Which of the following is/are true about pseudohypoparathyrodism?
A. Heterotopic calcification
B. ↓ed Ca2’ C. ↓edPO
D. ↓ed PTH
E. ↓ed response of urinary cAMP on PT
Ans.
A. Heterotopic calcification ; (D) ↓ed PTH
Pseudohypoparathyridism
is a hereditary disorder characterised by symptoms and signs of hypoparathyroidism, typically in association
with distinctive skeletal and developmental defects. The hypoparathyrodism is
due to deficiency of endorgan response to PTH. Hyperplasia of parathyroids, in response to hormone resistance,
causes elevation of PTH levels. There
are hypocalcemia, hyperphosphaemia,
heterotropic calcfication and skeletal
abnormalities.
— Defects in metatarsals and metacarpals with sometimes short phalanges.
— Typically short 4th and 5th metacarpals and metatarsals bilaterally.
— Exostoses and radius curvtis.
—Impaired olfaction and
taste and unusual dermatoglyphic abnormalities
