bluuebunny/crossref_metadata_2025 huggingface dataset:
Patient-Reported Outcome Measurement Information System (PROMIS) Scores in Pediatric Patients With Arthrogryposis
Authors: Hyer Lauren C. , Carson Lisa T. , Carpenter Ashley M. , Westberry David E.
Published: August 2021

Background:

Arthrogryposis multiplex congenita (AMC) is a clinical term that is used to describe congenital contractures that lead to childhood deformities. Treatment aims are to maximize function while minimizing pain and disability. Few studies have explored patient-reported outcomes in the pediatric arthrogrypotic population, particularly concerning mobility. The aim of this study was to report scores for the Patient-Reported Outcome Measurement Information System (PROMIS) questionnaire for pediatric patients with arthrogryposis with regards to mobility, upper extremity (UE) function, pain interference, and peer relationships.

Methods:

A retrospective chart review of 76 patients with AMC aged 5 to 17 who completed the PROMIS questionnaire between January 1, 2017 to March 24, 2020 was performed. Results were collected for four domains: mobility, UE function, pain interference, and peer relationships. Outcomes were stratified by type of arthrogryposis: Amyoplasia (Am), Distal Arthrogryposis (DA), and Other Diagnoses (OD). Results of subjects with isolated upper or lower extremity involvement were compared with subjects with involvement of upper and/or lower extremities. Outcomes were correlated with history of surgical intervention.

Results:

Children with Am and OD demonstrated moderate impairment of mobility (average: 35.2 and 35.9, respectively), while those with DA reported only mild impairment (average: 44.9). UE function was severely impaired for children with Am (average: 23.0), moderately impaired for OD (average: 33.0), and mildly impaired for DA (average: 43.4). All patient groups reported normal ranges of pain interference, as well as good peer relationships. A moderate negative correlation between number of surgical interventions and mobility scores, and a weak negative correlation between number of surgeries and pain interference scores were found.

Conclusions:

Children with AMC experience limited mobility and UE function, but normal levels of pain interference and good peer relationships. The average values provided in this study will serve as a baseline from which to evaluate the efficacy of both nonoperative and surgical interventions.

Level of Evidence:

Level II—prognostic study; retrospective study.
Intra-operative open-lung ventilatory strategy reduces postoperative complications after laparoscopic colorectal cancer resection
Authors: Li Hong , Zheng Zhi-Nan , Zhang Nan-Rong , Guo Jing , Wang Kai , Wang Wei , Li Lin-Gui , Jin Jing , Tang Jing , Liao Yao-Jun , Jin San-Qing
Published: August 2021

BACKGROUND

The role of the positive end-expiratory pressure (PEEP) and lung recruitment manoeuvre (LRM) combination (termed open-lung strategy, OLS) during intra-operative mechanical ventilation is not clear.

OBJECTIVE

To determine whether an open-lung strategy constituting medium PEEP (6–8 cmH2O) and repeated LRMs protects against postoperative complications in at-risk patients undergoing laparoscopic colorectal cancer resection under low-tidal-volume ventilation.

DESIGN

A prospective, assessor-blinded, randomised controlled trial.

SETTING

Single university-affiliated hospital, conducted from January 2017 to October 2018.

PATIENTS

A total of 280 patients at risk of pulmonary complications, scheduled for laparoscopic colorectal cancer resection under general anaesthesia and low-tidal-volume (6–8 ml kg−1 predicted body weight) ventilation.

INTERVENTION

The patients were randomly assigned (1 : 1) to a PEEP of 6–8 cmH2O with LRMs repeated every 30 min (OLS group) or a zero PEEP without LRMs (non-OLS group).

MAIN OUTCOME MEASURES

The primary outcome was a composite of major pulmonary and extrapulmonary complications occurring within 7 days after surgery. The secondary outcomes included intra-operative potentially harmful hypotension and the need for vasopressors.

RESULTS

A total of 130 patients from each group were included in the primary outcome analysis. Primary outcome events occurred in 24 patients (18.5%) in the OLS group and 43 patients (33.1%) in the non-OLS group [relative risk, 0.46; 95% confidence interval (CI), 0.26 to 0.82; P = 0.009). More patients in the OLS group developed potentially harmful hypotension (OLS vs. non-OLS, 15% vs. 4.3%; P = 0.004) and needed vasopressors (25% vs. 8.6%; P < 0.001).

CONCLUSION

Among at-risk patients undergoing laparoscopic colorectal cancer resection under low-tidal-volume ventilation, an open-lung strategy with a PEEP of 6–8 cmH2O and repeated LRMs reduced postoperative complications compared with a strategy using zero PEEP without LRMs. Of note, LRMs should be used with caution in patients with haemodynamic instability.

TRIAL REGISTRATION

Clinicaltrials.gov identifier: NCT03160144.
Pharmacogenetics of interaction between depot medroxyprogesterone acetate and efavirenz, rifampicin, and isoniazid during treatment of HIV and tuberculosis
Authors: Haas David W. , Mngqibisa Rosie , Francis Jose , McIlleron Helen , Robinson Jennifer A. , Kendall Michelle A. , Baker Paxton , Mawlana Sajeeda , Badal-Faesen Sharlaa , Angira Francis , Omoz-Oarhe Ayotunde , Samaneka Wadzanai P. , Denti Paolo , Cohn Susan E. ,   for the AIDS Clinical Trials Group A5338 Study Team
Published: August 2021

Objective

In AIDS Clinical Trials Group study A5338, concomitant rifampicin, isoniazid, and efavirenz was associated with more rapid plasma medroxyprogesterone acetate (MPA) clearance compared to historical controls without tuberculosis or HIV therapy. We characterized the pharmacogenetics of this interaction.

Methods

In A5338, women receiving efavirenz-based HIV therapy and rifampicin plus isoniazid for tuberculosis underwent pharmacokinetic evaluations over 12 weeks following a 150-mg intramuscular injection of depot MPA. Data were interpreted with nonlinear mixed-effects modelling. Associations between individual pharmacokinetic parameters and polymorphisms relevant to rifampicin, isoniazid, efavirenz, and MPA were assessed.

Results

Of 62 A5338 participants in four African countries, 44 were evaluable for pharmacokinetic associations, with 17 CYP2B6 normal, 21 intermediate, and 6 poor metabolizers, and 5 NAT2 rapid, 20 intermediate, and 19 slow acetylators. There were no associations between either CYP2B6 or NAT2 genotype and MPA C
min at week 12, apparent clearance, C
max, area under the concentration-time curve (AUC) or half-life, or unexplained interindividual variability in clearance, and uptake rate constant or mean transit time of the slow-release fraction (P > 0.05 for each). In exploratory analyses, none of 28 polymorphisms in 14 genes were consistently associated with MPA pharmacokinetic parameters, and none withstood correction for multiple testing.

Conclusions

Study A5338 suggested that more frequent depot MPA dosing may be appropriate for women receiving rifampicin, isoniazid, and efavirenz. The present results suggest that knowledge of CYP2B6 metabolizer or NAT2 acetylator status does not inform individualized DMPA dosing in this setting.
The functional role of inherited CDKN2A variants in childhood acute lymphoblastic leukemia
Authors: Li Chunjie , Zhao Xinying , He Yingyi , Li Ziping , Qian Jiabi , Zhang Li , Ye Qian , Qiu Fei , Lian Peng , Qian Maoxiang , Zhang Hui
Published: August 2021

Objective

Genetic alterations in CDKN2A tumor suppressor gene on chromosome 9p21 confer a predisposition to childhood acute lymphoblastic leukemia (ALL). Genome-wide association studies have identified missense variants in CDKN2A associated with the development of ALL. This study systematically evaluated the effects of CDKN2A coding variants on ALL risk.

Methods

We genotyped the CDKN2A coding region in 308 childhood ALL cases enrolled in CCCG-ALL-2015 clinical trials by Sanger Sequencing. Cell growth assay, cell cycle assay, MTT-based cell toxicity assay, and western blot were performed to assess the CDKN2A coding variants on ALL predisposition.

Results

We identified 10 novel exonic germline variants, including 6 missense mutations (p.A21V, p.G45A and p.V115L of p16INK4A; p.T31R, p.R90G, and p.R129L of p14ARF) and 1 nonsense mutation and 1 heterozygous termination codon mutation in exon 2 (p16INK4A p.S129X). Functional studies indicate that five novel variants resulted in reduced tumor suppressor activity of p16INK4A, and increased the susceptibility to the leukemic transformation of hematopoietic progenitor cells. Compared to other variants, p.H142R contributes higher sensitivity to CDK4/6 inhibitors.

Conclusion

These findings provide direct insight into the influence of inherited genetic variants at the CDKN2A coding region on the development of ALL and the precise clinical application of CDK4/6 inhibitors.
Fatty liver index and hypertension-mediated organ damage in never-treated hypertensive patients without diabetes mellitus
Authors: Siafi Eirini , Andrikou Ioannis , Konstantinidis Dimitris , Kakouri Niki , Iliakis Panagiotis , Koskinas John , Koullias Emmanouil , Thireos Eleftherios , Tousoulis Dimitris , Thomopoulos Costas , Tsioufis Costas
Published: August 2021

Background:

We investigated whether fatty liver index (FLI), a surrogate marker of nonalcoholic fatty liver disease (NAFLD), is associated with hypertension-mediated organ damage (HMOD) in never-treated hypertensive patients without diabetes mellitus.

Methods:

We performed both clinic and ambulatory blood pressure (BP) measurements, and calculated the FLI for all participants. A FLI of no less than 60 indicates a high-risk of underlying NAFLD, whereas a FLI of less than 60 indicates lower risk. We evaluated left ventricular mass (LVM) by echocardiography, arterial stiffness by carotid--femoral pulse wave velocity (PWV), capillary rarefaction by nailfold capillaroscopy, as well as urinary albumin-to-creatinine ratio (ACR). HMOD was defined according to the categorical thresholds for each domain, except for capillary rarefaction in which case the categorization of patients was made by the median.

Results:

We included 146 hypertensive patients (men, 43.8%; mean age, 56.6 ± 10.8 years; BMI, 30.3 ± 4.9 kg/m2; FLI, 57.2 ± 27.7; office, systolic/diastolic, and 24-h BP, 153.5 ± 15.8/94.7 ± 9.8 mmHg, and 140.5 ± 9.9/83.8 ± 9 mmHg, respectively). Patients with FLI at least 60 (n = 76) were younger, with higher BMI and 24-h SBP, compared with patients with FLI less than 60 (n = 70). FLI was associated with HMOD after adjustment (LVM indexed to height, P = 0.004; PWV, P = 0.047; reduced capillary density, P = 0.001; and logACR, P = 0.003). High-risk FLI phenotype and FLI z scores increased the likelihood of any HMOD by 3.8 (95% confidence interval, 1.6–7.1) and 5.4 (95% confidence interval, 2.3–15.0) times, respectively. However, the increased number of HMOD domains has progressively stopped being determined by the FLI z scores (P = 0.65).

Conclusion:

High-risk FLI pattern was associated with various HMOD, and may re-classify never-treated hypertensive patients without diabetes mellitus into a higher cardiovascular risk level.
“The Spinning Technique” for Unfolding Tightly Scrolled DMEK Grafts
Authors: Vasquez-Perez Alfonso , Phylactou Maria , Din Nizar , Liu Christopher
Published: August 2021

Purpose:

We present a no-touch Descemet membrane endothelial keratoplasty (DMEK) technique which assists in unfolding of tightly scrolled grafts from young donors.

Methods:

The technique was developed following a wet-laboratory experiment using human corneas created to observe the behavior of the graft during spinning within the artificial anterior chamber. This approach involves injecting short bursts of balanced salt solution to make the scroll spin continuously in a 360-degree manner. Spinning of the graft over the surface is accompanied by rotation on its horizontal axis resulting in opening in the correct orientation. Unfolding is then completed with standard bimanual tapping maneuvers. The spinning technique was then performed in 4 patients who all had tight DMEK scrolls from young donors.

Results:

The wet laboratory confirmed that directed jets of fluid to generate spinning and rotation open the graft in the correct orientation in most occasions. All 4 cases in this study received grafts from young donors (range 37–48 years old), and the technique achieved rapid unfolding with no complications. Two of our patients had glaucoma drainage devices, 1 had anterior chamber intraocular lens, and all other cases had clear corneas and good endothelial cell count after the 1-year follow-up.

Conclusions:

Our novel technique provides a safe and effective method to unscroll tight DMEK grafts that surgeons could incorporate into their armamentarium. Success is optimized by slightly undersizing the graft (7.5–8.0 mm).
One-Year Outcomes of Micropulse Cyclophototherapy for Primary Open-angle Glaucoma
Authors: Tong Weihan , Shen Tina Yu Ting , Wong Hung Chew , Aquino Maria Cecilia , Chew Paul , Lim Dawn
Published: August 2021

Precis:

In primary open-angle glaucoma (POAG), micropulse trans-scleral cyclophototherapy (MPTCP) is effective in lowering intraocular pressure (IOP), but its effects are not permanent. Hence, it can serve as a temporizing measure before definitive glaucoma surgery.

Purpose:

There is limited data on MPTCP in POAG. This is the first study that looks at MPTCP treatment specifically in POAG patients.

Patients and Methods:

This is an interventional, single-institution exploratory case series with 55 eyes of 48 patients with POAG. Data was collected from clinical records, including patient demographics, clinical information, number of glaucoma medications, MPTCP laser settings, complications, and clinical outcomes.

Results:

Patients had a mean age of 67.3±14.1 years with a preponderance of males. IOP was 24.8±1.0 mm Hg before MPTCP and decreased to 19.7±1.1, 21.9±1.1, and 21.8±1.1 mm Hg at postoperative month 3, 6, and 12 respectively. IOP remained below pretreatment levels throughout the postoperative period (P<0.05). Visual acuity and mean deviation remained stable before and after MPTCP. No eyes had complications. Number of glaucoma medications remained the same after MPTCP. Four eyes required additional oral acetazolamide at postoperative month 1 for IOP control. Seventeen eyes subsequently required further surgical intervention after 9.84 months. Maximal IOP decrease was greater when there were higher power settings, higher preoperative IOP, and better preoperative visual acuity.

Conclusions and Relevance:

The IOP lowering effect of MPTCP treatment in patients with POAG was found to be modest and transient with a similar medication burden, and definitive glaucoma surgery was needed in a number of patients.
Second Primary Neoplasms in Patients With Sebaceous Carcinoma of the Eyelid: A Population-based Study, 2000 to 2016
Authors: Tadrosse Abanoob F. , Tadrosse Marina F. , Ezzat Bishoy M. , Sadek Dina M. , Langer Paul D.
Published: August 2021

Purpose:

To investigate the risk of second primary neoplasms (SPNs) after primary sebaceous carcinoma of the eyelid (SCE).

Methods:

Data on patients diagnosed with primary SCE as their first malignancy were extracted from the Surveillance, Epidemiology, and End Results database from 2000 to 2016. Observed-to-expected ratios of SPNs were calculated to estimate standardized incidence ratios (SIRs). Patients were compared with a reference population (RP) matched for age, gender, and race.

Results:

Five hundred fifty-nine patients with primary SCE were identified, 16% of whom developed SPNs. SCE patients displayed a 61% increased risk of developing SPNs compared with the RP (p < 0.001). Overall, the risk of SPNs of the lungs (SIR = 1.82; p < 0.05), pancreas (SIR = 2.94; p < 0.05), salivary glands (SIR = 41.65; p < 0.001), and skin (SIR = 8.33; p < 0.05) was elevated. Only non-Hispanic Whites were at an increased risk (SIR = 1.51; p < 0.05). Patients 40–54 years old at the time of diagnosis were at the highest risk of developing SPNs compared with the RP (SIR = 3.15; p < 0.05). Women with SCE experienced an increased risk of breast cancer (SIR = 3.6; p < 0.05) and chronic lymphocytic leukemia (SIR = 8.8; p < 0.01).

Conclusion:

SCE patients are more likely to develop SPNs of the lungs, pancreas, salivary gland, and skin than the RP. Forty to fifty-four years old Caucasian patients are at the highest risk. Women are at an increased risk of developing breast malignancies and chronic lymphocytic leukemia. Clinicians should be cognizant of these risks when managing SCE patients.
Risk factors of recurrent carotid blowout syndrome and strategy of endovascular management
Authors: Luo Chao-Bao , Lee Chien-Hui , Chang Feng-Chi , Lin Chung-Jung
Published: August 2021

Background:

Carotid blowout syndrome (CBS) is a catastrophic complication after aggressive head and neck cancer treatment. Endovascular embolization is an effective modality to manage CBS. However, some CBS may have recurrent CBS (rCBS) after endovascular management. This study aims to report the potential rCBS risk and endovascular management strategy.

Methods:

Of the 225 patients with CBS referred for embolization in 13 years, 31 men and one woman (mean age, 55 years) with 35 rCBS with pseudoaneurysms formation were identified after endovascular management. Moreover, the rCBS preembolization angioarchitecture, rCBS cause, rCBS time interval, embolic materials selection, and final embolization clinical/angiographic outcomes were retrospectively analyzed.

Results:

rCBS with pseudoaneurysm due to disease progression (DP) occurred in 17 patients, while 15 patients had insufficient embolization (IE) with 18 rCBS. The mean rCBS timing interval was 76 days with 129 and 12 days due to DP or IE. The most common rCBS locations were the carotid bulb and the main trunk of the external carotid artery (n = 20, 57%), followed by internal carotid artery (n = 8, 23%), distal branch of the external carotid artery (n = 4, 11%), and common carotid artery (n = 3, 9%). Endovascular management was technically successful in all patients by reconstruction (n = 7, 20%) or destruction (n = 28, 80%) techniques. Three patients (9%) had procedure-related complications. No rCBS was observed in all affected arteries after the last embolization in a mean 11-month clinical follow-up.

Conclusion:

rCBS may result from DP or IE. The common location of IE-related rCBS usually occurred in the carotid branches. It occurred within two weeks of CBS largely because of the underestimation of the extension of the affected carotid artery. In addition, DP is natural in head and neck cancer after aggressive treatment. Thus, endovascular management remained an effective method to manage rCBS.
Reported Signs, Symptoms, and Diagnostic Tests Before Cardiotoxicity Among Women With Breast Cancer: A Pilot Study
Authors: Attin Mina , Reifenstein Karen , Mehta Sakshi , Arcoleo Kimberly , Lin C.D. , Storozynsky Eugene
Published: August 2021

Background

Cardiotoxicity after cancer treatment is a potentially preventable life-threatening complication among women with breast cancer. There is no algorithm to identify women with breast cancer at risk of cardiotoxicity.

Objectives

We quantified signs and symptoms as well as selected laboratory values among women with breast cancer who developed cardiotoxicity.

Methods

The clinical characteristics (n = 15) were collected from electronic health records. Spearman correlation coefficients and a nonparametric statistical test were used to analyze data.

Results

Significant statistical differences were detected in the laboratory values comparing the first and second half of 6 months before cardiotoxicity including alanine aminotransferase (U/L) (30.67 ± 26.27 and 42.31 ± 35.65, respectively; P = .03, Cohen’s d = 0.37). A negative correlation was found between estimated glomerular filtration rate and new onset of more than 1 sign or symptom (Spearman's ρ = −0.5, P = .06).

Conclusions

Investigating clinical characteristics before cardiotoxicity may determine the mechanism(s) and identify high-risk patients.
Effect of Miricorilant, a Selective Glucocorticoid Receptor Modulator, on Olanzapine-Associated Weight Gain in Healthy Subjects
Authors: Hunt Hazel J. , Donaldson Kirsteen , Strem Mark , Tudor Iulia Cristina , Sweet-Smith Suzanne , Sidhu Sharan
Published: August 2021

Purpose

Antipsychotic medications, including olanzapine, are associated with substantial weight gain and metabolic disturbances. We sought to determine whether coadministration of miricorilant, a selective glucocorticoid receptor modulator, with olanzapine can ameliorate these effects.

Methods

Sixty-six healthy men were enrolled in a 2-week, randomized, double-blind, placebo-controlled trial. The primary objective was to evaluate changes in body weight after 14 days coadministration of olanzapine (10 mg) + miricorilant (600 mg) compared with olanzapine (10 mg) + placebo. Secondary objectives included evaluating (a) the safety and tolerability of the combination; (b) the effects of the combination on glucose, insulin, insulin resistance, and triglycerides; and (c) the impact of the combination on hepatic enzymes.

Results

Subjects administered olanzapine + miricorilant gained less weight than subjects administered olanzapine + placebo (mean weight gain on day 15, 3.91 kg vs 4.98 kg; difference between groups, −1.07 kg; 95% confidence interval, −1.94 to −0.19; P = 0.017]). Compared with the placebo group, coadministration of miricorilant with olanzapine was associated with smaller increases in insulin (difference, −3.74 mIU/L; P = 0.007), homeostatic model assessment of insulin resistance (difference, −0.47; P = 0.007), triglycerides (difference, −0.29 mmol/L; P = 0.057), aspartate aminotransferase (difference, −32.24 IU/L; P = 0.009), and alanine aminotransferase (difference, −49.99 IU/L; P = 0.030).

Conclusions

Miricorilant may provide a promising option for ameliorating the detrimental effects of olanzapine, and investigation of this medication in patients affected by antipsychotic-induced weight gain is warranted. Two phase 2 studies of miricorilant in patients with recent and long-standing antipsychotic-induced weight gain are currently in progress.
Effectiveness of Clinical Simulation and Peer-Assisted Learning to Teaching About the Sexuality of People With Spinal Cord Injury
Authors: Hencklein Luana Cristina , da Silva Ruana Luiz Ferreira , Campos Daniel Gonçalves , Spagnol Gabriela Salim , Silva Juliany Lino Gomes , de Oliveira Sanfelice Clara Fróes , de Souza Oliveira-Kumakura Ana Railka
Published: August 2021

BACKGROUND: The sexuality of people with spinal cord injury (SCI) is a subject often neglected by healthcare professionals because of their lack of knowledge or understanding of how to proceed in this context. Different teaching strategies can be used to fill this knowledge gap, such as clinical simulation and peer-assisted learning. This study evaluates the effects of an educational intervention on nursing students' knowledge acquisition about the sexuality of individuals with SCI and on the students' self-confidence and satisfaction with the experience. METHODS: Quasi-experimental, pretest-posttest design was conducted with nursing students (N = 54). The intervention was the application by a senior-level nursing student of 2 scenarios of high-fidelity clinical simulation. The primary outcome was the knowledge about the sexuality of people with SCI. The secondary outcome was the satisfaction and self-confidence in learning. We performed descriptive and inferential statistics and built multiple linear regression models. RESULTS: The effect of the intervention on knowledge acquisition ranged from the mean of 1.26 in the pretest to 3.10 in the posttest. The means in the scores of satisfaction and self-confidence were considered excellent and correlated with knowledge acquisition. Previous attendance of a class on SCI positively interfered in the scale of knowledge acquisition. CONCLUSION: We verified an effect size ranging from medium to large magnitude for knowledge acquisition, and great satisfaction and self-confidence in learning through the use of simulated experience and peer-assisted learning.
Simultaneous Cochlear Implantation After Translabyrinthine Vestibular Schwannoma Resection: A Report of 41 Cases
Authors: Sanna Mario , Piccirillo Enrico , Kihlgren Caterina , Cagliero Gloria , Guidi Mariapaola , Saleh Essam
Published: August 2021

Objective:

To study the auditory outcome of simultaneous translabyrinthine vestibular schwannoma (VS) resection and cochlear implantation (CI) after successful cochlear nerve preservation.

Study Design:

A retrospective case series and patient questionnaire.

Setting:

Quaternary referral center for skull base pathologies.

Patients:

Patients with small (<2 cm) sporadic or neurofibromatosis 2 related tumors were included in this study.

Intervention:

Vestibular schwannoma resection + simultaneous cochlear implantation.

Main Outcome Measure:

Audiological performance postimplantation and perceived patients’ benefits.

Results:

Forty-one patients were included. Thirty-three were sporadic VS and eight were neurofibromatosis 2. Auditory perception postimplantation was achieved in 33 patients (80.5%). At the last follow-up, 20 patients (48.8%) were users and 21 (51.2%) were nonusers. In the users’ group, and after 1 year of implant activation, vowel identification was 75.3%, disyllabic word recognition 54%, sentence recognition 60.7%, and common phrase comprehension 61%, whereas in the nonusers’ group and after 1 year of implant activation, vowel identification was 22.9%, disyllabic word recognition 14.8%, sentence recognition 15.3%, and common phrase comprehension 14%. Sixteen users were classified into 10 high performers, three intermediate performers, and three poor performers. In the user’ group, the mean postimplantation pure tone average was 63.4 dB and the mean speech discrimination score was 63.7%.

Conclusions:

Simultaneous CI and VS resection is a viable option with many patients achieving auditory perception and nearly half the patients are CI users at long follow-up.
Neurodevelopmental outcomes in congenital heart disease through the lens of single ventricle patients
Authors: Burns Joseph , Varughese Robin , Ganigara Madhusudan , Kothare Sanjeev V. , McPhillips Lindsey A. , Dhar Arushi
Published: August 2021

Purpose of review

This review aims to summarize and organize the current body of literature on this contemporary topic, alongside a more general discussion of neurodevelopmental complications of congenital heart disease.

Recent findings

It is theorized that the causes of the neurodevelopment disabilities are multifactorial resulting from structural central nervous system abnormalities, haemodynamic alterations and/or biochemical changes. It is therefore imperative that all patients with single ventricle anatomy and physiology receive long-term neurologic and developmental assessments in addition to their cardiac monitoring.

Advancements in surgical techniques and medical management have improved survivorship of these medically complex patients. Neurodevelopmental sequelae are one of the most common comorbidities affecting this patient population leading to long-term challenges in motor, language, social and cognitive skills.
Osteoclast-Like Giant Cell Tumors of the Pancreas
Authors: Ueberroth Benjamin E. , Liu Alex J. , Graham Rondell P. , Bekaii-Saab Tanios S. , McWilliams Robert R. , Mahipal Amit , Truty Mark J. , Mody Kabir , Sonbol M. Bassam , Halfdanarson Thorvardur R.
Published: August 2021

Objectives

This study sought to better characterize patient characteristics, treatment options, and outcomes for osteoclast-like giant cell carcinoma of the pancreas, a rare subtype of pancreatic adenocarcinoma.

Methods

This is a retrospective study of all patients with osteoclast-like giant cell carcinoma of pancreatic origin treated at Mayo Clinic from 2000 to present. Baseline patient characteristics, treatment modalities utilized, and outcomes were compiled. Overall survival (OS) and progression-free survival were assessed using Kaplan-Meier analysis with a significance level of P ≤ 0.05.

Results

Fifteen patients met criteria for inclusion. Four patients had distant metastases at diagnosis, the remaining 11 with locoregional disease. Median OS for the entire cohort was 11 months. Metastatic disease was associated with significantly shorter OS (3.5 vs 14.1 months; P = 0.005). Three patients had no evidence of disease at time of analysis; all 3 were treated with complete resection followed by adjuvant chemotherapy.

Conclusions

Osteoclast-like giant cell carcinoma of the pancreas is an aggressive malignancy with poor prognosis. For patients with locoregional disease, surgical resection followed by adjuvant chemoradiation may play a role in extended disease-free survival. Metastatic disease presents a challenging entity to treat with little data to support any effective chemotherapy regimens.
Respiratory Nurses Have Positive Attitudes But Lack Confidence in Advance Care Planning for Chronic Obstructive Pulmonary Disease
Authors: Disler Rebecca , Cui Yuxiu , Luckett Tim , Donesky Doranne , Irving Louis , Currow David C. , Smallwood Natasha
Published: August 2021

Chronic obstructive pulmonary disease (COPD) is a progressive, life-limiting illness. Despite significant symptom burden, access to advance care planning (ACP) and palliative care are limited. Early initiation of ACP enables patients to articulate the values that underpin the decisions they would make if, in the future, they are unable to speak for themselves. Nurses constitute the majority of health care workforce and are well placed to initiate these discussions. This study explored knowledge, attitudes, and practice regarding ACP for patients with COPD among Australian and New Zealand respiratory nurses. A cross-sectional online survey tested knowledge about ACP and canvassed attitudes about current practice. Data were analyzed using descriptive statistics and content analysis of text data. The 112 participating respiratory nurses had substantial knowledge and positive attitudes regarding ACP in COPD; however, they lacked confidence and clarity regarding their role. Despite advances in end-of-life care provision for chronic disease, well-established barriers remained (inadequate training, fear of distressing patients, and time), and discussion triggers were still linked to acute deterioration, diagnosis of severe disease, and patient initiation. Better articulating the role of the respiratory nurse in ACP and building capacity and confidence within this workforce may improve ACP access for people living with COPD.
Palliative and End-of-Life Care Self-perceived Educational Needs of Physicians and Nurses in Rwanda
Authors: Moreland Patricia J. , Rosa William E. , Uwimana Philomene , Male Marcia A. , Sego Ruth , Nankundwa Evelyne , Byiringiro Samuel , Nsereko Etienne , Uwiringiyimana Emmanuel , Nyiringango Gerard , Baker Helen , Ntizimira Christian R.
Published: August 2021

Palliative care access is an urgent and ethical imperative to effectively manage the increasing burden of serious health-related suffering worldwide. Understanding the palliative care educational needs of health care professionals is critical to support evidence-based clinical practice in Rwanda and other low- and middle-income countries. A cross-sectional study was conducted at 5 hospitals in Kigali, Rwanda, to assess the palliative/end-of-life educational needs of nurses and physicians. The End-of-Life Professional Caregiver Survey and a demographic form were used to collect data. Descriptive analyses and independent t tests were conducted. A total of 420 health care professionals participated in the study (response rate, 72%). Participants perceived their knowledge and skills in providing palliative/end-of-life care as low. Those who received palliative/end-of-life care training in the past 5 years, third- and fourth-year residents, and health care providers with 5 years of experience or more had significantly higher (P ≤ .05) mean scores on the End-of-Life Professional Caregiver Survey. Nurses scored higher than physicians in the patient- and family-centered communication (P = .049) and cultural and ethical values (P = .037) subscales. Pain management was identified as an educational need by 77% of participants. Our survey suggests the need for increased governmental investments in palliative/end-of-life care education among nurses and physicians in Rwanda.
Clinical and Histopathologic Features Can Help Target Immunohistochemical Stain Use in the Diagnosis of Viral Esophagitis
Authors: Desai Nirali , Albahra Said , Lucas Elena , Singal Amit G. , Hammer Suntrea T.G. , Gopal Purva
Published: August 2021

Objectives:

Herpes simplex virus (HSV) and cytomegalovirus (CMV) immunohistochemical stains (IHC) are frequently applied on esophageal biopsies. Our aims were to identify IHC use patterns in viral esophagitis (VE), and clinicopathologic features of VE that could guide IHC use.

Methods:

We included 58 VE cases and 60 controls, defined as patients with negative HSV/CMV IHC between January 2006 and July 2017. Biopsies were reviewed and histologic features and clinical data recorded.

Results:

Thirteen cases required IHC for diagnosis. IHC was performed in 13 HSV and 5 CMV cases where diagnostic viral inclusions were present. VE patients were more likely to have endoscopic ulcer (P=0.002) and be immunocompromised (P<0.001). Pretest clinical concern for VE was common (P=0.006). Histologically, VE patients were more likely to have ulcer (P=0.004), ulcer exudate rich in neutrophils and histiocytes (P=0.001), neutrophils in squamous mucosa (P<0.001), histiocyte aggregates >15 (P<0.001) and spongiosis (P<0.001). Controls had frequent eosinophils, alone (P=0.008) or admixed with other inflammatory cells (P<0.0001).

Conclusions:

IHC is used in VE biopsies despite definite viral inclusions on hematoxylin and eosin and in patients without concerning histology or clinical concern for VE. History, endoscopic findings, and histology can be used to better target IHC use in VE.
Precision of Low-Cost Augmented Reality in Prefabricated Cutting Guide for Fibular Free Flap Surgery
Authors: Yodrabum Nutcha , Rudeejaroonrung Krit , Chaikangwan Irin , Prompattanapakdee Jiraya , Noraset Thanapon
Published: August 2021

Background:

Augmented reality (AR) is an imaging technology encompassing an interactive experience of a real-world environment enhanced by computer-generated perceptual information. It has been introduced to current medical practice to help the preoperative planning in many surgical fields.

Methods:

The authors applied AR to the computed tomography angiography of 8 patient's legs. Computed tomography angiography images were processed into Digital Imaging and communications in Medicine files to make a prefabricated cutting guide and customized titanium plate. Also, three-dimensional reconstruction of the arterial supply of the leg was performed to identify the perforators.

Results:

Followed by preoperative marking of operative details on patient's skins in antero-posterior view, lateral view, and combination of both views. Inaccuracy of measurement was confirmed by duplex ultrasound which average error of the combination of antero-posterior and lateral viewed of both legs was lowest (0.7 ± 0.2 cm). Followed by lateral view (1.0 ± 0.3 cm) and antero-posterior view (1.2 ± 0.4 cm), respectively.

Conclusions:

Augmented reality can improve patient's safety by directly locate the perforator and easily to design the skin paddle. Followed by satisfaction and confidence in patients and their relatives. Augmented reality also promoted understanding of operative steps for related assistants, residents, or fellows. Augmented reality can perform with existing equipment, mobile phone application, and can save the cost for preoperative planning. Distortion in the depth view can be more accurate by combining of AR in antero-posterior and lateral view.
Comparing fatal child abuse involving biological and surrogate parents
Authors: Presser Maxwell J. , Quiroz Hallie J. , Perez Eduardo A. , Sola Juan E. , Namias Nicholas , Thorson Chad M.
Published: August 2021

BACKGROUND

Nearly half of pediatric homicides younger than 5 years are attributable to child abuse. Parents are most commonly the perpetrators, but less is known about incidents involving biological versus surrogate parents. We sought to evaluate the characteristics of fatal child abuse involving biological and surrogate parents using the Georgia National Violent Death Reporting System, which we believe may differ in demographics and incident characteristics.

METHODS

This database was used to examine all homicides of children younger than 18 years from 2011 to 2017. Demographics and incident characteristics were analyzed using the existing National Violent Death Reporting System variables and incident narratives. Nonparametric and χ2 tests were used to compare fatal child abuse incidents involving biological and surrogate parents (e.g., adoptive, foster, step-parents, intimate partners of biological parent).

RESULTS

There were 452 pediatric homicides and 219 cases of fatal child abuse. Of all cases of fatal child abuse, 60% involved biological and 29% involved surrogate parents. Compared with children killed by biological parents, children killed by surrogate parents were older (4 vs. 3 years), more often male (71% vs. 51%), more likely to survive the initial injury and present to the emergency department before death (96% vs. 69%), and less likely to have a medical comorbidity (2% vs. 11%; all p < 0.05). Surrogate parents were more likely to be male (90% vs. 48%) and use a firearm (20% vs. 13%) to inflict the injury, both p < 0.05. The race/ethnicity of the child was not associated with the parent’s relationship.

CONCLUSION

Child abuse accounts for half of all pediatric homicides. Parents are the most common perpetrators of fatal child abuse, but surrogate parent perpetrators are almost exclusively male and more likely to use firearms. Most children have a history of abuse, leaving an opportunity to intervene on potentially preventable deaths if abuse is identified in a timely fashion.

LEVEL OF EVIDENCE

Prognostic and Epidemiologic, Level III
Emulating an RCT in observational research in chest trauma: The target trial approach
Authors: Cocco Annelise M. , Jennings Fiona L. , Lockwood David
Published: August 2021

BACKGROUND

While the concept of a “target trial”—optimizing the quality of observational studies by attempting to emulate the ideal world conditions of a randomized controlled trial—was first expounded over a decade ago, the take up of this concept in the design and analysis of trials in trauma is lacking. The target trial approach avoids common errors in observational research to increase its scientific validity as well as potentially enable causal questions to be answered without the expense and intricacies of a randomized controlled trial. This review article briefly introduces the reader to the concepts and utility of a “target trial” approach before providing demonstrations of its application in the subject area of chest trauma.

METHODS

Four articles published in the last 5 years—two case control and two cohort studies—are chosen and considered in terms of their causal question; study population; inclusion and exclusion criteria; designation of time 0; clarity of the follow-up period; study outcomes; methods to minimize confounding; results; overall issues regarding study time; and the presence of avoidable errors such as introduction of immortal time bias or information bias.

RESULTS

Two of the studies had an unclear causal question; none of the studies designated a time 0; the follow-up period was unclear for all but one of the studies; and one study had a serious issue with information bias resulting from differential misclassification.

CONCLUSION

Failure to emulate a “target trial” framework may lead to serious methodologic issues in observational research. Expansion of the awareness of this approach in trauma literature will improve the quality of our observational research and potentially translate into significant benefits for our patients.
Injection Drug use Practices and HIV infection among People Who Inject Drugs in Kigali, Rwanda
Authors: Twahirwa Rwema Jean Olivier , Nizeyimana Vianney , Menezes Neia Prata , Okonkwo Nneoma E. , Mazzei Amelia Aibina , Muhirwa Sulemani , Rukundo Athanase , Lucas Lisa , Niyigena Audace , Makuza Jean Damascene , Beyrer Chris , Baral Stefan D. , Kagaba Aflodis
Published: August 2021

Background

In Rwanda, epidemiological data characterizing people who inject drugs (PWID) and their burden of HIV are limited. We examined injecting drug use (IDU) history, practices, and HIV infection in a sample of PWID in Kigali.

Methods

From October 2019–February 2020, 322 PWID aged ≥18 were enrolled in a cross-sectional study using convenience sampling in Kigali. Participants underwent a structured interview and HIV testing. We used Poisson regression with robust variance estimation to assess IDU practices associated with HIV infection.

Results

The median age was 28 years(IQR:24-31) and 81%(248) were male. The median age at first injection was 23 years (IQR:20-27). HIV prevalence was 9.5%(95%CI:8.7-9.3).

In the six months preceding the study, heroin was the primary drug of choice for 99%(303); but cocaine and methamphetamine were also reported by 10%(31) and 4%(12) respectively. Furthermore, 31%(94) and 33%(103) of participants, shared or reused needles in the previous six months, respectively. Up to 43%(133) knew someone who died from a drug-related overdose. PWID reporting sharing needles at least half the time in the previous six months had increased likelihood of HIV-infection, compared to those who did not (aPR: 2.67; 95%CI:1.23–5.78).

Conclusion

HIV infection was common in this sample of PWID in Kigali. The high prevalence of needle reuse and sharing practices highlight significant risk for onward transmission and acquisition of HIV and hepatitis B and C. PWID-focused harm reduction services, including needle and syringe programs, safer injection education, naloxone distribution, and substance use disorder treatment programs, are needed in Rwanda.
Automated ELISA on-chip for the detection of anti-SARS-CoV-2 antibodies
Authors: González-González Everardo , Garcia-Ramirez Ricardo , Díaz-Armas Gladys Guadalupe , Esparza Miguel , Aguilar-Avelar Carlos , Flores-Contreras Elda A. , Rodríguez-Sánchez Irám Pablo , Delgado-Balderas Jesús Rolando , Soto-García Brenda , Aráiz-Hernández Diana , Abarca-Blanco Marisol , León José R.Yee-de , Velarde-Calvillo Liza P. , Abarca-Blanco Alejandro , León Juan F. Yee-de
Published: August 2021

The COVID-19 pandemic has been the most critical public health issue in modern his-tory due to its highly infectious and deathly potential; and the limited access to massive, low-cost, and reliable testing has significantly worsened the crisis. The recovery and the vaccination of millions of people against COVID-19, have made serological tests highly relevant to identify the presence and levels of SARS-CoV-2 antibodies. Due to its advantages, microfluidic-based technologies represent an attractive alternative to the conventional testing methodologies used for these purposes. In this work, we describe the development of an automated ELISA on-chip capable of detecting anti-SARS-CoV-2 antibodies in serum samples from COVID-19 patients and vaccinated individuals. The colorimetric reactions were analyzed with a microplate reader. No statistically significant differences were observed when comparing the results of our automated ELISA on-chip against the ones obtained from a traditional ELISA on a microplate. Moreover, we demonstrated that it is possible to carry out the analysis of the colorimetric reaction by performing basic image analysis of photos taken with a smartphone, which constitutes a useful alternative when lacking specialized equipment or a laboratory setting. Our automated ELISA on-chip has the potential to be used in a clinical setting and mitigate some of the burden caused by testing deficiencies.
Breastfeeding Practices Among Mothers During COVID-19 in India
Authors: Akinola Ajoke Akinola , Simon N. H. , Mohamed Nasteha Abdikadir
Published: August 2021

Introduction

The Covid-19 pandemic is disrupting normal life globally. The COVID-19 pandemic is an emerging concern regarding the potential effects during breastfeeding. The aim of this study was to conduct a systemic review of mother-to -child transmission of COVID-19 during breastfeeding.

Method

This study systematically searched electronic databases; google scholar, PubMed, Medline, up December 2020. The study was included studies relevant to transmission breast milk and respiratory droplets during breastfeeding of mothers with COVID-19 positive. To identify the quality of data, prism standard was used and Strobe checklist scale.

Result

A total of 3160 records were identified in this systemic review with eight relevant studies involving 159 mothers (63 mothers with COVID-19 positive, 55 of their breast milk samples tested negative for the-Covid-19. Twenty-one breast milk samples from 8 women tested positive for Covid-19. Of 73 infants were born to mothers with COVID-19 at the time of delivery. Two infants tested positive for Covid-19. The average mother-child separation time was 36•7 ± 21•1 days among mothers confirmed with COVID-19. Out of 22 mothers, ((37.5%) chose to breastfeed their babies after confirm covid-19 positive.

Conclusion

This study shown that breastfeeding practices were extremely impacted during the COVID-19 epidemic among both confirmed positive cases and suspected mothers. However, the risk of mother-to-infant transmission of Covid-19 vertically or horizontally, in the perinatal period is very low.
BCL6-dependent TCF-1+ progenitor cells maintain effector and helper CD4 T cell responses to persistent antigen
Authors: Xia Yu , Sandor Katalin , Pai Joy A. , Daniel Bence , Raju Saravanan , Wu Renee , Hsiung Sunnie , Qi Yanyan , Yangdon Tenzin , Okamoto Mariko , Schreiber Robert D. , Murphy Kenneth M. , Satpathy Ansuman T. , Egawa Takeshi
Published: August 2021

Shortly after priming, the fate of activated CD4 T cells is segregated into BCL6+ follicular helper T (Tfh) and BCL6− effector (Teff) cells. However, it remains unknown how these subsets are sustained in the presence of chronic antigen stimulation. Using a combination of single cell- and population-based approaches, we show that in chronic viral infection, activated CD4 T cells differentiate into BCL6-dependent TCF-1+ progenitor cells with superior capacity to expand and give rise to both Teff and Tfh. They share properties with progenitor-exhausted CD8 T cells and are required for the continued generation of Teff cells as antigen persists. In response to tumors, an analogous CD4 T cell population develops in draining lymph nodes. Our study reveals the heterogeneity and plasticity of CD4 T cells upon encountering persistent antigen and highlights their population dynamics through a stable bipotent intermediate state.