Related Experiment Video
Updated: May 13, 2026

Comparative Analysis of Human Growth Hormone in Serum Using SPRi, Nano-SPRi and ELISA Assays
Published on: January 7, 2016
Growth hormone therapy is safe and effective in patients with lysinuric protein intolerance
Harri Niinikoski1, Risto Lapatto, Matti Nuutinen
1Department of Pediatrics, University of Turku, Kiinamyllynkatu 4-8, Turku, Finland, harri.niinikoski@tyks.fi.
Background:
Lysinuric protein intolerance (LPI) is an autosomal recessive cationic amino acid transport defect characterized by episodes of postprandial hyperammonemias and spontaneous protein aversion. Subnormal growth is common in spite of appropriate nutritional therapy. Growth hormone (GH) therapy promotes appetite, protein synthesis and accretion, but its possible growth-promoting effects and safety in patients with LPI are poorly known.
Methods:
Four LPI children aged 7-16 years were treated with GH for a period of 3-4.5 years. Dietary intakes and plasma amino acid levels were analyzed frequently in addition to routine monitoring of GH therapy.
Results:
Insulin-like growth factor-1 concentration was low and bone age was delayed in all LPI patients, but GH provocative test was pathological in only one of the patients. During the 3-4.5 years of GH therapy (dose 0.035-0.050 mg/kg/day), bone age did not catch up but height standard deviation score (SDS) improved by 0.7-1.8 SDS. There were no episodes of hyperammonemias.
Conclusions:
Our data support safety and growth-promoting potential of long-term GH therapy in patients with LPI.
Related Concept Videos
Hypoglycemia and Glucagon
Glucagon-like Receptor Agonists
GLP-1, when administered in high doses intravenously, triggers insulin secretion, inhibits glucagon release, slows gastric emptying, reduces food intake, and restores normal insulin secretion. However, its rapid inactivation by the...
Lysosomal Hydrolases
Gene Therapy
Overview of Protein Metabolism
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
Insulin: Dosing Regimen and Adverse Effects
The basal dose constitutes about 40%-50% of the total daily dose, with the rest as premeal insulin. The mealtime insulin dose should mirror...
