Living-Donor Kidney Transplantation Between Mother and Son With Clinically Confirmed Hereditary Angioedema.

BACKGROUND

Hereditary angioedema (HAE) is a rare autosomal dominant disorder resulting from SERPING1-related C1-inhibitor deficiency. Surgical stress and airway manipulation may precipitate life-threatening attacks. Experience with kidney transplantation in patients with HAE remains extremely limited.

CASE REPORT: We describe a 19-year-old male with end-stage kidney disease secondary to congenital obstructive uropathy and nephrolithiasis, who underwent successful living-donor kidney transplantation from his mother, also diagnosed with HAE type I. Because danazol is unavailable in Argentina, both donor and recipient received compounded stanozolol 2 mg daily, starting 5 days preoperatively and continuing for 5 days postoperatively. Fresh frozen plasma (FFP, 10 mL/kg) was administered before extubation to raise C1-inhibitor levels, and icatibant was kept on standby for rescue. Neither donor nor recipient experienced perioperative angioedema. Both had uneventful recoveries, with the recipient maintaining stable graft function over 10 months of follow-up (latest serum creatinine 1.4 mg/dL).

CONCLUSIONS

This report demonstrates that living-donor kidney transplantation between two individuals affected by HAE can be performed safely with individualized prophylaxis and multidisciplinary coordination.