Diffuse Leukoencephalopathy Associated with Dialysis Disequilibrium Syndrome.

A 52-year-old woman, previously treated for gastric cancer, began hemodialysis (HD) to treat the onset of severe acidemia. After her initial HD sessions, she suffered from a prolonged coma for approximately ten days. Magnetic resonance imaging revealed diffuse leukoencephalopathy, with increased apparent diffusion coefficient. Magnetic resonance spectroscopy showed a reduction of the N-acetylaspartate/creatine ratio. Her neuroimaging findings gradually resolved. Her transient cerebral white matter lesions were thought to be interstitial edema derived from dialysis disequilibrium syndrome (DDS), which might have been amplified by subclinical brain injury due to past chemotherapy. Her history of cancer chemotherapy may be a risk factor for an exacerbation of DDS.

Effect of renal impairment on the pharmacokinetics of memantine.

The effect of renal impairment on the pharmacokinetics of a single oral dose of memantine (10 mg) was determined in Japanese subjects. Subjects were assigned to four groups based on baseline creatinine clearance (CL(CR)): normal renal function (> 80 mL/min, n = 6), and mild (50 to ≤ 80 mL/min, n = 6), moderate (30 to < 50 mL/min, n = 6), and severe renal impairment (5 to < 30 mL/min, n = 7). Mean memantine maximum plasma concentration (C(max)) was similar in the groups (12.66, 17.25, 15.75, and 15.83 ng/mL, respectively), as was mean time to C(max) (6.2, 5.2, 4.3, and 5.4 h, respectively). However, exposure to memantine determined from mean area under the plasma concentration-time curve was 1.62-, 1.97-, and 2.33-times higher in subjects with mild, moderate, and severe renal impairment, respectively, as compared to controls with normal renal function. Mean memantine plasma elimination half-life increased according to increasing renal impairment (61.15, 83.00, 100.13, and 124.31 h, respectively), while mean cumulative urinary recovery of unchanged memantine in 72 h after dosing decreased according to increasing renal impairment (33.68%, 33.47%, 23.60%, and 16.17%, respectively). These results are the same as those in the previous study on caucasian individuals, when compared per body weight. It is suggested that the dose of memantine should be halved in patients with renal impairment.

MPO-ANCA crescentic glomerulonephritis complicated by membranous nephropathy: MPO demonstrated in epimembranous deposits.

An elderly woman presented with haematuria and proteinuria accompanied by elevated serum myeloperoxidase (MPO)-specific anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A renal biopsy revealed mild mesangial proliferation with fibrocellular crescent formation and a membranous glomerular lesion. Immunofluorescence microscopy using FITC-labelled rabbit anti-human MPO antibodies revealed granular MPO deposition along the glomerular capillary walls (GCW) with a staining profile similar to that of glomerular IgG deposition. The one-year follow-up renal biopsy revealed minimal IgG and undetectable MPO deposition. Both MPO and MPO-ANCA might have been responsible for the IgG immune depositions along the GCW in this patient.

A case of lupus nephritis coexisting with podocytic infolding associated with Takayasu's arteritis.

A 61-year-old Japanese woman with both Takayasu's arteritis (TA) and systemic lupus erythematosus (SLE) presented with proteinuria due to glomerulopathy associated with podocytic infolding. She presented with unequal pulses in the upper extremities at 38 years old. TA was diagnosed on the basis of angiographic identification of stenosis in the left subclavian artery. Eight years after onset of TA, SLE was diagnosed on the basis of clinical and laboratory findings, including proteinuria, hematological and immunological abnormalities, high titer of antinuclear antibody, and a positive lupus band test on the skin. A renal biopsy showed lupus nephritis coexisting with podocytic infolding associated with TA, which has rarely been reported. After low-dose prednisolone therapy and immunosuppressive therapy by cyclosporine for 14 years, proteinuria has persisted without deterioration of serum creatinine levels.

Lupus nephritis associated with positive MPO-ANCA in a patient with underlying autoimmune hemolytic anemia.

A 19-year-old female was admitted with general malaise and systemic edema. She had been diagnosed as having autoimmune hemolytic anemia (AIHA) eight years earlier and was successfully managed with oral prednisolone. During the current admission, she was diagnosed as having systemic lupus erythematosus (SLE) based on the presence of renal involvement, hematological abnormalities, and antinuclear and anti-double-stranded DNA antibodies, along with a recurrence of AIHA; her serology revealed a high myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer. She was treated with prednisolone (50 mg day(-1)), but her renal function started to deteriorate. She responded to treatment with hemodialysis, plasmapheresis, and methylprednisolone pulse therapy; her MPO-ANCA titer and renal function improved. Treatment with intravenous cyclophosphamide gradually suppressed her AIHA and SLE activity. A renal biopsy revealed a diffuse proliferative lupus nephritis (class IV-G (A)) with necrotizing crescentic glomerulonephritis that was presumed to be associated with MPO-ANCA. The association of MPO-ANCA with SLE in this refractory case is discussed.

Elderly onset systemic lupus erythematosus (SLE) presenting with disseminated intravascular coagulation (DIC).

We describe an unusual case of elderly onset systemic lupus erythematosus (SLE) that presented with disseminated intravascular coagulation (DIC). An 86-year-old man who complained of general malaise was admitted for evaluation and treatment of thrombocytopenia. He was diagnosed as having SLE and DIC based on the criteria of the American College of Rheumatology for SLE (renal involvement, hematological abnormalities, and positivity for antinuclear antibody and lupus anticoagulant) and the criteria for DIC presented by the subcommittee on DIC of the ISTH (a large increase of fibrin degradation products [3 points] and a platelet count <50 x 10(3)/ml [2 points], resulting in a score of 5; a score > or =5 is compatible with DIC). The patient was treated with corticosteroid therapy (30 mg/day); the DIC and SLE remitted, and his renal function improved, but he developed pulmonary tuberculosis. Timely diagnosis, appropriate treatment, and an awareness of the potential for serious infections are of utmost importance when dealing with patients with elderly onset SLE.

Osteopontin expression in vascular smooth muscle cells in patients with end-stage renal disease.

beta-glycerophosphate, a phosphate donor, and uremic sera induce osteopontin (OPN) expression in bovine vascular smooth muscle cells (VSMCs). However, the correlations of serum phosphorus level with OPN expression, and blood urea nitrogen (BUN) level with OPN expression in humans have not previously been reported. The purpose of the current study is to compare the expression of OPN in VSMCs with clinical data in patients with end-stage renal disease (ESRD). The radial arteries of 33 patients (21 male and 12 female patients) were examined to determine the expression of OPN and collagen type I (Col I) by immunohistochemistry. The correlation of the expression of bone matrix proteins with clinical data was analyzed. Between the low-serum phosphorus (<6 mg/dL) group and high-serum phosphorus (> or =6 mg/dL) group, significant differences were detected in the expression of OPN (P = 0.0049) and the levels of BUN (P = 0.0005), serum phosphorus (P < 0.0001) and calcium x phosphorus products (P < 0.0001). Moreover, between the low-BUN (<70 mg/dL, N = 19) group and high-BUN (> or =70 mg/dL) group, significant differences were detected in the expression of OPN (P = 0.0039) and the levels of BUN (P = 0.0002), serum phosphorus (P = 0.0002) and calcium x phosphorus products (P = 0.0003). We have shown that hyperphosphatemia or azotemia is associated with the expression of OPN in VSMCs in patients with ESRD.

[Focal segmental glomerulosclerosis in a patient with polycythemia vera].

Herein we describe the case of a patient with focal segmental glomerulosclerosis (FSGS) following polycythemia vera (PV) on whom hemodialysis was started 7 years later. A 66-year-old woman who had been treated for PV with hydroxyurea and phlebotomy for three years was referred to our hospital because of nephrotic syndrome. Renal biopsy performed at her local hospital revealed FSGS. Although she had received prednisolone at an initial dose of 45 mg/day, no significant improvement of proteinuria was achieved. The dose of prednisolone was tapered because the second renal biopsy revealed sclerosing glomerulopathy. We considered that FSGS was associated with PV because renal hemodynamic alterations in PV could result in FSGS as in any other secondary FSGS and there was no proteinuria at the initial detection of PV. On January 29, 1999, she developed massive proteinuria (9.6 g/day) and the findings of the third renal biopsy worsened in comparison with that of the first renal biopsy. Thereafter, hydroxyurea or ranimustine was used in treating PV at an outpatient clinic. However severe thrombocytosis was difficult to control, and progressive renal dysfunction finally necessitated hemodialysis on January 18, 2005. In conclusion, physicians should be aware of the risk of progressive renal failure in patients with FSGS following PV, particularly in patients with persistent thrombocytosis.

Microscopic polyangiitis after silicone breast implantation.

We describe the case of a patient who developed microscopic polyangiitis (MPA) after silicone breast implantation. A 60-year-old woman who had undergone silicone breast implantation was admitted to our hospital with complaints of general malaise and hematoproteinuria. She was diagnosed as having MPA with evidence of acute progressive renal failure, pulmonary hemorrhage, and positivity for myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA). A renal biopsy showed severe necrotizing and crescentic glomerulonephritis with arteriolitis. The patient received high-dose steroids and plasma exchange treatment, but died of progressive pulmonary hemorrhage and multiple cerebral hemorrhage. Silicone implantation is associated with scleroderma, systemic lupus erythematosus, and rheumatoid arthritis. This case report indicates the possibility of the development of MPA after silicone breast implantation.

[Case of paroxysmal nocturnal hemoglobinuria complicated with IgA nephropathy who developed acute renal failure induced by hemolytic crisis].

A 60-year-old man, who had been diagnosed as having paroxysmal nocturnal hemoglobinuria(PNH) in 1994, was admitted to our hospital with general fatigue, and dark urine after a common-cold in January 2001. In the peripheral blood, the red blood cell count was 136 x 10(4)/microl, hemoglobin 4.0 g/dl and hematocrit 12.4%. The serum creatinine level was 9.9 mg/dl. Kidney biopsy revealed focal and segmental proliferation of mesangial cells, mesangial matrix expansion, acute tubular necrosis, interstitial fibrosis and hemosiderine deposits in the tubular epithelial cells confirmed by Berlin-blue staining. Immunofluorescence microscopy showed IgA and C3 deposition in the mesangium. Electron microscopy revealed electron dense deposits in the mesangial area and heavy electron-dense hemosiderin pigments in proximal tubular epithelial cells. After the transfusion of six units of washed red blood cells and two sessions of hemodialysis, the renal function returned to the levels before admission.

Acquired reactive perforating collagenosis in a nondiabetic hemodialysis patient: successful treatment with allopurinol.

The authors present a case of acquired reactive perforating collagenosis developed in a nondiabetic hemodialysis patient, who was treated successfully with allopurinol. Treatment of acquired reactive perforating collagenosis is difficult and often ineffective. The patient had been unresponsive to conventional treatments, but the pruritus was controlled, and skin lesions subsequently resolved after the treatment with allopurinol. Possible mechanisms of allopurinol treatment for acquired reactive perforating collagenosis are discussed.

Propylthiouracil-induced antineutrophil cytoplasmic antibody-associated crescentic glomerulonephritis in a patient with a predisposition to autoimmune abnormalities.

Abstract A 65-year-old woman with a history of primary biliary cirrhosis was diagnosed as having myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-associated crescentic glomerulonephritis (GN) during propylthiouracil (PTU) therapy for Graves' disease. Antinuclear antibodies, as well as various thyroid-associated autoantibodies, had been detected since the diagnosis of Graves' disease was made. The patient carried human leukocyte antigens (HLAs) DR4 and DR9, the two HLA haplotypes that have been reported to be related to ANCA-associated vasculitis. Withdrawal of PTU and the administration of prednisolone resulted in a decrease in the titer of MPO-ANCA, together with an improvement in renal function. It is suggested that in addition to the PTU therapy, her genetic predisposition to autoimmunity had played a role in the production of MPO-ANCA and the development of crescentic GN.