It is inherited in an X-linked dominant fashion 85% of the time; 15% are autosomal recessive.
Alport's is an abnormality in the gene that codes for type IV collagen.
It is associated with:
- abnormal GBM - 'basket weave' --> chronic renal failure, 30% nephrotic
- sensorineural deafness
- occular defects in 40%
- lenticonus = bulging lens capsule on slit lamp exam
- retinitis pigmentosa
Treatment is treating symptoms.
Onwards...
