Successful simultaneous pancreas kidney transplantation in a patient with partial congenital lipodystrophy

UVA P; LEON L; MINUE E; PILOTTI R; CABRERA I; GIUNIPPERO A; GALLO A; OSELLA F; TONIOLO F; CHULUYAN E; CASADEI D

Hong Kong

Congreso; 2016 TTS Congress Hong Kong; 2016

The Transplantation Society - TTS

IntroductionPartial congenital lipodystrophy is a genetic disorder characterized by reduction or absense of adipose tissue with regional lipoatrophy combined with zones of lipohypertrophy and is associated with hypertriglyceridemia, insulin resistance and diabetes mellitus. Patophysiology remains unclear, however, decreased subcutaneous deposits of adipocytes and leptine could lead to hyperphagia. This could produce high caloric intake favouring ectopic fat deposits leading to insulin resistance. This patients could present with high insulin requirements, hypertriglyceridemia, steatosis and cardiomyopathies among others. Leptin replacement could ameliorate insulin resistance and hypertriglyceridemia. We here present a case of partial congenital lipodystrophy with end stage renal disease with a successfull simultaneous pancreas kidney transplant. Case ReportThis is a 41 years-old female with partial congenital lipodystrophy. She was diagnosed with diabetes mellitus at age 15 and was started wih insulin therapy with high insulin requirements (115U/day). Her case was complicated with hypertriglyceridemia, bilateral retinopathy and end stage renal disease due to biopsy proven membranoproliferative glomerulonephitis type 1, on hemodialysis for the past 3 years. Her pre transplant studies revealed a non detectable C-peptide. Leptin analogues were not available in the Country for treatment. On September 7th, 2015 she received a simultaneous pancreas kidney transplant from an ideal donor. Induction therapy included Solumedrol and Antithymocyte Globulin (ATG Fresenius 15mg/kg). Maintenance immunosuppression included tacrolimus, sodium mycophenolate, steroids and belatacept. Belatacept was included to use lower tacrolimus levels. Both grafts function immediatly and required no postoperative insulin therapy and no hemodialysis. Her postoperative course was complicated by an intestinal obstruction leading to an intestinal anastomotic leak requiring conversion to a new Roux en Y duodenojejunostomy. She then presented a low output pancreatic fistula that closed with no other complications. Currently, at 1 year postransplant she has normal pancreatic and kidney function tests with a fructosamine of 237umol/L, HbA1c of 4.6, creatinine of 0.8mg/dL and no proteinuria. Tacrolimus level is kept at 6-8 ng/mL. Protocol biopsy has recently showed normal grafts.Current Leptine level is 2.5ng/dL.Conclusion: This is to our knowledge the first report of a successful SPK transplant in a patient with Congenital Partial Lipodystrophy