No abstract available.

No abstract available.
Hypercholesterolemia* ; Peritoneal Dialysis, Continuous Ambulatory* ; Risk Factors*

Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by the formation of hamartomas in multiple organs. TSC can show lesions including facial angiofibroma, shagreen patch on the skin, cortical tuber, subependymal nodule, astrocytoma in the brain, cardiac rhabdomyoma, and renal angiomyolipoma. In particular, renal angiomyolipoma may be a cause of end-stage renal disease (ESRD). On the other hand, sirolimus has regulatory effects on cellular growth and proliferation via its inhibitory effect on a protein, mammalian target of rapamycin. We report on a case of an 18-year-old male who underwent renal transplantation due to ESRD induced by TSC. Sirolimus played a role in successful treatment of TSC and effective immunosuppression for transplantation.
Adolescent ; Angiofibroma ; Angiomyolipoma ; Astrocytoma ; Brain ; Hamartoma ; Hand ; Humans ; Immunosuppression ; Kidney Failure, Chronic* ; Kidney Transplantation ; Male ; Rhabdomyoma ; Sirolimus* ; Skin ; Tuberous Sclerosis*

Focal segmental glomerular sclerosis (FSGS) accounts for recurrence in 20% to 40% of the renal allografts after transplantation, and it causes graft loss in 13% to 20% of the cases. We report here on successfully treating acute cellular rejection (ACR) combined with FSGS after a kidney transplantation with a combination treatment of plasmapheresis, rituximab and steroid pulse therapy. A 53-year-old female patient whose primary kidney disease was unknown developed massive proteinuria after living donor kidney transplantation. A urine protein/creatinine ratio of 13.42 and an elevated serum creatinine level was detected on postoperative days (POD) 10 and a renal biopsy showed acute cellular rejection (Banff IIb) combined with FSGS. We started steroid pulse therapy on POD 11. She underwent 5 plasmapheresis sessions in the first 3 week after transplantation and she received one dose of rituximab (375 mg/m2) on POD 12. The proteinuria decreased below the nephrotic range at POD 20 and the serum creatinine level was normalized. Three months later, the proteinuria was at 35 mg/day with stable graft function. Rituximab and plasmapheresis is a possible option to treat FSGS combined with a relapse of proteinuria after renal transplantation.
Antibodies, Monoclonal, Murine-Derived ; Biopsy ; Creatinine ; Female ; Humans ; Kidney Diseases ; Kidney Transplantation ; Living Donors ; Middle Aged ; Plasmapheresis ; Proteinuria ; Recurrence ; Rejection (Psychology) ; Rituximab ; Sclerosis ; Transplantation, Homologous ; Transplants

BACKGROUND/AIMS: Experimental studies using porcine non- heart beating donors to ameliorate graft injuries in liver transplantation has been conducted. Recently, it has been reported that cellular calcium may have an important role in ischemic injury, which consists of damage during ischemia and impairment at the time of reperfusion. therefore, it is possible that calcium channel blocker might prevent warm ischemic injury of the graft in liver transplantation when administered to the donor before harvesting and to the recipient at reperfusion. the purpose of this study was to investigate the protective effect of a calcium channel blocker diltiazem (DTZ) on hepatic ischemic injury using a porcine model. METHODS: Twenty pigs weighing 20 to 30 kg were enrolled in this study. Cardiac death was induced by direct cardiac injection of potassium chloride. The perfusion of UW (University of Wisconsin) solution started after 30 min of cardiac arrest. Orthotopic liver transplantation was perforated. Group A (experimental group) was administrated of DTZ at a dose of 70microgram/kg bolus iv injection before hepatic ischemia, perfused of 70microgram/L in UW solution and thereafter infused continuously 70microgram/L in 5% dextrose solution. RESULTS: Two ones death occurred among the ten transplant pigs. 24 hour survival rates were 80%. DTZ administrated group showed the hepatic blood flow and arterial ketone body ratio better compared with untreated controls (p<0.05). In addition, the increase of plasma lactate level was suppressed after ischemia (p<0.05). CONCLUSION: Our results suggest that DTZ has a protective effect on ischemic induced hepatic damage and might be useful in the prevention of primary graft failure caused by warm ischemia in liver transplantation.
Calcium ; Calcium Channels ; Death ; Diltiazem ; Glucose ; Heart ; Heart Arrest ; Humans ; Ischemia ; Lactic Acid ; Liver Transplantation* ; Liver* ; Perfusion ; Plasma ; Potassium Chloride ; Reperfusion ; Survival Rate ; Swine ; Tissue Donors* ; Transplants ; Warm Ischemia

Acute antibody-mediated rejection (AMR) developing simultaneously with acute cellular rejection has been rarely reported as a long-term complication of renal transplantation, and it can present on top of another chronic pathology affecting the graft. A 51-year-old female patient with chronic kidney disease of unknown etiology received renal transplantation 12 years ago from a living unrelated donor with 3 HLA mismatches. She received induction therapy with methylprednisolone and was maintained on steroids, mycophenolate mofetil and cyclosporine A (CsA). For a period of twelve years post-transplantation, she was clinically and biochemically stable. She presented with a rise in serum creatinine (SCr.) from 1.3 mg/dL to 2.4 mg/dL but did not have proteinuria. Graft biopsy revealed findings suggestive of acute cellular rejection on top of antibody-mediated rejection (type II) and chronic calcineurin inhibitor toxicity. Panel reactive antibody (PRA) test levels were 3.6%, 91.7% for class I and II respectively. The patient was treated with high-dose methylprednisolone for 3 days but serum creatinine was not fully normalised. After 2 weeks from initial methyl-PDS pulse therapy, she received intravenous immunoglobulin, plasma exchange and anti-CD20 (rituximab). Cyclosporine was changed to tacrolimus. She achieved a complete response, and SCr. was maintained at 1.3 mg/dL without proteinuria. Follow-up PRA test levels were 0%, 75% for class I and II. Current therapies have had considerable success in reversing mixed, acute humoral and cellular rejection since it is being identified quickly and treated aggressively. The best use of rituximab to treat AMR should be evaluated in controlled trials using dosing strategies that include longer courses or retreatment schedules.
Antibodies, Monoclonal, Murine-Derived ; Appointments and Schedules ; Biopsy ; Calcineurin ; Creatinine ; Cyclosporine ; Female ; Follow-Up Studies ; Graft Rejection ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Kidney Transplantation ; Methylprednisolone ; Middle Aged ; Mycophenolic Acid ; Plasma Exchange ; Plasmapheresis ; Proteinuria ; Rejection (Psychology) ; Renal Insufficiency, Chronic ; Retreatment ; Rituximab ; Steroids ; Tacrolimus ; Transplants ; Unrelated Donors

Thrombotic thrombocytopenic purpura (TTP) and the related hemolytic uremic syndrome (HUS) are disorders characterized by thrombocytopenia, microangiopathic hemolytic anemia, a variable degree of impairment of renal function and fluctuating neurological symptoms, which are thought to be due to platelet activation and subsequent formation of thrombi in the microcirculation. The fact that there was no clear-cut clinical and laboratory features that differentiate HUS from TTP has lead to view these two syndromes as a clinical continuum. Microvascular thrombosis is the typical lesion and closely related with endothelial injury and platelet activation. Pathologic alterations of the brain parenchyma are mainly manifested by small multiple infarcts. Numerous cases of CNS complications of these syndromes have been evaluated by using CT, but few reports have mentioned the MR findings. We experienced a case of TTP-HUS that had clinical features of cortical blindness and the brain lesion was confirmed by MRI showing cerebral infarct at the occipital area but it was reversible course. So we report this case with a brief review of literature.
Anemia, Hemolytic ; Blindness, Cortical* ; Brain ; Hemolytic-Uremic Syndrome* ; Magnetic Resonance Imaging* ; Microcirculation ; Platelet Activation ; Purpura, Thrombotic Thrombocytopenic* ; Thrombocytopenia ; Thrombosis

Goodpasture's syndrome is a clinical complex of anti-GBM (glomerular basement membrane) nephritis and lung hemorrhage. Anti-GBM nephritis is an autoimmune disease in which autoantibodies directed against type IV collagen induce RPGN (rapid progressive glomerulonephropathy) and crescentic glomerulonephritis. 50 to 70% of patients have pulmonary hemorrhage. We have one case of a successful renal transplantation in a patient with Goodpasture's syndrome. A 51 year old male had arrived in the emergency room due to dyspnea. 20 days prior to admission he had suffered from fever and then progressively developed nausea and weakness. He underwent hemodialysis with the dual lumen catheter which was inserted in the right internal jugular vein. Goodpasture's syndrome was confirmed by the measurement of serum anti-GBM Ab titer, renal biopsy and clinical manifestations of pulmonary hemorrhage. Renal biopsy findings showed diffuse proliferative glomerulonephritis on light microscopy and linear ribbon-like deposition of IgG along the GBM. The patient was placed on CAPD on the 30th hospital day. After six months of CAPD, the patient received a renal transplant from a HLA-haploidendical brother. Which was done after negative seroconversion of circulating antibody of GBM. With a induction of IL-2 receptor blocker (Basiliximab), cyclosporine-A and prednisone were administered for their immunosuppressants. He had good health for 37 months with excellent graft function.
Anti-Glomerular Basement Membrane Disease* ; Autoantibodies ; Autoimmune Diseases ; Biopsy ; Catheters ; Collagen Type IV ; Dyspnea ; Emergency Service, Hospital ; Fever ; Glomerulonephritis ; Hemorrhage ; Humans ; Immunoglobulin G ; Immunosuppressive Agents ; Jugular Veins ; Kidney Transplantation* ; Lung ; Male ; Microscopy ; Middle Aged ; Nausea ; Nephritis ; Peritoneal Dialysis, Continuous Ambulatory ; Prednisone ; Receptors, Interleukin-2 ; Renal Dialysis ; Siblings ; Transplants

Cryptococcosis is recognized as one of the most important complications in an organ transplant recipient. Cryptococcosis occurs in 2.5-39% of renal transplant recipients. This infection generally presents as symptomatic disseminated disease with an accelerated clinical course, involves multiple sites including the central nervous system, lungs, and skin. And if diagnosis or treatment is delayed, the prognosis is generally poor. The asymptomatic infection is rare and there are no case reports of asymptomatic disseminated cryptococcosis after renal transplantation in Korea. We experienced a case of asymptomatic cryptococcal multi-organ infection detected incidentally in a 51-year-old male received a living related renal transplant 35 months earlier for end-stage renal disease due to diabetic nephropathy. We treated successfully with amphotericin B and fluconazole and hereby report this case with a review of the relevant literature.
Amphotericin B ; Asymptomatic Infections ; Central Nervous System ; Cryptococcosis ; Diabetic Nephropathies ; Fluconazole ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation ; Korea ; Lung ; Male ; Middle Aged ; Prognosis ; Skin ; Transplants

Focal segmental glomerular sclerosis (FSGS) is known to recur in 20-40% of the renal allografts with graft loss in about half of these cases. We report a successful treatment of a recurrent FSGS after kidney transplantation with rituximab and plasmapheresis. An 16-year-old patient whose primary kidney disease was FSGS developed recurrence of proteinuria after living donor kidney transplantation despite preemptive plasmapheresis and one dose of rituximab (375 mg/m2). After kidney transplantation, nephrotic range proteinuria was detected. Kidney biopsy was done and showed recurrent FSGS. She undergone 11 times of plasmapheresis in the first 4 week post transplantation. In addition, she received additional one dose of rituximab (375 mg/m2) on day 14. Proteinuria was decreased below nephrotic range at 37 day. Ten months later, proteinuria was at 30 mg/day with excellent graft function. No significant adverse events related to rituximab or plasmapheresis were observed. Rituximab with plasmapheresis may be another option for recurrent FSGS after kidney transplantation.
Adolescent ; Antibodies, Monoclonal, Murine-Derived ; Biopsy ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney ; Kidney Diseases ; Kidney Transplantation ; Living Donors ; Plasmapheresis ; Proteinuria ; Recurrence ; Sclerosis ; Transplantation, Homologous ; Transplants ; Rituximab