A new physiological medium uncovers biochemical and cellular alterations in Lesch-Nyhan disease fibroblasts.

Background: Lesch-Nyhan disease (LND) is a severe neurological disorder caused by the genetic deficiency of hypoxanthine-guanine phosphoribosyltransferase (HGprt), an enzyme involved in the salvage synthesis of purines. To compensate this deficiency, there is an acceleration of the de novo purine biosynthetic pathway. Most studies have failed to find any consistent abnormalities of purine nucleotides in cultured cells obtained from the patients.

Randomized Trial on the Effect of an Oral Spleen Tyrosine Kinase Inhibitor in the Treatment of IgA Nephropathy.

Introduction: We reported increased spleen tyrosine kinase (SYK) expression in kidney biopsies of patients with IgA nephropathy (IgAN) and that inhibition of SYK reduces inflammatory cytokines production from IgA stimulated mesangial cells.

Methods: This study was a double-blind, randomized, placebo-controlled phase 2 trial of fostamatinib (an oral SYK inhibitor) in 76 patients with IgAN. Patients were randomized to receive placebo, fostamatinib at 100 mg or 150 mg twice daily for 24 weeks on top of maximum tolerated dose of renin-angiotensin system inhibitors.

In vitro synergistic antifungal activities of caspofungin in combination with fluconazole or voriconazole against Candida species determined by the Etest method.

Objectives: Increased resistance of Candida species, especially Candida. glabrata is problematic. Combination antifungal therapies were studied to solve the problem.

REPRINT OF: Relationship of Childhood Abuse and Household Dysfunction to Many of the Leading Causes of Death in Adults: The Adverse Childhood Experiences (ACE) Study.

Background: The relationship of health risk behavior and disease in adulthood to the breadth of exposure to childhood emotional, physical, or sexual abuse, and household dysfunction during childhood has not previously been described.

Methods: A questionnaire about adverse childhood experiences was mailed to 13,494 adults who had completed a standardized medical evaluation at a large HMO; 9,508 (70.5%) responded.

Foot Width Changes Following Hallux Valgus Surgery.

Background: Hallux valgus (HV) adversely affects quality of life. Patients frequently express concerns regarding postoperative foot appearance, foot width and footwear anticipations. However, only scarce data are available regarding postoperative foot width.

Lower Extremity Fractures in Hospitalized Pediatric Patients Following Road Traffic Accidents.

Lower extremity fractures (LEFs) caused by road traffic accidents (RTAs) can result in significant morbidity and account for a substantial part of nonfatal injuries requiring hospitalization. This study investigated the epidemiology of RTA-associated LEFs in the pediatric population. Based on the National Trauma Registry, data of 28,924 RTA hospitalized pediatric patients were reviewed.

Lateral malleolus closed reduction and internal fixation with intramedullary fibular rod using minimal invasive approach for the treatment of ankle fractures.

Background: Ankle fractures are extremely common and represent nearly one quarter of all lower-limb fractures. Techniques for fixation of displaced fractures of the lateral malleolus have remained essentially unchanged in recent decades. The current gold standard of treating unstable fractures is with open reduction and internal fixation (ORIF), using plates and screws construct.

Orthopaedic injuries among electric bicycle users.

Introduction: The use of electric bicycles (E-bike) has dramatically increased. E-bikes offer convenient, environmental-friendly, and less expensive alternative to other forms of transport. However, E-bikes provide a new public health challenge in terms of safety and injury prevention.

[Preliminary analysis of the effects of tailor-made notched music therapy on chronic idiopathic tinnitus].

Tailor-made notched music was applied to alleviate the symptoms of chronic idiopathic tinnitus and compared its effectiveness with other existing sound treatment of tinnitus. Subjects (=43; ears=75 )were recruited during June 2015 to October 2016 from the out-patients of our hospital. These patients had chronic (longer than 6months) and idiopathic tinnitus, with or without significant sensorineural hearing loss.

Factors affecting mortality after penetrating cardiac injuries: 10-year experience at urban level I trauma center.

Background: Despite the lethality of injuries to the heart, optimizing factors that impact mortality for victims that do survive to reach the hospital is critical.

Methods: From 2003 to 2012, prehospital data, injury characteristics, and clinical patient factors were analyzed for victims with penetrating cardiac injuries (PCIs) at an urban, level I trauma center.

Results: Over the 10-year study, 80 PCI patients survived to reach the hospital.

Penetrating cardiac injuries: A 36-year perspective at an urban, Level I trauma center.

Background: This study evaluates patterns of injuries and outcomes from penetrating cardiac injuries (PCIs) at Grady Memorial Hospital, an urban, Level I trauma center in Atlanta, Georgia, over 36 years.

Methods: Patients sustaining PCIs were identified from the Trauma Registry of the American College of Surgeons and the Emory Department of Surgery database; data of patients who died prior to any therapy were excluded. Demographics and outcomes were compared over three time intervals: Period 1 (1975-1985; n = 113), Period 2 (1986-1996; n = 79), and Period 3 (2000-2010; n = 79).

Salvage of avascular necrosis of the talus by combined ankle and hindfoot arthrodesis without structural bone graft.

Background: Osteonecrosis of the talus is a well-recognized pathology, which can result in significant hindfoot collapse resulting in poor function and pain. Treatment with intramedullary tibiotalocalcaneal arthrodesis (IMTCA) using a retrograde intramedullary nail is widely utilized for severe concomitant tibiotalar and subtalar pathologies. This study reports the results of ankle and hindfoot arthrodesis in patients with arthritis and deformity caused specifically by talar osteonecrosis.

Standard and advanced echocardiography in takotsubo (stress) cardiomyopathy: clinical and prognostic implications.

Echocardiography is frequently the initial noninvasive imaging modality used to assess patients with takotsubo cardiomyopathy (TTC). Standard transthoracic echocardiography can provide, even in the acute care setting, useful information about left ventricular (LV) morphology as well as regional and global systolic or diastolic function. It allows the differentiation of different LV morphologic patterns according to the localization of wall motion abnormalities.

Rapid laboratory diagnosis for respiratory infectious diseases by using MALDI-TOF mass spectrometry.

It is still challenging to prevent and treat respiratory infectious diseases. One critical step in the successful treatment of respiratory infections is rapid diagnosis by identifying the causative microorganisms in a timely fashion. However, traditional methods for identification of causative agents could not satisfy the need for rapid and accurate testing due to the limitations of technology-used.

Prior high-risk human papillomavirus testing and Papanicolaou test results of 70 invasive cervical carcinomas diagnosed in 2012: results of a retrospective multicenter study.

Context: Persistent high-risk human papillomavirus (hrHPV) infection is essential for the development of cervical cancer and its precursor lesions. High-risk HPV testing has a higher sensitivity than cytology does for detecting cervical epithelial lesions. However, a large study from a single institution showed 31% of patients with invasive cervical cancer had negative baseline hrHPV testing within 5 years preceding the diagnosis.

Base deficit as a marker of survival after traumatic injury: consistent across changing patient populations and resuscitation paradigms.

Background: Damage control resuscitation (DCR) has improved outcomes in severely injured patients. In civilian centers, massive transfusion protocols (MTPs) represent the most formal application of DCR principles, ensuring early, accurate delivery of high fixed ratios of blood components. Recent data suggest that DCR may also help address early trauma-induced coagulopathy.

Heterogeneity of primary glioblastoma cells in the expression of caspase-8 and the response to TRAIL-induced apoptosis.

Recent studies suggest that cancer stem cells (CSCs) are responsible for cancer resistance to therapies. We therefore investigated how glioblastoma-derived CSCs respond to the treatment of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL). Neurospheres were generated from glioblastomas, characterized for CSC properties including self-renewal, cell differentiation and xenograft formation capacity, and analyzed for TRAIL-induced apoptosis, CASP8 genomic status, and caspase-8 protein expression.

DR5-mediated DISC controls caspase-8 cleavage and initiation of apoptosis in human glioblastomas.

To explore the molecular mechanisms by which glioblastomas are resistant to tumour necrosis factor-related apoptosis-inducing ligand (TRAIL), we examined TRAIL signalling pathways in the tumours. TRAIL has four membrane-anchored receptors, death receptor 4/5 (DR4/5) and decoy receptor 1/2 (DcR1/2). Of these receptors, only DR5 was expressed consistently in glioblastoma cell lines and tumour tissues, ruling out the role of DcR1/2 in TRAIL resistance.

A prospective, open-label trial of sirolimus in the treatment of focal segmental glomerulosclerosis.

Calcineurin inhibitors are effective therapy for steroid-resistant focal segmental glomerulosclerosis (FSGS) but are associated with significant morbidity and nephrotoxicity. Sirolimus is a novel immunosuppressive agent that is structurally related to tacrolimus but demonstrates no long-term nephrotoxicity. For determination of the efficacy of sirolimus in reducing proteinuria, a prospective, open-label trial was conducted of 21 patients with idiopathic, steroid-resistant FSGS.

Costs of coronary restenosis (Lovastatin Restenosis Trial).

Within the Lovastatin Restenosis Trial, restenosis has been clearly shown to increase resource utilization and costs. While it is not possible to generalize these results to other patient populations, it is clear that successful efforts to decrease restenosis will certainly improve efficacy while decreasing follow-up costs and increasing the cost-effectiveness of intervention in the coronaries.