

4. Key role of various individual amino acids in host response to infection.

5. Peritoneal dialysis in maple-syrup-urine disease: studies on branched-chain amino and keto acids.

Review 6. The Banting Memorial Lecture 1971. Physiology of insulin in man.

7. Maple syrup urine disease: treatment of the acutely ill newborn.

8. Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease.

9. Insulin to inhibit protein catabolism after injury.

10. Outcome of early and long-term management of classical maple syrup urine disease.

Review 1. Acute management of propionic acidemia.

Review 2. Emergency treatment in glutaryl-CoA dehydrogenase deficiency.

3. Haemodialysis for metabolic decompensation in propionic acidaemia.

4. The early detection and management of inborn errors presenting acutely in the neonatal period.

5. Hudson memorial lecture. Neonatal management of organic acidurias. Clinical update.

Review 6. Emergency management of inherited metabolic diseases.

7. Glutaric aciduria type II: treatment with riboflavine, carnitine and insulin.