Development of an overdue outreach program to improve adherence to PKU management

Background: Phenylketonuria (PKU) is an inherited autosomal recessive disorder caused by variants in the PAH gene which encodes for phenylalanine hydroxylase (PAH). PKU, left untreated, can cause intellectual disability, psychiatric symptoms, and behavioral abnormalities. Lifelong management of PKU is challenging, and many adult patients become lost to follow-up, despite recommendations for lifelong management. Method(s): The PKU clinic team is a multidisciplinary team consisting of an APN Director, physician, dieticians, diet tech, genetic counselor, registered nurse, and social worker. After establishing formal guidelines, algorithms were created to determine thresholds for initiating patient outreach based on both age and type of PKU treatment. EMRbased data collection is used to track adherence to both clinic visits and consistent submission of Phe levels. Data was collected and analyzed for roughly 250 PKU patients. Baseline levels for adherence to clinic visits and filter card submission were collected at time of implementation. Data was then reviewed after 18 months, and has been further analyzed for a second 18 months (which correlates with the start of the COVID-19 pandemic) Results: Overall baseline adherence across the population for annual clinic visitswas 72% (144/200). Clinic visit adherence increased to 88% at 18 months, and then was similar at 86% through COVID-19 pandemic. In the adult population, 54% (57/106) were adherent at baseline with clinic follow-up. With implementation compliance increased to 80% initially and was then reported to be 74% during the pandemic. Baseline for all PKU patients showed 81% (161/200) filter card submission within the last 12 months. Submission increased to 91% after 18 months of overdue outreach, and as of October 2021, 85% of all patients had submitted a filter card within the last calendar year. Adult patients specifically showed an increase, with 53% at baseline to 69% after implementation. Conclusion(s): Implementation of an overdue outreach program is successful in re-engaging patients with the PKU clinic and improving adherence to treatment recommendations. We have seen increased patient adherence across all domains and have maintained this improved adherence despite the global COVID-19 pandemic.Copyright © 2022 Elsevier Inc. All rights reserved.

The role of Industry 4.0 in developing resilience for manufacturing companies during COVID-19

Humanity has faced many crises in the past, such as pandemics, wars, and economic crises, and other crises are certain to come in the future;however, emerging technologies have a role to play in improving companies' resilience in the face of such crises. The coronavirus (COVID-19) pandemic has led to human, technological, and managerial constraints for manufacturing companies due to scarce resources or supply chain (SC) disruptions. The research goal of this paper is to investigate whether Industry 4.0 implementation improved companies' resilience and whether companies' performance maintained stability during the COVID-19 outbreak. Composite-based structural equation modeling is applied to analyse data collected from 207 manufacturing companies. The theoretical model is grounded in the Practice-Based View (PBV) theory. The research findings show that operational responses based on Industry 4.0, smart manufacturing practices, and smart capabilities enable manufacturers to build resilience and quickly mitigate performance loss in times of global crisis. Therefore, the results demonstrate that Industry 4.0 implementation provides resilience for companies through flexibility, reliability, robustness, and responsiveness. The main practical implication of this study is to support managers in achieving manufacturing performance stability during disrupted times, such as the COVID-19 crisis, using Industry 4.0 approaches to make their companies more resilient and prepared to face future challenges and crises. © 2022 Elsevier B.V.

Impact of the COVID-19 pandemic on the generation of urban solid waste in the municipality of Limeira, São Paulo

Social distancing measures derived from the COVID-19 pandemic have changed the consumption habits of citizens and, consequently, the quantity and composition of urban solid waste, with new challenges for Brazilian municipalities. In this context, the present research aimed to carry out a comparative analysis, before and during the COVID-19 pandemic, of the generation of solid household waste, health services, selective collection, and bulky waste in Limeira, São Paulo, considering a time series from January 2016 to December 2020. To analyze the change in the amount of solid waste collected in the municipality due to the COVID-19 pandemic, Generalized Mixed Linear Models were adjusted, and descriptive statistical analysis was performed to examine the trend of the series. The increase in the collection of waste from health services from April 2020 was evident, with a positive relationship between the number of reported cases of COVID-19 and the generation of this waste. There was a reduction in waste from selective collection during all months of 2020 compared to previous years. Household waste showed no difference in generation in the months with and without COVID-19, while selective collection waste and bulky waste showed a reduction in generation in the months with COVID-19. The survey results are relevant as support for the contingency plan for solid waste management in pandemic situations in Limeira and for municipalities with similar characteristics. © 2022, ABES - Associacao Brasileira de Engenharia Sanitaria e Ambiental. All rights reserved.

The Impact of the Widened Demographic Gaps during the Pandemic on the rise in Unemployment

Demographic groups with the greatest prior socioeconomic vulnerabilities, such as women, youth, and people with fewer qualifications, have aggravated their structural challenges since the beginning of the pandemic in Colombia. Thus, it is necessary to isolate the impact explicitly caused by the widened demograph-ic gaps due to the pandemic on the fall in employment in the 23 cities and metropolitan areas. Through a difference-in-differenc-es model, it was estimated that about a fifth (3.1 pp) of the fall in total employment was associated with the widening of the gender gap;one-tenth (2.0 p.p.) with the extension of the generation gap;and a third (5.8 p.p.) with a higher skills gap. This shows the need to focus public policies on those more sensitive to the fall in labor demand as a result of the pandemic in the country.

The effects of the COVID-19 crisis on startups' performance: the role of resilience

Purpose This paper aims to evaluate the impacts of the COVID-19 crisis on startups performance and the moderating effects played by several resilience-related startup characteristics during times of crisis. Design/methodology/approach To achieve this, 94 Brazil-based startups were surveyed, and multivariate data techniques (PLS-SEM) were applied. Findings The results show that despite the startups performance having been affected by the pandemic crisis, the response measures, when influenced by the resilience characteristics of these companies, moderated this effect. Furthermore, our findings suggest the future challenges to be faced by these organisations in the post-pandemic period. Research limitations/implications Proposing a framework, our survey research contributes to the dynamic capabilities theory by showing that startups resilience is linked to the micro-foundations of sensing (e.g. innovation systems, resilience culture, pivoting practices, innovativeness products), seizing (e.g. leadership/focused skills, people development and selection, agility, clear vision of business process) and reconfiguring capabilities. Practical implications Not only for theory, but this paper also contributes insights and guidelines for business practice in the face of challenges arising from times of crisis. By demonstrating the positive effect of early response measures based on resilience, our findings provide genuine managerial input that can help managers, funders and decision-makers in these companies operations against turbulent crises early on, thereby supporting the traction phase and sustaining their performance. Originality/value Previous research has examined the effects of the COVID-19 crisis in several sectors and perspectives. However, this study is the first to empirically test and clarify how the resilience and singularities of these new business models based on innovation could react to the changes caused by the pandemic.

HISTIOCITOSE DE CÉLULAS DE LANGERHANS EM RECÉM-NASCIDO: RELATO DE CASO

Objetivos: Relatar um caso raro de Histiocitose de Células de Langerhans em um recém-nascido e atentar-se a importância do diagnóstico precoce e acompanhamento a longo prazo. Material e métodos: Estudo retrospectivo, descritivo, baseado em revisão de prontuário. Resultado: B.M.A.S, 1 mês e 14 dias, sexo masculino, nasceu de parto cesáreo por malformação do SNC, com boa vitalidade ao nascer, sem dismorfias, sem necessidade de manobras de reanimação. Apresentando, ao nascimento, lesões pustulosas e vesiculares em todo o corpo e mais intensa em face e irritabilidade intensa. Fez uso de 21 dias de aciclovir e 7 dias de oxacilina com melhora parcial das lesões. Mãe com sorologias negativas para TORCHS e swab vaginal para streptococo negativo. E paciente em questão apresentando sorologias negativas para TORCHS, Zika, Dengue e Chikungunya. A biópsia das lesões da pele congênitas foi conclusiva para Histiocitose de células de Langerhans. Paciente apresentava USTF com esquizencefalia de lábio aberto, RNM de crânio e TC de crânio com hidranencefalia. O mesmo evoluiu com infecção secundária nas lesões da pele, fazendo uso de oxacilina e fluconazol. Durante internação paciente evoluiu com síndrome respiratória aguda grave por COVID-19, com necessidade de suporte intensivo e choque séptico, evoluindo para óbito. Discussão: A histiocitose de células de Langerhans (HCL) é um termo genérico, que engloba doenças clinicamente distintas, mas que apresentam, como ponto em comum, a proliferação clonal de células de Langerhans com grânulos de Birbeck e S100/CD1A positivos na imuno-histoquímica. É considerada uma doença rara, com incidência anual na faixa pediátrica de três a quatro por um milhão. A pele é acometida em 50% dos casos, e em 10% é o único sítio. O curso varia desde formas localizadas e autolimitadas até formas multissistêmicas graves. Atualmente a HCL é entendida como uma doença espectral com ampla variabilidade de apresentações clínicas e curso clínico imprevisível. Os casos congênitos geralmente são exclusivamente cutâneos e autolimitados, com remissão espontânea em meses. O diagnóstico deve ser considerado nos quadros de dermatite seborreica e dermatite das fraldas que não apresentam boa resposta ao tratamento. O aspecto purpúrico das lesões deve chamar a atenção para esse diagnóstico. O exame histopatológico e a análise imunoistoquímica são diagnósticos. O principal critério é a positividade para CD1a ou langerina (CD207). Não há tratamento específico para a histiocitose congênita auto-limitada. A conduta orientada pela Histiocyte Society consiste em acompanhar o quadro e aguardar a regressão espontânea, não alcançada pelo paciente devido a óbito por choque séptico após 10 dias do diagnóstico conclusivo. Conclusão: A Histiocitose de Células de Langerhans em recém-nascido é rara e potencialmente fatal e apresenta ampla variabilidade de apresentações clínicas e curso clínico imprevisível por isso a importância do rápido diagnostico para abordagem precoce.

eP276: Development of an overdue outreach program to improve adherence to phenylketonuria management

Introduction: Phenylketonuria (PKU) is an inherited autosomal recessive disorder caused by variants in the PAH gene which encodes for phenylalanine hydroxylase (PAH). PAH deficiency leads to phenylalanine (Phe) accumulation, which untreated can cause intellectual disability, microcephaly, delayed speech, seizures, psychiatric symptoms, and behavioral abnormalities. Early detection of elevated Phe through newborn screening allows for rapid initiation of a Phe-restricted diet to prevent severe neurological outcomes;however, suboptimal Phe control throughout the lifespan is associated with increased rates of psychiatric illness and deficits in executive function even in early treated patients. Lifelong management of PKU is challenging, and it is well documented that many adult patients become lost to follow-up, despite the American College of Medical Genetics recommendation for lifelong management. Here we describe and evaluate efforts to improve follow-up care for patients with PKU of all ages at one center through formalization of clinic guidelines and creation of an overdue outreach program. Methods: The PKU clinic team is a multidisciplinary team consisting of an APN Director, physician, dieticians, diet tech, genetic counselor, registered nurse, and social worker. Regular meetings were scheduled with all clinic staff members to review PKU treatment guidelines, recommended lab monitoring, and visit frequency. After establishing formal guidelines, algorithms were created to determine thresholds for initiating patient outreach based on both age and type of PKU treatment. EMR-based data collection is used to track adherence to both clinic visits and consistent submission of Phe levels. Data was collected and analyzed for Lurie Children’s PKU program, which consists of roughly 250 patients. Baseline levels for adherence to clinic visits and filter card submission were collected at time of implementation. Data was then collected and analyzed initially after 18 months, and has been further analyzed for a second 18 months (which correlates with the start of the COVID-19 pandemic). Results: Overall baseline adherence across the PKU patient population for annual clinic visits was 72% (144/200). Clinic visit adherence increased to 88% at 18 months, and then was essentially unchanged at 86% through COVID-19 pandemic. In the pediatric patient population, annual clinic follow-up adherence was 92% (79/86) at baseline, which increased to 98% with implementation and maintained 98% during the pandemic. In the adult patient population, 54% (57/106) were adherent at baseline with clinic follow-up. With implementation compliance increased to 80% initially and was then reported to be 74% during the pandemic. Baseline for all PKU patients showed 81% (161/200) filter card submission within the last 12 months. Submission increased to 91% after 18 months of overdue outreach, and as of October 2021, 85% of all patients had submitted a filter card within the last calendar year. Adult patients specifically showed an increase, with 53% at baseline to 69% after implementation. Hyperphenylalaninemia (hyperphe) patients over the age of 2 showed an 18% (5/28) submission at baseline. With the overdue outreach program, this increased to 31% of patients initially and has further increased to 39%. Clinic visits for patients greater than 7 years old rose from 13% (3/24) initially to 57% and has further increased to 67%. Conclusion: Implementation of a coordinated overdue outreach program is successful in re-engaging patients with the PKU clinic and improving adherence to treatment recommendations. We have seen increased patient adherence across all domains, and have maintained this improved adherence despite the global COVID-19 pandemic. We believe that integrating overdue outreach guidelines into clinical practice is a replicable model for PKU clinics.

MENTAL ILLNESS: INTERFACES WITH THE WORKING ENVIRONMENT DURING THE COVID-19 PANDEMIC, FROM THE PERSPECTIVE OF NURSING PROFESSIONALS

The growth of invisible pathologies resulting from the psychological exhaustion of nursing professionals raises an alert to the lack of labor policies in institutions with a focus on prevention. This study aims to assess the perception of nursing professionals about mental illness in their work environment, enhanced by the current pandemic moment. It also aims to identify the actions that contracting health institutions adopt, as a preventive measure. This is a cross-sectional and descriptive study, in which the snowball technique was used, using the Google Forms® tool. The research complied with the ethical precepts of Resolution CNS 466/2012, being held in July and August 2020. 45 nurses and 56 nursing technicians answered the questions, with a prevalence of 85.14% being women. The high workload, the pressure in the work routine, the low pay and the precarious conditions for the exercise of the activity were the factors listed that most contribute to mental illness. Although 73 of the respondents did not leave work for prolonged periods, 30 were absent due to mental illness. It should be noted that 61 nursing professionals, who responded, are not aware of preventive initiatives for mental illness in their companies. This study reveals that the work environment, poor conditions for performing activities, work overload and fear of the unknown, such as the disease caused by the coronavirus, are considered the main factors that contribute to the mental illness of nursing professionals. Thus, it is essential that protective measures for self-care in mental health are offered by health institutions. © 2021 Slovak Academy of Sciences. All rights reserved.

A cheap immunoassay for determination of immune response to SARS-CoV-2 vaccine in capillary blood samples

The coronavirus disease 2019 (COVID-19) caused by SARS-Cov- 2 virus represents public health emergency of international concern. The recent development of SARS-CoV-2 vaccines offer great hope to fight against this worldwide pandemic. However, there are many unknowns moving forward, like how long immunogenicity lasts or how many doses are required to immunize a person. High-quality serology is a key tool in understanding the immunity to viruses, and the relevance of protection. The measurement of specific COVID-19 antibodies can be used and currently very necessary to verify the effectiveness of the different vaccines in the population. Here, we present an optimized ELISA-based serology protocol that can be applied in serum and in capillary blood. Validation was performed using serum collected from 113 hospitalized patients in Principe de Asturias University Hospital. Ninety-eight of them were diagnosed with COVID19 by a positive rRT-PCR result and fifteen samples were negative in rRT-PCR. We used an indirect ELISA assay, using the receptor binding domain (RBD) of the recombinant spike protein of SARS-Cov-2 to cover the plate. In addition, all patients were tested by commercial ELISA to compare with our cheap immunoassay. Results show that our ELISA assay is suitable for capillary blood samples and has a sensibility significantly higher than commercial ELISA. This study demonstrates the validity of our cheap immunoassay for the measurement of specific IgG in the serum of patients, which allows the safe and economic testing of all currently vaccinated people to ensure immunity in the population and the efficacy of vaccination. Surprisingly our assay works on capillary blood samples, which facilitates the process of obtaining the sample and can be carried out quickly and safely by the patients themselves. Therefore, our cheap immunoassay may be useful to provide population-based data on SARS-CoV-2 seropositivity, infection and immunity generated by the vaccines.

COVID-19 presentation in pediatric patients with visceral transplantation

Introduction: Coronavirus disease 2019 (COVID-19) pandemic has become one of the most challenging episodes in the history of modern public health, with particular emphasis in high risk population. However, the evidence regarding their response to the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-Cov-2), the agent responsible for COVID-19 is scant. Herein we present the clinical course of the SARSCoV- 2 infection in four pediatric patients that had underwent visceral transplantation. Methods: pediatric patients (3 with mulvisceral transplant and 1 hepatointestinal transplant;median age: 13,75 years, range: 12-17 years) were diagnosed with SARS-CoV-2 infection by nucleic acid amplification test, antigen test or serologic anti-SARS-CoV-2 IgM detection. Lymphocytes count, clinical signs and seroconversion were assessed. Results:3/4 consulted for symptoms compatible with COVID-19 and had a mild clinical presentation. Patient I: headache and muscle pain, Patient II: Anosmia and agusia, cough and rhinorrhea, Patient III: sore throat, cough and rhinorrhea. Patient IV was asymptomatic and consult for epidemiological environment. Lymphocytes count at the time of diagnosis was between normal range (media: 3600 cells/ul of blood, range 2600-4600 cells/ul of blood) and, interestingly, no gastrointestinal symptoms were reported. Of note, immunosuppression was not suspended nor diminished during the episode. 4/4 were receiving Tacrolimus (blood levels: median 7.7ng/ ml, range: 6.7-8.4 ng/ml) and 3/4 also corticoids (median 5 mg/day, range 2-10 mg/day). Finally, until today we could detect specific IgG antibodies against SARS-CoV-2 in serum after recovery in 3 of the patients (one is still pending). Conclusion: The results presented here suggest that SARS-CoV-2 infection in pediatric patients with visceral transplantation is asymptomatic/mild. Interestingly, despite their graft altered immune status, no gastrointestinal symptoms were observed. Additionally, despite the high immunosuppression levels used, seroconversion was achieved in all patients so far. All together, these results suggest that collaboration between B and T cells was not affected by the pharmacological treatment and that pediatric patients would not constitute a risk group.