Type : Other, mainly phenotypes with suspected mendelian basis;
Alternative titles and symbols : Pseudocorpus luteum insufficiency;
Description : Progesterone prepares the endometrium for blastocyst implantation and allows maintenance
of pregnancy. The major sources of progesterone are the corpus luteum during the second
half of the menstrual cycle and at the beginning of pregnancy, and the placenta. The
main hormones responsible for stimulation of progesterone secretion are luteinizing
hormone (LH) for the corpus luteum of the menstrual cycle and chorionic gonadotropin
for the corpus luteum of pregnancy. Complete end-organ resistance to progesterone
would be incompatible with reproductive competence in females. Males would not be
expected to be affected since progesterone has no known function in men. Failure of
the uterus to respond to progesterone would lead to the development of a 'constantly
proliferative' endometrium incompatible with blastocyst implantation. Partial resistance
to progesterone, on the other hand, would be expected to be associated with various
degrees of incomplete maturation of the endometrium, perhaps expressed clinically
as infertility or early abortions. The syndrome would present with the clinical and
histologic picture of a luteal phase defect in which the life span of the corpus luteum
and the plasma progesterone concentrations would be normal or elevated.;