Children with congenital heart defects (CHD) are often short/lightweight relative to peers. Limited growth, particularly height, may reflect energy deficits impacting physical activity. Latent class analyses of growth from birth and Bruce treadmill exercise data retrospectively identified for height, weight, and body mass index z-scores growth trajectories. Linear regression models examined exercise parameters by growth trajectory, adjusting for age/sex/CHD severity. A total of 213 children with CHD (39% female, 12.1 ± 2.9 years) achieved 85.8 ± 10.1% of the predicted peak heart rate. Peak heart rate among children whose height was consistently below average (class 1) was 15.2 ± 4.9 beats/min lower than children with other height trajectories. These children also attained a lower percentage of predicted peak heart rate. Children whose weight (p = 0.03) or body mass index (p < 0.001) z-score increased throughout childhood had significantly lower exercise duration (mean difference 1–2 min) than children whose growth trajectories were stable or declined. Children with above-average weight or an increasing body mass index also used a higher percentage of their heart rate reserve at each submaximal exercise stage. A very low height z-score trajectory is associated with decreased exercise capacity that may increase the risk for morbidities associated with a sedentary lifestyle. Future studies should examine potential mechanisms for the observed height deficits, such as an inadequate energy supply that could impact physical activity participation, congestive heart failure, cyanosis, pubertal stage, supplemental feeding history, or familial growth patterns. Prospective studies examining growth in relation to objective measures of daily physical activity are required.

In an Indian oncology setting, between August and December 2021, 56 patients, developed Burkholderia cenocepacia bacteremia. An investigation revealed a contaminated batch of the antiemetic drug palonosetron. The outbreak was terminated by withdrawing the culprit batch and the findings were reported promptly to regulatory authorities.

Background: The advent of real-time quaking-induced conversion (RT-QuIC) assays has transformed the diagnostic approach to sporadic Creutzfeldt-Jakob disease (CJD) facilitating earlier recognition of affected patients. Recognizing this, we evaluated the performance of clinical features and diagnostic tests for CJD in the modern era. Methods: Clinical data were extracted from the electronic medical records of 115 patients with probable or definite CJD assessed at Mayo Clinic from 2014-2021. Clinical features and diagnostic tests were evaluated at presentation, and associations with diagnosis and prognosis determined. Results: Mean age-at-symptom onset was 64.8±9.4 years; 68 patients were female (59%). The sensitivity of clinical markers (myoclonus) and tests historically considered in patients with suspected CJD was poor (stereotyped EEG abnormalities, 16%; CSF 14-3-3, 60%). Conversely, RT-QuIC (93%), t-tau >1149 pg/mL (88%), and characteristic signal abnormalities on MRI (77%) identified most patients. Multivariable linear regression confirmed shorter days-to-death in patients with myoclonus (125.9, CI95% 23.3-15.5, p=0.026), visual/cerebellar signs (180.19, CI95% 282.2-78.2, p<0.001), positive 14-3-3 (193, CI95% 304.9-82.9; p<0.001), and elevated t-tau (9.0, CI95% 1.0-18.0, for every 1000 pg/ml elevation; p=0.041). Conclusions: CSF RT-QuIC and elevated t-tau, and stereotyped MRI abnormalities were consistently detected in CJD patients. Myoclonus, EEG findings, and CSF protein 14-3-3 were less useful in the modern era.

The study of high-dimensional distributions is of interest in probability theory, statistics, and asymptotic convex geometry, where the object of interest is the uniform distribution on a convex set in high dimensions. The ℓp-spaces and norms are of particular interest in this setting. In this paper we establish a limit theorem for distributions on ℓp-spheres, conditioned on a rare event, in a high-dimensional geometric setting. As part of our proof, we establish a certain large deviation principle that is also relevant to the study of the tail behavior of random projections of ℓp-balls in a high-dimensional Euclidean space.

Bariosincosite is a new barium vanadium phosphate hydrate from the Spring Creek Mine, near Wilmington, South Australia. The new mineral occurs as irregular clusters of pale green, very thin platey crystals up to 250 µm across and 2 to 5 µm thick. The tetragonal crystals are tabular on {001} and the other form present is {100}. Associated with bariosincosite are quartz, cuprite, native copper, fluorapatite, whitlockite, baryte and springcreekite, BaV3 3+ (PO4)2(OH,H2O)6. Bariosincosite appears to have formed under supergene or low-temperature late-stage hydrothermal conditions. Electron microprobe analysis yielded: BaO 23.20; SrO 4.19; CaO 0.36; VO2 31.55; Fe2O3 0.20; Al2O3 0.50; P2O5 28.15; H2O 13.93 (calculated). These data give an empirical formula of (Ba0.77Sr0.20Ca0.03)∑1.00[(V0.96 4+ Al0.03Fe0.01 3+)∑1.00O(PO4)]2·4H2O, calculated on the basis of two P atoms. The simplified formula is Ba(V4+OPO4)2·4H2O. The mineral is transparent with a very pale green streak, a vitreous lustre and an estimated Mohs hardness of 3. The strongest lines in the X-ray powder pattern are [d obs (I obs) (hkl)] 6.414 (20) (110, 002); 5.748 (70) (111); 4.552 (30) (112, 200); 3.198 (20) (220, 004); 3.100 (100) (203, 221); 2.847 (40) (222, 114); 2.786 (80) (311); 2.368 (30) (313, 115); and 2.017 (100) (420, 332, 116). These data were indexed on a tetragonal cell, with a = 9.031(6), c = 12.755(8) Å and V = 1040(1) Å3; the space group is probably P4/n or P4/nmm. For Z = 4 and using the empirical formula, the calculated density is 3.306 gm/cm3. Bariosincosite is uniaxial negative with ω = 1.721(2) and ε = 1.715(2) (white light); pleochroism is weak from colourless (E) to pale green (O), absorption O > E. The mineral is named for the relationship to sincosite, Ca(V4+OPO4)2·4H2O.

Unlike planktonic systems, reaction rates in biofilms are often limited by mass transport, which controls the rate of supply of contaminants into the biofilm matrix. To help understand this phenomenon, we investigated the potential of magnetic resonance imaging (MRI) to spatially quantify copper transport and fate in biofilms. For this initial study we utilized an artificial biofilm composed of a 50:50 mix of bacteria and agar. MRI successfully mapped Cu2+ uptake into the artificial biofilm by mapping T 2 relaxation rates. A calibration protocol was used to convert T 2 values into actual copper concentrations. Immobilization rates in the artificial biofilm were slow compared to the rapid equilibration of planktonic systems. Even after 36 h, the copper front had migrated only 3 mm into the artificial biofilm and at this distance from the copper source, concentrations were very low. This slow equilibration is a result of (1) the time it takes copper to diffuse over such distances and (2) the adsorption of copper onto cell surfaces, which further impedes copper diffusion. The success of this trial run indicates MRI could be used to quantitatively map heavy metal transport and immobilization in natural biofilms.

Theoretical accounts placing episodic memory as central to constructive and communicative functions neglect the role of semantic memory. We argue that the decontextualized nature of semantic schemas largely supersedes the computational bottleneck and error-prone nature of episodic memory. Rather, neuroimaging and neuropsychological evidence of episodic-semantic interactions suggest that an integrative framework more accurately captures the mechanisms underpinning social communication.

Objectives: With comparable baseline performance on executive functions (EF) and memory between Alzheimer’s disease (AD) and behavioral-variant frontotemporal dementia (bvFTD), it is currently unclear if both diseases can be distinguished longitudinally on these measures reliably. Methods: A total of 111 participants (33 AD, 31 bvFTD, and 47 controls) were followed-up annually over a 4-year period and tested on measures of EF, memory, and orientation. Linear mixed-effect models were constructed using disease severity as a nuisance variable to examine profiles of neuropsychological performance decline. Results: At baseline, overlap in terms of cognitive impairment between bvFTD and AD on multiple EF, memory, and orientation measures was present. Longitudinally, only disinhibition (Hayling total errors) appeared sensitive to discriminating AD from bvFTD; however, only after the first annual follow-up. Subgroup analyses on smaller samples revealed comparable profiles on EF tasks at baseline and over time between bvFTD and AD who presented with impaired EF at presentation, and on memory and orientation tasks between AD and bvFTD who presented with severe amnesia. Conclusions: Our results replicate previous findings showing only moderate discriminability between AD and bvFTD at clinical presentation on EF and memory measures. More importantly, we also show that longitudinal trajectories strongly overlap for both dementias on these measures. Disinhibition emerged as the sole measure that in the long run was significantly more impaired in bvFTD. Future studies should use tests designed to target cortical regions that are specifically impaired in bvFTD, such as the ventromedial prefrontal cortex, to improve the accurate discrimination of these diseases. (JINS, 2017, 23, 34–43)