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Introduction: Patient registries are useful tools for assessing rare diseases. Our objective is to present the Spanish registry of patients with systemic lupus erythematosus (Registro español de pacientes con lupus eritematoso sistémico, RELES)., Patients and Methods: RELES was started in 2008 as an observational, prospective, multicentre cohort registry that included patients from the time they were diagnosed. The registry's objective is to analyse the incidence and noninflammatory complications of systemic lupus erythematosus (SLE). The departments of internal medicine of 38 Spanish hospitals participate in this registry., Results: A total of 298 patients with a mean age of 40.8±15.7 years were included, 88.9% of whom were women and 85.6% of whom were white. In the first visit, there was a predominance of joint manifestations (74.5%). One hundred and seventy-seven patients (59.4%) were positive for anti-native DNA. In these patients, there was a higher rate of lupus nephritis (26.7% vs. 14%, p=.009; relative risk [RR], 1.33), haemolytic anaemia (13.6% vs. 4.1%, p=.07; RR, 1.46) and lymphopenia (55.4% vs. 43.8%, p=.05; RR, 1.21). The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K) score was 9.64 points (interquartile range, 4-13). The patients treated with antimalarial drugs before the diagnosis of SLE had a median SLEDAI score in the first visit of 5, compared with 8 for those who were not treated with these drugs (p=.02)., Conclusions: RELES constitutes the first Spanish patient cohort with SLE recorded from the time of the diagnosis. The presence of anti-DNA has been related to severe manifestations such as nephritis and haemolytic anaemia. Treatment with antimalarial drugs before the diagnosis was associated with less active disease at the initial presentation., (Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.)

Background and Objective: Invasive pneumococcal disease (IPD) shows different epidemiological characteristics depending on age and pneumococcus serotype. The aims of the work were to analyze the clinical manifestations and mortality associated with IPD, the serotype isolated and the antibiotic resistance rates in different age groups., Patients and Method: Retrospectively, 141 patients with IPD diagnosed between 2002 and 2008 were studied. Patients were classified in 4 age groups: ≤ 2 year-old, 3-14 year-old, 15-64 year-old and ≥ 65 year-old., Results: Pneumonia was the most common manifestation in all age groups (71%). Pneumococcal meningitis was more prevalent in patients ≤ 2 year-old (28 vs. 9%, P=.054) and empyema was more frequent in those between 3-14 year-old (31 vs. 5%, P<.001). Mortality was associated with age ≥ 65 year-old (odds ratio [OR] 7, 95% confidence interval [95% CI] 1.9-28.9), primary bacteremia (OR 7, 95% CI 1.9-28.9) and orotracheal intubation (OR 9, 95% CI 1.9-41.1). The more prevalent serotypes among patients ≤ 2 year-old were 14, 19A and 19F. The serotype 1 was most common in patients between 3-14 year-old and serotype 3 in those ≥ 65 year-old. A higher rate of non-susceptible penicillin strains was observed in pediatric population (42 vs. 19%, P=.007)., Conclusions: Age was related to the clinical manifestations, mortality and antibiotic resistance rates. Primary bacteremia was one of the risk factors of mortality., (Copyright © 2013 Elsevier España, S.L.U. All rights reserved.)

This article lists the integral elements of the Sherlock Holmes method, which is based on the intelligent collection of information through detailed observation, careful listening and thorough examination. The information thus obtained is analyzed to develop the main and alternative hypotheses, which are shaped during the deductive process until the key leading to the solution is revealed. The Holmes investigative method applied to clinical practice highlights the advisability of having physicians reason through and seek out the causes of the disease with the data obtained from acute observation, a detailed review of the medical history and careful physical examination., (Copyright © 2013 Elsevier España, S.L. All rights reserved.)

Background: Osteonecrosis (ON) of the hip in human immunodeficiency virus (HIV)-infected patients has been related to corticosteroid use, dyslipidemia, alcoholism, lipodystrophy, antiretroviral drug use, and HIV infection, itself. The aim of this study was to evaluate the prevalence of silent ON of the hip in HIV-infected patients and its association with several risk factors., Methods: From a total of 1200 patients followed up at the HIV clinic of Xeral-Cies Hospital in Vigo (Spain), we selected those diagnosed with HIV infection before January 2006, aged 20-70 years, who came to the clinic during the period of March to May 2008. Patients with a diagnosis of ON of the hip and those with current symptoms were excluded. A magnetic resonance imaging (MRI) study of the hips was performed., Results: Ninety-seven Caucasian patients (68 men) with a median age of 44 years were included. ON of the hip was detected in 4 patients (4.1%), with bilateral involvement in 2 patients. All 4 patients were men, with a median age of 44.5 years. Several risk factors for ON of the hip were recorded in each patient. CD4 lymphocyte nadir (P=0.034), percentage of patients with CDC stage C (P=0.039), and number of patients with previous corticosteroid treatment (P=0.042) were significantly different between patients with ON of the hip and those with normal MRI findings., Conclusions: The prevalence of asymptomatic ON of the hip in HIV-infected patients was 4.1%. The most important risk factors for developing this condition were corticosteroid treatment, lower CD4 lymphocyte nadir, and AIDS-defining disease. Antiretroviral treatment was not associated with osteonecrosis., (Copyright 2008 Elsevier España, S.L. All rights reserved.)

Introduction: Although cocaine abuse is an increasingly important medical problem, many manifestations of its toxicity are not well understood. The aim of this study is to review the most serious clinical manifestations related to cocaine abuse., Patients and Method: We reviewed the medical records of all patients over 16 years of age admitted to the hospital from January 1994 to December 2005 where cocaine abuse was recorded in their clinical history., Results: A total of 170 patients, with 188 episodes and 268 pathologic manifestations related to cocaine abuse were included. Thirty two out of the 170 patients (18.8%) were females. Mean age was 33 +/- 11 years, with no significant difference between males and females. A total of 88.8% were smokers, 70% had alcohol abuse and 67.3% had other illegal drug abuses. The more frequent reason for their hospitalization was: pulmonary infection (29.6%), bronchial hyperreactivity (14%), acute psychotic attack related to drugs (12%), ischemic heart disease (10%), infectious endocarditis (7.8%), cerebrovascular disease (8.6%), seizures (6.2%) and severe abdominal complications (2.3%). Eight patients died (6.25%). The association with cocaine was only suspected in 46% of the toxic manifestations., Conclusions: Several clinical manifestations are associated to cocaine abuse and its consumption causes potentially fatal complications. The integral treatment of these patients could be improved if these complications are kept in mind.

Introduction: Removal of graft with intravenous antibiotics is traditionally considered the most effective treatment of vascular graft-associated infections. However, an increasing number of reports suggests that this complication can be effectively treated without graft removal., Methods: A prospective study to evaluate the outcome of conservative management of vascular graft-associated infection seen in our center was performed. The diagnosis of graft-associated infection was based on microbiological tests, and imaging studies. In our study, patients were hemodynamically stable with functioning vascular graft as per clinical and imaging evaluations. Conservative management included antibiotic treatment and local debridement without removal of vascular graft., Results: Fourteen patients with vascular graft-associated infection were enrolled in our study. Eleven out of 14 patients (78.6%) responded successfully to conservative management. Treatment failure was reported in 3 patients, 2 of whom required graft removal. After a two-year follow-up, these 14 patients were clinically stable without recurrence of infection., Conclusions: Our study has revealed that conservative management of vascular graft-associated infection seems to be effective and should be considered in hemodynamically stable patients with functioning vascular graft.

Objective: To analyze the characteristics of abscesses of the psoas muscle and assess the differences between pyogenic and tuberculous abscesses., Methods: Retrospective descriptive study of all patients with psoas abscess in our hospital over the period 1994 to 2004., Results: Fourteen patients were studied (10 males), with a mean age of 42 years. Half of them had had an underlying disease. The most frequent clinical manifestations were abdominal pain (64%), fever (57%), and back pain (43%). All the abscesses were secondary. In 7 patients the origin was gastrointestinal, in 6 osteoarticular, and 1 was related with infection of an aortobifemoral bypass. Computed tomography was the diagnostic imaging method in all patients. Culture of drainage specimens was positive in 92% of patients undergoing this procedure. Causal microorganisms included Mycobacterium tuberculosis (5), Streptococcus intermedius (4), Staphylococcus aureus (3), Escherichia coli (3) and Bacteroides fragilis (2). Tuberculous abscesses originated in spondylitis and the clinical presentation was longer prior to diagnosis. Drainage was performed in 12 patients (8 percutaneous and 4 surgical). Mean duration of antimicrobial therapy was 4 weeks. The infection resolved in all patients., Conclusions: Psoas abscess commonly had a gastrointestinal and osteoarticular origin. We underscore the high percentage of tuberculous etiology, which had a more insidious clinical and analytical presentation and was usually secondary to spondylitis. Prolonged antimicrobial treatment associated with drainage was effective therapy.

We performed a retrospective study about patients diagnosed of spinal epidural abscess without concurrent spondylodiscitis, collected in the last five years in our hospital. We describe four patients, all of them males, with ages ranged from 59 to 67 years old. All patients presented fever, elevated phase reactants and spinal pain, with cord compression in three of them. Two patients had risk factors: diabetes mellitus and non-Hodgkin lymphoma. S. aureus was isolated in three patients, and S. agalactiae in the other one. The posterior epidural space was the most frequently affected, with variable location along the spinal cord. Three patients required surgical operation, and the other one was treated only with antibiotics. All patients demonstrated a complete clinical, analytical and radiological resolution.

Objective: Analysis of Enterococcus faecalis bacteremia epidemiological, clinical, microbiological and prognostic characteristics., Methods: Retrospective analysis of clinical records of patients with E. faecalis bacteremia throughout 7 years (January 1995-December 2001)., Results: 95 episodes of bacteremia were documented, 83.2% with nosocomial origin, 85.3% associated to previous invasive procedures and 9.5% in neonates. 57.9% patients suffered an underlying disease and 41.1% had received previously broad-spectrum antibiotics without activity against enterococcus. 32.6% bacteremia episodes was considered primary and, in the rest, the most frequent associated sources of infection were cardiovascular, intra-abdominal, urogenital, and lung. The resistance study showed a single case of resistance to ampicillin and none to glucopeptides. Global mortality was 23.9%, although only in 9.9% was directly attributable to bacteremia. Inadecuate treatment and mechanical ventilation were factors of poor prognosis in the multivariate analysis., Conclusions: E. faecalis bacteremia is in our environment essentially a hospital-acquired infection in patients with severe underlying diseases, subject to invasive procedures, and previously treated with wide spectrum antibiotherapy. Ampicillin continues to be the treatment of choice. Inappropriateness of the initial empirical antibiotic treatment and mechanical ventilation are factors of poor prognosis.

Background: The etiology of multiple sclerosis (MS) is currently unknown. Different viruses have tentatively been involved as causative agents of MS that would trigger an autoimmune response leading to demyelination plaques. There is controversy regarding the role that the human herpesvirus 6 (HHV-6) might play in this condition, and high antibody titers have been detected to HHV-6. HHV-6 DNA has also been detected by PCR both in blood and cerebrospinal fluid by means of the Polymerase Chain Reaction (PCR). Immunohistochemistry studies were performed with histologic specimens from the white matters of patientes with MS. All this has led some authors to incriminate this virus as the triggering etiologic agent of this disease., Patients and Methods: CSF specimens from 23 patients with Relapsing-Remitting MS were studied. The CSF specimens from 23 patients undergoing rachianesthesia were used as controls, and none of them had neurologic disorders. A nested PCR was performed in the collected specimens to detect specific DNA sequences of HHV-6., Results: No DNA sequences of HHV-6, EBV, VZV, CMV and HSV were detected in the tested specimens., Conclusions: No HHV-6 DNA sequences were detected from CSF specimens of patients with MS. Further investigations on the association between HHV-6 and MS should be performed to elucidate the role of HHV-6 in the pathogenesis of this disease.

Objectives: Describe the clinico-evolutionary characteristics of cases of primary antiphospholipid syndrome (PAPLS) diagnosed at our center., Patients and Methods: Retrospective study of all patients with circulating anticoagulant type lupus (AL) (62 patients) and/or anticardiolipin antibodies (ACLA) found in our center since 1981 and 1988, respectively. TTPA, Kaolin's test (KT), and Russel's viper venom time (RVVT) and ACLA type IgG and IgM by ELISA: Only those patients meeting the criteria for PAPLS are included., Results: Twenty-four patients were diagnosed with PAPLS: Eleven patients were from the enlarged TTPA group, 11 of the total with positive ACLA, and two by KT and abnormal RVVT. As for gender, 18 were women. In the past three years, 19 patients were diagnosed. The diagnostic criteria were from the very beginning: venous thrombosis in nine patients, arterial thrombosis in four, aborted repetitions in three, and plateletopenia in eight. Six patients presented pulmonary thromboembolism, the cause of death in one patient. Thrombopenia was manifested in the evolutionary course of 14 patients, only one of which being severe. Two patients have died (one of them from neoplasia), and two others have had antiphospholipid antibodies become negative. Seven patients remain without treatment, the others undergoing anti-aggregating or anti-coagulant treatment., Conclusions: Plateletopenia, alone or accompanied by other symptoms, is the most common alteration of PAPLS in our area, followed by venous thrombosis which caused death by pulmonary thromboembolism in one patient. Arterial thrombotic events and aborted repetitions are by no means infrequent.

The presence of high serum concentrations of immunoglobulins and hypocomplementemia is commonly observed in alcoholic patients; however, the mechanism behind their production is unknown. We studied 70 subjects (52 alcoholics and 18 healthy controls) prospectively. All the patients were active drinkers who consumed more than 100 gr of ethanol daily for at least 10 years. A biopsy of the liver was performed for all the subjects, along with immunoglobulins, C3, C4, CH100 and tests of liver function. Of the 52 alcoholics in the study, 20 showed minimal changes in hepatic steatosis (group II), 6 isolated severe alcoholic hepatitis (group III), 11 cirrhosis of the liver without alcoholic hepatitis (group IV), and 15 cirrhosis with alcoholic hepatitis (group V). There were no significant differences between the various groups according to age or quantity and duration of intake. The highest concentrations of immunoglobulins was observed in those with cirrhosis of the liver (p < 0.001) regardless of the degree of inflammation. The best correlation was found between IgA and liver histology (r = 0.64; p < 0.001). Hypocomplementemia was a factor only in those patients belonging to histological groups IV and V. Both the immunoglobulins and the complement proteins were narrowly correlated with the tests of liver function, and its alterations were more pronounced in patients with cirrhosis in Child's stage C. We conclude that the hypergammaglobulinemia and hypocomplementemia observed in the alcoholic patients are conditioned fundamentally by the degree of deterioration in liver function, indicated by cirrhosis of the liver upon presentation.

19 patients diagnosed as non-tuberculous infectious spondylodiscitis (NTIS) have been studied retrospectively over the last 10 years in three general hospitals. The etiology of 13 cases was confirmed (there were nine cases of Staphylococcus aureus, two of Brucella, one of Pseudomonas aeruginosa and another of Salmonella typhi), and the diagnosis of the remaining cases was based on clinical and radiological data as well as on the evolution with antibiotic treatment. The age of the majority of these patients ranged between 40 and 60 years (p less than 0.05), showing a clear predominance of males (p less than 0.05). The most frequently appearing symptom at the beginning of the disease was local pain which was observed in nine patients; the rest presented mixed symptoms which led to misdiagnosis at admission; an exploratory laparotomy was carried out in one patient. The interval from the symptoms' onset to diagnosis ranged between two and sixteen weeks, with an average of 6.8 weeks. The VSG had values between 22 and 148 mm during the first hour, with an average of 83.3 mm. Seven patients underwent surgery, six of them presenting paraparesis-paraplegia, while another presented cervical spondylodiscitis. One patient passed away, two persisted with paraparesis-paraplegia as an irreversible sequela and one with persistent local pain.