Eculizumab Modifies Outcomes in Adults with Atypical Hemolytic Uremic Syndrome with Acute Kidney Injury.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with congenital or acquired genetic abnormalities that result in uncontrolled complement activation, leading to thrombotic microangiopathy and kidney failure. Until recently, the only treatment was plasma exchange or plasma infusion (PE/PI), but 60% of patients died or had permanent kidney damage despite treatment. Eculizumab, a complement inhibitor, has shown promising results in aHUS. However, data are mainly extracted from case reports or studies of heterogeneous cohorts, and no direct comparison with PE/PI is available. METHODS: An observational retrospective study of adult, dialysis-dependent aHUS patients with acute kidney injury (AKI) who were treated with either PE/PI alone or with second-line eculizumab in our center. We compared the effect of PE/PI and eculizumab on kidney function, hypertension, proteinuria, hematologic values, relapse, and death. RESULTS: Thirty-one patients were included (females, 18; sporadic aHUS, 29; mean age, 46 ± 20 years). Twenty-six patients were treated with PE/PI alone, and 5 were deemed to be plasma-resistant and received eculizumab after stopping PE/PI. Among patients receiving eculizumab, 80% attained complete recovery of kidney function, 100% stopped dialysis, 20% had decreased proteinuria, and no patient relapsed (vs. 38.5, 50, 15.4, and 11.5%, respectively, of patients receiving only PE/PI). At 1-year of follow-up, no deaths had occurred in either group. CONCLUSION: Eculizumab shows greater efficacy than PE/PI alone for the treatment of adult aHUS patients with AKI. Prospective studies and meta-analyses are warranted to confirm our findings and set guidelines for treatment, monitoring, and maintenance.

Identification of predictive factors of the degree of adherence to the Mediterranean diet through machine-learning techniques.

Food consumption patterns have undergone changes that in recent years have resulted in serious health problems. Studies based on the evaluation of the nutritional status have determined that the adoption of a food pattern-based primarily on a Mediterranean diet (MD) has a preventive role, as well as the ability to mitigate the negative effects of certain pathologies. A group of more than 500 adults aged over 40 years from our cohort in Northwestern Spain was surveyed. Under our experimental design, 10 experiments were run with four different machine-learning algorithms and the predictive factors most relevant to the adherence of a MD were identified. A feature selection approach was explored and under a null hypothesis test, it was concluded that only 16 measures were of relevance, suggesting the strength of this observational study. Our findings indicate that the following factors have the highest predictive value in terms of the degree of adherence to the MD: basal metabolic rate, mini nutritional assessment questionnaire total score, weight, height, bone density, waist-hip ratio, smoking habits, age, EDI-OD, circumference of the arm, activity metabolism, subscapular skinfold, subscapular circumference in cm, circumference of the waist, circumference of the calf and brachial area.

Spitzoid melanoma in children: clinicopathological study and application of immunohistochemistry as an adjunct diagnostic tool.

INTRODUCTION: The term spitzoid melanoma (SM) is reserved for a rare group of tumors with striking resemblance to Spitz nevus, often developing in children diagnosed in retrospect after the development of metastases. OBJECTIVES: To determine the biological significance of SM and to analyze the effectiveness of adjuvant diagnostic techniques. MATERIALS AND METHODS: A retrospective, observational study of 38 cases of SM in patients younger than 18 years. Histological type, Clark level and Breslow thickness, radial and vertical growth phase, mitotic count/mm(2), ulceration, regression, vascular and perineural invasion, satellitosis, cytology and associated nevi were reviewed. An immunohistochemical analysis with HMB45 and Ki67 was performed in 10 cases. These features were correlated to patient's stage and outcome. RESULTS: Analysis of histological and immunohistochemical features should allow accurate diagnosis in most cases. Given the low mortality rate, no conclusions about the prognostic significance of histological parameters of the primary tumor could be established. CONCLUSION: We report the largest series of SM from a unique center. Although these patients may have a better prognosis than adults, some patients with SM develop metastasis and die, particularly after age 11 years. Therefore, we recommend using the same treatments as in adults.

Factors influencing delay in the diagnosis of colorectal cancer: a study protocol.

BACKGROUND: Colorectal cancer (CRC) is the second most frequent tumor in developed countries. Since survival from CRC depends mostly on disease stage at the time of diagnosis, individuals with symptoms or signs suspicious of CRC should be examined without delay. Many factors, however, intervene between symptom onset and diagnosis. This study was designed to: 1) Describe the diagnostic process of CRC from the onset of first symptoms to diagnosis and treatment. 2) Establish the time interval from initial symptoms to diagnosis and treatment, globally and considering patient's and doctors' delay, with the latter due to family physician and/or hospital services. 3) Identify the factors related to defined types of delay. 4) Assess the concordance between information included in primary health care and hospital clinical records regarding onset of first symptoms. METHODS: Descriptive study, coordinated, with 5 participant groups of 5 different Spanish regions (Balearic Islands, Galicia, Catalunya, Aragon and Valencia Health Districts), with a total of 8 acute public hospitals and 140 primary care centers. Incident cases of CRC during the study period, as identified from pathology services at the involved hospitals. A sample size of 896 subjects has been estimated, 150 subjects for each participant group. Information will be collected through patient interviews and primary health care and hospital clinical records. Patient variables will include sociodemographic variables, family history of cancer, symptom perception, and confidence in the family physician; tumor variables will include tumor site, histological type, grade and stage; symptom variables will include date of onset, type and number of symptoms; health system variables will include number of patient contacts with family physician, type and content of the referral, hospital services attending the patient, diagnostic modalities and results; and delay intervals, including global delays and delays attributed to the patient, family physician and hospital. DISCUSSION: To obtain a nonrestricted sample of patients with CRC we have minimized selection risk by identifying the patients from pathology services. A greater constraint may be associated with information sources based on clinical records. Due to inherent features of coordinated studies, it is important to standardize the collection of information.

Comparative Study of Survival following Videothoracoscopic Lobectomy Procedures for Lung Cancer: Single- versus Multiple-port Approaches. / Estudio comparativo de la supervivencia tras procedimientos videotoracoscópicos para la lobectomía del cáncer de pulmón: abordaje por puerto único frente a múltiple.

INTRODUCTION: Video-assisted thoracoscopic surgery has become the technique of choice in the early stages of lung cancer in many centers although there is no evidence that all of the surgical approaches achieve the same long-term survival. METHOD: We carried out a retrospective review of 276 VATS lobectomies performed in our department, analyzing age, sex, comorbidities, current smoker, FEV1 and FCV, surgical approach, TNM and pathological stage, histologic type, neoadjuvant or coadjuvant chemotherapy, relapse and metastasis time, with the main aim of evaluating the survival rate and disease-free time, especially with regard to the two/three versus single port approach. RESULT: The one/four year global survival rate was 88.1 and 67.6% respectively. Bivariate analysis found that the variables associated with survival are comorbidity, histological type, stage, surgical approach and need for chemotherapy. When we independently analyzed the surgical approach, we found a lower survival rate in the single-port group vs. the two/three-port group (VATS). Stratifying by tumoral stage (stage I) and by tumor size (T2) survival was significantly lower for patients with single-port group in comparison to VATS approach. In the multivariate analysis, single-port group is associated with a higher risk of death (HR=1.78). In analyzing disease-free survival, differences were found in both cases in favor of two/three port VATS: p=.093 for local relapses and p=.091 for the development of metastasis. CONCLUSIONS: These results challenge the use of the single port technique in malignant lung pathologies, suggesting the need for clinical trials in order to identify the role this technique may have in lung cancer surgery.

Single-lung transplantation in emphysema: Retrospective study analyzing survival and waiting list mortality.

AIM: To performed remains a subject of debate and is the principal aim of the study. METHODS: This retrospective analysis included 73 patients with emphysema (2000-2012). The outcomes of patients undergoing single-lung transplantation (SL) (n = 40) or double-lung transplant (DL) (n = 33) were compared in a Cox multivariate analysis to study the impact of the technique, postoperative complications and acute and chronic rejection on survival rates. Patients were selected for inclusion in the waiting list according to the International Society of Heart Lung Transplantation criteria. Pre and postoperative rehabilitation and prophylaxis, surgical technique and immunosuppressive treatment were similar in every patients. Lung transplantation waiting list information on a national level and retrospective data on emphysema patient survival transplanted in Spain during the study period, was obtained from the lung transplantation registry managed by the National Transplant Organization (ONT). RESULTS: Both groups were comparable in terms of gender and clinical characteristics. We found significant differences in the mean age between the groups, the DL patients being younger as expected from the inclusion criteria. Perioperative complications occurred in 27.6% SL vs 54% DL (P = 0.032). Excluding perioperative mortality, median survival was 65.3 mo for SL and 59.4 mo for DL (P = 0.96). Bronchiolitis obliterans and overall 5-year survival were similar in both groups. Bacterial respiratory infection, cytomegalovirus and fungal infection rates were higher but not significant in SL. No differences were found between type of transplant and survival (P = 0.48). To support our results, national data on all patients with emphysema in waiting list were obtained (n = 1001). Mortality on the waiting list was 2.4% for SL vs 6.2% for DL. There was no difference in 5 year survival between 235 SL and 430 DL patients transplanted (P = 0.875). CONCLUSION: Our results suggest that SL transplantation in emphysema produce similar survival than DL with less postoperative complication and significant lower mortality in waiting list.

[Preoperative risk evaluation in beating-heart coronary artery bypass surgery]. / Evaluación preoperatoria del riesgo en la cirugía coronaria sin circulación extracorpórea.

INTRODUCTION AND OBJECTIVES: Operative risk stratification scales for use in cardiac surgery have been developed for patients who undergo procedures using extracorporeal circulation. The aims of the present study were to investigate the use of six preoperative risk stratification scales in patients undergoing beating-heart surgery and to identify risk factors for major complications and mortality in our group of patients who underwent revascularization using this approach. PATIENTS AND METHOD: Between January 1997 and December 2002, we performed 762 coronary artery bypass operations on the beating heart; 61 patients suffered major complications (8%) and 25 died (3.3%). Risk factors for major complications and death were identified using logistic regression analysis of prospectively collected data. The following risk scores were calculated for each patient: Parsonnet 95, Parsonnet 97, Euroscore, Cleveland, Ontario, and French. Receiver operating characteristic curves were used to compare the ability of each scale to predict mortality and major complications. RESULTS: In our patient group, the preoperative variables associated with increased risk were: need for cardiopulmonary resuscitation, renal dysfunction, peripheral vasculopathy, and the presence of severe left main coronary artery disease, three-vessel disease, or an impaired ejection fraction. CONCLUSIONS: Mortality and major complications were best predicted by the Parsonnet 95 and Euroscore scales.

A model for predicting mortality among critically ill burn victims.

OBJECTIVE: To develop a model for predicting mortality among burn victims. METHODS: All casualties admitted to our intensive care burn unit (ICBU) with a diagnosis of thermal or inhalation injury were studied. Age, total and full-thickness body surface area (BSA) burned, presence of inhalation injury, gender, mechanism of injury, delay to ICBU admission and mechanical ventilation during the first 72 h were recorded. The 851 participants were randomly divided into derivation (671) and validation (180) sets. From univariate and multivariate logistic regression analyses a mortality predictive equation was derived. RESULTS: Mortality was 17.6%. In univariate analysis, all variables were significantly associated with mortality except mechanism of injury and delay to ICBU admission. In multivariate analysis, age, total and full-thickness BSA burned, female gender and early mechanical ventilation were independently associated with mortality. CONCLUSIONS: We propose a mortality predictive equation for burned victims. In this model, MV and not inhalation injury is a mortality risk factor.

Peak expiratory flow rate as predictor of inpatient death in patients with chronic obstructive pulmonary disease.

OBJECTIVES: Few studies analyze hospital deaths and related factors in patients with acute exacerbation of chronic obstructive pulmonary disease who require hospitalization. METHODS: A cross-sectional study was done with 284 patients who had been admitted consecutively to the Short Stay Medical Unit at the Juan Canalejo Hospital in A Coruña. RESULTS: Eleven patients (3.9%) died. The independent variables for predicting death were the peak expiratory flow (OR, 0.96; 95% CI, 0.94 to 0.98), long-term oxygen therapy (OR, 12.46; 95% CI, 2.1 to 72.4), and body mass index (OR, 0.73; 95% CI, 0.59 to 0.90). A peak expiratory flow < 150 L/min showed the best specificity and positive predictive value with maximum sensitivity for predicting death. The results of the arterial blood gasses and the functional tests did not predict hospital death. CONCLUSIONS: Peak expiratory flow was the most important predictive value for determining the risk of death in patients who required hospitalization for acute exacerbation of chronic obstructive pulmonary disease. Additional studies are required to validate these findings.

[Incidence, risk factors and influence on survival of infectious complications in liver transplantation]. / Incidencia, factores de riesgo e influencia sobre la supervivencia de las complicaciones infecciosas en el trasplante hepático.

INTRODUCTION: Orthotopic liver transplantation (OLT) is successful therapy for patients with end-stage liver disease. Infection is currently a life-threatening complication for these patients. The aims of this study are to determine the incidence of various infections in patients with OLT, to study overall survival rates and survival as related to individual infections, and to investigate the risk factors associated with first episodes of bacterial (BI), fungal (FI), invasive fungal (IFI) and cytomegalovirus (CMV) infections. METHODS: The study includes 165 OLTs performed in 152 recipients from May 1994 to May 1998. A descriptive analysis estimating the 95% confidence interval was performed with 100 variables stratified according to preoperative, operative and postoperative conditions. Cox regression analysis was used to identify the variables associated with infection. Survival studies were carried out with the Kaplan-Meier method. RESULTS: Among the total, 66% of patients developed infection: 41.8% viral, 33.9% BI, 20.6% FI and 4.2% IFI. One-year and 4-year survival rates after transplantation were 90% and 75%, respectively. All the infections decreased survival. Multivariate analyses identified the following risk factors for the specific infections: BI - dialysis, mechanical ventilation, and time of organ ischemia during harvesting; FI - number of hours of surgery and pretransplantation plasma albumin concentrations; IFI - number of blood units transfused, pretransplantation plasma albumin and retransplantation. Cytomegalovirus infection was associated with FI and IFI in the univariate analysis, but the multivariate analysis identified no variables that independently increased the risk of developing this infection.

Presentation and prognosis of gastric cancer in patients aged 80 years and older.

The aim of this study was to analyze the characteristics of the presentation and prognosis of patients aged >/= 80 who were diagnosed with gastric adenocarcinoma. We have used a retrospective cohort study of 2334 patients diagnosed between 1975 and 1993 in northwestern Spain, 263 (11.3%)of whom were >/= 80 years of age. No differences were observed with respect to patients of a younger age at diagnosis regarding the site of the tumor, extension of the disease, or Laurén's histologic type. However, fewer resections with curative intent were performed in the older group (49.1% vs. 30.1%; p < 0.0001). Among those operated on with curative or palliative intent, at the end of the first month the survival probability was 0.9 and the 0.95% confidence interval (CI 95%) was 0.93-0.97 for patients < 80 years of age and 0.93 (CI 95% 0.89-0.98) for the older group ( p = 0.19). At the end of 5 years of follow-up these probabilities were 0.29 (CI 95% 0.27-0.31) and 0.18 (CI 95% 0.14-0.23), respectively ( p < 0.0001). If we consider only those patients undergoing curative resection, the survival probability for the two groups ( p = 0.4) was not statistically different. In conclusion, although the two groups showed similar characteristics at presentation, patients >/= 80 years of age underwent surgery with curative intent less frequently and their general prognosis was worse. Our data support the idea that curative surgery should not be ruled out exclusively for reasons of age.