[in Japanese]

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【Background】We examined the effects of once- or twice-monthly continuous erythropoietin receptor activator (CERA) administration on ferrokinetics, particularly the hepcidin-25 level and erythropoiesis-stimulating agent resistance index (ERI), in anemic patients undergoing hemodialysis.【Methods】A 1-year prospective study of 24 patients undergoing hemodialysis was performed. Group A (n=12) was administered CERA once a month, and Group B (n=12) was administered CERA twice a month. We compared the subjects' hepcidin-25, serum iron, transferrin saturation, ferritin, hemoglobin, and target maintenance hemoglobin levels, as well as their ERI values and the change in the CERA dose between the two groups.【Results】There were no differences in sex, age, hemodialysis duration, the baseline hemoglobin level, the ferritin level, or the CERA dose between the two groups. Both groups displayed significantly decreased hepcidin-25 levels at 1 week. Group B exhibited significantly lower hepcidin-25 levels than Group A at 1 month, 3 months, and 1 year. Similar tendencies were seen for the subjects' serum iron, transferrin saturation, and ferritin levels. The CERA dose decreased significantly in Group B, but not in Group A. The ERI increased by 0.4% in Group A, but decreased by 10.5% in Group B. However, no inter- or intra-group differences were noted.【Conclusion】Changes in the CERA dose had significant ferrokinetic effects, including on the hepcidin-25 level. Twice-monthly CERA administration might prevent neocytolysis and promote bone marrow hematopoiesis.

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A 43-year old male was admitted to our hospital because of nephrotic syndrome and an acute kidney injury. He had a history of lupus nephritis type V 8 years ago, but had achieved complete remission after treatment. Serological tests showed elevated anti-glomerular basement membrane antibody levels. A kidney biopsy examination showed crescentic glomerulonephritis without IgG deposits. Thus, a diagnosis of lupus nephritis type IV＋V was made. Steroid therapy was started, and plasma exchange was performed. Despite these therapies, the patient's renal function deteriorated, and hemodialysis was initiated. Intravenous cyclophosphamide pulse therapy was added, but the patient's renal function did not improve. A second kidney biopsy was performed on the 48th day. It showed the linear deposition of IgG along the capillary wall, which led to a diagnosis of anti-glomerular basement membrane disease. A further immunosuppressive regimen was considered to be ineffective. Therefore, the steroid therapy was tapered, and hemodialysis was continued. This case report demonstrates that anti-glomerular basement membrane disease can occur during the course of lupus nephritis.

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A 75-year-old male who underwent hemodialysis developed progressive anemia and thrombocytopenia, and hyperferritinemia despite being symptomless. He was admitted to our hospital because of intradialytic hypotension. The patient's laboratory data showed aggravated thrombocytopenia and hyperferritinemia together with high levels of C-reactive protein and procalcitonin. Cultures of the patient's blood and bone marrow aspirate confirmed the presence of methicillin-resistant Staphylococcus aureus. A bone marrow biopsy was performed, which demonstrated signs of the phagocytosis of blood components. Based on these findings, we diagnosed the patient with hemophagocytic syndrome (HPS). Intensive care, including steroid pulse therapy and continuous hemodiafiltration, had no effect, and the patient died of multiple organ failure. The autopsy demonstrated hemophagocytosis in the patient's bone marrow and severe purulent cystitis. In this case, the HPS initially exhibited a mild course, but then became rapidly progressive following the development of severe sepsis.The adequate diagnosis and treatment of infection-related HPS before it progresses to sepsis or the patient's general condition deteriorates are necessary to ensure the survival of hemodialysis patients.

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The patient was a 25-year-old male who was diagnosed with Alport syndrome at the age of 3. His renal function gradually decreased, and peritoneal dialysis was initiated at the age of 8. He received a living kidney transplantation at the age of 9. Unfortunately, his graft function gradually deteriorated because of chronic rejection. At the age of 19, he was diagnosed with hemophilia A due to repeated nasal hemorrhaging and his family history. His hemophilia A was mild, and his factor Ⅷ level was>10%. At the age of 21, a second round of renal replacement therapy was required. Hemodialysis without anticoagulation agents was only possible via the infusion of 500 units of factor Ⅷ at the end of the dialysis session. Dialysis treatment was successfully continued without severe hemorrhagic events or blood clotting within the dialysis circuit.

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