Pseudohypoparathyroidism

Labs Imaging Gross Pathology

Micro Pathology Treatment Discussion

Atlas Link

A 15-month-old Eskimo boy is brought to the pediamc clinic by his parents because of delayed dentition, poor growth and development, frequent crying, and weakness.

The infant's diet is deficient in eggs and dairy products, and he spends most of his time indoors (i.e., he has no exposure to sun-

Irritability; poor muscular development and muscle tone; ab- -pj dominal distention; hypotonia of all muscles; anterior fontanelle g open; softening of occipital and parietal bones with elastic re- g coil (craniotabes); frontal bossing; enlargement of costochon- ^

dral junctions (rachitic rosary); bowing of legs; lineal chest depression along diaphragm (Harrison's groove).

Serum calcium normal or slightly low; decreased serum phosphorus; increased alkaline phosphatase; low l,25(OH)2-vitamin D level.

XR: widening of growth plates; osteopenia of cranial and long bones; irregularity and cupping of distal ends of long bones; pseudofractures in metaphysis (looser\s lines).

Excess amount of uncalcified bone at junction of cartilage; bone stretched and pulled out of shape by gravity; increased osteoid seams; osteopenia; frontal bossing of skull; pigeon breast deformity.

Defective mineralization of osteoid in epiphysis and diaphysis. Increase calcium and vitamin D in diet.

Rickets is a disease of infancy and childhood involving defective mineralization of osteoid in bone skeleton and the neuromuscular system because of low vitamin D or calcium in the diet; it can also be due to low sunlight exposure (vitamin D conversion in skin) and chronic renal failure (BUN and phosphorus levels are high).

Lycm-zi MC-308

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