There are two main populations of<unk> ; the larger group is associated with cystic fibrosis and occurs because of a mutation in the CFTR gene, while the smaller group (estimated between 10 and 40%) is associated with unilateral renal agenesis (URA). The mutational spectrum of CFTR in the first group differs from that observed in classic cystic fibrosis, with milder missense or splice variants present on at least one chromosome. The genetic basis of the second group is not well understood. In the subset of males with both CBAVD and URA, the CFTR mutation has been shown to occur at a rate only slightly higher than the overall population. It has been suggested that another gene may be responsible for this condition. Mutation of the CFTR gene is found to result in obstructive azoospermia in postpubertal males with cystic fibrosis. Strikingly,<unk>  is one of the most consistent features of cystic fibrosis as it affects 98-99% of individuals in this CF patient population. In contrast, acute or persistent respiratory symptoms present in only 51% of total CF patients.
Congenital absence of the vas deferens
Recurrent toxin-mediated perineal erythema
Immunodeficiency with hyperimmunoglobulin M
Autoimmune autonomic ganglionopathy
Urethral foreign body
Ejaculatory duct obstruction
Bladder sphincter dyssynergia
Retrograde ejaculation