Endo

Question 1

Thyroid storm symptoms

Answer 1

Acute onset hyperthermia, tachy, heart failure, restlessness. Rapid progression to delirium, coma and death.
Precipitating events: trauma, pregnancy, infection or surgery

Question 2

Thyroid storm treatment

Answer 2

Start treatment before thyroid function tests return:
D5W for increased metabolic demand
Correct lyte abnormalities
Treat arrhythmias
Treat increased temperature 
Anti adrenergic = propanolol
Correct hyperthyroid state with high dose Propylthiouracil (inhibits conversion of t4 to t3)
Treat precipitating event (eg sepsis)

Question 3

OI triad

Answer 3

Fragile bones, blue sclera, and early deafness
Majority of cases are autosomal dominant, but often spontaneous mutation
Phenotype is very variable

Question 4

OI type 1

Answer 4

Mild, often found in large pedigrees. Paragraph blue sclera, recurrent fractures in childhood and presenile hearing loss [30 to 60%]
A and B subtypes depending on absence [a] or presence [b] of dentinogenesis imperfecta - opalescent teeth that wear quickly
Fractures result from mild to moderate trauma and decrease after puberty

Question 5

OI type 2

Answer 5

Perinatal, lethal

Stillborn or die in the first year of life. Birthweight and link are small for just stational age. Extreme fragility of skeleton and connective tissues. Intrauterine fractures of long bones.
Legs are held abducted at right angles to the body in frog leg position. The skull is large for body size with enlarged fontanelles. Sclera are dark blue gray.

Question 6

OI type 3

Answer 6

Progressive deforming. Most severe non-lethal form of OI results in significant physical disability.
Fractures in utero, relative macrocephaly and triangular facies.
Disorganization of the bone matrix results in popcorn appearance of the metaphyses
Scoliosis, vertebral compression, extreme short stature

Question 7

OI type 4

Answer 7

Moderately severe. May present at birth with in utero fractures or Bowing of lower long bones. Recurrent fractures after ambulation. May have moderate bowing even without fractures. Usually able to attain ambulation skills. Fractures rates decrease after puberty. Osteoporotic phones on radiograph with metaphyseal flaring and vertebral compression. Moderate short stature

Question 8

OI health supervision

Answer 8

• paint evaluation
• acute # management
• strategies for physical activity
• multi-d team
• progressive hearing loss –> audio gram after age 10