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General Reference ⬇

Nejm 1970;282:57; 1969;280:405

Pathophys and Cause ⬆ ⬇

Cause:All genetic, except occasionally due to amyloid (Nejm 1979;301:1050; 1979;304:827); all autosomal recessive

Pathophys:(Screening Tests of Clotting Function)

Epidemiology ⬆ ⬇

All very rare, <1/million

Signs and Symptoms ⬆ ⬇

Sx:

I and VII: no or little bleeding

II: mild bleeding, esp postsurgery

V and X: mild bleeding but hemarthroses

XIII: severe bleeding, hemarthroses, poor wound healing

Complications ⬆ ⬇

With II deficiency; r/o SLE anti-II antibodies

Lab and Xray ⬆ ⬇

Lab: Hem:Clotting w/u (Screening Tests of Clotting Function)

Treatment ⬆

Rx: For II, Konyne (Classic Hemophilia (Hemophilia A))