Espensenmikkelsen4824

To investigate ocular angiographic features of hereditary transthyretin amyloidosis with transthyretin Val30Met mutation (hATTR-V30M) in Japanese patients.

We retrospectively reviewed 102 eyes of 51 patients with hATTR-V30M who underwent fluorescein angiograms (FAs) and indocyanine green angiograms (ICGAs) between 2012 and 2018. Systemic severity score, FAs, ICGAs, and ocular amyloidosis presentations at the final angiograms and subsequent neovascular events were evaluated. Primary outcomes were the frequency of choroidal amyloid angiopathy (CAA) and retinal amyloid angiopathy (RAA). Secondary outcomes were their correlations to the systemic severity score.

Six eyes could not be evaluated by FA due to vitreous opacity. Of 96 eyes evaluated, RAA was detected in 36 (37.5%). Neovascularization was not detected. Vorinostat solubility dmso ICGA indicated CAA in 46/51 patients (90.2%), with distinct patterns─diffuse (n=6), focal (n=14), and punctiform (n=26)─based on late-phase hypercyanescence. RAA and CAA grades were associated with systemic severity (ρ=0.57 and 0.50, respectively; both p<0.05). At 35.4±28.4 (0-96) months, iris-rubeosis was observed in one eye and vitreous hemorrhage in two.

RAA was less common and CAA was frequent, and their severity correlated with the systemic severity score. The frequencies of RAA and subsequent neovascular events in this study may suggest regional differences in the ocular angiographic features of hATTR-V30M.

RAA was less common and CAA was frequent, and their severity correlated with the systemic severity score. The frequencies of RAA and subsequent neovascular events in this study may suggest regional differences in the ocular angiographic features of hATTR-V30M.

To characterize the spectrum of internal limiting membrane (ILM) disease in Alport syndrome (AS) using multimodal imaging, including widefield (WF) and ultra-widefield (UWF) modalities, and to report their relative prevalence according to the genetic pattern of inheritance.

Cross-sectional clinical study of patients diagnosed with AS. All patients underwent UWF color photography and autofluorescence, WF-optical coherence tomography angiography (OCTA), and spectral-domain OCT (SD-OCT). Demographics, past medical and ophthalmic history, and genetic mutation history were collected.

Forty-two eyes of 21 patients (11 males; age 36.6±12.9 years) were included. Macular SD-OCT revealed ILM granularity, more frequent in X-linked AS and corresponding to dot maculopathy on color fundus. Mid-peripheral SD-OCT scans revealed multi-lamellated ILM in 8 eyes (19%), presumably progressive, which corresponded to a cavitary pattern on en-face OCT. En-face OCT revealed multiple areas of retinal nerve fiber layer (RNFL) dehiscence in the macula, overlapping with vascular lacunae on OCTA, and a coarse arrangement of RNFL above and below the temporal raphe in 20 eyes (52%).

Multimodal imaging allowed for the detection/characterization of retinal findings (ILM granularity, progressive ILM lamellation, RNFL dehiscence, vascular lacunae, and coarse arrangement of RNFL toward the disc) as multifaceted manifestations of ILM disease in AS.

Multimodal imaging allowed for the detection/characterization of retinal findings (ILM granularity, progressive ILM lamellation, RNFL dehiscence, vascular lacunae, and coarse arrangement of RNFL toward the disc) as multifaceted manifestations of ILM disease in AS.

The American Geriatrics Society regularly updates the Beers Criteria for Potentially Inappropriate Medication (PIM) to improve prescribing safety.

This study assessed the impact of nurse practitioner (NP) practices on PIM prescribing across states in the United States and compared the change in PIM prescribing rates between 2016 and 2018.

We used data from a random selection of 20% of Medicare beneficiaries (66 years or older) from 2015 to 2018 to perform multilevel logistic regression. A PIM prescription was classified as initial or refill on the basis of medication history 1 year before a visit. PIM use after an outpatient visit was the primary study outcome.

We included 9 000 224 visits in 2016 and 9 310 261 in 2018. The PIM prescription rate was lower in states with full NP practice and lower among NPs than among physicians; these rates for both physicians and NPs decreased from 2016 to 2018.

Changes could be due to individual state practices.

Changes could be due to individual state practices.

In pediatric patients, the presentation of the nontraumatic acutely painful joint/limb poses a diagnostic dilemma due to the similarity of presentations of the most likely diagnoses [septic arthritis (SA), transient synovitis (TS), osteomyelitis]. Current tools employed to differentiate these diagnoses rely on nonspecific inflammatory markers, radiologic imaging, and arthrocentesis. Diagnostic algorithms utilizing these clinical, radiographic, and biochemical parameters have produced conflicting results. The purpose of this study was to identify a serum-based inflammatory signature which can differentiate SA from TS in pediatric patients.

Serum samples were collected from 22 pediatric patients presenting with joint/extremity pain whose working diagnosis included SA or TS. Each sample was analyzed for serum abundance of 72 distinct biomarkers and cytokines using enzyme linked immunosorbent assay based arrays. Linear discriminant analysis was performed to identify a combinatorial biomarker panel to predict l that accurately differentiates SA from TS in pediatric patients with joint/limb pain.

Level II-diagnostic study.

Level II-diagnostic study.Pierre Auguste Cot's painting, "Mireille giving alms at the door of Saint-Trophime," depicts a wealthy lady giving alms to a crippled beggar boy. The beautiful painting portrays the striking contrast between the rich and the poor. The painting also draws attention to the crude crutch the boy was using. This type of crutch was used over the centuries; improvements in the design of the axillary crutch occurred only in the 20th century.

Lyme arthritis often presents as acute monoarticular arthritis challenging to distinguish from septic arthritis. Typical management for Lyme arthritis entails antibiotic therapy, while septic arthritis usually warrants operative debridement. During the period when Western Pennsylvania transitioned to a Lyme-endemic region, many children underwent operative intervention who were ultimately diagnosed with Lyme arthritis due to diagnostic ambiguity. We examined the impact of the operative intervention on pediatric Lyme arthritis outcomes.

We conducted a retrospective cohort study of patients admitted to a tertiary care children's hospital who were diagnosed with Lyme arthritis from 2008 to 2018 using chart review. Inclusion criteria were positive Lyme serology by Centers for Disease Control and Prevention (CDC) definition, clinical arthritis, and negative bacterial cultures. We recorded clinical presentation, laboratory data, details of hospitalization, costs, and outcomes after therapy to compare the impact of antibiotics alone (nonoperative group) versus antibiotics plus operative debridement (operative group).

A total of 149 patients met the inclusion criteria. Overall, 47 (32%) patients underwent orthopaedic intervention. Operative management was associated with increased length (3.17 vs. 1.40 d) and cost ($27,850 vs. $10,716) of admission. The clinical resolution was documented in 57/58 patients (98%) in the nonoperative group and 41/42 patients (98%) in the operative group. The median duration to resolution was 21 days for both groups.

Operative management of pediatric patients with Lyme arthritis is associated with increased resource utilization and costs while being similarly efficacious to nonoperative management. As the US Lyme epidemic expands, improved diagnosis and management of acute undifferentiated arthritis may prevent unneeded operative intervention.

Level III-retrospective cohort study.

Level III-retrospective cohort study.Mucosal melanoma is a rare but devastating subtype of melanoma which typically has a worse prognosis than other melanoma subtypes. Large-scale next-generation sequencing studies, including our recent research, have also proved that the molecular landscape and potential oncogenic drivers of mucosal melanoma remain distinct from that of cutaneous melanoma. Recently, a number of selective cyclin-dependent kinase 4 (CDK4)/6 inhibitors have been approved for clinical application in breast cancer or entered phase III clinical trial in other solid tumors. Additionally, we have revealed that the dysregulation of cell cycle progression, caused by CDK4 amplification, is a key genetic feature in half of mucosal melanoma and targeting of CDK4 in selected mucosal melanoma patients is a potentially promising direction for precision cancer treatment by using molecular-characterized mucosal melanoma patient-derived-xenograft models. This review summarizes the current literature regarding CDK4/6 dysregulation in mucosal melanoma, preclinical and clinical studies of CDK4/6 inhibitors and potential combinational strategies in treating mucosal melanoma.

To define the symptomatology of SARS-CoV-2 infection in pregnancy and associations between occupation, sociodemographic factors, and comorbidities with the severity of COVID-19 disease in pregnancy in all trimesters, regardless of hospitalization.

We studied a retrospective cohort of a public health surveillance sample of persons with COVID-19 infection diagnosed during pregnancy. Data was collected March 2020-August 2020 regarding symptoms, disease severity, comorbidities, obstetric history, and occupation.

163 individuals were identified. Constitutional (64%) and lower respiratory symptoms (61%) were most common. Seventeen individuals (13.6%) were hospitalized, and one person (0.7%) died due to COVID-19. Risk factors for severe disease were age and an occupation that had high intensity exposure to people.

Occupational exposure is a risk factor for severe COVID-19 disease in pregnancy, justifying policy measures to ensure protection of this vulnerable population.

Occupational exposure is a risk factor for severe COVID-19 disease in pregnancy, justifying policy measures to ensure protection of this vulnerable population.

The aim of this study was to describe the self-reported physical and mental health over the course over 19 years of follow up of a population-based cohort of Gulf War and Gulf Era veterans.

A multi-modal health survey of 6338 Gulf War and Gulf Era veterans who participated in all three waves of the longitudinal study.

Gulf War and Gulf War Era veterans experienced an increase in prevalence of chronic disease over time. The adjusted odds ratios suggest that Gulf War veterans not only had significantly higher odds of reporting medical conditions, but also began to report them earlier.

The findings from this analysis suggest that Gulf War veterans are not only more likely than their non-deployed counterparts to report chronic disease, they were more likely to report it earlier.

The findings from this analysis suggest that Gulf War veterans are not only more likely than their non-deployed counterparts to report chronic disease, they were more likely to report it earlier.