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Background and Objective: Cardiovascular diseases (CVD) are the leading cause of death in Spain, and although the incidence rates have been declining for years due to improvements in prevention and treatment, there is still room for further reduction. Primary Care has a leading role in the prevention and control of these diseases. Determining the prevalence and incidence of cardiovascular risk factors and cardiovascular diseases in the Spanish adult population attended in Primary Care is the objective of the study to identify the Spanish population at cardiovascular and renal risk (IBERICAN)., Material and Methods: A prospective cohort study was conducted using a consecutive non-probabilistic sampling population between 18 and 85 years that attended Primary Care centres between April 1, 2014 and October 31, 2018. By reviewing the medical history, laboratory results, and medical visits, the Primary Care physicians collected socio-demographic information, personal and family history of the patients, as well as prevalence and incidence of cardiovascular risk factors and cardiovascular and renal disease. The incidents and associated factors will be estimated using proportional risk models of Cox and Kaplan-Meier Curves., Results: A total of 8,066 patients have been recruited by 531 Primary Care physicians from all Autonomous Communities of Spain as part of the IBERICAN cohort, with the exception of Ceuta. The mean age of the cohort is 57.9 years with 54.5% women. Obesity, dyslipidaemia, and hypertension are the most prevalent cardiovascular risk factors. There was a history of CVD in16.3%, and 25.5% had a subclinical organ lesion., Conclusion: The IBERICAN study is a cohort of people treated in Primary Care that expects to provide relevant information on the factors that determine the incidence of cardiovascular and renal disease in Spain., (Copyright © 2019 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España, S.L.U. All rights reserved.)

Treatment with nitisinone (NTBC) has brought about a drastic improvement in the treatment and prognosis of hereditary tyrosinemia type I (HT1). We conducted a retrospective observational multicentric study in Spanish HT1 patients treated with NTBC to assess clinical and biochemical long-term evolution.We evaluated 52 patients, 7 adults and 45 children, treated with NTBC considering: age at diagnosis, diagnosis by clinical symptoms, or by newborn screening (NBS); phenotype (acute/subacute/chronic), mutational analysis; symptoms at diagnosis and clinical course; biochemical markers; doses of NTBC; treatment adherence; anthropometric evolution; and neurocognitive outcome.The average follow-up period was 6.1 ± 4.9 and 10.6 ± 5.4 years in patients with early and late diagnosis respectively. All patients received NTBC from diagnosis with an average dose of 0.82 mg/kg/d. All NBS-patients (n = 8) were asymptomatic at diagnosis except 1 case with acute liver failure, and all remain free of liver and renal disease in follow-up. Liver and renal affectation was markedly more frequent at diagnosis in patients with late diagnosis (P < .001 and .03, respectively), with ulterior positive hepatic and renal course in 86.4% and 93.2% of no-NBS patients, although 1 patient with good metabolic control developed hepatocarcinoma.Despite a satisfactory global nutritional evolution, 46.1% of patients showed overweight/obesity. Interestingly lower body mass index was observed in patients with good dietary adherence (20.40 ± 4.43 vs 24.30 ± 6.10; P = .08) and those with good pharmacological adherence (21.19 ± 4.68 vs 28.58 ± 213.79).intellectual quotient was ≥85 in all NBS- and 68.75% of late diagnosis cases evaluated, 15% of which need pedagogical support, and 6.8% (3/44) showed school failure.Among the 12 variants identified in fumarylacetoacetate hydrolase gene, 1 of them novel (H63D), the most prevalent in Spanish population is c.554-1 G>T.After NTBC treatment a reduction in tyrosine and alpha-fetoprotein levels was observed in all the study groups, significant for alpha-fetoprotein in no NBS-group (P = .03), especially in subacute/chronic forms (P = .018).This series confirms that NTBC treatment had clearly improved the prognosis and quality of life of HT1 patients, but it also shows frequent cognitive dysfunctions and learning difficulties in medium-term follow-up, and, in a novel way, a high percentage of overweight/obesity.

Although a significant association between renal function and outcomes in patients with acute coronary syndromes (ACS) has been consistently described, little information exists about the magnitude of this association in patients at older ages. No study assessed the prognostic role of renal function according to frailty in patients with ACS. The LONGEVO-SCA registry included unselected ACS patients aged ≥80 years. Frailty was asessesed by the FRAIL scale, and baseline creatinine clearance was calculated by the Cockroff-Gault formula. We evaluated the impact of renal function on mortality or readmission at 6-months according to frailty status by the Cox regression method. A total of 473 patients were assessed, with a mean age of 84.2 years. The distribution of patients across estimated glomerular filtration rate (eGFR) subgroups was as follows: (1) <30 ml/min: n = 76 (16.1%); (2) 30 to 44 ml/min: n = 147 (31.1%); (3) 45 to 60 ml/min: n = 136 (28.8%); and (4) >60 ml/min: n = 114 (24.1%). Patients with lower eGFR values were older, had a higher proportion of comorbidities and other geriatric syndromes (p = 0.001) and underwent less often an invasive management during admission (p < 0.001). The incidence of mortality or readmission at 6 months progressively increased across renal function subgroups (p = 0.001). After adjusting for potential confounders, this association became nonsignificant (p = 0.802). The association between eGFR and outcomes was only significant in patients without frailty (p = 0.001). In conclusion, most patients aged ≥80 years with NSTEACS had renal function impairment at admission. The association between renal function and outcomes was different according to frailty status., (Copyright © 2018. Published by Elsevier Inc.)

Objective: The objective was to study the clinical course of children requiring continuous renal replacement therapy (CRRT) after cardiac surgery and to analyze the factors associated with mortality., Methods: A prospective observational study was performed that included all children requiring CRRT after cardiac surgery, comparing these patients with other critically ill children requiring CRRT. Univariate and multivariate analyses were performed to determine the influence of each factor on mortality., Results: Eighty-one (4.9%) of 1650 children undergoing cardiac surgery required CRRT; 65 of them (80.2%) presented multiorgan failure. Children starting CRRT after cardiac surgery had lower mean arterial pressure and lower urea and creatinine levels, and were more likely to require mechanical ventilation than other children on CRRT. The incidence of complications was similar. Cardiac surgery increased the probability of requiring CRRT for more than 14 days. Mortality was 43% in children receiving CRRT after cardiac surgery and 29% in other children (P = .05). Factors associated with mortality in the univariate analysis were age less than 12 months, weight less than 10 kg, higher Pediatric Risk of Mortality Score, hypotension, lower urea and creatinine on starting CRRT, and use of hemofiltration. In the multivariate analysis, the only factor associated with mortality was hypotension on starting CRRT (hazard ratio, 4.01; 95% confidence interval, 1.2-13.4; P = .024)., Conclusions: Although only a small percentage of children undergoing cardiac surgery required CRRT, mortality in these patients was high. Hypotension at the time of starting the technique was the only factor associated with a higher mortality., (Copyright © 2013. Published by Mosby, Inc.)

Objective: To study the impact on postoperative costs of a patient's antithrombin levels associated with outcomes after cardiac surgery with extracorporeal circulation., Design: An analytic decision model was designed to estimate costs and clinical outcomes after cardiac surgery in a typical patient with low antithrombin levels (<63.7%) compared with a patient with normal antithrombin levels (≥63.7%). The data used in the model were obtained from a literature review and subsequently validated by a panel of experts in cardiothoracic anesthesiology., Setting: Multi-institutional (14 Spanish hospitals)., Participants: Consultant anesthesiologists., Measurements and Main Results: A sensitivity analysis of extreme scenarios was carried out to assess the impact of the major variables in the model results. The average cost per patient was €18,772 for a typical patient with low antithrombin levels and €13,881 for a typical patient with normal antithrombin levels. The difference in cost was due mainly to the longer hospital stay of a patient with low antithrombin levels compared with a patient with normal levels (13 v 10 days, respectively, representing a €4,596 higher cost) rather than to costs related to the management of postoperative complications (€215, mostly owing to transfusions). Sensitivity analysis showed a high variability range of approximately ±55% of the base case cost between the minimum and maximum scenarios, with the hospital stay contributing more significantly to the variation., Conclusions: Based on this analytic decision model, there could be a marked increase in the postoperative costs of patients with low antithrombin activity levels at the end of cardiac surgery, mainly ascribed to a longer hospitalization., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Several epidemiologic investigations have confirmed that obesity is a significant risk factor for the appearance of proteinuria and end-stage kidney disease in a normal population. Weight loss induced by low-calorie diets, physical exercise, or bariatric surgery is accompanied by an important antiproteinuric effect. Reduction in proteinuria is already observed after a few weeks from the onset of weight loss and it is evident even in patients with modest weight losses. Reduction in proteinuria by weight loss has been described in chronic proteinuric nephropathies of different etiologies. The mechanisms through which weight loss may reverse proteinuria are likely to be plural: better control of blood pressure, improvement of serum lipid profile, improvement of insulin sensitivity, better glycemic control in diabetes patients, decrease of circulating leptin levels, reversal of glomerular hyperfiltration, and decreased activation of the renin-angiotensin-aldosterone system.

Background: The change from calcineurin inhibitors (CNI) to sirolimus (SRL) is a safe alternative in transplant patients with neoplasia (NEO) whereas the results of conversion for chronic allograft nephropathy (CAN) are controversial, depending on the histologic score, degree of proteinuria, and glomerular filtration rate (GFR). Our aim in this study was to compare GFR, proteinuria, albuminuria, blood pressure (BP) effects, and anemia after switching to sirolimus (SRL) among renal transplant recipients with CAN versus NEO., Methods: Fifty-five kidney transplant recipients with conversion from CNI to SRL owing to CAN or NEO were analyzed for the variables at 6 months before, at the time of, and at 6 months and 1, 2, and 3 years after the switch to SRL., Results: There were no differences between CAN and NEO in the slope of estimated GFR (mL/min/1.73 m(2) by Cockcroft-Gault formula) at 1 year (-5.5 vs 3.7; P = .007) and at 2 years (-3.86 vs -10.3; P = .01). The values of proteinuria (mg/24 h/1.73 m(2)) before (665 ± 136 vs 329 ± 69; P = .036) as well as at 1 (1,122 ± 306 vs 863 ± 190; P = .478) and at 2 years after conversion (1,360 ± 430 vs 457 ± 154; P = .045) showed some significant differences, as did the use of both antiangiotensin agents, angiotensin-converting enzyme inhibitor and angiotensin receptor blocker at the moment of switch (35% vs 0%; P = .005) at 1 year (69% vs. 6% P = .02) and at 2 years (67% vs 28%; P = .047). There were no differences in graft survival (log rank: P = .515). By logistic regression analysis, the best covariate associated with GFR >45 mL/min at 2 years was GFR >60 mL/min at the moment of switch to SRL (odds ratio, 1.33; 95% confidence interval, 1.002-1.74)., Conclusions: The evolution of renal damage was more important in the CAN group requiring greater use of 2 angiotensin antagonists for control of proteinuria. We probably need histologic and serologic biomarkers to show which patients with CAN will show a bad evolution after the change to SRL., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: To analyze the causes of death, survival, and risk factors for mortality in a large series of Spanish systemic sclerosis (SSc) patients followed over the last 25 years in a tertiary care university hospital., Methods: Demographic, clinical, and outcome data from all SSc patients followed in the rheumatology department were included in a database created in 1989. ANOVA, Kruskal-Wallis, or chi(2) tests were used to identify differences among groups; Kaplan-Meier analysis was used to estimate survival, and Cox proportional hazards regression analysis was used to identify factors associated with mortality., Results: A total of 204 patients were included, of whom 182 (89%) were women. Mean age at diagnosis was 49 +/- 17 years, and mean follow-up was 8 years. Over 1635 patient-years, 36 of 44 deaths were attributable to SSc: 28 related to cardiorespiratory involvement, 4 to peripheral vascular disease, 3 to gastrointestinal, and 1 to renal involvement. The main SSc-unrelated causes of death were malignancy (3 cases) and infections (2 cases). Survival rates from disease onset were 85, 75, and 55% at 5, 10, and 20 years, respectively, with poorer survival in patients with renal disease and pulmonary hypertension (PH). Independent prognostic factors for mortality were older age at diagnosis, diffuse skin involvement, proteinuria, PH, and elevated erythrocyte sedimentation rate., Conclusions: Ten-year survival is over 70% in Spanish SSc patients. The main causes of death are lung and cardiac involvement, and to a lesser extent, peripheral vascular disease and coexisting malignancy. Diffuse subset, proteinuria, PH, elevated erythrocyte sedimentation rate and older age at diagnosis are the main risk factors for mortality., ((c) 2010 Elsevier Inc. All rights reserved.)

Unlabelled: HIV nephropathy (HIVAN) is the most frequent cause of chronic renal failure in HIV-infected black patients. However, the prevalence of other glomerulopathies mediated by immunocomplexes has increased in the last years. We report on the glomerular diseases observed in HIV patients in our Hospital., Methods: A retrospective study of all patients with HIV infection and glomerular diseases diagnosed by renal biopsy., Results: We found 27 patients with the following glomerular diseases: membranoproliferative glomerulonephritis (MPGN) in 8 patients, non-collapsing focal segmental glomerulosclerosis (FSGS) in 7, IgA nephropaty (IgA N) in 6, collapsing glomerulosclerosis in 4 (HIVAN, and membranous nephropaty (MN) in 2. Most of patients were young white men. A high prevalence of coinfection with hepatitis C virus (HCV) (77.8%) and hepatitis B virus (HBV) (37%) was found. At diagnosis, most of patients (90%) had proteinuria, with nephrotic syndrome in 52% of them; 59% presented with acute renal failure. Nine patients (33%) showed malignant hypertension at diagnosis: this complication was particularly common among IgA N patients (4/6, 66%)., Conclusion: In our Hospital, immunocomplex-mediated glomerulonephritis were more frequent than HIVAN among HIV-infected patients. HCV-associated MPGN was the most frequently detected glomerular disease. A high prevalence of malignant hypertension was observed at diagnosis, particularly among patients with IgAN.

Murine typhus and "murine-typhus-like" disease are reemerging infectious diseases. In Canary Islands (Spain), a rather distinct clinical pattern characterized by higher incidence of complications, especially renal damage (including acute failure and urinalysis abnormalities), is apparent and highly suggestive. It could be related to different strains of Rickettsia typhi or other cross-reactive species.

Background: Improved immunosuppressive regimens and management strategies in renal transplantation (RT) have increased patient and graft survival during the last years. The aim of our study was to analyze the causes, risk factors, and evolution of mortality after renal transplantation., Methods: We studied 3365 renal transplant recipients in adults (>18 years) who survived at least 1 year after transplantation in Spain during 1990, 1994, and 1998. The mortality rates and risk factors were analyzed employing single and multivariate Cox regression., Results: The follow-up was shortest (maximum 2.5 years) for recipients transplanted in 1998. When we consider an identical follow-up period (2.5 years) for all patients, we did not observe a statistical difference in patient survival and causes of death in the three analyzed periods. Mortality was higher for men and for patients over 60 years. Cardiovascular diseases (CVD) and neoplasia were the most frequent causes of death. Graft dysfunction, as determined by creatinine level or proteinuria range in the first months, were significant factors associated with a higher risk for cardiovascular and infectious deaths., Conclusions: During the last decade in Spain, patient survival after RT (2.5 years follow-up) has remained stable. Recipient age (>60 years), male gender, and graft dysfunction in the first year were associated with a higher risk of death especially due to CVD.

Objective: To examine the outcome and risk factors for renal sequelae in an unselected population of adults with Henoch-Schonlein Purpura (HSP)., Methods: Retrospective study of adult patients (> 20 years) with biopsy proved cutaneous vasculitis diagnosed as having HSP seen at a single center between 1984 and 1998. Patients were classified as having HSP according to proposed criteria. Only those patients with a followup of at least 1 year were included in this study of renal sequelae., Results: Twenty-eight patients with a mean followup of 5.5 years fulfilled the inclusion criteria. When the study was concluded, 10 patients (36%) had renal sequelae and 2 (7%) had renal insufficiency. Men outnumbered women. However, neither a previous history of drugs, gender, nor age at disease onset was associated with a higher risk of permanent renal involvement. Patients with hematuria at disease onset or renal involvement during the course of the disease more commonly developed renal sequelae (p < 0.001). The presence of anemia (p = 0.05) at the time of diagnosis and the onset in summer (p < 0.05) were also more common in those with permanent renal involvement (renal sequelae). Patients with relapses had also a higher trend to develop renal sequelae (p = 0.07). All patients who fulfilled more than 2 of these 5 risk factors developed permanent renal involvement. With this model we were able to predict renal sequelae in 8 of the 10 patients who had this complication. The Goodman-Kruskal gamma test value was 0.92 (95% CI 0.78-1.00)., Conclusion: In unselected adults with HSP, permanent renal involvement (renal sequelae) is not uncommon. Hematuria at disease onset and persistence of renal manifestations during the course of the disease are significant indicators of possible development of renal sequelae. These manifestations plus other features such as onset in summer, anemia at disease onset, or relapses of the disease may predict the development of renal sequelae in most patients.

Objective: Henoch-Schonlein purpura (HSP) is a small sized vasculitis affecting mainly children. Intercellular adhesion molecule-1 (ICAM-1) gene polymorphisms have recently been implicated in the susceptibility to some vasculitides. To further investigate the clinical implication of ICAM-1 polymorphisms in HSP, we examined their potential association and influence in the development of severe complications in an unselected series of patients with HSP., Methods: Fifty-two patients, of which 41 were children, were diagnosed with HSP using classification criteria of Michel, et al at the Hospital Xeral-Calde (Lugo, Spain); 129 ethnically matched controls were included. Patients had at least one year of followup. Patients and controls were genotyped by allelic oligonucleotide techniques for ICAM-I polymorphism at codon 241 and 469., Results: The frequency distribution of the alleles and genotypes for each ICAM-1 polymorphism did not show significant differences between HSP patients and controls. Also, no differences between patients with or without renal manifestations were found. However, the frequency of the codon 469 K/E genotype was significantly decreased in patients without severe gastrointestinal manifestations compared to those with them (22.29 vs 65%, OR 0.1, p = 0.02, after correction for age, sex, and disease duration). None of the 11 adults exhibited the R/G genotype at codon 241 compared with 7 of 41 children (OR 0.0, 95% CI 0.0-2.9, p = 0.14). Patients with the R/G genotype were associated with low incidence of renal manifestations and none developed permanent renal involvement (renal sequelae); however, this finding did not achieve statistical significance., Conclusion: ICAM-1 polymorphisms alone are not associated with development of HSP, but patients not carrying the codon 469 K/E genotype are at decreased risk of developing severe gastrointestinal complications. The R/G polymorphism at codon 241 may reduce the risk of renal sequelae in the development of HSP in adulthood.

Thirty-three pigs affected by porcine dermatitis and nephropathy syndrome, 30 from Spain and three from the USA, were investigated in order to detect porcine circovirus (PCV) in their tissues. A standard in situ hybridisation technique using a specific DNA 317-bp probe based on a well-conserved sequence of PCV (which recognises both PCV-1 and PCV-2) was applied to formalin-fixed, paraffin-embedded tissues. Twenty-eight of the 30 Spanish pigs and all three American pigs had PCV in at least one tissue. Viral nucleic acid was detected mainly in lymphoid organs, and especially the lymph nodes. The viral genome was also found, in order of decreasing quantity, in Peyer's patches, tonsil, lung, spleen, kidney, liver, and skin. Viral nucleic acid was located mainly within the cytoplasm of monocyte/macrophage lineage cells, including follicular dendritic cells, macrophages, histiocytes and Kupffer cells. No viral nucleic acid was found in damaged glomeruli or arteriolar walls. In frozen samples available from three Spanish pigs, the virus was identified as type 2 by using the polymerase chain reaction and restriction fragment length polymorphism. Most of the pigs from which serum was available were seropositive against porcine respiratory and reproductive syndrome virus (PRRSV), and PRRSV antigen was detected in the lung of two of the Spanish pigs. These results suggested that PCV is present in tissues of almost all pigs affected by PDNS, and PCV has to be considered as a possible agent involved in the pathogenesis of the syndrome.

The physician Julián Gutiérrez de Toledo, born in the Imperial Town halfway through the Fifteenth Century and deceased around 1520, has a great relevance in the History of Urology for being the first writer of a treatise on urological topics in the Castilian language with his work "Cure of the stone and loin pain or/and renal colic" (Editor Melchor Gorricio, Printer Pedro de Hagembach, Toledo 1498). His work marks the transit from The Middle Ages to the Modern Times in what refers to awareness of the urinary tract diseases. The book "Cure of the stone..." has 85 pages written in two columns of 40 lines each with gothic characters. On the cover there is an engraving of the saint physicians Cosme and Damian, and on the last page is the printer's coat-of-arms. The book consists of five parts as all books from late Middle Ages: Of the Causes, Signs and Prognosis, Preservation, Of the Cure, Misgivings. The value of the treaty is that it compiles all that was known at the time on urinary lithiasis showing a marked Avicenna-like Galenism to which the author adds his own observations that show the Renaissance man. An exceptional witness of the reign of the Catholic King and Queen, this incunabular should be known by the Spanish speaking urologist as illustrious predecessor.

We present a series of cases of complex renal lithiasis, in which we carried out temporary internal urinary bypassing, prior to extracorporeal lithotripsy, by means of the retrograde insertion of a double-J urethral catheter, studying the time of stay, the presence or not of contamination in the catheter and its relationship with the calcifications found in them. We also carried out a metabolic-functional study of each patient, determining the urinary pH and the serum and urinary calcium, phosphorous and uric acid concentrations, and observing their possible influence on the above-mentioned complications. Of the 25 catheters studied, we observed calcification in 11 cases (44%). In these we observed urealytic germs (Proteus and Pseudomonas), without finding modifications of urinary pH. We observed no relationship between the calcium deposits and serum and urinary calcium, phosphorous and uric acid concentration.