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We evaluated a protocolized endoscopic necrosectomy approach with a lumen-apposing metal stent (LAMS) in patients with large symptomatic walled-off pancreatic necrosis (WON) comprising significant necrotic content, with or without infection.Randomized trials have shown similar efficacy of endoscopic treatment compared to surgery for infected WON.We conducted a regulatory, prospective, multicenter single-arm clinical trial examining the efficacy and safety of endoscopic ultrasound (EUS)-guided LAMS with protocolized necrosectomy to treat symptomatic WON ≥ 6 cm in diameter with 30% solid necrosis. After LAMS placement, protocolized WON assessment was conducted and endoscopic necrosectomy was performed for insufficient WON size reduction and persistent symptoms. Patients with radiographic WON resolution to ≤ 3 cm and/or 60-day LAMS indwell had LAMS removal, then 6-month follow-up. Primary endpoints were probability of radiographic resolution by 60 days and procedure-related serious adverse events (SAEs).Forty consecutive patients were enrolled September 2018 - March 2020, of whom 27 (67.5%) were inpatients and 19 (47.5%) had clinical evidence of infection at their index procedure. Mean WON size was 15.0 ± 5.6 cm with mean 53.2% ± 16.7% solid necrosis. Radiographic WON resolution was seen in 97.5% (95% CI, 86.8%, 99.9%) by 60 days, without recurrence in 34 patients with 6-month follow-up data. Mean time to radiographic WON resolution was 34.1 ± 16.8 days. SAEs occurred in 3 patients (7.5%), including sepsis, vancomycin-resistant enterococcal bacteremia and shock, and upper gastrointestinal bleeding. There were no procedure-related deaths.EUS-guided drainage with protocolized endoscopic necrosectomy to treat large symptomatic or infected walled-off necrotic pancreatic collections was highly effective and safe. Clinicaltrials.gov no: NCT03525808.

An 8-year-old girl presented with precocious puberty and an otherwise normal examination. MRI of the pituitary was normal; however, an incidental oval, non-enhancing T2 hyperintense lesion in the right temporal subcortical white matter was identified ([figure 1][1]). The neuro-radiographic

A 6-year-old boy presented for evaluation after a new onset focal seizure. CT demonstrated a large right hemispheric mass with midline shift ([figure 1A][1]). MRI confirmed a T2 hyperintense tumour with reduced diffusivity, minimal enhancement on postgadolinum sequences, and linear calcifications on

The optimal therapeutic approach for walled-off necrosis (WON) is not fully understood, given the lack of a validated classification system. We propose a novel and robust classification system based on radiologic and clinical factors to standardize the nomenclature, provide a framework to guide comparative effectiveness trials, and inform the optimal WON interventional approach.This was a retrospective analysis of patients who underwent endoscopic management of WON by lumen-apposing metal stent placement at a tertiary referral center. Patients were classified according to the proposed QNI classification system: quadrant ("Q"), represented an abdominal quadrant distribution; necrosis ("N"), denoted by the percentage of necrosis of WON; and infection ("I"), denoted as positive blood culture and/or systemic inflammatory response syndrome reaction with a positive WON culture. Two blinded reviewers classified all patients according to the QNI system. Patients were then divided into 2 groups: those with a lower QNI stratification (≤2 quadrants and ≤30% necrosis; group 1) and those with a higher stratification (≥3 quadrants, 2 quadrants with ≥30% necrosis, or 1 quadrant with60% necrosis and infection; group 2). The primary outcome was mean time to WON resolution. Secondary procedural and clinical outcomes between the groups were compared.Seventy-one patients (75% men) were included and stratified by the QNI classification; group 1 comprised 17 patients and group 2, 54 patients. Patients in group 2 had a higher number of necrosectomies, longer hospital stays, and more readmissions. The mean time to resolution was longer in group 2 than in group 1 (79.6 ± 7.76 days vs 48.4 ± 9.22 days, P = .02). The mortality rate was higher in group 2 (15% vs 0%, P = .18).Despite the heterogeneous nature of WON in severe acute pancreatitis, a proposed QNI system may provide a standardized framework for WON classification to inform clinical trials, risk-stratify the disease course, and potentially inform an optimal management approach.

A 16‐year‐old boy with negative past medical history presented to an outside hospital with a first time right‐sided focal seizure. Neurologic examination at presentation was normal. CT performed in the emergency room revealed a large left intra‐axial cerebral mass without haemorrhage ([

Background and study aims Gastric outlet obstruction (GOO) is traditionally managed with surgical gastroenterostomy (surgical-GE) and enteral stenting (ES). Endoscopic ultrasound-guided gastroenterostomy (EUS-GE) is now a third option. Large studies assessing their relative risks and benefits with adequate follow-up are lacking. We conducted a comparative analysis of patients who underwent EUS-GE, ES, or surgical-GE for GOO. Patients and methods In this retrospective comparative cohort study, consecutive patients presenting with GOO who underwent EUS-GE, ES, or surgical-GE at two academic institutions were reviewed and independently cross-edited to ensure accurate reporting. The primary outcome was need for reintervention. Secondary outcomes were technical and clinical success, length of hospital stay (LOS), and adverse events (AEs). Results A total of 436 patients (232 EUS-GE, 131 ES, 73 surgical-GE) were included. The median duration of follow-up of the entire cohort was 185.5 days (interquartile range 55.25–454.25 days). The rate of reintervention in the EUS-GE group was lower than in the ES and surgical-GE groups (0.9 %, 12.2 %, and 13.7 %, P Conclusion This large cohort study demonstrates the safety and palliation durability of EUS-GE as an alternative strategy for GOO palliation in select patients.

What is the relationship between oocyte donor characteristics and their pain perception, their expectation and experience of pain, and the interaction between pain and overall satisfaction with the donation process?Institutional Review Board approved, retrospective survey of commercial, US oocyte donors was emailed to recent donors recruited through Donor Egg Bank USA before 2020.Of the 503 opened emails, 246 individuals responded (48.9%). Most donors ranked their pain between minimal and moderate at all time points analysed. Altruistically motivated donors and parous donors were less likely to experience unexpected levels of pain. A high proportion (93.9%) of donors reported being informed of the risk of pain. Those who recalled being informed of the risk of pain were less likely to report higher levels of pain than expected. Although 94.2% of donors reported having an average to positive experience overall, 92.3% (12 out of 13 donors) in the group reporting a negative overall experience said they felt more pain than expected.Donors were well informed about the risk of pain. Altruistically motivated donors and parous donors were less likely to experience unexpected levels of pain. Report of unexpected levels of pain was highly related to low donor satisfaction.

Endoscopic ultrasound (EUS)-guided main pancreatic duct (PD) access may be used when conventional endoscopic retrograde cholangiopancreatography (ERCP) techniques fail. The use of a percutaneous transluminal angioplasty balloon (PTAB), originally developed for vascular interventions, can be used to facilitate transmural (To describe the technique, efficacy, and safety of PTABs for EUS-guided PD interventions.Patients who underwent EUS with use of a PTAB from March 2011 to August 2021 were retrospectively identified from a tertiary care medical center supply database. PTABs included 3-4 French angioplasty catheters with 3-4 mm balloons designed to use over a 0.018-inch guidewire. The primary outcome was technical success. Secondary outcomes included incidence of adverse events (AEs) and need for early reintervention.A total of 23 patients were identified (48% female, mean age 55.8 years). Chronic pancreatitis was the underlying etiology in 13 (56.5%) patients, surgically altered anatomy (SAA) with stricture in 7 (30.4%), and SAA with post-operative leak in 3 (13.0%). Technical success was achieved in 20 (87%) cases. Overall AE rate was 26% (EUS-guided use of PTABs for PD access and/or stricture management is feasible with an acceptable safety profile and can be considered in patients when conventional ERCP cannulation fails.

An 8-year-old boy with no significant medical history or family history presented with 1–2 weeks of decreased visual acuity and blurry vision. The patient denied any headaches, vomiting, dizziness, seizures or weakness. Physical examination was significant for poorly reactive pupils, constricted

Localized insulinoma is an uncommon entity that can result in substantial morbidity due to the associated hypoglycemia. Recent studies have suggested an increase in the incidence of insulinoma in recent decades that may possibly be secondary to increased awareness, incidental diagnoses, and better diagnostic methods. Diagnosing and localizing insulinoma within the pancreas can be challenging, but advances in nuclear imaging may improve diagnostic accuracy. Delays in diagnosis are common, but once a localized insulinoma is diagnosed and appropriately treated, the long-term prognosis is excellent. Surgical resection is considered the standard of care management option for localized insulinoma, but tumor ablation with endoscopic ultrasound guidance has also been shown to be an effective and safe method for therapy.

BACKGROUND A limited number of studies have investigated the presence of ongoing disease activity independent of clinical relapses in neuromyelitis optica spectrum disorder (NMOSD), and data are conflicting. The objective of our study was to examine whether patients with aquaporin-4 (AQP4)-IgG seropositive NMOSD exhibit progressive retinal neuroaxonal loss, independently of optic neuritis (ON) attacks. METHODS In this single-center, longitudinal study, 32 AQP4-IgG+ NMOSD patients and 48 healthy controls (HC) were followed with serial spectral-domain optical coherence tomography and visual acuity (VA) assessments. NMOSD patients with ON less than 6 months before baseline were excluded, whereas data from patients with ON during follow-up were censored at the last visit before ON. VA worsening was defined as a decrease in monocular letter acuity ≥5 letters for high-contrast VA and ≥7 letters for low-contrast VA. Analyses were performed with mixed-effects linear regression models adjusted for age, sex, and race. RESULTS The median follow-up duration was 4.2 years (interquartile range: 1.8-7.5). Relative to HC, NMOSD eyes had faster peripapillary retinal nerve fiber layer (pRNFL) (β = -0.25 µm/year faster, 95% confidence interval [CI]: -0.45 to -0.05, P = 0.014) and GCIPL thinning (β = -0.09 µm/year faster, 95% CI: -0.17 to 0, P = 0.05). This difference seemed to be driven by faster pRNFL and GCIPL thinning in NMOSD eyes without a history of ON compared with HC (GCIPL: β = -0.15 µm/year faster; P = 0.005; pRNFL: β = -0.43 µm/year faster, P < 0.001), whereas rates of pRNFL (β: -0.07 µm/year, P = 0.53) and GCIPL (β = -0.01 µm/year, P = 0.90) thinning did not differ between NMOSD-ON and HC eyes. Nine NMOSD eyes had VA worsening during follow-up. CONCLUSIONS In this longitudinal study, we observed progressive pRNFL and GCIPL atrophy in AQP4-IgG+ NMOSD eyes unaffected by ON. These results support that subclinical involvement of the anterior visual pathway may occur in AQP4-IgG+ NMOSD.

Endoscopic therapy is a key component in the management algorithm for variceal and nonvariceal hemorrhage related to portal hypertension. Band ligation is the endoscopic treatment of choice for acute esophageal variceal bleeding, and for preventing a first episode of bleeding (primary prophylaxis) and rebleeding (secondary prophylaxis). In contrast, endoscopic cyanoacrylate injection is the preferred modality for cardio-fundal variceal bleeding. Thrombin injection and EUS-guided angiotherapy are alternative treatment options for gastric variceal obliteration. The management of ectopic varices requires a multidisciplinary approach, and the choice of therapy is dictated, in part, by location of varices, vascular anatomy and available expertise. Endoscopic therapy is of limited value for the control of chronic blood loss from portal hypertensive gastropathy but is at the forefront in the management of gastric antral vascular ectasia associated with portal hypertension, which includes argon plasma coagulation, band ligation and radiofrequency ablation.

Background & aims Increased intrapancreatic fat is associated with pancreatic diseases; however, there are no established objective diagnostic criteria for fatty pancreas. On non-contrast computed tomography (CT), adipose tissue shows negative Hounsfield Unit (HU) attenuations (−150 to −30 HU). Using whole organ segmentation on non-contrast CT, we aimed to describe whole gland pancreatic attenuation and establish 5th and 10th percentile thresholds across a spectrum of age and sex. Subsequently, we aimed to evaluate the association between low pancreatic HU and risk of pancreatic ductal adenocarcinoma (PDAC). Methods The whole pancreas was segmented in 19,456 images from 469 non-contrast CT scans. A convolutional neural network was trained to assist pancreas segmentation. Mean pancreatic HU, volume, and body composition metrics were calculated. The lower 5th and 10th percentile for mean pancreatic HU were identified, examining the association with age and sex. Pre-diagnostic CT scans from patients who later developed PDAC were compared to cancer-free controls. Results Less than 5th percentile mean pancreatic HU was significantly associated with increase in BMI (OR 1.07; 1.03–1.11), visceral fat (OR 1.37; 1.15–1.64), total abdominal fat (OR 1.12; 1.03–1.22), and diabetes mellitus type 1 (OR 6.76; 1.68–27.28). Compared to controls, pre-diagnostic scans in PDAC cases had lower mean whole gland pancreatic HU (−0.2 vs 7.8, p = 0.026). Conclusion In this study, we report age and sex-specific distribution of pancreatic whole-gland CT attenuation. Compared to controls, mean whole gland pancreatic HU is significantly lower in the pre-diagnostic phase of PDAC.

Evidence on the impact of human immunodeficiency virus (HIV) drug resistance on regimens following treatment failure is varied and inconclusive. Differential medication adherence may explain this variation. We aimed to test the association between drug resistance at first-line antiretroviral therapy (ART) switch and adherence to and virologic failure on subsequent ART. We conducted a secondary analysis of data from an open-labeled randomized trial of second-line ART (ACTG A5234). ART susceptibility was determined from study entry plasma using the Stanford Drug Resistance database version 8.7. Adherence was measured with microelectronic monitors. Three adherence variables and rates of virologic failure (HIV-1 RNA ≥1000 copies/mL) on second-line ART were compared between participants with and without resistance at first-line ART failure. Of 214 participants switching to second-line ART with baseline resistance results, 113 (53%) were men, mean age was 39 years (standard deviation 10.3), and 37 (17%) had susceptible virus at study entry. Cumulative genotypic susceptibility score (cGSS) was inversely associated with adherence, adjusted odds ratio (aOR) 0.15, 95% confidence interval (CI) (0.05–0.40), p

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease defined by attacks on the central nervous system that cause irreversible damage. Recent approval of NMOSD therapies warrants investigations of comparative efficacy to inform treatment decisions. A network meta-analysis (NMA) of all U.S. Food and Drug Administration-approved therapies (eculizumab, inebilizumab, and satralizumab) for adults with aquaporin-4 immunoglobulin G-positive (AQP4+) NMOSD was conducted via a systematic literature review (SLR) using data from randomized controlled trials (RCTs). Database searches of MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials were executed for the SLR. A fixed-effects proportional hazards Bayesian NMA was used to estimate relative treatment effects based on data extracted from RCTs identified during the SLR (search end date: 11 September 2020). Four unique RCTs (N-MOmentum, PREVENT, SAkuraSky, and SAkuraStar) were identified, and data from 29 publications were extracted for analysis. Network scenarios describing the most comparable patient population groups (such as by treatment settings) were evaluated in our analyses. Relative treatment effects were evaluated based on time-to-first relapse and were expressed as hazard ratios (HRs) with 95% credible intervals (CrIs). In patients treated with a monoclonal antibody only, eculizumab was associated with a lower risk of relapse compared with satralizumab (HR 0.10, 95% CrI 0.01, 0.65) and inebilizumab (HR 0.11, 95% CrI 0.02, 0.68). In patients treated with monoclonal antibody with or without background immunosuppressive therapy (IST), patients treated with eculizumab ± IST were also less likely to relapse than patients treated with satralizumab ± IST (HR 0.24, 95% CrI 0.06, 0.98). The NMA results suggest that complement component 5 (C5) inhibition prevents NMOSD relapses more effectively than broader mechanisms of action. Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease characterized by inflammation that damages the brain and spinal cord. Many patients with NMOSD produce antibodies against a protein called aquaporin-4 (AQP4+). In the past two years, three drugs (eculizumab, inebilizumab, and satralizumab) have been approved by the U.S. Food and Drug Administration for the treatment of adults with AQP4+ NMOSD. Comparing the efficacy of these three drugs would help physicians make treatment decisions for their patients. In the absence of clinical trials directly comparing these three drugs, we conducted a Bayesian network meta-analysis in order to allow for simultaneous comparisons of these three drugs and estimate relative treatment effects between any pair of interventions in a connected network. With a Bayesian methodology, it is also possible to estimate the probability of being the best treatment out of all other interventions in a connected network. While all three drugs are safe and shown to prevent relapses in placebo-controlled trials, the results of our analysis suggests that eculizumab was the most efficacious in preventing relapses when compared with inebilizumab or satralizumab. These findings may help to inform physicians and their patients when determining the best treatment option for preventing the occurrence of relapses in adults with AQP4+ NMOSD.

Background & Aims: Duodenoscope-associated transmission of infections has raised questions about efficacy of endoscope reprocessing using high-level disinfection (HLD). Although ethylene oxide (ETO) gas sterilization is effective in eradicating microbes, the impact of ETO on endoscopic ultrasound (EUS) imaging equipment remains unknown. In this study, we aimed to compare the changes in EUS image quality associated with HLD vs HLD followed by ETO sterilization. Methods: Four new EUS instruments were assigned to 2 groups: Group 1 (HLD) and Group 2 (HLD + ETO). The echoendoscopes were assessed at baseline, monthly for 6 months, and once every 3 to 4 months thereafter, for a total of 12 time points. At each time point, review of EUS video and still image quality was performed by an expert panel of reviewers along with phantom-based objective testing. Linear mixed effects models were used to assess whether the modality of reprocessing impacted image and video quality. Results: For clinical testing, mixed linear models showed minimal quantitative differences in linear analog score (P =. 04; estimated change, 3.12; scale, 0–100) and overall image quality value (P =. 007; estimated change, −0.12; scale, 1–5) favoring ETO but not for rank value (P =. 06). On phantom testing, maximum depth of penetration was lower for ETO endoscopes (P

Neuromyelitis optica spectrum disorder (NMOSD) is a rare relapsing autoimmune disease of the central nervous system that preferentially targets the optic nerves and spinal cord, leading to visual loss and impaired mobility. Until 2019, no medications were FDA-approved for NMOSD treatment, and standard of care was based on mostly empiric and retrospective data. Therapies that target B cells emerged as a treatment strategy due to their fundamental role in disease pathogenesis. We explore different monoclonal antibodies directed at either CD20+ or CD19+ B cells that may have utilization in the treatment of NMOSD, discussing what is known regarding their efficacy and safety.

Introduction Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune condition characterized by unpredictable relapses that affect the optic nerves and spinal cord, which can lead to blindness, paralysis, and increased mortality rates. Evidence on the clinical and economic burden of NMOSD in the USA is currently lacking. Methods A retrospective, observational cohort study was conducted using data from the IQVIA PharMetrics Plus Healthcare Claims Database between January 1, 2012 and March 31, 2019. Adults (aged 18 years or more) with evidence of NMOSD and a matched group of comparison patients were identified. Outcomes, including NMOSD relapses, healthcare utilization, and healthcare expenditure (reported in 2018 US dollars), were evaluated during the follow-up period (maximum 6 years). Healthcare utilization and expenditure were assessed overall (all-cause) and during NMOSD relapses. Results The study included 1363 patients with NMOSD; the mean age was 44.9 years, and 75.3% were female. During the follow-up period (median 2.0 years), 47.7% of patients with NMOSD had one or more relapses, corresponding to an annualized relapse rate of 0.8 (95% confidence interval [CI] 0.7–0.9). When analyzing healthcare expenditure per patient, the mean annualized all-cause healthcare expenditure among patients with NMOSD was $60,599 (95% CI $52,112–66,716) compared with $8912 (95% CI $7084–10,727) among comparison patients, representing a difference of $51,687 (95% CI $43,820–58,664) attributable to NMOSD. The mean annualized total expenditure for NMOSD relapses was $10,070 (95% CI $7726–12,660) per patient, with hospital/inpatient care requiring more expenditure than ambulatory/outpatient care. Conclusion Findings of this large, retrospective, observational study indicate that relapses among patients with NMOSD are common in US clinical practice, leading to substantial healthcare utilization and expenditure. Therapies with the highest relapse risk reduction could lead to markedly lower relapse-associated healthcare utilization and clinical burden in patients with NMOSD. Supplementary Information The online version contains supplementary material available at 10.1007/s40120-021-00253-4., Plain Language Summary Neuromyelitis optica spectrum disorder (NMOSD) is a severely debilitating neurological disease that affects the nerves in the brain and spinal cord. People who have NMOSD may experience recurrent attacks, or relapses, that can cause blindness and disability. These relapses may lead to hospitalizations, doctor’s office visits, and pharmacy costs that are paid by health insurance plans. Overall, the cost of treating relapses in patients with NMOSD is substantial. Our study analyzed healthcare claims data from the USA. During a median follow-up time of 2.0 years, our study showed that 47.7% of patients with NMOSD experienced one or more relapses, resulting in hospital/inpatient admissions and ambulatory/outpatient treatments. In addition, the average healthcare cost among patients with NMOSD was $60,599 per year compared with $8912 per year for patients without NMOSD. This represents a difference of $51,687 per year, which can be attributed to NMOSD. Among patients with three or more relapses during the follow-up period, the average total healthcare cost was more than $83,000 per patient. Therefore, medicines that prevent relapses could lead to fewer relapse-associated hospitalizations and outpatient treatments for patients with NMOSD. Supplementary Information The online version contains supplementary material available at 10.1007/s40120-021-00253-4.

Massive unemployment during the COVID-19 pandemic could result in an eviction crisis in US cities. Here we model the effect of evictions on SARS-CoV-2 epidemics, simulating viral transmission within and among households in a theoretical metropolitan area. We recreate a range of urban epidemic trajectories and project the course of the epidemic under two counterfactual scenarios, one in which a strict moratorium on evictions is in place and enforced, and another in which evictions are allowed to resume at baseline or increased rates. We find, across scenarios, that evictions lead to significant increases in infections. Applying our model to Philadelphia using locally-specific parameters shows that the increase is especially profound in models that consider realistically heterogenous cities in which both evictions and contacts occur more frequently in poorer neighborhoods. Our results provide a basis to assess eviction moratoria and show that policies to stem evictions are a warranted and important component of COVID-19 control., Massive unemployment during the COVID-19 pandemic could result in an eviction crisis in US cities. Here, the authors model the effect of evictions on SARS-CoV-2 epidemics, simulating viral transmission within and among households in a theoretical and applied urban settings.

A crucial mutational mechanism in malignancy is structural variation, in which chromosomal rearrangements alter gene functions that drive cancer progression. Herein, the presence and pattern of structural variations were investigated in twelve prospectively acquired treatment‐naïve pancreatic cancers specimens obtained via endoscopic ultrasound (EUS). In many patients, this diagnostic biopsy procedure and specimen is the only opportunity to identify somatic clinically relevant actionable alterations that may impact their care and outcome. Specialized mate pair sequencing (MPseq) provided genome‐wide structural variance analysis (SVA) with a view to identifying prognostic markers and possible therapeutic targets. MPseq was successfully performed on all specimens, identifying highly rearranged genomes with complete SVA on all specimens with > 20% tumour content. SVA identified chimeric fusion proteins and potentially immunogenic readthrough transcripts, change of function truncations, gains and losses of key genes linked to tumour progression. Complex localized rearrangements, termed chromoanagenesis, with broad pattern heterogeneity were observed in 10 (83%) specimens, impacting multiple genes with diverse cellular functions that could influence theragnostic evaluation and responsiveness to immunotherapy regimens. This study indicates that genome‐wide MPseq can be successfully performed on very limited clinically EUS obtained specimens for chromosomal rearrangement detection and potential theragnostic targets.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory demyelinating disorder of the central nervous system (CNS). Aquaporin-4 (AQP4) antibodies in the serum are highly specific for the diagnosis of NMOSD, but the sensitivity remains under 90% allowing for diagnosis of AQP4 IgG seronegative disease. It remains of crucial importance to identify seronegative NMOSD myelitis as early as the first attack to initiate long-term treatment that will reduce future relapses and disability and to avoid potentially harmful treatments such as those of multiple sclerosis (MS). Over the years, many spinal imaging features have been reported to favour the diagnosis of NMOSD, but only longitudinally extensive transverse myelitis (LETM) was specific enough to make the diagnostic criteria in the AQP4 IgG seronegative cases. Bright spotty lesions (BSLs), which are defined as hyperintense lesions on axial T2-weighted images and sometimes associated with T1 low signal, are now reported to have a higher specificity and sensitivity compared to LETM in predicting a diagnosis of NMOSD against other causes of myelitis. In the review, we aim to highlight the position of BSLs in diagnosing NMOSD as well as its possible role as a prognostic factor for the clinical outcome.

Background: Neuromyelitis Optica Spectrum Disorder (NMOSD) is a chronic autoimmune disease of the central nervous system that causes recurrent attacks of optic neuritis, myelitis, and brainstem symptoms, resulting in severe neurological disability. Preventive treatment with immunosuppressive agents reduces relapse rate and improves long-term prognosis. In recent years, the potential therapeutical effect of new agents has been investigated. Two of these, the anti-interleukin 6 (IL-6) agents tocilizumab and satralizumab, have been studied in active NMOSD. Objective: To systematically review the current data regarding the efficacy and safety of anti-IL-6 agents in NMOSD. Result: Fourteen case reports and 5 case series of intravenous tocilizumab have shown beneficial clinical and paraclinical effects compared to commonly used therapies, and another case series of subcutaneous tocilizumab has shown it is as effective as the IV formulation. A phase 2 comparative trial has shown tocilizumab IV to be more effective than azathioprine for relapse prevention. A phase 3 trial of subcutaneous satralizumab versus placebo, has shown a lower risk of relapse in the sartralizumab-treated group, both as add-on therapy to stable immunosuppressant and as monotherapy. Tocilizumab also reduced pain severity in two trials and fatigue scores in one trial, but satralizumab did not significantly improve pain and fatigue. Adverse events with both agents were relatively mild and comparable to placebo and azathioprine. Conclusions: The anti-Il-6 agents tocilizumab and satralizumab show promising results in active NMOSD. Further randomized, larger-scale trials are needed to better define the role of these agents in the growing arsenal of NMOSD treatments.

Research question Ovarian stimulation during IVF cycles involves close monitoring of oestradiol, progesterone and ultrasound measurements of follicle growth. In contrast to blood draws, sampling saliva is less invasive. Here, a blind validation is presented of a novel saliva-based oestradiol and progesterone assay carried out in samples collected in independent IVF clinics. Design Concurrent serum and saliva samples were collected from 324 patients at six large independent IVF laboratories. Saliva samples were frozen and run blinded. A further 18 patients had samples collected more frequently around the time of HCG trigger. Saliva samples were analysed using an immunoassay developed with Salimetrics LLC. Results In total, 652 pairs of saliva and serum oestradiol were evaluated, with correlation coefficients ranging from 0.68 to 0.91. In the European clinics, a further 237 of saliva and serum progesterone samples were evaluated; however, the correlations were generally poorer, ranging from –0.02 to 0.22. In the patients collected more frequently, five out of 18 patients (27.8%) showed an immediate decrease in oestradiol after trigger. When progesterone samples were assessed after trigger, eight out of 18 (44.4%) showed a continued rise. Conclusions Salivary oestradiol hormone testing correlates well to serum-based assessment, whereas progesterone values, around the time of trigger, are not consistent from patient to patient.

Compared to adult AVMs, there is a paucity of data on the microsurgical treatment of pediatric AVMs. We report our institutional experience with pediatric AVMs treated by microsurgical resection with or without endovascular embolization and radiation therapy. We retrospectively reviewed all patients ≤ 18 years of age with cerebral AVMs that underwent microsurgical resection at Rady Children’s Hospital 2002–2019. Eighty-nine patients met inclusion criteria. The mean age was 10.3 ± 5.0 years, and 56% of patients were male. In total, 72 (81%) patients presented with rupture. Patients with unruptured AVMs presented with headache (n = 5, 29.4%), seizure (n = 9, 52.9%), or incidental finding (n = 3, 17.7%). The mean presenting mRS was 2.8 ± 1.8. AVM location was lobar in 78%, cerebellar/brainstem in 15%, and deep supratentorial in 8%. Spetzler-Martin grade was I in 28%, II in 45%, III in 20%, IV in 6%, and V in 1%. Preoperative embolization was utilized in 38% of patients and more frequently in unruptured than ruptured AVMs (62% vs. 32%, p = 0.022). Radiographic obliteration was achieved in 76/89 (85.4%) patients. Complications occurred in 7 (8%) patients. Annualized rates of delayed rebleeding and recurrence were 1.2% and 0.9%, respectively. The mean follow-up was 2.8 ± 3.1 years. A good neurological outcome (mRS score ≤ 2) was obtained in 80.9% of patients at last follow-up and was improved relative to presentation for 75% of patients. Our case series demonstrates high rates of radiographic obliteration and relatively low incidence of neurologic complications of treatment or AVM recurrence.