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. Author manuscript; available in PMC: 2018 Nov 29.
Published in final edited form as: Endocr Relat Cancer. 2015 Sep 25;23(1):R1–14. doi: 10.1530/ERC-15-0171

Table 1.

Some clinical features of hereditary syndromes of primary hyperplasia with hormone excess (See also Table 2).

Syndrome Normal serum stimulus of mutant sensor Hormone over-secreted Typical early age of onset of hormone excess Selected comments about expressions





NSHPT# Low Ca++ PTH In fetus Severe defects at birth reflect onset by fetal over-secretion of PTH
CNT TSH T4, T3 In fetus Severe defects at birth reflect onset by fetal over-secretion of iodo-thyronines
FMPP LH Testosterone 1-3 yr Not expressed in female carriers of the mutation
OHSS CG Estrogens, progestins, cytokines Pregnant female CG from the normal placenta stimulates the mutant FSH receptors in the corpus luteum of pregnancy
HAIIIA K+ Aldosterone 1 yr Hyperplasia is in the adrenal fasciculata with atrophy in the adrenal glomerulosa
#

Abbreviations: Congenital neonatal thyrotoxicosis CNT; Neonatal severe primary hyperparathyroidism NSHPT; hyperaldosteronism type III HAIII; Familial male-limited precocious puberty FMPP; Ovarian hyperstimulation syndrome OHSS