Abstract
Growth hormone (GH) insufficiency was demonstrated in a patient with methylmalonic aciduria. GH administration at 14–21U/m2 per week accelerated linear growth, stimulated lipolysis, and produced clinical improvement and reduced urinary methylmalonate excretion. The clinical and metabolic benefits were lost as the dose of GH was increased to 28U/m2 per week.
Conclusion
The use of GH in other patients with methylmalonic aciduria (and other disorders of intermediary metabolism demonstrating similar clinical and metabolic features) warrants further study.
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Abbreviations
- GH :
-
growth hormone
References
Bain MD, Borriello SP, Tracey BM, James M, Reed PJ, Chalmers RA, Stacey TE (1988) Contribution of gut bacterial metabolism to human metabolic disease. Lancet 1: 1078–1079
Chalmers RA (1994) Disorders of organic acid metabolism. In: Holton JB (ed) The inherited metabolic diseases. Churchill Livingston, Edinburgh, pp 115–204
Chalmers RA, Lawson AM (1982) Organic acids in man. Chapman and Hall, London
Harel Z, Tannenbaum GS (1993) Dietary protein restriction impairs both spontaneous and growth-hormone-releasing factor-stimulated growth hormone release in the rat. Endocrinology 133: 1035–1043
Rees L, Rigden SPA, Ward G, Preece MA (1990) Treatment of short stature in renal disease with recombinant human growth hormone. Arch Dis Child 65: 56–860
Sousa C de, Piesowicz AT, Brett EM, Leonard JV (1989) Focal changes in the globi pallidi associated with neurological dysfunction in methylmalonic acidaemia. Neuropediatrics 20: 199–201
Tanner JM, Whitehouse RH, Cameron N, Marshall WA, Healy MJR, Goldstein H (1985) Assessment of skeletal maturity and prediction of adult height. 2nd edn. Academic Press, London
Voerman HJ, Strack van Schijndel RJM, Groeneveld ABJ, deBoer H, Nauta JP, Veen EA van der, Thijs LG (1992) Effects of recombinant human growth hormone in patients with severe sepsis. Ann Surg 216: 648–655
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Bain, M.D., Nussey, S.S., Jones, M. et al. Use of human somatotrophin in the treatment of a patient with methylmalonic aciduria. Eur J Pediatr 154, 850–852 (1995). https://doi.org/10.1007/BF01959796
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DOI: https://doi.org/10.1007/BF01959796