Angiosarcoma of an Arteriovenous Fistula for Hemodialysis in a Kidney Transplant Recipient Affected by Lowe's Syndrome.

Objective/Background. To describe an uncommon, life-threatening condition such as angiosarcoma of a fistula for hemodialysis occurring in a transplant recipient affected by Lowe's syndrome. Summary. We present the case of a 56-year-old male kidney transplant recipient affected by Lowe's syndrome, also known as oculocerebrorenal syndrome, a rare X-linked disorder characterized by congenital cataracts, hypotonia, intellectual disability, and Fanconi-like renal tubular dysfunction, who was diagnosed with angiosarcoma of a functioning arteriovenous fistula for hemodialysis. Conclusion. Angiosarcoma is a rare soft tissue tumor, and only 22 cases of angiosarcoma of arteriovenous fistulae were described so far; although a correlation between Lowe's syndrome and a higher risk of tumor compared to the general population has not been described so far, the mechanisms of disease causation could be an interesting starting point for future studies on a possible connection between the two events.

Temporal trend of cadmium exposure in the United States population suggests gender specificities.

BACKGROUND: Over the years, environmental cadmium exposure has been linked to increased mortality. Over the years, the use of cadmium has generally decreased. AIMS: Although even relatively low levels of cadmium have been associated with increased mortality in the general population, whether this applies to blood cadmium is not well understood. METHODS: The authors analysed data of the National Health and Nutrition Examination Survey to study the temporal trend of cadmium exposure in the period 1988-2006 and the risk of all-cause, cancer and cardiovascular mortality associated with blood cadmium levels. RESULTS: Urinary cadmium decreased significantly over time in males (0.58 (0.01) mcg/g to 0.41 (0.01) mcg/g; P < 0.001) but not in females (0.71 (0.09) mcg/g to 0.63 (0.08) mcg/g; P= 0.66). All-cause mortality was significantly higher in the highest quartiles compared with the lowest quartile of blood cadmium in both males (hazard ratio 1.89, 95% confidence interval 1.22, 2.89; P= 0.005) and females (hazard ratio 2.03, 95% confidence interval 1.06, 3.89; P= 0.035) after adjustment for age, race/ethnicity, smoke status, alcohol intake, annual household income and body mass index. There was also a significant association with cardiovascular mortality in females (P= 0.025). CONCLUSIONS: Our data show that elevated blood cadmium levels are associated with elevated mortality, that there seem to be gender differences in temporal trends of cadmium exposure and that blood cadmium is a proxy of chronic cadmium exposure.

Cardiac calcium evaluation in hemodialysis patients with multisection spiral computed tomography.

AIM: The aim of this study is cardiac calcium content evaluation in hemodialysis patients by a new technique, based on ultrafast multisection CT (MTC). METHODS: The study was carried out on 30 HD patients, 14 F and 16 M, average age 57.7 +/- 13.9 years, average HD age 57.3 +/- 47.4 months. The intact PTH levels were 625.4 +/- 571 pg/mL. Serum calcium, phosphate and CaxP product were 9.75 +/- 0.84 mg/mL, 6.21 +/- 1.01 mg/dL and 60.2 +/- 10.7 mg2/dL2, respectively. RESULTS: The values obtained with the MTC technique were reported in terms of Agatson scores. Score values frankly in the pathologic range (>100) were found in 24 patients (80%). Correlation analysis has shown positive and significant correlation coefficients of the score with patients' age (p = 0.003), serum calcium (p = 0.012), CaxP (p = 0.015), iPTH (p = 0.049), and borderline, to HD age (p = 0. 06). CONCLUSION: Risk factors for cardiac calcification are mainly age, degree of hyperparathyroidism, increased CaxP and serum calcium levels. A control of calcium phosphate parameters in hemodialysis patients seems to be mandatory to avoid increased severity of coronary artery disease.

Antimicrobial activity of aqueous extracts and of berberine isolated from Berberis heterophylla.

The antimicrobial activity of Berberis heterophylla leaves, stems and root aqueous extracts was studied in vitro on Gram-positive and Gram-negative bacteria and fungi. The in vitro antifungal activity of berberine isolated from the same source against different Candida species was also investigated.

Plasmaperfusion on triptophan columns can improve the clinical outcome of patients affected with myasthenia gravis.

UNLABELLED: Myasthenia Gravis (MG) is a neuromuscular disease often associated with thymic pathology due to neuromuscular transmission impairment by circulating antibodies directed against the cholinergic postsynaptic receptor on the neuromuscular junction (Anti-AchR-Ab). The treatment of MG includes cholinesterase inhibitors, steroids and thymectomy. Plasmapheresis can remove Anti-AchR-Ab but more recently plasma-perfusion (PP), a more specific apheresis for selective removal of noxious plasma components, has been developed. AIM OF THE STUDY: To study the effect of PP treatment, performed by using specific immunocolumns for Anti-AchR-Ab, on the clinical outcome of MG patients non-responder to steroid therapy or thymectomy. MATERIALS AND METHODS: We treated 8 patients suffering from severe MG by a cycle of 6 sessions of PP. We used columns containing triptophan as a specific ligand for Anti-AchR-Ab. In order to evaluate the effectiveness of treatment we used functional tests (muscular tests, respiratory function, electromyography) and laboratory tests (Anti-AchR-Ab; immunoglobulins, complement fractions, immunocomplexes). RESULTS: After one to three PP sessions, early clinical improvement in bulbar and respiratory symptoms were found in all patients and EMG showed improvement of neuromuscular transmission. Serum concentration of immunological markers decreased progressively and significantly during the treatment. Clinical improvements were progressive despite the tendency for Anti-AchR-Ab to reach initial values between one session and another. We observed no side effects due to the type of immunocolumns used. CONCLUSIONS: Triptophan columns appear to be able to remove large quantities of Anti-AchR-Ab and immunological markers from plasma. Our experience shows that PP performed using triptophan columns in patients suffering from severe MG provides good clinical results, improving patients' outcome, without any risk linked to the procedure.

Resistive index in chronic nephropathies: predictive value of renal outcome.

UNLABELLED: The study of renovascular resistances by color Doppler ultrasound has become a useful diagnostic resource for nephrologists. In recent nephrological literature, many papers deal with the correlations between resistive index, anatomo-pathological patterns and renal function. AIMS: In our study, we have tried to discover if resistive index represents a prognostic index of progressive renal failure. MATERIAL AND METHODS: To this purpose we compared renal resistive index and blood creatinine obtained from 28 nephropathic patients at their first control, with blood creatinine values after a 3-year follow-up period. Using a linear regression test, we found a strong correlation between the initial value of resistive index and the value of creatinine variation (p = 0.006). RESULTS: All of the patients with normal resistive index at the beginning maintained a stable renal function. Conversely, the patients with high resistive index at their first control showed a progressive renal failure. CONCLUSION: Our study shows the reliability of resistive index in the prognostic evaluation of renal outcome.

Sodium and fluid modulation in dialysis: new approach.

Hypotension during hemodialysis is still an unsolved problem. The treatment of patients with cardiovascular instability is efficaciously carried out with the use of 'profiled dialysis' (PD) with computerized modulation of ultrafiltration and conductivity. We tested a new profile model which involves progressive decrease of ultrafiltration associated with variable conductivity ('bell pattern'). We observed 8 stable long-term patients receiving hemodialysis (4 men and 4 women, mean age 63.5 years) for 4 h three times a week. Before our test, sodium balance had reached a steady state in all patients and remained stable during the entire observation period. The sodium balance was established by means of a simple pattern suggested by Ursino and coworkers. The patients were observed for two periods of 1 month each (protocols A and B). The intradialytic mean arterial pressure was studied, checking every hour of dialysis. Statistical analysis was done by ANOVA for repeated measures. We compared standard dialysis with constant ultrafiltration rate and conductivity (protocol A) with sessions performed involving a progressive decrease of ultrafiltration together with a variable conductivity of -0.2, +0.2, +0.6, +0.6, 0, -0.4, -0.4, and -0.4 mS/s (protocol B). We found a lower incidence of hypotension (p < 0.01) with better cardiovascular stability during and after treatment in 'profiled dialysis'.

Calcitriol per os once, twice or three times a week: effect of different schedules of administration in hemodialysis patients.

Administration of a single dose of 1,25-OH(2)D(3) can lower PTH levels for up to 4 days in chronic hemodialysis patients. Our purpose was to verify the effects of the same weekly dose of calcitriol per os given in one, two or three administrations, to patients on dialysis with secondary hyperparathyroidism. Thirty patients were studied, divided in to three groups each of 10 patients. Calcitriol therapy in group A was given as a single weekly dose of 0.08 microg/kg b.w. In group B the same total weekly dose was divided in two equal doses. In group C the same total weekly dose was divided in three times. Treatment lasted 2 months. After 8 weeks of therapy the fall in intact PTH was statistically significant in each group, respectively with one-way ANOVA: p<0.02 (A); p<0.002 (B); p<0.001 (c). Two-way ANOVA for comparison of PTH % variation among the three groups was statistically significant p<0.003. Significance was due to difference between group A and groups B and C. The present study confirms the efficacy of single dose in suppressing significantly intact PTH. However, when the same weekly dose is divided into two or in three time-spaced administrations, the suppressive effects are definitely increased.

Myasthenia gravis (MG) treatment with immunoadsorbent columns.

We performed ten plasma perfusion (PP) treatments on eight patients affected by Myasthenia Gravis (MG) with high serum levels of autoantibodies against acetylcholine receptors (anti AChR-AB), and one PP treatment on a patient with MG of probable genetic origin and without specific antibodies. All patients (Osserman group III-IV) had undergone thymectomy and immunosuppressive therapy. Each patient received a treatment cycle of six PP sessions. Clinical conditions were assessed before and after the treatment with evaluation of muscular strength, ventilatory function, and electromyographic testing. Immunologic markers were tested before and after each PP. The patient without specific antibodies showed no improvement with PP and was excluded from our study. All the other patients showed continued improvement, with increased muscle strength and improved respiratory function. Four patients (follow-up 16-24 months) still maintain the clinical improvement; two, unexpectedly relapsed 11-12 months after PP, received a new treatment successfully (follow-up 9-10 month).

Computerised monitoring of sodium and fluid during haemodialysis.

Four stable chronic haemodialysis patients were studied during two periods of 3 weeks: at first a 'standard' haemodialysis (S/HD) was used, followed by 'computer-modulated' haemodialysis (CM/HD). We used a monitor Hospal Monitral-S with Hospal DPM (Display Programmer Module) that allows the programming of 'weight loss rate' and 'dialysate osmolality'. During standard haemodialysis fluid was removed at a constant rate of 1 kg/h with constant sodium dialysate of 143 mmol/l. During computer-modulated haemodialysis the dialysate sodium concentration and the fluid removal of 4 kg per session was modulated by display programmer module.

Plasma perfusion in myasthenia gravis.

Seven patients with myasthenia gravis (MG) unresponsive to thymectomy and steroid treatment (Osserman group III) underwent plasma perfusion (PP). All patients showed palpebral ptosis, diplopia, dysphonia, dysphagia, and muscle weakness; five of them had impaired ventilatory function. Separated plasma was perfused onto a column to adsorb anti-AChR-Ab. Each patient received a treatment cycle of six PP sessions. Clinical conditions were assessed before and after the treatment, with evaluation of muscular strength, ventilatory function, and electromyographic testing (RSS). Immunologic markers were tested before and after each PP. Clinical improvement in bulbar symptoms and respiratory function was noted in all patients after one to three PP. Limb muscle strength began to improve later. Serum concentration decreased (mean % +/- SD) after each PP:anti-AChR-Ab 36.47 +/- 17.43; IgA 20.44 +/- 11.26; IgG 21.24 +/- 32.56; IgM 23.22 +/- 11.40; C3 36.78 +/- 10.15; C4 42.69 +/- 14.82. In five of seven patients the improvement continues (follow-up 1 to 10 months). In one patient it lasted only 1 month, and in another a relapse occurred after 10 months of benefit, but was successfully reversed by retreatment.

Extracorporeal blood recirculation and beta-2-microglobulin plasma levels during hemodialysis.

Beta-2-microglobulin (beta 2m) plasma levels during hemodialysis have recently been considered markers of membrane biocompatibility. The aim of this study was to assess if generation of beta 2m from blood cells in contact with the membrane could account for changes in beta 2m plasma concentration during hemodialysis. The role of heparin was studied as well by comparing conventional with heparin-free dialysis. beta 2m plasma levels were measured in six patients during 12 Cuprophan (Cu) and 12 Eval (E) non-consecutive hemodialysis sessions without ultrafiltration. E membranes were compared with and without heparin. beta 2m plasma levels significantly increased with Cu but not with E. A total of 200 ml of blood from four other patients was recirculated ex vivo before Cu hemodialysis in Cu or E and then reinfused into the patient. A drop in beta 2m concentration was observed in the recirculating blood, recovery of beta 2m from the washed filter was higher with E, and reinfusion of recirculated blood to the patient was not associated with significant increases in beta 2m plasma levels. Hemodialysis with a new Cu filter, following reinfusion, did not produce significant increase in plasma beta 2m concentration. In conclusion, 1) beta 2m plasma levels change during Cu, but are not associated with hemoconcentration from ultrafiltration nor with local release from the blood leaving the filter, 2) beta 2m binding to the membrane may account for decreased levels in recirculated blood, 3) E membranes both with and without heparin do not increase beta 2m plasma levels and reinfusion of recirculated blood blunts the beta 2m changes observed with Cu.