Surgery
Surgery such as the "Nesbit operation", which is named after Reed M. Nesbit (1898–1979), an American urologist at University of Michigan), is considered a last resort and should only be performed by highly skilled urological surgeons knowledgeable in specialized corrective surgical techniques. A penile implant may be appropriate in advanced cases. Epidemiology
It is estimated to affect between .4 to 20% of men. The condition becomes more common with age. The mean age at onset of disease is 55–60 years although many cases have been recorded in adolescence and early 20’s.The overall prevalence of PD is about .4 to 20 percent in men. Rates range from 3.2 percent in a community-based survey of 4432 men (mean age of sample 57.4) to 16 percent among 488 men undergoing evaluation for erectile dysfunction (mean age 52.8). The prevalence of PD among the 4432 men in the community based study who responded by self report positively for palpable plaque, newly occurring angulation or curvature and painful erection was 1.5 percent between the ages of 30 and 39, 3 percent between 40 and 49, 3 percent between 50 and 59, 4 percent between 60 and 69, and 6.5 percent over 70. In 534 men undergoing routine prostate screening for cancer detection (without a specific urologic complaint), the prevalence of PD was 8.9 percent. In this study, the mean age of those with PD was 68.2 years compared with 61.8 years of those without PD.
Bleeding is more frequent and severe in women of child-bearing age; these women may suffer miscarriages, menometrorrhagia, and excessive bleeding during child birth and/or the postpartum period. Excessive bleeding following major or minor surgery, including dental extractions, occurs in both females and males with the disorder. Thrombotic complications of the disorder are often (~50%) recurrent and can involve central and peripheral arteries, deep and superficial veins. Thrombotic events may be serious and involve occlusion of a cerebral artery leading to stroke, splanchnic venous thrombosis, and pulmonary thrombosis presumptively secondary to deep vein thrombosis. Fibrinogen
Circulating fibrinogen is a glycoprotein made of two trimers each of which is composed of three polypeptide chains, Aα (also termed α) encoded by the FGA gene, Bβ (also termed β) encoded by the FGB gene, and γ encoded by the FGG gene. All three genes are located on the long or "q" arm of human chromosome 4 (at positions 4q31.3, 4q31.3, and 4q32.1, respectively) and are the sites where mutations occur that code for a dysfunctional fibrinogen and/or reduced fibrinogen levels which are the cause of congenital hypodysfibrinogenemia. Pathophysiology
Congenital hypodysfibrinogenemia is inherited as an autosomal dominant disorder caused by at least 32 different types of single mutations. Ten of these mutations are in the fibrinogen alpha chain gene (also termed the FGA gene), 5 in the fibrinogen beta chain gene (also termed the FGB gene), and 17 in the fibrinogen gamma chain gene (also termed the FGG gen).