bluuebunny/crossref_metadata_2025_split huggingface dataset:
Family studies of warts, hypogammaglobulinemia, immunodeficiency, myelokathexis syndrome
Authors: Dale David C. , Dick Emily , Kelley Merideth , Makaryan Vahagn , Connelly Jim , Bolyard Audrey Anna 
Published: October 2019

Purpose of review
WHIM syndrome (warts, hypogammaglobulinemia, immunodeficiency, myelokathexis, or WHIMs) is a very rare autosomal dominant immunodeficiency disorder attributable to mutations in CXCR4. We reviewed clinical manifestations in 24 patients in 9 families to expand understanding of this syndrome.

Recent findings
Warts, cellulitis and respiratory infections are common in patients with WHIMs. Less commonly these patients have congenital heart disease, human papilloma virus-associated malignancies (cervical and vulvular) and lymphomas. Hearing loss because of recurrent otitis media is another important complication. Treatment with granulocyte colony-stimulating factor is controversial; this review indicates that it is effective to prevent and treat infections based upon long-term observations of patients enrolled in the Severe Chronic Neutropenia International Registry. Understanding the natural history and diversity of this syndrome are important for ongoing clinical trials of novel agents to treat WHIMs.

Summary
WHIM syndrome has diverse manifestations; some features occur consistently in almost all patients, for example, neutropenia, lymphocytopenia and mild hypogammaglobulinemia. However, the clinical consequences are quite variable across patient cohorts and within families. Each complication is important as a cause for morbidity and a source for patient and family concerns.
Laryngeal amyloidosis
Authors: Burns Hannah , Phillips Nicholas 
Published: October 2019

Purpose of review
Laryngeal amyloidosis is a rare hematological disorder of plasma cells. The cause is still considered idiopathic. The otolaryngology literature predominantly comprises case reports and short series. The present review summarizes the amyloid literature more generally in order to assist the otolaryngologist managing this disorder.

Recent findings
Distinguishing localized amyloidosis from systemic disease continues to be challenging. Both radiological and hematological investigations may assist. Surgery continues to be the predominant modality for treatment, but radiation and potentially medical approaches are on the horizon.

Summary
When localized, this benign laryngeal disease carries an excellent prognosis. Clinicians should however be aware of systemic presentations and ensure patients receive long-term follow-up.
Early Obesity
Authors: Cuzmar Valeria , Alberti Gigliola , Uauy Ricardo , Pereira Ana , García Cristián , De Barbieri Florencia , Corvalán Camila , Santos José L. , Mericq Verónica , Villarroel Luis , Gana Juan Cristóbal 
Published: October 2019

ABSTRACTNonalcoholic fatty liver disease (NAFLD), defined as fat accumulation greater than 5% in hepatocytes, may progress to fibrosis or cirrhosis later in life. NAFLD prevalence in adolescents has increased significantly in direct relation with obesity prevalence. Fatty liver has become the most frequent indication for liver transplantation in adults.Objective:The aim of the study was to identify anthropometric variables during the first 10 years of life associated to the risk of developing NAFLD in adolescence.Methods:Longitudinal cohort study 'Growth and Obesity Chilean Cohort Study’ (GOCS) consisting of 513 children born in 2002 to 2003, with yearly anthropometric data collected over a 10‐year period. The presence of intrahepatic fat in the livers of subjects 14 to 16 years of age was determined using abdominal ultrasound. In addition, elastography was performed on all participants with ultrasound evidence of NAFLD.Results:9.7% of the participants presented findings compatible with NAFLD. After 2 years of age, obesity significantly and progressively increased the probability of NAFLD occurrence in adolescence. Obesity at 5 years of age was associated with the highest OR for NAFLD, reaching values of 8.91 (95% CI 3.03–16.11). Among participants with NAFLD, those with altered liver elasticity (≥7 kPa) had greater weight, BMI z‐score, waist and hip circumference, and altered liver enzymes (P < 0.05).Conclusion:The risk of developing NAFLD in adolescence increases progressively with early obesity starting at age 2 years.
Pregnant African American Women's Perceptions of Neighborhood, Racial Discrimination, and Psychological Distress as Influences on Birth Outcomes
Authors: Dove-Medows Emily , Deriemacker Amanda , Dailey Rhonda , Nolan Timiya S. , Walker Deborah S. , Misra Dawn P. , Kavanaugh Karen , Giurgescu Carmen 
Published: October 2019

Abstract

Background:
African American women are more likely to experience preterm birth compared with White women. Social factors such as neighborhood disorder and experiences of racial discrimination, which disproportionately affect African American women, may partially explain these disparities.

Purpose:
The purpose of this study was to examine pregnant African American women's perceptions of neighborhood disorder, racial discrimination, and psychological distress and whether these concepts were viewed as influences on birth outcomes.

Study Design and Methods:
Using a mixed-methods approach, seven pregnant African American women completed questionnaires including scales for neighborhood disorder (Ross Neighborhood Disorder Scale), racial discrimination (Experiences of Discrimination), and psychological distress (Center for Epidemiological Studies Depression Scale; Psychological General Well-Being Index). All constructs were also assessed by semistructured interviews. Within- and across-case analyses were conducted to compare agreement and discordance between the data sources for each construct and to note patterns in the data.

Results:
The qualitative interviews provided data about women's experiences that were not captured by questionnaires alone. All of the women disclosed concerns about neighborhood conditions, experiences of discrimination, and psychological distress either reported on the questionnaires, during the qualitative interviews, or both. The mixed-methods approach provided a rich source of data that brought into focus the depth of the perceptions around these constructs.

Clinical Implications:
Maternal–child nurses should assess perceptions of neighborhood environment, racial discrimination, and psychological distress, as these factors may increase the risk for adverse pregnancy and birth outcomes.
The Relationship Between Experiential Learning and Nursing Management Competency
Authors: Kuraoka Yumiko 
Published: October 2019

OBJECTIVE
This study aimed to examine the relationship between experiential learning and nursing management competency among nurse managers (NMs) in the early years of a supervisory role.

BACKGROUND
The relevance of experiential learning to the development of NMs has been explored in an effort to harvest the knowledge embedded in shared NM practice narratives. However, the relationship between experiential learning and nursing management competency has not been empirically measured.

METHODS
The author developed an experiential learning-based program for NMs in the initial 3 years of a supervisory role. Sixty-three NMs were enrolled. The relationship between experiential learning and nursing management competency was evaluated using multiple regression analysis after completing this program.

RESULTS
The results of multiple regression analysis revealed a significant relationship between experiential learning and nursing management competency (P < .001).

CONCLUSIONS
A significant relationship was identified between experiential learning and nursing management competence.
Effect of Personalized Music Intervention in Mechanically Ventilated Children in the PICU: A Pilot Study*
Authors: Liu Mei-hua , Zhu Li-hui , Peng Jian-xiong , Zhang Xin-ping , Xiao Zheng-hui , Liu Qiong-jie , Qiu Jun , Latour Jos M. 
Published: October 2019

Objectives:
To determine the feasibility of a personalized music intervention with mechanically ventilated patients in the PICU.

Design:
Pilot study with a quasi-experimental design.

Setting:
Tertiary children’s hospital in China with a 40-bed PICU.

Patients:
Children, 1 month to 7 years, with mechanical ventilation were recruited and assigned to music group (n = 25) and control group (n = 25).

Interventions:
Children in the music group received their own favorite music and listened for 60 minutes three times a day. The control group receive routine care without music.

Measurements and Main Results:
Primary outcome measure was comfort measured with the COMFORT Behavior scale 5 minutes before and after the music. Secondary outcome measures were physiologic variables; heart rate, respiration, blood pressure, oxygen saturation. Mechanical ventilation time, length of stay, and sedation medication were also collected. Qualitative analysis revealed that nurses had a positive attitude in delivering the interventions and identified improvements for the main trial. Children in the music group had lower COMFORT Behavior scores (15.7 vs 17.6; p = 0.011). Children in the music group had better physiologic outcomes; heart rate (140 vs 144; p = 0.039), respiration rate (40 vs 43; p = 0.036), systolic blood pressure (93 vs 95 mm Hg; p = 0.031), oxygen saturation (96% vs 95%; p < 0.001), diastolic blood pressure was not significantly (52 vs 53 mm Hg; p = 0.11). Children in the music group had a shorter ventilation time (148.7 vs 187.6; p = 0.044) and a shorter length of stay, but not significant (11.2 vs 13.8; p = 0.071). Children in the control group had higher total amount of on-demand midazolam (29 vs 33 mg; p = 0.040).

Conclusions:
Our pilot study indicates that personalized music intervention is feasible and might improve the comfort of children with mechanical ventilation. Further studies are needed to provide conclusive evidence in confirming the effectiveness of music interventions comforting critically ill children in PICUs.
Predictors of Viremia in Postpartum Women on Antiretroviral Therapy
Authors: Hoffman Risa M. , Warshaw Meredith G. , Amico K. Rivet , Pilotto Jose , Masheto Gaerolwe , Achalapong Jullapong , Machado Elizabeth , Chokephaibulkit Kulkanya , Duarte Geraldo , João Esau , Graham Kathleen K. , Knapp Katherine M. , Stek Alice M. , Scott Gwendolyn B. , Coletti Anne , Loftis Amy J. , Chakhtoura Nahida , Currier Judith S. ,   for the PROMISE 1077HS Team
Published: October 2019

Background:
HIV-infected, postpartum women on antiretroviral therapy (ART) have high rates of viremia. We examined predictors of postpartum viremia in the PROMISE study.

Methods:
Women with pre-ART CD4+ T-cell counts ≥400 cells/mm3 who started ART during pregnancy were randomized postpartum to continue ART (CTART) or discontinue ART (DCART). Viral load and self-reported adherence were collected every 12 weeks, up to 144 weeks. Women in DCART reinitiated therapy when clinically indicated. Viremia was defined as 2 consecutive viral loads >1000 copies/mL after 24 weeks on ART. Adherence was dichotomized as missing versus not missing ART doses in the past 4 weeks. Predictors of viremia were examined using Cox proportional hazards regression with adherence as a time-varying covariate.

Results:
Among 802 women in the CTART arm, median age at entry was 27 years and median CD4+ T-cell count 696 cells/mm3. Of 175 women in CTART with viremia (22%), 141 had resistance data, and 12% had resistance to their current regimen. There was an estimated 0.12 probability of viremia by week 48 and 0.25 by week 144. Predictors of viremia included missed ART doses within the past 4 weeks, younger age, shorter duration of pre-entry ART, and being from the South American/Caribbean region. Of 137 women in DCART who reinitiated therapy, probability of viremia was similar to CTART (0.24 by week 96; 0.27 by week 144).

Conclusions:
Rates of postpartum viremia are high and viremia is more likely in younger postpartum women who start ART later in pregnancy. Interventions should target these higher-risk women.
Asymptomatic Metastasis to Thyroid Cartilage Detected by 18F-Choline and 64Cu-PSMA PET/CT as a Single Site of Disease Relapse in a Patient With Castration-Resistant Prostate Carcinoma
Authors: Annovazzi Alessio , Faiella Adriana , Pescarmona Edoardo , Sanguineti Giuseppe , Sciuto Rosa 
Published: October 2019

AbstractMetastatic involvement of the larynx is rare due to the absence of vessels within the cartilaginous tissue. The probability of metastatic spread increases with aging as a result of larynx ossification. The secondary involvement of larynx is more frequently associated with melanoma and renal cell carcinoma. Few observations have been reported also in prostate cancer patients, usually associated with advanced disease and diffuse metastatic bone involvement. We report a case of an 83-year-old man with castration-resistant prostate cancer in whom a metastasis to the thyroid cartilage was the only site of relapse.
Simultaneous Correction of Complex Secondary Deformities of Cleft Lip Using Autogenous Costal Cartilage Combined With Rib Grafts
Authors: Zhang Lu , Wei Shuqiang , Qi Lin , Huang Weilong , Yu Chunbo , Cheng Liying , Jin Rong , Zhang Ying , Sun Xiaoming , Zhang Yuguang 
Published: October 2019

Objective:
Complex secondary deformities of cleft lip include nasal and labial deformities, alveolar cleft, oronasal fistula, and so on. In this report, the authors present a method to simultaneously repair the above complex deformities with autogenous costal cartilage and rib grafts.

Methods:
The surgical procedure of simultaneous correction includes

Results:
Satisfactory outcomes were achieved in all 22 patients. Fifteen patients were followed up for 6 to 24 months and exhibited good nasal shape, repaired alveolar cleft, improved labial shape, and closed oronasal fistula. Three patients presented with red nasal tips. One patient had local infection, which was cured by antibiotics.

Conclusion:
This method
As this method has led to positive outcomes, it has the potential to be widely used in clinical practice.
Treatment of Intraosseous Hemangioma With Embolization in Mandible
Authors: Yalcin Mustafa , Ozer Nedim 
Published: October 2019

Abstract
The authors report a rare case of intraosseous hemangioma of the mandible in a 14-year-old male. Surgical intervention with embolization is as important as histological and radiological examination to reduce the bleeding complication. Magnetic resonance angiography is paramount of importance in these lesions to detect the supplier arteries.
The Effect of Burn Resuscitation Volumes on the Gut Microbiome in a Swine Model
Authors: McIntyre Matthew K. , Winkler Charlotte J. , Gómez Belinda I. , Lapierre Jean-Paul , Little Joshua S. , Dubick Michael A. , Nicholson Susannah E. , Burmeister David M. 
Published: October 2019

ABSTRACT

Introduction:
While recent reports underscore the significance of the gut microbiome (GM) in health and disease, its importance in burn outcomes remains unclear. Moreover, aggressive intravenous (IV) fluid resuscitation of patients may alter intestinal flora. Herein, we describe GM changes following a large burn in swine randomized to different volumes of IV Lactated Ringers’ (LR).

Methods:
Anesthetized Yorkshire swine sustained 40% total body surface area full-thickness burns and were randomized to different volumes of IV LR: none (n = 5), 15 mL/kg/d (low; n = 6), or 80 mL/kg/d (high; n = 6). At baseline and days 1 and 2, fecal swabs were collected for 16s rDNA sequencing. Proximal jejunum was collected immediately after euthanasia (day 2) for western blot, histopathology, and cytokine analyses.

Results:
Burns produced significant shifts in β-diversity and non-significant reductions in α-diversity that did not recover regardless of treatment group. Burn-induced increases in Proteobacteria and decreases in Firmicutes were attenuated by IV fluids in a dose-dependent manner, and also correlated with α-diversity. IV fluids caused a dose-dependent increase in Bacteroides and prevented a transient increase in the opportunistic pathogen Haemophilus parainfluenzae. While high volumes of IV fluids increased intestinal Hsp70 levels (P = 0.0464), they reduced SGLT1 (P = 0.0213) and caspase3 (P = 0.0139) levels. IV fluids elicited a non-specific cytokine response; however, Bacteroidetes levels correlated with intestinal IL18 levels (P = 0.0166, R2 = 0.4201).

Conclusions:
We present the first report on the gut microbiome in a porcine burn model, and present data to suggest that IV fluids may influence GM and gut functional proteins following a burn. Overall, burn-induced GM diversity shifts may expose diagnostic and/or therapeutic targets to improve outcomes.
Medical Student Skill Retention After Cardiopulmonary Resuscitation Training
Authors: Saad Rafael , Sampaio Favarato Maria Helena , Ferreira de Paiva Edison , do Patrocinio Tenorio Nunes Maria 
Published: October 2019

Introduction
The retention of cardiopulmonary resuscitation skills and the ideal frequency of retraining remain unanswered. This study investigated the retention of cardiopulmonary resuscitation skills by medical students for up to 42 months after training.

Methods
In a cross-sectional study, 205 medical students received 10 hours of training in basic life support in 3 practical classes, during their first semester at school. Then, they were divided into 4 groups, according to the time elapsed since the training: 73 after 1 month, 55 after 18 months, 41 after 30 months, and 36 after 42 months. Nineteen cardiopulmonary resuscitation skills and 8 potential technical errors were evaluated by mannequin-based simulation and reviewed using filming.

Results
The mean retention of the skills was 90% after 1 month, 74% after 18 months, 62% after 30 months, and 61% after 42 months (P < 0.001). The depth of chest compressions had the greatest retention over time (87.8%), with no significant differences among groups. Compressions performed greater than 120 per minute were less likely to be done with adequate depth. Ventilation showed a progressive decrease in retention from 93% (n = 68) after 1 month to 19% (n = 7) after 42 months (P < 0.001). All 205 students were able to turn the automated external defibrillator on and deliver the shock.

Conclusions
The depth of chest compressions and the use of an automated external defibrillator were the skills with the highest retention over time. Based on a skills retention prediction curve, we suggest that 18 to 24 months as the minimum retraining interval to maintain at least 70% of skills.
Aging Adults' Preferences for Wellness Program Activities and Delivery Characteristics
Authors: Talley Kristine M. C. , Cheung Corjena , Mathiason Michelle A. , Schorr Erica , McMahon Siobhan , Wyman Jean F. 
Published: October 2019

Background:
Lifestyle wellness programs help prevent and manage chronic diseases, yet few are designed for aging adults.

Purpose:
Identify characteristics associated with aging adults' preferences for wellness program activities and delivery characteristics.

Subjects/Methods:
Cross-sectional, self-administered survey of a convenience sample of 386 adults aged 55 years and older. Logistic regression models identified characteristics influencing preferences.

Results:
Current healthy behaviors, gender, and age influenced many preferences, while body mass index, multiple chronic conditions, self-rated general health status, and quality of life did not.

Discussion:
Incorporating aging adults' preferences for wellness programs will help design appealing and engaging programs.
Outcomes of Patients Suspended From the National Kidney Transplant Waiting List in the United Kingdom Between 2000 and 2010
Authors: Wallace David , Robb Matthew , Hughes Winter , Johnson Rachel , Ploeg Rutger , Neuberger James , Forsythe John , Cacciola Roberto 
Published: October 2019

Background.
In the United Kingdom, 1 in 3 patients on the National Kidney Transplant Waiting List (NKTWL) is suspended from the list at least once during their wait. The mortality of this large cohort of patients remains underreported and poorly described.

Methods.
We linked patient records from the UK transplant registry to mortality data from the Office of National Statistics and evaluated the impact of a clinically induced suspension event by estimating hazard ratios (HRs) that compared mortality and graft survival between those who had experienced a suspension event and those who had not.

Results.
Between January 1, 2000, and December 31, 2010, 16.7% (2221/13 322) of all patients registered on the NKTWL were suspended. Forty-eight percent (588/1225) of those who were suspended and who were never transplanted died, most often from cardiothoracic causes. A suspension event was associated with increased mortality from the time of listing (adjusted HR [aHR], 1.79; 1.64-1.95) and from the time of transplantation (aHR, 1.20; 1.06-1.37; P = 0.005). Graft survival was also poorer in those who had been suspended (aHR, 1.13; 1.01-1.28; P = 0.04).

Conclusions.
Patients suspended on the NKTWL have a significantly higher rate of mortality both on the waiting list and following transplantation. Earlier prioritization of patients at risk of experiencing a suspension event may improve their outcomes.
Delayed Implantation of Pumped Kidneys Decreases Renal Allograft Futility in Combined Liver–Kidney Transplantation
Authors: Lunsford Keri E. , Agopian Vatche G. , Yi Stephanie G. , Nguyen Duc T.M. , Graviss Edward A. , Harlander-Locke Michael P. , Saharia Ashish , Kaldas Fady M. , Mobley Constance M. , Zarrinpar Ali , Hobeika Mark J. , Veale Jeffrey L. , Podder Hemangshu , Farmer Douglas G. , Knight Richard J. , Danovitch Gabriel M. , Gritsch H. Albin , Li Xian C. , Ghobrial R. Mark , Busuttil Ronald W. , Gaber A. Osama 
Published: October 2019

Background.
Combined liver–kidney transplantation (CLKT) improves survival for liver transplant recipients with renal dysfunction; however, the tenuous perioperative hemodynamic and metabolic milieu in high-acuity CLKT recipients increases delayed graft function and kidney allograft failure. We sought to analyze whether delayed KT through pumping would improve kidney outcomes following CLKT.

Methods.
A retrospective analysis (University of California Los Angeles [n = 145], Houston Methodist Hospital [n = 79]) was performed in all adults receiving CLKT at 2 high-volume transplant centers from February 2004 to January 2017, and recipients were analyzed for patient and allograft survival as well as renal outcomes following CLKT.

Results.
A total of 63 patients (28.1%) underwent delayed implantation of pumped kidneys during CLKT (dCLKT) and 161 patients (71.9%) received early implantation of nonpumped kidneys during CLKT (eCLKT). Most recipients were high-acuity with median biologic model of end-stage liver disease (MELD) score of, 35 for dCLKT and 34 for eCLKT (P = ns). Pretransplant, dCLKT had longer intensive care unit stay, were more often intubated, and had greater vasopressor use. Despite this, dCLKT exhibited improved 1-, 3-, and 5-year patient and kidney survival (P = 0.02) and decreased length of stay (P = 0.001), kidney allograft failure (P = 0.012), and dialysis duration (P = 0.031). This reduced kidney allograft futility (death or continued need for hemodialysis within 3 mo posttransplant) for dCLKT (6.3%) compared with eCLKT (19.9%) (P = 0.013).

Conclusions.
Delayed implantation of pumped kidneys is associated with improved patient and renal allograft survival and decreased hospital length of stay despite longer kidney cold ischemia. These data should inform the ethical debate as to the futility of performing CLKT in high-acuity recipients.