Emergency department CT examinations demonstrate no evidence of early viral circulation at the start of the COVID-19 pandemic-a multicentre epidemiological study.

BACKGROUND: Biological studies suggested that the COVID-19 outbreak in France occurred before the first official diagnosis on January 24, 2020. We investigated this controversial topic using a large collection of chest CTs performed throughout French emergency departments within 6 months before the 1st lockdown. RESULTS: Overall, 49,311 consecutive patients (median age: 60 years, 23,636/49,311 [47.9%] women) with available chest CT images and reports from 61 emergency departments between September 1, 2020, and March 16, 2020 (day before the 1st French lockdown), were retrospectively included in this multicentre study. In the macroscopic analysis of reports automatically (labelled for presence of ground glass opacities [GGOs], reticulations, and bilateral and subpleural abnormalities), we found a significant breakpoint on February 17, 2020, for the weekly time series with 1, 2 and ≥ 3 of these 4 radiological features, with 146/49,311 (0.3%) patients showing bilateral abnormalities and ground glass opacities (GGOs) from that day. According to radiologists, 22/146 (15.1%) CT images showed typical characteristics of COVID-19, including 4/146 (2.7%) before February 2020. According to hospital records, one patient remained without microbial diagnosis, two patients had proven influenza A and one patient had concomitant influenza A and mycoplasma infection. CONCLUSION: These results suggest that SARS-CoV-2 was not circulating in the areas covered by the 61 emergency departments involved in our study before the official beginning of the COVID-19 outbreak in France. In emergency patients, the strong resemblance among mycoplasma, influenza A and SARS-CoV-2 lung infections on chest CT and the nonspecificity of CT patterns in low prevalence periods is stressed. CRITICAL RELEVANCE STATEMENT: We proposed here an innovative approach to revisit a controversial 'real' start of the COVID-19 pandemic in France based on (1) a population-level approach combining text mining, time series analysis and an epidemiological dataset and (2) a patient-level approach with careful retrospective reading of chest CT scans complemented by analysis of samples performed contemporarily to the chest CT. We showed no evidence that SARS-CoV-2 was actively circulating in France before February 2020.

How artificial intelligence improves radiological interpretation in suspected pulmonary embolism.

OBJECTIVES: To evaluate and compare the diagnostic performances of a commercialized artificial intelligence (AI) algorithm for diagnosing pulmonary embolism (PE) on CT pulmonary angiogram (CTPA) with those of emergency radiologists in routine clinical practice. METHODS: This was an IRB-approved retrospective multicentric study including patients with suspected PE from September to December 2019 (i.e., during a preliminary evaluation period of an approved AI algorithm). CTPA quality and conclusions by emergency radiologists were retrieved from radiological reports. The gold standard was a retrospective review of CTPA, radiological and clinical reports, AI outputs, and patient outcomes. Diagnostic performance metrics for AI and radiologists were assessed in the entire cohort and depending on CTPA quality. RESULTS: Overall, 1202 patients were included (median age: 66.2 years). PE prevalence was 15.8% (190/1202). The AI algorithm detected 219 suspicious PEs, of which 176 were true PEs, including 19 true PEs missed by radiologists. In the cohort, the highest sensitivity and negative predictive values (NPVs) were obtained with AI (92.6% versus 90% and 98.6% versus 98.1%, respectively), while the highest specificity and positive predictive value (PPV) were found with radiologists (99.1% versus 95.8% and 95% versus 80.4%, respectively). Accuracy, specificity, and PPV were significantly higher for radiologists except in subcohorts with poor-to-average injection quality. Radiologists positively evaluated the AI algorithm to improve their diagnostic comfort (55/79 [69.6%]). CONCLUSION: Instead of replacing radiologists, AI for PE detection appears to be a safety net in emergency radiology practice due to high sensitivity and NPV, thereby increasing the self-confidence of radiologists. KEY POINTS: • Both the AI algorithm and emergency radiologists showed excellent performance in diagnosing PE on CTPA (sensitivity and specificity ≥ 90%; accuracy ≥ 95%). • The AI algorithm for PE detection can help increase the sensitivity and NPV of emergency radiologists in clinical practice, especially in cases of poor-to-moderate injection quality. • Emergency radiologists recommended the use of AI for PE detection in satisfaction surveys to increase their confidence and comfort in their final diagnosis.

Emergency teleradiological activity is an epidemiological estimator and predictor of the covid-19 pandemic in mainland France.

BACKGROUND: COVID-19 pandemic highlighted the need for real-time monitoring of diseases evolution to rapidly adapt restrictive measures. This prospective multicentric study aimed at investigating radiological markers of COVID-19-related emergency activity as global estimators of pandemic evolution in France. We incorporated two sources of data from March to November 2020: an open-source epidemiological dataset, collecting daily hospitalisations, intensive care unit admissions, hospital deaths and discharges, and a teleradiology dataset corresponding to the weekly number of CT-scans performed in 65 emergency centres and interpreted remotely. CT-scans specifically requested for COVID-19 suspicion were monitored. Teleradiological and epidemiological time series were aligned. Their relationships were estimated through a cross-correlation function, and their extremes and breakpoints were compared. Dynamic linear models were trained to forecast the weekly hospitalisations based on teleradiological activity predictors. RESULTS: A total of 100,018 CT-scans were included over 36 weeks, and 19,133 (19%) performed within the COVID-19 workflow. Concomitantly, 227,677 hospitalisations were reported. Teleradiological and epidemiological time series were almost perfectly superimposed (cross-correlation coefficients at lag 0: 0.90-0.92). Maximal number of COVID-19 CT-scans was reached the week of 2020-03-23 (1 086 CT-scans), 1 week before the highest hospitalisations (23,542 patients). The best valid forecasting model combined the number of COVID-19 CT-scans and the number of hospitalisations during the prior two weeks and provided the lowest mean absolute percentage (5.09%, testing period: 2020-11-02 to 2020-11-29). CONCLUSION: Monitoring COVID-19 CT-scan activity in emergencies accurately and instantly predicts hospitalisations and helps adjust medical resources, paving the way for complementary public health indicators.

Practical clinical and radiological models to diagnose COVID-19 based on a multicentric teleradiological emergency chest CT cohort.

Our aim was to develop practical models built with simple clinical and radiological features to help diagnosing Coronavirus disease 2019 [COVID-19] in a real-life emergency cohort. To do so, 513 consecutive adult patients suspected of having COVID-19 from 15 emergency departments from 2020-03-13 to 2020-04-14 were included as long as chest CT-scans and real-time polymerase chain reaction (RT-PCR) results were available (244 [47.6%] with a positive RT-PCR). Immediately after their acquisition, the chest CTs were prospectively interpreted by on-call teleradiologists (OCTRs) and systematically reviewed within one week by another senior teleradiologist. Each OCTR reading was concluded using a 5-point scale: normal, non-infectious, infectious non-COVID-19, indeterminate and highly suspicious of COVID-19. The senior reading reported the lesions' semiology, distribution, extent and differential diagnoses. After pre-filtering clinical and radiological features through univariate Chi-2, Fisher or Student t-tests (as appropriate), multivariate stepwise logistic regression (Step-LR) and classification tree (CART) models to predict a positive RT-PCR were trained on 412 patients, validated on an independent cohort of 101 patients and compared with the OCTR performances (295 and 71 with available clinical data, respectively) through area under the receiver operating characteristics curves (AUC). Regarding models elaborated on radiological variables alone, best performances were reached with the CART model (i.e., AUC = 0.92 [versus 0.88 for OCTR], sensitivity = 0.77, specificity = 0.94) while step-LR provided the highest AUC with clinical-radiological variables (AUC = 0.93 [versus 0.86 for OCTR], sensitivity = 0.82, specificity = 0.91). Hence, these two simple models, depending on the availability of clinical data, provided high performances to diagnose positive RT-PCR and could be used by any radiologist to support, modulate and communicate their conclusion in case of COVID-19 suspicion. Practically, using clinical and radiological variables (GGO, fever, presence of fibrotic bands, presence of diffuse lesions, predominant peripheral distribution) can accurately predict RT-PCR status.

Left atrial shape is independent predictor of arrhythmia recurrence after catheter ablation for atrial fibrillation: A shape statistics study.

BACKGROUND: Markers of left atrial (LA) shape may improve the prediction of postablation outcomes in atrial fibrillation (AF). Correlations to LA volume and AF persistence limit their incremental value over current clinical predictors. OBJECTIVE: To develop a shape score independent from AF persistence and LA volume using shape-based statistics, and to test its ability to predict postablation outcome. METHODS: Preablation computed tomography (CT) images from 141 patients with paroxysmal (57%) or persistent (43%) AF were segmented. Deformation of an average LA shape into each patient encoded patient-specific shape. Local analysis investigates regional differences between patient groups. Linear regression was used to remove shape variations related to LA volume and AF persistence, and to build a shape score to predict postablation outcome. Cross-validation was performed to evaluate its accuracy. RESULTS: Ablation failure rate was 23% over a median 12-month follow-up. Regions associated with ablation failure mostly consisted of a large area on posteroinferior LA, mitral isthmus, and left inferior vein. On univariate analysis, strongest predictors were AF persistence (P = .005), LA indexed volume (P = .02), and the proposed shape score (P = .001). On multivariate analysis, all 3 were independent predictors of ablation failure, with the LA shape score showing the highest predictive value (odds ratio [OR] = 6.2 [2.5-15.8], P < .001), followed by LA indexed volume (OR = 3.1 [1.2-7.9], P = .019) and AF persistence (OR = 2.9 [1.2-7.6], P = .022). CONCLUSION: Posteroinferior LA, mitral isthmus, and left inferior vein are the regions whose shape have the highest impact on outcome. LA shape predicts AF ablation failure independently from, and more accurately than, atrial volume and AF persistence.

The accuracy of teleradiologists in diagnosing COVID-19 based on a French multicentric emergency cohort.

OBJECTIVES: To evaluate the accuracy of diagnoses of COVID-19 based on chest CT as well as inter-observer agreement between teleradiologists during on-call duty and senior radiologists in suspected COVID-19 patients. MATERIALS AND METHODS: From March 13, 2020, to April 14, 2020, consecutive suspected COVID-19 adult patients who underwent both an RT-PCR test and chest CT from 15 hospitals were included in this prospective study. Chest CTs were immediately interpreted by the on-call teleradiologist and were systematically blind reviewed by a senior radiologist. Readings were categorised using a five-point scale: (1) normal; (2) non-infectious findings; (3) infectious findings but not consistent with COVID-19 infection; (4) consistent with COVID-19 infection; and (5) typical appearance of COVID-19 infection. The diagnostic accuracy of chest CT and inter-observer agreement using the kappa coefficient were evaluated over the study period. RESULTS: In total, 513 patients were enrolled, of whom 244/513 (47.6%) tested positive for RT-PCR. First readings were scored 4 or 5 in 225/244 (92%) RT-PCR+ patients, and between 1 and 3 in 201/269 (74.7%) RT-PCR- patients. The data were highly consistent (weighted kappa = 0.87) and correlated with RT-PCR (p < 0.001, AUC1st-reading = 0.89, AUC2nd-reading = 0.93). The negative predictive value for scores of 4 or 5 was 0.91-0.92, and the PPV for a score of 5 was 0.89-0.96 at the first and second readings, respectively. Diagnostic accuracy was consistent over the study period, irrespective of a variable prevalence rate. CONCLUSION: Chest CT demonstrated high diagnostic accuracy with strong inter-observer agreement between on-call teleradiologists with varying degrees of experience and senior radiologists over the study period. KEY POINTS: • The accuracy of readings by on-call teleradiologists, relative to second readings by senior radiologists, demonstrated a sensitivity of 0.75-0.79, specificity of 0.92-0.97, NPV of 0.80-0.83, and PPV of 0.89-0.96, based on "typical appearance," as predictive of RT-PCR+. • Inter-observer agreement between the first reading in the emergency setting and the second reading by the senior emergency teleradiologist was excellent (weighted kappa = 0.87).

OCT and CMR for the Diagnosis of Patients Presenting With MINOCA and Suspected Epicardial Causes.

OBJECTIVES: Among all patients presenting with myocardial infarction with nonobstructive coronary arteries (MINOCA), epicardial causes may be suspected when there is a correlation between electrocardiogram (ECG) changes and regional wall motion abnormalities (WMAs). We evaluated the diagnostic yield of intravascular optical coherence tomography (OCT) and cardiac magnetic resonance (CMR) in this specific setting. BACKGROUND: OCT is able to identify different morphologic features of coronary plaques that are well known causes of MINOCA. Furthermore, CMR has become the gold standard for detection of myocardial infarction in the setting of MINOCA. METHODS: In a prospective 2-center study, consecutive patients with MINOCA including ECG features of ischemia associated with corresponding WMAs underwent OCT and CMR. RESULTS: Forty patients (mean age: 50 ± 11 years, 62.5% male, 32.5% with ST-segment elevation) were enrolled. Coronary arteries were normal on coronary angiography in 10 patients (25%); 18 patients (45%) presented minimal lumen irregularities, whereas the remaining 12 patients (30%) showed mild to moderate (≥30% but <50%) coronary lesions. Plaque rupture, eruptive calcific nodule, plaque erosion, lone thrombus, and spontaneous coronary artery dissection were found in 14 (35%), 1 (2.5%), 12 (30%), 3 (7.5%), and 2 (5%) patients, respectively. Acute myocardial infarction was evident at CMR in 31 of 40 patients (77.5%). Twenty-three patients (57.5%) had a substrate and/or diagnosis supported by both techniques with an evident relationship between the findings obtained by the 2 techniques. By coupling OCT with CMR, a substrate and/or diagnosis was found in 100% of cases. CONCLUSIONS: OCT coupled with CMR can provide a clear substrate and/or diagnosis in the vast majority of patients presenting with MINOCA including ECG features of ischemia associated with corresponding WMAs.

COVID-19 impact assessment on the French radiological centers: a nationwide survey.

PURPOSE: To determine the impact of the COVID-19 on the CT activities in French radiological centers during the epidemic peak. MATERIALS AND METHODS: A cross-sectional prospective CT scan survey was conducted between March 16 and April 12, 2020, in accordance with the local IRB. Seven hundred nine radiology centers were invited to participate in a weekly online survey. Numbers of CT examinations related to COVID-19 including at least chest (CTcovid) and whole chest CT scan activities (CTchest) were recorded each week. A sub-analysis on French departments was performed during the 4 weeks of the study. The impact of the number of RT-PCRs (reverse transcriptase polymerase chain reactions) on the CT workflow was tested using two-sample t test and Pearson's test. RESULTS: Five hundred seventy-seven structures finally registered (78%) with mean response numbers of 336 ± 18.9 (323; 351). Mean CTchest activity per radiologic structure ranged from 75.8 ± 133 (0-1444) on week 12 to 99.3 ± 138.6 (0-1147) on week 13. Mean ratio of CTcovid on CTchest varied from 0.36 to 0.59 on week 12 and week 14 respectively. There was a significant relationship between the number of RT-PCR performed and the number of CTcovid (r = 0.73, p = 3.10-16) but no link with the number of positive RT-PCR results. CONCLUSION: In case of local high density COVID-19, CT workflow is strongly modified and redirected to the management of these specific patients. KEY POINTS: • Over the 4-week survey period, 117,686 chest CT (CTtotal) were performed among the responding centers, including 61,784 (52%) CT performed for COVID-19 (CTcovid). • Across the country, the ratio CTcovid/CTtotal varied from 0.36 to 0.59 and depended significantly on the local epidemic density (p = 0.003). • In clinical practice, in a context of growing epidemic, in France, chest CT was used as a surrogate to RT-PCR for patient triage.

High-Resolution Late Gadolinium Enhancement Magnetic Resonance for the Diagnosis of Myocardial Infarction With Nonobstructed Coronary Arteries.

OBJECTIVES: The aim of this study was to assess the diagnostic yield of cardiac magnetic resonance (CMR) including high-resolution (HR) late gadolinium enhancement (LGE) imaging using a 3-dimensional respiratory-navigated method in patients with myocardial infarction with nonobstructed coronary arteries (MINOCA). BACKGROUND: CMR plays a pivotal role for the diagnosis of patients with MINOCA. However, the diagnosis remains inconclusive in a significant number of patients, the results of CMR being either negative or uncertain (i.e., compatible with multiple diagnoses). METHODS: Consecutive patients categorized as having MINOCA after blood testing, electrocardiography, coronary angiography, and echocardiography underwent conventional CMR, including cine, T2-weighted, first-pass perfusion, and conventional breath-held LGE imaging. HR LGE imaging using a free-breathing method allowing improved spatial resolution (voxel size 1.25 × 1.25 × 2.5 mm) was added to the protocol when the results of conventional CMR were inconclusive and was optional otherwise. Diagnoses retained after reviewing conventional CMR were compared with those retained after the addition of HR LGE imaging. RESULTS: From 2013 to 2016, 229 patients were included (mean age 56 ± 17 years, 45% women). HR LGE imaging was performed in 172 patients (75%). In this subpopulation, definite diagnoses were retained after conventional CMR in 86 patients (50%): infarction in 39 (23%), myocarditis in 32 (19%), takotsubo cardiomyopathy in 13 (8%), and other diagnoses in 2 (1%). In the remaining 86 patients (50%), results of CMR were inconclusive: negative in 54 (31%) and consistent with multiple diagnoses in 32 (19%). HR LGE imaging led to changes in final diagnosis in 45 patients (26%) and to a lower rate of inconclusive final diagnosis (29%) (p < 0.001). In particular, HR LGE imaging could reveal or ascertain the diagnosis of infarction in 14% and rule out the diagnosis of infarction in 12%. HR LGE imaging was particularly useful when the results of transthoracic echocardiography, ventriculography, and conventional CMR were negative, with a 48% rate of modified diagnosis in this subpopulation. CONCLUSIONS: HR LGE imaging has high diagnostic value in patients with MINOCA and inconclusive findings on conventional CMR. This has major diagnostic, prognostic, and therapeutic implications.

Focal scar and diffuse myocardial fibrosis are independent imaging markers in repaired tetralogy of Fallot.

AIMS: To identify the correlates of focal scar and diffuse fibrosis in patients with history of tetralogy of Fallot (TOF) repair. METHODS AND RESULTS: Consecutive patients with prior TOF repair underwent electrocardiogram, 24-h Holter, transthoracic echocardiography, exercise testing, and cardiac magnetic resonance (CMR) including cine imaging to assess ventricular volumes and ejection fraction, T1 mapping to assess left ventricular (LV) and right ventricular (RV) diffuse fibrosis, and free-breathing late gadolinium-enhanced imaging to quantify scar area at high spatial resolution. Structural imaging data were related to clinical characteristics and functional imaging markers. Cine and T1 mapping results were compared with 40 age- and sex-matched controls. One hundred and three patients were enrolled (age 28 ± 15 years, 36% women), including 36 with prior pulmonary valve replacement (PVR). Compared with controls, TOF showed lower LV ejection fraction (LVEF) and RV ejection fraction (RVEF), and higher RV volume, RV wall thickness, and native T1 and extracellular volume values on both ventricles. In TOF, scar area related to LVEF and RVEF, while LV and RV native T1 related to RV dilatation. On multivariable analysis, scar area and LV native T1 were independent correlates of ventricular arrhythmia, while RVEF was not. Patients with history of PVR showed larger scars on RV outflow tract but shorter LV and RV native T1. CONCLUSION: Focal scar and biventricular diffuse fibrosis can be characterized on CMR after TOF repair. Scar size relates to systolic dysfunction, and diffuse fibrosis to RV dilatation. Both independently relate to ventricular arrhythmias. The finding of shorter T1 after PVR suggests that diffuse fibrosis may reverse with therapy.

Relationship between atrial scar on cardiac magnetic resonance and pulmonary vein reconnection after catheter ablation for paroxysmal atrial fibrillation.

INTRODUCTION: Pulmonary vein (PV) reconnection is frequent in patients showing atrial fibrillation (AF) recurrence after PV isolation (PVI). Its detection with cardiac magnetic resonance (CMR) may help predict outcome and guide redo procedures. We assessed the relationship between scar on CMR and PV reconnection after catheter ablation for paroxysmal AF. METHODS AND RESULTS: Fifty-one patients with paroxysmal AF underwent CMR before PVI using either a conventional single-electrode catheter (N = 28) or a circular multielectrode catheter (N = 23). At 3 months, a second CMR study was performed, followed by a systematic electrophysiological procedure to look for PV reconnection, regardless of AF recurrence. Preablation fibrosis and postablation scar were quantified and mapped from late gadolinium-enhanced CMR. CMR results were compared to the distribution and extent of PV reconnection. CMR and electrophysiological findings were compared between catheter types. Three months after successful PVI, scar gaps were found in 39 (76%) patients, and 78 (39%) veins. Electrical PV reconnection was detected in 45 (88%) patients, and 99 (50%) veins. The extent of PV reconnection related closely to the number of gaps (R = 0.55; P < .001), and to scar burden (R = -0.63; P < .001). However, the agreement was only fair for the localization of PV reconnection (k = 0.37; P < .001), scar gaps particularly lacking sensitivity in areas of pre-existing fibrosis. The circular catheter was associated with shorter procedures (P < .001), more scar (P = .01), less gaps (P = .01), and less reconnected veins (P = .03). CONCLUSION: PV reconnection is extremely frequent after PVI. CMR scar imaging accurately predicts its extent, but poorly predicts its location. Multielectrode circular catheters induce more complete ablation.

Clinical and genetic heterogeneity in familial steroid-sensitive nephrotic syndrome.

BACKGROUND: Familial steroid-sensitive nephrotic syndrome (SSNS) is a rare condition. The disease pathophysiology remains elusive. However, bi-allelic mutations in the EMP2 gene were identified, and specific variations in HLA-DQA1 were linked to a high risk of developing the disease. METHODS: Clinical data were analyzed in 59 SSNS families. EMP2 gene was sequenced in families with a potential autosomal recessive (AR) inheritance. Exome sequencing was performed in a subset of 13 families with potential AR inheritance. Two variations in HLA-DQA1 were genotyped in the whole cohort. RESULTS: Transmission was compatible with an AR (n = 33) or autosomal dominant (AD, n = 26) inheritance, assuming that familial SSNS is a monogenic trait. Clinical features did not differ between AR and AD groups. All patients, including primary (n = 7) and secondary steroid resistant nephrotic syndrone (SRNS), (n = 13) were sensitive to additional immunosuppressive therapy. Both HLA-DQA1 variations were found to be highly linked to the disease (OR = 4.34 and OR = 4.89; p < 0.001). Exome sequencing did not reveal any pathogenic mutation, neither did EMP2 sequencing. CONCLUSIONS: Taken together, these results highlight the clinical and genetic heterogeneity in familial SSNS. Clinical findings sustain an immune origin in all patients, whatever the initial steroid-sensitivity. The absence of a variant shared by two families and the HLA-DQA1 variation enrichments suggest a complex mode of inheritance.

Age, atrial fibrillation, and structural heart disease are the main determinants of left atrial fibrosis detected by delayed-enhanced magnetic resonance imaging in a general cardiology population.

INTRODUCTION: We studied the extent and distribution of left atrial (LA) fibrosis on delayed-enhanced (DE) MRI in a general cardiology population. METHODS AND RESULTS: One hundred ninety consecutive patients referred for cardiac MRI underwent DE imaging using a free breathing method. The population comprised 60 AF patients and 130 patients without AF, including 75 with structural heart disease (SHD). DE was quantified using histogram thresholding, expressed in % of the wall. Regression analysis was performed to identify predictors of DE. Additionally, DE was registered on a template to study its distribution in subpopulations. In the total population, age, AF, and SHD were independently associated with DE. DE was increasingly observed from 11.1 ± 4.7% in patients with no SHD nor AF, 18.8 ± 7.8% in SHD and no AF history, 22.9 ± 7.8% in paroxysmal AF, to 27.8 ± 7.7% in persistent AF. Among non-AF patients, age and SHD were independently associated with DE. Among AF patients, female gender and AF persistence were independently associated with DE. DE was variably distributed but more frequently detected in the posterior wall. CONCLUSION: Age, history of AF, and SHD are the most powerful predictors of atrial fibrosis, as detected by MRI, in a general cardiology population. Atrial fibrosis predominates in the posterior LA wall.

Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study.

BACKGROUND: Data on anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis are scarce in children. The current study is aimed at describing the clinical features and outcomes of childhood-onset ANCA-associated vasculitis (AAV). METHODS: We conducted a retrospective French multicentre study involving patients in whom AAV was diagnosed before the age of 18 years. Inclusion criteria were (i) granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) according to classification criteria of the European League Against Rheumatism/Paediatric Rheumatology European Society, and (ii) ANCA positivity. Patient and renal survival were analysed. RESULTS: Among 66 children included, 80% were female, 42% had GPA and 58% MPA including renal-limited vasculitis, 67% were pANCA+ and 33% cANCA+. The mean incidence of reported cases increased to 0.45 per million children/year in the period 2006-10. Median age at diagnosis was 11.5 years, and median time to diagnosis was 1 month. Initial symptoms included fever and fatigue (79%), skin lesions (41%), arthritis (42%), pulmonary (45%) and renal involvement (88%). Clinical features were similar between GPA and MPA with the exception of upper airway impairment (28%) specific of GPA. Ninety percent of the patients achieved remission after induction treatment. After a median follow-up of 5.2 years, 4 patients (6%) died, corresponding to a mortality rate of 1.2 per 100 person-years, and 22 patients (34%) developed end-stage renal disease (ESRD). Renal survival was 74, 70 and 59% at 1, 5 and 10 years, respectively. In a multivariable Cox regression model, baseline glomerular filtration rate, ethnic origin, histopathological classification and era of treatment were associated with the occurrence of ESRD. Relapse-free survival was 57% at 5 years and 34% at 10 years of follow-up. Patient and renal outcome did not significantly differ between GPA and MPA. CONCLUSION: Childhood-onset AAV is a rare disease characterized by female predominance, delayed diagnosis, frequent renal impairment and a high remission rate. Baseline GFR and new histopathological classification system are strong predictors of ESRD. Renal survival in childhood AAV has improved over time.

C3 nephritic factor associated with C3 glomerulopathy in children.

BACKGROUND: C3 glomerulopathy (C3G) is characterized by predominant C3 deposits in glomeruli and dysregulation of the alternative pathway of complement. Half of C3G patients have a C3 nephritic factor (C3NeF). C3G incorporated entities with a range of features on microscopy including dense deposit diseases (DDD) and C3 glomerulonephritis (C3GN). The aim of this work was to study children cases of C3G associated with C3NeF. METHODS: We reviewed 18 cases of C3G with a childhood onset associated with C3NeF without identified mutations in CFH, CFI, and MCP genes. RESULTS: Clinical histories started with recurrent hematuria for seven patients, nephrotic syndrome for four, acute post-infectious glomerulonephritis for three and acute renal failure for four. Twelve patients had a low C3 at first investigation. Kidney biopsy showed ten C3GN and eight DDD. Twenty-three percent of the patients tested presented elevated sC5b9. Seven patients relapsed 3 to 6 years after the onset. At the end of follow-up, two patients were under dialysis, 11 had a persistent proteinuria, five had none; four patients did not follow any treatment. Steroids were first used in 80 % of cases. CONCLUSIONS: C3NeF associated C3G has a heterogeneous presentation and outcome. Anti-proteinuric agents may control the disease during follow-up, even after nephrotic syndrome at the onset. The efficiency of immunosuppressive therapy remains questionable.

Kidney transplant with multiple renal artery grafts from deceased donors: are long-term graft and patient survival compromised?

BACKGROUND: Kidneys with multiple arteries are often transplanted. However, the long-term outcome of such kidneys recovered exclusively from deceased donors is not clear. OBJECTIVE: To determine whether use of renal grafts with multiple arteries affects long-term graft survival and function. METHODS: The outcomes of 259 consecutive kidney transplants between 1996 and 2000 were retrospectively reviewed. Patients were divided into 2 groups, multiple renal artery graft recipients (n = 70) and single renal artery graft recipients (n = 189). Short-term complications and long-term outcomes (survival rates, blood pressure after transplant, creatinine clearance, and proteinuria levels at 1, 3, 5, and 7 years after transplant) were compared between the 2 groups. RESULTS: Early vascular complications were more common (P = .02) in multiple artery graft recipients (18.6%) than in single artery graft recipients (7.9%), mainly because of occlusion of a polar artery in grafts with multiple renal arteries (7.1%). Urologic complications were no more frequent in one group than in the other (5.7% vs 5.3%; P = .89). The 2 groups did not differ significantly (P = .33) in long-term graft survival, with a median follow-up of 9.05 years (range, 0.1-12.7 years). Mean (SD) for creatinine clearance (59.4 [22.6] vs 55.9 [20.3] mL/min; P = .47), proteinuria (0.77 [2.1] vs 0.4 [0.8] g/24 h; P = .19), and systolic blood pressure (133.6 [14.5] vs 133.7 [17.5] mm Hg; P = .85) did not differ significantly between the 2 groups 7 years after transplant. CONCLUSIONS: Kidney transplant with grafts containing multiple renal arteries rather than grafts with a single renal artery does not significantly influence patient and graft outcomes.

Renal resistive index as a new independent risk factor for new-onset diabetes mellitus after kidney transplantation.

Pulse pressure and urinary albumin excretion were recently identified as risk factors of new-onset diabetes after renal transplantation (NODAT), suggesting that microvascular injury may be implicated in NODAT. However, the relationship between of microvascular injury and NODAT is unknown. In the present long-term (median follow-up: 5.7years; observation period: 4908 patient-years) retrospective study in 656 renal transplant recipients, the association between baseline renal resistance index (RI, used as a marker of widespread microvascular damage) and the incidence of NODAT was assessed. The incidence of NODAT was 11.2% and 14.6% at 5 and 10years, respectively, after transplantation. RI at 3months was a risk factor for NODAT [hazard ratio (HR) per 0.1: 2.19 (1.55-3.09), P<0.0001]. RI >0.75 (vs. 0≤0.75) was a potent a predictor of NODAT [HR: 3.29 (1.91-5.67), P<0.0001], even after adjustments [HR: 3.29 (1.50-7.24), P=0.0030] on age, weight, glucose, nephropathy, and arterial pressure. Similar results were observed when RI was measured at 1month [HR per 0.1:1.74 (1.33-2.27), P<0.0001] and 12months [HR per 0.1:1.74 (1.33-2.27), P<0.0001] after transplantation. High RI early after renal transplantation is a long-term risk factor for NODAT, and could be used to refine the individual risk of NODAT.

An unusual case of central venous catheter-related bacteremia.

Some hemodialysed patients need definitive central venous catheterization. One of the main complications is catheter infection, and each infection must be treated. We report a case of an unusual cause of central venous catheter (CVC) infection: physical examination and catheter opacification demonstrated two pin-holes in the catheter. It was possible to salvage the catheter following a treatment regimen combining systemic antibiotics, antibiotic locks, fibrinolytics, and removal of a catheter segment.

Doubling of serum creatinine in clinical trials, cost-effectiveness studies, and individual patients: adequate use in renal transplantation.

BACKGROUND: The predictive value of doubling of serum creatinine (DSC) has never been assessed in renal transplantation. We evaluated it in terms of its use for clinical trials, cost-effectiveness studies, and individual patients. METHODS: Retrospective longitudinal study in 896 renal transplant recipients. RESULTS: Death-censored graft loss occurred in 133 patients, during follow-up (up to 21 years). DSC was a risk factor for graft loss; however, the relative risk was different in patients with glomerular filtration rate less than 40 vs. more than or equal to 40 mL/min (hazard ratio: 14.5 [95% confidence interval: 7.4-28.4] vs. 47.8 [28.4-80.6], P=0.0051). Parameters influencing creatinine value (weight, age, sex) did not modify DSC's predictive value. The use of the composite endpoint DSC or death-censored graft loss instead of death-censored graft loss alone in clinical trials would reduce sample size by 7.1% to 9.0%. The annual probability of DSC to graft loss transition decreased from 76% (follow-up <1 year) to 5% (follow-up ≥10 years). Median graft half-life after DSC was 10 months [95% confidence interval: 6-18] but varied with increasing time to DSC (<1 year: 1 month [0.5-6]; 3-4.9 years: 15 months 5/67) and reference creatinine (<130 µmol/L: 3 months 2/6); ≥130 µmol/L: 25 months 15/37). CONCLUSIONS: DSC may be adequately used to refine the risk of death-censored graft loss for individual patients. However, the use of DSC as an endpoint in clinical trials marginally affects sample size, and the probability of DSC to graft loss transition is not constant, which limits the use of DSC in cost-effectiveness analyses of renal transplantation.