Evaluation of the association of serum glypican-4 with prevalent and future kidney function.

Serum glypican-4 (GPC4) has been identified as an insulin-sensitizing adipokine serving as a marker for body mass index and insulin resistance in humans. The association of circulating GPC4 with kidney function is to date largely unexplored. Therefore, we aimed to evaluate the association between serum GPC4 and prevalent as well future kidney function in a prospective cohort study. The study included 456 Caucasian coronary angiography patients. After a median follow up period of 3.4 years, data on kidney function was reassessed in all patients. Chronic kidney disease (CKD) was defined by decreased estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m2 or albuminuria. At baseline, serum GPC4 was significantly associated with decreased eGFR (adjusted odds ratio (OR) per standard deviation = 4.75 [2.66-8.48]; P < 0.001), albuminuria (OR = 1.49 [1.15-1.92]; P = 0.002), and, accordingly, with CKD (OR = 1.75 [1.35-2.26]; P < 0.001). GPC4 levels also significantly and independently predicted the incidence of newly diagnosed decreased eGFR (OR = 2.74 [1.82-4.14]; P < 0.001, albuminuria (OR = 1.58 [1.01-2.46]; P = 0.043, and CKD (OR = 2.16 [1.45-3.23]; P < 0.001). ROC analysis indicated an additional predictive value of GPC4 to a basic prediction model for newly diagnosed CKD and eGFR < 60 mL/min/1.73 m2. Our study, therefore, indicates that high serum GPC4 is associated with decreased prevalent and future kidney function.

Does the choice of intraoperative fluid modify abdominal aneurysm repair outcomes?: A cohort analysis.

Intraoperatively administered hydroxyethyl starch could be a risk indicator for postoperative acute kidney injury (AKI) in vascular surgical patients.In a single-center retrospective cohort analysis, we assessed the impact of hydroxyethyl starch and other risk indicators on AKI and mortality in 1095 patients undergoing elective open abdominal aneurysm repair (AAA-OR) or endovascular aortic repair (EVAR). We established logistic regression models to determine the effect of various risk indicators, including hydroxyethyl starch, on AKI, as well as Cox proportional hazard models to assess the effect on mortality.The use of intravenous hydroxyethyl starch was not associated with an increased risk of AKI or mortality. Patients undergoing EVAR were less likely to develop AKI (4% vs 18%). Multivariate risk indicators associated for AKI included suprarenal or pararenal aortic cross-clamp [odds ratio (OR), 4.44; 95% confidence interval (95% CI), 2.538-7.784; P < .001] and procedure length (OR, 1.005; 95% CI, 1.003-1.007; P < .001), and favored EVAR (OR, 0.351; 95% CI, 0.118-0.654; P < .01). Main multivariate risk indicators associated with mortality included patients needing an urgent procedure [hazard ratio (HR), 2.294; 95% CI, 1.541-3.413; P < .001], those with suprarenal or pararenal aortic cross-clamp (HR, 1.756; 95% CI, 1.247-2.472; P < .01), and patients undergoing EVAR (HR, 1.654; 95% CI, 1.292-2.118; P < .001).We found neither a benefit nor a negative effect of hydroxyethyl starch on the risk of AKI or mortality. Instead, other variables and comorbidities were found to be relevant for the development of postoperative AKI and survival. Nevertheless, clinicians should be aware of the high risk of postoperative AKI, particularly among those undergoing AAA-OR procedures.

Detection of pneumothoraces in patients with multiple blunt trauma: use and limitations of eFAST.

BACKGROUND: Extended focused assessment with sonography for trauma (eFAST) has been shown to have moderate sensitivity for detection of pneumothorax in trauma. Little is known about the location or size of missed pneumothoraces or clinical predictors of pneumothoraces in patients with false-negative eFAST. METHODS: This retrospective cross-sectional study includes all patients with multiple blunt trauma diagnosed with pneumothorax who underwent both eFAST and CT performed in the ED of a level 1 trauma centre in Switzerland between 1 June 2012 and 30 September 2014. Sensitivity of eFAST for pneumothorax was determined using CT as the gold standard. Demographic and clinical characteristics of those who had a pneumothorax detected by eFAST and those who did not were compared using the Mann-Whitney U or Pearson's χ2 tests. Univariate binary logistic regression models were used to identify predictors for pneumothoraces in patients with negative eFAST examination. RESULTS: The study included 109 patients. Overall sensitivity for pneumothorax on eFAST was 0.59 and 0.81 for pneumothoraces requiring treatment. Compared with those detected by eFAST, missed pneumothoraces were less likely to be ventral (30 (47.6%) vs 4 (9.3%), p <0.001) and more likely to be apical and basal (7 (11.1%) vs 15 (34.9%), p=0.003; 11 (17.5%) vs 18 (41.9%), p=0.008, respectively). The missed pneumothoraces were smaller than the detected pneumothoraces (left side: 30.7±17.4 vs 12.1±13.9 mm; right side: 30.2±10.1 vs 6.9±10.2 mm, both p <0.001). No clinical variables were identified which predicted pneumothoraces in falsely negative eFAST. Among those pneumothoraces missed by eFAST, 30% required tube thoracostomy compared with 88.9% of those detected with eFAST. CONCLUSION: In our study, pneumothoraces missed by eFAST were smaller and in atypical locations compared with those detected by eFAST and needed thoracic drainage less often.

High sensitive cardiac troponin T: Testing the test.

BACKGROUND: High sensitive cardiac troponin T (hs-TnT) found its way into everyday clinical routine to diagnose acute myocardial infarction (AMI). However, its levels vary considerably based on the underlying pathophysiology of the patients. Hence we sought to test the applicability of the currently only available hs-TnT assay (Roche Diagnostics, Switzerland) to diagnose acute myocardial infarction. METHODS AND PATIENTS: Retrospectively, we analyzed the hs-TnT results of 1573 patients admitted to a level A university hospital emergency department. Overall 323 patients had an acute cardiac event defined as Non-ST Elevated Myocardial Infarction (NSTEMI) and 286 patients had a ST-Elevated Myocardial Infarction (STEMI). 964 patients served as controls, consisting of patients with other cardiac and non-cardiac morbidity. RESULTS: The sensitivity of hs-TnT for detecting an acute cardiac event was more than 92% overall. The specificity varied around 35% depending on the respective patient cohort. ROC curve analysis of the initial hs-TnT results showed that the AUC in total cardiac events (STEMI and NSTEMI) was 0.81. Detailed analysis resulted in an AUC of 0.79 in NSTEMI and 0.84 in STEMI patients detected via the initial hs-TnT. We further tested the ESC algorithm for detecting NSTEMI and obtained a sensitivity of about 83%, while 43% of all non-NSTEMIs are classified as NSTEMIs. CONCLUSION: We show that the specificity of hs-TnT for AMI is very low and conclude that the current assay including its delta values represents myocardial damage of any origin. This damage alone does not substantiate an AMI diagnosis even when international algorithms are applied.

Somnambulism: Emergency Department Admissions Due to Sleepwalking-Related Trauma.

INTRODUCTION: Somnambulism is a state of dissociated consciousness, in which the affected person is partially asleep and partially awake. There is pervasive public opinion that sleepwalkers are protected from hurting themselves. There have been few scientific reports of trauma associated with somnambulism and no published investigations on the epidemiology or trauma patterns associated with somnambulism. METHODS: We included all emergency department (ED) admissions to University Hospital Inselspital, Berne, Switzerland, from January 1, 2000, until August 11, 2015, when the patient had suffered a trauma associated with somnambulism. Demographic data (age, gender, nationality) and medical data (mechanism of injury, final diagnosis, hospital admission, mortality and medication on admission) were included. RESULTS: Of 620,000 screened ED admissions, 11 were associated with trauma and sleepwalking. Two patients (18.2%) had a history of known non-rapid eye movement parasomnias. The leading cause of admission was falls. Four patients required hospital admission for orthopedic injuries needing further diagnostic testing and treatment (36.4%). These included two patients with multiple injuries (18.2%). None of the admitted patients died. CONCLUSION: Although sleepwalking seems benign in the majority of cases and most of the few injured patients did not require hospitalization, major injuries are possible. When patients present with falls of unknown origin, the possibility should be evaluated that they were caused by somnambulism.

Interprofessional and interdisciplinary simulation-based training leads to safe sedation procedures in the emergency department.

BACKGROUND: Sedation is a procedure required for many interventions in the Emergency department (ED) such as reductions, surgical procedures or cardioversions. However, especially under emergency conditions with high risk patients and rapidly changing interdisciplinary and interprofessional teams, the procedure caries important risks. It is thus vital but difficult to implement a standard operating procedure for sedation procedures in any ED. Reports on both, implementation strategies as well as their success are currently lacking. This study describes the development, implementation and clinical evaluation of an interprofessional and interdisciplinary simulation-based sedation training concept. METHODS: All physicians and nurses with specialised training in emergency medicine at the Berne University Department of Emergency Medicine participated in a mandatory interdisciplinary and interprofessional simulation-based sedation training. The curriculum consisted of an individual self-learning module, an airway skill training course, three simulation-based team training cases, and a final practical learning course in the operating theatre. Before and after each training session, self-efficacy, awareness of emergency procedures, knowledge of sedation medication and crisis resource management were assessed with a questionnaire. Changes in these measures were compared via paired tests, separately for groups formed based on experience and profession. To assess the clinical effect of training, we collected patient and team satisfaction as well as duration and complications for all sedations in the ED within the year after implementation. We further compared time to beginning of procedure, time for duration of procedure and time until discharge after implementation with the one year period before the implementation. Cohen's d was calculated as effect size for all statistically significant tests. RESULTS: Fifty staff members (26 nurses and 24 physicians) participated in the training. In all subgroups, there is a significant increase in self-efficacy and knowledge with high effect size (d z = 1.8). The learning is independent of profession and experience level. In the clinical evaluation after implementation, we found no major complications among the sedations performed. Time to procedure significantly improved after the introduction of the training (d = 0.88). DISCUSSION: Learning is independent of previous working experience and equally effective in raising the self-efficacy and knowledge in all professional groups. Clinical outcome evaluation confirms the concepts safety and feasibility. CONCLUSION: An interprofessional and interdisciplinary simulation-based sedation training is an efficient way to implement a conscious sedation concept in an ED.

Outcome and features of acute kidney injury complicating hypoxic hepatitis at the medical intensive care unit.

BACKGROUND: Hypoxic hepatitis (HH) is a frequent and potentially life-threatening event typically occurring in critically ill patients as a consequence of hemodynamic impairment. While acute kidney injury (AKI) has been well described in patients with acute liver failure, incidence and outcome of AKI accompanying HH are unclear. The aim of this study was to assess incidence, clinical implications and outcome of AKI and renal replacement therapy (RRT) in critically ill patients with HH. METHODS: A total of 1948 consecutive critically ill admissions were studied at the Medical University of Vienna. Laboratory and clinical parameters as well as the presence of HH and AKI were assessed on a daily basis. Outcome, renal recovery and length of stay were assessed and documented, and patients were followed for 1 year. RESULTS: A total of 295 admissions (15 %) developed HH. Main precipitators were cardiogenic (44 %) and septic shock (36 %). Occurrence of HH was significantly associated with AKI [OR 4.50 (95 % CI 3.30-6.12)] and necessity of renal replacement therapy [RRT; OR 3.36 (95 % CI 2.58-4.37)], p < 0.001 for both. Two hundred forty admissions with HH (81 %) developed AKI, 159 of whom (66 %) had AKI stage 3. Both HH and AKI were significantly linked to mortality. AKI stage 3, international normalized ratio (INR, during HH) and the presence of septic shock were identified as independent predictors of 28-day mortality in admissions with HH, whereas RRT was identified as an independent protective factor. There was a synergistic effect of HH and AKI on length of stay at the ICU. Of all HH survivors treated with RRT, 71 % showed renal recovery during follow-up. CONCLUSION: HH is frequently complicated by occurrence of AKI. Severity of HH, AKI stage and the presence of septic shock seem to contribute to poor outcome in these patients. Initiation of RRT in HH with AKI may enable renal recovery and should not be withheld in medical ICU patients.

Age is not associated with intracranial haemorrhage in patients with mild traumatic brain injury and oral anticoagulation.

BACKGROUND: Patients admitted to emergency departments with traumatic brain injury (TBI) are commonly being treated with oral anticoagulants. In contrast to patients without anticoagulant medication, no guidelines, scores or recommendations exist for the management of mild traumatic brain injury in these patients. We therefore tested whether age as one of the high risk factors of the Canadian head CT rule is applicable to a patient population on oral anticoagulants. METHODS: This cross-sectional analysis included all patients with mild TBI and concomitant oral anticoagulant therapy admitted to the Emergency Department, Inselspital Bern, Switzerland, from November 2009 to October 2014 (n = 200). Using a logistic regression model, two groups of patients with mild TBI on oral anticoagulant therapy were compared - those with and those without intracranial haemorrhage. RESULTS: There was no significant difference in age between the patient groups with (n = 86) and without (n = 114) intracranial haemorrhage (p = 0.078). In univariate logistic regression, GCS (OR = 0.419 (0.258; 0.680)) and thromboembolic event as reason for anticoagulant therapy (OR = 0.486 (0.257; 0.918)) were significantly associated with intracranial haemorrhage in patients with mild TBI and anticoagulation (all p < 0.05). However, there was no association with age (p = 0.078, OR = 1.024 (0.997; 1.051)), the type of accident or additional medication with acetylsalicylic acid or clopidogrel ((both p > 0.05; 0.552 (0.139; 2.202) and 0.256 (0.029; 2.237), respectively). CONCLUSION: Our study found no association between age and intracranial bleeding. Therefore, until further risk factors are identified, diagnostic imaging with CCT remains necessary for mild TBI patients on oral anticoagulation of all ages, especially those with therapeutic anticoagulation because of thromboembolic events.

Use of Diuretics is not associated with mortality in patients admitted to the emergency department: results from a cross-sectional study.

BACKGROUND: Patients with diuretic therapy are at risk for drug-induced adverse reactions. It is unknown if presence of diuretic therapy at hospital emergency room admission is associated with mortality. METHODS: In this cross sectional analysis, all emergency room patients 2010 and 2011 at the Inselspital Bern, Switzerland were included. A multivariable logistic regression model was performed to assess the association between pre-existing diuretic medication and 28 day mortality. RESULTS: Twenty-two thousand two hundred thirty-nine subjects were included in the analysis. A total of 8.5%, 2.5%, and 0.4% of patients used one, two, or three or more diuretics. In univariate analysis spironolactone, torasemide and chlortalidone use were associated with 28 day mortality (all p < 0.05). In a multivariate cox regression model no association with mortality was detectable (p > 0.05). No difference existed between patients with or without diuretic therapy (P > 0.05). Age and creatinine were independent risk factors for mortaliy (both p < 0.05). CONCLUSION: Use of diuretics is not associated with mortality in an unselected cohort of patients presenting in an emergency room.

Use of Calcium Channel Blockers is Associated with Mortality in Patients with Chronic Kidney Disease.

BACKGROUND/AIMS: The use of antihypertensive medicines has been shown to reduce proteinuria, morbidity, and mortality in patients with chronic kidney disease (CKD). A specific recommendation for a class of antihypertensive drugs is not available in this population, despite the pharmacodynamic differences. We have therefore analysed the association between antihypertensive medicines and survival of patients with chronic kidney disease. METHODS: Out of 2687 consecutive patients undergoing kidney biopsy a cohort of 606 subjects with retrievable medical therapy was included into the analysis. Kidney function was assessed by glomerular filtration rate (GFR) estimation at the time point of kidney biopsy. Main outcome variable was death. RESULTS: Overall 114 (18.7%) patients died. In univariate regression analysis the use of alpha-blockers and calcium channel antagonists, progression of disease, diabetes mellitus (DM) type 1 and 2, arterial hypertension, coronary heart disease, peripheral vascular disease, male sex and age were associated with mortality (all p<0.05). In a multivariate Cox regression model the use of calcium channel blockers (HR 1.89), age (HR 1.04), DM type 1 (HR 8.43) and DM type 2 (HR 2.17) and chronic obstructive pulmonary disease (HR 1.66) were associated with mortality (all p < 0.05). CONCLUSION: The use of calcium channel blockers but not of other antihypertensive medicines is associated with mortality in primarily GN patients with CKD.

Risk Factors for Intracranial Haemorrhage in Accidents Associated with the Shower or Bathtub.

BACKGROUND: There has been little research on bathroom accidents. It is unknown whether the shower or bathtub are connected with special dangers in different age groups or whether there are specific risk factors for adverse outcomes. METHODS: This cross-sectional analysis included all direct admissions to the Emergency Department at the Inselspital Bern, Switzerland from 1 January 2000 to 28 February 2014 after accidents associated with the bathtub or shower. Time, age, location, mechanism and diagnosis were assessed and special risk factors were examined. Patient groups with and without intracranial bleeding were compared with the Mann-Whitney U test.The association of risk factors with intracranial bleeding was investigated using univariate analysis with Fisher's exact test or logistic regression. The effects of different variables on cerebral bleeding were analysed by multivariate logistic regression. RESULTS: Two hundred and eighty (280) patients with accidents associated with the bathtub or shower were included in our study. Two hundred and thirty-five (235) patients suffered direct trauma by hitting an object (83.9%) and traumatic brain injury (TBI) was detected in 28 patients (10%). Eight (8) of the 27 patients with mild traumatic brain injuries (GCS 13-15), (29.6%) exhibited intracranial haemorrhage. All patients with intracranial haemorrhage were older than 48 years and needed in-hospital treatment. Patients with intracranial haemorrhage were significantly older and had higher haemoglobin levels than the control group with TBI but without intracranial bleeding (p<0.05 for both).In univariate analysis, we found that intracranial haemorrhage in patients with TBI was associated with direct trauma in general and with age (both p<0.05), but not with the mechanism of the fall, its location (shower or bathtub) or the gender of the patient. Multivariate logistic regression analysis identified only age as a risk factor for cerebral bleeding (p<0.05; OR 1.09 (CI 1.01;1.171)). CONCLUSION: In patients with ED admissions associated with the bathtub or shower direct trauma and age are risk factors for intracranial haemorrhage. Additional effort in prevention should be considered, especially in the elderly.

Hyperphosphatemia Is an Independent Risk Factor for Mortality in Critically Ill Patients: Results from a Cross-Sectional Study.

BACKGROUND: Phosphate imbalances or disorders have a high risk of morbidity and mortality in patients with chronic kidney disease. It is unknown if this finding extends to mortality in patients presenting at an emergency room with or without normal kidney function. METHODS AND PATIENTS: This cross sectional analysis included all emergency room patients between 2010 and 2011 at the Inselspital Bern, Switzerland. A multivariable cox regression model was applied to assess the association between phosphate levels and in-hospital mortality up to 28 days. RESULTS: 22,239 subjects were screened for the study. Plasma phosphate concentrations were measured in 2,390 patients on hospital admission and were included in the analysis. 3.5% of the 480 patients with hypophosphatemia and 10.7% of the 215 patients with hyperphosphatemia died. In univariate analysis, phosphate levels were associated with mortality, age, diuretic therapy and kidney function (all p<0.001). In a multivariate Cox regression model, hyperphosphatemia (OR 3.29, p<0.001) was a strong independent risk factor for mortality. Hypophosphatemia was not associated with mortality (p>0.05). CONCLUSION: Hyperphosphatemia is associated with 28-day in-hospital mortality in an unselected cohort of patients presenting in an emergency room.

Calcium Disorders in the Emergency Department: Independent Risk Factors for Mortality.

BACKGROUND: Calcium disorders are common in both intensive care units and in patients with chronic kidney disease and are associated with increased morbidity and mortality. It is unknown whether calcium abnormalities in unselected emergency department admissions have an impact on in-hospital mortality. METHODS: This cross-sectional analysis included all admissions to the Emergency Department at the Inselspital Bern, Switzerland from 2010 to 2011. For hyper- and hypocalcaemic patients with a Mann-Whitney U-test, the differences between subgroups divided by age, length of hospital stay, creatinine, sodium, chloride, phosphate, potassium and magnesium were compared. Associations between calcium disorders and 28-day in-hospital mortality were assessed using the Cox proportional hazard regression model. RESULTS: 8,270 patients with calcium measurements were included in our study. Overall 264 (3.2%) patients died. 150 patients (6.13%) with hypocalcaemia and 7 patients with hypercalcaemia (6.19%) died, in contrast to 104 normocalcaemic patients (1.82%). In univariate analysis, calcium serum levels were associated with sex, mortality and pre-existing diuretic therapy (all p<0.05). In multivariate Cox regression analysis, hypocalcaemia and hypercalcaemia were independent risk factors for mortality (HR 2.00 and HR 1.88, respectively; both p<0.01). CONCLUSION: Both hypocalcaemia and hypercalcaemia are associated with increased 28-day in-hospital mortality in unselected emergency department admissions.

Hypermagnesemia is a strong independent risk factor for mortality in critically ill patients: results from a cross-sectional study.

BACKGROUND: Patients with electrolyte imbalances or disorders have a high risk of mortality. It is unknown if this finding from sodium or potassium disorders extends to alterations of magnesium levels. METHODS AND PATIENTS: In this cross-sectional analysis, all emergency room patients between 2010 and 2011 at the Inselspital Bern, Switzerland, were included. A multivariable logistic regression model was performed to assess the association between magnesium levels and in-hospital mortality up to 28days. RESULTS: A total of 22,239 subjects were screened for the study. A total of 5339 patients had plasma magnesium concentrations measured at hospital admission and were included into the analysis. A total of 6.3% of the 352 patients with hypomagnesemia and 36.9% of the 151 patients with hypermagnesemia died. In a multivariate Cox regression model hypermagnesemia (HR 11.6, p<0.001) was a strong independent risk factor for mortality. In these patients diuretic therapy revealed to be protective (HR 0.5, p=0.007). Hypomagnesemia was not associated with mortality (p>0.05). Age was an independent risk factor for mortality (both p<0.001). CONCLUSION: The study does demonstrate a possible association between hypermagnesemia measured upon admission in the emergency department, and early in-hospital mortality.

Kidney biopsy results versus clinical parameters on mortality and ESRD progression in 2687 patients with glomerulonephritis.

BACKGROUND: Physicians refer proteinuric patients to kidney biopsy in order to clarify the issue of underlying renal disease. We compared kidney biopsy results with classical outcome parameters in a large cohort of patients with biopsy proven glomerulonephritis (GN). METHODS: In a retrospective analysis, 2687 patients with different forms of GN from 123 Austrian centres were investigated. Patient characteristics, the diagnosis of GN and its respective subtype and clinical symptoms such as arterial hypertension, haematuria, amount of proteinuria and estimated glomerular filtration rate (eGFR) were tested for their association with all-cause mortality and progression to end-stage renal disease (ESRD). RESULTS: During a median follow-up of 129·9 months (IQR 89·6; 177·7), 688 patients (25·6%) died and 718 patients required dialysis (29·4%). In multivariate Cox's regression analysis age (HR 1·06), female sex (HR 0·71), eGFR (HR 0·74), the diagnosis of GN and its subtypes predicted patient survival (all P < 0·01), whereas the amount of proteinuria was not associated with patient survival. The incidence of progression to ESRD was associated with female sex (HR 0·71), eGFR (HR 0·65), amount of proteinuria (HR 1·15) and the diagnosis of GN and its subtypes (all P < 0·01). Nephrotic or nephritic syndromes were not associated with patient survival or progression to ESRD and did not add further predictive value to outcome of GN. CONCLUSIONS: Our study demonstrates histological diagnosis of GN and its specific subtype predicts patient survival and dialysis incidence. Therefore, kidney biopsy should be an integral part of routine diagnostic assessment in patients with any forms of suspected GN.

Independent or synergistic relationship of proteinuria and glomerular filtration rate on patient and renal survival in patients with glomerulonephritis?

BACKGROUND: Glomerular filtration rate (GFR) in patients with chronic kidney disease (CKD) identifies patients at risk for death or end-stage renal disease (ESRD). CKD staging by GFR should incorporate proteinuria to augment risk stratification. We therefore tested the predictive power of the combination of GFR with proteinuria in patients with different histologically-diagnosed types of glomerulonephritis (GN). METHODS: In a retrospective analysis, 2,687 patients with different forms of GN from 123 Austrian centres were investigated. Full data sets were available from 1,892 subjects. Classes of CKD on the basis of estimated GFR (eGFR) and of proteinuria grouped as <1, 1-3.5, and >3.5 g/24 h were tested for their association with all-cause mortality and ESRD. RESULTS: During a median follow-up of 130 months [interquartile range (IQR) 90; 178] 478 patients (25.3 %) died. Median eGFR was 49 ml/min/1.73 m(2) (IQR 24; 81) and proteinuria 3.8 g/24 h (IQR 1.7; 8.0). Adjusted multivariate Cox regression indicated that renal survival but not overall survival is related to proteinuria >3.5 g/24 h [as opposed to <1 g/24 h; hazard ratio (HR) 1.91] and shows progression to ESRD. However, subgroup analyses revealed that this risk with proteinuria >3.5 g/24 h exists only in patients with immunoglobulin (Ig)A GN (HR 4.93), miscellaneous GN (HR 1.74), and CKD stage 5 (HR 2.50). Additionally, proteinuria is a risk factor for renal survival in males more than in females with GN and proteinuria >3.5 g/24 h (HR 1.91). CONCLUSION: Proteinuria is a strong risk factor for renal survival particularly in patients with proteinuria >3.5 g/24 but not for all types of GN, nor for all CKD stages. Proteinuria is not a risk factor for overall survival in patients with GN.

A single lung transplant in a patient with fabry disease: causality or far-fetched? A case report.

Introduction. Fabry disease is a rare X-linked lysosomal storage disorder, characterized by an α-galactosidase A deficiency resulting in globotriaosylceramide storage within cells. Subsequently, various organ systems are involved, clinically the most important are kidneys, the heart, and the peripheral and central nervous systems. Although obstructive lung disease is a common pathological finding in Fabry disease, pulmonary involvement is a clinically disregarded feature. Case Presentation. We report a patient with a diagnosis of chronic obstructive pulmonary disease (COPD) who received a single lung transplant in 2007. Later, a kidney biopsy revealed the diagnosis of Fabry disease, which was confirmed by enzymatic and genetic testing. Ultrastructural changes in a native lung biopsy were consistent with the diagnosis. Although the association of a lung transplant and Fabry disease appears far-fetched on first sight, respiratory impairment cannot be denied in Fabry disease. Conclusion. With this case presentation, we would like to stimulate discussion about rare differential diagnoses hidden beneath widespread disease and that a correct diagnosis is the base of an optimal treatment strategy for each patient. Overall, the patient might have benefited from specific enzyme replacement therapy, especially in view of the chronic kidney disease.

Long-term mortality in patients with chronic obstructive pulmonary disease following extracorporeal membrane oxygenation for cardiac assist after cardiovascular surgery.

PURPOSE: Information on predisposing risk factors influencing long-term survival after extracorporeal membrane oxygenation (ECMO) support remains scarce. In critically ill patients chronic obstructive pulmonary disease (COPD) is an independent risk factor for mortality and morbidity. We assessed the influence of COPD on cardiovascular and all-cause mortality in patients undergoing ECMO therapy. METHODS: We prospectively included 191 patients undergoing veno-arterial ECMO therapy following cardiovascular surgery at a university-affiliated tertiary care center into our registry. RESULTS: The median follow-up time was 51 months (IQR 34-71 months) corresponding to 4,197 overall months of follow-up. A total of 125 patients (65 %) died; 88 % of deaths were due to cardiovascular causes. Long-term survival was decreased in patients with COPD after 1 year (23 % vs. 44 %) and after 6 years (14 % vs. 35 %) compared to patients without COPD. COPD was independently associated with all-cause mortality with a hazard ratio of 4.22 (95 % CI 1.04-17.11, p = 0.04) and cardiovascular mortality with a hazard ratio of 5.87 (95 % CI 1.41-24.47, p = 0.02). CONCLUSIONS: We identified COPD as a strong and independent predictor of long-term all-cause mortality and cardiovascular mortality in patients undergoing ECMO therapy following cardiovascular surgery. The current study presents valuable information for a comprehensive decision-making process prior to ECMO implantation and helps to identify high-risk patients that may benefit from intensified treatment of co-morbidities and close check-ups after hospital discharge.