In Japanese patients treated with hemodialysis, blood concentrations of sulfur-containing substances such as cysteine or sulfite are increased. Recently, hydrogen sulfide has been recognized as the third gaseous mediator that functions in tissues such as brain and vessel. It is produced from cysteine by cystathione β-synthase (CBS) or cystathionine γlyase (CSE) in the mammalian body. However, it is difficult to measure the concentration of hydrogen sulfide in blood, because of several interfering substances that impede analysis. In this study, we measured blood concentrations of hydrogen sulfide in hemodialysis patients by Savage's method using high performance liquid chromatography (HPLC). Concentrations of hydrogen sulfide were gradually decreased in water and plasma. Furthermore, there was a tendency for the concentrations of hydrogen sulfide to decrease much faster in blood. In patients treated with hemodialysis, mean plasma hydrogen sulfide was significantly higher than that in healthy controls. Mean plasma hydrogen sulfide in patients with diabetes mellitus was significantly lower than that in patients with glomerulonephritis. Concentrations of hydrogen sulfide were significantly decreased during hemodialysis treatment. Hydrogen sulfide was detected in liquid from the dialyzer at the initial step of each therapy session. Therefore, increase in hydrogen sulfide in blood caused by renal failure is removed by hemodialysis.

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Because there have only been a few reports evaluating the absolute value of circulating blood volume (CBV) in hemodialysis (HD) patients, we measured CBV in 15 HD patients using pulse dye-densitometry (PDD) in this study. We evaluated the correlation between CBV by PDD (CBV-PDD) and the calculated CBV using three prediction formulas (CBV1 : body weight×0.077, CBV2 : body surface area : 2.68, CBV3 : ogawa & fujita method), respectively. Furthermore, we also evaluated differences in calculating the plasma refilling rate (PRR) and mean plasma refilling coefficient (mean Kr) by CBV-PDD or CBV1. First, there was a significant and positive linear correlation between CBV-PDD and CBV1, CBV2 and CBV3 before HD (CBV-PDD vs. CBV1 ; r=0.56, vs. CBV2 ; r=0.60, vs. CBV3 ; r=0.58, p<0.05, respectively) and there was no difference on analysis by Bland-Altman plots. Second, there was no difference between PRR by CBV-PDD and by CBV1 (CBV-PDD ; 86.9±1.8, CBV1 ; 87.9±1.7%), and mean Kr calculated by CBV-PDD and CBV1 (CBV-PDD ; 2.5±0.4, CBV1 ; 2.6±0.4 mL/min/mmHg). Therefore, we concluded that the evaluation of CBV and vascular permeability in HD patients showed almost the same values measured by PDD and calculated by prediction formulas. Therefore, the evaluation of body fluid using either prediction formulas or PDD is clinically useful.

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Sixty-one patients at this hospital have been undergoing outpatient hemodialysis for <2 years. Thirty-two of the 61 patients (age : 59.2±12.7 years ; dialysis period : 12.6±7.3 months) underwent 320-row computed tomography (CT) imaging to evaluate coronary stenosis. We excluded 6 patients >80 years of age, 10 who underwent conventional coronary angiography (CAG), and 13 who did not consent to participation in this study. Among the 32 patients, CT imaging demonstrated that 6 had coronary stenosis, and 20 did not have coronary stenosis. The remaining 6 patients were indeterminate (5, advanced calcification ; 1, poor image quality). The average age of patients with coronary stenosis (67.3±9.5 years) and indeterminate cases (68.3±9.7 years) was significantly higher (p<0.05 and p<0.01, respectively) than that of those without coronary stenosis (54.0±11.8 years). The calcification score of indeterminate cases (1088±907) was significantly higher (p<0.05 and p<0.01, respectively) than that of patients with (351±356) or without (100±217) coronary stenosis. Four of the 6 coronary stenosis patients also had diabetes (67%), which was higher than the rate of 5/20 patients without coronary stenosis (25%), but the difference was not significant. Five of 6 patients with CT-detected coronary stenosis who underwent CAG had significant stenosis requiring treatment. One patient underwent coronary artery bypass surgery, 2 percutaneous coronary intervention (PCI), and the other 2 were administered drugs. One of 5/6 patients who were indeterminate on CT was found to have significant coronary artery stenosis, and PCI was performed. CT coronary artery screening detected coronary artery disease requiring treatment in 6/32 (19%) asymptomatic hemodialysis patients, on whom CAG was performed. Since 320-row CT coronary artery screening can detect untreated and latent coronary stenosis, it is useful to screen patients at the initiation of maintenance hemodialysis.

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A 67-year-old woman with a 16-year history of hemodialysis because of diabetic kidney disease received maintenance dialysis therapy for 3.5 hours 3 times a week. Weight gain was approximately 4% of her basic weight (40.2 kg). Blood pressure was 190/90 mmHg on awakening, 120/60 mmHg in a sitting position before dialysis, and rose to 200/90 mmHg in a supine position soon after the start of dialysis. It decreased to 90/50 mmHg upon standing after dialysis, and thereafter the patient could hardly stand up for the rest of the day. The application of transdermal nitroglycerin (Nitroderm TTS^®) at the start of dialysis was effective for preventing the increase in supine blood pressure during dialysis, with the blood pressure maintained at 150/80 mmHg. Long-term administration of oral amezinium metilsulfate (Risumic^® 10 mg) was ineffective for preventing orthostatic decrease in blood pressure. Only slight improvement was observed with the administration of oral droxidopa (DOPS^® 100 mg) before and after the end of dialysis. The additional administration of an oral acetylcholinesterase inhibitor, distigmine (Ubretid^®), before the end of dialysis significantly reduced the incidence of general malaise and gait disturbance. Symptoms were markedly improved to the extent that the patient could perform daily life activities, including shopping and household chores. The coexistence of supine hypertension and orthostatic hypotension is not rare in diabetic hemodialysis patients. The application of transdermal nitroglycerin at the start of dialysis prevented the increase in supine blood pressure during dialysis. The use of acetylcholinesterase inhibitors to treat orthostatic decrease in blood pressure at the end of dialysis led to an improvement in the patient's QOL.

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A 62-year-old woman with no contributory medical history transferred to our hospital. Three years before presentation, she had complained of sausage-like fingers, Raynaud's phenomenon, digital-tip ulcers and decreased wrinkles around her mouth. One year before presentation, she had dyspnea on effort. Though Chinese herbal medicine was administered, these symptoms did not improve. Three days before presentation, she had been admitted to a local hospital because of orthopnea. On admission, hypertension with papilledema, massive pericardial effusion, congestive heart failure, pulmonary fibrosis, red cell fragmentation on peripheral blood smear and renal impairment were present. On anti-hypertensive therapy, blood pressure decreased to 150/80 mmHg and red cell fragmentation on peripheral blood smear disappeared. However, renal dysfunction and edema of extremities developed and she transferred to our hospital. On admission, skin scleroderma, skin pigmentation, systolic murmur, fine crackles and edema of extremities were present. Urinalysis showed both proteinuria and microhematuria and chest X ray showed cardiomegaly and lung congestion. Echocardiography demonstrated massive pericardial effusion and chest computed tomography scan showed mild interstitial change of the bilateral lower lung fields. Taking positive anti-Scl-70 antibody into consideration, she was diagnosed with scleroderma renal crisis and congestive heart failure with pericardial effusion. Both ACE inhibitor and hemodialysis were started and blood pressure before one session of hemodialysis decreased to 138/68 mmHg. Since congestion and edema of extremities improved, she was discharged from hospital on the 27^th hospital day. The frequency of scleroderma renal crisis is lower in Japan than in western countries. We report a case of severe scleroderma renal crisis with pericardial effusion that progressed to end stage renal disease.

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A 73-year-old man was admitted to our hospital because of fever, proteinuria, microscopic hematuria and elevated serum creatinine level (0.9→2.3 mg/dL) in July 2008. MPO-ANCA was detected in serum (83 EU), and renal biopsy showed pauci-immune crescentic glomerulonephritis. He was diagnosed with MPO-ANCA-related RPGN and was treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone. Serum creatinine and MPO-ANCA titers decreased to 1.2 mg/dL and less than 10 EU, respectively. With tapering of the prednisolone dose, he was followed in the outpatient department. In January 2010, the titer of MPO-ANCA was re-elevated to 212 EU. In April 2010, serum creatinine was elevated to 4.5 mg/dL, and he was readmitted on May 17, 2010. Moreover anti-GBM antibody was detected (50 EU), and he received intravenous methylprednisolone pulse therapy to treat RPGN followed by oral prednisolone, and plasma exchange was initiated. Hemodialysis was performed because serum creatinine had increased to 6.1 mg/dL, but MPO-ANCA and anti-GBM antibody titers both decreased to less than 10 EU. From the beginning of July, his platelet count remained low, and fragmentation of red blood cells on peripheral blood smear and decreased haptoglobin levels were observed. A diagnosis of TTP was made, and plasma exchange was reinitiated. Plasma exchange was repeatedly performed in addition to administration of prednisolone, but the disease became resistant to extensive plasma exchange, leading to the death of the patient from pancreatitis and MOF on August 14. There have been several reports of a combination of TTP and either ANCA-related disease or anti-GBM antibody-related disease, but the combination of TTP and double antibody-positive RPGN is very rare. Here, we report a patient with double antibody-positive crescentic glomerulonephritis who developed refractory TTP.

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