bluuebunny/crossref_metadata_2025_split huggingface dataset:
Therapeutic benefits of distal ventricular pacing in mid-cavity obstructive hypertrophic cardiomyopathy
Authors: Malcolmson James W. , Hughes Rebecca K. , Joshi Abhishek , Cooper Jackie , Breitenstein Alexander , Ginks Matthew , Petersen Steffen E. , Mohiddin Saidi A. , Dhinoja Mehul B. 
Published: August 2022

Introduction: Hypertrophic cardiomyopathy (HCM) patients with left ventricular (LV) mid-cavity obstruction (LVMCO) often experience severe drug-refractory symptoms thought to be related to intraventricular obstruction. We tested whether ventricular pacing, guided by invasive haemodynamic assessment, reduced LVMCO and improved refractory symptoms. Methods: Between December 2008 and December 2017, 16 HCM patients with severe refractory symptoms and LVMCO underwent device implantation with haemodynamic pacing study to assess the effect on invasively defined LVMCO gradients. The effect on the gradient of atrioventricular (AV) synchronous pacing from sites including right ventricular (RV) apex and middle cardiac vein (MCV) was retrospectively assessed. Results: Invasive haemodynamic data were available in 14 of 16 patients. Mean pre-treatment intracavitary gradient was 77 ± 22 mmHg (in sinus rhythm) versus 21 ± 21 mmHg during pacing from optimal ventricular site (95% CI: −70.86 to −40.57, p < 0.0001). Optimal pacing site was distal MCV in 12/16 (86%), RV apex in 1/16 and via epicardial LV lead in 1/16. Pre-pacing Doppler-derived gradients were significantly higher than at follow-up (47 ± 15 versus 24 ± 16 mmHg, 95% CI: −37.19 to −13.73, p < 0.001). Median baseline NYHA class was 3, which had improved by ⩾1 NYHA class in 13 of 16 patients at 1-year post-procedure ( p < 0.001). The mean follow-up duration was 4.6 ± 2.7 years with the following outcomes: 8/16 (50%) had continued symptomatic improvement, 4/16 had symptomatic decline and 4/16 died. Contributors to symptomatic decline included chronic atrial fibrillation (AF) ( n = 5), phrenic nerve stimulation ( n = 3) and ventricular ectopy ( n = 1). Conclusion: In drug-refractory symptomatic LVMCO, distal ventricular pacing can reduce intracavitary obstruction and may provide long-term symptomatic relief in patients with limited treatment options. A haemodynamic pacing study is an effective strategy for identifying optimal pacing site and configuration.
Risk factors for hip displacement in cerebral palsy: A population-based study of 121 nonambulatory children
Authors: Terjesen Terje , Horn Joachim 
Published: August 2022

Purpose: The aim was to evaluate which clinical and radiographic variables are independent (true) risk factors for hip subluxation in nonambulatory children below 5 years of age with cerebral palsy. Methods: Patients were recruited from a population-based hip surveillance program. Inclusion criteria were birth during 2002–2006, age below 5 years, and gross motor function classification system levels III–V. In all, 121 children (71 boys) met these criteria. Gross motor function classification system was level III in 29 patients, level IV in 28, and level V in 64. Anteroposterior radiographs at diagnosis and during follow-up were assessed, and only the worst hip of each patient was used for the analyses. The mean age at the initial radiograph was 2.5 years (range: 0.7–4.9 years), and the mean follow-up time was 4.0 years (range: 0.5–11.8 years). Results: At the last follow-up, 67 children had a clinically significant hip displacement, defined as migration percentage ≥40%. Univariable regression analysis defined these risk factors: gross motor function classification system level V, spastic bilateral cerebral palsy, initial migration percentage, yearly rate of migration percentage progression, and initial acetabular index. When these variables were analyzed with multivariable regression in 107 patients with initial migration percentage <50% and follow-up ≥1.0 year, the independent risk factors were initial migration percentage (p = 0.003) and yearly rate of migration percentage progression (p < 0.001). Conclusion: The parameters that need to be assessed in hip surveillance in children below 5 years of age are initial migration percentage and rate of migration percentage progression. Acetabular index and femoral head–shaft angle might be useful later for decision-making regarding choice of treatment. Level of evidence: Level II, development of diagnostic criteria.
Outcomes for patients with infantile idiopathic scoliosis by casting table type
Authors: Marrache Majd , Prasad Niyathi , Thompson George H , Li Ying , Glotzbecker Michael , Sponseller Paul D 
Published: August 2022

Purpose: Serial casting is an effective treatment for infantile idiopathic scoliosis. The most common casting table types are Mehta, Risser, and spica tables. We compared major curve correction between patients with infantile idiopathic scoliosis treated using pediatric hip spica tables versus Risser or Mehta tables. Methods: In this multicenter retrospective study, we included 52 children younger than 3 years (mean ± standard deviation age, 1.6 ± 0.68 years) treated with ≥2 consecutive casts for infantile idiopathic scoliosis between September 2011 and July 2018. We compared major curve angle (measured using the Cobb method) before and after treatment and improvement in curve angle between the spica tables group ( n = 12) and the Risser or Mehta tables group ( n = 40). The primary outcome was the difference in percentage correction of the major curve according to radiographs taken after first casting and at final follow-up. Results: The mean major curve was 47° ± 18° before casting. A median of six casts (range: 2–14) were applied. Mean follow-up after treatment initiation was 22 months (range: 7–86 months). At baseline, the major curve was significantly larger in the spica tables group (58°) than in the Risser or Mehta tables group (43°) ( p = 0.01). We found no differences in the percentage curve correction in the spica tables group versus Risser or Mehta tables group after first casting or at final follow-up. Conclusion: Serial casting was associated with substantial major curve correction in patients with infantile idiopathic scoliosis. Curve correction did not differ between patients treated with a spica table versus a Risser or Mehta table. Level of Evidence: Level III, retrospective cohort study
Sinonasal Papillomas: 10-Year Retrospective Analysis of Etiology, Epidemiology, and Recurrence
Authors: Archang Maani , Chew Leila , Han Albert Yoon-Kyu , Sajed Dipti , Vorasubin Nopawan , Wang Marilene 
Published: August 2022

Objectives To examine the epidemiology, subtypes, trends over time, and predictive factors for recurrence and malignant transformation of sinonasal papillomas. Methods A retrospective chart review of 118 patients with sinonasal papillomas from 2009 to 2019 was conducted at the University of California, Los Angeles. This study is a follow-up to a previously published study from 2000 to 2009 at the same academic center. Results The mean age was at presentation was 58.5 years, with a 2:1 male to female ratio, and average follow-up of 30.1 months. The rate of recurrence after complete resection was 19% with an average of 32.6 months to recurrence. The time to recurrence followed a bimodal distribution with 57% of cases recurring within 24 months (mean = 10) and 43% from 40 to 103 months (mean = 61). The proportion of the inverted papillomas rose from 38% in 2000-2004 to 89.6% in 2015-2019. Patients presenting at a younger age had a higher chance of recurrence (mean age 52 with recurrence vs. 61 without recurrence). Age did not correlate with histopathologic transformation in surgical pathology. Furthermore, histopathological transformation did not raise the chance of recurrence. Smoking, alcohol use, chronic rhinosinusitis, and allergic rhinitis were not associated with any of the outcome measures in this study. The most significant factor predicting recurrence, beside age at presentation, was the history of two or more prior sinus surgeries for papillomas or other reasons (OR = 3.52 and 5.81). Conclusion This study explored the features of sinonasal papillomas as well as the risk factors for recurrence and transformation. Younger age at presentation and two or more prior surgeries for papillomas were associated with recurrence. Time to recurrence followed a bimodal distribution, with late recurrences happenning from 40 to 103 months after surgery, emphasizing the importance of long-term follow-up for timely resection of tumors and prevention of malignancy.
Feline intralenticular Encephalitozoon cuniculi: three cases from California
Authors: Lin Joie , Nell Barbara , Horikawa Taemi , Zarfoss Mitzi 
Published: August 2022

Case series summary Three domestic shorthair cats from California presented to veterinary ophthalmologists with immature cataracts. Other presenting clinical signs included corneal edema, anisocoria, anterior uveitis, elevated intraocular pressure, blepharospasm and/or lethargy. All patients were immunocompromised due to concurrent diseases and/or immunomodulatory drugs. Diagnostics included serial comprehensive ophthalmic examinations with tonometry, ocular ultrasound, electroretinogram and testing for other causes of feline uveitis. Testing for Encephalitozoon cuniculi included serology, histopathology and/or PCR of aqueous humor, lens material or paraffin-embedded whole eye. Treatments included antiparasitic medication, anti-inflammatory medication and supportive care in all three cases. Surgical treatment included enucleation (one case), bilateral phacoemulsification and unilateral intraocular lens placement (one case) and bilateral phacoemulsification with bilateral endolaser ciliary body ablation and bilateral intraocular lens implantation (one case). Both cats for which serologic testing for E cuniculi was performed were positive (1:64–1:4096). In all cats, diagnosis of intraocular E cuniculi was based on at least one of the following: lens histopathology or PCR of aqueous humor, lens material or paraffin-embedded ocular tissue. The clinical visual outcome was best in the patient undergoing phacoemulsification at the earliest stage of the cataract. Relevance and novel information Encephalitozoon cuniculi should be considered as a differential cause of cataracts and uveitis in cats in California, the rest of the USA and likely worldwide.
Magnetic Resonance Imaging of Lateral Meniscus Root Tears in the Pediatric and Adolescent Knee: Is the Diagnosis Missed, Mentioned, or Made?
Authors: Schlechter John , Pak Theresa , Gornick Bryn , McDonald Edward 
Published: August 2022

Background: Failure to address meniscus root tears may place undue loads on anterior cruciate ligament (ACL) reconstructive surgery in the adult population. Because the intraoperative management of lateral meniscus posterior root tears (LMPRTs) may diverge from standard meniscal work and requires specialty items, preoperative diagnosis may be advantageous. Purpose: To evaluate the reliability of radiologist interpretations of preoperative knee magnetic resonance imaging (MRI) scans of lateral meniscus root pathology in a mixed pediatric and adolescent population. Study Design: Cohort study (diagnosis); Level of evidence, 2. Methods: A retrospective review of medical records was performed to identify patients younger than 18 years with an arthroscopically confirmed LMPRT who underwent knee arthroscopy between March 1, 2010, and April 1, 2020. Arthroscopic findings were compared with the reading radiologist’s preoperative MRI interpretations, and patients were stratified into 2 groups: (1) LMPRT diagnosis made preoperatively or (2) diagnosis missed or only mentioned to describe pathology nonspecific to the root. Variables such as body mass index (BMI), open physes, time from injury to MRI, time from MRI to surgery, MRI magnet field strength, musculoskeletal radiologist designation, insurance type, and tear grade were assessed between groups. Results: Overall, 1116 knee arthroscopies were performed, with 49 LMPRTs found; all 49 LMRPTs were found concomitantly with ACL tears (49/535; 9.2%). The average patient age was 15.97 years (range, 11.52-17.97 years). There were 50 MRI scans for 49 patients. An LMPRT was diagnosed based on preoperative MRI scans in 12 of these 50 scans (24%) and mentioned or missed in 38 of the 50 scans (76%). No significant difference was seen between the diagnosis-made versus diagnosis-mentioned/missed groups in BMI, skeletal maturity, time from injury to MRI, time from MRI to surgery, MRI magnet strength, fellowship training of the reading radiologist, tear grade, or insurance type. Conclusion: In 76% of patients, a definitive diagnosis of LMPRT was not made on preoperative MRI scans. Notably, all LMPRTs found intraoperatively were found concomitantly with ACL tears.
Factors Associated With Diabetes Self-Care Behaviors of People With Visual Impairment: A Cross-Sectional Study
Authors: Chang Sun Ju , Kim Hee Jung , Lee Kyoung-eun , Yang Eunjin 
Published: August 2022

Purpose: The purpose of the study was to examine the factors associated with diabetes self-care behaviors (DSCB) in people with visual impairment (PVI), including demographics, disease-related characteristics, and psychosocial factors (self-efficacy, depression, and family support). Methods: A descriptive cross-sectional study was conducted. Adults with visual impairment and diabetes were recruited online and through posting notices, and a total of 141 participants were included. Measurements were completed using email and telephone surveys. Multiple linear regression analysis was performed to examine the factors associated with DSCB. Results: Higher self-efficacy and positive family support were significant predictors of better DSCB, whereas higher negative family support was a significant predictor of worse DSCB. Diabetes medication regimen was another major influencing factor on DSCB. However, depression was not associated with DSCB. Conclusion: Diabetes interventions for the visually impaired should have strategies to improve vulnerable areas of diabetes self-care, such as blood glucose monitoring. Health care providers are needed to consider diabetes education programs focused on improving self-efficacy or include family members in interventions to promote DSCB in PVI. Further studies are required to understand the problems and solutions for diabetes self-care in this population.
Experiences of Using Wearable Continuous Glucose Monitors in Adults With Diabetes: A Qualitative Descriptive Study
Authors: Kang Hee Sun , Park Hyang Rang , Kim Chun-Ja , Singh-Carlson Savitri 
Published: August 2022

Purpose: The purpose of the study was to understand the experiences of adults with diabetes wearing a continuous glucose monitor (CGM). Methods: This qualitative, descriptive study included 19 adults with type 1 diabetes or type 2 diabetes, who had used the FreeStyle Libre CGM for at least 4 weeks, from an outpatient clinic at a university-affiliated hospital in Korea. Data were collected through in-depth interviews and analyzed using an inductive content analysis approach. Results: The content analysis revealed 3 major themes—navigating glucose level fluctuations, reframing diabetes self-care and improving quality of life, and device improvement and service quality. Participants reported that CGMs offered convenient glucose level monitoring, allowed early response to rapid glucose changes, and facilitated effective patient-clinician communication. Participants expressed concerns about the financial burden and limited services, recommending improvements for devices, consumer services, and health insurance coverage. Conclusions: Study findings indicated that using wearable CGMs could improve self-care and quality of life in adults with diabetes. Using CGMs could improve patients’ understanding of how diabetes self-care management affects real-time glucose levels. Health care providers could support patients’ self-care by using device data. Improvements in quality, services, and insurance coverage could increase user satisfaction and promote self-care.
Arthroscopic Repair of a “Floating” Posterior Inferior Glenohumeral Ligament
Authors: Greiner Justin J. , Setliff Joshua C. , Dworkin Joshua D. , Lin Albert 
Published: August 2022

Background: Combined posterior glenoid labrum lesions with posterior humeral avulsion of the glenohumeral ligament, also known as the “floating” posterior inferior glenohumeral ligament (PIGL), occur infrequently. These combined lesions are frequently missed on magnetic resonance imaging in the workup of posterior shoulder instability. Correct identification of the “floating” PIGL lesion allows for appropriate preoperative planning and treatment to decrease the risk of recurrent posterior shoulder instability. Indications: A “floating” PIGL lesion is a cause of posterior shoulder instability and demonstrates increased translation when compared with isolated labral lesions. Surgical repair of an acute “floating” PIGL lesion with concomitant superior labral tear is described. Technique Description: The patient is placed in the lateral decubitus position. Standard posterior and anterior portals are created. In this case, a superior labral tear with anterior labral tear extension was also identified and repaired. The posterior labrum was repaired prior to the posterior humeral avulsion of the glenohumeral ligament (HAGL). The torn posterior labrum is mobilized and glenoid bony bed prepared. Short, 2.9-mm biocomposite knotless suture anchors loaded with suture tape are used for labral fixation. A 70° arthroscope is used to visualize the posterior HAGL from the anterior cannula and an additional posterior inferior portal established. The footprint of PIGL on the humerus is identified, debrided, and two 3.0-mm anchors loaded with suture placed. The sutures are passed through the capsule and PIGL and tied in a mattress pattern external to the capsule and ligament. The posterior portals are closed with nonabsorbable suture. Results: While few outcomes are described in the literature for the “floating” PIGL, the literature suggests good outcomes following surgical repair. Conclusion: The “floating” PIGL lesion is a rare cause of posterior shoulder instability. It is important to perform a thorough evaluation for concomitant pathology in patients with posterior shoulder instability as multiple structures can be injured. Arthroscopic repair of the posterior labrum and posterior humeral avulsion of the glenohumeral ligament can be performed to restore posterior stability to the shoulder in the setting of a “floating” PIGL.
Ventricular arrhythmias and sudden death events following acalabrutinib initiation
Authors: Bhat Seema A. , Gambril John , Azali Leylah , Chen Sunnia T. , Rosen Lindsay , Palettas Marilly , Wiczer Tracy E. , Kalathoor Sujay , Zhao Qiuhong , Rogers Kerry A. , Kittai Adam , Grever Michael , Awan Farrukh , Ruz Patrick , Byrd John C. , Woyach Jennifer , Addison Daniel 
Published: August 2022

Abstract
Acalabrutinib, a next-generation Bruton’s tyrosine kinase inhibitor (BTKi), associates with dramatic efficacy against B-cell malignancies. Recently, unexplained ventricular arrhythmias (VAs) with next-generation BTKi-therapy have been reported. Yet, whether acalabrutinib associates with VAs in long-term follow-up is unknown. Leveraging a large-cohort of 290 consecutive B-cell malignancy patients treated with acalabrutinib from 2014 to 2020, we assessed the incidence of VAs. The primary-endpoint was incident VA development (ventricular fibrillation, ventricular tachycardia, and symptomatic premature ventricular contractions). Probability-scores were assessed to determine likelihood of acalabrutinib-association. Incident rates as function of time-on-therapy were calculated. Weighted average observed incidence rates were compared with expected population rates using relative-risks. Absolute excess risk (AER) for acalabrutinib-associated VAs was estimated. Over 1063 person-years of follow-up, there were 8 cases of incident-VAs, including 6 in those without coronary disease (CAD) or heart failure (HF) and 1 sudden-death; median time-to-event 14.9 months. Among those without prior ibrutinib-use, CAD, or HF, the weighted average incidence was 394 per 100 000 person years compared with a reported incidence of 48.1 among similar-aged non–BTKi-treated subjects (relative risk, 8.2; P &lt; .001; AER, 346). Outside of age, no cardiac or electrocardiographic variables associated with VA development. Collectively, these data suggest VAs may be a class-effect of BTKi therapies.
SUMOylation inhibition overcomes proteasome inhibitor resistance in multiple myeloma
Authors: Heynen Guus J. J. E. , Baumgartner Francis , Heider Michael , Patra Upayan , Holz Maximilian , Braune Jan , Kaiser Melanie , Schäffer Isabell , Bamopoulos Stefanos A. , Ramberger Evelyn , Murgai Arunima , Ng Yuen Lam Dora , Demel Uta Margareta , Laue Dominik , Liebig Sven , Krüger Josefine , Janz Martin , Nogai Axel , Schick Markus , Mertins Philipp , Müller Stefan , Bassermann Florian , Krönke Jan , Keller Ulrich , Wirth Matthias 
Published: August 2022

Abstract
Proteasome inhibition is a highly effective treatment for multiple myeloma (MM). However, virtually all patients develop proteasome inhibitor resistance, which is associated with a poor prognosis. Hyperactive small ubiquitin-like modifier (SUMO) signaling is involved in both cancer pathogenesis and cancer progression. A state of increased SUMOylation has been associated with aggressive cancer biology. We found that relapsed/refractory MM is characterized by a SUMO-high state, and high expression of the SUMO E1-activating enzyme (SAE1/UBA2) is associated with poor overall survival. Consistently, continuous treatment of MM cell lines with carfilzomib (CFZ) enhanced SUMO pathway activity. Treatment of MM cell lines with the SUMO E1-activating enzyme inhibitor subasumstat (TAK-981) showed synergy with CFZ in both CFZ-sensitive and CFZ-resistant MM cell lines, irrespective of the TP53 state. Combination therapy was effective in primary MM cells and in 2 murine MM xenograft models. Mechanistically, combination treatment with subasumstat and CFZ enhanced genotoxic and proteotoxic stress, and induced apoptosis was associated with activity of the prolyl isomerase PIN1. In summary, our findings reveal activated SUMOylation as a therapeutic target in MM and point to combined SUMO/proteasome inhibition as a novel and potent strategy for the treatment of proteasome inhibitor–resistant MM.