High Dose Infliximab in the Treatment of Refractory Uveitis: Does Dose Matter?

Background. Infliximab (INF) has been shown to be beneficial in treating refractory uveitis, however, no data exist on optimal dosing and the efficacy of higher dosing. Objectives. To compare the efficacy of low-dose (LD) (<10 mg/kg), moderate-dose (MD) (≥10–15 mg/kg), and high-dose (HD) INF (≥15–20 mg/kg) in the treatment of uveitis. Methods. Retrospective chart review children with uveitis diagnosed at Childrens Hospital Los Angeles and Millers Children's Hospital, CA, USA. Results. Of the 34 INF-treated children, 6 patients received LD, 19 received MD, and 9 received HD. Average disease duration prior to therapy was 10.6, 24.6, and 37.1 months each group, respectively. Topical steroids were discontinued after an average of 3 months, 9.5 months, and 10.2 months in the LD, MD, and HD groups, respectively. We found that 66% of patients receiving LD, 42% of MD, and 66% receiving HD INF failed therapy and required either dose escalation or alternate medication for disease control. Conclusions. INF is beneficial in the treatment of uveitis, and dose escalation up to 4 times above the approved dose is often necessary to achieve disease control in patients with uveitis. Doses < 10 mg/kg every 4 weeks may not be sufficient to control disease

A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis

X-linked agammaglobulinemia (XLA) is a primary immune deficiency characterized by recurrent bacterial infections and profoundly depressed serum immunoglobulin levels and circulating mature B cells. We describe a 12-year-old boy with XLA and enthesitis-related arthritis (ERA). To date, there has been a paucity of reports of noninfectious inflammatory arthritis in children with XLA. This case illustrates that functional B cells and/or immunoglobulin are not required for ERA pathogenesis. In addition, this case suggests a possible link between immune deficiency, immune dysregulation, and rheumatic illness

Activities of Daily Living

The activities of daily living (ADLs) is a term used to collectively describe fundamental skills that are required to independently care for oneself such as eating, bathing, and mobility. The term activities of daily living was first coined by Sidney Katz in 1950. ADL is used as an indicator of a person’s functional status. The inability to perform ADLs results in the dependence of other individuals and/or mechanical devices. The inability to accomplish essential activities of daily living may lead to unsafe conditions and poor quality of life. Measurement of an individual’s ADL is important as these are predictors of admission to nursing homes, need for alternative living arrangements, hospitalization and use of paid home care. The outcome of a treatment program can also be assessed by reviewing a patient’s ADLs. Nurses are often the first to note when patients\u27 functionality declines during hospitalization; therefore, routine screening of ADLs is imperative and nursing assessment of ADL\u27s is performed on all hospitalized patients. Hospitalization for an acute or chronic illness may influence a person’s ability to meet personal goals and sustain independent living. Chronic illnesses progress over time, resulting in a physical decline that may lead to a loss of ability to perform ADL\u27s. In 2011, the United States National Health Interview Survey determined that 20.7% of adults aged 85 or older, 7% of those aged 75 to 84 and 3.4% of those aged 65 to 74 needed help with ADLs

Abatacept in the Treatment of Juvenile Dermatomyositis-Associated Calcifications in a 16-Year-Old Girl

Calcinosis is a feared complication of JDM that may be seen in up to 40% of children with JDM. It is associated with negative impact on the patients’ quality of life due to weakness, functional disability, joint contractures, muscle atrophy, skin ulcers, and secondary infections. Calcinosis can present as superficial nodules or plaques, larger nodular deposits extending into deeper tissue layers, accumulation of calcifications along the fascial planes of muscles or tendons, or an exoskeleton of calcium leading to limitations in mobility and joint contractures. Currently, there are no known effective treatments for calcinosis and current therapy is based on anecdotal retrospective studies and cases series. We report the case of a child with JDM-associated calcinosis with extensive intramuscular calcifications who failed conventional therapies but demonstrated improvement as evident by decrease in calcinosis and improved physical function with use of abatacept. We found that use of abatacept was associated with improvement in functional outcome and recurrence did not occur. This case suggests use of abatacept as a safe and effective treatment option for calcinosis due to JDM. Furthermore, large-scale clinical studies are needed to validate our findings and to evaluate the long-term outcomes

Childhood-Onset ANCA- Associated Vasculitis: single center experience from Central California

Abstract Background Childhood-onset ANCA-associated vasculitides (AAV) are characterized by necrotizing inflammation and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pediatric data is scare and there have been no prior studies examining the characteristics of pediatric AAV in Central California. Methods This retrospective study comprised AAV patients ≤18 years of age, diagnosed between 2010 and 2021, in Central California. We analyzed initial presentation including demographics, clinical, laboratory characteristics, treatment, and initial outcomes. Results Of 21 patients with AAV, 12 were categorized as MPA and 9 with GPA. Median age at diagnosis was 13.7 years in MPA cohort and 14 years in GPA. MPA cohort were majority females (92% versus 44%). 57% of the cohort were racial/ethnic minority including Hispanics (n = 9), Asians (n = 2), multiracial (n = 1) and 43% were white (n = 9). MPA patients were more frequently Hispanic (67%), meanwhile GPA patients were frequently white (78%). Median duration of symptoms prior to diagnosis was 14 days in MPA cohort and 21 days in GPA cohort. Renal involvement was frequent (100% in MPA and 78% in GPA). GPA cohort had frequent ear, nose and throat (ENT) involvement (89%). All patients were ANCA positive. All Hispanic patients were MPO positive, meanwhile 89% of white patients were PR3 positive. MPA cohort tended towards more severe disease with 67% requiring ICU admission and 50% requiring dialysis. Two deaths were reported in MPA cohort, related to Aspergillus pneumonia and pulmonary hemorrhage. In MPA cohort, 42% received cyclophosphamide in combination with steroids and 42% received rituximab in combination with steroids. GPA patients received cyclophosphamide, either with steroids alone (78%) or in combination with steroids and rituximab (22%). Conclusions Microscopic polyangiitis was the most frequent AAV subtype with female preponderance, shorter duration of symptoms at onset and higher proportion of racial/ ethnic minority patients. Hispanic children demonstrated frequent MPO positivity. Trends towards higher rates of ICU requirement and need for dialysis upon initial presentation was noted in MPA. Patients with MPA received rituximab more frequently. Future prospective studies are needed to understand differences in presentation and outcomes in childhood onset AAV between diverse racial-ethnic groups

The role of HRM in leadership development, talent retention, knowledge management, and employee engagement.

The purpose of this paper is to examine how the principles of leadership development, talent retention, knowledge management and employee engagement are determined to enhance survival during recovery after a recession within organisations. This paper critically reviews the literature pertaining to trends in leadership development and talent retention, with integration to knowledge management. Pragmatic insights into how organisations can enhance knowledge management through strategic and tactical implementation are offered. Key areas are identified that organisations need to implement to enhance knowledge management and people advantage. The integration of concepts with respect to employee engagement, involvement, challenges and opportunities for organisations in the recovery stage of the recession are highlighted. Practice based opportunities for organisations are discussed to enhance knowledge management by focussing on human and social capital; furthermore, an integration of leadership development initiatives across three countries, South Africa, Australia and New Zealand. Some recommendations are for HRM to take the lead in developing leader

The role of HRM in leadership development, talent retention, knowledge management and employee engagement

The purpose of this paper is to examine how the principles of leadership development, talent retention, knowledge management and employee engagement are determined to enhance survival during recovery after a recession within organisations. This paper critically reviews the literature pertaining to trends in leadership development and talent retention, with integration to knowledge management. Pragmatic insights into how organisations can enhance knowledge management through strategic and tactical implementation are offered. The significance of this paper is that key areas are identified that organisations need to implement to enhance knowledge management , people advantage, the integration of concepts with respect to employee engagement, involvement, challenges and opportunities for organisations in the recovery stage of the recession Practice based opportunities for organisations are discussed to enhance knowledge management by focussing on human and social capital; furthermore, an integration of leadership development initiatives across three countries, South Africa, Australia and New Zealand. Some recommendations are for HRM to take the lead in developing leaders

Perceptions on Business Strategy of Small and Medium-Sized Enterprises. Paper delivered at the Auckland, New Zealand

The purpose of this paper is to examine how the perceptions of employees could have an influence in business strategy. This paper critically reviews the literature pertaining to perceptions and attitudes. Perception involves a sophisticated thinking process, starting from obtaining data from the external environment, analysing, and converting it through the cognitive process. The result of this process may vary among individuals. People’s perceptions of reality have greater influence on their behaviour than reality itself. The significance of this paper is that it is important for SMEs in Lao PDR in order to develop more understanding about the influence of employees’ perceptions on the outcome of business strategy. The findings show strong evidence that supports the relationship between employees’ perceptions about strategy and business performance. Employees’ perceptions are important and cannot be ignored. Further results demonstrated that employees who had some interest in business strategy accounted for 37%, those with a moderate interest in strategy were at 42%, and those who were very interested in strategy represented at 16%. Some recommendations are given at the end of the paper before the conclusions sectio

The influence of employees' perceptions on business strategy of small and medium-sized enterprises

The purpose of this research is to understand the influence of employees’ perceptions about business strategy in order to identify the factors that contribute to a successful strategy. The research also examines employees’ perceptions about strategy formulation and implementation process of SMEs in Laos, and how these perceptions would affect the outcome of strategy. This research studies the phenomenon by exploring the reality in a scientific way. As a result, this research is based on the positivist paradigm by applying a quantitative research method. The target population of this research is employees of SMEs in Lao PDR, which comprises 100 respondents as the sample size. The findings show that employees who work for profitable SMEs believe business strategy is a key factor that drives superior business performance; certain degrees of interest in business strategy among employees work for SMEs regardless of their gender, age groups, and levels of education; employees who were satisfied with the strategy of their companies performed well in their jobs; and employees who value the formulation process are those who believe in the management system of their companies