Transcription factor paralogs may share a common role in staged or overlapping expression in specific tissues, as in the Hox family. In other cases, family members have distinct roles in a range of embryologic, differentiation or response pathways (as in the Tbx and Pax families). For the interferon regulatory factor (IRF) family of transcription factors, mice deficient in Irf1, Irf2, Irf3, Irf4, Irf5, Irf7, Irf8 or Irf9 have defects in the immune response but show no embryologic abnormalities. Mice deficient for Irf6 have not been reported, but in humans, mutations in IRF6 cause two mendelian orofacial clefting syndromes, and genetic variation in IRF6 confers risk for isolated cleft lip and palate. Here we report that mice deficient for Irf6 have abnormal skin, limb and craniofacial development. Histological and gene expression analyses indicate that the primary defect is in keratinocyte differentiation and proliferation. This study describes a new role for an IRF family member in epidermal development.

No abstract

No abstract

To assess the sensitivity of detecting the most commonly encountered foreign bodies in Emergency Radiology using all imaging modalities (conventional radiography, computed tomography, ultrasound, and magnetic resonance imaging). The following materials were inserted into a pig-leg phantom and imaged using conventional radiography, computed tomography, ultrasound, and magnetic resonance imaging: Plastics #1, 2, 3, 5, and compostable plastic; dry and wet wood, aluminum, gravel, glass (tinted and non-tinted), and Salmon and Halibut fish bones. The visibility of plastic is variable on both conventional radiography and computed tomography, depending on composition, but all types of plastic are well visualized on ultrasound. Wood is most easily identified and localized on both computed tomography and ultrasound, is only faintly visible on conventional radiography, and is not well visualized on magnetic resonance imaging. Gravel, glass, and aluminum are well visualized on all modalities, with the exception of magnetic resonance imaging, where there is significant artifact surrounding the foreign body. Fish bones (Halibut and Salmon) are well visualized on conventional radiography, computed tomography, and ultrasound. Conventional radiography and computed tomography are great modalities for detecting foreign bodies of various compositions. Computed tomography is particularly useful at localizing the foreign body and determining its relationship to surrounding structures and its depth of involvement. All foreign bodies are visualized on ultrasound if the location is known and the foreign body is in the plane of the transducer. Magnetic resonance imaging is not helpful in detecting foreign bodies.

Vascular anomalies are a diverse group of pathologic conditions. They have different manifestations, natural histories, and treatments. Compared with other vascular malformations, arteriovenous malformations (AVMs) are considered the most symptomatic and difficult to manage. AVMs inherently progress and have a high rate of recurrence after treatment. Imaging helps provide an accurate and early diagnosis, which can then be used to direct appropriate management, with embolization evolving as the primary therapy. Thus, radiology plays a crucial role in the detection, workup, and management of AVMs. Ultrasonography (US) is a useful initial imaging modality, particularly when AVMs involve the extremities or a superficial or accessible location. Limitations include poor identification of soft-tissue and bone components, as well as suboptimal evaluation of deep or complex AVMs. Magnetic resonance (MR) angiography is the preferred imaging modality for AVMs and should be considered in any symptomatic patient or in the initial evaluation of vascular anomalies that are equivocal at US. Computed tomographic angiography should be reserved for those patients who are unable to undergo MR angiography or for evaluation of acute symptoms, such as bleeding or airway compromise. Conventional catheter-based angiography is useful for real-time depiction and evaluation of AVMs, particularly in the planning and execution of endovascular treatment and in the diagnosis of an AVM when findings from noninvasive imaging are equivocal for a high-flow component. As with the diagnostic workup, MR angiography is the preferred posttreatment modality. (©)RSNA, 2016.

Massive hemoptysis occurs in a minority of patients with cystic fibrosis, with an annual incidence of 1%. Although rare, massive hemoptysis can be a severe and potentially fatal complication of this disease. Beyond the acute life-threatening event, hemoptysis in patients with cystic fibrosis has been associated with faster decline in lung function, accelerated need for lung transplant, and increased mortality. The bronchial arteries are the culprit vessels in over 90% of cases of hemoptysis. This normally quiescent vascular system undergoes remarkable hypertrophy, collateralization, and angiogenesis before the onset of hemoptysis, introducing numerous pitfalls for the interventionalist. However, in experienced hands, bronchial artery embolization is a safe and potentially lifesaving therapy. Preprocedural noninvasive imaging, specifically computed tomographic angiography, has been repeatedly validated for helping to localize the likely site of bleeding, characterizing pertinent arterial anatomy, and promoting efficient and effective intervention; it has been recommended for all stable patients with hemoptysis. Success in the angiographic suite requires a thorough understanding of normal and variant bronchial arterial anatomy, appropriate patient selection, and a meticulous embolization technique. A meticulous approach to imaging and intervention, conscientious of both visualized and nonvisualized collateral pathways and nontarget vessels, can minimize potentially devastating complications. This review summarizes the current literature, modern procedural techniques, and emerging controversies, serving to guide an evolving approach to management of patients with cystic fibrosis and hemoptysis.RSNA, 2018.

This series suggests that TIPS can be performed in selective pregnant patients with portal hypertension, with little added risk to the mother or fetus.

BackgroundAngioembolization has been the gold standard for management of pelvic arterial bleeding, but applicability has been limited by delays in access at many trauma centers. We hypothesized that a quality improvement program to reduce time to start of angiography would be associated with lower in-hospital mortality in patients with pelvic fractures and shock.MethodsRetrospective study of adults with a pelvic fracture and vital signs consistent with shock admitted to a level I trauma center after the initiation of a quality improvement project to reduce the time to angioembolization (2012 to 2016). Time from admission to procedure start for hemorrhage control was examined based on destination and time of day. In-hospital mortality was the primary outcome and was compared with US benchmarks in the literature.ResultsThe study group included 424 patients with a mean Injury Severity Score of 41±14. Of these, 212 (50%) responded to resuscitation and were admitted to the intensive care unit; 143 (34%) patients went directly to interventional radiology (IR) with a median time to start of angiography of 86 minutes (IQR 66 to 116); and 69 (16%) patients went directly to the OR with a median time to start of operation of 52 minutes (IQR 37 to 73). There were no significant differences in time to procedures based on time of day or transfer status. In-hospital mortality for patients in shock on admission was 15%.DiscussionPatients with pelvic fracture and hemorrhagic shock, with a median time to angioembolization of <90 min, had a lower in-hospital mortality compared with published US benchmarks. These times were achieved by protocolization of pelvic fracture management that includes expeditious mobilization of the IR team, bypassing the CT scanner, and institutional quality metrics for compliance.Study typeCase series.Level of evidenceIV.