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간행물 검색
Eventful yet Successful Renal Transplantation in a case of TNF Receptor Associated Periodic Syndrome
Aviral Kashyap
2024 ; 2024(1):
논문분류 :
춘계학술대회 초록집
Tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS) is a rare and potentially florid autoinflammatory disorder which is seldom diagnosed . It may cause a multi system involvement and lead to secondary amyloidosis with renal failure . There is very little awareness of this condition and literature on renal failure and transplantation in individuals affected by this condition is scarce . We report a case of familial TRAPS with secondary amyloidosis and renal failure . The patient , suffering from TRAPS since infancy with numerous flare ups of the condition had developed ckdChronic Kidney Disease stage 5 and was on Haemodialysis for a year .He was having multiple family members suffering from the same condition and we had limited options for transplantation.A live donor transplantation with his grandmother as the donor was planned .Induction with Anti- Thymocyte Globulin was done .After an uneventful initial course,the patient developed acute rejection , which was successfully managed with plasmapheresis and ivig.Graft function stabilized and patient is now doing well on regular follow up.Long term follow up will be required to see the graft function in this patient .
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