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Archives of Disease in Childhood 2000;82:236-237; doi:10.1136/adc.82.3.236
Copyright © 2000 BMJ Publishing Group Ltd & Royal College of Paediatrics and Child Health.
Arch Dis Child 2000;82:236-237 ( March )

Article

Fits, pyridoxine, and hyperprolinaemia type II Valerie Walkera, Graham A Millsb, Sheila A Petersc, W Louis Mertond

a Department of Chemical Pathology, Southampton General Hospital, Tremona Road, Southampton SO16 6YD, UK, b School of Pharmacy and Biological Sciences, University of Portsmouth, White Swan Road, Portsmouth PO1 2DT, UK, c Department of Paediatrics, St Mary's Hospital, Milton Road, Portsmouth PO3 6AD, UK, d Department of Neurophysiology, St Mary's Hospital

Correspondence to: Dr Walker

Accepted 22 November 1999

The rare inherited disorder hyperprolinaemia type II presents with fits in childhood, usually precipitated by infection. A diagnosis of hyperprolinaemia type II and vitamin B6 deficiency was made in a well nourished child with fits. It is thought that pyridoxine deficiency was implicated in her fits and was the result of inactivation of the vitamin by the proline metabolite, pyrroline-5-carboxylate.


Keywords: pyridoxine; fits; hyperprolinaemia type II; pyrroline-5-carboxylate


© 2000 by Archives of Disease in Childhood

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This article has been cited by other articles:

  • Mitsubuchi, H., Nakamura, K., Matsumoto, S., Endo, F. (2008). Inborn Errors of Proline Metabolism. J. Nutr. 138: 2016S-2020S [Abstract] [Full Text]  
  • VIVEKANANDAN, S (2000). Fits, pyridoxine, and hyperprolinaemia type II. Arch. Dis. Child. 83: 87d-87 [Full Text]  
  • Farrant, R. D., Walker, V., Mills, G. A., Mellor, J. M., Langley, G. J. (2001). Pyridoxal Phosphate De-activation by Pyrroline-5-carboxylic Acid. INCREASED RISK OF VITAMIN B6 DEFICIENCY AND SEIZURES IN HYPERPROLINEMIA TYPE II. J. Biol. Chem. 276: 15107-15116 [Abstract] [Full Text]  

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Fits, pyridoxine and hyperprolinaemia type II
S Vivekanandan
ADC Online, 31 Mar 2000 [Full text]

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