Literature DB >> 6425121

Chronic vomiting in a case of citrullinaemia detected after treatment by total parenteral nutrition.

A Benque, G Bommelaer, G Rozental, P Cales, L Cathelineau, D Pham Dinh, A Ribet.   

Abstract

We report a case of a 56 year old woman who presented with a long history of chronic attacks of vomiting. On admission to hospital she was cachectic, and attempted parenteral nutrition induced coma. The illness was found to be due to citrullinaemia, a metabolic disorder of the urea cycle. Our patient is the oldest with this disorder so far described in the literature. The main points of the case and its investigation are outlined: hyperammonaemia, amino acid chromatogram, measurement of enzyme activity in skin and liver biopsy material. The therapeutic measures which led to cure are of particular interest.

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Year:  1984        PMID: 6425121      PMCID: PMC1432457          DOI: 10.1136/gut.25.5.531

Source DB:  PubMed          Journal:  Gut        ISSN: 0017-5749            Impact factor:   23.059


  11 in total

1.  Periodic hyperammonemia, hyperlysinemia, and homocitrullinuria associated with decreased argininosuccinate synthetase and arginase activities.

Authors:  H Sogawa; K Oyanagi; T Nakao
Journal:  Pediatr Res       Date:  1977-09       Impact factor: 3.756

Review 2.  Inherited hyperammonemic syndromes.

Authors:  Y E Hsia
Journal:  Gastroenterology       Date:  1974-08       Impact factor: 22.682

3.  Citrullinemia: a new case, with implications concerning adaptation to defective urea synthesis.

Authors:  A Scott-Emuakpor; J V Higgins; A F Kohrman
Journal:  Pediatr Res       Date:  1972-07       Impact factor: 3.756

4.  Citrullinemia: elevated serum citrulline levels in healthy siblings.

Authors:  H Wick; T Brechbühler; J Girard
Journal:  Experientia       Date:  1970-08-15

5.  Improved method for the antenatal diagnosis of citrullinemia.

Authors:  L Cathelineau; D Pham Dinh; J Boué; J M Saudubray; J P Farriaux; P Kamoun
Journal:  Clin Chim Acta       Date:  1981-10-08       Impact factor: 3.786

6.  Citrullinemia with defective urea production.

Authors:  G Morrow; L A Barness; M L Efron
Journal:  Pediatrics       Date:  1967-10       Impact factor: 7.124

7.  Treatment of hyperammonemia.

Authors:  B Levin; A Russell
Journal:  Am J Dis Child       Date:  1967-01

8.  Treatment of adult-type citrullinemia with administration of citrate.

Authors:  Y Yajima; T Hirasawa; T Saheki
Journal:  Tohoku J Exp Med       Date:  1981-07       Impact factor: 1.848

9.  The effect of keto-analogues of essential amino acids in severe chronic uremia.

Authors:  M Walser; A W Coulter; S Dighe; F R Crantz
Journal:  J Clin Invest       Date:  1973-03       Impact factor: 14.808

10.  Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion.

Authors:  M L Batshaw; S Brusilow; L Waber; W Blom; A M Brubakk; B K Burton; H M Cann; D Kerr; P Mamunes; R Matalon; D Myerberg; I A Schafer
Journal:  N Engl J Med       Date:  1982-06-10       Impact factor: 91.245

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  1 in total

Review 1.  Food triggers and inherited metabolic disorders: a challenge to the pediatrician.

Authors:  Evelina Maines; Annunziata Di Palma; Alberto Burlina
Journal:  Ital J Pediatr       Date:  2018-01-25       Impact factor: 2.638

  1 in total

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