Endocrine dysfunction in the diencephalic syndrome of emaciation in infancy.
A 10-month-old boy with the clinical features of the diencephalic syndrome of emaciation due to a suprasellar spongioblastoma is described. The patient showed high basal levels of growth hormone (GH greater than 80 muU/ml on several occasions). In addition, elevated concentration of plasma testosterone (125.5 ng/100ml) was combined with a relatively high LH-increase to LHRH (45.6 mU/ml). After completion of irradiation basal GH-levels had been normalized, and GH responses to insulin induced hypoglycemia (IIH) and propranolol-glucagon (PG) were adequate. Complete clinical remission of emaciation occurred soon after radiation therapy and went parallel with the normalization of GH-regulation.
D001254:Astrocytoma / Q000503:physiopathology*; D001786:Blood Glucose / Q000032:analysis; D001932:Brain Neoplasms / Q000097:blood / Q000503:physiopathology*; D004614:Emaciation / Q000097:blood / Q000503:physiopathology* / Q000628:therapy; D005640:Follicle Stimulating Hormone / Q000097:blood; D013006:Growth Hormone / Q000097:blood; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007031:Hypothalamus / Q000503:physiopathology*; D007223:Infant; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D010903:Pituitary Gland, Anterior / Q000503:physiopathology; D011388:Prolactin / Q000097:blood; D013577:Syndrome; D013739:Testosterone / Q000097:blood; D013963:Thyroid Hormones / Q000097:blood
D001786:Blood Glucose; D013963:Thyroid Hormones; D013739:Testosterone; D011388:Prolactin; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D013006:Growth Hormone; D006854:Hydrocortisone