ABSTRACTPathogenic sequence variants in the nuclear bile acid receptor FXR, encoded by NR1H4, have been reported in a small number of children with low-γ-glutamyl transferase (GGT) cholestasis ...progressing to liver failure. We describe 3 additional children from 2 unrelated families with cholestasis and liver failure because of pathologic variants in NR1H4. One patient underwent liver transplantation and has had good clinical outcomes in 6 years of follow-up. Although that patient has biochemical evidence of increased bile acid synthetic activity, he has not experienced post-transplant diarrhea or allograft steatosis, as has been reported among other transplanted patients.
Pathogenic sequence variants in the nuclear bile acid receptor FXR, encoded by NR1H4, have been reported in a small number of children with low-γ-glutamyl transferase (GGT) cholestasis progressing to ...liver failure. We describe 3 additional children from 2 unrelated families with cholestasis and liver failure because of pathologic variants in NR1H4. One patient underwent liver transplantation and has had good clinical outcomes in 6 years of follow-up. Although that patient has biochemical evidence of increased bile acid synthetic activity, he has not experienced post-transplant diarrhea or allograft steatosis, as has been reported among other transplanted patients.
Stick balancing at the fingertip is a powerful paradigm for the study of the control of human balance. Here we show that the mean stick balancing time is increased by about two-fold when a subject ...stands on a vibrating platform that produces vertical vibrations at the fingertip (0.001 m, 15-50 Hz). High speed motion capture measurements in three dimensions demonstrate that vibration does not shorten the neural latency for stick balancing or change the distribution of the changes in speed made by the fingertip during stick balancing, but does decrease the amplitude of the fluctuations in the relative positions of the fingertip and the tip of the stick in the horizontal plane, A(x,y). The findings are interpreted in terms of a time-delayed "drift and act" control mechanism in which controlling movements are made only when controlled variables exceed a threshold, i.e. the stick survival time measures the time to cross a threshold. The amplitude of the oscillations produced by this mechanism can be decreased by parametric excitation. It is shown that a plot of the logarithm of the vibration-induced increase in stick balancing skill, a measure of the mean first passage time, versus the standard deviation of the A(x,y) fluctuations, a measure of the distance to the threshold, is linear as expected for the times to cross a threshold in a stochastic dynamical system. These observations suggest that the balanced state represents a complex time-dependent state which is situated in a basin of attraction that is of the same order of size. The fact that vibration amplitude can benefit balance control raises the possibility of minimizing risk of falling through appropriate changes in the design of footwear and roughness of the walking surfaces.
Chelonid alphaherpesviruses 5 and 6 (ChHV5 and ChHV6) are viruses that affect wild sea turtle populations. ChHV5 is associated with the neoplastic disease fibropapillomatosis (FP), which affects ...green turtles (
) in panzootic proportions. ChHV6 infection is associated with lung-eye-trachea disease (LETD), which has only been observed in maricultured sea turtles, although antibodies to ChHV6 have been detected in free-ranging turtles. To better understand herpesvirus prevalence and host immunity in various green turtle foraging aggregations in Florida, USA, our objectives were to compare measures of innate and adaptive immune function in relation to (1) FP tumor presence and severity, and (2) ChHV5 and ChHV6 infection status. Free-ranging, juvenile green turtles (
= 45) were captured and examined for external FP tumors in Florida's Big Bend, Indian River Lagoon, and Lake Worth Lagoon. Blood samples were collected upon capture and analyzed for ChHV5 and ChHV6 DNA, antibodies to ChHV5 and ChHV6, in vitro lymphocyte proliferation using a T-cell mitogen (concanavalin A), and natural killer cell activity. Despite an overall high FP prevalence (56%), ChHV5 DNA was only observed in one individual, whereas 20% of turtles tested positive for antibodies to ChHV5. ChHV6 DNA was not observed in any animals and only one turtle tested positive for ChHV6 antibodies. T-cell proliferation was not significantly related to FP presence, tumor burden, or ChHV5 seroprevalence; however, lymphocyte proliferation in response to concanavalin A was decreased in turtles with severe FP (
= 3). Lastly, green turtles with FP (
= 9) had significantly lower natural killer cell activity compared to FP-free turtles (
= 5). These results increase our understanding of immune system effects related to FP and provide evidence that immunosuppression occurs after the onset of FP disease.
Introduction
The purpose of this study is to (1) describe a pre-operative planning technique using non-reformatted CT images for insertion of multiple transiliac–transsacral (TI–TS) screws at a ...single sacral level, (2) define the parameters of a sacral osseous fixation pathway (OFP) that will allow for insertion of two TI–TS screws at a single level, and (3) identify the incidence of sacral OFPs large enough for dual-screw insertion in a representative patient population.
Methods
Retrospective review at a level-1 academic trauma center of a cohort of patients with unstable pelvic injuries treated with two TI–TS screws in the same sacral OFP, and a control cohort of patients without pelvic injuries who had CT scans for other reasons.
Results
Thirty-nine patients had two TI–TS screws at S1. Eleven patients, all with dysmorphic osteology, had two TI–TS screws at S2. The average pathway size in the sagittal plane at the level the screws were placed was 17.2 mm in S1 vs 14.4 mm in S2 (
p =
0.02). Twenty-one patients (42%) had screws that were intraosseous and 29 (58%) had part of a screw that was juxtaforaminal. No screws were extraosseous. The average OFP size of intraosseous screws was 18.1 mm vs. 15.5 mm for juxtaforaminal screws (
p =
0.02). Fourteen millimeters was used as a guide for the lower limit of the OFP for safe dual-screw fixation. Overall, 30% of S1 or S2 pathways were ≥ 14 mm in the control group, with 58% of control patients having at least one of the S1 or S2 pathways ≥ 14 mm.
Conclusions
OFPs ≥ 7.5 mm in the axial plane and 14 mm in the sagittal plane on non-reformatted CT images are large enough for dual-screw fixation at a single sacral level. Overall, 30% of S1 and S2 pathways were ≥ 14 mm and 58% of control patients had an available OFP in at least one sacral level.
Background
The number of patients requiring home enteral nutrition (HEN) continues to increase. Many of these patients are interested in using blended food instead of, or in addition to, commercial ...enteral formula (CEF). Increased risk of food‐borne illness is a concern of blenderized tube‐feeding (BTF). This project assessed a standard procedure for minimizing bacterial growth of BTF prepared in the home setting.
Methods
Fifty participants prepared BTF in their kitchens using a standard preparation procedure to minimize bacterial contamination. BTF was assessed for growth of aerobic microorganisms, Escherichia coli, Staphylococcus aureus, and coliforms at baseline, 24‐hour, and 48‐hour intervals after preparation for a total of 150 colony forming units (CFU) counts performed.
Results
No sample had zero aerobic microbial counts; yet no substantial increase in microbial counts was observed during the 48 hours. At baseline and 24 hours, 5/50 (10%) had a CFU count of >104, and at 48 hours, 6/50 (12%) exceeded 104 CFUs. Out of 150 CFU counts, 2 (1.3%) were just over 105 CFU/mL. Samples exceeding 104 CFU/mL were likely contaminated by common endospore‐forming bacteria found in soil or by bacteria in milk that was close to its expiration date.
Conclusion
In this study, 88% of the samples met the US Food Code criteria for safe food consumption; 10.7% met guidelines for marginal safety by other standards; and 1.3% slightly exceeded 105 CFUs. Established safe food‐handling procedures can minimize bacterial contamination of BTF and consequently reduce risk of food‐borne infection in HEN patients.
Abstract
Heifer pregnancy (HPG) and Stayability (STAY) are female reproductive traits that have EPD reported by the Red Angus Association of America. Challenges arise when making genetic predictions ...for these traits. Specifically, HPG and STAY phenotypes can only be collected on females retained in the breeding herd and have low heritability estimates. Additionally, STAY is measured late in an animal’s life. The objective of this research was to investigate the genetic relationships between HPG or STAY and 13 other traits, which included measurements of growth, carcass, ultrasound, and scrotal circumference. For STAY relationships between mature weight (MW), body condition score (BCS), teat score (TS), and udder suspension score (US) were also evaluated. Data from 142,146 and 164,235 animals were used in the analyses for HPG and STAY, respectively. Genetic relationships were investigated using a series of 2 trait animal models and a REML procedure. In all analyses, the appropriate contemporary groups were included as a fixed effect, and direct genetic as a random effect. Additional fixed effects included as follows: sex for weight, carcass and ultrasound traits, age of dam for weight traits, and age of measurement for ultrasound, carcass, BCS, udder traits, and MW. Maternal genetic effects for preweaning gain (Pre-WG), weaning weight (WW), and yearling weight (YW) were also modeled. Permanent environmental effects of the dam were modeled for the traits Pre-WG and WW. Permanent environment of the individual for the traits MW, BCS, TS, and US was also included. Heritability estimates were 0.12 ± 0.01 and 0.10 ± 0.01 for HPG and STAY, respectively. Heritability estimates for direct genetic effects of production traits were moderate to high in magnitude, maternal heritability estimates were low, and permanent environmental effects accounted for 0.00 to 0.18 of the total variation. The strongest genetic correlations were those among Pre-WGD (0.24 ± 0.08), WWD (0.18 ± 0.08), YWD (0.20 ± 0.07), ultrasound rib eye area direct (0.16 ± 0.08), and ultrasound backfat direct (0.14 ± 0.08) and HPG. The highest genetic correlations were between STAY and WWM (0.54 ± 0.05), YWM (0.36 ± 0.07), backfat (0.53 ± 0.20), marbling score (0.40 ± 0.20), UREA (0.19 ± 0.07), ultrasound backfat (0.37 ± 0.07), TS (0.30 ± 0.11), and US (0.23 ± 0.11). Relationships between HPG or STAY and other traits were minimal. These results suggest that genetic relationships exist between HPG or STAY and other more densely recorded traits.
To identify molecular alterations in skeletal muscle in rheumatoid arthritis (RA) that may contribute to ongoing disability in RA.
Persons with seropositive or erosive RA (n = 51) and control ...subjects matched for age, gender, race, body mass index (BMI), and physical activity (n = 51) underwent assessment of disease activity, disability, pain, physical activity and thigh muscle biopsies. Muscle tissue was used for measurement of pro-inflammatory markers, transcriptomics, and comprehensive profiling of metabolic intermediates. Groups were compared using mixed models. Bivariate associations were assessed with Spearman correlation.
Compared to controls, patients with RA had 75% greater muscle concentrations of IL-6 protein (p = 0.006). In patients with RA, muscle concentrations of inflammatory markers were positively associated (p < 0.05 for all) with disease activity (IL-1β, IL-8), disability (IL-1β, IL-6), pain (IL-1β, TNF-α, toll-like receptor (TLR)-4), and physical inactivity (IL-1β, IL-6). Muscle cytokines were not related to corresponding systemic cytokines. Prominent among the gene sets differentially expressed in muscles in RA versus controls were those involved in skeletal muscle repair processes and glycolytic metabolism. Metabolic profiling revealed 46% higher concentrations of pyruvate in muscle in RA (p < 0.05), and strong positive correlation between levels of amino acids involved in fibrosis (arginine, ornithine, proline, and glycine) and disability (p < 0.05).
RA is accompanied by broad-ranging molecular alterations in skeletal muscle. Analysis of inflammatory markers, gene expression, and metabolic intermediates linked disease-related disruptions in muscle inflammatory signaling, remodeling, and metabolic programming to physical inactivity and disability. Thus, skeletal muscle dysfunction might contribute to a viscous cycle of RA disease activity, physical inactivity, and disability.
Background: It has been postulated that exposure to bacterial endotoxins and animals early in life might confer protection against the development of asthma and allergies. Objective: We investigated ...the longitudinal effects of exposure to house dust endotoxin (HDE), allergen levels, and the presence of a dog in the home on wheezing in young children over a 4-year period. Methods: Two hundred twenty-six children younger than 5 years were followed for 4 years. Endotoxin and allergen levels were measured from house dust collected at baseline. Longitudinal associations were investigated by using a proportional hazards technique that allowed for multiple outcomes per subject. Results: Exposure to high concentrations of HDE of greater than the median level was associated with an increased risk for wheezing over the period of observation (multivariate relative risk, 1.52; 95 % CI, 1.07-2.14), but this risk rapidly decreased over time (P for trend = .005). Exposure to cockroach allergen was associated with increased risk for wheezing, whereas exposure to cat allergen and the presence of a dog in the home were both associated with decreased risk for wheezing. The risks associated with cockroach allergen, cat allergen, and dog did not change over the period of observation. Conclusion: The negative associations between exposures to dogs and cat allergen and wheeze appear to be independent of the effects of endotoxin and suggest that separate mechanisms might mediate the effects of HDE exposure and pet exposure on the developing immune system. (J Allergy Clin Immunol 2002;110:736-42.)
Community-based participatory research (CBPR) is defined by the Kellogg Community Health Scholars Program as a collaborative process that equitably involves all partners in the research process and ...recognizes the unique strengths that each community member brings. The CBPR process begins with a research topic of importance to the community, with the goal of combining knowledge and action with social change to improve community health and eliminate health disparities. CBPR engages and empowers affected communities to collaborate in defining the research question; sharing the study design process; collecting, analyzing, and disseminating the data; and implementing solutions. A CBPR approach in radiology has several potential applications, including removing limitations to high-quality imaging, improving secondary prevention, identifying barriers to technology access, and increasing diversity in the research participation for clinical trials. The authors provide an overview with the definitions of CBPR, explain how to conduct CBPR, and illustrate its applications in radiology. Finally, the challenges of CBPR and useful resources are discussed in detail.
RSNA, 2023 Quiz questions for this article are available in the supplemental material.
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