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A 60-year-old man who had been receiving dialysis for more than 30 years was admitted for treatment of cellulitis in his right thigh on November 7, 2003. He suffered from an ileus on December 14 and was found to have a huge, 7-cm-diameter, well-circumscribed fecalith, incarcerated at the splenic flexure of the colon. It was proving difficult to pass this naturally and surgical removal was thought to be too risky. Using a colonoscope and a water-jet probe, the fecalith was broken up; the ileus then improved and the patient was able to take oral fluids. Unfortunately, he died of cardiac failure on February 13, 2004. We conducted an autopsy, with his family's consent, and found generalized amyloidosis. Deposits of amyloid were seen in all layers of the colon. Because of this, we hypothesized that peristalsis had been poor and this had led to paralytic ileus due to stasis, which, in turn, had led to the formation of the huge fecalith. In Japan it is not rare for a patient to be on dialysis for more than 25 years and it may be that this is a cause of generalized amyloidosis. There have been no such cases of fecalith associated with gastrointestinal amyloidosis described previously, which is why we decided to report this case here.

Postmenopausal women on maintenance haemodialysis (MHD) has considerably higher risk of bone fracture than general population with combination of postmenopausal osteoporosis and renal osteodystrophy. However, the treatment of osteoporosis on MHD has not been established. Evidence indicates raloxifene (RLX), a selective estrogen receptor modulator, is effective for a protection of bone fracture without increasing of breast cancer and endometrial cancer. We hereby report short-term use experience of RLX for the postmenopausal MHD patients. Fifteen postmenopausal MHD patients with less than 80% of YAM bone density in DEXA administrated 60 mg RLX on every HD days (3 days/week). Serum NTX level significantly decreased after 6 months (180 +/- 18 vs. 95 +/- 12 nmol/BCE/L, p< 0.05), however, i-PTH did not have the significant difference. (115 +/- 23 vs. 157 +/- 29 pg/mL). RLX is effective for bone biomarker improvement in postmenopausal MHD patients. Further evaluation for the effectiveness and safety of RLX is necessary in the long term.

Thirteen patients, who received parathyroidectomy within 5 years after the initiation of hemodialysis, were discussed on their clinical characteristics and their prognoses. 204 patients received the first parathyroidectomy due to secondary hyperparathyroidism in our department, 13 of which were selected on dialysis duration. 9 patients were female and origin of CRF were analgesics-induced interstitial nephritis in 2 and prune-belly syndrome in 1. There were 2 patients who suffered from renal disease more than 30 years. Concomitant primary hyperparathyroidism was suspected in 1 case. In all cases, PTx achieved adequate control of 2HPT after the operation.

Elevated parathyroid hormone (PTH) levels and hyperphosphatemia are thought to be associated with the development of calciphylaxis. We report a patient on hemodialysis who developed proximal calciphylaxis with consistently low PTH levels after parathyroidectomy. A 31-year-old man was admitted to our hospital because of abdominal skin ulcerations. Calciphylaxis spread to the penis, and simultaneous progressive lung calcification was evident on chest X-ray, suggestive of pulmonary calciphylaxis on 99mTc-methylene disphosphonate scintigraphy. The patient died of respiratory failure despite intensive treatment including hyperbaric oxygen therapy. This is the first report of a patient on hemodialysis who developed calciphylaxis involving the penis after parathyroidectomy.

Aims: It has been reported that taste acuity for the four primary tastes, sour, sweet, salty and bitter, is impaired in hemodialysis (HD) patients. However, there have been no studies reported on taste acuity of diabetic HD patients. The present study aimed to quantify and compare the taste acuity of diabetic and non-diabetic HD patients, and further to determine if there were correlations between diminished taste acuity and certain blood serum parameters typically askew in hemodialysis patients., Methods: In a test group of 24 diabetic and 24 non-diabetic HD patients matched for age, body mass index and duration of HD, taste acuity for the four tastes was determined by asking patients to identify them at varying concentrations., Results: Statistical analyses indicate that bitter and total taste acuity were significantly impaired in diabetic HD patients. In diabetic HD patients, correlation was found between sweet, salty or total taste acuity and blood urea nitrogen or normalized protein catabolic rate., Conclusions: We conclude that taste acuity is partially impaired in diabetic HD patients, and suggest this contributes to reduced appetite, leading to malnutrition and poor prognoses., (Copyright 2003 S. Karger AG, Basel)

Dialysis-related amyloidosis (DRA) is one of the most serious complications interfering with rehabilitation in dialysis patients. Here, we report a case of beta2-microglobulin (beta2M)-related amyloidosis, in which the patient developed a severe intestinal pseudo-obstruction. The patient was a 42-year-old male who had been undergoing hemodialysis for 13 years, and who had no history of osteoarticular involvement of DRA. The first symptoms of the disease were severe abdominal fullness and nausea after meals. The whole intestinal wall biopsy revealed massive amyloid deposition in the propria muscularis. The patient became malnourished and died of acute subendocardial infarction 3 years after the onset. An autopsical examination revealed a massive deposition of amyloid, which was positively stained with anti-beta2M antibody but not AA amyloid, predominantly in the gastrointestinal muscular layer, including the tongue, esophagus, stomach, small intestines, colon, and rectum. These results suggest that the gastrointestinal involvement of beta2M-related amyloidosis might occur during the course of hemodialysis treatment, and that this possibility should be considered if patients suffer from intestinal pseudo-obstruction without osteoarticular symptoms.

The clinical results of open surgical treatment for hemodialysis-related arthropathy in the shoulder were evaluated, and the pathology was investigated. Fifteen shoulders of 11 patients (6 female and 5 male) were treated during the past 6 years. The average age was 56.7 years. The duration of hemodialysis averaged 17.5 years. Simple curettage was performed in 7 shoulders, and curettage and bone graft were performed in 5 shoulders. Repair of the rotator cuff was performed in 3 shoulders. Pathologic foci always existed in the intertubercular sulcus and the greater tuberosity of the humerus. Marked proliferations of the bursa and multiple cystic bone lesions were found, and amyloid depositions were observed on histologic evaluation in all cases. Open surgical treatment was quite effective, and relief from the clinical symptoms was maintained for a satisfactory period of time. Enthesopathy induced by the amyloid deposition is considered the main pathology.

Background: Advanced glycation end product-modified beta2-microglobulin (AGE-beta2m) is an important component of dialysis-related amyloidosis (DRA). Its presence induces monocyte chemotaxis and the release of the proinflammatory cytokines through macrophage activation. Transforming growth factor-beta (TGF-beta) is a multifunctional cytokine that also has chemotactic activity for monocytes at very low (0.1 to 10 pg/mL) concentrations and inhibits proinflammatory cytokine production of macrophages. In this study, we investigated the role of TGF-beta in the pathogenesis of DRA., Methods: We performed an immunohistochemical study of DRA tissues (8 cases) to confirm the existence of TGF-betas and their receptors; we also performed a chemotaxis assay of human monocytes as well as enzyme-linked immunosorbent assay (ELISA) of TGF-beta1, tumor necrosis factor-alpha (TNF-alpha), interleukin-1beta (IL-1beta), and interleukin-1 receptor antagonist (IL-1Ra) in the supernatant of human monocyte-derived macrophage cell culture under varying conditions of incubation with TGF-beta1, AGE-beta2m, and TGF-beta1 antibody additions., Results: There was positive staining for TGF-betas (types 1, 2, and 3) and their receptors (types I, II, and III) in infiltrated macrophages (CD68+), synovial lining cell, as well as vascular walls around amyloid deposition. AGE-beta2m also induced TGF-beta1 production by macrophages in a dose-dependent manner (410 +/- 80 pg/mL at 12.5 microg/mL, 621 +/- 62 pg/mL at 25 microg/mL, and 776 +/- 62 pg/mL at 50 microg/mL of AGE-beta2m). AGE-beta2m induced significant TNF-alpha and IL-1Ra production by macrophage. The addition of exogenous TGF-beta1 (0.1 to 10 ng/mL) decreased AGE-beta2m-induced TNF-alpha production and increased IL-1Ra production in a dose-dependent fashion. IL-1beta production was not effected by any experimental conditions. In chemotaxis assay, anti-TGF-beta1 antibody (0.1 to 10 microg/mL) attenuated AGE-beta2m-induced monocyte chemotaxis., Conclusions: These results provide the first evidence to our knowledge for the presence of TGF-beta in DRA tissue, as well as the stimulatory action of AGE-beta2m on tissue macrophages. In turn, TGF-beta suppresses the proinflammatory activation of macrophages, suggesting a dual role for TGF-beta in the inflammatory process of DRA. These observations may provide a pathophysiologic link between TGF-beta and DRA.

Background: Parathyroid hormone (PTH) has an adverse effect on the immune system and may cause immunologic disorders in patients with chronic renal failure. The in vivo effects of a parathyroidectomy on the immunologic parameters was examined., Methods: Thirty-four patients under dialysis therapy received a parathyroidectomy (PTx) for secondary hyperparathyroidism (HPT). They were prospectively studied regarding serum immunoglobulins, complements, CD markers, and serum soluble IL-2 receptor (sIL-2R) until 12 months after PTx., Results: The serum levels of IgG, IgA and IgM showed significant increase until 12 months after PTx (P<0.001, respectively). C3, C4, and CH50 also indicated significant increase at 12 months after PTx. In cellular immunity, only serum sIL-2R showed significant increase 2 weeks after PTx (P = 0.028). The hematocrit and serum albumin also improved significantly at 12 months., Conclusions: PTx showed beneficial effects on humoral immunological markers. The effects are probably due to the remarkable PTH reduction and partly improved nutritional state after PTx.

We reviewed 546 operative cases in haemodialysis patients during the past 8 years between June 1990 and May 1998. The average age of the 257 male and the 289 female patients was 57.1 years. The average period of haemodialysis was 14 years and 4 months. We discussed the etiology, pathological focus, frequency, symptoms, problems and operative indications. Carpal tunnel syndrome occurred most frequently, occupying 289 cases. Short-term postoperative results were positive in the initial case within 6 months after the occurrence of the first symptoms. Because no effective method preventing recurrence existed, synovectomy was used as much as possible in the initial operation. Surgical treatment was used for amyloid arthropathy in 15 shoulders. It was effective in cases resistant to any conservative treatment. For the knee joint, the arthroscopic synovectomy was performed in 8 cases, and total knee replacement in 6 cases. The results of the total knee replacement cases were good. However, recurrences were observed in 40% of the synovectomy cases. In the hip joint, the curettage and bone grafting were performed in 7 hips of 6 patients. All bone grafts were consolidated and there was no case of postoperative pathological fracture. In some cases with destructive spondyloarthropathy, the vertebrae involved spontaneously fused without severe kyphotic deformity. A case with mild pain and without neurological deficit can be treated conservatively. A case with severe pain, instability, and myelopathy indicates operative measures must be taken. Attention must be paid for a possible collapse of the grafted bone after the cervical level two anterior fusion. Haemodialysis patients are vulnerable to infection. This diagnosis is difficult to distinguish due to the weakened state of the immune system. Rigorous attention is required to prevent and detect infection in cases using artificial joints or instrumentation. The treatment of bone and joint disturbances from dialysis-related amyloidosis has become surgical due to the advancement of dialysis control. However, it remains one of the more conservative treatments. Determining the cause and establishing a method of treatment are desired as early as possible.

In dialysis patients, the prevalence of severe left ventricular (LV) hypertrophy and systolic failure, important predictors of cardiovascular morbidity and mortality, has been reported to be very high. Therefore, we investigated cardiac function in 17 long-term CAPD patients (dialysis duration: 76.5 +/- 13.2 months; L-CAPD) by echocardiography and pulsed Doppler, and then compared with 16 short-term CAPD patients (dialysis duration: 28.9 +/- 11.9 months; S-CAPD), 21 long-term hemodialysis patients (dialysis duration: 165.1 +/- 52.7 months; L-HD), and 22 short-term hemodialysis patients (dialysis duration: 71.3 +/- 28.9 months; S-HD), except for the cases with diabetes mellitus, ischemic heart disease, cardiac surgery or overt congestive heart failure. We selected 13 normotensive patients with normal kidney function as normal control group matched for sex and age (Control). Concerning with L-CAPD, S-CAPD, L-HD, and S-HD, these four groups were matched for age and original diseases. We examined blood pressure (BP), cardiothoracic rate (CTR), antihypertensive (AHT) drugs and laboratory data. Wall thickness, left atrium, ventricular chamber size, ejection fraction (EF) and left ventricular mass (LV mass) [Devereux et al. 1986] were measured by echocardiograph. Peak early diastolic flow velocity (E), peak atrial filling velocity (A), A/E ratio and deceleration time of peak early diastolic flow velocity (DT) were calculated by analyzing transmitral flow, recorded by pulsed Doppler. BP control, CTR and EF were significantly worse in L-CAPD than in other patient groups. A/E as one of parameters for cardiac diastolic function was significantly higher in L-CAPD than in HD patients. LVMI (LV mass index: LV mass/body surface area) was significantly higher in L-CAPD than in other groups. LVMI in CAPD patients was shown to be significantly worse as time goes. Volume control by itself without AHT drugs could achieve good BP control in the long-term CAPD patients who were changed to maintenance hemodialysis because of peritoneal sclerosis. We concluded that LV hypertrophy and systolic dysfunction tend to progress in CAPD patients as time goes on. Also it is suggested that the cause of cardiac dysfunction in CAPD patients was mainly based on poor BP control probably due to overhydration, and therefore, appropriate volume control in CAPD patients is especially important.

Background: Incidences of the variety of postoperative complications in surgical treatment for patients with chronic renal failure on hemodialysis is the clinically accepted opinion. The aim of this study was to ascertain the safety of surgical treatment for patients of biliary lithiasis with chronic renal failure on hemodialysis., Methods: Eleven consecutive patients with biliary lithiasis, who had a history of chronic renal failure treated with hemodialysis, were selected. The peri-operative course in-each of these cases was investigated., Results: There was no incidence of peri-operative problems and postoperative complications in cases treated with laparoscopic cholecystectomy. One case treated with cholecystectomy with laparotomy had a slight degree of postoperative pneumonia, which soon improved after administration of antibiotics. Only one case, treated with choledochotomy, resulted in operative death due to accidental septic shock from an unknown origin., Conclusions: Surgical treatment of patients with biliary lithiasis and chronic renal failure on hemodialysis can be safely performed with appropriate operative indications and noninvasive surgical techniques.

Dialysis-related amyloidosis (DRA) predominantly occurs in the osteoarticular structures. However, according to studies in the increasing number of long-term hemodialysis patients, DRA has also been systemically found to appear in the other tissues and organs as well. In this study, we investigated lingual amyloidosis in relation to systemic DRA. A total of 472 patients were studied who were on regular hemodialysis for more than 10 years, including 103 patients for more than 20 years. Eight of these patients (7 males and 1 female, mean age 59 +/- 8 years, range 46 to 68 years) developed lingual amyloidosis, seemingly as a result of beta2-microglobulin (beta2m) deposits. All patients demonstrating lingual amyloidosis had been treated with regular hemodialysis for more than 20 years (mean HD duration 23.6 +/- 1.4 years), and its morbidity was 7.8% in the 103 patients and 20% (6 patients) in the 30 patients treated for more than 23 years with hemodialysis. Hemodialysis (HD) duration with bioincompatible unsubstituted cellulose membranes in the 8 patients was longer than that in the control group without lingual amyloidosis (P < 0.05). Lingual amyloid nodules were whitish-yellow in color and varied in size, at least over 1 mm in diameter. Their consistency was firmer than the intact tongue. The location of the amyloid nodules could be classified into two types: (1) diffuse type (diffusely distributed over the tongue), and (2) lateral type (localized only in the lateral side of the tongue). Five of the eight patients with lingual amyloidosis complained of functional disturbances in the tongue, such as abnormal taste, or difficulty in mobility and articulation. No macroglossia was observed in any of these cases. It was thus concluded that DRA of the tongue is a very rare complication, occurring in the late stage of long-term hemodialysis patients, that disturbs their quality of life.

Superficial repositioning of the artery (SRA) is a modality of the blood access operation for chronic hemodialysis that has been previously used in cases of cardiac failure. We performed 42 SRAs from 1986 to 1993; thereafter, we retrospectively investigated the operative indications, postoperative complications, and long-term results. Superficial repositioning of the artery was indicated for the lack of an appropriate vein (17 cases; 40%), frequent and early access failure due to arteriovenous fistula or polytetrafluoroethylene grafts (six cases; 14%), venous hypertension (five cases; 12%), and cardiac failure (two cases; 5%). The patency rates of the SRAs were 87% at 3 years and 58% at 4.5 years. There was some difficulty in finding the returning veins in five of 28 functioning SRAs (18%). The SRA is thus considered to be a secondary-selected blood access operation; however, it also may be used as an efficient blood access for an extended period of time without any serious complications.

We retrospectively reviewed 17 hip arthroplasties in 15 patients having haemodialysis for chronic renal failure. The duration of haemodialysis before the operation averaged 8.6 years and the average age of the patients was 61 years. All patients were followed for more than two years (mean 4.6 years). Six arthroplasties in four patients had failed due to loosening, and one of these patients died from undiagnosed infection of both hips at 7.6 years after the operation. General skeletal abnormalities caused by maintenance haemodialysis may explain the high incidence of loosening and it is important to be aware of the danger of postoperative infection. The risk-to-benefit ratio of hip arthroplasty is high in patients on haemodialysis.

Hemodynamic changes in diabetic patients were studied in both acetate and bicarbonate modes of hemodialysis (HD) where both body weight and plasma volume did not change. Diabetic patients showed a significant decrease in mean arterial pressure and cardiac output in both HD modes when compared to the predialysis values. Despite a decreased cardiac output, total peripheral vascular resistance did not increase, probably because of the sympathetic nerve dysfunction in diabetic patients. In addition, diabetic patients with acetate HD demonstrated peripheral vasodilation at a later stage of acetate HD. Increase in plasma acetate may have caused peripheral vasodilation in these patients. It is concluded that intradialytic hypotension in diabetic patients is mainly associated with a decrease in cardiac output and loss of compensatory increase in peripheral vascular tone.

We reviewed 29 patients who had developed destructive arthropathy of the spine during long-term haemodialysis. Their mean age when haemodialysis began was 43.8 years; at diagnosis they had been dialysed for an average of 8.6 years. In 26 patients, the lesions were between C4 and C7; in six they were between L4 and S1, three having lesions in both regions. Sixteen patients had had previous surgery for carpal tunnel syndrome. Spinal surgery was performed in nine patients with satisfactory results in only five. We demonstrated beta-2 microglobulin amyloid deposits in the discs and surrounding ligaments in all biopsied cases. The natural history and management of this condition are not yet clear.

After successful subtotal parathyroidectomy (PTX) in 10 chronic haemodialysis patients, significant elevation of Epo was observed, from 48.4 +/- 17.8 mU/ml(M +/- SEM) at preoperative state to 103.3 +/- 34.7 mU/ml at 6 h and 163.4 +/- 50.2 mU/ml at 12 h after PTX. Significant reductions in both PTH-m and ionized calcium (iCa) were confirmed. Since Epo did not increase in the cases with an inadequate PTX and ovariectomy, an abrupt reduction in PTH with a decrease in iCa may play some role in the elevation of Epo.