Nevin Manimala

J Pediatr Gastroenterol Nutr. 2021 May 13. doi: 10.1097/MPG.0000000000003153. Online ahead of print.

ABSTRACT

OBJECTIVES: To advance our understanding of monogenic forms of intrahepatic cholestasis.

METHODS: Analyses included participants with pathogenic biallelic mutations in ABCB11 (bile salt export pump; BSEP) or ATP8B1 (familial intrahepatic cholestasis; FIC1), or those with monoallelic or biallelic mutations in ABCB4 (multidrug resistance; MDR3), prospectively enrolled in the Longitudinal Study of Genetic Causes of Intrahepatic Cholestasis (LOGIC; NCT00571272) between 11/2007-12/2013. Summary statistics were calculated to describe baseline demographics, history, anthropometrics, laboratory values, and mutation data.

RESULTS: Ninety-eight participants with FIC1 (n = 26), BSEP (n = 53, including 8 with biallelic truncating mutations [severe] and 10 with p.E297G or p.D482G [mild]), or MDR3 (n = 19, including 4 monoallelic) deficiency were analyzed. Thirty-five had surgical interruption of the enterohepatic circulation (sEHC), including 10 who underwent liver transplant (LT) after sEHC. Onset of symptoms occurred by age 2 years in most with FIC1 and BSEP deficiency, but was later and more variable for MDR3. Pruritus was nearly universal in FIC1 and BSEP deficiency. In participants with native liver, failure to thrive was common in FIC1 deficiency, high ALT was common in BSEP deficiency, and thrombocytopenia was common in MDR3 deficiency. sEHC was successful after more than 1 year in 7 of 19 participants with FIC1 and BSEP deficiency. History of LT was most common in BSEP deficiency. Of 102 mutations identified, 43 were not previously reported.

CONCLUSIONS: In this cohort, BSEP deficiency appears to be correlated with a more severe disease course. Genotype-phenotype correlations in these diseases are not straightforward and will require study of larger cohorts.

An infographic is available for this article at: http://links.lww.com/MPG/C343.

PMID:34016879 | DOI:10.1097/MPG.0000000000003153

Prosthet Orthot Int. 2021 May 18. doi: 10.1097/PXR.0000000000000017. Online ahead of print.

ABSTRACT

BACKGROUND: The Scottish Specialist Prosthetics Service has provided microprocessor knees (MPKs) through the National Health Service since 2014, predominantly to high-activity transfemoral amputations (TFAs). Benefits of MPKs to the lower-activity TFA are less established in the literature.

OBJECTIVES: This study aimed to compare patient-reported and functional measures in low-activity TFAs transitioning from a mechanical knee to a MPK.

STUDY DESIGN: This is a retrospective cohort analysis of low-activity individuals with a unilateral TFA provided with an MPK.

METHODS: Patient-reported measures were recorded in routine clinical care before and 6 months after MPK provision. These included HR-QoL (EQ-5D-5L Health Index), Activities Balance Confidence score, Prosthetic Limb User Survey of Mobility, falls frequency, use of walking aids, and Socket Comfort Score. Functional measures included the two-minute walk test (2MWT), L-test, and Amputee Mobility Predictor score, and three-dimensional gait analysis was used to generate a Gait Profile Score (GPS). The primary outcomes were HR-QoL and GPS.

RESULTS: Forty-five participants fulfilled the inclusion criteria. Thirty-one had pre-MPK and post-MPK measures, of which 15 had three-dimensional gait analysis. The mean age (n = 31) was 60 years (SD 11), and 68% were male. HR-QoL and GPS did not significantly improve with MPK provision (p = 0.014 and p = 0.019); Amputee Mobility Predictor score, L-Test, 2MWT, falls, and Activities Balance Confidence score showed a significant improvement with MPK provision (p < 0.001).

CONCLUSIONS: Although no statistically significant change in the primary outcomes was measured, there were sufficient data to support MPK provision in low-activity prosthetic users with participants demonstrating improvements in balance, 2MWT, falls frequency, and confidence.

PMID:34016872 | DOI:10.1097/PXR.0000000000000017

Prosthet Orthot Int. 2021 May 18. doi: 10.1097/PXR.0000000000000016. Online ahead of print.

ABSTRACT

BACKGROUND: Microprocessor controlled knees (MPKs) have been shown to improve gait outcomes for individuals with unilateral transfemoral amputation (TFA); however, previous studies have limitations, which may affect their applicability.

OBJECTIVES: To compare gait outcomes between non-MPK and MPK conditions within a large and diverse cohort of established TFAs in the pragmatic context of an operational clinical service and to identify any demographic variables that correlate with changes in gait outcomes.

STUDY DESIGN: This is a retrospective cohort study.

METHODS: Full-body three-dimensional motion capture and a portable breath gas analyser were used to evaluate gait-specific outcomes both pre-MPK and 6 months post-MPK provisions in TFAs (n = 32). The primary outcome measure was gait profile score, along with the following secondary outcome measures: walking velocity, step length, step length symmetry ratio, step time symmetry ratio, vertical ground reaction force symmetry index, base of support, center of mass deviation, and oxygen cost of walking.

RESULTS: Data showed statistically significant improvement in gait profile score from 11.2° ± 2.2° to 10.1° ± 2.1° (p < 0.01) between non-MPK and MPK conditions. Other outcomes which showed significant improvement were walking velocity (p < 0.01), step length (p < 0.01), vertical ground reaction force symmetry index (p < 0.01), and center of mass deviation (p < 0.05).

CONCLUSIONS: MPK provision offered significant gait improvements to this cohort of TFAs in an operational clinical setting. There still remains a large gap in gait outcomes between this cohort and those of healthy unimpaired adults. Improvements in socket design, prosthetic foot design, and physiotherapy intervention could further reduce this gap.

PMID:34016870 | DOI:10.1097/PXR.0000000000000016

J Cardiovasc Nurs. 2021 May 19. doi: 10.1097/JCN.0000000000000821. Online ahead of print.

ABSTRACT

OBJECTIVES: Frailty is a syndrome characterized by increased vulnerability and reduced ability to maintain homeostasis after stressful events that results in an increased risk for poor outcomes. Frailty screening could potentially be valuable in cardiac surgery risk assessment. The purpose of this review is to evaluate the current literature linking multicomponent frailty assessment and invasive cardiac surgery outcomes.

METHODS: We searched PubMed, EMBASE, and CINAHL; 1887 articles met the search criteria, and each was independently reviewed by 2 reviewers.

RESULTS: The 19 eligible studies assessed 52 291 subjects using 17 different frailty measurements. The most commonly used instruments were the Fried Frailty Phenotype and the Clinical Frailty Scale. Between 9% and 61% of participants were found to be frail in each study. All 19 studies included mortality as an outcome, 12 included surgical complications, 12 included hospital length of stay, 3 included quality of life, and 2 included functional status. Nine found statistically significant differences in survival between frail and nonfrail patients, 6 of 12 found that frail patients had a longer length of stay, 4 of 12 found that frail patients were more likely to experience major complications, and 2 of 2 found that frail patients were more likely to have a decrease in functional status.

CONCLUSION: Although some studies lacked power, the majority confirmed that frail patients are more likely to experience poor outcomes. Further research is needed to determine which frailty measure provides the best predictive validity and to identify interventions to mitigate the risks that major cardiac surgery poses to frail patients.

PMID:34016838 | DOI:10.1097/JCN.0000000000000821

J Dev Behav Pediatr. 2021 May 19. doi: 10.1097/DBP.0000000000000964. Online ahead of print.

ABSTRACT

OBJECTIVE: To estimate the prevalence of screen time (ST) and its associated effects, including emotional and behavioral changes, sleep disturbances, and physical activity levels, in children aged 2 to 5 years.

METHODS: We conducted a cross-sectional study among 400 randomly selected children aged 2 to 5 years in Chandigarh, North India. We used a validated, pretested, semistructured digital-screen exposure and physical activity questionnaire; an abbreviated standard Child Sleep Habit Questionnaire; and a standard Preschool Child Behavior Checklist. We considered ST as excessive if it was more than 1 hour per day as per the American Academy of Pediatrics 2016 guidelines. IBM SPSS Statistics for Macintosh, version 25.0, was used to perform linear regression model analysis and stepwise binary multivariate logistic regression.

RESULTS: Approximately 59.5% of children (mean age 3.5 ± 0.9 years) had excessive ST. ST was higher on weekdays (58.5%) compared with the weekends (56.8%). Higher ST correlated positively with the mother’s level of education (r = +0.219) and higher per capita family income (r = +0.227). Screen time was also positively correlated with attending daycare centers (adjusted odd’s ratio [aOR] = 1.82, confidence interval [CI] = 1.18-2.81), caregivers ST of more than 2 hours (aOR = 1.68, CI = 1.12-2.56), and the absence of digital-media rules at home (aOR = 3.3, CI = 0.92-11.65). It was negatively correlated (i.e., protective) with lower mother’s education (i.e., up to middle school) (aOR = 0.62, CI = 0.41-0.96) and watching educational digital-media content (aOR = 0.53, CI = 0.33-0.87).

CONCLUSION: We observed a high prevalence of excessive ST among children aged 2 to 5 years. Screen time was significantly associated with being in daycare centers, higher caregivers’ ST, lower mothers’ educational status, and educational digital content.

PMID:34016828 | DOI:10.1097/DBP.0000000000000964

J Cataract Refract Surg. 2021 May 18. doi: 10.1097/j.jcrs.0000000000000702. Online ahead of print.

ABSTRACT

PURPOSE: To evaluate the accuracy of the Hill-RBF 3 formula, with and without direct measurements of total corneal power, using a heteroscedastic statistical method for analysis.

SETTING: Department of Ophthalmology, Shaare Zedek Medical Center, Jerusalem, Israel.

STUDY DESIGN: Retrospective, consecutive case series.

METHODS: Records of consecutive patients who underwent routine cataract surgery between February 2018 and June 2020 were retrospectively reviewed. The prediction accuracy of the Hill-RBF 3.0 formula was compared to the Barrett Universal II (BUII), Emmetropia Verifying Optical 2.0 (EVO), Haigis, HILL-RBF 2.0, Hoffer Q, Holladay 1, Holladay 2, Kane, Olsen, and SRK/T formulas, based on biometry measurements by swept-source optical coherence tomography (SS-OCT) with standard keratometry )K(, SS-OCT with total keratometry (TK), and an optical low-coherence reflectometer (OLCR). Statistical analysis was applied according to a heteroscedastic statistical method with a standard deviation (SD) of prediction errors as the main parameter for formula performance.

RESULTS: The study included 153 eyes of 153 patients. The SD values that were obtained by HILL-RBF 3.0 (0.266-0.285D) were significantly lower compared to Hill-RBF 2.0 (0.290-0.309D), Hoffer Q (0.387-0.407D), Holladay 1 (0.367-0.385D), Holladay 2 (0.386-0.401D), and SRK/T (0.377-0.399D) formulas, P < .036. The prediction accuracy of the Hill-RBF 3.0 was similar across the SS-OCT (K), SS-OCT (TK), and OLCR methods of measurement (P > .51).

CONCLUSIONS: HILL-RBF 3.0 was more accurate than Hill-RBF 2.0 and older generation formulas and had similar prediction accuracy compared to new generation formulas. The use of total keratometry did not provide significant improvement to its prediction accuracy.

PMID:34016821 | DOI:10.1097/j.jcrs.0000000000000702

Indian Pediatr. 2021 May 20:S097475591600330. Online ahead of print.

ABSTRACT

OBJECTIVE: To study the serum vitamin D levels in neonatal seizures and vitamin D status of the mothers whose babies had vitamin D deficiency.

METHODS: For this case-control study, vitamin D levels were studied in term and late preterm neonates admitted to NICU with seizures in a tertiary care center in South India. Controls were term and late preterm healthy neonates admitted in the postnatal ward with the mothers in the same center.

RESULT: 30 cases and 30 controls were enrolled. The mean (SD) serum vitamin D was 19.33 (7.76) ng/mL among cases and 16.83 (6.74) ng/mL among controls (P=0.18). We tested maternal vitamin D levels in babies with seizures and low vitamin D levels. The mean (SD) serum vitamin D level among these mothers (n=11) was 13.25 (6.17) ng/mL.

CONCLUSION: There was no statistically significant association between serum vitamin D levels and seizures among neonates in our study. However, we found low vitamin D levels in lactating mothers and newborn population of this region.

PMID:34016803

J Dr Nurs Pract. 2021 May 20:JDNP-D-20-00036. doi: 10.1891/JDNP-D-20-00036. Online ahead of print.

ABSTRACT

BACKGROUND: Quality improvement (QI) projects comprise the majority of University of Maryland School of Nursing (UMSON) Doctor of Nursing Practice (DNP) projects.

METHODS: An online survey was completed by 51% (n = 38) of faculty, who teach or mentor DNP students, and was analyzed using quantitative and descriptive methods.

RESULTS: Faculty were somewhat or not familiar with developing a QI charter 68.4%, human error theory and error proofing 63.2%, driver diagrams 60.5%, characteristics of high-reliability organizations 60.5%, and Standards for Quality Improvement Reporting Excellence (SQUIRE) guidelines 55.3%. The faculty were most interested in learning more about (n = 97 responses) were human error theory and error proofing (28.9%), SQUIRE guidelines (26.3%), statistical process control (21.1%), and implementation strategies and tactics (21.1%). The most commonly identified challenges included identifying QI projects (24%), project time constraints (16%), keeping up-to-date on QI concepts, methods, and tools (12%), and balancing professional workload (10%).

CONCLUSIONS: Gaps in self-reported QI knowledge indicate there is a need for further development of DNP and PhD prepared faculty at the UMSON.

PMID:34016785 | DOI:10.1891/JDNP-D-20-00036

Science. 2021 May 21;372(6544):860-864. doi: 10.1126/science.abg1685.

ABSTRACT

Global vegetation over the past 18,000 years has been transformed first by the climate changes that accompanied the last deglaciation and again by increasing human pressures; however, the magnitude and patterns of rates of vegetation change are poorly understood globally. Using a compilation of 1181 fossil pollen sequences and newly developed statistical methods, we detect a worldwide acceleration in the rates of vegetation compositional change beginning between 4.6 and 2.9 thousand years ago that is globally unprecedented over the past 18,000 years in both magnitude and extent. Late Holocene rates of change equal or exceed the deglacial rates for all continents, which suggests that the scale of human effects on terrestrial ecosystems exceeds even the climate-driven transformations of the last deglaciation. The acceleration of biodiversity change demonstrated in ecological datasets from the past century began millennia ago.

PMID:34016781 | DOI:10.1126/science.abg1685

J Nucl Med. 2021 May 20:jnumed.120.261545. doi: 10.2967/jnumed.120.261545. Online ahead of print.

ABSTRACT

Purpose: The assessment of gliomas by ¹⁸F-FDOPA PET imaging in adjunct to MRI showed high performance by combining static and dynamic features to non-invasively predict the isocitrate dehydrogenase (IDH) mutations and the 1p/19q co-deletion, which the World Health Organization classified as significant parameters in 2016. The current study evaluates whether other ¹⁸F-FDOPA PET radiomics features further improve performance and the contributions of each of these features to performance. Methods: Our study included seventy-two, retrospectively selected, newly diagnosed, glioma patients with ¹⁸F-FDOPA PET dynamic acquisitions. A set of 114 features, including conventional static features and dynamic features as well as other radiomics features were extracted and machine-learning models trained to predict IDH mutations and the 1p/19q co-deletion. Models were based on a machine-learning algorithm built from stable, relevant, and uncorrelated features selected by hierarchical clustering followed by a bootstrapped feature selection process. Models were assessed by comparing area under the curve (AUC) using a nested cross-validation approach. Feature importance was assessed using SHapley Additive exPlanations (SHAP) values. Results: The best models were able to predict IDH mutations (logistic regression with L2 regularization) and the 1p/19q co-deletion (support vector machine with radial basis function kernel) with an AUC of 0.831[0.790;0.873] and 0.724[0.669;0.782] respectively. For the prediction of IDH mutations, dynamic features were the most important features in the model (TTP: 35.5%). In contrast, other radiomics features were the most useful for predicting the 1p/19q co-deletion (up to 14.5% of importance for the small zone low grey level emphasis) . Conclusion: ¹⁸F-FDOPA PET is an effective tool for the non-invasive prediction of glioma molecular parameters using a full set of amino-acid PET radiomics features. The contribution of each feature set shows the importance of systematically integrating dynamic acquisition for the prediction of the IDH mutations as well as developing the use of radiomics features in routine practice for the prediction the 1p/19q co-deletion.

PMID:34016731 | DOI:10.2967/jnumed.120.261545