Sodium thiosulfate not always resolves calciphylaxis: an ambiguous response.

Calciphylaxis is a severe "vascular ossification-calcification," associated with a very high mortality rate that involves arterial wall, venular wall, and nerves resulting in ischemia and necrosis of skin, subcutaneous fat, visceral organs, and skeletal muscles. Sodium thiosulfate has recently been used as a novel treatment option for calciphylaxis because of its dual role as an antioxidant and a chelator. Multiple case reports demonstrated that such therapy has resulted in pain relief and healing of skin ulceration. We report a case of calciphylaxis of large severity that had an ambiguous response to sodium thiosulfate treatment (improvement of symptomatology and skin lesions, improvement of blood parameters, worsening of general conditions, and consciousness until death).

Phosphorus: the philosopher's stone discovered in 1669.

Recently the importance in nephrology of phosphorus as phosphate has been highlighted by chronic renal failure patients, in whom the toxic effect of phosphate is widely acknowledged, given the association of phosphate serum level with cardiovascular risk. This association is not limited to chronic renal failure and hemodialysis patients as high serum phosphate. Recently high serum phosphate levels were associated with increased risk for cardiovascular disease in subjects free from chronic kidney disease, and cardiovascular disease as well, and with progression of atherosclerosis. It is useful to know the history of phosphorus from its discovery in 1669, because that history gives us more evidence to better understand the negative and/or toxic effects of high phosphate serum levels and to identify phosphorus as a physiologically crucial anion.

Impotence in the 18th and 19th century: concepts of etiology and approaches to therapy.

The old word impotence is derived from the Latin word impotencia, which literally translated means "lack of power." Impotence, in the course of the history, has been attributed to mental pathology, anxiety, or demons or witches. Historically, the pharmacological treatments for impotence started in Greek times, when a myriad of herbal medications were applied locally to the genitals to enhance "sexual strength." In the 18th century, theories about the main factors inducing impotence saw it as an abnormal state of the fibers, a defect in the solid or liquid substances or a bad structure (tumor, inflammation, abscess, ulcer or foreign body). According to these mechanisms, when impotence depended on the state of the muscular fibers, treatment included a tepid bath and a clyster. In very fat or very weak people, who get particularly tired, it was important to use the remedies able to give energy to the fibers, such as ferrous mineral waters, for a month. Moreover, other suggestions were to ride a horse, to sleep few hours, to breathe good country air, to take a purge every 2 weeks, to drink half a glass of wine from Borgogne or to distract the mind continuously. In the 19th century, therapies regarding impotence included slight electric stimulation through the application of stimulators on the scrotum in the testis or epididymis areas, until pain was induced. In the same period, another method for treating impotence was flagellation. This method consisted of little flagellations with leather strips.

Hepatitis status and mortality in hemodialysis population.

The role of hepatitis B (HBV) and C (HCV) virus infection in mortality among MHD patients is poorly understood. Recent studies have shown that HCV positivity is associated with significantly higher cardiovascular mortality, especially in dialysis patients younger than 65 years. However, little information is available in European renal registries about mortality among HBV and HCV positive MHD patients. We prospectively followed all patients (prevalents and incidents) attending the dialysis center in the Sicilian region since January 1, 1999, up to December 31, 2000. Those who died for any cause after the starting point were identified and included in the cases population. In all, 698 eligible cases were found. For each case, three controls extracted from the Registry were matched by age at death (within five years) and sex. We calculated the sample size of 698 cases and three controls for each case, assuming the power of the study to be 80%, with an estimated prevalence of exposure among controls of 3.0%. The chi(2) and the t-test were used to evaluate possible differences among cases and controls for the different variables under investigation. The ORs of the association between hepatitis infection and mortality, adjusted for each of the possible confounding factors, was calculated using the Mantel-Haenszel test. The prevalence of Hepatitis C (HCV) was much higher among case compared with controls, both in males (23.4% vs. 17.7 %) and females (25.0% vs. 22.4%). In the multivariate model, the association between HCV and mortality maintained a significant association only among women aged <65 years with an OR of 1.77 (95% CI: 1.12-2.79). We also observed a correlation between increased risk of mortality in hemodialysis and HCV-positive patients with a longer time on dialysis. Our results suggest that HCV positivity among MHD patients is associated with significantly higher mortality in female aged <65 years. For this reason we should be more aggressive in identifying, preventing, and treating HCV infection among patients with end stage renal disease.

Survival in octogenarian dialysis patients: analysis in two Southern Italian regions.

Dialysis for octogenarian and nonagenarian patients has increased dramatically in recent decades. Worldwide Registries of dialysis and transplantations show how the incidence rate of patients older than 80 years of age is almost doubled. This increase is probably due to liberalisation of acceptance criteria for dialysis, together with the ageing population. In recent years, the Sicilian and Campanian Registries of dialysis and transplantations have also observed a progressive increase in the elderly population. In order to study survival in this class of patients, we performed a study on a sample of Sicilian and Campanian patients. Regarding Sicilian patients, we considered a sample of 497 patients and grouped them into 3 categories of risk: in the first group (low risk) patients included were <70 years old without any co-morbidities; in the second group (average risk), we selected patients between 70 and 80 years old with one or more co-morbidities: in the third group (high risk patients), we included patients aged more than 80 years or with a high number of co-morbidities. The probabilities of survival in the low risk patients after 48 months of treatment was 96.1%; this probability fell to 82.9% for patients included in the high risk group; while the probability of survival for average risk patients was 91.7%. Given the high risk presented by older patients, we focused the second part of our analysis only on octogenarians, studying both Sicilian and Campanian patients. 64 Sicilian patients (33 males and 31 females--51.56% and 48.43% respectively) were observed: and their survival was 81.25% after 48 months. The sample of Campanian octogenarian patients considered for this study included 26 patients (12 males--53.85%--and 14 females--46.15%), observed for a shorter period (36 months). The probabilities of survival after 36 months was 84.61%. To sum up, elderly patients represent a growing reality on dialysis registries in Sicily and Campania. Future research concerning this class of patients should be developed in order to individualize suitable stratification risk indices; knowing patterns and probability of survival might help physicians in the dialysis decision-making process.

Changes in serum fetuin-A and inflammatory markers levels in end-stage renal disease (ESRD): effect of a single session haemodialysis.

BACKGROUND: The aim of the present study was to evaluate the effect of a single haemodialysis (HD) session on serum fetuin-A levels, considered a negative acute phase response marker; moreover, we evaluated the behaviour of fibrinogen and high sensitivity C-reactive protein (hsCRP) as acute phase response and chronic/subclinical inflammation markers, respectively, after a single HD session. METHODS: Serum fetuin-A, albumin, hsCRP and fibrinogen were measured in 72 patients before and after a single HD session. RESULTS: After a single HD session, we observed a significant increase in fibrinogen levels, while fetuin-A levels decreased (p<0.05). Also, hsCRP levels were significantly increased. CONCLUSIONS: The significant decrease of fetuin-A levels after a single HD session is consistent with the hypothesis of HD-induced inflammation; activated acute phase response and fetuin-A deficiency might account for increased cardiovascular risk and accelerated atherogenesis in dialysis patients.

Management of secondary hyperparathyroidism in uremic patients: the role of the new vitamin D analogs.

Secondary hyperparathyroidism - a common comorbid condition in patients with chronic renal insufficiency - is considered a consequence of critical determinants such as hypocalcemia, phosphate retention and reduced levels of calcitriol production. In this complex mechanism, the skeletal apparatus and the nonskeletal targets such as vascular and heart valves are often involved, thus explaining the increased risk of cardiovascular morbidity and mortality of uremic patients. In this review we will focus on the major role played by Calcitriol deficiency as a trigger of secondary hyperparathyroidism and the crucial need for obiquitous vitamin D receptor activation in order to have an optimal PTH control and to obtain a modulation between inhibitors and inducers of soft tissue calcification. This review will also elucidate the possible role of paricalcitol - a new vitamin D analog - in conditioning morbidity and mortality of patients on renal replacement therapy (RRT).

Prevalence and severity of anaemia in patients with type 2 diabetic nephropathy and different degrees of chronic renal insufficiency.

BACKGROUND/AIM: Type 2 diabetes mellitus is the single most common cause of chronic kidney disease (CKD); however its real impact on renal anaemia has not been established. The aim of this study was to evaluate whether onset, severity, and prevalence of anaemia during the course of CKD is different between type 2 diabetic and non-diabetic patients. METHODS: We enrolled 281 patients with: (1) type 2 diabetes and no CKD (n = 75); (2) type 2 diabetes plus CKD (n = 106), and (3) CKD without type 2 diabetes (n = 100). According to K/DOQI guidelines, the patients with renal insufficiency (i.e., those with a glomerular filtration rate <60 ml/min) were subgrouped into three tertiles of CKD: (1) stage 3 (creatinine clearance 60-30 ml/min); (2) stage 4 (creatinine clearance 29-15 ml/min), and (3) stage 5 (creatinine clearance <15 ml/min). RESULTS: Anaemia was observed in 16% of the diabetic patients without CKD; it was more frequent in the diabetic patients with CKD than in the non-diabetic patients with CKD (61.7 vs. 52%, p < 0.05). The comparison among the tertiles showed that the prevalence of anaemia was significantly higher only in diabetic CKD patients of stages 4 and 5. The prevalence was higher in females independently of type 2 diabetes mellitus. In diabetics with a normal renal function, the haemoglobin levels were higher than in diabetics and non-diabetics with CKD, but the diabetics showed lower levels of haemoglobin than non-diabetics at stage 3 and stage 4 of CKD. CONCLUSIONS: Diabetic patients with CKD of stages 4 and 5 have a higher prevalence of anaemia than non-diabetic patients with comparable glomerular filtration rate. A higher awareness of this risk will allow earlier diagnosis and treatment.

Anderson-Fabry disease: a protean clinical behavior and a chance diagnosis.

Anderson-Fabry disease is a rare inborn X-linked glycosphingolipid storage disorder in which the deficient activity of the enzyme alfa-galactosidase A (alfa-gal A) leads to the progressive tissular accumulation of lipidic molecules which, in turn, cause a protean pattern of multi-organ disfunction. Enzyme replacement therapy has recently become available and has proved to be effective in controlling the disorder. We present and discuss the case of a family with this disease, with special attention to the variability of clinical features and the difficulty of a correct diagnosis.

Anti-C1q autoantibodies in lupus nephritis: prevalence and clinical significance.

Recently, anti-C1q autoantibodies have been proposed as a useful marker in systemic lupus erythematosus (SLE) since their occurrence correlates with renal involvement and, possibly, with nephritic activity. We aimed to evaluate the prevalence of anti-C1q antibodies in patients with SLE, with and without renal involvement, and to correlate these markers' presence and levels with the activity of the disease and nephropathy. We studied 61 patients with SLE, 40 of whom had biopsy-proven lupus nephritis; 35 patients with other connective tissue diseases; and 54 healthy controls. In addition, 18 lupus nephritis patients were followed up during the disease time course. Anti-C1q antibodies were measured using "homemade" ELISA with high salt concentration (1 M sodium chloride). High anti-C1q antibody titers (> 55 AU) were present in 27 of 61 (44%) SLE patients and in 4% and 0% of normal blood donors and pathologic controls, respectively. Anti-C1q antibodies were found in 60% of patients with lupus nephritis compared with only 14% of SLE patients without nephropathy (P < 0.05). Moreover, patients who were positive for anti-C1q antibodies had a higher European Consensus Lupus Activity Measurement (ECLAM) score (4.35 vs. 2.2); 89% of patients with active lupus nephritis showed high titers of anti-C1q antibodies compared with 0% of patients with inactive nephritis. Anti-C1q and anti-dsDNA antibodies agreed in 79% of cases. Our results confirm that anti-C1q antibodies are present in a significant percentage of SLE patients, and that their presence and levels correlate with disease activity-in particular, during renal flare-ups.

Circulating levels of soluble adhesion molecules in patients with ANCA-associated vasculitis.

BACKGROUND: During inflammation, activated vascular endothelial cells and other cell types express various adhesion molecules, which facilitate the binding of circulating leukocytes and their extravasation in surrounding tissue (i.e. renal tissue). The serum concentration of circulating soluble adhesion molecules is supposed to reflect the degree of this activation. OBJECTIVE: In the first part of the study, we determined if the serum levels of the soluble intercellular adhesion molecule (sICAM)-1 and the soluble endothelial cell-leukocyte adhesion molecule (sELAM)-1, in patients affected by microscopic polyangiitis (MPA), associated with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibodies (ANCA), were related to the active and the inactive vasculitis phase. In the second part of the study, we examined the changes in circulating sICAM-1 and sELAM-1 levels and the clinical outcome of renal function in these patients. METHODS: We examined 20 MPO-ANCA-positive MPA patients in an acute phase and in a remission phase, after 6 months of treatment, and 50 subjects as controls, 30 with autosomal dominant polycystic kidney disease (ADPKD) in stable chronic renal failure (CRF) and 20 healthy volunteers (HS) with normal renal function. RESULTS: Regarding serum creatinine (Cr) concentration, no significant differences were found comparing active and inactive phases in the MPA group and the CRF group. Mean serum adhesion molecule levels in the MPA group were higher in the active phase compared to the inactive phase and to the CRF and HS groups. In addition, considering the outcome of serum Cr concentrations in the MPA group, the serum adhesion molecule levels were higher and decreased more slowly in patients with final high serum Cr concentrations than in patients with final normal serum Cr concentrations. CONCLUSION: Our data suggest that in MPO-ANCA-positive MPA patients, higher sICAM-1 and sELAM-1 levels during the active phase and their slower decline during the treatment period, could be a prognostic risk factor for CRF development.

Prevalence of biliary lithiasis in a Sicilian population of chronic renal failure patients.

BACKGROUND: The aim of this study was to evaluate the prevalence of biliary lithiasis (BL) and associated risk factors in a population of undialysed patients with chronic renal failure (CRF), and to compare these with findings we had obtained previously in chronic haemodialysis (HD) patients and in subjects from the general population located in the same geographic region. METHODS: A total of 118 CRF patients on conservative treatment were included in the study. In all subjects, we measured several clinical and humoral parameters potentially correlated with BL. Liver and biliary tract ultrasonography was performed with a 3.5 MHz linear probe after at least 12 h of fasting. RESULTS: The prevalence of BL in CRF patients was 22%, which was higher than in the general population (chi(2) = 9.4, P < 0.002) but lower than in HD patients (chi(2) = 25.9, P < 0.0001). Age was similar in the three groups. Body mass index (BMI) was significantly higher in the CRF group than in both HD patients (P < 0.0001) and the general population (P < 0.0001). When the CRF group was divided into subjects with or without BL, the only difference was lower serum calcium levels in the subgroup with BL (P < 0.04). CONCLUSIONS: The prevalence of BL in a Sicilian population of CRF patients was higher than in the general population, but lower than in patients with CRF on chronic HD. Apart from BMI, none of the risk factors traditionally associated with BL in the general population were related to BL in the CRF patients. These data suggest that other factors inherent to kidney pathology contribute to the high prevalence of BL in CRF patients.