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

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 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

Transplant renal artery stenosis (TRAS) is a common surgical complication after kidney transplantation (KTP) and is the cause of allograft dysfunction. TRAS is a potentially curable cause of refractory hypertension and allograft dysfunction which accounts for approximately 1% to 5% of cases of post-transplant hypertension. Acute cellular rejection (ACR) is also common after KTP, which is the main cause of allograft dysfunction. Although the incidence of ACR has declined with the advent of new immunosuppressive drugs, it is still around 15% worldwide. Although each disease is frequently seen individually, seeing both together is rare. A 42-year-old man with end stage renal disease underwent KTP, and the donor was his younger brother. Four months after KTP, his serum creatinine was increased to 2.1 mg/dL, and renal biopsy showed interstitial lymphocytic infiltration and tubulitis. With the diagnosis of acute T-cell mediated rejection, steroid pulsing therapy was started, but it was resisted. Therefore thymoglobulin 60 mg (1 mg/kg/day) was administered for 6 days, but serum creatinine was 1.8 mg/dL. Abdomen magnetic resonance angiography showed TRAS, stenosis at the anastomosis site and lobar artery in the lower pole. Percutaneous transluminal angiography was performed successfully. After balloon angioplasty, the stenotic lesion showed a normal size and blood flow. The patient's renal function returned to normal levels and he is currently being followed up for 9 months.
Abdomen ; Adult ; Allografts ; Angiography ; Angioplasty, Balloon ; Arteries ; Biopsy ; Constriction, Pathologic ; Creatinine ; Diagnosis ; Humans ; Hypertension ; Incidence ; Kidney Failure, Chronic ; Kidney Transplantation ; Magnetic Resonance Angiography ; Renal Artery Obstruction* ; Renal Artery* ; Siblings ; T-Lymphocytes ; Tissue Donors ; Transplantation*

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

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

Peritonitis is a major cause of morbidity in continuous ambulatory peritoneal dialysis (CAPD) patients. Achromobacter xylosoxidans is a rarely reported cause of peritonitis in CAPD patients. In this report, a peritonitis case due to Achromobacter xylosoxidans in a 60-year-old male patient with end-stage renal failure receiving CAPD for 7 years, has been reported. White blood cell (WBC) count in peritoneal fluid was 3,160/mm3 with 95% neutrophil. Gram staining of the peritoneal fluid yielded gram negative rod. Empirical antibiotic therapy with ceftriaxone was initiated intraperitoneally. But drug sensitivity test revealed these regimens were resistant. On fourth hospital day, Achromobacter xylosoxidans was cultured from peritoneal effluent, the antibiotic regimen was switched to piperacillin/tazobactam intraperitoneally. The patient rapidly recovered and the WBC count of the peritoneal effluent decreased. The therapy was continued for 14 days and then the patient was discharged. The peritoneal catheter was not removed.
Achromobacter ; Achromobacter denitrificans ; Ascitic Fluid ; Catheters ; Ceftriaxone ; Humans ; Kidney Failure, Chronic ; Leukocytes ; Male ; Middle Aged ; Neutrophils ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis

Secondary hyperparathyroidism is a major complication in ESRD patients undergoing dialysis. In hemodialysis patients with secondary hyperparathyroidism, intravenous administration of paricalcitol became widely utilized. In CAPD patients, however, the intravenous administration of paricalcitol which requires frequent visits to the clinic is not practical. The subject of this study was one CAPD patient with secondary hyperparathyroidism. He had already received oral calcitriol pulse therapy for 6 months and thereafter refused parathyroidectomy and intravenous paricalcitol which required frequent visits to the hospital. Furthermore, paricalcitol capsule is not yet introduced in Korea. Consequently, intraperitoneal paricalcitol therapy was tried whereby the patient was taught how to inject the paricalcitol (5 ug) directly into the dialysate for three times per week before bedtime. Blood samples for measurement of intact parathyroid hormone (iPTH), serum ionized calcium, serum phosphate, serum total alkaline phosphatase levels were obtained at baseline and after 1, 2, 3 and 4 months of treatment. After usage of intraperitoneal paricalcitol for 2 months, there was a significant decrease in iPTH level. In conclusion, intraperitoneal paricalcitol therapy might be effective for suppressing iPTH in CAPD patients with secondary hyperparathyroidism. A large-scale and long-term study must be conducted for safety and clinical effect.
Administration, Intravenous ; Alkaline Phosphatase ; Calcitriol ; Calcium ; Dialysis ; Ergocalciferols ; Humans ; Hyperparathyroidism, Secondary ; Injections, Intraperitoneal ; Kidney Failure, Chronic ; Korea ; Parathyroid Hormone ; Parathyroidectomy ; Peritoneal Dialysis, Continuous Ambulatory ; Renal Dialysis