Variation of Diagnostic Approaches and Treatment Practices for Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome Among Pediatric Subspecialists.

OBJECTIVE: To assess the diagnostic and treatment practices among a variety of subspecialists at pediatric institutions in the US. STUDY DESIGN: Using a web-based survey, we assessed the consultation, diagnostic, and treatment preferences of providers from the different pediatric subspecialties who care for pediatric patients with hemophagocytic lymphohistiocytosis (HLH)/macrophage activating syndrome (MAS). Domains included demographics, provider training level and specialty, experience and comfort level with the diagnosis and treatment of HLH/MAS, and institutional approaches toward the diagnosis and management of HLH/MAS. Participants also were given 2 case scenarios: one describing Epstein-Barr virus-associated HLH and another describing an underlying rheumatologic condition with MAS. RESULTS: Of 263 respondents, 23%, 29%, 39%, and 7% identified as hematology/oncology, rheumatology, general pediatrics/critical care/hospitalist, and allergy/immunology, respectively. For Epstein-Barr virus/HLH, hematology/oncology was the preferred first consultant by most respondents other than rheumatologists, of whom only 47% agreed. For MAS, 92% of respondents from all specialties favored a rheumatology consultation. Preferred diagnostic tests varied by subspecialty, with hematology/oncology more likely than rheumatology to order an infectious workup, natural killer cell function, soluble interleukin-2 receptor, bone marrow biopsy, and genetic testing. First-line therapy also varied, with hematology/oncology preferring dexamethasone and etoposide and rheumatology more often preferring methylprednisolone and anakinra. One-half of respondents were unaware of institutional algorithms for diagnosis and treatment of HLH/MAS. Most (85.6%) favored the development of treatment algorithms for HLH/MAS, and 90% supported a multidisciplinary approach. CONCLUSIONS: Current consulting patterns, diagnostic workup, and treatment approaches of HLH/MAS vary by specialty, highlighting the need for standardized management algorithms and institutional multidisciplinary HLH/MAS teams.

Wide variation in glucocorticoid dosing in paediatric ANCA-associated vasculitis with renal disease: a paediatric vasculitis initiative study.

OBJECTIVES: High-dose glucocorticoids for remission-induction of ANCA-associated vasculitis are recommended and commonly used in adults, but recent studies suggest lower glucocorticoid doses can reduce toxicity without reducing efficacy. No paediatric-specific data exists to inform optimal glucocorticoid dosing in paediatric ANCA-associated vasculitis (pAAV). Our objectives were to describe glucocorticoid use in pAAV-related renal disease, and to explore associations between glucocorticoid dose, baseline patient characteristics and 12-month outcomes. METHODS: Youth <18 years with pAAV, biopsy-confirmed pauci-immune glomerulonephritis and 12-month follow-up data were included from an international paediatric vasculitis registry. Presenting features and 12-month outcomes (eGFR, glucocorticoid-related adverse effects), were compared between patients receiving no, low-moderate (≤90mg/kg) and high (>90mg/kg) cumulative intravenous methylprednisolone (IVMP), and low (<0.5mg/kg/day prednisone equivalent), moderate (0.5-1.5mg/kg/day) and high (>1.5mg/kg/day) starting doses of oral glucocorticoids. RESULTS: Among 131 patients (101 granulomatosis with polyangiitis, 30 microscopic polyangiitis), 27 (21%) received no IVMP, 64 (49%) low-moderate and 29 (22%) high-dose IVMP, while 9 (7%) received low, 75 (57%) moderate and 47 (36%) high initial doses of oral glucocorticoids. Renal failure at diagnosis (p=0.022) and plasmapheresis use (p=0.0001) were associated with high-dose IVMP. Rates of glucocorticoid-related adverse effects ranged from 15-31% across dose levels, and glucocorticoid dosing did not associate with 12-month outcomes. CONCLUSIONS: Glucocorticoid dosing for pAAV-related renal disease was highly variable, and rates of adverse effects were high across all dosing groups. A significant proportion of patients received oral glucocorticoid or IVMP doses that were discordant with current adult guidelines. Higher glucocorticoid doses did not associate with improved outcomes.

Sperm cryopreservation in an Australian skink (Eulamprus quoyii).

Assisted reproductive technologies for population and genetic management for threatened herpetofauna have grown substantially in the past decade. Here we describe experiments to optimise sperm cryopreservation in a model squamate, the eastern water skink Eulamprus quoyii . Small, concentrated volumes of highly motile spermatozoa were reliably collected from adult male E. quoyii by non-lethal ventral massage. Samples were used to: (1) test whether protein-rich diluents, namely Beltsville poultry semen extender (BPSE) and TES and Tris (TEST) yolk buffer (TYB), improve post-thaw quality metrics compared with Dulbecco's phosphate-buffered saline (DPBS); and (2) compare the efficacy of these diluents in combination with either 1.35M glycerol or 1.35M dimethyl sulfoxide (DMSO) at two freezing rates, fast (approximately -20°C min-1 ) versus slow (-6°C min-1 ). Glycerol and DMSO performed equally well in preserving spermatozoa under slow freezing rates. Under these conditions, the use of the complex diluents BPSE and TYB significantly improved post-thaw total motility compared with DPBS. Complex interactions occurred between cryodiluent type, cryoprotectant and freezing rate when testing fast versus slow freezing rates among treatment groups. Under slow freezing rates, DMSO was better at preserving membrane integrity and motility, regardless of diluent type, but successful fast freezing required complex diluents to support motility and membrane integrity, which has implications for implementation in a field setting.

Trends in referrals to liaison psychiatry teams from UK emergency departments for patients over 65.

INTRODUCTION: The number of people over the age of 65 attending Emergency Departments (ED) in the United Kingdom (UK) is increasing. Those who attend with a mental health related problem may be referred to liaison psychiatry for assessment. Improving responsiveness and integration of liaison psychiatry in general hospital settings is a national priority. To do this psychiatry teams must be adequately resourced and organised. However, it is unknown how trends in the number and type referrals of older people to liaison psychiatry teams by EDs are changing, making this difficult. METHODS: We performed a national multi-centre retrospective service evaluation, analysing existing psychiatry referral data from EDs of people over 65. We described trends in the number, rate, age, mental health presentation, and time taken to assessment over a 7 years period. RESULTS: Referral data from 28 EDs across England and Scotland were analysed (n = 18,828 referrals). There was a general trend towards increasing numbers of people referred to liaison psychiatry year on year. Variability in referral numbers between different departments, ranged from 0.1 to 24.3 per 1000 ED attendances. The most common reasons for referral were mood disorders, self-harm and suicidal ideas. The majority of referrals were assessed within 60 min, however there is variability between departments, some recording waits over 11 h. DISCUSSION: The data suggests great inter-departmental variability in referral numbers. Is not possible to establish the cause of variability. However, the data highlights the importance of asking further questions about why the differences exist, and the impact that has on patient care.

Civic engagement and mental health system strengthening in Indonesia: a qualitative examination of the views of health professionals and national key stakeholders.

BACKGROUND: Mental health services in Indonesia are developing rapidly in response to national and global health policy to support people living with psychosis. This presents a unique opportunity for civic engagement, the active involvement of patients, carers and communities in mental health care, to shape emergent services. In-depth explorations of the views of professionals and other key stakeholders in mental health care on the use of civic engagement in Indonesia are lacking which contributes to a limited understanding of its potential in this regard. The study aimed to explore contemporary professionals' and other key stakeholders' perspectives on the current use of and potential for civic engagement to strengthen mental health systems in Indonesia. METHODS: Qualitative interviews were undertaken and analysed using thematic analysis underpinned by a critical realist approach. Eighteen multi-disciplinary professionals and lay health workers involved in mental health care in Jakarta and Bogor and 10 national key stakeholders were recruited. RESULTS: Despite high levels of awareness of and support for civic engagement amongst mental health professionals and policy makers combined with a nascent grass roots movement, analysis revealed unstructured and insufficient mechanisms for civic engagement which resulted in ad-hoc and mostly superficial levels of involvement activity. Civic engagement was thought to require a marked shift in existing practices as well as organisational and societal cultures. Challenging stigma is a key feature of civic engagement and our analysis highlights the relevance of social contact methods which are locally and culturally contextualised in this regard. Our findings point to a need to expand current definitions of civic engagement which focus on indivdiual enablement to ones that also encompass environmental and organisational enablement to optimise the future use of civic engagement in mental health settings. CONCLUSIONS: Key mental health stakeholders have identified that central aspects of Indonesian culture are well aligned to the ethos of civic engagement which has the potential to facilitate the enactment of recent global health policy. However, full realisation is likely to be impeded by prevailing paternalistic cultures in mental health services and high levels of stigma and discrimination towards those with mental illness in Indonesia without intervention.

Exploring the potential use of patient and public involvement to strengthen Indonesian mental health care for people with psychosis: A qualitative exploration of the views of service users and carers.

BACKGROUND: Patient and public involvement (PPI) has the potential to strengthen mental health systems in Indonesia and improve care for people living with psychosis. Current evidence from other parts of the world demonstrates the need to understand the contexts in which PPI is to be enacted to ensure optimal implementation. OBJECTIVE: To understand service users' and carers' views on the current use and potential applicability of PPI within Indonesian mental health services. DESIGN: Qualitative study incorporating focus groups analysed using thematic analysis. SETTING AND PARTICIPANTS: Participants included 22 service users and 21 carers recruited from two study sites in Indonesia (Jakarta and Bogor). All participants had experience of psychosis either as a service user or carer. RESULTS: Despite the value attributed to PPI in relation to improving services and promoting recovery, current use of such activities in Indonesian mental health services was limited. Participants expressed a desire for greater levels of involvement and more holistic care but felt community organizations were best placed to deliver this because PPI was considered more congruent with the ethos of third-sector organizations. Additional barriers to PPI included stigma and low levels of mental health literacy in both health services and communities. DISCUSSION AND CONCLUSION: Participants felt that there was potential value in the use of PPI within Indonesian mental health services with careful consideration of individual contexts. Future aspirations of involvement enactment should ensure a central design and delivery role for third-sector organizations. Facilitators to global collaborative research in the context of the current study are also discussed.

HIV Care Trajectories as a Novel Longitudinal Assessment of Retention in Care.

Consistent engagement in care is associated with positive health outcomes among people living with HIV (PLWH). However, traditional retention measures ignore the evolving dynamics of engagement in care. To understand the longitudinal patterns of HIV care, we analyzed medical records from 2008 to 2015 of PLWH ≥ 18 years-old receiving care at a public, hospital-based HIV clinic (N = 2110). Using latent class analysis, we identified five distinct care trajectory classes: (1) consistent care (N = 1281); (2) less frequent care (N = 270); (3) return to care after initial attrition (N = 192); (4) moderate attrition (N = 163); and (5) rapid attrition (N = 204). The majority of PLWH in Class 1 (73.9%) had achieved sustained viral suppression (viral load ≤ 200 copies/mL at last test and > 12 months prior) by study end. Among the other care classes, there was substantial variation in sustained viral suppression (61.1% in Class 2 to 3.4% in Class 5). Care trajectories could be used to prioritize re-engagement efforts.

Evaluation of Radiation Exposure During Sentinel Lymph Node Biopsy in Breast Cancer: A Retrospective Study.

INTRODUCTION: Sentinel node biopsy (SNB) is the standard of care for nodal staging in breast cancer. Currently, radioisotope-guided SNB is the standard technique. We have undertaken this study to analyse radiation exposure during SNB for breast cancer over a significant period of time. METHODS: The study was conducted over a period of 41 months and included 183 patients. The radioisotope was usually administered the day preceding the surgery at a dose of 40 MBq (±10%), and a lower dose of 20 MBq (±10%) was given if injected on the day of surgery. The radiation exposure was measured by dosimeters worn by the surgeon and surgical assistant. RESULTS: The surgical procedures performed were mastectomy and SNB (56 patients), wide local excision and SNB (109 patients), and SNB alone (18 patients). The median time interval between the injection and surgery was 19.3 h, and median duration of surgery 93 min. The annual exposure for the surgeon was 0.125 mSv (range 0.01-0.03 mSv) and for the assistant was 0.265 (range 0.01-0.13 mSv). CONCLUSION: Contrary to other studies, we found the radiation exposure in the assistant was higher than that in the surgeon and we think this is because the assistant is positioned closer to the injection site during the procedure. However, the cumulative levels of radiation with SNB were very low and significantly below the permissible limits. The timing of injection, type of breast surgery and duration of procedure did not affect the levels of radiation exposure.

Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis.

OBJECTIVES: To characterise the clinical course and outcomes of a cohort of children with granulomatosis with polyangiitis (GPA). METHODS: Retrospective cohort study of children diagnosed with GPA in a tertiary care facility from 2000-2014. All subjects met the American College of Rheumatology 1990 criteria for GPA or the 2008 European League against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society criteria for GPA. Predictors of readmission were determined using univariate logistic regression. Kaplan-Meier analysis was used to demonstrate the relapse-free survival probability in follow-up. RESULTS: Twenty-eight children (median age 14.7 years) were diagnosed during the study period. At presentation 14 (50%), 5 (18%), and 4 (14%) children required intensive care unit care, ventilator support, and dialysis, respectively. One-third of the children in our cohort had gastrointestinal involvement, one-quarter of whom were previously diagnosed with inflammatory bowel disease. Two-thirds of children were readmitted. Renal failure and infections accounted for most readmissions. Twenty-three (85%) patients achieved remission, however, 11 subsequently flared (median time to flare 21.5 months). Haematuria at diagnosis was significantly associated with readmission (OR 6.25). At a median follow-up of 3.3 years (range 5 months to 6 years) 10 (37%) children had chronic kidney disease (> stage 2) and none of the children died. CONCLUSIONS: Children with GPA frequently have severe disease presentations including significant renal, respiratory and gastrointestinal involvement. While most children with GPA achieve remission, nearly half have subsequent relapses.

Predictors of effective de-escalation in acute inpatient psychiatric settings.

AIMS AND OBJECTIVES: To explore the factors that influence the use of de-escalation and its success in halting conflict in acute psychiatric inpatient setting. BACKGROUND: De-escalation is the use of verbal and nonverbal communication to reduce or eliminate aggression and violence during the escalation phase of a patient's behaviour. Although de-escalation is a first-line intervention in aggression management in acute psychiatric settings, little is known about the use or effectiveness of this technique. DESIGN: A retrospective case note analysis. METHODS: For each patient (n = 522), their involvement in conflict (e.g. aggression) or containment (e.g. coerced medication) during the first two weeks of their admission was recorded. The frequency and order of the conflict and containment events were identified during each shift. The sequences of events occurring in shifts involving de-escalation were analysed. Sequences where de-escalation ended the pattern of conflict or containment were categorised as 'successful', and all others were categorised as 'unsuccessful'. RESULTS: Over half of patients (53%) experienced de-escalation during the first two weeks of admission, with the majority of these (37%) experiencing multiple episodes. De-escalation was successful in approximately 60% of cases. Successful de-escalations were preceded by fewer, and less aggressive, conflict events, compared with unsuccessful de-escalations, which were most frequently followed by administration of pro re nata medication. Patients with a history of violence were more likely to experience de-escalation, and it was more likely to be unsuccessful. CONCLUSIONS: De-escalation is frequently effective in halting a sequence of conflict in acute inpatient settings, but patients with a history of violence may be specifically challenging. RELEVANCE TO CLINICAL PRACTICE: These findings provide support for de-escalation in practice but suggest that nurses may lack confidence in using the technique when the risk of violence is greater. Providing evidence-based staff training may improve staff confidence in the use of this potentially powerful technique.

Protocol for a multicentre randomised controlled trial of STeroid Administration Routes For Idiopathic Sudden sensorineural Hearing loss: The STARFISH trial.

Idiopathic sudden sensorineural hearing loss (ISSNHL) is the rapid onset of reduced hearing due to loss of function of the inner ear or hearing nerve of unknown aetiology. Evidence supports improved hearing recovery with early steroid treatment, via oral, intravenous, intratympanic or a combination of routes. The STARFISH trial aims to identify the most clinically and cost-effective route of administration of steroids as first-line treatment for ISSNHL. STARFISH is a pragmatic, multicentre, assessor-blinded, three-arm intervention, superiority randomised controlled trial (1:1:1) with an internal pilot (ISRCTN10535105, IRAS 1004878). 525 participants with ISSNHL will be recruited from approximately 75 UK Ear, Nose and Throat units. STARFISH will recruit adults with sensorineural hearing loss averaging 30dBHL or greater across three contiguous frequencies (confirmed via pure tone audiogram), with onset over a ≤3-day period, within four weeks of randomisation. Participants will be randomised to 1) oral prednisolone 1mg/Kg/day up to 60mg/day for 7 days; 2) intratympanic dexamethasone: three intratympanic injections 3.3mg/ml or 3.8mg/ml spaced 7±2 days apart; or 3) combined oral and intratympanic steroids. The primary outcome will be absolute improvement in pure tone audiogram average at 12-weeks following randomisation (0.5, 1.0, 2.0 and 4.0kHz). Secondary outcomes at 6 and 12 weeks will include: Speech, Spatial and Qualities of hearing scale, high frequency pure tone average thresholds (4.0, 6.0 and 8.0kHz), Arthur Boothroyd speech test, Vestibular Rehabilitation Benefit Questionnaire, Tinnitus Functional Index, adverse events and optional weekly online speech and pure tone hearing tests. A health economic assessment will be performed, and presented in terms of incremental cost effectiveness ratios, and cost per quality-adjusted life-year. Primary analyses will be by intention-to-treat. Oral prednisolone will be the reference. For the primary outcome, the difference between group means and 97.5% confidence intervals at each time-point will be estimated via a repeated measures mixed-effects linear regression model.

Identification of the "minimal triangle" and other common event-to-event transitions in conflict and containment incidents.

Although individual conflict and containment events among acute psychiatric inpatients have been studied in some detail, the relationship of these events to each other has not. In particular, little is known about the temporal order of events for individual patients. This study aimed to identify the most common pathways from event to event. A sample of 522 patients was recruited from 84 acute psychiatric wards in 31 hospital locations in London and the surrounding areas during 2009-2010. Data on the order of conflict and containment events were collected for the first two weeks of admission from patients' case notes. Event-to-event transitions were tabulated and depicted diagrammatically. Event types were tested for their most common temporal placing in sequences of events. Most conflict and containment occurs within and between events of the minimal triangle (verbal aggression, de-escalation, and PRN medication), and the majority of these event sequences conclude in no further events; a minority transition to other, more severe, events. Verbal abuse and medication refusal were more likely to start sequences of disturbed behaviour. Training in the prevention and management of violence needs to acknowledge that a gradual escalation of patient behaviour does not always occur. Verbal aggression is a critical initiator of conflict events, and requires more detailed and sustained research on optimal management and prevention strategies. Similar research is required into medication refusal by inpatients.

SARS-CoV-2 Infection and Increased Risk for Pediatric Stroke.

BACKGROUND: There is an increased risk of stroke in adults with severe acute respiratory syndrome coronavirus 2 (coronavirus disease 2019 [COVID-19]) infection, but whether there is a similar association with stroke in children is unclear. Our objective was to determine whether there is a correlation between COVID-19 infection, multisystem inflammatory syndrome in children (MIS-C), and pediatric ischemic stroke. METHODS: This was a retrospective, population-based cohort analysis between March 1, 2020, and June 30, 2021, conducted at a children's hospital. Pediatric patients with a diagnosis of ischemic stroke were identified using ICD-10 diagnoses of ischemic stroke, cerebrovascular accident, or cerebral infarction. RESULTS: We identified 16 patients, seven male and nine female, with ischemic stroke. Ages were 8 months to 17 years (median 11.5 years). More Asian (6%) and black (13%) patients had strokes compared with population prevalence (2% each, respectively). No patients had active COVID-19 infection. COVID-19 antibodies were identified in five of 11 patients tested (45%), of whom three were diagnosed with MIS-C. 82% of the strokes occurred between February and May 2021. The peak incidence was in February 2021, which was two months after peak incidence of pediatric cases of COVID-19 and one month after the peak of MIS-C cases. CONCLUSIONS: Our study suggests that prior COVID-19 infection, but not acute infection, is correlated with a risk for stroke in the pediatric population. The risk for stroke appears to be distinct from the risk for MIS-C.

Patient characteristics and behaviours associated with self-harm and attempted suicide in acute psychiatric wards.

AIMS AND OBJECTIVES: The study examined events before and after incidents of self-harm and attempted suicide and the characteristics of patients who engage in these behaviours. BACKGROUND: Psychiatric inpatient populations have an elevated risk of self-harm and suicide, but relatively little is known about the circumstances of these events during an admission. DESIGN: Retrospective case note analysis. METHOD: Data were collected on conflict (aggression, rule breaking, etc.) and containment (coerced medication, restraint, etc.) during the first two weeks of admission for a sample of 522 acute psychiatric inpatients. RESULTS: One in 10 patients self-harmed, and 4% attempted suicide. Aggression, attempting to leave the ward without permission and medication refusal were frequent precursors to incidents. Pro re nata medication and de-escalation were the most frequent interventions to follow an incident. Self-harm and attempted suicides during the current admission were significantly associated with younger age and a history of self-harm. CONCLUSIONS: A minority of the sample were involved in these behaviours, but incidents occurred soon after admission and sometimes repeatedly during the course of a day. Assessment of risk should be completed as early as possible. RELEVANCE TO CLINICAL PRACTICE: At-risk patients should be monitored for signs of withdrawal from ward activity, wanting to leave the ward without permission or non-compliance with medication to enable early intervention.

Left ventricular thrombus in the multisystem inflammatory syndrome in children associated with COVID-19.

A 3-year-old girl presenting with fever, mucocutaneous inflammation, and acute gastrointestinal symptoms met criteria for the multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C). Echocardiography showed severely decreased left ventricular (LV) function with an apical mass. After treatment with intravenous (IV) immunoglobulin, IV steroids, anakinra, milrinone, and systemic anticoagulation, her LV function rapidly improved and the mass became increasingly mobile. Given the risk of systemic embolization, the mass was excised through left ventriculotomy and pathology confirmed a thrombus.

Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

OBJECTIVE: There is no standardized approach to the treatment of pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Because of the rarity of pediatric AAV, randomized trials have not been feasible. The present study of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) was undertaken to establish consensus treatment plans (CTPs) for severe pediatric AAV to enable the future study of comparative effectiveness and safety. METHODS: A workgroup of CARRA members (rheumatologists and nephrologists) formed the AAV Workgroup. This group performed a literature review on existing evidence-based treatments and guidelines for the management of AAV. They determined that the target population for CTP development was patients <18 years of age with new-onset granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or renal-limited AAV (eosinophilic GPA was excluded), with presentation confined to those with severe disease (i.e., organ- or life-threatening). Face-to-face consensus conferences employed nominal group techniques to identify treatment strategies for remission induction and remission maintenance, data elements to be systematically collected, and outcomes to be measured over time. RESULTS: The pediatric AAV Workgroup developed 2 CTPs for each of the remission induction and remission maintenance of severe AAV. A glucocorticoid-weaning regimen for induction and maintenance, a core data set, and outcome measures were also defined. A random sample of CARRA membership voted acceptance of the CTPs for remission induction and remission maintenance, with a 94% (75 of 80) and 98% (78 of 80) approval rate, respectively. CONCLUSION: Consensus methodology established standardized CTPs for treating severe pediatric AAV. These CTPs were in principle accepted by CARRA-wide membership for the evaluation of pragmatic comparative effectiveness in a long-term registry.

Postdischarge Glucocorticoid Use and Clinical Outcomes of Multisystem Inflammatory Syndrome in Children.

Importance: Minimal data are available regarding the postdischarge treatment of multisystem inflammatory syndrome in children (MIS-C). Objectives: To evaluate clinical characteristics associated with duration of postdischarge glucocorticoid use and assess postdischarge clinical course, laboratory test result trajectories, and adverse events in a multicenter cohort with MIS-C. Design, Setting, and Participants: This retrospective cohort study included patients with MIS-C hospitalized with severe illness and followed up for 3 months in an ambulatory setting. Patients younger than 21 years who were admitted between May 15, 2020, and May 31, 2021, at 13 US hospitals were included. Inclusion criteria were inpatient treatment comprising intravenous immunoglobulin, diagnosis of cardiovascular dysfunction (vasopressor requirement or left ventricular ejection fraction ≤55%), and availability of complete outpatient data for 3 months. Exposures: Glucocorticoid treatment. Main Outcomes and Measures: Main outcomes were patient characteristics associated with postdischarge glucocorticoid treatment, laboratory test result trajectories, and adverse events. Multivariable regression was used to evaluate factors associated with postdischarge weight gain (≥2 kg in 3 months) and hyperglycemia during illness. Results: Among 186 patients, the median age was 10.4 years (IQR, 6.7-14.2 years); most were male (107 [57.5%]), Black non-Hispanic (60 [32.3%]), and Hispanic or Latino (59 [31.7%]). Most children were critically ill (intensive care unit admission, 163 [87.6%]; vasopressor receipt, 134 [72.0%]) and received inpatient glucocorticoid treatment (178 [95.7%]). Most were discharged with continued glucocorticoid treatment (173 [93.0%]); median discharge dose was 42 mg/d (IQR, 30-60 mg/d) or 1.1 mg/kg/d (IQR, 0.7-1.7 mg/kg/d). Inpatient severity of illness was not associated with duration of postdischarge glucocorticoid treatment. Outpatient treatment duration varied (median, 23 days; IQR, 15-32 days). Time to normalization of C-reactive protein and ferritin levels was similar for glucocorticoid duration of less than 3 weeks vs 3 or more weeks. Readmission occurred in 7 patients (3.8%); none was for cardiovascular dysfunction. Hyperglycemia developed in 14 patients (8.1%). Seventy-five patients (43%) gained 2 kg or more after discharge (median 4.1 kg; IQR, 3.0-6.0 kg). Inpatient high-dose intravenous and oral glucocorticoid therapy was associated with postdischarge weight gain (adjusted odds ratio, 6.91; 95% CI, 1.92-24.91). Conclusions and Relevance: In this multicenter cohort of patients with MIS-C and cardiovascular dysfunction, postdischarge glucocorticoid treatment was often prolonged, but clinical outcomes were similar in patients prescribed shorter courses. Outpatient weight gain was common. Readmission was infrequent, with none for cardiovascular dysfunction. These findings suggest that strategies are needed to optimize postdischarge glucocorticoid courses for patients with MIS-C.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease.

OBJECTIVE: To provide evidence-based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists. METHODS: Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question. We used the Grading of Recommendations, Assessment, Development and Evaluation method to assess the quality of evidence and formulate recommendations. Each recommendation required consensus from at least 70% of the Voting Panel. RESULTS: We present 1 good practice statement, 11 recommendations, and 1 ungraded position statement to guide the management of KD and clinical scenarios of suspected KD. These recommendations for KD are focused on situations in which input from rheumatologists may be requested by other managing specialists, such as in cases of treatment-refractory, severe, or complicated KD. The good practice statement affirms that all patients with KD should receive initial treatment with intravenous immunoglobulin (IVIG). In addition, we developed 7 strong and 4 conditional recommendations for the management of KD or suspected KD. Strong recommendations include prompt treatment of incomplete KD, treatment with aspirin, and obtaining an echocardiogram in the setting of unexplained macrophage activation syndrome or shock. Conditional recommendations include use of IVIG with other adjuvant agents for patients with KD and high-risk features of IVIG resistance and/or coronary artery aneurysms. These recommendations endorse minimizing risk to the patient by using established therapy promptly at disease onset and identifying situations in which adjunctive therapy may be warranted. CONCLUSION: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and use of echocardiography in patients with suspected or confirmed KD.