Living donor kidney transplantation from relatives with mild urinary abnormalities in Alport syndrome: long-term risk, benefit and outcome.
BACKGROUND
Alport syndrome is a hereditary nephropathy leading to renal failure during adolescence. This study evaluates the outcome of living donor transplantation (Tx) from heterozygous mothers to their affected children.


METHODS
Seven mothers were evaluated, and donation was refused in one because of proteinuria.


RESULTS
All of the remaining six donors had microhaematuria, and one had proteinuria. Renal function was monitored after Tx (average 6.7 years in donors and 5.3 years in acceptors). Three of six donors developed new onset hypertension, and two new onset of proteinuria. Renal function declined significantly in four donors: (1) -35% after 2 years; (2) -25% after 3 years; (3) -30% after 4 years and (4) -60% after 14 years versus before Tx. However, creatinine clearance remained >40 ml/min in all donors. All transplanted kidneys worked well 1 and 5 years after Tx, and one failed after 10 years. One patient died from meningitis, and the remaining four remained stable.


CONCLUSIONS
Living donor Tx from relatives in Alport families is an ambivalent option. Proteinuria should be an exclusion criterion. Yet, even in donors with isolated microhaematuria, families and their physicians should be aware of an increased risk of renal failure in donor and recipient. This risk might be minimized by careful donor evaluation including biopsy and nephroprotective strategies after Tx in both donor and recipient.
Gross
Oliver
O
Department of Nephrology and Rheumatology, University of Goettingen, Robert-Koch Str. 40, D-37075 Goettingen, Germany. gross.oliver@med.uni-goettingen.de
Weber
Manfred
M
Fries
Jochen W U
JW
Müller
Gerhard-Anton
GA
D000293:Adolescent; D000328:Adult; D001706:Biopsy; D005190:Family; D005260:Female; D005500:Follow-Up Studies; D006417:Hematuria / Q000150:complications*; D006579:Heterozygote; D006801:Humans; D007668:Kidney / Q000473:pathology; D016030:Kidney Transplantation; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D009034:Mother-Child Relations; D009394:Nephritis, Hereditary / Q000235:genetics* / Q000601:surgery*; D051437:Renal Insufficiency / Q000453:epidemiology*; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult