bluuebunny/crossref_metadata_2025 huggingface dataset:
Talus osteomyelitis by Candida krusei with multiple huge cystic lesions: a case report and review of literatures
Authors: Kim Hyungtae , Bae Su-Young
Published: July 2022

Background

Osteomyelitis due toCandida kruseiare extremely rare, given that only six cases have been reported, all of which are limited to the patients with immunocompromising risk factors. Here we report a case ofC. kruseiosteomyelitis in an immunocompetent patient, presenting with multiple huge cystic lesions of talus.

Case presentation

A 66-year-old female presented with one year history of painful swelling of right ankle and a draining sinus around lateral malleolus. Five months and three months ago, she had undergone arthroscopic synovectomy and bursectomy which revealed no causative organism. Open bursectomy with sinus tract excision was performed and intravenous antibiotic was administered. Two year after the surgery, the patient revisited the clinic for recurrent painful swelling with pus drainage at the same location. Multiple huge cystic lesions with osteolysis and sclerotic rim of talus were found andC. kruseiwas isolated from tissue culture. The patient received surgical debridement and prolonged antifungal treatment comprising caspofungin and voriconazole.

Conclusions

In this case,C. kruseiinfection showed atypically aggressive osteolysis shown as multiple huge cystic abscess. High index of suspicion is critical for early diagnosis and treatment to prevent such devastating results even in an immunocompetent patient.
Surgeons' requirements for a surgical support system to improve laparoscopic access
Authors: Spiller Moritz , Bruennel Marcus , Grosse Victoria , Sühn Thomas , Esmaeili Nazila , Stockheim Jessica , Turial Salmai , Croner Roland , Boese Axel , Friebe Michael , Illanes Alfredo
Published: July 2022

Creating surgical access is a critical step in laparoscopic surgery. Surgeons have to insert a sharp instrument such as the Veress needle or a trocar into the patient’s abdomen until the peritoneal cavity is reached. They solely rely on their experience and distorted tactile feedback in that process, leading to a complication rate as high as 14% of all cases. Recent studies have shown the feasibility of surgical support systems that provide intraoperative feedback regarding the insertion process to improve laparoscopic access outcomes. However, to date, the surgeons’ requirements for such support systems remain unclear. This research article presents the results of an explorative study that aimed to acquire data about the information that helps surgeons improve laparoscopic access outcomes. The results indicate that feedback regarding the reaching of the peritoneal cavity is of significant importance and should be presented visually or acoustically. Finally, a solution should be straightforward and intuitive to use, should support or even improve the clinical workflow, but also cheap enough to facilitate its usage rate. While this study was tailored to laparoscopic access, its results also apply to other minimally invasive procedures.
Isolated ACTH deficiency following immunization with the BNT162b2 SARS-CoV-2 vaccine: a case report
Authors: Morita Shuhei , Tsuji Tomoya , Kishimoto Shohei , Uraki Shinsuke , Takeshima Ken , Iwakura Hiroshi , Furuta Hiroto , Nishi Masahiro , Inaba Hidefumi , Matsuoka Taka-aki
Published: July 2022

Background

The global COVID-19 pandemic requires urgent development of new vaccines. Endocrinological adverse effects following the new mRNA vaccine against COVID-19 have been reported in several cases. Specific to the involvement of pituitary function; however, only a single case with hypophysis has been reported. This is the first case of isolated adrenocorticotropic hormone (ACTH) deficiency (IAD) following mRNA vaccination against COVID-19.

Case presentation

A healthy 31-year-old man received the BNT162b2 SARS-CoV-2 mRNA vaccine. The first injection was uneventful. One day after the second injection, he noticed general fatigue and fever. In the following several days, he additionally developed headaches, nausea, and diarrhea. Four days after the vaccine injection, he visited a hospital with worsening of these symptoms. Physical examination revealed slight disorientation but no other deficits. Laboratory tests revealed hyponatremia, hypoglycemia, and extremely low plasma ACTH and serum cortisol levels (ACTH < 1.5 pg/ml, cortisol 1.6 μg/dl). He was diagnosed with adrenal crisis and was emergently treated with hydrocortisone. The symptoms responded well and he recovered within a few days. Magnetic resonance images after the replacement with hydrocortisone revealed an atrophic pituitary gland. The patient was referred to our tertiary hospital for further endocrinological examination. Pituitary endocrine load tests revealed isolated adrenocortical response deficiency. After other clinical assessments, he was diagnosed as having isolated ACTH deficiency. After initiation of hydrocortisone replacement, there has been no recurrence of symptoms related to adrenocortical insufficiency nor involvement of other pituitary functions.

Conclusion

This is the first reported case of IAD potentially associated with COVID-19 immunization. Recent reports have emphasized the importance of adjuvants in the mRNA vaccine that induce the endocrinological adverse effects through disturbance of the autoimmune system, but details are still unclear. Given the broad and rapid spread of vaccinations against COVID-19, it is clinically important to consider that there could be cases with a rare but emergent adrenal crisis even among those who present common symptoms of adverse effects following inactive SARS-CoV-2 mRNA vaccination.
The effect of clinical pharmacists’ intervention in adverse drug reaction reporting: a retrospective analysis with a 9-year interrupted time series
Authors: Lan Tianwei , Wang Hua , Li Xin , Yin Hang , Shao Dan , Jiang Yueyao , Yu Qian
Published: July 2022

Background

In China, 85.4% of adverse drug reactions (ADRs) are spontaneously reported by healthcare facilities. As a result, many ADRs are not reported due to lack of mandatory reporting requirements. As healthcare professionals, clinical pharmacists (CPhs) serve as a bridge between clinical work and medication and ensure rational drug use. In China, A team of CPhs implemented an intervention for ADRs reporting, with the goal of improving the number of ADRs reports, the number of unreported ADRs, and the standardized reporting rate.

Methods

On June 01, 2015, a team of CPhs implemented an intervention for ADRs reporting at a Grade A, Class 3 hospital in China. The drug review catalogue (DRC) was used to screen physician orders for having visible symptoms of ADRs across departments, pooled the ADRs, and submitted them to the Center for Advanced Drug Monitoring (CNCAM). We retrospectively analysed the effect of a CPhs ADRs reporting intervention on the number of clinical ADRs reports, the number of unreported ADRs, and the standardized reporting rate over a 9-year period by interrupted time series (ITS). The method was implemented at the hospital on June 1, 2015, and a segmented regression model was used to analyse the data from January 1, 2010, to December 31, 2019.

Results

After the CPhs ADRs reporting intervention, the number of inpatient ADRs reports submitted to the CNCAM immediately increased by approximately 63 (62.658, P < 0.01) and then decreased by approximately 1 (0.701, P = 0.000151 < 0.01) per month afterward; the number of unreported ADRs was immediately reduced by approximately 44 (44.091, P < 0.01) and remained largely unchanged over time (P > 0.05); the standardized ADRs reporting rate per month immediately increased by 63.634% (P < 0.01) and remained largely unchanged over time (P > 0.05).

Conclusion

The CPhs ADRs reporting intervention had an immediate effect on improving ADRs reporting, which highlights the severity of ADRs underreporting in Chinese hospitals. The method is practical and should be used more widely in clinical practice. For example, the method can adjust and establish a DRC catalog that meets the actual situation of the implementing hospital based on the hospital's drug use habits and has the characteristics of good adaptability. However, it does have some limitations; for example, it may be difficult to detect early ADRs without visible symptoms.
The association between primary care use and potentially-preventable hospitalization among dual eligibles age 65 and over
Authors: Oh N. Loren , Potter Andrew J. , Sabik Lindsay M. , Trivedi Amal N. , Wolinsky Fredric , Wright Brad
Published: July 2022

Background

Individuals dually-enrolled in Medicare and Medicaid (dual eligibles) are disproportionately sicker, have higher health care costs, and are hospitalized more often for ambulatory care sensitive conditions (ACSCs) than other Medicare beneficiaries. Primary care may reduce ACSC hospitalizations, but this has not been well studied among dual eligibles. We examined the relationship between primary care and ACSC hospitalization among dual eligibles age 65 and older.

Methods

In this observational study, we used 100% Medicare claims data for dual eligibles ages 65 and over from 2012 to 2018 to estimate the likelihood of ACSC hospitalization as a function of primary care visits and other factors. We used linear probability models stratified by rurality, with subgroup analyses for dual eligibles with diabetes or congestive heart failure.

Results

Each additional primary care visit was associated with an 0.05 and 0.09 percentage point decrease in the probability of ACSC hospitalization among urban (95% CI: − 0.059, − 0.044) and rural (95% CI: − 0.10, − 0.08) dual eligibles, respectively. Among dual eligibles with CHF, the relationship was even stronger with decreases of 0.09 percentage points (95% CI: − 0.10, − 0.08) and 0.15 percentage points (95% CI: − 0.17, − 0.13) among urban and rural residents, respectively.

Conclusions

Increased primary care use is associated with lower rates of preventable hospitalizations for dual eligibles age 65 and older, especially for dual eligibles with diabetes and congestive heart failure. In turn, efforts to reduce preventable hospitalizations for this dual-eligible population should consider how to increase access to and use of primary care.
Generic substitution for prescribed brand medicines in Ethiopia: knowledge, attitude and practice among pharmacy professionals in community drug retail outlets
Authors: Alemu Sintayehu , Tadesse Natnael , Mulugeta Tidenek , Assefa Desta
Published: July 2022

Background

Generic substitution is a good approach to reduce pharmaceutical expenses without compromising healthcare quality. Yet, the practice of generic substitution has been contentious due to concerns on quality and efficacy.

Objective

This study was aimed to assess knowledge, attitude and practice among pharmacy professionals toward generic substitution in community drug retail outlets in Jimma town, Southwest Ethiopia.

Methods

A descriptive cross-sectional study was conducted among pharmacy professionals working in community drug retail outlets. Data was collected using a self-administered questionnaire. Knowledge was tested using a 3-point response format consisting of “Yes,” “No” and “I am not sure.” Attitude was evaluated using the 5-point Likert scale ranging from 1(strong agreement) to 5 (strong disagreement). The practice was examined as never, seldom, sometimes, often, and always with scores ranging from 0 to 4. The influences of socio-demographic factors on knowledge, attitude, and practice were tested using the Mann–Whitney U and Kruskal–Wallis tests as appropriate. P ≤ 0.05 was considered statistically significant.

Results

The mean knowledge score of participants regarding generic medicines was 5.75 ± 1.79. Only 32 respondents (30.2%) of the participants were knowledgeable about the generic substitution. 54 (50.9%) of respondents had positive attitude toward generic substitution and 52 (49.1%) had practiced generic substitution. The year of experience had a significant effect on knowledge (X2 = 9.14, p = 0.01) and practice (X2 = 4.71, p = 0.03) of generic substitution.

Conclusions

Our study found that pharmacy professionals working in community drug retail outlets in Jimma town had lack of knowledge about generic substitution. Conversely, an enormous amount of participants had positive attitude toward generic substitution and nearly half of them had practiced generic substitution. The year of experience had a significant effect on knowledge and practice of generic substitution.
Prevalence of and factors associated with unintended pregnancies among sexually active undergraduates in mainland China
Authors: Yuan Yan , Ruan Fang , Liu Yusi , Wu Lei , Pan Mingliang , Ye Zijie , Zhao Youxiong , Lin Lu , Zhang Li , Liu Jiajun , Luo Dongsheng , Zhu Bangzheng , Liao Xinyu , Hong Mengsi , Wang Siyi , Chen Jilun , Li Zihao , Yang Gaoming , Jiang Hongfang , Fu Guochen , Wang Junfang
Published: July 2022

Background

Unintended pregnancies (UIP) among unmarried sexually active college students in mainland China have emerged as a major reproductive health issue with detrimental personal and socioeconomic consequences. This cross-sectional study aimed to determine the prevalence and factors associated with UIP among sexually active undergraduates in mainland China.

Methods

Between September 8, 2019 and January 17, 2020, a total of 48,660 participants were recruited across the Chinese mainland to complete the self-administered, structured, online questionnaire. This analysis was restricted to 6347 sexually experienced, never-married 15–26 year old undergraduates. Pearson’s Chi square tests and multivariate Logistic regression analyses were performed to identify sociodemographic, familial and individual variables associated with UIP.

Results

The overall prevalence of UIP was 17.7%. More specifically, 19.5% of male college students reported they had unintentionally gotten a partner pregnant, while 14.9% of female college students became unintentionally pregnant. Students who experienced UIP were more likely to belong to the older age group (23–26 years), live with only one parent or live without parents at home, report that their family members approve of premarital sex, initiate sexual activity younger than 14 years old and have casual sex partners. Furthermore, females with multiple partners and males who came from low- income households, experienced sexual abuse, perceived difficulties in acquiring condoms and did not know how to use condoms correctly were also at higher risk of experiencing an unintended pregnancy.

Conclusion

In order to prevent UIP, a comprehensive intervention measure should be taken to target older students and those engaging in risky sexual behaviors, work with young male students to improve condom use skills, improve the availability of free condoms, optimize the involvement of parents and other family members in their children’s sex education.
Prevalence and associated factors of sexual violence experienced by housemaids in Ethiopia: a systematic review and meta-analysis
Authors: Mekonnen Birye Dessalegn , Lakew Zemene Habtu , Melese Endalkachew Belayneh
Published: July 2022

Background

Housemaids often experience different types of sexual violence by different perpetrators. Sexual violence against housemaids remains usually concealed as victims cannot report such offenses. Except for fragmented studies with varying reports, there is no national prevalence studies conducted on sexual violence among housemaids in Ethiopia. Thus, this systematic review and meta-analysis aimed to determine the pooled prevalence and associated factors of sexual violence amongst housemaids in Ethiopia.

Methods

A systematic search of PubMed, Embase, Science Direct, HINARI, Scopus, Cochrane Library, and Google Scholar was conducted using relevant search terms. Data were extracted using the Joanna Briggs Institute (JBI) tool. The quality of all selected articles was evaluated using JBI critical appraisal checklist. Data analysis was performed using STATA Version 14 statistical software. Egger’s test and funnel plot were used to evaluate publication bias. Heterogeneity was assessed using Cochran’s chi-squared test and quantified by I2 values. A random-effects model was applied during meta-analysis if heterogeneity was exhibited; otherwise, a fixed-effects model was used.

Results

After reviewing 37,849 articles, 8 studies involving 3,324 housemaids were included for this systematic review and meta-analysis. The pooled prevalence of life time sexual violence among housemaids in Ethiopia was 46.26% (95% CI: 24.69, 67.84). The pooled prevalence was 55.43% (95% CI: 26.38, 84.47) for sexual harassment, 39.03% (95% CI: 14.55, 63.52) for attempted rape, and 18.85% (95% CI: 7.51, 30.19) for rape. Sexual violence is more likely among housemaid who previously lived rural residence (AOR = 2.25; 95% CI: 1.41, 3.60), drinks alcohol (AOR = 2.79 95% CI: 1.02, 4.56), and employer alcohol consumption (AOR = 6.01; 95% CI: 1.10, 32.96).

Conclusion

This study revealed that the prevalence of sexual violence against housemaids in Ethiopia is high. Of the forms of sexual violence against housemaids, sexual harassment is high. Male employers are the vast majority of perpetrators of their housemaids. Thus, concerned stakeholders should develop and implement interventions that could empower housemaids in their struggle toward the elimination of sexual violence, create awareness for men, control and monitor the implementation of legislation and policies, and prompt punishment of the perpetrators.

Systematic review and meta-analysis registration PROSPERO CRD42021160511.
Clinical profiles of treated and untreated adults with hypophosphatasia in the Global HPP Registry
Authors: Dahir Kathryn M. , Seefried Lothar , Kishnani Priya S. , Petryk Anna , Högler Wolfgang , Linglart Agnès , Martos-Moreno Gabriel Ángel , Ozono Keiichi , Fang Shona , Rockman-Greenberg Cheryl
Published: July 2022

Background

The clinical signs and symptoms of hypophosphatasia (HPP) can manifest during any stage of life. The age at which a patient’s symptoms are reported can impact access to targeted treatment with enzyme replacement therapy (asfotase alfa), as this treatment is indicated for patients with pediatric-onset HPP in most countries. As such, many patients reported to have adult-onset HPP typically do not receive treatment. Comparison of the disease in treated and untreated adult patients is confounded by the approved indication. To avoid this confounding factor, a comparison between baseline disease manifestations prominent among treated versus untreated adult patients was limited to those with pediatric-onset HPP using data collected from the Global HPP Registry. The hypothesis was that treated adults will have a greater disease burden at baseline than untreated adults. The analysis of disease manifestations in adults with adult-onset HPP was conducted separately.

Results

A total of 398 adults with HPP were included; 213 with pediatric-onset (114 treated, 99 untreated) and 141 with adult-onset HPP (2 treated and 139 untreated). The treated, pediatric-onset patients were more likely to have a history of pain (prevalence ratio [PR]: 1.3, 95% confidence interval [CI] 1.1, 1.4), skeletal (PR: 1.3, 95% CI 1.1, 1.6), constitutional/metabolic (PR: 1.7, 95% CI 1.3, 2.0), muscular (PR: 1.8, 95% CI 1.4, 2.1) and neurological (PR: 1.7, 95% CI 1.1, 2.3) manifestations of HPP, and also had poorer measures for health-related quality of life, pain, and disability compared with untreated pediatric-onset patients. In patients with adult-onset HPP, the most frequent signs and symptoms were chronic bone pain (52.5%), dental manifestations (42.6%), fatigue (23.4%), recurrent fractures or pseudofractures (22.0%), and generalized body pain (22.0%).

Conclusions

Along with the more classical skeletal signs and symptoms, pain, muscular, and constitutional/metabolic manifestations are common in adults with HPP, regardless of age of disease onset, highlighting a full spectrum of HPP manifestations.
Characterization of hearing-impairment in Generalized Arterial Calcification of Infancy (GACI)
Authors: Theng Elizabeth H. , Brewer Carmen C. , Oheim Ralf , Zalewski Christopher K. , King Kelly A. , Delsmann Maximillian M. , Rolvien Tim , Gafni Rachel I. , Braddock Demetrios T. , Jeffrey Kim H. , Ferreira Carlos R.
Published: July 2022

Background and importance

Hearing loss (HL) has been sporadically described, but not well characterized, in Generalized Arterial Calcification of Infancy (GACI), a rare disease in which pathological calcification typically presents in infancy.

Objectives

This study aims to describe the clinical audiologic and otologic features and potential etiology of hearing impairment in GACI and gain pathophysiological insight from a murine model of GACI.

Design

Cross-sectional cohort study of individuals with GACI. Murine ossicle micromorphology of the ENPP1asj/asj mutant compared to wild-type.

Setting

Clinical research hospital; basic science laboratory.

Participants

Nineteen individuals with GACI who met clinical, biochemical, and genetic criteria for diagnosis.

Main outcomes and measures

Clinical, biochemical, and radiologic features associated with hearing status.

Results

Pure-tone thresholds could be established in 15 (n = 30 ears) of the 19 patients who underwent audiological assessments. The prevalence of HL was 50% (15/30) of ears, with conductive HL in 80% and sensorineural HL in 20%. In terms of patients with HL (n = 8), seven patients had bilateral HL and one patient had unilateral HL. Degree of HL was mild to moderate for 87% of the 15 ears with hearing loss. Of those patients with sufficient pure-tone and middle ear function data, 80% (8/10) had audiometric configurations suggestive of ossicular chain dysfunction (OCD). Recurrent episodes of otitis media (ROM) requiring pressure-equalizing tube placement were common. In patients who underwent cranial CT, 54.5% (6/11) had auricular calcification. Quantitative backscattered electron imaging (qBEI) of murine ossicles supports an OCD component of auditory dysfunction in GACI, suggesting loss of ossicular osteocytes without initiation of bone remodeling.

Conclusions and relevance

Hearing loss is common in GACI; it is most often conductive, and mild to moderate in severity. The etiology of HL is likely multifactorial, involving dysfunction of the ossicular chain and/or recurrent otitis media. Clinically, this study highlights the importance of early audiologic and otologic evaluation in persons with GACI. Novel findings of high rates of OCD and ROM may inform management, and in cases of unclear HL etiology, dedicated temporal bone imaging should be considered.
Global epidemiology of amyloid light-chain amyloidosis
Authors: Kumar Nishant , Zhang Nicole J. , Cherepanov Dasha , Romanus Dorothy , Hughes Michael , Faller Douglas V.
Published: July 2022

Background

Amyloid light-chain (AL) amyloidosis is an ultra-rare disease associated with significant morbidity and mortality. Few studies have examined the global epidemiology of this condition.

Methods

This study estimated the diagnosed incidence and 1-year, 5-year, 10-year, and 20-year period prevalence of AL amyloidosis in 2018 for countries in and near Europe, and in the United States (US), Canada, Brazil, Japan, South Korea, Taiwan, and Russia. A systematic literature review (SLR) was conducted to identify country-specific, age- and gender-specific diagnosed incidence of AL amyloidosis and observed survival data-point inputs for an incidence-to-prevalence model. Extrapolations were used to estimate incidence and prevalence for countries without registry or published epidemiological data.

Results

Of 171 publications identified in the SLR, 10 records met the criteria for data extraction, and two records were included in the final incidence-to-prevalence model. In 2018, an estimated 74,000 AL amyloidosis cases worldwide were diagnosed during the preceding 20 years. The estimated incidence and 20-year prevalence rates were 10 and 51 cases per million population, respectively.

Conclusions

Orphan medicinal product designation criteria of the European Medicines Agency or Electronic Code of Federal Regulations indicate that a disease must not affect > 5 in 10,000 people across the European Union or affect < 200,000 people in the US. This study provides up-to-date epidemiological patterns of AL amyloidosis, which is vital for understanding the burden of the disease, increasing awareness, and to further research and treatment options.
A recurrent single-amino acid deletion (p.Glu500del) in the head domain of ß-cardiac myosin in two unrelated boys presenting with polyhydramnios, congenital axial stiffness and skeletal myopathy
Authors: Bader Ingrid , Freilinger M. , Landauer F. , Waldmüller S. , Mueller-Felber W. , Rauscher C. , Sperl W. , Bittner R. E. , Schmidt W. M. , Mayr J. A.
Published: July 2022

Background

Alterations in the MYH7 gene can cause cardiac and skeletal myopathies. MYH7-related skeletal myopathies are extremely rare, and the vast majority of causal variants in the MYH7 gene are predicted to alter the rod domain of the of ß-cardiac myosin molecule, resulting in distal muscle weakness as the predominant manifestation. Here we describe two unrelated patients harboring an in-frame deletion in the MYH7 gene that is predicted to result in deletion of a single amino acid (p.Glu500del) in the head domain of ß-cardiac myosin. Both patients display an unusual skeletal myopathy phenotype with congenital axial stiffness and muscular hypertonus, but no cardiac involvement.

Results

Clinical data, MRI results and histopathological data were collected retrospectively in two unrelated boys (9 and 3.5 years old). Exome sequencing uncovered the same 3-bp in-frame deletion in exon 15 (c.1498_1500delGAG) of the MYH7 gene of both patients, a mutation which deletes a highly conserved glutamate residue (p.Glu500del) in the relay loop of the head domain of the ß-cardiac myosin heavy chain. The mutation occurred de novo in one patient, whereas mosaicism was detected in blood of the father of the second patient. Both boys presented with an unusual phenotype of prenatal polyhydramnios, congenital axial stiffness and muscular hypertonus. In one patient the phenotype evolved into an axial/proximal skeletal myopathy without distal involvement or cardiomyopathy, whereas the other patient exhibited predominantly stiffness and respiratory involvement. We review and compare all patients described in the literature who possess a variant predicted to alter the p.Glu500 residue in the ß-cardiac myosin head domain, and we provide in-silico analyses of potential effects on polypeptide function.

Conclusion

The data presented here expand the phenotypic spectrum of mutations in the MYH7 gene and have implications for future diagnostics and therapeutic approaches.
Clinical and genetic spectrum of primary ciliary dyskinesia in Chinese patients: a systematic review
Authors: Peng Bo , Gao Yong-hua , Xie Jia-qi , He Xiao-wen , Wang Cong-cong , Xu Jin-fu , Zhang Guo-jun
Published: July 2022

Background

Primary ciliary dyskinesia (PCD) represents a highly heterogenous disorder with extensive clinical and genetic patterns among populations of different geographic location and ethnic origin. However, data about Chinese patients are limited. We aimed to summarize the clinical and genetic spectrum of Chinese PCD patients based on all available literatures.

Methods

We searched Embase, Pubmed, Web of Science and Chinese databases including CNKI, SinoMed and Wanfang from 1981 to 2021, to identify articles reporting patients with PCD in China, which had included information about transmission electron microscopy and/or genetic testing.

Results

A total of 244 Chinese PCD patients in 52 articles were included. Of these patients, the mean age was 13.1 years, and 55 patients (22.5%) were diagnosed with PCD after 18 years old. Compared with patients diagnosed with PCD in childhood or infancy, patients diagnosed with PCD in adulthood had a higher prevalence of chronic wet cough, sinusitis, Pseudomonas aeruginosa (PA) isolation and radiological bronchiectasis as well as worse lung function. 25 PCD-related genes were identified in 142 patients, and DNAH5, DNAH11, CCDC39 and CCDC40 were the most frequently detected mutations. More than half of genetic variants were loss-of-function mutations, and the majority of these variants were seen only once. Correlations between PCD phenotype, genotype and ciliary ultrastructure were also evidenced.

Conclusions

Diagnostic delay and under-recognition of PCD remain a big issue in China, which contributes to progressive lung disease and PA infection indicating worse outcome. Specialist equipment and expertise are urgently required to facilitate the early diagnosis and treatment of PCD.

Trial registry

PROSPERO; No.: CRD42021257804; URL:www.crd.york.ac.uk/prospero/
Neurodevelopmental outcome of Italian preterm ELBW infants: an eleven years single center cohort
Authors: Caporali Camilla , Longo Stefania , Tritto Giovanna , Perotti Gianfranco , Pisoni Camilla , Naboni Cecilia , Gardella Barbara , Spinillo Arsenio , Manzoni Federica , Ghirardello Stefano , Borgatti Renato , Orcesi Simona ,   ELBWI Neurodevelopmental Follow-up Study Group, Olivieri Ivana , La Piana Roberta , Tonduti Davide , Decio Alice , Ravelli Claudia , Olivotto Sara , Ariaudo Giada , Spairani Silvia , Figar Tiziana
Published: July 2022

Background

Preterm extremely low birth weight infants (ELBWi) are known to be at greater risk of developing neuropsychiatric diseases. Identifying early predictors of outcome is essential to refer patients for early intervention. Few studies have investigated neurodevelopmental outcomes in Italian ELBWi. This study aims to describe neurodevelopmental outcome at 24 months of corrected age in an eleven-year single-center cohort of Italian ELBWi and to identify early risk factors for adverse neurodevelopmental outcomes.

Methods

All infants born with birth weight < 1000 g and admitted to the Neonatal Intensive Care Unit of the “Fondazione IRCCS Policlinico San Matteo” hospital in Pavia, Italy, from Jan 1, 2005 to Dec 31, 2015 were eligible for inclusion. At 24 months, Griffiths’ Mental Developmental Scales Extended Revised (GMDS-ER) were administered. Neurodevelopmental outcome was classified as: normal, minor sequelae (minor neurological signs, General Quotient between 76 and 87), major sequelae (cerebral palsy; General Quotient ≤ 75; severe sensory impairment). Univariate and multivariate multinomial logistic regression models were performed to analyze the correlation between neonatal variables and neurodevelopmental outcome.

Results

176 ELBWi were enrolled (mean gestational age 26.52 weeks sd2.23; mean birthweight 777.45 g sd142.89). 67% showed a normal outcome at 24 months, 17% minor sequelae and 16% major sequelae (4.6% cerebral palsy on overall sample). The most frequent major sequela was cognitive impairment (8.52%).

In the entire sample the median score on the Hearing-Speech subscale was lower than the median scores recorded on the other subscales and showed a significantly weaker correlation to each of the other subscales of the GMDS-ER.

Severely abnormal cUS findings (RRR 10.22 p 0.043) and bronchopulmonary dysplasia (RRR 4.36 p 0.008) were independent risk factors for major sequelae and bronchopulmonary dysplasia for minor sequelae (RRR 3.00 p 0.018) on multivariate multinomial logistic regression.

Conclusions

This study showed an improvement in ELBWI survival rate without neurodevelopmental impairment at 24 months compared to previously reported international cohorts. Cognitive impairment was the most frequent major sequela. Median scores on GMDS-ER showed a peculiar developmental profile characterized by a selective deficit in the language domain. Severely abnormal cUS findings and bronchopulmonary dysplasia were confirmed as independent risk factors for major sequelae.
The potential roles of type I interferon activated neutrophils and neutrophil extracellular traps (NETs) in the pathogenesis of primary Sjögren’s syndrome
Authors: Peng Yu , Wu Xunyao , Zhang Shulan , Deng Chuiwen , Zhao Lidan , Wang Mu , Wu Qingjun , Yang Huaxia , Zhou Jiaxin , Peng Linyi , Luo Xuan , Chen Yingying , Wang Anqi , Xiao Qiufeng , Zhang Wen , Zhao Yan , Zeng Xiaofeng , Fei Yunyun
Published: July 2022

Objective

Neutrophils and aberrant NETosis have been implicated in the pathogenesis of diverse autoimmune diseases; however, their roles in primary Sjögren’s syndrome (pSS) remain unclear. We aimed to reveal the potential roles of neutrophils and neutrophil extracellular traps (NETs) in pSS.

Methods

pSS patients were enrolled and NETosis markers were measured in plasma and labial glands using ELISA and immunofluorescence. The gene signatures of neutrophils were assessed by RNA-Seq and RT-PCR. Reactive oxygen species (ROS), mitochondrial ROS (MitoSOX) production, and JC-1 were measured by flow cytometry.

Results

NETosis markers including cell-free DNA (cf-DNA) and myeloperoxidase (MPO) in plasma and labial glands from pSS patients were significantly higher than healthy controls (HCs) and were associated with disease activity. RNA sequencing and RT-qPCR revealed activated type I IFN signaling pathway and higher expression of genes related to type I interferon in pSS neutrophils. Further stimulating with IFN-α 2a in vitro significantly induced ROS production and JC-1 monomer percentage in pSS neutrophils.

Conclusions

Our data suggest the involvement of neutrophils and enhanced NETosis in pSS patients. Further mechanism study in vitro revealed that type I IFN activation in pSS neutrophils led to mitochondrial damage and related ROS production which finally result in the generation of NETs.
Myeloid-derived suppressor cells in hematologic malignancies: two sides of the same coin
Authors: Yu Shunjie , Ren Xiaotong , Li Lijuan
Published: July 2022

Myeloid-derived suppressor cells (MDSCs) are a heterogeneous population of bone marrow cells originating from immature myeloid cells. They exert potent immunosuppressive activity and are closely associated with the development of various diseases such as malignancies, infections, and inflammation. In malignant tumors, MDSCs, one of the most dominant cellular components comprising the tumor microenvironment, play a crucial role in tumor growth, drug resistance, recurrence, and immune escape. Although the role of MDSCs in solid tumors is currently being extensively studied, little is known about their role in hematologic malignancies. In this review, we comprehensively summarized and reviewed the different roles of MDSCs in hematologic malignancies and hematopoietic stem cell transplantation, and finally discussed current targeted therapeutic strategies.Affiliation: Kindly check and confirm the processed affiliations are correct. Amend if any.correct
Regional landscape planning for the innovation of urban planning. Municipal implementation of the city-country pact in Apulia
Authors: Calace Francesca , Paparusso Olga Giovanna
Published: July 2022

The adoption of the European Landscape Convention (ELC) has wrought a profound evolution in the landscape planning discipline, contributing to the integration of the landscape into planning policy. This paper explores the operative significance of including the landscape dimension in local urban planning policies. Adopting an inter-scalar approach, the research analyses the renewal of local urban planning in light of the innovations introduced by the Apulia Regional Landscape Plan (PPTR), an exemplary case of ELC implementation in Italy. One of the spatial strategies indicated in the PPTR provides valuable insights into the complex relationships between the city and the countryside. That strategy, the City-Country Pact, interacts with urban transformations and, as a result, with municipal planning. The paper utilises PPTR indicators to explore how some Apulian municipalities interpret and implement the City-Country Pact. A comparative analysis of case studies outlines the approaches and conditions by which urban planning can effectively renew itself through the ELC landscape approach, influencing plan philosophy, regulations and practices. The paper also points out the current limitations of such processes.
COVID-19 epidemic lockdown-induced changes of cereals and animal protein foods consumption of Iran population: the first nationwide survey
Authors: Nikooyeh Bahareh , Rabiei Samira , Amini Maryam , Ghodsi Delaram , Rasekhi Hamid , Doustmohammadian Azam , Abdollahi Zahra , Minaie Mina , Sadeghi Farzaneh , Neyestani Tirang R.
Published: July 2022

Background

The COVID-19 epidemic has affected diverse issues of life including economy, health and nutrition. This nationwide study was conducted to evaluate the effects of the epidemic lockdown-induced socio-economic changes on animal source proteins and cereals intakes of Iran population for the first time.

Methods

This was a cross-sectional descriptive-analytical study using a web-based electronic self-administered questionnaire. A questionnaire was designed with the aim of detecting any changes in the dietary pattern of the Iranian household following coronavirus epidemic.

Results

A total of 21,290 households participated in the study. Approximately 33%, 24%, 14.2% and 7% of the households had decreased the weekly consumption of red meat, white meat, eggs and rice/bread, respectively, following COVID-19 epidemic. The results of ordered logistic regression showed that the female-headed households, as compared with male-headed ones, were 30% more likely to decrease their weekly consumption of white meat (OR = 1.3, 95% CI: 1.1–1.5). About 8.6% of the households had reduced all three major dietary sources of animal protein. Close to half of the households (46.9%) who decreased their intake of the animal protein sources had increased their intake of rice or bread, too. The major reasons for these changes were decrease of income and job loss.

Conclusion

In conclusion, animal protein food consumption decreased during epidemic lockdown but the amounts of rice and bread, as the major sources of dietary energy, have increased. These changes, if persist long enough, can seriously affect micronutrient status of the whole population. Early nutritional interventions for needy families are warranted.
Scoping review of 30 years of suicide prevention in university students around the world: efficacy, effectiveness, and cost-effectiveness
Authors: Cecchin Hareli Fernanda Garcia , Murta Sheila Giardini , de Macedo Etiene Oliveira Silva , Moore Rafael Alberto
Published: July 2022

A scoping review of systematic reviews was carried out to identify evidence of efficacy, effectiveness, and cost-effectiveness of universal and selective suicide prevention programs among university students worldwide. Five databases were reviewed using terms in English, Spanish, and Portuguese. The following were the inclusion criteria: systematic review or meta-analysis or meta-synthesis, suicide prevention in college students, evaluation of the efficacy, effectiveness and/or cost-effectiveness of interventions, and peer-reviewed studies. The quality of reviews was assessed. The field of study features three decades of publication in high-income countries. The strategy used, the components of the program, and the target audience to which they are delivered interfere with efficacy. In the psychoeducation strategy, the experiential and didactic components are more efficacious in the knowledge about suicide. And the motivational enhancement component promotes greater self-efficacy in suicide prevention. Programs that take a multimodal approach are effective in increasing short-term attitudes related to suicide and reducing rates of completed suicide. The gatekeeper strategy delivered to peer counselors is the most effective one in the outcomes, including short-term and long-term knowledge about suicide and its prevention and self-efficacy in suicide prevention. A greater number of evaluated studies of gatekeeper interventions were identified, indicating a trend in this research field. No review addressed the effects on subgroups that were classified based on sex, racial or sexual minorities, and special (indigenous) populations. Only one study addressed cost-effectiveness, pointing out that the psychoeducation and gatekeeper strategies have relevant net benefit rates, but the gatekeeper strategy has a higher cost–benefit ratio compared to the psychoeducation strategy. The findings indicate that psychoeducation and gatekeeper interventions tend to be more efficacious when they combine education and skills training to intervene in suicidal behavior. The components of the intervention and the target audience to which it is delivered influence efficacy. Multimodal interventions evaluate completed suicide outcomes, but require greater implementation efforts, in terms of human and financial resources and more time for the evaluation.
Experiences of physiotherapists regarding a standard set of measurement instruments to improve quality of care for patients with chronic obstructive pulmonary disease: a mixed methods study
Authors: Verburg Arie C. , Zincken Jessica , Kiers Henri , van Dulmen Simone A. , van der Wees Philip J.
Published: July 2022

Rationale

The quality of physiotherapy care for patients with chronic obstructive pulmonary disease (COPD) can be improved by comparing outcomes of care in practice.

Aim

To evaluate the experiences of physiotherapists implementing a standard set of measurement instruments to measure outcomes and improve the quality of care for patients with COPD.

Methods

This sequential explanatory mixed methods study was performed in two parts. In the quantitative part, a survey of 199 physiotherapists was conducted to evaluate their attitudes and knowledge, as well as the influence of contextual factors (i.e., practice policy and support from colleagues), in the implementation of the standard measurement set. In the qualitative part, 11 physiotherapists participated in individual interviews to elucidate their experiences using a thematical framework.

Results

The survey showed that, on average, 68.4% of the physiotherapists reported having a positive attitude about using the standard set, 85.0% felt they had sufficient knowledge of the measurement instruments, and 84.7% felt supported by practice policy and colleagues. In total, 80.3% of physiotherapists thought the standard set had added value in clinical practice, and 90.3% indicated that the measurement instruments can be valuable for evaluating treatment outcomes. The physiotherapists mentioned several barriers, such as lack of time and the unavailability of the entire standard set of measurement instruments in their practice. Moreover, the physiotherapists indicated that the measurement instruments have added value in providing transparency to policymakers through the anonymized publication of outcomes.

Conclusion

Physiotherapists support the use of the standard set of measurement instruments to improve the quality of physiotherapy treatment for patients with COPD.
Comparative study on pathological changes in sheep and goats experimentally infected with Haemonchus Contortus
Authors: Abosse Jirata Shiferaw , Terefe Getachew , Teshale Bethlehem Mesfin
Published: July 2022

Background

The parasites causes localized and generalized infections to the host depends on the parasite attachment organs, depth of penetration, site of location and worm burdens.

Methods

The experimental study was done between December, 2019 and April, 2020 in the fly-proof experimental animal facility located in the premise of the College of Veterinary Medicine and Agriculture at Bishoftu, Ethiopia. A total of 14 male goat (G1 and G2) and 14 male sheep (G3 and G4) were allotted in to four equal groups. Single dose of 10,000 of infective larvae ofHaemonchus contortus(L3) was orally administered to each animal in G1 and G3. Parameters such as body weight, PCV, haemoglobin, worm count, serum total protein, serum albumin, alkaline phosphatase and aspartate aminotransferase were measured. Moreover, histopathological sections were stained and examined for general changes as well as for changes in specific cells such as tissue eosinophilia and parietal cell population.

Results

The findings show that 1) All infected sheep and goats developed the infection with higher mean worm burden in goats (5590) than sheep (2887) and the difference was significant (P < 0.05); 2) All infected sheep and goats exhibited a progressive anaemia; the level being more severe in goats than in sheep (P < 0.05) with mean PCVs of 13 and 18.6% respectively; 3) While body weight gain was minimal in sheep, goats have lost significant weight compared to pre-infection levels, to control animals or in relation to infected sheep (P < 0.05); 4) analysis of biochemical changes revealed marked reduction in serum total protein and albumin which was much more significant in goats than in sheep (P < 0.05); 5) the abomasum of infected sheep and goats have shown thickening, nodule development, eosinophilic infiltration and damage to parietal cells. Tissue eosinophilia was more prominent in sheep while parietal cell loss was severe in goats.

Conclusion

In conclusion, goats under experimental infection and similar management condition with sheep develop much more severe infection and associated pathology compared to sheep and hence deserve special attention.
An analysis of the recent fire regimes in the Angolan catchment of the Okavango Delta, Central Africa
Authors: van Wilgen Brian W. , de Klerk Helen M. , Stellmes Marion , Archibald Sally
Published: July 2022

Background

This paper presents an analysis of fire regimes in the poorly studied Angolan catchment of the Okavango Delta in Botswana. We used MODIS data to examine the frequency and seasonality of fires over 20 years (from 2000 to 2020) in three dominant vegetation types (miombo woodlands, open woodlands and grasslands, and short closed to open bushlands), and in areas where people were present, and where they were absent.

Results

The median fire return intervals for both open woodlands and grasslands and short bushlands were relatively short (1.9 and 2.2 years respectively). In miombo woodlands, fires were less frequent (median return periods of 4.5 years). Human population density had no discernible effect on the fire return intervals, but about 14% of the miombo woodlands experienced no fires over 20 years. Ongoing shifting cultivation within miombo woodlands has led to structural changes and the introduction of fire into this vegetation type where fires were rare or absent in the past. About 12% of the miombo did not burn during the period examined where people were present, whereas close to 20% of the sites remained unburnt where people were absent. This suggests that people did not change the fire return interval in any of the vegetation types studied, but that they altered the amount of the landscape that is flammable in miombo vegetation. Fires occurred between June and September, with a peak in the late dry season (August and September).

Conclusions

Historical research indicates that late dry-season fires are detrimental to miombo woodlands, and our analysis suggests that degradation in parts of the catchment has led to the introduction of fire to this previously fire-free and fire-sensitive vegetation type. Deforestation of miombo woodlands, and the consequent introduction of fire, is a cause for concern with respect to the ecological stability of the Okavango Delta. Managers should therefore aim to protect the remaining closed-canopy miombo stands from further clearing and to attempt to shift the timing of burns to the early dry season to reduce their intensity.