Dermato-Radiological Evaluation of Congenital Limb Overgrowth Vascular Syndromes.

International Society for the Study of Vascular Anomalies classification defines Congenital Limb Overgrowth Vascular Syndromes (CLOS) as a subset of vascular syndromes with other abnormalities that present with unilateral limb overgrowth. It includes Klippel-Trenaunay Syndrome, Parkes-Weber Syndrome, CLOVES (Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Spinal/Skeletal Anomalies/Scoliosis) Syndrome, Proteus Syndrome, PTEN Hamartomatous Syndrome, and Fibroadipose Vascular Anomaly. Due to their rare and complex nature, a multidisciplinary approach to diagnosis and treatment is required. A thorough clinical and radiological workup can go miles in reflecting on the patient's outcome. Here we report five cases of CLOS with their detailed dermato-radiological profiles.

Assessment of Lung Structure and Regional Function Using 0.55 T MRI in Patients With Lymphangioleiomyomatosis.

OBJECTIVES: Contemporary lower-field magnetic resonance imaging (MRI) may offer advantages for lung imaging by virtue of the improved field homogeneity. The aim of this study was to evaluate the utility of lower-field MRI for combined morphologic imaging and regional lung function assessment. We evaluate low-field MRI in patients with lymphangioleiomyomatosis (LAM), a rare lung disease associated with parenchymal cysts and respiratory failure. MATERIALS AND METHODS: We performed lung imaging on a prototype low-field (0.55 T) MRI system in 65 patients with LAM. T2-weighted imaging was used for assessment of lung morphology and to derive cyst scores, the percent of lung parenchyma occupied by cysts. Regional lung function was assessed using oxygen-enhanced MRI with breath-held ultrashort echo time imaging and inhaled 100% oxygen as a T1-shortening MR contrast agent. Measurements of percent signal enhancement from oxygen inhalation and percentage of lung with low oxygen enhancement, indicating functional deficits, were correlated with global pulmonary function test measurements taken within 2 days. RESULTS: We were able to image cystic abnormalities using T2-weighted MRI in this patient population and calculate cyst score with strong correlation to computed tomography measurements (R = 0.86, P < 0.0001). Oxygen-enhancement maps demonstrated regional deficits in lung function of patients with LAM. Heterogeneity of oxygen enhancement between cysts was observed within individual patients. The percent low-enhancement regions showed modest, but significant, correlation with FEV1 (R = -0.37, P = 0.007), FEV1/FVC (R = -0.33, P = 0.02), and cyst score (R = 0.40, P = 0.02). The measured arterial blood ΔT1 between normoxia and hyperoxia, used as a surrogate for dissolved oxygen in blood, correlated with DLCO (R = -0.28, P = 0.03). CONCLUSIONS: Using high-performance 0.55 T MRI, we were able to perform simultaneous imaging of pulmonary structure and regional function in patients with LAM.

Oxygen-enhanced functional lung imaging using a contemporary 0.55 T MRI system.

The purpose of this study was to evaluate oxygen-enhanced pulmonary imaging at 0.55 T with 3D stack-of-spirals ultrashort-TE (UTE) acquisition. Oxygen-enhanced pulmonary MRI offers the measurement of regional lung ventilation and perfusion using inhaled oxygen as a contrast agent. Low-field MRI systems equipped with contemporary hardware can provide high-quality structural lung imaging by virtue of the prolonged T2 *. Fortuitously, the T1 relaxivity of oxygen increases at lower field strengths, which is expected to improve the sensitivity of oxygen-enhanced lung MRI. We implemented a breath-held T1 -weighted 3D stack-of-spirals UTE acquisition with a 7 ms spiral-out readout. Measurement repeatability was assessed using five repetitions of oxygen-enhanced lung imaging in healthy volunteers (n = 7). The signal intensity at both normoxia and hyperoxia was strongly dependent on lung tissue density modulated by breath-hold volume during the five repetitions. A voxel-wise correction for lung tissue density improved the repeatability of percent signal enhancement maps (coefficient of variation = 34 ± 16%). Percent signal enhancement maps were compared in 15 healthy volunteers and 10 patients with lymphangioleiomyomatosis (LAM), a rare cystic disease known to reduce pulmonary function. We measured a mean percent signal enhancement of 9.0 ± 3.5% at 0.55 T in healthy volunteers, and reduced signal enhancement in patients with LAM (5.4 ± 4.8%, p = 0.02). The heterogeneity, estimated by the percent of lung volume exhibiting low enhancement, was significantly increased in patients with LAM compared with healthy volunteers (11.1 ± 6.0% versus 30.5 ± 13.1%, p = 0.01), illustrating the capability to measure regional functional deficits.

Opportunities in Interventional and Diagnostic Imaging by Using High-Performance Low-Field-Strength MRI.

Background Commercial low-field-strength MRI systems are generally not equipped with state-of-the-art MRI hardware, and are not suitable for demanding imaging techniques. An MRI system was developed that combines low field strength (0.55 T) with high-performance imaging technology. Purpose To evaluate applications of a high-performance low-field-strength MRI system, specifically MRI-guided cardiovascular catheterizations with metallic devices, diagnostic imaging in high-susceptibility regions, and efficient image acquisition strategies. Materials and Methods A commercial 1.5-T MRI system was modified to operate at 0.55 T while maintaining high-performance hardware, shielded gradients (45 mT/m; 200 T/m/sec), and advanced imaging methods. MRI was performed between January 2018 and April 2019. T1, T2, and T2* were measured at 0.55 T; relaxivity of exogenous contrast agents was measured; and clinical applications advantageous at low field were evaluated. Results There were 83 0.55-T MRI examinations performed in study participants (45 women; mean age, 34 years ± 13). On average, T1 was 32% shorter, T2 was 26% longer, and T2* was 40% longer at 0.55 T compared with 1.5 T. Nine metallic interventional devices were found to be intrinsically safe at 0.55 T (<1°C heating) and MRI-guided right heart catheterization was performed in seven study participants with commercial metallic guidewires. Compared with 1.5 T, reduced image distortion was shown in lungs, upper airway, cranial sinuses, and intestines because of improved field homogeneity. Oxygen inhalation generated lung signal enhancement of 19% ± 11 (standard deviation) at 0.55 T compared with 7.6% ± 6.3 at 1.5 T (P = .02; five participants) because of the increased T1 relaxivity of oxygen (4.7e-4 mmHg-1sec-1). Efficient spiral image acquisitions were amenable to low field strength and generated increased signal-to-noise ratio compared with Cartesian acquisitions (P < .02). Representative imaging of the brain, spine, abdomen, and heart generated good image quality with this system. Conclusion This initial study suggests that high-performance low-field-strength MRI offers advantages for MRI-guided catheterizations with metal devices, MRI in high-susceptibility regions, and efficient imaging. © RSNA, 2019 Online supplemental material is available for this article. See also the editorial by Grist in this issue.

Optimized reconstructions of compressively sampled two-dimensional infrared spectra.

Compressive sampling has the potential to dramatically accelerate the pace of data collection in two-dimensional infrared (2D IR) spectroscopy. We have previously introduced the Generic Iteratively Reweighted Annihilating Filter (GIRAF) reconstruction algorithm to solve the reconstruction in 2D IR compressive sampling. Here, we report a thorough assessment of this method and comparison to our earlier efforts using the Total Variation (TV) algorithm. We show that the GIRAF algorithm has some distinct advantages over TV. Although it is no better or worse in terms of ameliorating the impacts of compressive sampling on the measured 2D IR line shape, we find that the nature of those effects is different for GIRAF than they were for TV. In addition to assessing the impacts on the line shape of a single oscillator, we also test the ability of the algorithm to reconstruct spectra that have transitions from more than one oscillator, such as the coupled carbonyl oscillators in rhodium dicarbonyl. Finally, and perhaps most importantly, we show that the GIRAF algorithm has a distinct denoising effect on the signal-to-noise ratio (SNR) of the 2D IR spectra that can increase the SNR by as much as 4× without any additional signal averaging and collecting fewer data points, which should further enhance the acceleration of data collection that can be achieved using compressive sampling and enable even more challenging experimental measurements.

DENOISING AND DEINTERLEAVING OF EPSI DATA USING STRUCTURED LOW-RANK MATRIX RECOVERY.

Echo-planar spectroscopic imaging (EPSI) sequence with spectrally interleaving is often used to rapidly collect metabolic MRI data. The main problem in using it on high field scanners is the presence of spurious peaks resulting from phase distortions between interleaves as well as the low signal to noise ratio. We introduce a novel structured low-rank framework for the simultaneous denoising and deinterleaving of spectrally interleaved EPSI data. The proposed algorithm exploits annihilation relations resulting from the linear predicability of exponential signals as well as due to uncorrected phase relations between interleaves. The algorithm is formulated as a structured nuclear norm minimization of a block Hankel matrix, derived from the interleaves. Experiments using hyperpolarized 13 C mouse kidney EPSI data demonstrate the ability of the algorithm to remove ghost peaks from EPSI data collected using bipolar readout gradients.

Accelerating two-dimensional infrared spectroscopy while preserving lineshapes using GIRAF.

We introduce a computationally efficient structured low-rank algorithm for the reconstruction of two-dimensional infrared (2D IR) spectroscopic data from few measurements. The signal is modeled as a combination of exponential lineshapes that are annihilated by appropriately chosen filters. The annihilation relations result in a low-rank constraint on a Toeplitz matrix constructed from signal samples, which is exploited to recover the unknown signal samples. Quantitative and qualitative studies on simulated and experimental data demonstrate that the algorithm outperforms the discrete compressed sensing algorithm, both in uniform and non-uniform sampling settings.

Compressively Sampled Two-Dimensional Infrared Spectroscopy That Preserves Line Shape Information.

Two-dimensional infrared (2D IR) spectroscopy is a powerful tool to investigate molecular structures and dynamics on femtosecond to picosecond time scales and is applied to diverse systems. Current technologies allow for the acquisition of a single 2D IR spectrum in a few tens of milliseconds using a pulse shaper and an array detector, but demanding applications require spectra for many waiting times and involve considerable signal averaging, resulting in data acquisition times that can be many days or weeks of laboratory measurement time. Using compressive sampling, we show that we can reduce the time for collection of a 2D IR data set in a particularly demanding application from 8 to 2 days, a factor of 4×, without changing the apparatus and while accurately reproducing the line-shape information that is most relevant to this application. This result is a potent example of the potential of compressive sampling to enable challenging new applications of 2D IR.

Compartmentalized low-rank recovery for high-resolution lipid unsuppressed MRSI.

PURPOSE: To introduce a novel algorithm for the recovery of high-resolution magnetic resonance spectroscopic imaging (MRSI) data with minimal lipid leakage artifacts, from dual-density spiral acquisition. METHODS: The reconstruction of MRSI data from dual-density spiral data is formulated as a compartmental low-rank recovery problem. The MRSI dataset is modeled as the sum of metabolite and lipid signals, each of which is support limited to the brain and extracranial regions, respectively, in addition to being orthogonal to each other. The reconstruction problem is formulated as an optimization problem, which is solved using iterative reweighted nuclear norm minimization. RESULTS: The comparisons of the scheme against dual-resolution reconstruction algorithm on numerical phantom and in vivo datasets demonstrate the ability of the scheme to provide higher spatial resolution and lower lipid leakage artifacts. The experiments demonstrate the ability of the scheme to recover the metabolite maps, from lipid unsuppressed datasets with echo time (TE) = 55 ms. CONCLUSION: The proposed reconstruction method and data acquisition strategy provide an efficient way to achieve high-resolution metabolite maps without lipid suppression. This algorithm would be beneficial for fast metabolic mapping and extension to multislice acquisitions. Magn Reson Med 78:1267-1280, 2017. © 2016 International Society for Magnetic Resonance in Medicine.

Tyrosine phosphorylation stimulates activity of human RAD51 recombinase through altered nucleoprotein filament dynamics.

The DNA strand exchange protein RAD51 facilitates the central step in homologous recombination, a process fundamentally important for accurate repair of damaged chromosomes, restart of collapsed replication forks, and telomere maintenance. The active form of RAD51 is a nucleoprotein filament that assembles on single-stranded DNA (ssDNA) at the sites of DNA damage. The c-Abl tyrosine kinase and its oncogenic counterpart BCR-ABL fusion kinase phosphorylate human RAD51 on tyrosine residues 54 and 315. We combined biochemical reconstitutions of the DNA strand exchange reactions with total internal reflection fluorescence microscopy to determine how the two phosphorylation events affect the biochemical activities of human RAD51 and properties of the RAD51 nucleoprotein filament. By mimicking RAD51 tyrosine phosphorylation with a nonnatural amino acid, p-carboxymethyl-l-phenylalanine (pCMF), we demonstrated that Y54 phosphorylation enhances the RAD51 recombinase activity by at least two different mechanisms, modifies the RAD51 nucleoprotein filament formation, and allows RAD51 to compete efficiently with ssDNA binding protein RPA. In contrast, Y315 phosphorylation has little effect on the RAD51 activities. Based on our work and previous cellular studies, we propose a mechanism underlying RAD51 activation by c-Abl/BCR-ABL kinases.

COMPARTMENTALIZED LOW-RANK REGULARIZATION WITH ORTHOGONALITY CONSTRAINTS FOR HIGH-RESOLUTION MRSI.

We introduce a novel compartmental low rank algorithm for high resolution MR spectroscopic imaging. We model the field inhomogeneity compensated MRSI dataset as the sum of a lipid dataset and a metabolite dataset using the spatial compartmental information obtained from water reference data. Both these datasets are modeled as low-rank subspaces, and are assumed to be orthogonal to each other. We formulate the recovery of the dataset from spiral measurements as a low-rank recovery problem. Experiments using numerical phantom and in-vivo data demonstrates the ability of the algorithm to provide improved spatial resolution and nuisance signal free spectra.