Diagnosis: Idiopathic focal segmental glomerulosclerosis post kidney transplant
Treatment: Velcade
The treatment with Velcade is medically necessary.

In treating children with this complex rejection presentation, commitment to prolong and rescue this first kidney allograft using the therapies available to control the process and resolve if it is possible must be made. Most of the immunosuppressive medications do not have absolute FDA indication for immunosuppression in organ transplantation. The use of these medications is based on clinical retrospective trials, small series, and most importantly, the need to solve complex immunological phenomena by learning from other disciplines such as Pediatric Oncology and others.

Previous review labeled the use of Bortezomib as unproven, with no RCT (randomized controlled trials) for the evaluation of the efficacy of Bortezomib for the treatment of antibody mediated rejection. This child has undergone the standard therapy for antibody mediated rejection and has had no evidence of improvement. The use of Bortezomib for the treatment of antibody mediated rejection in children has been done and published in small series demonstrating that Bortezomib stabilizes the immunological process and may preserve renal function in the majority of children treated.

This child has a very high possibility of losing her graft with the levels of antibodies and specially after failing to respond to standard therapy. Bortezomib is the last option to attempt to preserve renal function, by eliminating plasma cells that are the producers of antibodies. This kidney graft does not have significant chronic injury.

In this reviewer's opinion, and supported by literature in pediatric kidney transplant patients, Bortezomib is justified for the treatment of non-responding acute antibody mediated rejection on this child's care. This child needs adequate renal function in the next years to accomplish adequate growth and development. Graft failure and dialysis will be a devastating complication. This is a very difficult disorder in pediatric organ transplantation, lacking good agents to treat patients with high levels of antibodies injuring the graft effectively. In patients like this, it takes a full combination of all agents available to attempt to provide control and possible resolution of this disorder.