In:
Hormone Research in Paediatrics, S. Karger AG, Vol. 78, No. 3 ( 2012), p. 193-200
Abstract:
〈 b 〉 〈 i 〉 Background: 〈 /i 〉 〈 /b 〉 Laron syndrome is caused by a mutation in the growth hormone (GH) receptor and manifests as insulin-like growth factor-I (IGF-I) deficiency, severe short stature, and early hypoglycemia. We report a case with postprandial hyperglycemia, an abnormality not reported previously. Postprandial hyperglycemia was due to chronic IGF-I deficiency, and was reversed by IGF-I replacement therapy. 〈 b 〉 〈 i 〉 Methods: 〈 /i 〉 〈 /b 〉 A Moroccan girl referred for short stature at 7 years and 8 months of age had dwarfism [height, 78 cm (–9 SDs); weight, 10 kg (–4 SDs)] , hypoglycemia, and truncal obesity. Her serum IGF-I level was very low, and her baseline serum GH level was elevated to 47 mIU/l. Molecular analysis showed a homozygous mutation in the GH receptor gene. 〈 b 〉 〈 i 〉 Results: 〈 /i 〉 〈 /b 〉 Continuous glucose monitoring before treatment showed asymptomatic hypoglycemia with postprandial hyperglycemia (2.5 g/l, 13.75 mmol/l). Treatment with recombinant human IGF-I (mecasermin, Increlex®) was started. The blood glucose profile improved with 0.04 µg/kg/day and returned to normal with 0.12 µg/kg/day. 〈 b 〉 〈 i 〉 Conclusion: 〈 /i 〉 〈 /b 〉 Postprandial hyperglycemia is a metabolic consequence of chronic IGF-I deficiency. The beneficial effect of IGF-I replacement therapy may be ascribable to improved postprandial transfer of glucose.
Type of Medium:
Online Resource
ISSN:
1663-2818
,
1663-2826
Language:
English
Publisher:
S. Karger AG
Publication Date:
2012
detail.hit.zdb_id:
2540224-9
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