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Growth and endocrine changes in the hepatic glycogenoses.

Dunger, DB; Holder, AT; Leonard, JV; Okae, J; Preece, MA; (1982) Growth and endocrine changes in the hepatic glycogenoses. Eur J Pediatr , 138 (3) pp. 226-230.

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Abstract

The biochemical and endocrine responses of 13 patients with hepatic glycogen storage disease (HGSD) (type I-six patients, type Ib-two, type III-three, type IX-two patients) to an oral glucose load have been investigated. Longitudinal growth data was available in all patients. The height velocity standard deviation score (HVSDS) was positively correlated with the plasma somatomedin and inversely correlated with the glucose-insulin ratio, plasma cortisol and plasma growth hormone concentrations. There was no correlation between plasma glucagon and HVSDS with treatment was accompanied by a rise in plasma somatomedin and a fall in growth hormone and cortisol. In two patients the glucose-insulin ratio decreased. Growth retardation in HGSD can be explained as part of the adaptation to the inability to maintain normal glucose homeostasis.

Type: Article
Title: Growth and endocrine changes in the hepatic glycogenoses.
Location: Germany
Keywords: Adolescent, Blood Glucose, Body Height, Child, Female, Glucagon, Glycogen Storage Disease, Glycogen Storage Disease Type I, Glycogen Storage Disease Type III, Growth, Growth Hormone, Humans, Hydrocortisone, Infant, Insulin, Male, Somatomedins
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health > ICH Infect, Imm, Infla. and Physio Med
URI: http://discovery.ucl.ac.uk/id/eprint/1371426
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