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*

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

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

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

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

Thrombotic microangiopathy (TMA) is a serious complication of solid organ transplantation. Drug-induced TMA is typically caused by immunosuppressants, particularly calcineurin inhibitors. Withdrawing the causative drug can be one of the treatments for TMA. However, the more immunosuppressants are reduced, the more risk of rejection increases. Even if TMA is successfully resolved, the outcomes of patient and graft survival would be worse than expected. Therefore, it is necessary to maintain efficient and safe immunosuppression therapy. We report on a case of de novo TMA after kidney transplantation triggered by tacrolimus and reactivated by sirolimus. Belatacept, a novel CTLA4 Ig fusion protein, was administered for maintenance immunosuppressant with mycophenolate mofetil and prednisolon. The patient had excellent early graft outcome, and there have been no adverse events so far.
Abatacept ; Calcineurin ; Graft Survival ; Humans ; Immunosuppression* ; Immunosuppressive Agents ; Kidney Transplantation* ; Kidney* ; Organ Transplantation ; Sirolimus ; Tacrolimus ; Thrombotic Microangiopathies* ; Transplants

Acute antibody-mediated rejection is the major cause of graft failure in the early stage of kidney transplantation. Preoperative treatment and early diagnosis of acute rejection is very important to prevent graft loss in sensitized patients. High panel reactive antibody (PRA) means a likelihood of acute rejection, and the recipient of high PRA needs adequate pretreatment for kidney transplantation. However, there is not sufficient time and chances for desensitization in deceased kidney transplants. We report a successful renal transplant outcome in a 47-year-old-woman with high PRA levels (Class I 97.5%, Class II 36.7%). The cross match was negative on the CDC (ELISA) and flowcytometric methods. Plasma exchange was performed on the recipient before transplantation (fresh frozen plasma replacement, 1.3 plasma volume) and immediately after plasma exchange she was given 200 mg of rituximab. She received basiliximab and methyl prednisolone induction therapy and was maintained on steroids, mycophenolate mofetil, and tacrolimus. Graft function was normal immediately after transplantation, but decreased urinary output and elevated serum creatinine was noted on POD 5. On POD 6, a graft biopsy revealed acute cellular rejection (Type IIa) and antibody-mediated rejection (Type II). On 9~13 days after transplantation, additional plasma exchange was performed every other day, and steroid pulse therapy was performed 3 times. After normalization of urinary output and serum creatinine, the patient was discharged and is being followed up on. In conclusion, immunologically careful preparation and pretransplant treatment may be needed on the negative cross match in cadaveric kidney recipients with high levels of PRA.
Antibodies, Monoclonal ; Antibodies, Monoclonal, Murine-Derived ; Biopsy ; Cadaver ; Centers for Disease Control and Prevention (U.S.) ; Creatinine ; Early Diagnosis ; Graft Rejection ; Humans ; Immunization ; Kidney ; Kidney Transplantation ; Mycophenolic Acid ; Plasma ; Plasma Exchange ; Prednisolone ; Recombinant Fusion Proteins ; Rejection (Psychology) ; Rituximab ; Steroids ; Tacrolimus ; Tissue Donors ; Transplants

It has been well known that long-term immune suppression in renal transplant patients increases the possibility of complications. Infectious disease is one of the representative complications. We experienced a case of nocardiosis with cytomegalovirus infection after third renal transplantation in China. Nocardiosis is an important opportunistic infection in immunosuppressed patients, lymphoma, sarcoidosis, and organ transplant patients. CMV can cause severe hepatitis, pneumonitis, enteritis, endometritis, and encephalitis. It can depress bone marrow, and impair the immune system so as to increase other bacterial infection and trigger rejections. Third renal transplantation causes long-term immune suppression or over-immune suppression on transplant patients. Very few cases of third renal transplantation have been reported in Korea. We reduced the dose of immune- suppressants, and treated it successfully with ganciclovir and Trimethoprim/Sulfamethoxazole (Bactrim(R)).
Bacterial Infections ; Bone Marrow ; China* ; Communicable Diseases ; Cytomegalovirus ; Cytomegalovirus Infections ; Encephalitis ; Endometritis ; Enteritis ; Female ; Ganciclovir ; Hepatitis ; Humans ; Immune System ; Kidney Transplantation* ; Korea ; Lymphoma ; Nocardia Infections* ; Opportunistic Infections ; Pneumonia ; Sarcoidosis ; Transplants