Psychiatric symptoms in Salla disease

Salla disease (SD) is a rare lysosomal storage disorder characterised by intellectual disability ataxia, athetosis, nystagmus, and central nervous system demyelination. Although the neurological spectrum of SD's clinical phenotype is well defined, psychotic symptoms in SD remain unreported. We reviewed the presence of psychiatric symptoms in patients diagnosed with SD. Medical records of all SD patients at Oulu University Hospital during the years 1982-2015 were systematically reviewed to evaluate the presence of psychiatric symptoms. Psychiatric symptoms were frequently associated with SD (10/24, 42%), and two patients were described as developing psychosis as adolescents. We reported their clinical characteristics in detail and assessed the prevalence of psychiatric symptoms in a cohort of 24 patients. Other psychiatric factors associated with SD were sleeping disorders (8/24, 32%), aggressive behaviour disorders or restlessness (6/24, 25%), and off-label antipsychotic medication (4/24, 17%). This report expands the knowledge of the phenotypic spectrum of SD and demonstrates the importance of recognising the possibility of psychiatric symptoms, including psychosis, in persons with SD

"As protective gear began to run low, guidance on protection became looser" - Healthcare workers' perspectives on infection prevention and control during the COVID-19 pandemic

ObjectivesThe COVID-19 pandemic has posed several risk factors to healthcare workers' (HCWs') emotional distress. The purpose of the study was to enhance understanding of the experiences and feelings of HCWs during the COVID-19 pandemic, with specific reference to infection prevention and control (IPC) practices and guidance, focusing on the quality and availability of personal protective equipment (PPE), guidelines, and management. With a qualitative approach, we aimed to enable a wider narrative; to gain a more detailed understanding related to PPE use and identify experiences that can be overlooked in forced-choice questionnaires. MethodsAn online questionnaire was conducted among HCWs of the City of Helsinki and Helsinki University Hospital between 12.6.2020 and 5.4.2021. Altogether 1,580 HCWs participated in the study, from whom 579 shared 1,666 free-text responses. These responses were analyzed qualitatively, and the results were combined with statistical data on the participants' working conditions and backgrounds. ResultsWe identified problems in PPE availability and changing guidelines as factors causing the most distress in the participants. Regarding availability, running out of masks and respirators emerged as the most worrying issue, and inadequate PPE was associated with the excessive workload (OR 1.51, CI 95% 1.01-2.25). The results also highlight the importance of transparent and clear communication regarding IPC instructions and guidance, and clear IPC guidance was associated with better levels of reported recovery from work (OR 1.51, CI 95% 1.06-2.14). ConclusionsOur study highlights the importance of adequate PPE provision, transparent communication, clear guidance, and supportive supervisory work in this ongoing pandemic and potential new ones. We suggest more rigorous preparation, with crisis communication planning and emergency storage of PPE.Peer reviewe

Työolot perusterveydenhuollossa ja erikoissairaanhoidossa covid-19 pandemian aikana

Objectives: The COVID-19 pandemic has globally affected healthcare workers’ (HCWs) health and wellbeing. Most studies on COVID-19 have focused on tertiary healthcare. The aim of this study was to increase the knowledge on the effects of the pandemic on working conditions in tertiary and primary healthcare. Material and Methods: The comparative cross-sectional study consisted of an online questionnaire sent to HCWs of the City of Helsinki (primary healthcare) and Helsinki University Hospital (tertiary healthcare). Altogether 1580 HCWs with direct patient contact participated in the study: 895 from tertiary and 685 from primary healthcare. Statistical analysis used SPSS 25 from IBM. The tests used were the χ2 test, Fisher’s exact test, and binary logistic regression analysis. Results: Primary HCWs were less likely to treat COVID-19 patients (OR = 0.45, 95% CI: 0.37–0.56). However, both groups reported a similar number of COVID-19 infections, primary HCWs 4.9% and tertiary HCWs 5.0%, and work-related quarantine was significantly more prevalent (OR = 1.96, 95% CI: 1.38–2.79) among primary HCWs. In addition, work-related wellbeing was poorer among primary HCWs than tertiary HCWs in terms of feeling more stressed at work (OR = 3.20, 95% CI: 2.55–4.02), not recovering from work (OR = 0.49, 95% CI: 0.39–0.62), reported mental wellbeing below normal levels (OR: 1.59, 95% CI: 1.26–2.00), and increased working hours (OR = 1.63, 95% CI: 1.25–2.12). Conclusions: The study demonstrates how the pandemic has affected the wellbeing and working conditions of not only tertiary but also less studied primary HCWs. The authors’ findings suggest that the challenges identified during the COVID-19 pandemic in the health and wellbeing of healthcare workers are even greater in primary care than in tertiary care.Peer reviewe

Psychiatric symptoms in Salla disease

Abstract Salla disease (SD) is a rare lysosomal storage disorder characterised by intellectual disability ataxia, athetosis, nystagmus, and central nervous system demyelination. Although the neurological spectrum of SD’s clinical phenotype is well defined, psychotic symptoms in SD remain unreported. We reviewed the presence of psychiatric symptoms in patients diagnosed with SD. Medical records of all SD patients at Oulu University Hospital during the years 1982–2015 were systematically reviewed to evaluate the presence of psychiatric symptoms. Psychiatric symptoms were frequently associated with SD (10/24, 42%), and two patients were described as developing psychosis as adolescents. We reported their clinical characteristics in detail and assessed the prevalence of psychiatric symptoms in a cohort of 24 patients. Other psychiatric factors associated with SD were sleeping disorders (8/24, 32%), aggressive behaviour disorders or restlessness (6/24, 25%), and off-label antipsychotic medication (4/24, 17%). This report expands the knowledge of the phenotypic spectrum of SD and demonstrates the importance of recognising the possibility of psychiatric symptoms, including psychosis, in persons with SD