bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment of Facial Asymmetry Using Distraction Osteogenesis in a Mandible First Approach
Authors: Rachmiel Adi , Capucha Tal , Ginini Jiriys George , Emodi Omri , Aizenbud Dror , Shilo Dekel 
Published: September 2023

Background:
Facial asymmetry includes several etiologies, among them trauma to the condylar area during early childhood and congenital malformations such as hemifacial microsomia. This article describes the management of facial asymmetry in adolescents and young adults using a mandible first approach by distraction osteogenesis, followed by maxillary Le-Fort I as a second stage.

Methods:
Eighteen patients 14–25 years of age presented with unilateral hypoplasia of the jaws which manifested clinically by deviation of the chin and canting of the occlusal plane. Etiology included hemifacial microsomia and trauma injuries at early childhood.
All patients underwent orthodontic treatment and two phases of surgical treatment. Surgical treatment included unilateral mandibular distraction followed by Le-Fort I osteotomy for alignment of the maxilla. Additional bone graft in the affected side and sliding genioplasty were done as required.

Results:
Marked ramal elongation of 18.94 mm concomitant with mandibular forward traction of 12.5 mm was noted while achieving symmetry. In all cases, the maxilla was centered to the midline in proper occlusion. Post distraction, posteroanterior cephalometric radiographs demonstrated elongation of the affected ramus, improvement in facial symmetry, and correction of the occlusal canting. Relapse was minimal based on long-term follow-ups of 47.4 months.

Conclusions:
The two-stage surgical approach that includes elongation of the mandible as a first stage followed by adaptation of the maxilla is useful in correcting facial asymmetry. Using this protocol at the correct age (14–18) is very stable, as demonstrated by our results, yet one should always remember the transverse deficiency in the gonial angle requires additional bone grafting or patient specific implants.
Sorafenib induces ferroptosis by promoting TRIM54-mediated FSP1 ubiquitination and degradation in hepatocellular carcinoma
Authors: Liu Man-ru , Shi Ce , Song Qiu-ya , Kang Meng-jie , Jiang Xin , Liu Hui , Pei Dong-sheng 
Published: September 2023

Background:
Ferroptosis is a unique form of regulated cell death that provided a new opportunity for cancer therapy. Ferroptosis suppressor protein 1 (FSP1) is a key regulator in the NAD(P)H/FSP1/CoQ10 antioxidant system, which sever as an oxide redox enzyme to scavenge harmful lipid hydroperoxides and escape from ferroptosis in cells. This study aimed to investigate the role of FSP1 on sorafenib-induced ferroptosis and disclosed the underlying mechanisms.

Methods:
Cell viability, malondialdehyde (MDA), glutathione (GSH), and lipid reactive oxygen species levels were assessed using indicated assay kits. The levels of FSP1 and glutathione peroxidase 4 (GPX4) in the patients with HCC were analyzed based on the database. Western blot and quantitative real-time PCR were performed to detect the protein and mRNA expression. Co-immunoprecipitation was applied to detect the interaction between proteins. Tumor xenograft experiments were used to evaluate whether overexpression of FSP1-inhibited sorafenib-induced ferroptosis in vivo.

Results:
We verified that sorafenib-induced ferroptosis in HCC. Furthermore, we found that sorafenib decreased the protein level of FSP1, and knockdown FSP1 rendered HCC cells susceptible to sorafenib-induced ferroptosis. Co-immunoprecipitation and ubiquitination assays showed that sorafenib accelerated the TRIM54-mediated FSP1 ubiquitination and degradation. Sorafenib-induced ferroptosis was abrogated by TRIM54 suppression. Mechanically, sorafenib-promoted TRIM54 ubiquitinated and degraded FSP1 by means of the ERK pathway. Moreover, FSP1 enhanced tumor development and decreased HCC cellular susceptibility to sorafenib in vivo.

Conclusions:
Sorafenib facilitated the TRIM54-mediated FSP1 ubiquitination through the ERK pathway, thereby inducing ferroptosis in HCC cells.
MELD, MELD 3.0, versus Child score to predict mortality after acute variceal hemorrhage: A multicenter US cohort
Authors: Buckholz Adam , Wong Rochelle , Curry Michael P. , Baffy Gyorgy , Chak Eric , Rustagi Tarun , Mohanty Arpan , Fortune Brett E. 
Published: September 2023

Background:
Acute variceal hemorrhage is a major decompensating event in patients with cirrhosis and is associated with high 6-week mortality risk. Many prognostic models based on clinical and laboratory parameters have been developed to risk stratify patients on index bleeding presentation, including those based on the Model for End-Stage Liver Disease (MELD) and Child-Turcotte-Pugh (CTP). However, consensus on model performance remains unclear.

Methods:
Using a large US multicenter cohort of hospitalized patients with cirrhosis who presented with acute variceal hemorrhage, this study evaluates, recalibrates, and compares liver severity index-based models, including the more recent MELD 3.0 model, to investigate their predictive performance on 6-week mortality. Models were also recalibrated and externally validated using additional external centers.

Results:
All recalibrated MELD-based and CTP-based models had excellent discrimination to identify patients at higher risk for 6-week mortality on initial presentation. The recalibrated CTP score model maintained the best calibration and performance within the validation cohort. Patients with low CTP scores (Class A, score 5–6) were strongly associated with < 5% mortality, while high CTP score (Class C, score > 9) were associated with > 20% mortality.

Conclusion:
Use of liver severity index-based models accurately predict 6-week mortality risk for patients admitted to the hospital with acute variceal hemorrhage and supports the utilization of these models in future clinical trials as well as their use in clinical practice.
Effect of 24-h blood pressure dysregulations and reduced ocular perfusion pressure in open-angle glaucoma progression
Authors: Melgarejo Jesus D. , Van Eijgen Jan , Wei Dongmei , Maestre Gladys E. , Al-Aswad Lama A. , Liao Chia-Te , Mena Luis J. , Vanassche Thomas , Janssens Stefan , Verhamme Peter , Zhang Zhen-Yu , Van Keer Karel , Stalmans Ingeborg 
Published: September 2023

Background:
Low ocular perfusion pressure (OPP), which depends on the mean arterial pressure (MAP) and intraocular pressure (IOP), is associated with glaucoma. We studied 24-h MAP dysregulations and OPP in relation to the progression of glaucoma damage.

Methods:
We retrospectively analyzed 155 normal-tension glaucoma (NTG) and 110 primary open-angle glaucoma (POAG) patients aged 18 years old followed at the University Hospital Leuven with repeated visual field tests (n = 7000 measures, including both eyes) who underwent 24-h ambulatory blood pressure monitoring. Twenty-four-hour MAP dysregulations were variability independent of the mean (VIM), and the five lowest dips in MAP readings over 24 h. OPP was the difference between 2/3 of the MAP and IOP. Glaucoma progression was the deterioration of the visual field, expressed as decibel (dB) changes in mean deviation analyzed by applying multivariable linear mixed regression models.

Results:
The mean age was 68 years (53% were women). High 24-h VIMmap was associated with glaucoma progression in POAG (P < 0.001) independently of the 24-h MAP level. The estimated changes in mean deviation in relation to dip MAP measures ranged from −2.84 dB [95% confidence interval (CI) −4.12 to −1.57] to −2.16 dB (95% CI −3.46 to −0.85) in POAG. Reduced OPP along with high variability and dips in MAP resulted in worse mean deviation deterioration.

Conclusion:
The progression of glaucoma damage associates with repetitive and extreme dips in MAP caused by high variability in MAP throughout 24 h. This progression exacerbates if 24-h MAP dysregulations occur along with reduced OPP.
Acquired bleeding disorders secondary to immune checkpoint inhibitors: a case report and systematic literature review
Authors: Archibald William J. , Kouides Peter A. , Refaai Majed A. , Lachant Neil A. 
Published: September 2023

Acquired bleeding disorders because of an autoimmune phenomenon are rare events. Acquired von Willebrand disease (aVWD) has been estimated as having a prevalence of 400 per million in the general population. Acquired hemophilia A (AHA), the most common of the acquired hemophilias, has an estimated incidence of 1.3–1.5 cases per million per year. Immune checkpoint inhibitors (ICI) targeting PD-1, PD-L1, and CTLA-4 are being used with increasing frequency for hematologic and oncologic disorders. Acquired hemophilias and aVWD have been reported with the use of ICI therapy.
We performed a systematic review of the literature to identify cases of acquired bleeding disorders with ICI therapy and contribute our own institution's experience with a case of AHA after pembrolizumab therapy. Six cases of AHA, one case of aVWD, and one case of factor V inhibitor were identified in the literature. Inhibitors were successfully eradicated in five of the eight cases identified. We propose that a centralized registry, possibly through the Scientific and Standardization Subcommittee on Plasma Coagulation Inhibitors through the International Society on Thrombosis and Hemostasis (ISTH), be developed to record treatment and outcomes of this rare ICI complication in order to prognosticate risk and better understand optimal treatment strategies.
Multicenter validation of FIB-6 as a novel machine learning non-invasive score to rule out liver cirrhosis in biopsy-proven MAFLD
Authors: Anushiravani Amir , Alswat Khalid , Dalekos George N , Zachou Kalliopi , Örmeci Necati , Al-Busafi Said , Abdo Ayman , Sanai Faisal , Mikhail Nabiel NH , Soliman Riham , Shiha Gamal 
Published: September 2023

Background and aimsWe previously developed and validated a non-invasive diagnostic index based on routine laboratory parameters for predicting the stage of hepatic fibrosis in patients with chronic hepatitis C (CHC) called FIB-6 through machine learning with random forests algorithm using retrospective data of 7238 biopsy-proven CHC patients. Our aim is to validate this novel score in patients with metabolic dysfunction-associated fatty liver disease (MAFLD).MethodPerformance of the new score was externally validated in cohorts from one site in Egypt (n = 674) and in 5 different countries (n = 1798) in Iran, KSA, Greece, Turkey and Oman. Experienced pathologists using METAVIR scoring system scored the biopsy samples. Results were compared with FIB-4, APRI, and AAR.ResultsA total of 2472 and their liver biopsy results were included, using the optimal cutoffs of FIB-6 indicated a reliable performance in diagnosing cirrhosis, severe fibrosis, and significant fibrosis with sensitivity = 70.5%, specificity = 62.9%. PPV = 15.0% and NPV = 95.8% for diagnosis of cirrhosis. For diagnosis of severe fibrosis (F3 and F4), the results were 86.5%, 24.0%, 15.1% and 91.9% respectively, while for diagnosis of significant fibrosis (F2, F3 and F4), the results were 87.0%, 16.4%, 24.8% and 80.0%). Comparing the results of FIB-6 rule-out cutoffs with those of FIB-4, APRI, and AAR, FIB-6 had the highest sensitivity and NPV (97.0% and 94.7%), as compared to FIB-4 (71.6% and 94.7%), APRI (36.4% and 90.7%), and AAR (61.2% and 90.9%).ConclusionFIB-6 score is an accurate, simple, NIT for ruling out advanced fibrosis and liver cirrhosis in patients with MAFLD.
Predictors of 30-day mortality in patients diagnosed with hepatic encephalopathy on admission to the emergency department
Authors: Yuksek Ali , Acehan Selen , Satar Salim , Gulen Muge , Balcik Muhammet , Sevdimbas Sarper , Ince Cagdas , Koca Ahmet Naci , Tas Adnan 
Published: September 2023

Background
The aim of this study is to compare the laboratory findings and disease severity scores of patients diagnosed with hepatic encephalopathy (HE) in the emergency department (ED) to predict 30-day mortality.

Method
The patients over 18 years old and diagnosed HE in the ED of a tertiary hospital were included in the study. Demographic and clinical characteristics, laboratory parameters, predisposing causes and outcomes of the patients included in the study were recorded in the data form. Severity of liver disease was assessed by Child Pugh Score (CPS), End-stage liver disease model (MELD), MELD-Na and MELD-Lactate scores.

Results
Two hundred fifty-four patients diagnosed with HE were included in the study. 59.1% of the patients were male. The mean age of the patients was 65.2 ± 12.6 years. The mortality rate of the patients was 47.2%. When the receiver operating characteristic (ROC) analysis, which determines the predictive properties of laboratory parameters and disease severity scores, was examined, the area under curve value of the MELD-Lactate score (0.858 95% CI 0.812–0.904, P < 0.001) was the highest. Binary logistic regression analysis for the estimation of patients’ 30-day mortality showed that CPS and MELD-Lactate scores and blood ammonia and B-type natriuretic peptide levels were independent predictors of mortality.

Conclusion
According to the study data, MELD-Lactate and BNP levels in patients diagnosed with HE in the ED may help the clinician in the prediction of 30-day mortality in the early period.
Mixed donor chimerism following stem cell transplantation for sickle cell disease
Authors: Shah Niketa C. , Rangarajan Hemalatha G. , Ngwube Alexander , Shenoy Shalini 
Published: September 2023

Sickle cell disease is a debilitating hemoglobinopathy with high morbidity and mortality. Hematopoietic stem cell transplantation (HCT) is curative, but the presence of mixed donor/recipient chimerism post-HCT raises concerns about disease control long-term. Mixed donor/recipient chimerism is reported in significant numbers even after aggressive HCT conditioning regimens. Post-HCT, adequacy of donor erythropoiesis is crucial for disease control. This review explores the relationship between mixed donor/recipient chimerism and outcomes post-HCT.
Serial chimerism analysis in lineage specific manner in erythroid or myeloid cells post-HCT predicts for disease control and HCT success. Adequate and stable donor-derived erythropoiesis is essential for reversing SCD manifestations. Myeloid lineage chimerism mirrors erythropoiesis is commercially available, and a reliable indicator of adequacy. Using this tool, the minimum threshold of donor chimerism is required to prevent SCD-related complications and maintain sickle hemoglobin less than 50% is approximately 20–25% even when a donor has Hb S trait. Curative interventions should, at a minimum, meet this goal long-term.
Achieving a balance between successful engraftment while minimizing toxicity is important in patients vulnerable because of age or preexisting morbidity and is the objective of recent clinical trials. As HCT and gene therapies evolve, efficient long-term follow-up that includes durability assessment of mixed donor/recipient chimerism will be crucial.
Results of laminoplasty versus laminectomy and posterior fusion for multilevel cervical spondylotic myelopathy
Authors: Nguyen Trong Yen , Nguyen Khac Hieu , Tran Quang Dung , Pham Quang Anh , Lam Viet Anh 
Published: September 2023

Background:
The authors compared the clinical, MR, and clinical outcomes for patients with multilevel cervical spondylotic myelopathy (MCSM) who underwent laminoplasty (LP) versus laminectomy with fusion (LPSF).

Materials and methods:
The authors evaluated 65 patients with MCSM (2019–2021) with 31 undergoing LP versus 34 having LPSF. Variables studied included JOA scores, T2W MR hyperintense cord signals, preoperative lordosis, number of stenotic levels, and neurological outcomes.

Results:
Both groups showed similar preoperative JOA scores, number of stenotic levels, T2-weighted MR hyperintense cord signals, and nearly identifcal 12-month postoperative outcomes (good for 83.9 in the LP group vs. 85.3% in the LPSF group). Differences included: higher preoperative visual analog scale in the LPSF versus LP group, plus the LPSF patients significantly less preoperative cervical lordosis versus LP patients.

Conclusion:
LP and LPSF used to treat MCSM resulted in similar clinical improvement and 1-year postoperative outcomes. However, our recommendation would be for patients with neck pain, instability, and/or cervical kyphosis to undergo LPSF.
Recurrent uveitis in a patient with CREST syndrome: a case report
Authors: Khalayli Naram , Hodifa Yara , Tarcha Raghad , Hodaifa Amr , Kudsi Maysoun 
Published: September 2023

Introduction:
Systemic sclerosis is a connective tissue condition presented with clinical manifestations, including ocular involvement in a small percentage. A few cases of uveitis were described in the literature, especially in CREST syndrome.

Case presentation:
The authors reported a case of a 48-year-old male with a blurred vision in his left eye that diagnosed with recurrent uveitis in the setting of CREST syndrome, treated with prednisolone, methotrexate, and golimumab, with improvement.

Discussion:
Ocular manifestations of systemic sclerosis include palpebral alterations, keratoconjunctivitis, sicca syndrome, cataracts, pinguecula, and blepharitis. To our knowledge, this is the fifth case of uveitis in a setting of CREST syndrome in the literature, and the first one in sex involvement, as the previous cases were females, and in its treatment by golimumab, an anti-tumor necrosis factor inhibitor.

Conclusion:
Although this association is low, we believe that it should be taken into consideration when treating these situations to obtain better treatment results. Collaboration between rheumatologists and ophthalmologists is necessary in deciding on treatment.
Misdiagnosed periappendiceal and intestinal tuberculosis during the COVID-19 pandemic
Authors: Cui Zhaoyang , Le Yi , Liu Hu , Feng Linjing , Zhang Shaogeng 
Published: September 2023

Introduction and importance:
The coronavirus disease 2019 (COVID-19) was first reported in Wuhan, Hubei Province, China. It mainly involves the respiratory system, causing fever, cough, chest tightness, and other symptoms. However, when combined with other common or rare diseases, such as appendicitis and intestinal tuberculosis (TB), it can cause other systemic lesions, thus making the original disease lose its specific clinical manifestations. This case highlights the importance of early identification and clinical precision medicine diagnosis and treatment.

Case presentation:
A young woman presented with intermittent pain and discomfort in the right lower quadrant. Ultrasonography suggested appendicitis with a peripheral abscess. The nucleic acid test of COVID-19 was positive, and the chest computed tomography scan showed pulmonary involvement. She was sent for surgery. Postoperative body temperature increased regularly, and the TB T-cell test was positive.

Clinical discussion:
Multiple infections caused by common bacteria, pandemic virus, and specific mycobacterium TB cause a series of nonspecific clinical manifestations, which brings challenges to clinical diagnosis and treatment. Therefore, when facing a complex infection case, the authors should consider the possibility of multiple infections and give targeted treatment for the pathogens.

Conclusions:
During the epidemic of COVID-19, the incidence of intestinal TB is relatively low, which is easy to be overlooked and misdiagnosed, especially in the case of appendicitis. Therefore, clinicians must be highly vigilant in the diagnosis process to avoid missed diagnosis or misdiagnosis, so as to provide the best diagnosis and treatment plan.
Home-based HIV testing strategies for middle-aged and older adults in rural South Africa
Authors: Marcus Maja E. , Mahlalela Nomsa , Drame Ndeye D. , Rohr Julia K. , Vollmer Sebastian , Tollman Stephen , Berkman Lisa , Kahn Kathleen , Gómez-Olivé Francesc Xavier , Manne-Goehler Jennifer , Bärnighausen Till 
Published: September 2023

Objective:
More than one in four adults over 40 years with HIV in South Africa are unaware of their status and not receiving antiretroviral therapy (ART). HIV self-testing may offer a powerful approach to closing this gap for aging adults. Here, we report the results of a randomized comparative effectiveness trial of three different home-based HIV testing strategies for middle-aged and older adults in rural South Africa.

Design:
Two thousand nine hundred and sixty-three individuals in the ‘Health and Ageing in Africa: a Longitudinal Study of an INDEPTH Community in South Africa (HAALSI)’ cohort study were randomized 1 : 1 : 1 to one of three types of home-based and home-delivered HIV testing modalities: rapid testing with counseling; self-testing, and both rapid testing with counselling and self-testing.

Method:
In OLS regression analyses, we estimated the treatment effects on HIV testing and HIV testing frequency at about 1 year after delivery. Finally, we assessed the potential adverse effects of these strategies on the secondary outcomes of depressive symptom as assessed by the CESD-20, linkage to care, and risky sexual behavior.

Results:
There were no significant differences in HIV testing uptake or testing frequency across groups. However, respondents in the self-testing treatment arms were more likely to shift from testing at home and a facility [self-testing (HIVST), −8 percentage points (pp); 95% confidence interval (CI) −14 to −2 pp; self-testing plus rapid testing and counselling (ST+RT+C); −9 pp, 95% CI −15 to −3 pp] to testing only at home (HIVST 5 pp; 95% CI 2 to 9 pp; ST+RT+C: 5 pp, 95% CI 1 to 9 pp) – suggesting a revealed preference for self-testing in this population. We also found no adverse effects of this strategy on linkage to care for HIV and common comorbidities, recent sexual partners, or condom use. Finally, those in the self-testing only arm had significantly decreased depressive symptom scores by 0.58 points (95% CI −1.16 to −0.01).

Conclusion:
We find HIV self-testing to be a well tolerated and seemingly preferred home-based testing option for middle-aged and older adults in rural South Africa. This approach should be expanded to achieve the UNAIDS 95–95–95 targets.
Urine point-of-care tenofovir test demonstrates strong predictive clinical and research utility
Authors: Mustanski Brian , Ryan Daniel T. , Spinelli Matthew A. , Gandhi Monica , Newcomb Michael E. 
Published: September 2023

Background:
Preexposure prophylaxis (PrEP) significantly reduces HIV infection risk but is dependent on adherence. Available approaches to measuring adherence have limitations related to accuracy, cost, practicality, and timeliness. This study compared the performance of two methods implementable in clinics and research studies [interview and urine point of care (POC) assay] to the gold-standard for measuring recent and longer term adherence in dried blood spots (DBS).

Methods:
Participants were recruited from RADAR, a cohort study of young MSM, or via online advertisements. At 3 monthly visits, an interviewer administered 7-day timeline follow-back (TLFB) questionnaire, DBS samples were tested for tenofovir-diphosphate (TFV-DP) to estimate average dosing over the prior month and emtricitabine-triphosphate (FTC-TP) to assess recent dosing (past 2–3 days), and a urine POC TFV test to qualitatively assess recent adherence (past 4 days).

Results:
Eighty-three PrEP users contributed 163 observations. At visit 1, self-reported adherence was 86% (4+ doses in last 7 days), versus urine TFV (74%), DBS FTC-TP (76%), and DBS TFV-DP (69%). The objective measures of short-term adherence performed similarly well in predicting longer term adherence. In multivariable logistic regression analyses, the urine assay was a significant predictor of DBS TFV-DP (adjusted OR = 19.4, P < 0.0001); self-report did not add significantly.

Conclusion:
The urine POC TFV assay had excellent predictive values for adherence and self-report did not add significantly to prediction. The POC assay provides results in several minutes to enable same-visit counseling, requires no specialized training, and is projected to be low-cost.