Sebaceous trichofolliculoma, which is a variant of trichofolliculoma, is a rare disease that clinically show a centrally depressed lesion usually singly on the nose, and histologically a centrally located cavity, lined by squamous epithelium, with numerous sebaceous lobules connected to them. We describe a case of sebaceous trichofolliculoma in a 27-year-old woman who had several brown papules on the nose. We think that this is the first report of sebaceous trichofolliculloma in Korean literature.
Adult ; Epithelium ; Female ; Humans ; Nose ; Rare Diseases

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

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

Left ventricular pseudoaneurysm is a rare but fatal complication of acute myocardial infarction. It occurs as a consequence of rupture of the ventricular free wall and is confined by a portion of pericardium. The pseudoaneurysm extended to lateral side of the left atrium is rare. We report a case of left ventricular pseudoaneurysm extended to lateral side of the left atrium in a 83-year-old man.
Aged, 80 and over ; Aneurysm, False* ; Heart Atria* ; Humans ; Myocardial Infarction* ; Pericardium ; Rupture

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

Hypercalcemia is a common complication in CAPD patients treated with calcium-containing phosphate binders and using the standard dialysate(Ca++ : 3.5 mEq/L). Furthermore, the high calcium concentration in standard dialysate may have a suppressive effect on parathyroid hormone(iPTH) level, contributing to the high prevalence of low-urnover bone disease. We studied the effect of low calcium dialysate(Ca++ : 2.5 mEq/L) for those patients with high risk of low- turnover bone disease. Among 386 patients(1996. 1.- 1999. 12.) who had been stable on CAPD for at least 3 months, 46 patients were included in this study. The patients were divided into 3 groups on the basis of the iPTH levels(<150 pg/mL) and/or corrected serum calcium levels(>10 mg/dL) before the conversion to low calcium dialysate. Group 1(n=29), iPTH <150 pg/mL and Ca++>10 mg/dL; Group 2 (n=14), iPTH <150 pg/mL and Ca++<10 mg/dL; Group 3(n=3), iPTH >150 pg/mL and Ca++ >10 mg/ dL. During a 2-month run-in period, those patients were treated with standard dialysate. After that, a 12-month therapy with low calcium dialysate was followed. Biochemical data including calcium, phosphorus, iPTH and alkaline phosphatase were measured regularly and daily phosphate binder and calcitriol intake(pill counting) were assessed during the run-in and therapy period. We obtained the following result: the prevalence of hypercalcemia(Ca++>10.5 mg/dL) was 5.7%(22/ 386 patients). Serum calcium levels decreased during the therapy period(12 months)(10.5+/-1.4 vs 9.4+/-1.3 mg/dL, p<0.05). Serum phosphorus levels remained unchanged. Mean serum alkaline phosphatase level increased(203.0+/-92.9 vs 257.2+/-103.4 U/L, p<0.05). Serum iPTH levels increased (92.7+/-128.8 vs 225.3+/-237.3 pg/mL,p<0.05). The mean intake of oral phosphate binders was not significantly different between run-in period and therapy period. But calcitriol doses increased 0.038+/-0.087 at run-in period to 0.158+/-0.288 tablets/person/day at therapy period(p<0.05). In the six patients, low calcium dialysate was converted to standard dialysate due to high iPTH level (n=3), symptomatic hypo calcemia(n=2), and uncontrolled edema(n=1). In conclusion, in the study of 46 patients over 12 month period, the usage of 2.5 mEq/L calcium dialysate resulted in a significant decrement in calcium levels and increased iPTH levels. Therefore, we propose that dialysis with a low calcium dialysate is an acceptable form of therapy for the patients with high risk of low-turnover bone disease showing hypercalcemia and low iPTH level. However, further study will be needed for evaluating the effect of low calcium dialysate in low-turnover bone disease.
Alkaline Phosphatase ; Bone Diseases* ; Calcitriol ; Calcium* ; Dialysis ; Humans ; Hypercalcemia ; Peritoneal Dialysis, Continuous Ambulatory* ; Phosphorus ; Prevalence

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

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