bluuebunny/crossref_metadata_2025_split huggingface dataset:
Diagnostic performance of ultrasonography, dual-phase 99mTc-MIBI scintigraphy, early and delayed 99mTc-MIBI SPECT/CT in preoperative parathyroid gland localization in secondary hyperparathyroidism
Authors: Zhang Rongqin , Zhang Zhanwen , Huang Pinbo , Li Zhi , Hu Rui , Zhang Jie , Qiu Wanglin , Hu Ping 
Published: August 2020

AbstractBackgroundSecondary hyperparathyroidism (SHPT) usually requires parathyroidectomy when drug regimens fail. However, obtaining an exact preoperative map of the locations of the parathyroid glands is a challenge. The purpose of this study was to compare the diagnostic performance of US, dual-phase99mTc-MIBI scintigraphy, early and delayed99mTc-MIBI SPECT/CT in patients with SHPT.MethodsSixty patients with SHPT who were undergoing dialysis were evaluated preoperatively by US, dual-phase99mTc-MIBI scintigraphy, early and delayed99mTc-MIBI SPECT/CT. Postoperative pathology served as the gold standard. The sensitivity, specificity, and accuracy were determined for each method. Spearman correlation analysis was used to analyse the correlation of hyperplastic parathyroid calcification with serum alkaline phosphatase (ALP) and parathyroid hormone (PTH).ResultsA total of 229 lesions in 60 patients were pathologically confirmed to be parathyroid hyperplasia, with 209 lesions in typical sites, 15 lesions in the upper mediastinum and 5 lesions in the thyroid. A total of 88.33% (53/60) of patients had four lesions. US, early and delayed99mTc-MIBI SPECT/CT had significantly higher sensitivity and accuracy than dual-phase99mTc-MIBI scintigraphy (P < 0.001). Furthermore, early99mTc-MIBI SPECT/CT had significantly higher sensitivity (P < 0.001) and accuracy (P = 0.001 andP < 0.001) than US and delayed99mTc-MIBI SPECT/CT. In patients with ectopic hyperplastic parathyroid glands, the sensitivity of early99mTc-MIBI SPECT/CT (90%) was significantly higher than that of US (55%) and dual-phase99mTc-MIBI scintigraphy (50%) (P < 0.05). The Spearman correlation results showed a significant albeit weak association between calcification and both serum PTH and ALP (P = 0.002).ConclusionThe ability of early99mTc-MIBI SPECT/CT to detect hyperplastic parathyroid glands in patients with SHPT is superior to that of US, dual-phase99mTc-MIBI scintigraphy and delayed99mTc-MIBI SPECT/CT; furthermore, dual-phase99mTc-MIBI SPECT/CT is not essential.
Novel imaging and clinical phenotypes of CONDSIAS disorder caused by a homozygous frameshift variant of ADPRHL2: a case report
Authors: Aryan Hajar , Razmara Ehsan , Farhud Dariush , Zarif-Yeganeh Marjan , Zokaei Shaghayegh , Hassani Seyed Abbas , Ashrafi Mahmoud Reza , Garshasbi Masoud , Tavasoli Ali Reza 
Published: August 2020

Abstract
Background
Stress-induced childhood-onset neurodegeneration with variable ataxia and seizures (CONDSIAS) is an autosomal recessive disorder caused by defects in the ADP-Ribosylhydrolase Like 2 (ADPRHL2; OMIM: 618170) gene. This gene encodes the ADP-ribosylhydrolase enzyme (ARH3) that eliminates the addition of poly-ADP ribose (PAR) in the cellular stress onto proteins in the ADP-ribosylation process in which adding one or more ADP-ribose moieties onto the target proteins in the post-translational modification have occurred. In this study, we report a new case of CONDSIAS in the Iranian population. A literature review of CONDSIAS is also included.

Case presentation
A four-year-old female patient, born to a consanguineous Iranian family, was referred with various clinical symptoms including impaired speech, variable ataxia, infrequent seizures, and gradual onset of truncal hypotonia. Over time, she developed complete motor and speech regression, bilateral sensorineural hearing loss, infrequent seizures, abdominal distension and gastrointestinal (GI) intolerance, and loss of consciousness. To better molecularly diagnose, trio-whole-exome sequencing (WES) was performed on the proband and her parents. Sanger sequencing was also applied to investigate co-segregation analysis. Using in silico predictive tools, the possible impacts of the variant on the structure and function of ADPRHL2 protein were predicted. All basic metabolic tests were normal, while serial coronal magnetic resonance imaging (MRI) showed progressive cerebral and cerebellar atrophy in addition to cerebral white matter signal changes as a novel neuroimaging finding. GI intolerance was another novelty of clinical scenarios in the patient. An auditory brainstem response test showed a severe bilateral sensorineural hearing loss. An electroencephalogram also confirmed focal seizures. From the molecular perspective, a novel homozygous frameshift variant in the ADPRHL2 gene (NM_017825.2; c.636_639del, p.(Leu212fs)) was identified by WES.

Conclusions
CONDSIAS is an ultra-rare neurodegenerative disorder. In the present study, we introduced extra-neurological and neuroimaging findings of this disorder in a female child caused by a novel frameshift variation in the ADPRHL2 gene.
Using airway resistance measurement to determine when to switch ventilator modes in congenital diaphragmatic hernia: a case report
Authors: Kimura Sasagu , Toyoshima Katsuaki , Shimokaze Tomoaki , Hoshino Rikuo 
Published: August 2020

Abstract
Background
Congenital diaphragmatic hernia is a deficiency of the fetal diaphragm resulting in herniation of the abdominal viscera into the thoracic cavity. The best method of respiratory management of congenital diaphragmatic hernia is unclear, but high frequency oscillatory ventilation is often used as the initial ventilator mode for severe congenital diaphragmatic hernia. When it becomes impossible to maintain the pre-ductal saturations, the timing of successful switching of the ventilation mode from high frequency oscillatory ventilation to conventional mechanical ventilation remains unclear. Herein, we reported two cases in which airway resistance measurements based on pulmonary function tests were used for making the decision to switch the ventilator mode from high frequency oscillatory ventilation to conventional mechanical ventilation in patients with left isolated congenital diaphragmatic hernia.

Case presentation
Two 0-day-old infants with congenital diaphragmatic hernia were admitted to our hospital. In both patients, high frequency oscillatory ventilation was started initially, and the levels of saturation gradually rose within a few hours after birth. After 24 h of high frequency oscillatory ventilation, the level of saturation decreased, and the dissociation of pre-ductal and post-ductal saturation re-occurred. The respiratory-system resistance was 515 and 403 cmH2O·kg/L/s, respectively. Because the respiratory-system resistance was elevated, we decided to change the ventilator mode from high frequency oscillatory ventilation to conventional mechanical ventilation. After switching to conventional mechanical ventilation, the patients’ heart rate and saturation increased immediately.

Conclusions
In patients with congenital diaphragmatic hernia, resistance levels of > 400 cmH2O·kg/L/s may indicate high airway resistance and suggest greater alveolar vibration attenuation. When respiratory-system resistance reaches over 400 cmH2O·kg/L/s, it may be an optimal time for switching from high frequency oscillatory ventilation to conventional mechanical ventilation.
Neonatal hand, foot, and mouth disease due to coxsackievirus A6 in Shanghai
Authors: Xu Shanshan , Li Huajun , Qiao Peng , Xu Guofeng , Zhao Dongying , Lin Xiaoyan , Qin Yu , Yu Huiju , Zhang Xi , Zhang Wanju , Huang Lisu 
Published: August 2020

Abstract
Background
Evidence of hand, foot, and mouth disease (HFMD) in neonates is limited. The aim of this study was to evaluate the clinical symptoms, pathogens, possible transmission routes, and prognosis of neonatal HFMD in Shanghai.

Methods
This was a case-control study based on the HFMD registry surveillance system. All neonates and infected family members were enrolled between 2016 and 2017 in Shanghai. Neonates with HFMD were followed for at least half a year. Detailed questionnaires, medical history, and physical examination were recorded. Routine blood examination, liver and renal function, immunophenotypes of peripheral blood lymphocytes (CD3, CD4, and CD8 T-cells; NK cells), immunoglobulin (Ig) M, IgG, and IgA, and cytokine interleukin (IL-1β, IL-2R, IL-6, IL-8, IL-10, and TNF-α) levels were measured. All rectal swab specimens were collected and genotyped for enterovirus, and phylogenetic analysis based on the VP1 sequences of coxsackievirus A6 (CV-A6) was performed to investigate molecular and evolutionary characteristics. T-test or nonparametric test was used to evaluate the differences. Logistic analysis was applied to calculate the risk of clinical manifestations in the group of HFMD neonates and their paired siblings.

Results
There were 16 neonates among the 12,608 diagnosed patients with HFMD, accounting for 0.13%. All neonatal infections were transmitted by other members of the family, mainly the elder siblings, and were caused by CV-A6. CV-A6 was the emerging and predominant causative agent of HFMD in Shanghai. None of the neonates with HFMD experienced fever, onychomadesis, or severe complications. However, two elder sibling patients showed lethargy, and one developed hypoperfusion. In the elder siblings with HFMD, the proportion of white blood cells was generally higher than in neonates with HFMD. The immunologic function of the neonates with HFMD was basically normal. The levels of inflammatory markers were higher in both neonates and elder siblings with HFMD compared to age-matched controls. The clinical symptoms receded about 1 week after onset. None of the neonates had sequelae.

Conclusions
In our study, CV-A6 infection in neonates was benign, but had the character of family clustering. Due to the two-child policy in China, elder siblings may be the main route of HFMD transmission.
Improvement of locomotive syndrome with surgical treatment in patients with degenerative diseases in the lumbar spine and lower extremities: a prospective cohort study
Authors: Kato Satoshi , Kurokawa Yuki , Kabata Tamon , Demura Satoru , Matsubara Hidenori , Kajino Yoshitomo , Okamoto Yoshiyuki , Kimura Hiroaki , Shinmura Kazuya , Igarashi Kentaro , Shimizu Takaki , Yonezawa Noritaka , Yokogawa Noriaki , Tsuchiya Hiroyuki 
Published: August 2020

Abstract
Background
The epidemiology, risk factors, and prevention of locomotive syndrome (LS) have been reported. However, the number of clinical studies about the efficacy of LS treatment, including surgery, has been limited. This study aimed to evaluate LS and its improvement in patients undergoing surgeries for degenerative disease of the lumbar spine and lower extremities, and to discuss the effects of surgery on LS and the issues of LS assessment in these patients.

Methods
We enrolled 257 patients aged ≥60 years that underwent surgery for degenerative diseases of the lumbar spine and lower extremities and agreed to participate in the preoperative and 6- and 12-month postoperative LS examinations. According to the disease location, patients were divided into the lumbar (n = 81), hip (n = 106), knee (n = 43), and foot and ankle (n = 27) groups. Patients underwent LS risk tests, including the stand-up test, two-step test, and 25-Question Geriatric Locomotive Function Scale (GLFS-25) assessment.

Results
The preoperative prevalence of LS stage 2 was 95%. Only the hip group showed significant improvements in the stand-up test. The knee group showed the worst results in the stand-up and two-step tests at all time points. All four groups had significant improvements in GLFS-25 scores. Approximately 40% of all patients had improvement in their LS stage postoperatively. However, > 90% of the patients in the knee group had LS stage 2 postoperatively.

Conclusion
Nearly all elderly patients requiring surgeries for degenerative diseases of the lumbar spine and lower extremities had advanced conditions (LS stage 2). Surgeries could be beneficial in alleviating LS. The LS stage 3 criteria should be established, and the use of the GLFS-25 assessment can be appropriate for advanced LS patients with severe musculoskeletal diseases requiring surgeries.
Frequency of different types of facial melanoses referring to the Department of Dermatology and Venereology, Nepal Medical College and Teaching Hospital in 2019, and assessment of their effect on health-related quality of life
Authors: Amatya Bibush , Jha Anil Kumar , Shrestha Shristi 
Published: August 2020

Abstract
Background
Abnormalities of facial pigmentation, or facial melanoses, are a common presenting complaint in Nepal and are the result of a diverse range of conditions.

Objectives
The objective of this study was to determine the frequency, underlying cause and impact on quality of life of facial pigmentary disorders among patients visiting the Department of Dermatology and Venereology, Nepal Medical College and Teaching Hospital (NMCTH) over the course of one year.

Methods
This was a cross-sectional study conducted at the Department of Dermatology and Venereology, NMCTH. We recruited patients with facial melanoses above 16 years of age who presented to the outpatient department. Clinical and demographic data were collected and all the enrolled participants completed the validated Nepali version of the Dermatology Life Quality Index (DLQI).

Results
Between January 5, 2019 to January 4, 2020, a total of 485 patients were recruited in the study. The most common diagnoses were melasma (166 patients) and post acne hyperpigmentation (71 patients). Quality of life impairment was highest in patients having melasma with steroid induced rosacea-like dermatitis (DLQI = 13.54 ± 1.30), while it was lowest in participants with ephelides (2.45 ± 1.23).

Conclusion
Facial melanoses are a common presenting complaint and lead to substantial impacts on quality of life. Accurate diagnosis and management can prevent or treat many facial melanoses, including those that lead to substantial loss of quality of life, such as melasma with steroid induced rosacea-like dermatitis. Health care systems in low and middle-income countries should dedicate resources to the identification, prevention and treatment of these conditions to improve quality of life.
The association between doctors’ presenteeism and job burnout: a cross-sectional survey study in China
Authors: Pei Pei , Lin Guohua , Li Gaojie , Zhu Yifan , Xi Xiaoyu 
Published: August 2020

AbstractBackgroundIt is necessary to examine doctors working with illness from a professional point of view, because it is not only related to their occupational health, but more importantly, will affect the treatment effect of patients and the overall medical level of the hospital. The purpose of this study was to explore the relationship between doctors’ presenteeism and job burnout, and to identify other factors that are associated with presenteeism.MethodsA cross-sectional survey involving doctors (except for primary doctors) was conducted in China. Using one item measure about presenteeism and a 15-item Chinese version of the BMI-GS questionnaire, this study investigated prevalence of doctors’ presenteeism and job burnout, and determined the relationship between presenteeism and job burnout by logistical model.ResultsRelationship between presenteeism and job burnout were explored, and the influence of work factors were evaluated. The survey was completed by 1376/1547 hospital doctors, with a response rate of 88.9%. Presenteeism was reported by 30.7% of participants. Using MBI-GS, 86.8% of all doctors had moderate job burnout and 6.0%(n = 82) were severe job burnout. Logistic regression analysis showed that doctors with medium, high degree of emotional exhaustion and high degree of cynicism were more likely to practice presenteeism (allp < 0.05). In addition, two other work-related factors, including the doctors’ department and position, were also likely to relate with presenteeism (allp < 0.05).ConclusionsBy examining the relationship between presenteeism and job burnout, this study determined that there is indeed a significant correlation between the two. This result has a certain reference value for the development of work health, especially presenteeism and job burnout theory, and also makes a certain contribution to the relevant research literature.
Predictive values of ANGPTL8 on risk of all-cause mortality in diabetic patients: results from the REACTION Study
Authors: Zou Huajie , Xu Yongping , Chen Xi , Yin Ping , Li Danpei , Li Wenjun , Xie Junhui , Shao Shiying , Liu Liegang , Yu Xuefeng 
Published: August 2020

Abstract
Background
Angiopoietin-like protein 8 (ANGPTL8), an important regulator of lipid metabolism, is increased in diabetes and is associated with insulin resistance. However, the role of ANGPTL8 in the outcomes of diabetic patients remains unclear. This study aimed to investigate circulating levels of ANGPTL8 in participants with and without diabetes and its potential associations with clinical outcomes in a 5 year cohort study.

Methods
Propensity-matched cohorts of subjects with and without diabetes from the Risk Evaluation of Cancers in Chinese Diabetic Individuals: A longitudinal (REACTION) study were generated on the basis of age, sex and body mass index at baseline. The primary outcome was all-cause mortality. The secondary outcomes were a composite of new-onset major adverse cardiovascular events, hospitalization for heart failure, and renal dysfunction (eGFR < 60/min/1.73 m2).

Results
We identified 769 matched pairs of diabetic patients and control subjects. Serum ANGPTL8 levels were elevated in patients with diabetes compared to control subjects (618.82 $$\pm$$
±
318.08 vs 581.20 $$\pm$$
±
299.54 pg/mL, p = 0.03). Binary logistic regression analysis showed that elevated ANGPTL8 levels were associated with greater risk ratios (RRs) of death (RR in quartile 4 vs. quartile 1, 3.54; 95% CI 1.32–9.50) and renal dysfunction (RR in quartile 4 vs. quartile 1, 12.43; 95% CI 1.48–104.81) only in diabetic patients. Multivariable-adjusted restricted cubic spline analyses revealed a significant, linear relationship between ANGPTL8 and all-cause mortality in diabetic patients (p for nonlinear trend = 0.99, p for linear trend = 0.01) but not in control subjects (p for nonlinear trend = 0.26, p for linear trend = 0.80). According to ROC curve analysis, the inclusion of ANGPTL8 in QFrailty score significantly improved its predictive performance for mortality in patients with diabetes.

Conclusion
Serum ANGPTL8 levels were associated with an increased risk of all-cause mortality and could be used as a potential biomarker for the prediction of death in patients with diabetes.
Malaria outbreak in Riaba district, Bioko Island: lessons learned
Authors: Guerra Carlos A. , Fuseini Godwin , Donfack Olivier Tresor , Smith Jordan M. , Ondo Mifumu Teresa Ayingono , Akadiri Gninoussa , Eyang Delicias Esono Mba , Eburi Consuelo Oki , Motobe Vaz Liberato , Micha Victor Mba , Okenve Leonor Ada , Janes Christopher R. , Andeme Ramona Mba , Rivas Matilde Riloha , Phiri Wonder P. , Slotman Michel A. , Smith David L. , García Guillermo A. 
Published: August 2020

AbstractAt the beginning of 2019, a sudden surge of malaria cases was observed in the district of Riaba, Bioko Island. Between January and April, confirmed malaria cases increased 3.8-fold compared to the same period in 2018. Concurrently, anopheline human biting rate (HBR) increased 2.1-fold. During the outbreak, 82.2% of the district population was tested for malaria with a rapid diagnostic test; 37.2% of those tested had a detectable infection and were treated according to national guidelines. Vector control interventions, including indoor residual spraying and larval source management were scaled-up. After the interventions, the number of confirmed cases decreased by 70% and the overall parasite prevalence in the communities by 43.8%. Observed prevalence in a follow up malaria indicator survey, however, was significantly higher than elsewhere on the island, and higher than in previous years. There was no significant reduction in HBR, which remained high for the rest of the year. The surge was attributed to various factors, chiefly increased rainfall and a large number of anthropogenic anopheline breeding sites created by construction works. This case study highlights the need for sustained vector control interventions and multi-sector participation, particularly in malaria control and elimination settings with persistently high local malaria receptivity.
Estimating health service utilization potential using the supply-concentric demand-accumulation spatial availability index: a pulmonary rehabilitation case study
Authors: Matthews Kevin A. , Gaglioti Anne H. , Holt James B. , Wheaton Anne G. , Croft Janet B. 
Published: August 2020

AbstractThe potential for a population at a given location to utilize a health service can be estimated using a newly developed measure called the supply-concentric demand accumulation (SCDA) spatial availability index. Spatial availability is the amount of demand at the given location that can be satisfied by the supply of services at a facility, after discounting the intervening demand among other populations that are located nearer to a facility location than the given population location. This differs from spatial accessibility measures which treat absolute distance or travel time as the factor that impedes utilization. The SCDA is illustrated using pulmonary rehabilitation (PR), which is a treatment for people with chronic obstructive pulmonary disease (COPD). The spatial availability of PR was estimated for each Census block group in Georgia using the 1105 residents who utilized one of 45 PR facilities located in or around Georgia. Data was provided by the Centers for Medicare & Medicaid Services. The geographic patterns of the SCDA spatial availability index and the two-step floating catchment area (2SFCA) spatial accessibility index were compared with the observed PR utilization rate using bivariate local indicators of spatial association. The SCDA index was more associated with PR utilization (Morans I = 0.607, P < 0.001) than was the 2SFCA (Morans I = 0.321, P < 0.001). These results suggest that the measures of spatial availability may be a better way to estimate the health care utilization potential than measures of spatial accessibility.