Deficiency of non-suppressible insulin-like activity in thalassaemia major. 1981

G A Werther, and R Matthews, and H G Burger, and A C Herington

The commonly occurring short stature in the condition of thalassaemia major was investigated with respect to the possible role of the somatomedin growth factor low molecular weight non-suppressible insulin-like activity (NSILAs). Nineteen affected patients (12 boys, 7 girls) aged between 2 and 21 years were studied. Twelve of them were on or below the 10th centile for height of whom 7 were on or below the 3rd centile for height. Serum immunoreactive growth hormone responses to exercise were normal in 9 of 11 subjects tested. Using an isolated fat cell bioassay NSILAs was undetectable in 10 and was more than 2 SD below the normal mean value in the other 9 subjects. High molecular weight NSILA (not a growth factor) was very low or undetectable in all 9 subjects tested. Low molecular weight NSILAs did not show the normal correlation with age in childhood, nor was there any correlation with height, height velocity, or bone age. The 2 children above the 50th centile for height had undetectable NSILAs. There was no evidence of iron or ferritin interfering in the bioassay, and mixing experiments showed no evidence of inhibitory activity towards NSILAs in thalassaemic sera. Low circulating levels of the somatomedin NSILAs may contribute to the short stature in thalassaemia major, but other factors may permit normal growth in some affected children.

UI MeSH Term Description Entries
D008297 Male Males
D008970 Molecular Weight The sum of the weight of all the atoms in a molecule. Molecular Weights,Weight, Molecular,Weights, Molecular
D009632 Nonsuppressible Insulin-Like Activity A blood protein (NSILA) which mimics the biological activity of insulin in serum, but is not suppressed by insulin antibodies. During acid-ethanol extraction of Cohn fraction III, 10% of the activity is found in the supernatant (NSILA-S) and the remaining activity in the precipitate (NSILA-P). The latter is a large molecular compound, much less stable than the soluble fraction. NSILA-S is a more potent growth factor than insulin and exhibits sulfation activity. NSILA,NSILA-P,NSILA-S,Nonsuppressible Insulinlike Activity,Activity, Nonsuppressible Insulin-Like,Activity, Nonsuppressible Insulinlike,Insulin-Like Activity, Nonsuppressible,Insulinlike Activity, Nonsuppressible,Nonsuppressible Insulin Like Activity
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D006130 Growth Disorders Deviations from the average values for a specific age and sex in any or all of the following: height, weight, skeletal proportions, osseous development, or maturation of features. Included here are both acceleration and retardation of growth. Stunted Growth,Stunting,Disorder, Growth,Growth Disorder,Growth, Stunted,Stuntings
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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