Preemptive Kidney Transplantation in a Pediatric Patient With Factor
XI
Deficiency: A Case Report
Ghina Sadek, Chakib Khoury, Farah Ghobar, Edmond Abboud, Maroun Abou Jaoude ABSTRACT
Background
Hemophilia C, or factor XI deficiency (FXID), is a rare bleeding disorder with variable clinical manifestations that correlate poorly with factor levels. These characteristics present unique challenges in major surgical procedures, such as kidney transplantation (KT), especially in perioperative management. Reports describing the coexistence of FXID and KT are exceedingly scarce, with only one adult case documented to date.
Methods
We describe a 14‐year‐old male, who underwent preemptive living donor heterotopic KT from his brother for end‐stage renal disease secondary to congenital posterior urethral valves. Despite factor XI activity of 10% and a markedly prolonged activated partial thromboplastin time, the patient had no prior bleeding history. A multidisciplinary perioperative plan incorporated two units of fresh frozen plasma (FFP) without the use of antifibrinolytic agents.
Results
The surgery proceeded uneventfully, with minimal blood loss, immediate graft function, and normalization of coagulation parameters postoperatively with gradual return to baseline. Renal profile remained stable throughout follow‐up.
Conclusions
This case represents the first reported pediatric preemptive KT in a patient with FXID and demonstrates that successful transplantation can be achieved with individualized care and minimal transfusion requirements. FXID should not be considered a contraindication to KT. Larger studies and multicenter collaborations are needed to establish evidence‐based guidelines.