Predictors of a relapsing course in myelin oligodendrocyte glycoprotein antibody-associated disease.

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described demyelinating disorder, and children represent about 50% of all cases. Almost half of the patients experience relapses, but very few studies have evaluated predictors of relapse risk, challenging clinical management. The study aimed to identify predictors at MOGAD onset that are associated with a relapsing course. METHODS: Prospectively collected data from paediatric patients with MOGAD seen by the US Network of Paediatric MS Centres were leveraged. Univariable and adjusted multivariable models were used to predict recurrent disease. RESULTS: We identified 326 MOGAD cases (mean age at first event 8.9 years [SD 4.3], 57% female, 77% white and 74% non-Hispanic) and 46% relapsed during a mean follow-up of 3.9 years (SD 4.1). In the adjusted multivariable model, female sex (HR 1.66, 95% CI 1.17 to 2.36, p=0.004) and Hispanic/Latino ethnicity (HR 1.77, 95% CI 1.19 to 2.64, p=0.005) were associated with a higher risk of relapsing MOGAD. Maintenance treatment initiated before a second event with rituximab (HR 0.25, 95% CI 0.07 to 0.92, p=0.037) or intravenous immunoglobulin (IVIG) (HR 0.35, 95% CI 0.14 to 0.88, p=0.026) was associated with lower risk of a second event in multivariable analyses. Conversely, maintenance steroids were associated with a higher estimated relapse risk (HR 1.76, 95% CI 0.90 to 3.45, p=0.097). CONCLUSION: Sex and ethnicity are associated with relapsing MOGAD. Use of rituximab or IVIG therapy shortly after onset is associated with a lower risk of the second event. Preventive treatment after a first event could be considered for those with a higher relapse risk.

Susceptibility-based imaging aids accurate distinction of pediatric-onset MS from myelin oligodendrocyte glycoprotein antibody-associated disease.

BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) and pediatric-onset multiple sclerosis (POMS) share clinical and magnetic resonance imaging (MRI) features but differ in prognosis and management. Early POMS diagnosis is essential to avoid disability accumulation. Central vein sign (CVS), paramagnetic rim lesions (PRLs), and central core lesions (CCLs) are susceptibility-based imaging (SbI)-related signs understudied in pediatric populations that may help discerning POMS from MOGAD. METHODS: T2-FLAIR and SbI (three-dimensional echoplanar imaging (3D-EPI)/susceptibility-weighted imaging (SWI) or similar) were acquired on 1.5T/3T scanners. Two readers assessed CVS-positive rate (%CVS+), and their average score was used to build a receiver operator curve (ROC) assessing the ability to discriminate disease type. PRLs and CCLs were identified using a consensual approach. RESULTS: The %CVS+ distinguished 26 POMS cases (mean age 13.7 years, 63% females, median EDSS 1.5) from 14 MOGAD cases (10.8 years, 35% females, EDSS 1.0) with ROC = 1, p < 0.0001, (cutoff 41%). PRLs were only detectable in POMS participants (mean 2.1±2.3, range 1-10), discriminating the two conditions with a sensitivity of 69% and a specificity of 100%. CCLs were more sensitive (81%) but less specific (71.43%). CONCLUSION: The %CVS+ and PRLs are highly specific markers of POMS. After proper validation on larger multicenter cohorts, consideration should be given to including such imaging markers for diagnosing POMS at disease onset.

Parenteral nutrition-related complications in older patients with acute intestinal failure: A descriptive cohort study.

INTRODUCTION: Reported outcomes for parenteral nutrition (PN)-related complications in older adult patients with acute intestinal failure who are receiving PN in the acute hospital setting are limited. Our study aims to compare PN-related complications between older and younger adult patients. METHODS: A retrospective descriptive study of inpatients who were administered PN from January 1, 2019, to December 31, 2019, was performed. Patients were categorized into older (≥65 years old) and younger (<65 years old) adult groups. RESULTS: Two hundred thirty-five patients were included. There were 103 patients in the older adult group (mean age: 73.9 [SD: 6.9] years) and 132 patients in the younger adult group (mean age: 52.4 [SD: 12.5] years). There was a significantly higher Charlson Comorbidity Index score and lower Karnofsky score in the older adult group. The older adult group received significantly lower total energy (20.8 [SD: 7.8] vs 22.8 [SD: 6.3] kcal/kg/day), dextrose (3.1 [SD: 1.4] vs 3.6 [SD: 1.4] g/kg/day), and protein (1.1 [SD: 0.4] vs 1.2 [SD: 0.3] g/kg/day) than the younger group received. The mean length of stay was significantly shorter in the older adult group (35.9 [SD: 21.3] vs 59.8 [SD: 55.3]; P < 0.05). There was no significant difference in PN-related complications and clinical outcomes (catheter-related bloodstream infections, hypoglycemia or hyperglycemia, fluid overload, or inpatient mortality) between the two groups. CONCLUSION: Despite more comorbidities in the older adult, the usage of PN in older adult patients with acute intestinal failure was associated with neither an increased rate of PN-related complications nor worse clinical outcomes when compared with that of younger patients.

High titers of myelin oligodendrocyte glycoprotein antibody are only observed close to clinical events in pediatrics.

BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-IgG is increasingly detected in children with CNS demyelinating diseases. Due to the clinical overlap in children with CNS demyelination with and without MOG-IgG positivity, identifying distinct characteristics would help early diagnosis. OBJECTIVE: To compare the specific features that may help differentiate MOG-IgG positive from negative children with CNS demyelinating diseases. To compare characteristics of patients with high and low MOG-IgG titers. METHODS: Children with CNS demyelinating disorders with onset before 18 years of age who were tested for MOG-IgG at the University of California San Francisco were included. This retrospective study collected the following by chart review: demographic, clinical, MRI, CSF, and treatment data. Serum was tested for MOG-IgG at Mayo Clinic by live cell-based fluorescent activated cell sorting assay with titer ≥1:20 confirming positivity. RESULTS: We assessed 65 Mog-IgG positive and 65 MOG-IgG negative patients. Median (IQR) age of onset was 7.6 (6.6) years for MOG-IgG positive and 13.8 (5.8) years for MOG-IgG negative (p<0.001). The female to male ratio was approximately 1:1 for the MOG-IgG positive group and 3:1 for the negative group (p=0.042). The most common initial diagnosis was demyelinating disease not otherwise specified (52.3%) in the positive group, compared to relapsing-remitting multiple sclerosis (41.5%) in the negative group (p<0.01). Optic nerve involvement (52.3%) was the most common clinical localization at onset for the MOG-IgG positive group, while brainstem/cerebellar (49.2%) localization predominated in the MOG-IgG negative group. The positive group also presented more often with a severe event at disease onset than the negative group (81.5% vs 60.3%; p< 0.002). MOG-IgG positive children had a lower frequency of oligoclonal bands (15.8% vs 57.4%; p<0.001). The frequency of baseline brain and spinal cord MRI abnormalities were similar in both groups; however, MOG-IgG positive patients more often had T2 hyperintense lesions in the optic nerves (26/43 vs 10/41; p<0.001). Disease-modifying medications were used in 64.6% of MOG-IgG positive patients versus 80% of negative children. Of the 32 positive patients with follow-up titers, seven reverted to negative while two who tested negative initially converted to positive. Positive titers greater than 1:160 were only observed within four months of a clinical event (disease onset or relapse). Patients with high and low MOG-IgG titers were comparable in demographic and clinical characteristics. CONCLUSION: Despite some clinical overlap, we report notable demographic, MRI and CSF differences between MOG-IgG positive and negative children with CNS demyelinating disorders at disease onset. High MOG-IgG titers were only observed close to a clinical event.

A Descriptive Study of enteral tube feeding among adults in an acute care tertiary hospital-patient selection, characteristics and complications.

BACKGROUND: It is vital to develop a better understanding of the use of different modalities for enteral feeding and its associated complications, given differences in funding support, community resources and infrastructure available to support home enteral feeding in an acute care tertiary hospital. AIM: To provide a description of the clinical characteristics of patients on long-term enteral feeding and incidence of associated complications. METHODS: A retrospective case records review study design was adopted. Medical records of patients discharged from a tertiary hospital with long-term nasogastric tube (NGT) or percutaneous endoscopic gastrostomy (PEG) feeding for the first time during the period of January 2010 to June 2017 were reviewed. Data collected include patient's demographics, reason for enteral feeding, morbidity and nutritional status upon initiation of NGT and PEG feeding, readmission episodes and documented complications (associated with enteral feeding) within one-year post discharge. RESULTS: Records of 120 NGT and 118 PEG patients were analysed. Significant age and gender differences were found with older patients being more likely to be placed on NGT [NGT (Mean 79.1, SD 11.3) vs. PEG (Mean 67.1, SD 12.6)] and higher number of females in the NGT group as compared to the PEG group (NGT 59.2% vs. PEG 31.4%). Majority of patients were fed by caregivers in the NGT (99.2%) as compared to the PEG (51.7%) group. Patients with cancer were more likely to be on PEG feeding (NGT 5%, PEG 70.3%), whereas patients with stroke-related diagnoses were more likely to be on NGT feeding (NGT 48% vs. PEG 8.5%). The total Charlson Comorbidity score was also significantly different between the NGT (mean = 5.7; SD = 1.5) and PEG (mean = 4.5; SD = 2.0) groups. A higher number of patients with PEG feeding had no complications (47.5%) as compared to the NGT group (8.3%). Patients who received NGT feeding were more likely experience tube blockage [OR 0.03, 95% CI (0.001-0.72), p = 0.03], secondary displacement of tube [OR 0.04, 95% CI (0.002-0.72), p = 0.03] and accidental tube removal [OR 0.03, 95% CI (0.004-0.21), p < 0.001]. CONCLUSION: Overall, patients who received NGT feeding experienced more complications than those who had PEG feeding. The choice for NGT or PEG feeding may be influenced by patient related factors as well as the presence of caregivers, which need to be considered in the improvement of enteral nutrition services in the local context.

A retrospective cohort study of plasma exchange in central nervous system demyelinating events in children.

BACKGROUND: Plasma exchange (PLEX) may improve recovery of acute central nervous system (CNS) demyelinating events related to multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), transverse myelitis (TM), acute disseminated encephalomyelitis (ADEM), and MOG-antibody associated demyelination (MOG) if recovery with pulse steroids (PS) is incomplete. Although there is a single randomized controlled trial in adults, there are limited case series in children. We aimed to describe the effectiveness and safety of PLEX in children with acute events of MS, NMOSD, TM, ADEM, and MOG with limited improvement after PS. METHODS: This was a retrospective cohort study of children with acute CNS demyelinating events seen at a single tertiary referral center who received PLEX as a second- or third-line therapy between 2006 and 2018. Through chart review of clinical notes, presence of clinical improvement by physician assessment was recorded pre- and post-PS and pre- and post-PLEX. Expanded Disability Status Scale (EDSS) scores were collected pre- and post-PLEX. We evaluated the number who improved clinically with PLEX and compared pre- and post-PLEX EDSS with Wilcoxon matched pairs signed-rank test. RESULTS: 26 patients followed at the Pediatric MS Center at the University of California, San Francisco received PLEX for acute events of MS (n = 15), NMOSD (n = 7), MOG (n = 2), TM (n = 1), and ADEM (n = 1). At time of PLEX initiation, median age was 13.5 years (range 3-17) and median time between the acute event onset and PLEX initiation was 22 days (range 3-94). 14 of 24 patients had documented clinical improvement after PS. Of those who improved during PS (n = 14), 13 had additional improvement after PLEX. Of those with no improvement after PS (n = 10), 8 improved after PLEX. 16 of 26 patients had pre- and post-PLEX EDSS scores available. Median pre-PLEX EDSS score was 4.0 (range 3.0-8.0), and median post-PLEX EDSS score was 3.75 (range 0-8.0) (p = 0.062). 5 patients had improved EDSS scores by 1 or more points. Adverse events during PLEX included hypotension (n = 3), nausea (n = 2), headache (n = 2), hypocalcemia (n = 2), hypofibrinogenemia (n = 2), thrombocytopenia (n = 1), spinal cord hemorrhage (n = 1), acute non-occlusive thrombosis of internal jugular vein (n = 1), occlusion of the central line (n = 1), edema of the neck (n = 1), and gastrointestinal discomfort (n = 1). CONCLUSIONS: PLEX is an overall well-tolerated second-line treatment option for pediatric patients with severe acute CNS demyelinating events with limited response to PS.

Effect of alternative intravenous lipid emulsion in clinical outcome in non-critically ill patients.

INTRODUCTION: Most studies on alternative intravenous lipid emulsion (IVLE) versus conventional IVLE have been conducted in the critically ill patients. The benefits of alternative IVLE in non-critically ill patients is uncertain. We aim to determine clinical outcome difference between alternative IVLE versus conventional IVLE in non-critically ill patients. METHOD: All patients on parenteral nutrition (PN) from July 2007 to September 2010 were identified. Patients were stratified into two groups: conventional IVLE (soybean oil-based) and alternative IVLEs, namely MCT oil-based, olive oil-based and fish oil-containing IVLE. RESULT: Three hundred and eighty-eight patients were included in the study. Ninety-one patients received soybean-based IVLE, 59 patients received MCT oil-based IVLE, 141 patients received olive oil-based IVLE and 97 patients received fish oil-containing IVLE. Adjusting the effect of baseline covariates in separate multiple linear/logistic regression models, there were no differences in mortality, readmission, length of stay and infection between conventional IVLE group and alternative IVLEs group, the adjusted p-value was 0.64, 0.06, 0.36 and 0.18 respectively. However, there was a significant change in day 5 CRP between these two groups (8.43 g/L (SD 112.2) vs -41.2 (SD 106.4); adjusted p-value = 0.01). There was no difference in day 5 albumin between these two group (-1.03 (SD 5.1) vs -0.1 (SD 5.3); adjusted p-value = 0.08). CONCLUSION: Our study showed that pertinent clinical outcomes in non-critically ill patients who received either conventional IVLE or alternative IVLEs were the same. However, there was significant reduction in day-5 CRP in alternative IVLE compared to conventional IVLE.