An Unusual Case of Erdheim Chester Disease (ECD) with Knee Pain: A Case Report.

Background: Erdheim Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology that occurs in multiple organs. The clinical characteristics of ECD are unknown, making it difficult to diagnose. Case presentation: A 61-year-old woman presented with left knee pain and contracture. She had recent medical problems such as recurrent urinary tract infection, pericardial effusion, and pleural effusion. Simple radiography and magnetic resonance imaging of the knee revealed an osteosclerotic lesion. Under suspicion of malignancy, other radiologic modalities were performed, but there were no significant results showing malignancy. A bone biopsy of the knee lesion led to a final diagnosis of ECD. The patient was treated with systemic steroids and was ultimately tried on PEG-interferon. Conclusion: This report describes an unusual presentation of ECD involving the skeletal system and multiple extraskeletal organs. Owing to its non-specific nature, ECD was notably difficult to diagnose. Therefore, if a patient has knee pain and other multiorgan presentations without malignancy, clinicians should suspect ECD.

Ulnar shortening osteotomy for posttraumatic ulnar impaction syndrome in adolescent (younger than 18 years) - Based on the Cha & Shin assessment.

PURPOSE: We performed ordinary ulnar shortening osteotomy (USO) in patients younger than 18 years old with secondary ulnar impaction syndrome (UIS) after traumatic events. Here, we report the clinical and radiologic outcomes with a review of the previous literature through a retrospective case series. METHODS: Twenty-two adolescents treated by USOs from 2006 to 2018 were investigated. The amount of shortening was classified into three categories. The first category was for a still open physis on the medial half of the radius in those younger than 15. In this category, we osteotomized the ulna for the physis level to be left neutral or negative by 1-2 mm. The second category had no growth potency in the radius. If the patient was younger than 15, we considered only residual growth of the ulna, thus performing USO for the ordinary UV to be negative by 2-3 mm. For patients aged 15-18 years old, if growth potency was nearly absent in the ulna, we performed traditional USO with a neutral ulnar variance (UV). RESULTS: Categories 1, 2, and 3 for the amount of USO were determined for 4, 4, and 14 patients, respectively. All USOs properly healed without substantial complications. The mean preoperative UV was 2.91 mm, and the final value decreased to 0.23 with statistical significance (p < 0.001). The range of wrist motion was improved after USO from 133.86° and 132.73° to 154.77° and 160.68° (all, p < 0.001 in flexion-extension and pronation-supination arcs, respectively). The preoperative VAS and MMWS scores also improved from 2.77 to 75.00 to 0.18 and 88.86, respectively, at the final follow-up (all, p < 0.001). CONCLUSIONS: UIS in adolescent populations after trauma in their children/younger adolescents could be properly treated by USO. Even with an open physis at the ulna, neutral UVs could be achieved, and the clinical outcomes were satisfactory. However, long-term follow-up is still needed regarding TFCC and DRUJ status. LEVEL OF EVIDENCE: Level IV, retrospective case series.

Systematic scoping review on moral distress among physicians.

BACKGROUND: Concepts of moral distress (MD) among physicians have evolved and extend beyond the notion of psychological distress caused by being in a situation in which one is constrained from acting on what one knows to be right. With many accounts involving complex personal, professional, legal, ethical and moral issues, we propose a review of current understanding of MD among physicians. METHODS: A systematic evidence-based approach guided systematic scoping review is proposed to map the current concepts of MD among physicians published in PubMed, Embase, PsycINFO, Web of Science, SCOPUS, ERIC and Google Scholar databases. Concurrent and independent thematic and direct content analysis (split approach) was conducted on included articles to enhance the reliability and transparency of the process. The themes and categories identified were combined using the jigsaw perspective to create domains that form the framework of the discussion that follows. RESULTS: A total of 30 156 abstracts were identified, 2473 full-text articles were reviewed and 128 articles were included. The five domains identified were as follows: (1) current concepts, (2) risk factors, (3) impact, (4) tools and (5) interventions. CONCLUSIONS: Initial reviews suggest that MD involves conflicts within a physician's personal beliefs, values and principles (personal constructs) caused by personal, ethical, moral, contextual, professional and sociocultural factors. How these experiences are processed and reflected on and then integrated into the physician's personal constructs impacts their self-concepts of personhood and identity and can result in MD. The ring theory of personhood facilitates an appreciation of how new experiences create dissonance and resonance within personal constructs. These insights allow the forwarding of a new broader concept of MD and a personalised approach to assessing and treating MD. While further studies are required to test these findings, they offer a personalised means of supporting a physician's MD and preventing burn-out.

Doxorubicin loading fucoidan acetate nanoparticles for immune and chemotherapy in cancer treatment.

In order to develop immuno- and chemotherapy agents, self-organized acetylated fucoidan (AcFu) nanoparticles were designed. Doxorubicin (DOX), used as a model drug, was loaded into the AcFu nanoparticles by dialysis. The DOX loading efficacy and content were 71.1% and 3.6%, respectively. Approximately 140 nm of spherical nanoparticles were obtained. DOX-loaded AcFu nanoparticles (DOX-AcFu) exhibited first-order drug release behavior for 5 days. Interestingly, AcFu treated Raw264.7 macrophages overexpressed various anti-tumor cytokines, such as tumor necrosis factor-alpha (TNF-α) and granulocyte-macrophage colony-stimulating factor (GM-CSF). The ability of DOX-AcFu to suppress drug efflux was revealed by confocal microscope images and FACS analysis in multidrug resistance (MDR) cells. IC50 (50% inhibitory concentration) value of DOX-AcFu was lower than that of free DOX in the MDR model cells. Based on these results, we strongly suggest that AcFu nanoparticles have a promising potential for development as a one-step therapy containing agents for both immuno- and chemotherapy.

Optical imaging of intracellular reactive oxygen species for the assessment of the cytotoxicity of nanoparticles.

The generation of intracellular reactive oxygen species (ROS) was optically monitored using ROS-sensitive gold nanoprobes in response to an exposure of nanoparticles (NPs). Fluorescent dye-labeled hyaluronic acid was grafted onto the surface of gold nanoparticles (HF-AuNPs) for imaging intracellular ROS. The ultrasensitive detection of intracellular ROS was utilized as a powerful analytical tool to assess early cellular toxicities of monodisperse polystyrene (PS) particles with different sizes and different functional groups on the surface. The effect of PEGylation on the surface of PS NPs was also investigated by evaluating intracellular ROS generation. For various PS NPs, the extent of intracellular ROS was well correlated with cellular uptake, apoptosis inducing activity, and cytotoxic effect of NPs. In addition to the nanoparticles, commonly used polymeric gene carriers such as linear and branched polyethylenimine (PEI) were tested to analyze their extent of intracellular ROS generation related to cellular toxicity. This study demonstrated that sensitive and optical detection of intracellular ROS generation can provide a valuable toxicity index value for a wide range of NPs as an early indicator for cellular responses.

Biomechanical comparison of anterior and posterior stabilization methods in atlantoaxial instability.

OBJECT: The authors compared the biomechanical stability of two anterior fixation procedures--anterior C1-2 Harms plate/screw (AHPS) fixation and the anterior C1-2 transarticular screw (ATS) fixation; and two posterior fixation procedures--the posterior C-1 lateral mass combined with C-2 pedicle screw/rod (PLM/APSR) fixation and the posterior C1-2 transarticular screw (PTS) fixation after destabilization. METHODS: Sixteen human cervical spine specimens (Oc-C3) were tested in three-dimensional flexion-extension, axial rotation, and lateral bending motions after destabilization by using an atlantoaxial C1-2 instability model. In each loading mode, moments were applied to a maximum of 1.5 Nm, and the range of motion (ROM), neutral zone (NZ), and elastic zone (EZ) were determined and values compared using the intact spine, the destabilized spine, and the postfixation spine. The AHPS method produced inferior biomechanical results in flexion-extension and lateral bending modes compared with the intact spine. The lateral bending NZ and ROM for this method differed significantly from the other three fixation techniques (p < 0.05), although statistically significant differences were not obtained for all other values of ROM and NZ for the other three procedures. The remaining three methods restored biomechanical stability and improved it over that of the intact spine. CONCLUSIONS: The PLM/APSR fixation method was found to have the highest biomechanical stiffness followed by PTS, ATS, and AHPS fixation. The PLM/APSR fixation and AATS methods can be considered good procedures for stabilizing the atlantoaxial joints, although specific fixation methods are determined by the proper clinical and radiological characteristics in each patient.