What is the problem of providing a kidney source for kidney transplantation in a family with hereditary nephritis?

  Women in families with hereditary nephritis, especially mothers, are often courageous enough to request a kidney source for a relative kidney transplant for their children with ESRD (mostly sons) because of kinship and its complex psychology, while relatively few fathers are available as donors. Indeed the mother’s day donor kidney may enter ESRD for a shorter period of time and require renal replacement therapy, with a higher reported incidence. In view of this, medical ethics committees, including nephrologists and transplant surgeons, need to provide more scientifically accurate counseling and ethical validation of XLAS families, with specific recommendations such as: 1 careful preoperative family investigation, especially genetic genealogical mapping and characterization of XLAS families to determine the relationship between the donor and recipient family lines 2
Further determination of the diagnosis of XLAS and analysis of the various bases supporting the diagnosis of XLAS, including the pathologic basis of the preexisting patient, analysis of the donor-recipient skin biopsy for type IV collagen molecule a5 chain staining, or/and genotype identification.  3
Renal function tests and urinalysis of the donor, audiometry and fundus and crystalline examination of the eyes, renal function tests should include isotope examination (ECT examination) of the fractional renal function. Urinalysis includes proteinuria and microalbuminuria analysis.  Suspected patients with XLAS or clear mutation gene carriers identified by the above tests should not be used as donor for relative kidney transplantation. Female members over 40 years of age who still do not clinically present with proteinuria and high-frequency hearing loss may be carefully considered as child relative kidney transplant donors, while all other female XLAS patients should not be candidates for relative living donor kidney transplantation. The chance of developing anti-GBM antibodies after kidney transplantation in male XLAS patients is approximately 8-10%, and the possibility of developing anti-GBM nephritis in the transplanted kidney exists, whereas the chance of developing anti-GBM antibodies after kidney transplantation in female patients may be much lower than that in male recipients. In contrast, the rules for pro-kidney transplantation in ARAS or ADAS families can be referred to the rules for normal pro-kidney transplantation.