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1.
Recently, there have been reports of beta 2-microglobulin (beta 2 m) related amyloid deposition in perineural and periarticular tissues in patients receiving long-term hemodialysis, but it has been rarely described in bone. We, therefore, examined previously obtained bone biopsy specimens in patients receiving long-term hemodialysis to determine the prevalence of beta 2 m deposition in bone and to assess the relationship between beta 2 m deposits and bone histomorphometry. We found beta 2 m deposits in bone in 8% of 224 patients examined. Bone deposition of beta 2 m was absent in patients who were on dialysis for less than six years, but was present in 19% who dialyzed longer than 10 years. beta 2 m deposits were found in specimens from the iliac crest, femoral bone, tibia, vertebra and rib. In the iliac crest beta 2 m deposition was localized predominantly to the periosteum. Among these patients with beta 2 m in iliac crest periosteum, 62% had suffered a femoral neck fracture compared to only 4% of matched patients who had negative staining for beta 2 m in the iliac crest (P less than 0.001). Histologically, osteitis fibrosa seemed more common in patients positive for beta 2m than in patients negative for beta 2m deposition. We conclude that beta 2m deposition in bone is common in uremic patients who have received hemodialysis longer than 10 years. The high prevalence of femoral neck fracture in patients with beta 2m localized to the periosteum of the iliac crest suggests that this involvement may be useful to predict susceptibility to femoral fracture.  相似文献   

2.
beta 2-microglobulin (beta 2M) has been implicated in the pathogenesis of amyloidosis in long-term dialysis patients. beta 2M levels were measured in patients with chronic renal failure: before and after conventional hemodialysis in 30, before and after high-flux (HF) hemodialysis in 35, and during the first hemodialysis treatment in five patients, as well as in the serum and peritoneal fluid of 13 patients who were receiving continuous ambulatory peritoneal dialysis (CAPD) and in the serum and urine of three patients who had received kidney transplants. Dialysis patients had markedly elevated beta 2M levels; prehemodialysis values were not significantly different for patients receiving conventional v HF hemodialysis. Most of these patients were functionally anephric, and the beta 2M levels did not correlate with age, sex, or time on dialysis. In patients receiving conventional hemodialysis using cellulose acetate membrane, beta 2M levels increased 25.4% after hemodialysis, whereas in patients receiving HF hemodialysis using polysulfone membrane, beta 2M levels decreased significantly (43.0%) after hemodialysis. End-stage renal disease patients dialyzed for the first time had beta 2M values significantly lower than the other two groups because of residual glomerular filtration rate (GFR). CAPD patients also had lower values because they had an estimated loss of 80.4 mg/d of beta 2M in the dialysate fluid. In patients with chronic renal failure, beta 2M levels paralleled the increase in serum creatinine. Patients who received kidney transplants had a dramatic decrease in beta 2M levels that correlated with improvement in GFR. beta 2M correlated with the residual GFR, and its removal was membrane-dependent.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   

3.
We describe a 69-year-old male patient on maintenance hemodialysis for 24 years who developed a fatal left psoas abscess with osteomyelitis at the hip joint following acute enterocolitis. He had systemic beta(2)-microglobulin amyloid deposition in colon epithelium and psoas muscle. Cultures from abscess fluid and femoral bone marrow yielded Bacteroides fragilis. To our knowledge, this is the first case on hemodialysis having a psoas abscess following acute gastrointestinal infection. This rare case suggested that a secondary psoas abscess could be one of the occult infections in patients undergoing long-term maintenance hemodialysis.  相似文献   

4.
The diagnosis of dialysis-related amyloid (AB-amyloid) has been based usually on clinical and radiological criteria. Following the discovery that beta 2-microglobulin was the major protein of this amyloid, we isolated and radiolabelled uremic plasma beta 2-microglobulin. After intravenous injection, gamma-camera images of selected joint areas were obtained from 42 patients who were on regular hemodialysis therapy. Positive scans involving the shoulder, hip, knee and carpal regions were found in 13 of 14 patients treated for more than 10 years and 10 of 16 patients treated for 5 to 10 years. Patients treated for less time had negative scans. Specificity was indicated by negative scans in non-amyloid inflammatory lesions in control hemodialysis patients. Up to 48-fold tracer enrichment was detected in excised AB-amyloid containing tissue as compared to amyloid-free tissue. These findings suggest that circulating radiolabelled beta 2-microglobulin is taken up by the amyloid deposits. This method may non-invasively detect tissue infiltrates of amyloid. It may also permit prospective evaluation of the efficacy of prophylactic dialysis strategies which are designed to prevent or delay the onset of this complication of long-term dialysis.  相似文献   

5.
Since Warren and Otieno reported carpal tunnel syndrome in patients on intermittent hemodialysis in 1975, a number of related reports have been published. However, there are few reports associated with neurosurgery about carpal tunnel syndrome in patients on long term hemodialysis. We reviewed this disease and reported our case. We treated a patient who complained of bilateral hand numbness and atrophy of the right thenar muscle. He had been suffering from chronic renal failure and had been treated with hemodialysis for ten years. We diagnosed carpal tunnel syndrome based on the findings concerning Tinel's sign, Phalen test, and the conduction velocity of the median nerve. We performed decompression surgery of the median nerve. However, although there was no recovery from thenar muscle atrophy, there was improvement of hand numbness. Histologically, amyloid deposits within the hypertrophic transverse carpal ligament on the right side, could be found but on the left side where the internal shunt had been made amyloid deposits were absent. The reason why patients receiving long term hemodialysis develop carpal tunnel syndrome is controversial, but it seems that beta 2 microglobulin may play an important role in developing carpal tunnel syndrome in hemodialysis patients. This was reported by Gejyo in 1985. There may be uremic and/or diabetic neuropathy in these patients, and these neuropathies may be responsible for the more rapid deterioration and poorer surgical results in carpal tunnel syndrome associated with hemodialysis than in idiopathic cases. It is most important that carpal tunnel syndrome has to be diagnosed early and that surgical decompression is performed while the disease is in its early stage.  相似文献   

6.
These studies were designed to track the cutaneous deposition of beta 2-microglobulin (beta 2M) in patients on chronic hemodialysis, patients with chronic renal insufficiency and patients with successful renal transplants. Immunoperoxidase staining of skin biopsies from dialysis patients demonstrated significantly increased amounts of beta 2M compared to controls (p < 0.01). There was a strong positive correlation between the skin beta 2M content and the years of dialysis treatment. Renal transplant recipients had decreased skin content of beta 2M compared to hemodialysis patients. There was no difference in the skin beta 2M content in patients with chronic renal insufficiency not on hemodialysis and controls. No dialysis patient had amyloid in the skin by Congo red stain. We conclude that beta 2M accumulates in the skin of patients on chronic hemodialysis. This beta 2M is not in the form of amyloid. Successful renal transplantation allows for the removal of beta 2M from the skin indicating that beta 2M not in the form of amyloid can be mobilized from tissue sites.  相似文献   

7.
Amyloid urinary-tract calculi in patients on chronic dialysis   总被引:1,自引:0,他引:1  
Urinary calculi found in 4 patients on chronic hemodialysis or continuous ambulatory peritoneal dialysis (CAPD) were identified as protein calculi by infrared spectroscopic analysis. Positive Congo red staining and immunological assessment revealed that the calculi were composed of amyloid protein derived from beta 2-microglobulin. A comparison of the patients who excreted calculi with 10 patients on chronic dialysis without urinary calculi showed no significant differences in the urinary and serum levels of beta 2-microglobulin. The mechanism of amyloid calculus formation may involve factors independent of the concentration of beta 2-microglobulin in urine or serum. Urinary calculi found in patients on chronic hemodialysis or CAPD were composed of amyloid protein derived from beta 2-microglobulin.  相似文献   

8.
beta 2-Microglobulin (beta 2M)-derived amyloidosis has become a major concern in long-term hemodialysis patients. Clinical symptomatology is largely restricted to the articular and periarticular sites and in rare cases systemic manifestations have been described. We present a long-term hemodialysis patient, who after 16 years of hemodialysis with regenerated cellulosic membranes not only had a bilateral carpal tunnel syndrome, cystic bone translucencies and humeroscapular periarthritis but also developed two subcutaneous tumors in both gluteal regions, causing discomfort when sitting. Histology, immunohistology and electron microscopy of the tumor from the right side showed that it consisted of beta 2M-derived amyloid with concurrent scattered amyloid infiltration of the overlying skin. This report therefore adds a new clinical manifestation to the symptomatology of this type of amyloid.  相似文献   

9.
Serum from 8 undialyzed patients and 30 dialyzed patients was examined by immunoblotting using anti beta 2-microglobulin (beta 2M) serum after two-dimensional gel electrophoresis (2.DE). One major spot and three minor spots were detected in the ultrafiltrate as well as in the serum. One major spot was determined to be native beta 2M and three minor spots were found to be novel beta 2M. Novel beta 2M had a lower molecular weight (MW) and a higher acidic isoelectric point (pI). Novel beta 2M was recognized in the sera of 5 out of 20 hemodialysis (HD) patients without carpal tunnel syndrome (CTS), 2 of whom had been on HD from 5 to 10 years and 3 for more than 10 years, as well as in the sera of all 10 patients with CTS. By chromatofocusing, pI of novel beta 2M was 5.2, while pI of native beta 2M was 5.7. When the tissue specimen of transverse carpal ligament of 2 HD patients with CTS was examined by immunoblotting after 2.DE, the spot of novel beta 2M was larger than that of native beta 2M. It is possible that some metabolic abnormality of beta 2M occurs through long-term hemodialysis, and it is possible that novel beta 2M might relate to amyloidogenic predisposition.  相似文献   

10.
Beta 2-microglobulin (beta 2M) amyloidosis is common in patients on long-term hemodialysis, but the clinical conditions associated with disease activity are poorly understood. This study was designed to determine if the serum amyloid P (AP) component concentration is predictive of beta 2M amyloid disease activity. Serum AP component concentrations were determined by rocket immunoelectrophoresis and beta 2M concentrations by a commercially available kit. Radiographic evidence of beta 2M amyloidosis was determined from bone films of the hips, shoulders, and hands. Serum AP component concentrations were not different in dialysis and control patients. However, AP component concentrations in long-term (greater than or equal to 5 years) dialysis patients were significantly lower than in short-term (less than 5 years) dialysis patients (43.0 +/- 16.9 micrograms/mL [n = 28] v 56.0 +/- 18.3 micrograms/mL [n = 31], P less than 0.05). The patients on hemodialysis for 5 or more years who had radiographic evidence of severe beta 2 M amyloidosis were significantly older (57.9 +/- 9.5 v 38.3 +/- 11.3 years, P less than 0.001) and their serum AP concentrations were significantly lower (34.3 +/- 15.0 v 50.1 +/- 15.6 micrograms/mL, P less than 0.05) than long-term dialysis patients without radiographic evidence of disease. Stepwise regression analysis showed that the patient's age and serum AP component concentration were predictors of radiographic evidence of beta 2 M amyloidosis. Thus, serum AP component concentrations are decreased in long-term dialysis patients, suggesting accelerated deposition into amyloid deposits.(ABSTRACT TRUNCATED AT 250 WORDS)  相似文献   

11.
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.  相似文献   

12.
Background and purpose Whether or not uncemented total hip arthroplasty (THA) can achieve durable fixation of implants to bone in patients on chronic hemodialysis is unknown. We analyzed the 2–13-year clinical outcomes of cementless THA in patients with end-stage renal diseases who were maintained on long-term hemodialysis.

Patients and methods We reviewed the outcome of 23 consecutive uncemented THAs undertaken between 1993 and 2004, in patients with chronic renal failure who had been on long-term hemodialysis (2–18 years). 1 patient died and 2 patients were lost to follow-up within 2 years, leaving 20 hips (20 patients, median age 66 (38–81) years at the time of THA, 11 females) that were reviewed at median 7 (2–13) years postoperatively.

Results Radiographic bone-ingrowth fixation of the components was found in 19 patients. 1 patient had aseptic loosening requiring revision surgery. The median d'Aubigne and Postel score was 10 (8–14) preoperatively and 15 (12–18) at final review. No prosthetic infections were found in any of the patients.

Interpretation Uncemented THA shows promising medium-term results in patients receiving long-term hemodialysis.  相似文献   

13.
Postmortem examinations were carried out in 13 patients (6 males, 7 females, age 58 +/- 9 years) who had been on regular hemodialysis treatment for 10-90 months using disposable regenerated cellulose membrane dialyzers. The prevalence of beta 2-microglobulin (beta 2m)-derived AB-amyloid deposits in different sites was determined. At autopsy, specimens were obtained from different joints, paravertebral tissue, intervertebral discs and from visceral organs. During life, routine laboratory parameters and radiographic studies had been carried out at 6-month intervals. Serum levels of beta 2m were elevated in all patients (57.5 +/- 13.4 mg/l). Synovial AB-amyloid deposits were shown in different joints of 4 patients, aged between 59 and 73 years, and dialysed for 10-90 months, respectively. All had been unremarkable by X-ray and asymptomatic. No amyloid could be detected in the intervertebral discs of 2 further patients suffering from destructive spondylarthropathy. In 11 of the 13 patients, extracellular beta 2m deposits were observed by immunohistochemistry in different tissues. The results document that (a) AB-amyloidosis may occur in elderly patients even when dialysed for less than 5 years; (b) most cases are completely asymptomatic; the appearance of symptoms must be dependent on additional factors, e.g., site of AB-amyloid deposition and intensity of inflammatory reaction, and (c) AB-amyloid is not the exclusive cause of destructive spondylarthropathy, as 2 typical cases were observed who were devoid of amyloid.  相似文献   

14.
BACKGROUND: End-stage renal disease (ESRD) patients undergoing hemodialysis treatment have reduced plasma L-carnitine levels; however, the relationship between dialysis age and carnitine status is poorly understood. This study examined the relationship between duration of dialysis and plasma and skeletal muscle concentrations of L-carnitine and its esters in ESRD patients. METHODS: Blood samples were collected from 21 patients at baseline and throughout the first 12 months of hemodialysis. In 5 patients, muscle samples were obtained after 0, 6, and 12 months of hemodialysis. Blood and muscle samples were collected from an additional 20 patients with a mean dialysis age of 5.10 years. L-carnitine, acetyl-L-carnitine, and total L-carnitine were measured by high-performance liquid chromatography (HPLC). RESULTS: The mean +/- SD plasma L-carnitine concentration in ESRD patients who had not yet started hemodialysis was 50.6 +/- 20.0 micromol/L. Significantly lower concentrations were observed after 12 months (29.7 +/- 10.5 micromol/L) and >12 months (22.0 +/- 5.4 micromol/L) of hemodialysis treatment. Acetyl-L-carnitine also declined with dialysis age, while plasma nonacetylated acylcarnitines continued to increase with the progression of hemodialysis therapy. An inverse relationship between dialysis age and muscle L-carnitine concentrations was observed. CONCLUSION: Long-term hemodialysis treatment is associated with a significant reduction in endogenous plasma and muscle L-carnitine levels and a significant increase in plasma acylcarnitines. The majority of the change in plasma L-carnitine concentrations occurs within the first few months of hemodialysis, while muscle levels continue to decline after 12 months of treatment.  相似文献   

15.
Tissue distribution of dialysis amyloidosis   总被引:7,自引:0,他引:7  
Twenty-three uremic patients on intermittent hemodialysis for eight to eighteen years provided the material for the present pathological study. In all of them, there was evidence for dialysis related amyloidosis based on previous clinical or histological findings or both. The material examined consisted of nine skin biopsies, five abdominal fat aspirates, eight trans-iliac bone biopsies and numerous post-mortem specimens of various visceral organs from eight cases. None of the skin biopsies or fat aspirates showed amyloid deposits. In only one bone biopsy could a small Congo red positive area be recognized that showed characteristic birefringence under polarizing light. Autopsy material findings were negative except for one case: this patient had been dialyzed for 18 years. Very minute amyloid deposits with a positive immunofluorescence staining for beta 2-microglobulin (beta 2-M) were found in the walls of small vessels from her lung, heart, liver and intestine. Thus, in chronic hemodialysis patients the accumulation of beta 2-M amyloid fibrils in tissues other than joints and juxta-articular structures appears to have a low incidence, to occur lately and to be of limited size. Although extra-articular amyloid deposits may progressively occur and extend with increasing survival time on dialysis, tiny deposits such as those observed in only two of our patients will hardly lead to serious complications.  相似文献   

16.
Summary A case of hemodialysis-associated amyloidosis in a patient who had been on hemodialysis for 10 years is described. Bone lesions were found in the humeral heads, carpal bones, and femoral heads and necks. Specimens obtained from the pathologically fractured left femoral neck revealed amyloid deposits histologically. Immunoperoxidase staining for beta-2 microglobulin was positive in the amyloid at the light-microscopic level. We reconfirmed that bone lesions associated with long-term hemodialysis are manifestations of amyloidosis of beta-2 microglobulin origin. Hemodialysis-associated amyloidosis should be considered in the treatment of long-term hemodialysis patients.  相似文献   

17.
High frequency of amyloid lymphadenopathy in uremic patients   总被引:1,自引:0,他引:1  
Amyloid lymphadenopathy has only been reported in case report form, or in small groups of patient groups within large series. We believe that amyloid lymphadenopathy is common in uremic patients, and thus designed this study to determine the frequency of this condition in hemodialysis patients, and to assess its types and patterns. We reevaluated 46 uremic patients' lymph node biopsies for amyloid deposits. We also immunohistochemically identified the protein origin of these deposits using Amyloid A, kappa, lambda, beta2 microglobulin, and transthyretin antibodies. Histopathologically, we observed for vascular involvement, follicular deposition, and diffuse deposition. We detected amyloid deposits in 10 of the 46 (22%) patients' lymph nodes. The patterns of deposition were vascular involvement alone in six specimens, vascular involvement plus follicular deposition in three, and vascular involvement plus diffuse deposition in one specimen. Amyloid AA type protein was present in seven nodes, beta2 microglobulin-related amyloid in two nodes, and immunoglobulin-derived protein (AL) in one node. We assessed these 10 patients for causes of end-stage renal disease (ESRD) and other conditions that might relate to amyloidosis. The cause of ESRD in the seven patients with AA amyloid were renal amyloidosis secondary to Familial Mediterranean Fever in four, glomerulonephritis in one patient who had bronchiectasis and Castleman's disease, unknown in one patient who had bronchial asthma, and pyelonephritis in one patient who had no characteristics that could be linked with AA type amyloidosis. The causes of ESRD in the two individuals with beta2 microglobulin-related amyloidosis who had been on long-term hemodialysis were pyelonephritis and glomerulonephritis. The cause of ESRD in the patient with AL type protein was glomerulonephritis, and this patient had no systemic disease. We conclude that amyloid lymphadenopathy is, indeed, common in uremic patients. Amyloid type AA is the most prevalent form of amyloid protein in uremic patients, but amyloid type does not always correspond with underlying cause of renal failure, or with the presence of systemic disease.  相似文献   

18.
Patients who are on hemodialysis commonly experience muscle wasting and weakness, which have a negative effect on physical functioning and quality of life. The objective of this study was to determine whether anabolic steroid administration and resistance exercise training induce anabolic effects among patients who receive maintenance hemodialysis. A randomized 2 x 2 factorial trial of anabolic steroid administration and resistance exercise training was conducted in 79 patients who were receiving maintenance hemodialysis at University of California, San Francisco-affiliated dialysis units. Interventions included double-blinded weekly nandrolone decanoate (100 mg for women; 200 mg for men) or placebo injections and lower extremity resistance exercise training for 12 wk during hemodialysis sessions three times per week using ankle weights. Primary outcomes included change in lean body mass (LBM) measured by dual-energy x-ray absorptiometry, quadriceps muscle cross-sectional area measured by magnetic resonance imaging, and knee extensor muscle strength. Secondary outcomes included changes in physical performance, self-reported physical functioning, and physical activity. Sixty-eight patients completed the study. Patients who received nandrolone decanoate increased their LBM by 3.1 +/- 2.2 kg (P < 0.0001). Exercise did not result in a significant increase in LBM. Quadriceps muscle cross-sectional area increased in patients who were assigned to exercise (P = 0.01) and to nandrolone (P < 0.0001) in an additive manner. Patients who exercised increased their strength in a training-specific fashion, and exercise was associated with an improvement in self-reported physical functioning (P = 0.04 compared with nonexercising groups). Nandrolone decanoate and resistance exercise produced anabolic effects among patients who were on hemodialysis. Further studies are needed to determine whether these interventions improve survival.  相似文献   

19.
BACKGROUND: Fosfestrol drip infusion therapy is an available endocrinotherapy for prostate cancer. But since there have been few reports of its use in chronic dialysis patients, the pharmacokinetics of fosfestrol in these patients remains unclear. We conducted fosfestrol drip infusion therapy as an induction therapy in chronic hemodialysis patients with prostate cancer. METHODS: Two male patients were included in this study. One was a 68-year-old man who had been in hemodialysis for 15.7 years and had stage B2 prostate cancer. The other was a 74-year-old man who had been in hemodialysis for 4.4 years and had stage C prostate cancer. A total of 250 mg of fosfestrol was dissolved in 250 mL of 5% glucose solution and administered by drip infusion. The drug was given subcutaneously during 14 consecutive days and a luteinizing hormone-releasing hormone agonist was injected on day 15. RESULTS: Serum fosfestrol levels increased rapidly after the drip infusion was started and remained at high levels during infusion, but fell quickly after the treatment ended. Diethylstilbestrol (DES) was also detected in blood after the infusion was started and its levels peaked when infusion ended. But on the next day, neither fosfestrol nor DES were detected in the blood of the patients. Moreover, neither fosfestrol nor DES was detected in the blood of the two patients before administering fosfestrol on day 15. Fosfestrol was quickly eliminated from the blood after hemodialysis was started, while DES remained in the blood during hemodialysis. The adverse reactions were mild hepatic dysfunction and gynecomastia. CONCLUSIONS: Fosfestrol drip infusion therapy appeared to be safe as an endocrinotherapy for prostate cancer in chronic hemodialysis patients.  相似文献   

20.
AIM: The aim of this study was to determine the effect of l-carnitine on quality of life (QOL) in chronic hemodialysis patients. PATIENTS AND METHODS: This trial used a randomized, prospective, placebo-controlled, double-blind, crossover design. Inclusion criteria were patients who were older than 18 years, had been on dialysis for a minimum of one year, and had at least two of the following symptoms: intradialytic hypotension, muscle cramping, lack of energy, muscle weakness or myopathy, cardiomyopathy, or lack of responsiveness to erythropoietin (EPO). Patients were excluded if they were mentally incompetent to complete a QOL questionnaire. Sixteen patients were randomized to receive either l-carnitine (20 mg/kg) or placebo (normal saline) after each dialysis session for 12 weeks, followed by a 6-week washout, then the crossover therapy for 12 weeks. The Kidney Dialysis Questionnaire was the assessment tool used to evaluate QOL. RESULTS: There was no significant effect of l-carnitine on QOL irrespective of treatment order. There were also no differences found in any of the secondary outcomes including incidence of muscle cramping, intradialytic hypotension, EPO requirements or hemoglobin. Adverse effects consisted of gastrointestinal symptoms, with a similar incidence between l-carnitine and placebo. CONCLUSION: L-carnitine did not have a benefit on QOL in our patient population.  相似文献   

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