bluuebunny/crossref_metadata_2025_split huggingface dataset:
Time to Transplant in X-Linked Adrenoleukodystrophy
Authors: Bonkowsky Joshua L. , Wilkes Jacob 
Published: March 2022

Objectives Cerebral X-linked adrenoleukodystrophy (cALD) is an inflammatory demyelination of the brain that can lead to death unless treated by hematopoietic stem cell transplantation. Survival and improved outcomes for cerebral adrenoleukodystrophy are associated with hematopoietic stem cell transplantation at earliest evidence of disease on magnetic resonance imaging (MRI). Our goal was to determine average duration between diagnosis of cALD and hematopoietic stem cell transplantation. Methods This was a retrospective review of data of patients aged 18 years or younger, using a nationwide administrative health care database (Pediatric Health Information System), with an International Classification of Diseases, Tenth Revision ( ICD-10) diagnosis of adrenoleukodystrophy. Time range was October 1, 2015, through June 30, 2021. We determined time to hematopoietic stem cell transplantation by duration between index brain MRI and a code for hematopoietic stem cell transplantation. Results We identified 27 patients with cerebral adrenoleukodystrophy. Total charges for the cohort was $53 million. Time to transplant averaged 97 days. For Hispanic patients, time to transplant was 117 days, compared with 80 days for White, non-Hispanic patients. Comparison of different hospitals showed significant variability in time to hematopoietic stem cell transplantation. Discussion We found that time to hematopoietic stem cell transplantation was >3 months for patients with cerebral adrenoleukodystrophy in the hospitals we evaluated. We noted differences in average time by race/ethnicity and by hospital. Our findings suggest opportunity to reduce time to transplant in cerebral adrenoleukodystrophy.
Synthesis and characterization of new aromatic copoly(ether amide)s bearing 4-trifluoromethylphenyl-2,6-diphenylpyridyl moieties in the main chain
Authors: Yu Yang-Ting , Zhao Jing-Wei , Wei Mei-Hong , Huang Zhi-Hui , Liu Xiao-Ling 
Published: March 2022

A series of new aromatic fluorinated copoly(ether amide)s containing 4-(4-trifluoromethylphenyl)-2,6-diphenylpyridyl moiety with high molecular weight were prepared by a low-temperature solution polycondensation of terephthaloyl chloride with 4-(4-trifluoromethylphenyl)-2,6-bis(4-aminophenyl)pyridine and 1,4-bis(4-amino-2-trifluoromethylphenoxy)benzene with various mole ratios of the two monomers ranged from 20/80 to 80/20. All polymers were amorphous and soluble in common organic solvents such as N-methyl-2-pyrrolidone, N,N-dimethylacetamide and tetrahydrofuran at room temperature or upon heating at 70oC, and could be solution cast into transparent, tough, and flexible films with tensile strengths of 66.9–80.2 MPa, tensile moduli of 1.84–2.03 GPa, and elongations at break of 11.8–20.7%. These new polyamides displayed good thermal stability with gradually increasing of glass temperatures of 259–285oC as an increase of the 4-(4-trifluoromethylphenyl)-2,6-diphenylpyridyl component, the temperature at 10% weight loss of 476–505oC, and the residue of 55–61% at 750oC in nitrogen. They also exhibited low dielectric constants of 3.11–3.38 (1 MHz), low moisture absorption in the range of 1.43–1.75%, and high transparency with an UV–vis absorption cut-off wavelength in the 350–368 nm range.
Diabetes mellitus and the causes of hospitalisation in people with heart failure
Authors: Malik Anam , Garland Ellis , Drozd Michael , Palin Victoria , Giannoudi Marilena , Straw Sam , Jex Nick , Walker Andrew MN , Gierula John , Paton Maria , Witte Klaus K , Kearney Mark T , Levelt Eylem , Cubbon Richard M 
Published: March 2022

Introduction Diabetes mellitus (DM) is associated with increased risk of hospitalisation in people with heart failure and reduced ejection fraction (HFrEF). However, little is known about the causes of these events. Methods Prospective cohort study of 711 people with stable HFrEF. Hospitalisations were categorised by cause as: decompensated heart failure; other cardiovascular; infection or other non-cardiovascular. Rates of hospitalisation and burden of hospitalisation (percentage of follow-up time in hospital) were compared in people with and without DM. Results After a mean follow-up of 4.0 years, 1568 hospitalisations occurred in the entire cohort. DM (present in 32% [ n=224]) was associated with a higher rate (mean 1.07 vs 0.78 per 100 patient-years; p<0.001) and burden (3.4 vs 2.2% of follow-up time; p<0.001) of hospitalisation. Cause-specific analyses revealed increased rate and burden of hospitalisation due to decompensated heart failure, other cardiovascular causes and infection in people with DM, whereas other non-cardiovascular causes were comparable. Infection made the largest contribution to the burden of hospitalisation in people with and without DM. Conclusions In people with HFrEF, DM is associated with a greater burden of hospitalisation due to decompensated heart failure, other cardiovascular events and infection, with infection making the largest contribution.
Venous outflow-targeted coil embolization of direct carotid-cavernous fistulas
Authors: Lee Sang Hyub , Park Hyun , Lee Kwangho , Hwang Soo Hyun , Lee Chul Hee , Kang Dong Ho , Go Kyeong-O 
Published: March 2022

Background Coil embolization is the mainstay treatment for carotid-cavernous fistulas (CCFs). However, few studies have reported entire occlusion of engorged veins to interrupt venous outflow. We report our experience with venous outflow-targeted coil embolization of direct CCFs. Methods We retrospectively reviewed all the patients diagnosed with direct CCFs treated with venous outflow-targeted coil embolization between November 2013 and February 2020. Venous outflow-targeted coil embolization of the CCFs was performed as follows. First, transarterial stent-assisted coil embolization of CCFs was performed. If the venous outflow to the engorged veins persisted after transarterial stent-assisted coil embolization, entire occlusion of the engorged veins and additional coil packing within the cavernous sinus were performed to interrupt the venous outflow. Results Ten patients had undergone venous outflow-targeted coil embolization, 6 women (60%) and 4 men (40%). Transfemoral cerebral angiography showed high-flow, direct CCFs in all the patients. Venous outflow occurred through the superior ophthalmic vein (SOV) in all the patients and was completely interrupted by the entire occlusion of the engorged veins with fibered coils. Three patients (30%) had undergone additional treatment in a supplementary manner because of recurrent symptoms (chemosis in 1 patient, faint tinnitus in 2 patients) in the early postprocedural period (1 to 4 weeks). All the symptoms were resolved on follow-up. No additional recurrence was found during follow-up (1–75 months). No peri-procedural complications were encountered. Conclusions Venous outflow-targeted coil embolization of CCFs would be a safe and effective treatment method.
Topical Administration of Carvacrol Improves Healing in Nasal Septal Perforation: An Experimental Animal Study
Authors: Çengel Kurnaz Senem , Kuruca Nilüfer , Güvenç Dilek , Kaya Muhammed Taha , Güvenç Tolga 
Published: March 2022

Background Clinicians are exploring alternative treatments for nasal septum perforation since surgical treatment is challenging. Objective The effects of topical carvacrol on the healing of nasal septal perforation were investigated in an animal model. Method Twenty-one male New Zealand rabbits were randomly divided into three equal groups. A 5-mm circular biopsy punch was used to perforate the nasal septum behind the columella. For 14 days, bilateral gelatin sponges impregnated with carvacrol in olive oil in group 1, and only olive oil in group 2 were placed. Perforation only was performed in the control group. Animals were then sacrificed, and their nasal septums were removed. The closure of the perforation was measured, and samples were examined histopathologically. MMP-9 reactivity was evaluated using the immunoperoxidase technique. Histopathologic parameters were scored as 0 = none, 1 = mild, 2 = moderate, and 3 = strong. Results The septum perforation closure in the carvacrol group was statistically significant compared with the other groups (p < 0.001). Cartilage regeneration, connective tissue density, and MMP-9 immunoreactivity were significantly higher in the carvacrol group (p = 0.020, p = 0.009, and p = 0.008, respectively). Conclusion Topically administered carvacrol enhances wound healing in rabbit nasal septum perforation. It accelerated perforation closure by increasing cartilage regeneration, connective tissue, and MMP-9 expression.
Cellular therapies for the treatment and prevention of SARS-CoV-2 infection
Authors: Conway Susan R. , Keller Michael D. , Bollard Catherine M. 
Published: March 2022

Abstract
Patients with blood disorders who are immune suppressed are at increased risk for infection with severe acute respiratory syndrome coronavirus 2. Sequelae of infection can include severe respiratory disease and/or prolonged duration of viral shedding. Cellular therapies may protect these vulnerable patients by providing antiviral cellular immunity and/or immune modulation. In this recent review of the field, phase 1/2 trials evaluating adoptive cellular therapies with virus-specific T cells or natural killer cells are described along with trials evaluating the safety, feasibility, and preliminary efficacy of immune modulating cellular therapies including regulatory T cells and mesenchymal stromal cells. In addition, the immunologic basis for these therapies is discussed.
Identification of novel γ-globin inducers among all potential erythroid druggable targets
Authors: Yu Lei , Myers Greggory , Schneider Emily , Wang Yu , Mathews Raven , Lim Kim Chew , Siemieniak David , Tang Vi , Ginsburg David , Balbin-Cuesta Ginette , Singh Sharon A. , Phuwakanjana Pongpon , Jearawiriyapaisarn Natee , Khoriaty Rami , Engel James Douglas 
Published: March 2022

Abstract
Human γ-globin is predominantly expressed in fetal liver erythroid cells during gestation from 2 nearly identical genes, HBG1 and HBG2, that are both perinatally silenced. Reactivation of these fetal genes in adult red blood cells can ameliorate many symptoms associated with the inherited β-globinopathies, sickle cell disease, and Cooley anemia. Although promising genetic strategies to reactivate the γ-globin genes to treat these diseases have been explored, there are significant barriers to their effective implementation worldwide; alternatively, pharmacological induction of γ-globin synthesis could readily reach the majority of affected individuals. In this study, we generated a CRISPR knockout library that targeted all erythroid genes for which prospective or actual therapeutic compounds already exist. By probing this library for genes that repress fetal hemoglobin (HbF), we identified several novel, potentially druggable, γ-globin repressors, including VHL and PTEN. We demonstrate that deletion of VHL induces HbF through activation of the HIF1α pathway and that deletion of PTEN induces HbF through AKT pathway stimulation. Finally, we show that small-molecule inhibitors of PTEN and EZH induce HbF in both healthy and β-thalassemic human primary erythroid cells.
Comparing clinical outcomes in patients with diabetes undergoing coronary artery bypass graft and percutaneous coronary intervention in real world practice in Iranian population
Authors: Arabi Arezo , Naghshtabrizi Behshad , Baradaran Hamid Reza , Moradi Yousef , Asadi-Lari Mohsen , Mehrakizadeh Ali 
Published: March 2022

Abstract
Background
Coronary artery bypass graft (CABG) is generally regarded as one the treatment options for coronary artery disease (CAD) in patients with diabetes. In recent years, with the advent of drug-eluting stents (DES), percutaneous coronary intervention (PCI) was introduced as a suitable alternative for CABG. The aim of this study was to compare the incidence of major adverse cardiac and cerebrovascular event (MACCE) during mid-term period in patients with diabetes treated with 2 revascularization strategies.

Methods
This historical cohort study was conducted on 750 consecutives patients with diabetes in a single cardiovascular center from July 2009 to March 2012 in Iran. We included previously known case of DM treated with antidiabetic medications (with or without end organ damage) and patient with preoperational evaluation FBS test > 126 (not on the day of the surgery) who were revascularized by 2 strategies. We excluded those patients whose follow-up was not possible.

Results
Finally, out of 697 eligible patients, 355 patients underwent a CABG and 342 underwent a PCI: 53 patients were lost to follow-up (27 in CABG and 26 in PCI groups). The mean follow-up time was 900.68 ± 462.03 days in the CABG and 782.60 ± 399.05 in PCI groups. There were 17 (9.13%) cardiac deaths in the CABG group and 8 (4.45%) in the PCI group; this difference was not significant (P = .11). There was 14 (7.58%) cerebrovascular accident in the CABG group and 4 (2.31%) in the PCI group; this difference was significant (P = .04). Moreover, the frequency of the target vessel revascularization in the CABG and PCI groups was 6 (3.32%) and 31 (17.11%) (P < .001), respectively. Myocardial infarction in the CABG group was 5 (2.77%) and 14 (7.86%) in the PCI group (P = .009). Finally, the frequency of MACCE in the CABG and PCI groups was 41(20.70%) and 47(24.16%) respectively; this difference was not statistically significant (P = .195).

Conclusion
Patients with CABG in this study experienced more CVA, while the frequency of TVR and non-fatal MI was higher in the PCI arm.
Fall determinants in hospitalised older patients: a nested case control design - incidence, extrinsic and intrinsic risk in Malaysia
Authors: Lee Fatt Soon , Sararaks Sondi , Yau Weng Keong , Ang Zen Yang , Jailani Anis-Syakira , Abd Karim Zulkarnain , Naing Lin , Krishnan Thillainathan , Chu Ai Reen , Junus Suria , Ahmad Mohd Shahril , Sapiee Norhayaty , Veloo Vicneas Wary , Manoharan Sangetavani , A. Hamid Maimunah 
Published: March 2022

Abstract
Background
The older person is at greater risk of falls due to multiple intrinsic and extrinsic factors. This is compounded when the elderly is admitted to hospitals, as they are acutely ill and placed in an unfamiliar environment. Delirium and polypharmacy further complicate these problems. As falls reflect quality of care with potential for grave outcomes, this study aimed to identify the extent and risk of falls in public hospitals.

Methods
We conducted a nested case control study in 12 public hospitals in Malaysia. In the cohort section, we screened all inpatients 60 years of age and above daily until discharge, or the end of the study period. Daily, we identified those who fell, inclusive of near falls, in the preceding 24 h. Our enumerators interviewed patients on experience of fall, and supplemented data from the nurses and caregivers. For each case, ten controls were chosen.

Results
The incidence of falls/near falls was 1.0 per 1000 patient days (95% CI: 0.9, 1.1). Intrinsic risk factors found to be significant included patients who were not from a nursing home or not cared for by a domestic helper prior to admission, had prior history of indoor fall either in home or hospital, had four or more clinical diagnoses or exited from the bed on the weak side. Significant extrinsic factors were the absence of transfer bar in toilet, call bells, light switches or walking aids that were not within reach, as well as not having a walking aid. Non-sturdy chair was associated with lesser falls than when sturdy chairs with armrests were present.

Conclusion
Querying patients for falls produced better results than incident reporting. Several intrinsic factors such as history of indoor or in-hospital fall, having four or more clinical diagnoses or exiting from weaker side and residence history may help to identify those at higher risk. Addressing significant extrinsic factors such as transfer bars and the identification of switches may help in reducing falls risk in hospitals.

Trial registration
This study was registered in National Medical Research Register of Malaysia (NMRR-07-772-1044; date 26/05/2008) with Ethics Approval from Medical Research and Ethics Committee (MREC: MRG-07-LOI-HSR-1).
A trespasser from a foreign land? A case report of primary mucosal leishmaniasis
Authors: Fleissig Yoram , Dan-Gur Mary , Michael-Gayego Ayelet , Maly Alexander , Tabib Rami , Jaffe Charles L. , Korem Maya 
Published: March 2022

Abstract
Background
We report a clinically challenging and unusual case of L. donovani oral mucosal leishmaniasis.

Case presentation
Israeli resident with a former travel to central and North Africa, with no documented or prior cutaneous lesions presented with oral lesions of the maxillary gingiva and the upper lip. A delay in diagnosis and treatment have led to progression of the maxillary gingival lesions towards the hard palatal and the soft palate that could have potentially compromised the upper airway.

Conclusions
This case highlights the importance of early diagnosis of leishmaniasis in patients with oral lesions and the laboratory workup necessary to appropriately characterize and treat the disease.
Newly arrived Asian-born gay men in Australia: exploring men’s HIV knowledge, attitudes, prevention strategies and facilitators toward safer sexual practices
Authors: Phillips Tiffany R. , Medland Nicholas , Chow Eric P. F. , Maddaford Kate , Wigan Rebecca , Fairley Christopher K. , Bilardi Jade E. , Ong Jason J. 
Published: March 2022

Abstract
Background
Asian-born gay, bisexual and other men who have sex with men (gbMSM) newly arrived in Australia are more than four times as likely than their Australian-born counterparts to be diagnosed with incident HIV. Our aim was to explore experiences of Asian-born gbMSM newly arrived in Australia and attending a sexual health centre with regards to their knowledge of and preference for HIV prevention strategies.

Results
Twenty-four gbMSM aged 20–30 years attending Melbourne Sexual Health Centre who were born in Asia and arrived in Australia in the preceding four years, participated in semi-structured face-to-face interviews from 8th May 2019 and 23rd December 2019. Men were excluded if they were living with HIV. Interviews were recorded, transcribed verbatim and analysed thematically. Men reported little knowledge of HIV prevention strategies outside of condom use prior to coming to Australia. Although participants reported basic knowledge of HIV transmission and treatment, exposure to sexual identity and HIV-related stigma in their countries of birth meant they imagined a HIV diagnosis would be devastating. Most relied on condoms to stay HIV negative however their consistency of use varied. Seven men were on pre-exposure prophylaxis (PrEP); all but one started PrEP after coming to Australia. Many indicated interest in PrEP but described it as too expensive given they do not have access to government-subsidized healthcare. Sexual health counselling and connections with LGBTQI community groups appeared to facilitate PrEP and consistent condom use.

Conclusions
Asian-born gbMSM newly-arrived to Australia may have limited knowledge of HIV prevention strategies aside from condom use. Increased connections with sexual health services and LGBTQI communities may facilitate more effective HIV prevention strategies.
A sharp decrease in reported non-COVID-19 notifiable infectious diseases during the first wave of the COVID-19 epidemic in the Rotterdam region, the Netherlands: a descriptive study
Authors: van Deursen Babette , Hagenaars Margot , Meima Abraham , van Asten Liselotte , Richardus Jan Hendrik , Fanoy Ewout , Voeten Helene 
Published: March 2022

Abstract
Background
The Public Health Services in the Rotterdam region, the Netherlands, observed a substantial decrease of non-COVID-19 notifiable infectious diseases and institutional outbreaks during the first wave of the COVID-19 epidemic. We describe this change from mid-March to mid-October 2020 by comparing with the pre-COVID-19 situation.

Methods
All cases of notifiable diseases and institutional outbreaks reported to the Public Health Services Rotterdam-Rijnmond between 1st January and mid-October 2020 were included. Seven-day moving averages and cumulative cases were plotted against time and compared to those of 2017–2019. Additionally, Google mobility transit data of the region were plotted, as proxy for social distancing.

Results
Respiratory, gastrointestinal, and travel-related notifiable diseases were reported 65% less often during the first wave of the COVID-19 epidemic than in the same weeks in 2017–2019. Reports of institutional outbreaks were also lower after the initially imposed social distancing measures; however, the numbers rebounded when measures were partially lifted.

Conclusions
Interpersonal distancing and hygiene measures imposed nationally against COVID-19 were in place between mid-March and mid-October, which most likely reduced transmission of other infectious diseases, and may thus have resulted in lower notifications of infectious diseases and outbreaks. This phenomenon opens future study options considering the effect of local outbreak control measures on a wide range of non-COVID-19 diseases. Targeted, tailored, appropriate and acceptable hygiene and distancing measures, specifically for vulnerable groups and institutions, should be devised and their effect investigated.
Tranexamic acid for reducing blood loss following vaginal delivery: a double-blind randomized controlled trial
Authors: Igboke Francis Nwabueze , Obi Vitus Okwuchukwu , Dimejesi Benedict Ikechukwu , Lawani Lucky Osaheni 
Published: March 2022

Abstract
Background
Postpartum haemorrhage (PPH) is a major cause of maternal morbidity and mortality worldwide. Tranexamic acid (TXA) is a useful drug for prevention of PPH and merits evaluation in Nigeria, where PPH is the leading cause of maternal death (25%) and severe maternal morbidity. This study evaluates the efficacy of TXA in reducing blood loss following vaginal delivery.

Methods
This was a double-blind randomized placebo-controlled study on the efficacy and safety of intravenous TXA in reducing blood loss in women undergoing vaginal delivery in a tertiary hospital. Data analysis was conducted with IBM SPSS software (version 20, Chicago II, USA). P-value < 0.05 was considered statistically significant.

Results
The mean estimated blood loss was lower in TXA compared with the placebo group. (174.87 ± 119.83 ml versus 341.07 ± 67.97 ml respectively; P < 0.0001). PPH (blood loss > 500 ml) was 5.13% in the study arm compared to the control arm 7.14%- risk ratio (RR) 0.71; 95% CI: 0.38–1.79, p = 0.5956]. Additional uterotonics was required more in the control group compared to the treatment group 14(16.67%) versus 3(3.85%), p-value= 0.007. There were no major complications noticed in the treatment group.

Conclusion
This study demonstrated that intravenous administration of TXA reduced blood loss following vaginal delivery. It also reduced the need for additional uterotonics. However, blood loss greater than 500 was not significantly reduced.

Trial registration
This trial was registered retrospectively.
Pan African Clinical Trial Registry: PACTR202010828881019 on 12/10/2020.