Calciphylaxis is a rare and often fatal complication of end-stage renal disease, and is characterized by painful skin ulceration. The main pathological findings are medial calcification and edematous intimal proliferation of small arteries. There had been little information regarding the prevalence and clinical features of calciphylaxis in Japan before our group conducted a nationwide survey in a Japanese dialysis population in 2009 as the study of “Research on Intractable Disease of Health and Labour Sciences Research Grants” from the Ministry of Health and Labour. The prevalence of calciphylaxis was speculated to be much lower than that in Western countries, and the lack of knowledge on the disease was suggested be related to this low prevalence. Therefore, to facilitate the diagnosis of calciphylaxis and spread knowledge of it, we proposed diagnostic criteria based on nationwide surveillance. These criteria must be reviewed by the accumulation of cases, while this attempt should promote caution regarding calciphylaxis.

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Objective: To investigate the safety and efficacy of pregabalin in patients with peripheral neuropathic pain undergoing maintenance dialysis. Subjects：A pain questionnaire was conducted in patients undergoing maintenance dialysis at this facility or its associated clinic, of whom 137 persons fulfilled the inclusion criteria, and consent was obtained from 32 subjects with peripheral neuropathic pain. The subjects were 20 men and 12 women, with a mean age of 69 years and a mean dialysis time of 92 months. Methods: Patients with peripheral neuropathic pain were treated with an initial dose of pregabalin at 25mg, and, thereafter, surveyed with a questionnaire, an SF-8, a VAS scale, and given HS-CRP (high-sensitivity C-reactive protein) tests at Weeks 2, 4, and 8. The dose was increased up to a maximum of 75mg depending on the patient. Results: Twelve subjects discontinued because of dizziness, lightheadedness, or a lack of efficacy. Seven subjects continued at 25mg, 6 at 50mg, and 7 at 75mg. While significant reductions were noted 8 weeks after the start based on the questionnaire results, the VAS scale, and the SF-8 as compared to before the study initiation, a significant difference was not noted with HS-CRP. Also, some patients complained of drowsiness or dizziness as adverse reactions; however, no serious adverse drug reactions were noted. Conclusions: If side effects are carefully watched for with pregabalin administration and the doses used are prudently decided upon, the efficacy of this agent for peripheral neuropathic pain can be expected.

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In Japan, cardiovascular disease was the most common cause of death in 2010. Cilostazol is recommended in the guidelines for the treatment of cerebral apoplexy and peripheral artery disease, and also reportedly increases the serum level of high-density lipoprotein (HDL) cholesterol and decreases that of triglycerides. However, the effects of cilostazol on lipid levels in hemodialysis patients have not previously been reported. We examined the effects of switching from aspirin to cilostazol on the lipid profiles of hemodialysis patients. Eight hemodialysis patients with peripheral arterial disease or a history of cerebral infarction who were being treated with aspirin were enrolled in this study. The patients’prescriptions were switched from aspirin (100mg/day) to cilostazol (100mg/day). We examined the serum levels of albumin, triglycerides, low-density lipoprotein (LDL) cholesterol, HDL cholesterol, apolipoprotein A1 (Apo A-1), apolipoprotein A2 (Apo A-2), and apolipoprotein B (Apo B) before and 1 month after the start of treatment with cilostazol. The serum levels of HDL cholesterol, Apo A-1, and Apo A-2 increased significantly (45.8±11.5 to 52.4±12.3mg/dL, p=0.003; 123.8±21.3 to 139.4±19.1mg/dL, p=0.004; and 26.4±4.3 to 28.9±3.6mg/dL, p=0.003; respectively). The serum level of triglycerides tended to decrease (103.0±70.0 to 83.1±39.5mg/dL, p=0.186). In hemodialysis patients, cilostazol might increase the serum level of HDL cholesterol, Apo A-1, and Apo A-2, similarly to its effects in non-hemodialysis patients.

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Two years ago, a 23-year-old woman attended a private clinic and was diagnosed with systemic scleroderma based on the detection of Raynaud's phenomenon, scleroderma of her hands, and positivity for the anti-topoisomerase I antibody. She suffered from nausea, vomiting, and loss of appetite in November 2010, and was referred to our hospital in December 2010 because of severe renal dysfunction (blood urea nitrogen, 92.1mg/dL; creatinine, 12.76mg/dL). Hemodialysis was initiated immediately on admission. Her condition was initially considered to be scleroderma renal crisis because she showed severe hypertension. However, urinalysis revealed massive proteinuria (6.8g/gCr) and hematuria (urinary red blood cells, 50-99/HPF), and the myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer was elevated to 78EU. Percutaneous renal biopsy was performed for an accurate diagnosis. The pathological diagnosis was pauci-immune type crescentic glomerulonephritis. Oral prednisolone (30mg daily) was commenced following methylprednisolone pulse therapy, but her renal function did not improve. She chose peritoneal dialysis (PD) as a maintenance dialysis method. Total creatinine clearance (Ccr) was low (25.0L/week) under automatic PD at night. Hence, the therapy was switched to continuous ambulatory PD (CAPD). Total Ccr increased to 35.0L/week, and she was discharged in March 2011. Her CAPD filtration was maintained at the level of 1,000 to 1,200mL/day, and a peritoneal equilibration test in June 2011 showed a low average result. In December 2011, however, her CAPD filtration volume decreased to 800mL/day, and her weight increased. Next, an icodextrin dialysate night dwell was initiated, and her filtration volume increased to 1,000 to 1,200mL/day. Many cases of scleroderma complicated with MPO-ANCA-positive crescentic glomerulonephritis have been reported. However, this case was unique because there have been only a few reported cases of scleroderma patients treated with PD.

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In Japan, hemodialysis patients have been increasing. Both the age of patients starting hemodialysis and that of patients receiving hemodialysis have become older than ever. Consequently, the number of hemodialysis patients with dementia has been growing. Cerebrovascular disease (CVD) is more common in hemodialysis patients than in non-hemodialysis patients. The prevalence of dementia in hemodialysis patients with CVD is also higher than without CVD. This factor also contributes to the high prevalence of dementia in hemodialysis patients. In addition, patients are often medically sedated for their symptoms. It is often difficult to perceive the change in the consciousness level of hemodialysis patients. Here, we report a non-diabetic hemodialysis patient who suffered from dementia due to CVD, and was diagnosed with primary adrenal insufficiency, indicated by hypoglycemic coma. In the case of comatose hemodialysis patients, therefore, we should consider hypoglycemia as one of the causes of coma regardless of diabetes.

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A 73-year-old diabetic patient on maintenance hemodialysis was admitted to our hospital for MRSA arteriovenous graft (AVG) infection and pyogenic vertebral osteomyelitis. Because it is difficult to establish AV fistulation, hemodialysis was converted to peritoneal dialysis. Antibiotics were started and AVG was removed with stable infection control. Myoclonus and generalized convulsion developed 86 days after admission. No evidence of an acute CNS lesion was discovered, nor electrolyte and glycemic disturbance. Because the patient was malnourished, Wernicke's encephalopathy was suspected. The intravenous administration of thiamine was started, with the rapid resolution of myoclonus and generalized convulsion.

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A 63-year-old man on hemodialysis for 10 years due to chronic renal failure caused by diabetic nephropathy was examined by our department in January 2011 due to pain in the glans penis region. Examination revealed black, gangrenous tissue in the glans and annular groove regions, and necrotic debridement was conducted at the beginning of February. However, as the gangrenous region enlarged and he became febrile, partial penile resection was conducted on hospital day8 to prevent infection spread. Postoperative wound infection was absent and he was discharged at the end of February. Subsequently, from around April, black necrotic tissue reappeared at the penile resection site and wound dehiscence occurred. Debridement was conducted followed by conservative follow-up, but dialysis intolerance and the systemic condition gradually worsened, and he died at home in the middle of May.

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A 44-year-old man noticed purpura on his lower legs in Feb. 2008. One or two weeks before, he experienced very severe pain in his lower legs. The purpura turned into necrosis and ulcers. There were no infection. He consulted a plastic surgeon because his skin necrosis and ulcers were difficult to treat. The plastic surgeon resected the lesions and substituted the resected area with skin grafts eight times. In June 2008, He underwent resection of 4 parathyroids. He also received on operation of minced parathyroid autografts on his right forearm. After parathyroidectomy, the necrosis and ulcers attenuated. The histological findings supported the clinical course of our CUA case. Recently, research on the pathophysiology of calcification in the mid-membrane of arterioles has made marked progress. Phosphatemia transforms vascular smooth muscle cells into osteogenic cells producing osteocalcin, which combine with Ca²⁺. Consequently, active de novo calcification in the mid-membrane of arterioles occurs. We presented a new idea for a CUA mechanism concerning controversial reports about parathyroidectomy. The essential point is to maintain the dynamic turnover of bone remodeling. The effect of cinacalcet against CUA supports this idea. New treatments for CUA are also emerging. In the near future, parathyroidectomy will be replaced by STS and cinacalcet.

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We estimated the future dialysis population in Japan based on the registry data of the Japanese Society for Dialysis Therapy (JRDR) during 2001 to 2010. We used the prevalence of dialysis patients in the annual JRDR from 2001 to 2010, and calculated the changes in number of dialysis patients from 2002 to 2010 (9 years). The annual rate of increase was plotted by linear model using the prevalent number in each year. (y=450.372044-0.222751x, R-square=0.7227, p=0.0037). Based on this formula, we estimated annual rate of increase and prevalence in the following years. Results were that the prevalence would reach to its maximum prevalence of 348,873 in 2021 (90% confidence interval: 302,868 to 401,119), then start to decline gradually.

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