[Recurrent thromboembolisms despite full anticoagulation in a patient with antiphospholipid syndrome]. / Rezidivierende Thrombembolien trotz Vollantikoagulation bei Patientin mit Antiphospholipidsyndrom.

We report on a female patient with confirmed secondary antiphospholipid syndrome (APS) due to underlying systemic lupus erythematosus (SLE). Despite a thromboplastin time within the normal range (international normalized ratio, INR) under treatment with a vitamin K antagonist (VKA), a recurrent thrombotic event occurred, this time as pulmonary embolism due to bilateral deep vein thrombosis. Despite an INR value in the therapeutic range, clotting factors II, VII, IX and X were found to be insufficiently decreased suggesting inefficient anticoagulation. Thus, the anticoagulation regimen was changed to the direct oral anticoagulant dabigatran. This case demonstrates that the INR in APS patients may be artificially prolonged in rare cases, despite a normal activated partial thromboplastin time (aPTT) and cannot be used for monitoring VKA anticoagulant therapy. Suspicion of ineffective anticoagulation despite VKA therapy should prompt measurement of the individual clotting factors.

[Management of vascular anomalies in children]. / Prise en charge des anomalies vasculaires chez l'enfant.

Vascular anomalies, which are broadly identified as "angiomas", are rare entities and often unknown by the medical sphere. They are divided in two different categories which carry different prognosis and management: "vascular tumors" and "vascular malformations". Their precise identification is crucial and involves a good knowledge of the biological classification published by Mulliken and Glowacki and that has recently been updated by the International Society for the Study of Vascular Anomalies (ISSVA). Vascular tumors are benign, common, inborn or not and most of the time disappear with growth. Vascular malformations are always congenital and growth with the child. They can involve type of vessels solely or combined with others. A rheologic differentiation between slow and fast flow malformations is essential in order to characterize the seriousness of the lesion. Frequently, their diagnosis is clinically established and the anamnesis is conducted to answer three questions that are the time of revelation of the lesion ("When?"), its aspect ("What?") and its evolution ("How?"). Further investigations are usually not required but a non-invasive imaging technique such as Doppler ultrasound could be useful if a doubt exists. Surgery is not mandatory and must always be well thought because its consequences might be disastrous. It must be left to cosmetic sequelae of these lesions or to lesions that are totally resectable without causing any unacceptable deformation.

Detection of opsonic antibodies against Enterococcus faecalis cell wall carbohydrates in immune globulin preparations.

Three different commercially available polyvalent immune globulins (IG) were investigated for the existence of antibodies against cell wall carbohydrates of four different E. faecalis serotypes (using a cell wall carbohydrate-enzyme-linked immunosorbent assay), and whether these antibodies mediated opsonic killing (using an opsonic-killing assay). All three IG preparations contained antibodies against all four serotypes (CPS-A to CPS-D). However, only one of the three IG preparations showed opsonic killing against all four serotypes. Average killing was higher against serotypes A and B (72 and 79 %, respectively) than against serotypes C and D (30 and 37 %, respectively). Such IG preparations could play a role as an adjuvant therapeutic option in life-threatening infections with E. faecalis, particularly when resistant strains are involved.

The effect of spironolactone on diastolic function in haemodialysis patients.

Heart failure with preserved ejection fraction (HFpEF) is highly prevalent in patients on maintenance haemodialysis (HD) and lacks effective treatment. We investigated the effect of spironolactone on cardiac structure and function with a specific focus on diastolic function parameters. The MiREnDa trial examined the effect of 50 mg spironolactone once daily versus placebo on left ventricular mass index (LVMi) among 97 HD patients during 40 weeks of treatment. In this echocardiographic substudy, diastolic function was assessed using predefined structural and functional parameters including E/e'. Changes in the frequency of HFpEF were analysed using the comprehensive 'HFA-PEFF score'. Complete echocardiographic assessment was available in 65 individuals (59.5 ± 13.0 years, 21.5% female) with preserved left ventricular ejection fraction (LVEF > 50%). At baseline, mean E/e' was 15.2 ± 7.8 and 37 (56.9%) patients fulfilled the criteria of HFpEF according to the HFA-PEFF score. There was no significant difference in mean change of E/e' between the spironolactone group and the placebo group (+ 0.93 ± 5.39 vs. + 1.52 ± 5.94, p = 0.68) or in mean change of left atrial volume index (LAVi) (1.9 ± 12.3 ml/m2 vs. 1.7 ± 14.1 ml/m2, p = 0.89). Furthermore, spironolactone had no significant effect on mean change in LVMi (+ 0.8 ± 14.2 g/m2 vs. + 2.7 ± 15.9 g/m2; p = 0.72) or NT-proBNP (p = 0.96). Treatment with spironolactone did not alter HFA-PEFF score class compared with placebo (p = 0.63). Treatment with 50 mg of spironolactone for 40 weeks had no significant effect on diastolic function parameters in HD patients.The trial has been registered at clinicaltrials.gov (NCT01691053; first posted Sep. 24, 2012).

A young source of optical emission from distant radio galaxies.

DISTANT radio galaxies provide valuable insights into the properties of the young Universe-they are the only known extended optical sources at high redshift and might represent an early stage in the formation and evolution of galaxies in general. This extended optical emission often has very complex morphologies, but the origin of the light is still unclear. Here we report spectroscopic observations for several distant radio galaxies (0.75≤ z ≤ 1.1) in which the rest-frame spectra exhibit featureless continua between 2,500 Å and 5,000 Å. We see no evidence for the break in the spectrum at 4,000 Å expected for an old stellar population1-3, and suggest that young stars or scattered emissions from the active nuclei are responsible for most of the observed light. In either case, this implies that the source of the optical emission is com-parable in age to the associated radio source, namely 107 years or less.

Multicentre analysis of hyperglycaemic hyperosmolar state and diabetic ketoacidosis in type 1 and type 2 diabetes.

AIMS: To compare diabetes patients with hyperglycaemic hyperosmolar state (HHS), diabetic ketoacidosis (DKA), and patients without decompensation (ND). METHODS: In total, 500,973 patients with type 1 or type 2 diabetes of all ages registered in the diabetes patient follow-up (DPV) were included. Analysis was stratified by age (≤ / > 20 years) and by manifestation/follow-up. Patients were categorized into three groups: HHS or DKA-during follow-up according to the most recent episode-or ND. RESULTS: At onset of diabetes, HHS criteria were met by 345 (68.4% T1D) and DKA by 9824 (97.6% T1D) patients. DKA patients had a lower BMI(-SDS) in both diabetes types compared to ND. HbA1c was higher in HHS/DKA. During follow-up, HHS occurred in 1451 (42.2% T1D) and DKA in 8389 patients (76.7% T1D). In paediatric T1D, HHS/DKA was associated with younger age, depression, and dyslipidemia. Pump usage was less frequent in DKA patients. In adult T1D/T2D subjects, metabolic control was worse in patients with HHS/DKA. HHS and DKA were also associated with excessive alcohol intake, dementia, stroke, chronic kidney disease, and depression. CONCLUSIONS: HHS/DKA occurred mostly in T1D and younger patients. However, both also occurred in T2D, which is of great importance in the treatment of diabetes. Better education programmes are necessary to prevent decompensation and comorbidities.

[Coarctation of the aorta presenting as a pulsatile occipital mass]. / Coarctation de l'aorte révélée par une masse occipitale pulsatile.

A healthy 18 months old boy, is referred to our hospital for a thrilling pulsatile mass behind the left ear. Aortography in the ascending aorta shows a severe coarctation with almost interruption of the aorta between the left carotid and left subclavian artery. Both external carotid arteries provide an important collateral pathway through occipital arteries to two dilated vertebral arteries. Descending aorta is feeding by a reverse blood flow into vertebral and subclavian arteries. The child has been operated. This is an original presentation of severe coarctation of the aorta with development of an important and vital collateral pathway.

Influence of treatment modality on angiographic outcome after coronary stenting in diabetic patients: a controlled study.

OBJECTIVES: This retrospective study was designed to determine the six-month angiographic outcome after stenting of native coronary arteries in insulin-treated (ITDM) and non-ITDM patients with diabetes mellitus (DM) and compare the results with those in non-DM patients. BACKGROUND: The influence of the treatment modality for DM on restenosis in patients undergoing coronary artery stenting has not been elucidated sufficiently. METHODS: A total of 1,439 (70%) of 2,061 patients underwent repeated angiography within six months of coronary stenting. The ITDM and non-ITDM (oral hypoglycemic drugs or diet) were documented in 48 (3.3%) and 177 patients (12.3%), respectively, leaving 1,214 non-DM patients. RESULTS: Baseline reference vessel diameter tended to be smaller in ITDM patients (mean, 2.73 mm) than in non-DM and non-ITDM patients (2.88 mm and 2.85 mm, respectively). However, percent diameter stenosis was not different. The median number of stents deployed was 1; median stent length was 15 mm. Statistically significant differences were present after stenting for the means of minimal lumen diameter (MLD) and acute gain between ITDM patients (MLD: 2.67 mm, acute gain: 1.98 mm) and non-DM patients (MLD: 2.81 mm, acute gain: 2.16 mm). At follow-up, percent diameter stenosis, late lumen loss and loss index were significantly higher in both non-ITDM lesions (42%, 1.14 mm and 0.56, respectively) and ITDM lesions (48%, 1.26 mm and 0.65, respectively) than in non-DM lesions (35%, 0.96 mm and 0.45, respectively). The corresponding differences between non-ITDM and ITDM lesions did not reach statistical significance. Restenosis rates in non-DM, non-ITDM and ITDM lesions were 23.8%, 32.8% (p = 0.013 vs. non-DM) and 39.6% (p = 0.02 vs. non-DM, p = 0.477 vs. non-ITDM), respectively. CONCLUSIONS: This study showed that compared with stenting in non-DM patients, stenting of native coronary arteries in DM patients is associated with significantly increased lumen renarrowing, regardless of the treatment modality for DM.

Changes in coronary blood flow and myocardial metabolism during aortic balloon valvuloplasty.

The effects of balloon inflation on myocardial perfusion and metabolism were studied during aortic valvuloplasty in 17 patients with aortic stenosis, including 6 with associated coronary artery disease. Coronary sinus flow and blood samples were obtained before and during the first inflation, and 5 to 10 minutes after the last inflation. During inflation, coronary blood flow decreased (272 +/- 111 standard deviation to 166 +/- 92 ml/min; p less than 0.05), myocardial oxygen uptake fell and transcardiac lactate handling shifted from extraction to production (35 +/- 54 to -41 +/- 48 mumol/min; p less than 0.01). At the end of the procedure, aortic valve area had increased from 0.51 +/- 0.22 to 0.81 +/- 0.48 cm2 (p less than 0.002). Coronary sinus flow increased slightly above control values (+6%; difference not significant) and myocardial oxygen and lactate uptakes were back to control values. However, myocardial alanine production had increased from -3.6 to -6.6 mumol/min (p less than 0.05) and glutamine production was reduced or replaced by extraction (-3.3 +/- 2.1 to 3.5 +/- 3.8 mumol/min; p less than 0.05). Recovery of coronary flow, oxygen and lactate uptakes was not significantly different in patients with or without coronary artery disease, although the former patients tended to have less glutamine extraction and less improvement in their ejection fraction at the end of the procedure. Thus, aortic balloon valvuloplasty produces brief episodes of low-flow ischemia. Recovery of oxidative metabolism is almost immediate after deflation and no detrimental effect seems to persist at the end of the procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Ku autoimmune antigen is involved in placental regulation of rat P450c17 gene transcription.

The steroidogenic enzyme P450c17 (17alpha hydroxylase/C17,20 lyase) regulates a key branchpoint in steroidogenesis, as its activity directs the steroid biosynthetic pathways toward glucocorticoid or sex hormone synthesis. Expression of the P450c17 gene is transcriptionally regulated in steroidogenic tissues by cAMP. We showed that DNA between -84 and -55 in the rat P450c17 gene was bound uniquely by steroidogenic factor-1 (SF-1), which regulated both basal and cAMP-stimulated transcription in mouse adrenocortical and Leydig cells. SF-1 gene ablation experiments in mice indicate that SF-1 is not mandatory for placental steroidogenesis. We studied P450c17 gene regulation in the placenta using human placental JEG-3 trophoblast cells. Transfection of reporter luciferase gene constructs containing serial deletions of the 5' flanking region of the rat P450c17 gene showed that DNA between -98 and +13 mediated basal and cAMP-regulated transcription in placental JEG-3 cells, as it did in adrenal and Leydig cells. DNase footprints further identified a region between -88 and the TATA box that was bound by protein. Transfection of luciferase reporter constructs containing -84 to -55 of the rat P450c17 DNA ligated to the minimal promoter of the thymidine kinase gene showed that this DNA increased both basal and cAMP-simulated luciferase activity. Gel mobility shift assays identified two DNA-protein complexes with JEG-3 cell nuclear extracts that were different from complexes formed with MA-10 cell extracts and did not involve SF-1. Mutational analysis of the -84/-55 DNA showed that JEG-3 nuclear proteins bound to a site containing, but not identical to, the SF-1 sequence. One complex involved Ku autoimmune antigen, which bound to DNA sequence specifically. Overexpression of Ku antigen in MA-10 cells stimulated rat P450c17 gene transcription, thus demonstrating a biologic effect of Ku. Ku also bound to a similar region of the human P450c17 gene, and the DNA region to which Ku bound was transcriptionally active in JEG-3 cells. Ku was also found in extracts from rat placenta and bound to the -84/-55 rat P450c17 DNA. These data demonstrate a role of Ku in regulating P450c17 gene expression. These data further indicate that although human P450c17 is not normally expressed in the placenta, factors that could activate this gene are indeed present.

Sufentanil potentiates the inhibitory effect of epinephrine on intestinal motility.

OBJECTIVE: The aim of our study was to evaluate the effect of the combination of different catecholamines and sufentanil on peristalsis of the isolated guinea pig small bowel in vitro. DESIGN: In vitro study on excised guinea pig small-bowel segments (8-10 segments per substance tested). SETTING: Laboratory for experimental studies at the University. SUBJECTS: Isolated guinea pig small-bowel segments. INTERVENTIONS: Excised segments of guinea pig small bowel were mounted in a tissue bath (37 degrees C) in Tyrode's solution and bubbled with carbogen (95% O2/5% CO2). The lumina were perfused with Tyrode's solution at 0.5 ml/min. The test drugs (epinephrine, norepinephrine, dobutamine, sufentanil, and a combination of these catecholamines with sufentanil) were added to the tissue bath and peristalsis recorded via changes in the intraluminal pressure. One-way and two-way ANOVA were used for statistical analysis. MEASUREMENTS AND RESULTS: All the tested substances, both individually and in combination, inhibited intestinal peristalsis in a dose-dependent manner. High doses resulted in a complete blockade of peristalsis. Preexposure of the segments to sufentanil at 0.1 nM barely influenced the effects of the catecholamines on peristalsis. However, sufentanil at 0.3 nM enhanced the antiperistaltic activity of epinephrine in a supraadditive manner, whereas the effect on norepinephrine and dobutamine was less pronounced. CONCLUSIONS: Our experimental data suggest that the combination of epinephrine and sufentanil might be the worst choice for the intensive care setting. This is due to its pronounced inhibitory effect on peristalsis in vitro at moderate and higher concentrations.

Vasospasm after subarachnoid hemorrhage: diagnosis with MR angiography.

BACKGROUND AND PURPOSE: The possibility of treating intracranial vasospasm has increased the significance of its diagnosis and follow-up; however, so far, no ideal method is available. The goal of this study was to assess the accuracy of MR angiography versus intraarterial angiography (IA-DSA) in detecting vasospasm. METHODS: The study included 42 patients with acute spontaneous subarachnoid hemorrhage (SAH). Serial MR angiograms (minimum, two per patient within 10 days after the event; total, 149) were obtained prospectively using a 3D time-of-flight technique covering the circle of Willis at 0.5 T. Forty-seven MR angiograms could be compared with intraarterial angiograms obtained within 24 hours of MR angiography. Vascular narrowing on both studies was rated consensually by two pairs of neuroradiologists using a scale from 0 (no narrowing) to 3 (severe narrowing). Categories 0 and 1 were considered an absence of vasospasm and categories 2 and 3 a presence of vasospasm. RESULTS: Agreement between MR angiography and IA-DSA (assessed with weighted kappa statistics) was substantial for the middle and anterior cerebral arteries (MCA and ACA) but moderate for the internal carotid artery (ICA). The sensitivity, specificity, accuracy, and positive and negative predictive values of MR angiography for detecting patients with vasospasm were 92%, 98%, 96%, 92%, and 98%, respectively. Considering each vessel separately, specificity was high for all locations (95-99%) and sensitivity was excellent for the ACA (100%) but poorer for the ICA (25%) and MCA (56%). CONCLUSION: MR angiography at 0.5 T is capable of identifying vasospasm after acute SAH but is less sensitive than IA-DSA for depicting vasospasm in the ICA and MCA.

Diagnosis of intracranial aneurysms: accuracy of MR angiography at 0.5 T.

PURPOSE: Our goal was to determine the accuracy of MR angiography at 0.5 T for the diagnosis of intracranial aneurysms. METHODS: We retrospectively studied 140 patients, 70 with acute subarachnoid hemorrhage, who were either at high or low risk for intracranial aneurysm. Three-dimensional time-of-flight MR angiography was typically performed to cover the circle of Willis, with a volume thickness of 30 mm. Conventional spin-echo MR images and MR angiograms were reviewed together, and the results were compared with those obtained at intraarterial cerebral angiography to determine the sensitivity and specificity of MR angiography. RESULTS: Eighty-nine aneurysms (size range, 2 to 27 mm; 25 aneurysms < 5 mm) were identified at intraarterial cerebral angiography. Six aneurysms were missed by MR angiography and two were doubtful (sensitivity, 91% to 93%; specificity, 100%). Missed aneurysms were located outside the MR angiographic acquisition volume (n = 3) or on the carotid siphon (n = 3; size = 2, 3, and 5 mm). CONCLUSION: Even if MR angiography presents some restrictions in acquisition volume and spatial resolution, the detection rate of intracranial aneurysms is excellent at 0.5 T in both asymptomatic patients and in those with subarachnoid hemorrhage. A midfield system is not a restriction to the detection of intracranial aneurysms by MR examination.

[Imaging of intestinal ischemia]. / Imagerie des ischémies intestinales.

Ischemic bowel disease includes acute and chronic mesenteric ischemia, and colon ischemia. Cross-sectional imaging, and more particularly computed tomography, has an increasing role in the detection of acute and chronic mesenteric ischemia. Vascular obstructions or stenoses and changes in the bowel wall can be observed. Functional information can be added with MRI by using sequences that are sensitive to oxygen saturation in the superior mesenteric vein. Arteriography remains the reference examination in patients with acute mesenteric ischemia.

[Chronic arterial mesenteric ischemia: Doppler color ultrasonography demonstration of collateral flow]. / Ischémie mésentérique chronique d'origine artérielle: mise en évidence de réseaux de vicariance par échographie Doppler couleur.

Two cases of atypical mesenteric ischemia where color Doppler US demonstration of the underlying arterial abnormality and collateral supply was possible are presented. Significant stenosis of the celiac axis and thrombosis of the SMA were clearly depicted, along with the presence of collateral arterial supply. Endovascular treatment was successful in both cases. These cases confirm the possibility of detecting collateral flow at Doppler imaging in patients with mesenteric ischemia, both for diagnosis of mesenteric ischemia and endovascular treatment planning.

Saccular aneurysm of the external jugular vein, an unusual cause of neck swelling.

Enlargement of neck swelling on Valsalva maneuver raises the diagnosis of laryngocele or jugular aneurysm. When considering this latter type of lesion, though fusiform cervical venous aneurysms represent a frequent occurrence, sacciform aneurysms are much rarer. We report two exceptional cases of saccular aneurysms of the external jugular vein which presenting symptom was a neck lump that enlarged on Valsalva maneuver. Although computed tomography and selective venography allow accurate diagnosis, ultrasonography with color flow duplex imaging is the gold standard for the diagnosis of such venous aneurysms of the neck.

Delayed endovascular coil extrusion after embolisation for post-tonsillectomy haemorrhage: case report and literature review.

OBJECTIVE: To report a rare case of delayed endovascular coil extrusion following embolisation of a lingual artery pseudoaneurysm. CASE REPORT: A 23-year-old woman presented with dysphagia and odynophagia 11 months after having experienced massive post-tonsillectomy haemorrhage. At that time, the bleeding had been stopped by embolisation of a lingual artery pseudoaneurysm and the external carotid artery. Clinical examination at admission showed extrusion of the embolisation coils in the lateral lower pharyngeal wall. The coils were removed under general anaesthesia in the presence of an interventional radiologist. The procedure and post-operative period were without complication and no bleeding was observed. The dysphagia and pain disappeared and the subsequent seven-month follow-up period was uneventful. CONCLUSION: Although selective embolisation is a safe and effective treatment for severe post-tonsillectomy haemorrhage, the possibility of delayed coil extrusion should be kept in mind.