TY - JOUR
TI - Anterior pituitary function in the prader-labhart-willi (PLW) syndrome
AU - Tolis, G.
AU - Lewis, W.
AU - Verdy, M.
AU - Friesen, H.G.
AU - Solomon, S.
AU - Pagalis, G.
AU - Pavlatos, F.
AU - Fessas, P.
AU - Rochefort, J.G.
JO - JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM
PY - 1974
VL - 39
TODO - 6
SP - 1061-1066
PB - 
SN - 0021-972X
TODO - 10.1210/jcem-39-6-1061
TODO - chorionic gonadotropin;  clomifene;  estradiol;  follitropin;  gonadorelin;  growth hormone;  luteinizing hormone;  prolactin;  protirelin;  radioisotope;  testosterone;  thyrotropin, adenohypophysis;  adrenal cortex;  congenital adrenal hyperplasia;  cryptorchism;  diagnosis;  ear cartilage;  etiology;  hypogonadism;  hypophysis;  hypothalamus;  hypothalamus hypophysis system;  karyotype;  major clinical study;  mental deficiency;  methodology;  micrognathia;  muscle hypotonia;  obesity;  sella turcica;  sexual development;  sexual maturity;  strabismus;  tooth malformation, 17-Ketosteroids;  Adolescent;  Adult;  Carbohydrate Metabolism, Inborn Errors;  Chorionic Gonadotropin;  Clomiphene;  Estradiol;  Female;  Follicle Stimulating Hormone;  Glucose Tolerance Test;  Gonadotropin-Releasing Hormone;  Growth Hormone;  Humans;  Hydrocortisone;  Hypothalamo-Hypophyseal System;  Iodine Radioisotopes;  Luteinizing Hormone;  Male;  Mental Retardation;  Metyrapone;  Obesity;  Pituitary Gland;  Pituitary Gland, Anterior;  Prolactin;  Radioimmunoassay;  Syndrome;  Testosterone;  Tetrahydrocortisol;  Tetrahydrocortisone;  Thyrotropin
TODO - Serum FSH, LH, PRL, TSH, GH, estradiol (E2) and testosterone (T) were measured in 2 male and 3 female patients with the Prader- Labhart-Willi Syndrome. Adrenal and thyroid function was within normal limits, as was GH secretion for 3 of the patients. Serum TSH and hPRL were normal in all patients tested before and after TRH administration. In contrast, LRH administration caused a variable increase in serum FSH, LH, E2 and T with the largest increments observed in patients with more advanced sexual maturation. Clomiphene did not alter serum FSH, LH and T. Following human chorionic gonadotropin (hCG) administration the increase in serum T was variable in the two male patients tested. The above data suggest that there is a considerable degree of variation in the function of the hypothalamic-pituitary- gonadotropin axis in the Prader-Labhart-Willi syndrome. Long-term administration of LRH or clomiphene is the most helpful procedure that can be used to allow a precise evaluation of the functional reserve of the hypothalamic-pituitarygonadotropin axis in such patients. © 1974 by The Endocrine Society.
ER -