Long-term nephrotic syndrome recurrence risk of kidney transplantation in two siblings with ADCK4-associated glomerulopathy.

Abstract

BACKGROUND\nMutations in the ADCK4\xa0gene cause steroid-resistant nephrotic syndrome (NS), namely ADCK4-associated glomerulopathy. Reportedly, 38.5% of patients with ADCK4-associated glomerulopathy had end-stage renal disease (ESRD) at the time of diagnosis of ADCK4-associated glomerulopathy, requiring renal replacement therapy, such as dialysis and kidney transplantation. However, long-term NS recurrence risk of kidney transplantation in patients with ADCK4-associated glomerulopathy is unknown.\n\n\nMETHODS\nThe clinical data and mutations in ADCK4 of two siblings with steroid-resistant NS were collected. The long-term prognosis of the two siblings who received kidney transplantation was evaluated.\n\n\nRESULTS\nWe describe two siblings with ADCK4-associated glomerulopathy who received deceased donor kidney transplantation and showed no clinical evidence of recurrence of NS during more than 10\xa0years of follow-up.\n\n\nCONCLUSIONS\nThis suggests that long-term NS recurrence risk of kidney transplantation is low in patients with ADCK4-associated glomerulopathy who progress to ESRD.