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Information

 Bone and Soft-Tissue Disorders

Differences between Various Types of Hypoparathyroidism

HypoPTPseudo HypoPTPseudopseudo HypoPT
Serum Ca
Serum P
AlkaPhosor or
Response to PTH-Injection
Urine cAMP
Urine P
Plasma AMP

Differential Signs between PHypoPT and PPHypoPT

Radiographic SignsPHypoPTPPHypoPT
calcification of basal ganglia44%8%
soft-tissue calcifications55%40%
metacarpal shortening (4 + 5 always involved)75%90%
metatarsal shortening (3 + 4 involved)70%99%

Idiopathic Hypoparathyroidism  !!navigator!!

= rare condition of unknown cause

  • round face, short dwarflike, obese; mental retardation
  • cataracts; dry scaly skin, atrophy of nails
  • dental hypoplasia (delayed tooth eruption, impaction of teeth, supernumerary teeth)

Secondary Hypoparathyroidism  !!navigator!!

= accidental removal / damage to parathyroid glands in thyroid surgery / radical neck dissection (5%); 131I therapy (rare); external beam radiation; hemorrhage; infection; thyroid carcinoma; hemochromatosis (iron deposition)

Pseudohypoparathyroidism  !!navigator!!

= PHypoPT = congenital X-linked dominant abnormality with renal + skeletal resistance to PTH due to

  1. Endorgan resistance
  2. Presence of antienzymes
  3. Defective hormone

May be associated with: hyperparathyroidism hypocalcemia; F >M

  • short obese stature, round face, mental retardation
  • abnormal dentition (hypoplasia, delayed eruption, excessive caries); corneal + lenticular opacity
  • hypocalcemia + hyperphosphatemia (resistant to PTH injection); normal levels of PTH
  • brachydactyly in bones in which epiphysis appears latest: metacarpal, metatarsal bones I, IV, V (75%)
  • accelerated epiphyseal maturation resulting in dwarfism + coxa vara / valga
  • multiple diaphyseal exostoses (occasionally)
  • calcification of basal ganglia + dentate nucleus
  • calcification / ossification of skin + subcutaneous tissue

Pseudopseudohypoparathyroidism  !!navigator!!

= PPHypoPT = different expression of same familial disturbance with identical clinical + radiographic features as pseudohypoparathyroidism but normocalcemic

Cause: end-organ resistance to PTH

  • short stature, round facies
  • NO blood chemical changes = normal calcium + phosphorus
  • normal response to injection of PTH
  • brachydactyly

 Outline