Multihormonal resistance to parathyroid hormone, thyroid stimulating hormone, and other hormonal and neurosensory stimuli in patients with pseudohypoparathyroidism.
In patients with pseudohypoparathyroidism, hormonal resistance first affects parathyroid hormone (PTH), which leads to calcipenia, a decrease in renal vitamin D activation, and a tendency to bone receptor remodeling. However, because G proteins are ubiquitously distributed, multiple hormonal resistance occurs in pseudohypoparathyroidism type Ia and type Ic, impairing responses to other calciotropic hormones (PTHrP, calcitonin), TSH, and also pituitary and hypothalamic hormones, and to neurosensory stimuli. The diversity of multihormonal resistance contributes to the various phenotypes of the disease. Some clinical discomfort and medical consequences of the disease can be treated or prevented with hormone supplementation or modulation.
Wémeau
Jean-Louis
JL
Clinique Endocrinologique Marc Linquette, USNA, CHRU, Lille, France. jl-wemeau@chru-lille.fr
Balavoine
Anne-Sophie
AS
Ladsous
Miriam
M
Velayoudom-Cephise
Fritz-Line
FL
Vlaeminck-Guillem
Virginie
V
D006309:Hearing; D006728:Hormones / Q000502:physiology*; D006801:Humans; D010281:Parathyroid Hormone / Q000502:physiology; D044162:Parathyroid Hormone-Related Protein / Q000378:metabolism; D010641:Phenotype; D011547:Pseudohypoparathyroidism / Q000145:classification / Q000378:metabolism* / Q000503:physiopathology; D044169:Receptors, Calcium-Sensing / Q000378:metabolism; D012684:Sensory Thresholds / Q000502:physiology*; D012903:Smell; D013649:Taste; D013972:Thyrotropin / Q000502:physiology; D014785:Vision, Ocular
D006728:Hormones; D010281:Parathyroid Hormone; D044162:Parathyroid Hormone-Related Protein; D044169:Receptors, Calcium-Sensing; D013972:Thyrotropin