The demonstration of insulinopenia in familial dysautonomia

• Published in: Pediatrics (Evanston) Vol. 52; no. 1; pp. 137 - 139
• Main Author: Cole, H S
• Format: Journal Article
• Language: English
• Published: United States 01-07-1973
• Subjects: Adolescent; Blood Glucose - analysis; Child; Child, Preschool; Dysautonomia, Familial - blood; Dysautonomia, Familial - genetics; Glucose Tolerance Test; Growth Disorders - blood; Growth Disorders - etiology; Growth Hormone - blood; Humans; Insulin - blood; Jews
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.52.1.137

Children with familial dysautonomia have a marked retardation in height and weight. Nine children with familial dysautonomia and seven control children were studied by means of rapid IVGTTs. The KG and serum levels in insulin and GH were obtained to determine the existence of abnormalities in any of these parameters which might be responsible for growth failure.
There were no significant differences in the KG or GH values between the groups. The insulin values of the dysautonomic children were lower than those of the control children at all the sampling times during the 1-hour test and were statistically significantly lower when the total insulin areas were compared.
These lower levels of serum insulin growth might reduce its effectiveness as an anabolic agent and result in the growth failure of dysautonomic children.