CIRSE Standards of Practice on Peri-operative Anticoagulation Management During Interventional Radiology Procedures.

This CIRSE Standards of Practice document is aimed at interventional radiologists and provides best practices for peri-operative anticoagulation management during interventional radiology procedures.

CIRSE standards of practice on gynaecological and obstetric haemorrhage.

This CIRSE Standards of Practice document provides best practices for obstetric haemorrhage embolisation (OHE) in the management of postpartum haemorrhage (PPH). The document is aimed at interventional radiologists involved in treating postpartum haemorrhage, and has been developed by a writing group established by the CIRSE Standards of Practice Committee.CIRSE Standards of Practice documents are not clinical practice guidelines and do not intend to impose a standard of care, rather provide reasonable approaches to and best practices for specific interventional radiology treatments and techniques.

Inflammation-associated gene expression in RAW 264.7 macrophages induced by toxins from fungi common on damp building materials.

Most fungi that grow on damp building materials produce low molecular weight compounds, some of which are known to be toxic. In this study, we tested the hypothesis that exposure to some metabolites of fungi common on damp building materials would result in time-, dose-, and compound-specific responses in the production of various chemokines by RAW 264.7 cells. Cell cultures were exposed to a 10-7M or 10-8M metabolite dose for 2, 4, 8 or 24h. Metabolite concentrations used were based on those that might be expected in alveolar macrophages due to inhalation exposure from living or working in a damp building. Compared to controls, exposure provoked significant time-, dose- and compound-specific responses manifest as differentially elevated secretion of three of nine cytokines tested in culture supernatant of treated cells. The greatest number of cytokines produced in response to the metabolites tested were in andrastin A-treated cells (GM-CSF, TGFß1, Tnf-α) followed by koninginin A (TGFß1 and Tnf-α) and phomenone (GM-CSF, TGFß1). Chaetoglobosin A, chaetomugilin D and walleminone exposures each resulted in significant time-specific production of Tnf-α only. This investigation adds to a body of evidence supporting the role of low molecular weight compounds from damp building materials as pathogen associated molecular patterns (PAMPs). Along with fungal glucan and chitin, these compounds contribute to the non-allergy based respiratory outcomes for people living and working in damp buildings.

Sustained benefit at 2 years for covered stents versus bare-metal stents in long SFA lesions: the VIASTAR trial.

PURPOSE: The hypothesis that covered stents are superior to bare-metal stents (BMS) in long femoropopliteal artery disease was tested. The one-year results of the VIASTAR trial revealed a patency benefit of covered stents in the treatment-per-protocol (TPP) analysis only. METHODS: A prospective, randomized, single-blind, multicenter study evaluated 141 patients with symptomatic peripheral arterial disease (PAD) after treatment with heparin-bonded covered stents (VIABAHN(®) Endoprosthesis) or BMS. Clinical outcomes and patency rates were assessed at 1, 6, 12, and 24 months. Mean lesion length was 19.0 ± 6.3 cm in the VIABAHN(®) versus 17.3 ± 6.6 cm in the BMS group. RESULTS: The 24-month primary patency rates in the VIABAHN(®) and BMS group were: intention-to-treat 63.1 (95 % CI 0.52-0.76) versus 41.2 % (95 % CI 0.29-0.57; log rank p = 0.04) and TPP 69.4 (95 % CI 0.58-0.83) versus 40.0 % (95 % CI 0.28-0.56; log rank p = 0.004). Freedom from target-lesion-revascularization (TLR) was 79.4 (95 % CI 0.70-0.90) versus 73.0 % (95 % CI 0.63-0.85) for VIABAHN(®) versus BMS (log rank p = 0.37). For the TPP group in lesions ≥20 cm, the 24-month patency rates were 65.2 (95 % CI 0.50-0.85) versus 26.7 % (95 % CI 0.12-0.59; log rank p = 0.004) for VIABAHN(®) versus BMS, and freedom from TLR was 80.0 (95 % CI 0.68-0.94) versus 61.9 % (95 % CI 0.44-0.87; log rank p = 0.13). The ankle brachial index was 0.89 ± 0.18 versus 0.91 ± 0.17 (p = 0.76) at 24-month in the VIABAHN(®) versus the BMS group, respectively. CONCLUSION: At 24-month, this trial in PAD patients with long femoropopliteal lesions demonstrated a significantly improved primary patency rate for heparin-bonded covered stents compared to BMS, however, without a significant impact on clinical outcomes and TLR rate (Reg. Nr. ISRCTN48164244).

Heparin-bonded covered stents versus bare-metal stents for complex femoropopliteal artery lesions: the randomized VIASTAR trial (Viabahn endoprosthesis with PROPATEN bioactive surface [VIA] versus bare nitinol stent in the treatment of long lesions in superficial femoral artery occlusive disease).

OBJECTIVES: The hypothesis that endovascular treatment with covered stents has equal risks but higher efficacy than bare-metal stents (BMS) in long femoropopliteal artery disease was tested. BACKGROUND: Although endovascular treatment of short superficial femoral artery lesions revealed excellent results, efficacy in long lesions remains unsatisfactory. METHODS: In a prospective, randomized, single-blind, multicenter study, 141 patients with symptomatic peripheral arterial disease were assigned to treatment with heparin-bonded, covered stents (Viabahn 72 patients) or BMS (69 patients). Clinical outcomes and patency rates were assessed at 1, 6, and 12 months. RESULTS: Mean ± SD lesion length was 19.0 ± 6.3 cm in the Viabahn group and 17.3 ± 6.6 cm in the BMS group. Major complications within 30 days were observed in 1.4%. The 12-month primary patency rates in the Viabahn and BMS groups were: intention-to-treat (ITT) 70.9% (95% confidence interval [CI]: 0.58 to 0.80) and 55.1% (95% CI: 0.41 to 0.67) (log-rank test p = 0.11); treatment per-protocol (TPP) 78.1% (95% CI: 0.65 to 0.86) and 53.5% (95% CI: 0.39 to 0.65) (hazard ratio: 2.23 [95% CI: 1.14 to 4.34) (log-rank test p = 0.009). In lesions ≥20 cm, (TransAtlantic Inter-Society Consensus class D), the 12-month patency rate was significantly longer in VIA patients in the ITT analysis (VIA 71.3% vs. BMS 36.8%; p = 0.01) and the TPP analysis (VIA 73.3% vs. BMS 33.3%; p = 0.004). Freedom from target lesion revascularization was 84.6% for Viabahn (95% CI: 0.72 to 0.91) versus 77.0% for BMS (95% CI: 0.63 to 0.85; p = 0.37). The ankle-brachial index in the Viabahn group significantly increased to 0.94 ± 0.23 compared with the BMS group (0.85 ± 0.23; p < 0.05) at 12 months. CONCLUSIONS: This randomized trial in symptomatic patients with peripheral arterial disease who underwent endovascular treatment for long femoropopliteal lesions demonstrated significant clinical and patency benefits for heparin-bonded covered stents compared with BMS in lesions ≥20 cm and for all lesions in the TPP analysis. In the ITT analysis for all lesions, which was flawed by major protocol deviations in 8.5% of the patients, the difference was not significant. (GORE VIABAHN® endoprosthesis with bioactive propaten surface versus bare nitinol stent in the treatment of TASC B, C and D lesions in superficial femoral artery occlusive disease; ISRCTN48164244).

Induction of Dectin-1 and asthma-associated signal transduction pathways in RAW 264.7 cells by a triple-helical (1, 3)-ß-D glucan, curdlan.

People living in damp buildings are typically exposed to spore and mycelial fragments of the fungi that grow on damp building materials. There is experimental evidence that this exposure to triple-helical (1, 3)-ß-D glucan and low molecular weight toxins may be associated with non-atopic asthma observed in damp and moldy buildings. However, the mechanisms underlying this response are only partially resolved. Using the pure (1, 3)-ß-D glucan, curdlan, and the murine macrophage cell line, RAW 264.7, there were two objectives of this study. The first was to determine whether signal transduction pathways activating asthma-associated cell signaling pathways were stimulated using mouse transduction Pathway Finder(®) arrays and quantitative real-time (QRT) PCR. The second objective was to evaluate the dose and temporal responses associated with transcriptional changes in asthma-associated cytokines, the signal transduction receptor gene Dectin-1, and various transcription factor genes related to the induction of asthma using customized RT-PCR-based arrays. Compared to controls, the 10(-7) M curdlan treatment induced significant changes in gene transcription predominately in the NFkB, TGF-ß, p53, JAK/STAT, P13/AKT, phospholipase C, and stress signaling pathways. The 10(-8) M curdlan treatment mainly induced NFkB and TGF-ß pathways. Compared to controls, curdlan exposures also induced significant dose- and time-dependent changes in the gene translations. We found that that curdlan as a non-allergenic potentiator modulates a network of transduction signaling pathways not only associated with TH-1, TH-2, and TH-3 cell responses including asthma potentiation, but a variety of other cell responses in RAW 264.7 cells. These results help provide mechanistic basis for some of the phenotypic changes associated with asthma that have been observed in in vitro, in vivo, and human studies and open up a hypothesis-building process that could explain the rise of non-atopic asthma associated with fungi.

Percutaneous transluminal angioplasty versus turbostatic carbon-coated stents in infrapopliteal arteries: InPeria II trial.

PURPOSE: To determine the clinical outcome and the success of stent application for high-grade lesions of the infrapopliteal arteries compared with treatment with percutaneous transluminal angioplasty (PTA) in critical limb ischemia (CLI). MATERIALS AND METHODS: In this ethics board-approved randomized prospective study, PTA or stent application was performed on 131 lesions in 88 patients with CLI. The primary end points were clinical improvement after endovascular treatment and limb salvage rate. Secondary end points were defined by the minimal lumen diameter (MLD) before and after the revascularization procedure, percentage of residual diameter stenosis (DS), binary restenosis rate (>50% DS and >70% DS), and incidence of target lesion revascularization at 9-month follow-up. RESULTS: At 3 months, the clinical status in the PTA group was less improved than that in the stent group (P = .008). At 9 months, there had been five minor and two major amputations in the PTA group and five major and five minor amputations in the stent group. MLD was significantly larger and the percentage of DS was significantly less in the stent group at completion angiography. At 9 months, the angiographic control showed better trends for the stent group in comparison to the PTA group despite that no significant differences were detected (MLD, 1.19 mm ± 0.92 vs 1.02 mm ± 1.02; DS, 38.68% ± 25.47 vs 43.31% ± 28.37). CONCLUSION: Infrapopliteal stent application is an effective treatment modality in CLI. The PTA and stent groups were essentially equal at 3 and 9 months except for the difference in clinical improvement in the stent group at 3 months.

Effects of low molecular weight fungal compounds on inflammatory gene transcription and expression in mouse alveolar macrophages.

The inflammatory potential and molecular mechanisms underscoring inflammatory responses of lung cells to compounds from fungi that grow on damp building materials is poorly understood in vitro. In this study we evaluated the effect of pure fungal compounds on potentiating acute inflammatory response in primary mouse alveolar macrophages (AMs) and tested the hypothesis that AM responses to low molecular weight fungal compounds exhibit temporal and compound specificity that mimic that observed in the whole lung. Transcriptional responses of 13 inflammation/respiratory burst-associated genes (KC=Cxcl1, Cxcl2, Cxcl5, Cxcl10, Ccl3, Ccl112, Ccl20, IL-1ß, Il-6, ifi27 Tnfα, iNOS and Blvrb) were evaluated in mouse AMs exposed to a 1ml (10(-8)mol) dose of either pure atranone C, brevianimide, cladosporin, curdlan, LPS, neoechinulin A & B, sterigmatocystin or TMC-120A for 2h, 4h and 12h PE using customized reverse transcription (RT)-PCR based arrays. Multianalyte ELISA was used to measure expression of 6 pro-inflammatory cytokines common to the transcriptional assays (Cxcl1, Cxcl10, Ccl3, IL1ß, Ifn-λ and Tnf-α) to determine whether gene expression corresponded to the transcription data. Compared to controls, all of these compounds induced significant (≥2.5-fold or ≤-2.5-fold change at p≤0.05) time- and compound-specific transcriptional gene alterations in treatment AMs. The highest number of transcribed genes were in LPS treatment AMs at 12h PE (12/13) followed by neoechinulin B at 4h PE (11/13). Highest fold change values (>30) were associated with KC, Cxcl2, Cxcl5 and IL1ß genes in cells exposed to LPS. Compound exposures also induced significant (p≤0.05) time- and compound-specific pro-inflammatory responses manifest as differentially elevated Cxcl1, Cxcl10, Ccl3, Ifn-λ and Tnf-α concentrations in culture supernatant of treatment AMs. Dissimilarity in transcriptional responses in AMs and our in vivo model of lung disease is likely attributable to whole lung vs. isolated cell responsive and dose differences between the two studies. The results not only indicate that low molecular weight compounds from fungi that grow in damp built environments are potently pro-inflammatory in vitro, it further highlights the important role AMs play in innate lung defence, and against exposure to low molecular weight fungal compounds. These observations further support our position that exposure to low molecular weight compounds from indoor-associated fungi may provoke some of the inflammatory health effects reported from humans in damp building environments. They also open up a hypothesis building process that could explain the rise of non-atopic asthma associated with fungi.

Dectin-1 and inflammation-associated gene transcription and expression in mouse lungs by a toxic (1,3)-beta-D glucan.

The form of (1-3)-beta-D glucan found in the cell walls of the anamorphic Trichocomaceae that grow on damp building materials is considered to have potent toxic and inflammatory effects on cells of the respiratory system. It is also considered to have a potential role in the development of non-allergenic respiratory health effects. While human studies involving experimental exposures all point to the inflammatory potential of pure curdlan, a linear (1-3)-beta-D glucan in a triple helix configuration, animal experiments result in conflicting conclusions concerning the inflammatory potency of this glucan. However, because mice appear to be a better model than guinea pigs for exploring the respiratory effects of curdlan and because molecular mechanisms associated with this glucan remain largely unknown, we conducted further work to clarify the role of curdlan on the inflammatory response using our mouse model of lung disease. This study used in situ hybridization (ISH) to probe dectin-1 mRNA transcription with a digoxigenin-labeled cDNA probe, with reverse transcription (RT)-PCR based arrays used to measure inflammation gene and receptor transcriptional responses. Also, immunohistochemistry (IHC) was used to probe dectin-1 as well as anti-mouse Ccl3, Il1-alpha, and TNF-alpha expression to evaluate dose and time-course (4 and 12 h) postexposure (PE) response patterns in the lungs of intratracheally instilled mice exposed to a single 50 mul dose of curdlan at 10(-7), 10(-8), 10(-9), and 10(-10) M/animal (=4 mug to 4 ng curdlan/kg lung wt). Dectin-1 mRNA transcription and expression was observed in bronchiolar epithelium, alveolar macrophages (AMs), and alveolar type II cells (ATIIs) of lungs exposed to 4 mug to 40 ng curdlan/kg lung wt, at both time points. Compared to controls, array analysis revealed that 54 of 83 genes assayed were significantly modulated by curdlan. mRNA transcription patterns showed both dose and time dependency, with highest transcription levels in 10(-7) and 10(-8) M treatment animals, especially at 4-h PE. Nine gene mRNA transcripts (Ccl3, Ccl11, Ccl17, Ifng, Il1alpha, Il-20, TNF-alpha, Tnfrsf1b, and CD40lg) were significantly expressed at all doses suggesting they may have a central role in immunomodulating curdlan exposures. IHC revealed Ccl3, Il1-alpha, and TNF-alpha expression in bronchiolar epithelium, AMs and ATIIs illustrate the important immunomodulatory role that these cells have in the recognition of, and response to glucan. Collectively, these results confirm the inflammatory nature of curdlan and demonstrate the complex of inflammation-associated gene responses induced by (1-3)-beta-D glucan in triple helical forms. These observations also provide a biological basis for the irritant and inflammatory response to curdlan observed in humans and animals in experimental studies.

Infragenicular stent implantation for below-the-knee atherosclerotic disease: clinical evidence from an international collaborative meta-analysis on 640 patients.

PURPOSE: To report a systematic review of the literature published on the outcomes of stenting for below-the-knee disease in patients with critical limb ischemia (CLI). METHODS: Potentially relevant studies of stent implantation in the infragenicular arteries in >or=5 patients with >or=1-month follow-up were systematically sought in BioMedCentral, ClinicalTrials.gov, The Cochrane Collaboration Register of Controlled Trials (CENTRAL), Google Scholar, and PubMed. Data were abstracted and pooled with a random-effect model to generate risk estimates with 95% confidence intervals (CI). Interaction tests were performed to compare different stent types. A risk of bias assessment was conducted separately, as were appraisals for small study bias, statistical heterogeneity, and inconsistency. RESULTS: Eighteen nonrandomized studies were retrieved comprising 640 patients. After a median follow-up of 12 months, binary in-stent restenosis occurred in 25.7% (95% CI 11.6% to 40.0%), primary patency in 78.9% (95% CI 71.8% to 86.0%), improvement in Rutherford class in 91.3% (95% CI 85.5% to 97.1%), target vessel revascularization in 10.1% (95% CI 6.2% to 13.9%), and limb salvage in 96.4% (95% CI 94.7% to 98.1%). Head-to-head comparisons showed that sirolimus-eluting stents were superior to balloon-expandable bare metal stents in preventing restenosis and increasing primary patency (both p<0.001); sirolimus-eluting stents were also better than paclitaxel-eluting stents in terms of primary patency (p<0.001) and repeat revascularizations (p = 0.014). CONCLUSION: Percutaneous infragenicular stent implantation after failed or unsuccessful balloon angioplasty is associated with favorable clinical results in patients with CLI. Notwithstanding limitations of primary studies, sirolimus-eluting stents appear superior to bare metal and paclitaxel-eluting stents in terms of angiographic and/or clinical outcomes.

Family clusters of variant X-linked chronic granulomatous disease.

Chronic granulomatous disease (CGD) is a rare inherited immunodeficiency disorder. The clinical presentation is varied depending on the degree of involvement of the NADPH oxidase system responsible for the oxidative burst of neutrophils. We present 3 cases of variant X-linked CGD in an effort to introduce the disease and highlight the importance and limitations of CGD screening. The variant X-linked form of CGD results in a less severe phenotype and frequently presents later in life. Variant X-linked CGD is difficult to diagnose, but is becoming more readily recognized based on improved testing methods. A high index of suspicion in the setting of unusual infections such as Burkholderia cepacia pneumonia is essential to make the diagnosis. Family screening can lead to early intervention, prophylaxis, and appropriate genetic counseling.

Translumbar treatment of type II endoleaks after endovascular repair of abdominal aortic aneurysm.

The modality of treatment and the appropriate time point to treat type II endoleaks after endovascular repair of abdominal aortic aneurysms (EVAR) remain controversial issues. The purpose of the present study was to assess the efficacy of translumbar embolization of type II endoleaks after endovascular repair of aortic aneurysm repair. Eighty-four consecutive patients after EVAR were analyzed for the onset of type II endoleaks. Of these, five patients had experienced translumbar embolization after ineffective intraartrial approach to exclude the endoleak. A combination of several liquid embolic agents was used as sealant. Post-procedural contrast-enhanced ultrasound (CEUS) was used to document the outcome of the embolization. Translumbar embolization was successful in four patients. Complete sealing of the nidus was seen on CEUS 24 h after the procedure. In one patient with a duplication of the inferior vena cava, the procedure was aborted because an additional type Ib endoleak was found. The procedure was well tolerated by all patients. The translumbar approach to treat growing aneurysm sacs in patients with persistent type II endoleaks is safe and well tolerated. The immediate post-interventional outcome as documented on CEUS is promising. Long-term follow-ups are yet to be performed.

Drug-loaded microspheres for the treatment of liver cancer: review of current results.

Transarterial chemoembolization (TACE) involves the emulsification of a chemotherapeutic agent in a viscous drug carrier, delivered intra-arterially to liver tumor for maximum effect. TACE reduces arterial inflow, diminishes washout of the chemotherapeutic agent, and decreases systemic exposure. Despite evidence of some clinical success with TACE, a new type of microspheres with drug-eluting capabilities may offer a precisely controlled and sustainable release of the chemotherapeutic agent into the tumor bed. In animal trials tumor necrosis (approaching 100%) was greatest at 7 days, with significantly lower plasma concentrations of doxorubicin than in control animals treated with doxorubicin intra-arterially. Clinically, drug-eluting microspheres loaded with doxorubicin, either at 75 mg/m(2) or at a fixed dose of 150 mg, were used recently and no severe disorders of the hepatic function were observed postprocedure, while a substantial reduction of the fetoprotein levels occurred. An interim analysis of the first 15 patients from the Hong Kong group at 3 months showed an objective response rate of 61.54% and 53.84% according to EASL criteria and RECIST criteria, respectively, and a survival rate of 93.3%. In this paper we present how to use microspheres loaded with doxorubicin and review their clinical value and preliminary performance for treatment of unresectable liver cancer.

Immunohistochemical and immunocytochemical detection of SchS34 antigen in Stachybotrys chartarum spores and spore impacted mouse lungs.

The purpose of this study was to evaluate the distribution of a 34 kD antigen isolated from S. chartarum sensu lato in spores and in the mouse lung 48 h after intra-tracheal instillation of spores by immuno-histochemistry. This antigen was localized in spore walls, primarily in the outer and inner wall layers and on the external wall surfaces with modest labelling observed in cytoplasm. Immuno-histochemistry revealed that in spore impacted mouse lung, antigen was again observed in spore walls, along the outside surface of the outer wall and in the intercellular space surrounding spores. In lung granulomas the labelled antigen formed a diffusate, some 2-3x the size of the long axis of spores, with highest concentrations nearest to spores. Collectively, these observations indicated that this protein not only displayed a high degree of specificity with respect to its location in spores and wall fragments, but also that it slowly diffuses into surrounding lungs.

Endovascular management performed percutaneously of isolated iliac artery aneurysms.

PURPOSE: To report about the endovascular treatment of isolated iliac artery aneurysms (IIAA) with stentgraft placement and transluminal or CT-guided embolization of the internal iliac artery or the combination of these methods. METHODS AND MATERIALS: Over a period of 5.6 years, 36 interventions were performed in 20 patients with 23 IIAAs. In a retrospective analysis patient records were reviewed. The CT-angiography follow-up was evaluated for the presence of re-perfusion of the IIAA and for change of aneurysm diameter. RESULTS: Primary success was achieved in 15/23 aneurysms (65%), and secondary success in 21/23 aneurysms (91%). In 5/23 cases two interventions and in 1/23 cases three interventions were necessary to achieve secondary success. Embolization alone, as a therapy for aneurysms involving only the internal iliac artery, had a success rate of 27%. No procedure-related minor or major complications occurred. Mean decrease of aneurysm size during a mean observation period of 14.1 months was 6.9% which was not significant (p=0.3; 95% confidence interval +7-21%). CONCLUSION: Endovascular therapy of isolated iliac artery aneurysms performed percutaneously has become a treatment alternative to open surgical repair. This method is feasible and safe with low procedure-related morbidity and mortality. However, on average more than one intervention has to be performed to achieve successful permanent exclusion of the aneurysm and embolization alone in isolated internal iliac artery aneurysms is not sufficient.

The role of fungal proteinases in pathophysiology of Stachybotrys chartarum.

The adverse health effects of Stachybotrys chartarum have often been linked to exposure to the trichothecene mycotoxins. Recent studies have shown that in addition to mycotoxins this fungus is capable of producing and secreting in vivo proteins such as hemolysins and proteinases. Spore extracts obtained from a high trichothecene producing isolate JS 58-17 exhibited a significantly lower proteolytic activity compared to the low trichothecene producer, JS 58-06. Growing isolates on rice or potato dextrose agar results in higher proteolytic activity of the spores compared to those grown on drywall. Proteinases in the spore extracts can hydrolyze gelatin and collagen I and IV. Analysis of zymograms shows the presence of several proteins with proteolytic activity in the spores of S. chartarum. Human tracheal epithelial cells exposed to spore extracts produced significantly higher levels of IL-6, IL-8, and TNF-alpha than control cells. This stimulation of cytokine production was completely abolished by Pefabloc, a serine protease inhibitor. Neutrophil numbers and proinflammatory cytokine (IL1-beta and TNF-alpha) concentrations were highly elevated in the lungs of 7 day old rat pups exposed intratracheally to 4 x 10(4) spores/gm body weight compared to control. No significant differences in those inflammatory indices in vivo were noted between the treatments with the high trichothecene producer, isolate JS 58-17 and JS 58-06, which does not produce macrocyclic trichothecenes. Immunohistochemistry revealed reduced collagen IV labeling in spore-induced lung granulomas in rat pups exposed to both isolates. These results suggest that proteinases from S. chartarum spores significantly contribute to lung inflammation and injury.

Leukocyte adhesion deficiency in a female patient without delayed umbilical cord separation.

Leukocyte adhesion deficiency type 1 (LAD-1), a rare disorder of neutrophil function, is classically characterised by delayed umbilical cord separation. We report a case of LAD-1 in a female infant whose umbilical cord separated within 2 weeks of birth and review the literature pertaining to the timing of umbilical cord separation.

CTA in the detection and quantification of vertebral artery pathologies: a correlation with color Doppler sonography.

INTRODUCTION: We evaluated the feasibility of multidetector CT angiography (MDCTA) in the examination of vertebral artery (VA) pathologies and correlated the results with those of color Doppler sonography (CDS). METHODS: In this retrospective cohort analysis, we identified 65 patients with suspected cerebrovascular disease, who underwent MDCTA and CDS of the supraaortic vessels within a maximum period of 1 month. We evaluated the feasibility and image quality of MDCTA in this indication, compared the value of reformatted images and axial source images in the grading of stenoses and correlated these results with those of CDS. RESULTS: The image quality of the MDCTA examination was classified as good in 64 patients (98.5%) and as moderate in 1 patient (1.5%). Axial source images and reformatted images agreed perfectly in terms of stenosis detection and grading as well as the detection of hypoplastic VAs (kappa = 1). The correlation between MDCTA and CDS was moderate (kappa = 0.56) in terms of stenosis detection and quantification and poor (kappa = 0.35) in terms of detection of hypoplasia of the VA. CONCLUSION: MDCTA is a feasible method for the evaluation of VA pathologies providing a good image quality. Image reformatting does not add any diagnostic value to the interpretation of axial source images. The correlation between MDCTA and CDS is only moderate, reflecting the clinically important limitations of CDS in this indication.

Endovascular repair of acute type B aortic dissection: long-term follow-up of true and false lumen diameter changes.

BACKGROUND: The aim of this study was to determine the technical success after endovascular treatment of acute type B aortic dissections and to evaluate true and false lumen diameter changes at long-term follow-up. METHODS: Twenty-eight patients with acute type B-dissection who were treated by stent graft repair presented with rupture (n = 1), contained rupture (n = 2), compromised branch vessels (n = 14), pleural effusion (n = 11), rapid aortic diameter progression (n = 5), persistent pain (n = 3), refractory hypertension (n = 10), and an aortic diameter of more than 4 cm (n = 4). Taking into account the perfusion status of the false lumen, diameter changes were monitored in the thoracic aorta at the level of the stented segment (L1), distal to the stent graft (L2), and at the level of the celiac trunk (L3). RESULTS: Severe complications in 9 patients (32%) resulted in 3 deaths for a 30-day mortality rate of 10.7%. Primary sealing of the entry tear was achieved in 86%. At all levels, the true lumen diameter increased significantly after stent graft placement. At the 1-year follow-up, the false lumen in L1 was thrombosed in 90% and the mean difference of diameter reduction was highly significant. In L2, complete false lumen thrombosis occurred in 60% with a significant diameter decrease. In L3, the false lumen thrombosed in only 22%, and the mean difference of false lumen diameter increase reached significance at the 2-year follow-up. CONCLUSIONS: Ninety percent of patients were treated successfully with thrombosis of the false lumen in the stented segment. False lumen perfusion distal to the stent graft resulted in diameter increase in several patients leaving these segments an area of concern.