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

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

PURPOSE: The presence of C4d in peritubular capillaries (C4d (PTC)) as a diagnostic in-situ marker of acute humoral rejection and CD20 as marker of B-cell deposition in graft kidney has been reported to be related to steroid resistance and poor outcome. In this retrospective study, we evaluated the clinical significance of C4d and CD20 in allograft renal biopsies by immunohistochemistry technique. And we also evaluated the relationships between C4d and CD20 positive B lymphocytes. METHODS: We studied 22 patients who had been biopsied for suspected acute rejection. Biopsies were classified by updated Banff 97 criteria. Of the 22 cases, borderline rejection and Banff 1A were 11 cases respectively and no case had a vascular lesion. Paraffin sections were stained with monoclonal antibodies (anti-C4d and -CD20) using an immunohistochemistry technique and the results of immunohistochemistry were analyzed by clinical data. RESULTS: Of the 22 cases, 22.7% (5/22) showed diffuse and 40.9% (9/22) showed focal C4d positivity in peritubular capillaries. The grafts failed to survive in 20% (1/5) of the diffuse (P), 44.4% (4/9) of the focal, and 0% (0/8) of the negative group for 2 years since postbiopsies, however, the C4d staining was not statistically related to graft loss and graft survival rates (P=0.091, P=0.106 respectively). The C4d positivity was significantly related to the level of serum creatinine (P=0.042) and to steroid pulsing therapy resistance (P=0.030). However C4d deposition was not associated with recipient gender, age, type of donor (living vs deceased), HLA matching, induction, and Banff classification. On the CD20 immunostaining, 50.0% (11/22) showed negative reactivity, 9.1% (2/22) one nodule, 40.9% (9/22) 2 nodules. The presence and the number of CD20 positive nodules were not correlated to the C4d clinical data. But, the degree of C4d staining was statistically related with the presence of CD20 positive nodules (P=0.029). CONCLUSION: The peritubular capillary C4d is clinically important however, not likely a significant predictor of grafts survival rates in mild rejection. The clinical implication of CD20 positive B lymphocyte nodules in acute rejection was not demonstrated in this study. But, CD20 positive B lymphocyte may be a positive linkage with C4d and participate in humoral rejection.
Antibodies, Monoclonal ; B-Lymphocytes ; Biopsy ; Capillaries ; Creatinine ; Graft Survival ; Humans ; Immunohistochemistry ; Kidney ; Lymphocytes ; Paraffin ; Rejection (Psychology) ; Retrospective Studies ; Survival Rate ; Tissue Donors ; Transplantation, Homologous ; 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