Continuous erythropoietin receptor activator (CERA) is a novel erythropoiesis-stimulating agent. It has a prolonged half-life compared with recombinant human erythropoietin (rHuEPO) through the integration of amide bonds between amino groups and methoxy polyethylene glycol-succinimidyl butanoic acid. Moreover, CERA has a longer mode of action than rHuEPO, enabling successful anemia treatment with prolonged dosing intervals. We previously reported the effects of CERA administered once every 2 weeks (Q2W) for 28 weeks in hemodialysis (HD) patients with anemia. However, the long-term effect of CERA Q2W switched from epoetin beta (EPO) has not been reported. Thus, we investigated the efficacy of switching from EPO to CERA Q2W, and the transition of patients achieving the target hemoglobin (Hb) level and the maintenance dose of CERA over the long term. The results suggest switching from EPO to CERA Q2W effectively maintained the Hb level within the target Hb±0.5 g/dL in HD patients with anemia over the long-term. Mean dosage of CERA decreased over time from 65 μg at switching to 32 μg at 55 weeks. Intravenous iron use was significantly decreased, and the Erythropoietin Resistance Index score was significantly decreased. CERA Q2W successfully improved the hematopoiesis of patients. Thus, switching from EPO to long-term maintenance therapy with CERA Q2W is sufficient for improvement of anemia as well as maintaining Hb levels of HD patients.

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A 62-year-old man with no evidence of a bleeding tendency or a family history of hereditary bleeding disease began receiving dialysis therapy for the treatment of end-stage renal disease arising from benign nephrosclerosis in July 2009 at another hospital; he was subsequently referred to our hospital for regular dialysis treatment. For several years, no difficulties in treating this patient were encountered. In July 2012, however, we began to note that a longer period of time seemed to be required for the patient to stop bleeding from the vascular access puncture sites after the completion of his dialysis treatments. On one occasion, the application of pressure hemostasis for more than one hour was required before the bleeding could be stopped and the patient was able to go home. However, the patient subsequently returned to the hospital and was admitted because of a massive subcutaneous hemorrhage extending from his left upper arm to the chest due to re-bleeding from the puncture sites. During the period of hospitalization, since intractable subcutaneous upper arm bleeding occurred on repeated occasions, we examined his bleeding and coagulate functions. His activated partial thromboplastin time was markedly extended and his factor VIII activity level was significantly decreased, with the presence of 10 Bethesda units/mL of factor VIII inhibitory activity. Accordingly, we diagnosed the patient as having acquired hemophilia A. Considering his old age and his uremic immune-compromised state, the early cessation of steroid treatment was desired. Consequently, we treated him using bypass therapy and the removal of inhibitory antibodies in combination with a low-dose, short-term steroid administration period. Double filtration plasmapheresis (DFPP) was used to remove the antibodies effectively. After 6 sessions of DFPP, the inhibitors disappeared and his hemostasis eventually recovered to normal. We concluded that DFPP is an effective modality for treating patients with acquired hemophilia A, especially those with an immune-compromised state such as uremia, when used in combination with low-dose steroid administration.

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A 64-year-old male patient began to receive peritoneal dialysis due to end-stage renal disease caused by nephrosclerosis in October 2008. However, peritoneal dialysis was switched to hemodialysis in September 2010 because of repeated episodes of peritonitis during continuous ambulatory peritoneal dialysis. Peritoneal lavage after withdrawal of peritoneal dialysis was performed to prevent the encapsulating peritoneal sclerosis. Then, small renal cell carcinoma was detected by abdominal computed tomography, and retroperitoneal laparoscopic radical nephrectomy was carried out. After surgery, peritoneal lavage was continued uneventfully in the same manner as before. Retroperitoneal laparoscopic radical nephrectomy is useful for patients undergoing peritoneal lavage after withdrawal of peritoneal dialysis.

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A 38-year-old woman on maintenance hemodialysis for 16 years due to IgA nephropathy was admitted to our hospital from 20 weeks of gestation in order to manage her pregnancy. After admission, we started 4 hours of hemodialysis six times per week to maintain pre-dialysis BUN<50 mg/dL and increased her dry weight by 200-300 g per week to match the growth of the fetus. Although we assessed the change of circulation blood volume (%BV) estimated by optical hematocrit measurements, the diameter of the inferior vena cava (IVCd), human atrial natriuretic peptide (hANP) and amniotic fluid volume, hANP increased gradually from 28 weeks of gestation. In the evening of 33 weeks 3 days of gestation, acute dyspnea suddenly occurred and we diagnosed congestive heart failure by chest X-ray and echocardiography. Then, urgent caesarean section was performed. She was treated in an intensive care unit and discharged 24 days after surgery. We detected the presence of atypical prolactin (16 kDa) and increased cathepsin D activity in this case. This suggested that peripartum cardiomyopathy was involved in this episode of acute congestive heart failure.

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We encountered a case of penile necrosis assumed to be associated with calciphylaxis. The patient was a 58-year-old male who had received hemodialysis for 15 years for IgA nephropathy and chronic renal failure. In August 2012, redness and pain of the glans were observed, and the course was followed by conservative treatment with antibiotics, but the condition exacerbated, and whitening and necrosis of the glans occurred in September 2012. The patient had a past medical history of oral warfarin treatment. Partial penectomy was performed in September 2012. On pathological examination, calcification of middle-small muscular-type arteries, arterial stenosis, and obstruction were noted. Referring to the draft diagnostic criteria of calciphylaxis, calciphylaxis-associated penile necrosis was diagnosed on the basis of clinical and histopathological findings. No problem was noted in wound healing after surgery, and there was no recurrence. When painful penile necrosis is observed in dialysis patients, it should be treated, considering this disease in the differential diagnosis. In addition, course observation may be important because symptoms may develop in other regions.

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