European Journal of Pediatrics

, Volume 148, Issue 4, pp 344–348

Chronic renal failure in methylmalonic acidaemia

Authors

  • J. H. Walter
    • Department of Child HealthInstitute of Child Health
  • A. Michalski
    • Department of Child HealthInstitute of Child Health
  • W. M. Wilson
    • Department of Paediatric NephrologyInstitute of Child Health
  • J. V. Leonard
    • Department of Child HealthInstitute of Child Health
  • T. M. Barratt
    • Department of Paediatric NephrologyInstitute of Child Health
  • M. J. Dillon
    • Department of Paediatric NephrologyInstitute of Child Health
Metabolic Diseases

DOI: 10.1007/BF00444131

Cite this article as:
Walter, J.H., Michalski, A., Wilson, W.M. et al. Eur J Pediatr (1989) 148: 344. doi:10.1007/BF00444131

Abstract

The renal function of 12 patients with non vitamin B12 responsive methylmalonic acidaemia has been investigated. Eight patients had reduced glomerular filtration rates, but the plasma creatinine concentration was only raised in those with values of less than 40ml/min per 1.73m2 surface area. The reduction in glomerular filtration was a function of the age and the severity of the disease. Plasma urate concentrations were increased in four patients but this may be secondary to the renal disease rather than its cause.

Key words

Methylmalonic acidaemiaChronic renal failureTubulo-interstitial nephritis

Abbreviations

51 Cr-EDTA

51-chromium ethylenediamine tetraacetic acid

GFR

glomerular filtration rate

MMA

methylmalonic acidaemia

SA

surface area

TRP

tubular reabsorption of phosphate

Ua/Uc

urinary albumin/creatinine concentration ratio

Ul/Uc

urinary lysozyme/creatinine concentration ratio

Copyright information

© Springer-Verlag 1989