The risk of recurrence of hemolytic uremic syndrome after renal transplantation in children

Abstract

We reviewed the literature to analyze the risk of recurrence of hemolytic uremic syndrome (HUS) after renal transplantation in children. Among 118 children transplanted after post-diarrheal (D+) HUS, 1 (0.8%) had recurrence with graft loss. Among 63 children transplanted after HUS not associated with a prodrome of diarrhea (D-) of unknown mechanism, 13 (21%) had recurrence with graft loss. Of 11 patients with HUS associated with factor H deficiency who were transplanted, 5 lost the graft because of recurrence. Of 7 patients with HUS associated with normal factor H concentration but mutations in factor H gene who were transplanted, probably 2 had recurrence. Three patients with HUS associated with low serum C3, but no factor H deficiency or mutation lost their graft because of recurrence. The risk of recurrence in the autosomal recessive forms of HUS of unknown mechanism is not documented in children, but is around 60% in adults. A similar risk has been reported in the autosomal dominant forms. The only transplant patient with a constitutional deficiency of von Willebrand factor-cleaving protease had recurrence. Further efforts to document the post-transplant course of patients with D- HUS and progress in the understanding of the mechanisms and genetics of the disease are needed to allow more accurate prediction of the recurrence risk and to define therapeutic approaches.

Post‐appendectomy thrombotic thrombocytopenic purpura: A case report and review of the literature

“…Although infrequent, postoperative TTP has been reported after cardiac, vascular, and transplant surgeries [3][4][5][6][7][8][9][10][11]. A National Library of Medicine search identified only eight cases of postoperative TTP after abdominal or general surgical procedures: four cases after bowel resection, two after cholecystectomy, and two post-hysterectomy (Table I) [11][12][13][14][15][16][17].…”

“…Multiple conditions have been associated with TTP including infections, certain medications, pregnancy, and stem cell or solid organ transplantation [2,[3][4][5][6][7][8]. However, in the majority of cases, TTP is considered idiopathic.…”

Hemolytic Uremic Syndrome

“…Indeed, the outcome of renal transplantation is good in children with Stx-HUS: Recurrence rates range from 0 to 10% (85,86), and graft survival at 10 yr is even better than in control children who had other diseases and received a transplant (87).…”

Hemolytic uremic syndrome

“…ADAMTS13 is an enzyme produced by a special kind of cells in the liver. It acts as a von Willebrand factor (VWF) cleaving protease, and its deficiency is associated with the circulation in the plasma of the ultralarge multimeric form of VWF (ULVWF), which binds the platelets, leading to thrombocytopenia and embolisation of small vessels causing tissue ischemia (18,19,23).…”