Acute respiratory failure due to pulmonary exacerbation in children with cystic fibrosis admitted in a pediatric intensive care unit: outcomes and factors associated with mortality.

BACKGROUND: Children with advanced pulmonary disease due to cystic fibrosis (CF) are at risk of acute respiratory failure due to pulmonary exacerbations leading to their admission to pediatric intensive care units (PICU). The objectives of this study were to determine short and medium-term outcomes of children with CF admitted to PICU for acute respiratory failure due to pulmonary exacerbation and to identify prognosis factors. METHODS: This retrospective monocentric study included patients less than 18 years old admitted to the PICU of a French university hospital between 2000 and 2020. Cox proportional hazard regression methods were used to determine prognosis factors of mortality or lung transplant. RESULTS: Prior to PICU admission, the 29 patients included (median age 13.5 years) had a severe lung disease (median Forced Expiratory Volume in 1 s percentage predicted at 29%). Mortality rates were respectively 17%, 31%, 34%, 41% at discharge and at 3, 12 and 36 months post-discharge. Survival rates free of lung transplant were 34%, 32%, 24% and 17% respectively. Risk factors associated with mortality or lung transplant using the univariate analysis were female sex and higher pCO2 and chloride levels at PICU admission, and following pre admission characteristics: home respiratory and nutritional support, registration on lung transplant list and Stenotrophomonas Maltophilia bronchial colonization. CONCLUSION: Children with CF admitted to PICU for acute respiratory failure secondary to pulmonary exacerbations are at high risk of death, both in the short and medium terms. Lung transplant is their main chance of survival and should be considered early.

Spirituality, Mental Health, and COVID-19.

This issue of JORH presents a broad range of articles that consider spirituality and spiritual care from various international perspectives. It also looks at a diverse range of articles relating to mental health disorders and addictions. Lastly, this issue considers the aftermath of COVID-19. Readers are also reminded of the European Congress on Religion, Spirituality, and Health (ECRSH) (Salzburg, Austria), as well as the inaugural International Moral Injury and Wellbeing Conference (IMIWC), Brisbane, Australia, 2024.

Nurses, Clergy, Chaplains, Parkinson's Disease, Workplace Religiosity, Women's Health and Family Issues.

This issue of JORH explores a broad range of topics looking at the professions of nursing, clergy and chaplains. This issue also concludes the series on Parkinson's disease (Part 2), and for the first time, JORH presents a collation of articles relating to workplace religiosity. Finally, this issue revisits the topics of women's health and family issues in relation to religiosity and spirituality.

Tribal Healing, Suicide, Ethical Issues, Cancer and Measuring Religiosity and Spirituality.

This issue of JORH considers the 'good, the bad and the ugly' of tribal or traditional healers, as well as articles relating to ethical challenges due to contemporary medicine and environmental issues. The concluding series on suicide (Part 2) is also finalized in this issue, as well as a number of research articles from multiple countries relating to cancer. Similar to previous issues, JORH once again adds to its increasing collection of articles relating to the empirical measurement of religion, spirituality and health. Readers are also reminded of the European Congress on Religion, Spirituality and Health (ECRSH) (Salzburg, Austria, May 2024), as well as the inaugural International Moral Injury and Wellbeing Conference (IMIWC) (Brisbane, Australia, September 2024).

Home monitoring in asthma: towards digital twins.

PURPOSE OF REVIEW: We highlight the recent advances in home monitoring of patients with asthma, and show that these advances converge towards the implementation of digital twin systems. RECENT FINDINGS: Connected devices for asthma are increasingly numerous, reliable and effective: new electronic monitoring devices extend to nebulizers and spacers, are able to assess the quality of the inhalation technique, and to identify asthma attack triggers when they include a geolocation function; environmental data can be acquired from databases and refined by wearable air quality sensors; smartwatches are better validated. Connected devices are increasingly integrated into global monitoring systems. At the same time, machine learning techniques open up the possibility of using the large amount of data collected to obtain a holistic assessment of asthma patients, and social robots and virtual assistants can help patients in the daily management of their asthma. SUMMARY: Advances in the internet of things, machine learning techniques and digital patient support tools for asthma are paving the way for a new era of research on digital twins in asthma.

Children's views on artificial intelligence and digital twins for the daily management of their asthma: a mixed-method study.

New technologies enable the creation of digital twin systems (DTS) combining continuous data collection from children's home and artificial intelligence (AI)-based recommendations to adapt their care in real time. The objective was to assess whether children and adolescents with asthma would be ready to use such DTS. A mixed-method study was conducted with 104 asthma patients aged 8 to 17 years. The potential advantages and disadvantages associated with AI and the use of DTS were collected in semi-structured interviews. Children were then asked whether they would agree to use a DTS for the daily management of their asthma. The strength of their decision was assessed as well as the factors determining their choice. The main advantages of DTS identified by children were the possibility to be (i) supported in managing their asthma (ii) from home and (iii) in real time. Technical issues and the risk of loss of humanity were the main drawbacks reported. Half of the children (56%) were willing to use a DTS for the daily management of their asthma if it was as effective as current care, and up to 93% if it was more effective. Those with the best computer skills were more likely to choose the DTS, while those who placed a high value on the physician-patient relationship were less likely to do so.   Conclusions: The majority of children were ready to use a DTS for the management of their asthma, particularly if it was more effective than current care. The results of this study support the development of DTS for childhood asthma and the evaluation of their effectiveness in clinical trials. What is Known: • New technologies enable the creation of digital twin systems (DTS) for children with asthma. • Acceptance of these DTSs by children with asthma is unknown. What is New: • Half of the children (56%) were willing to use a DTS for the daily management of their asthma if it was as effective as current care, and up to 93% if it was more effective. •Children identified the ability to be supported from home and in real time as the main benefits of DTS.

Surface Measure to Depth (SMeTD): a new low-budget system for 3D water temperature measurements for combining with UAV-based thermal infrared imagery.

Characterising spatial patterns in water temperature is important for monitoring aquatic habitats and understanding physical and biogeochemical processes to support environmental management decisions. As freshwater bodies exhibit high spatial and temporal variability, high-resolution 3D temperature data are essential to understand local anomalies. The acquisition of simultaneously high spatial and temporal datasets in the field has so far been limited by costs and/or workload associated with commonly used monitoring systems.We present a new, low-cost, spatially and temporally flexible 3D water temperature monitoring system, Surface Measures to Depth (SMeTD). SMeTD can be used to provide information on the relation of water surface temperature to changes with depth, characterise water temperature in 3D and ground truth remotely sensed thermal infrared data. The systems performance was tested under laboratory conditions and under controlled conditions in the field. This revealed an accuracy comparable to established but more expensive monitoring systems. Field testing of SMeTD involved 1-min data collection of 3D water temperature for a full diurnal cycle in a lake. The 3D temperature patterns were supported by a thermal infrared image of the lakes surface. The field dataset demonstrated higher water temperatures and higher water temperature variation at the surface compared to deeper layers. SMeTD can be used to observe a broad range of hydrological processes in natural and artificial aquatic environments and help to understand processes involved with energy budgets, infiltration, limnology, or groundwater surface water exchange.

Chaplaincy, Judaism, Ukraine, COVID-19 and JORH Jubilee.

This first issue of JORH for 2023 considers (1) the ministry of chaplains, (2) Judaism, (3) the people of war-torn Ukraine, (4) the ongoing saga of COVID-19 and, on a happier note, (5) we celebrate a belated jubilee by presenting a bibliometric analysis of the Journal of Religion and Health (1961-2021). To conclude this issue, a book review is presented, "The Desperate Hours" by award winning journalist Marie Brenner, focusing on one hospital's fight to save New York City during COVID-19. A reminder is also provided to readers on the call for papers regarding a future issue on religion, spirituality, suicide and its prevention.

Pediatrics, Students, Allied Health and COVID-19.

This second issue of JORH for 2023 considers research relating to (1) pediatrics, (2) students, (3) various allied health professions and their related practices, and lastly, (4) COVID-19. An additional reminder is also provided to readers on the call for papers regarding a future issue on "Religion, Spirituality, Suicide, and its Prevention", as well as a new call for papers with respect to "Spiritual Care for People with Parkinson's Disease and their Caregivers".

Chaplaincy, Clergy, Prayer, Cancer and Measuring Religion and Health.

This third issue of JORH for 2023 revisits a number of themes previously highlighted in JORH, along with the addition of two new themes. Since JORH's first special issue on 'Chaplaincy' (JORH, 2022, 61:2), this area of research within JORH has now flourished, with a total of three JORH issues now incorporating the allied health discipline of chaplaincy. Two new article collections in this JORH issue relate to clergy 'faith leaders' and research related to 'prayer.' This issue also revisits the topic of cancer-a recurrent focus within JORH which has, over the past six decades, examined nearly every type of known cancer in the context of religion/spirituality. Finally, JORH collates once again, a number of articles relating to the empirical measurement of religion and health-an increasingly important area of research.

Care of the Aged, Women's Health, and Measuring Religion, Spirituality and Health.

This issue of JORH explores various concerns related to the care of the elderly within a number of countries (namely China, India, Iran, Israel, Turkey, USA). Issues relating to Women's Health are also considered across the life span but particularly with regard to gynaecology, paediatrics, cancer, mental health and wellbeing. Research is presented on the empirical measurement of religion, spirituality and health with scales developed and/or tested in Iran, India, Haiti, Taiwan, Jordan and the Netherlands. Finally, readers are reminded of the 9th European Congress on Religion, Spirituality and Health (ECRSH) during May 2024, 16-18th at the Paracelsus Medical University in Salzburg, Austria.

Suicide, Moral Injury, Parkinson's Disease, Diabetes and Dialysis.

This issue of JORH presents the first of a two-part series specifically exploring suicide. Research relating to moral injury is also included-a topic which has previously been discussed within earlier editions of JORH and an issue that is increasingly recognised as being associated with suicide. Other topic areas explored within this issue are Parkinson's Disease, Diabetes, and Haemodialysis. Finally, readers are once again reminded of the 9th European Congress on Religion, Spirituality and Health (ECRSH) to be held in May 2024, 16-18th at the Paracelsus Medical University in Salzburg, Austria. We would also like to announce a proposed inaugural International Moral Injury and Wellbeing Conference (IMIWC), 19-20 September 2024, Brisbane Exhibition and Convention Centre, Australia.

Medical Practice, the Church and COVID-19.

This issue of JORH explores the importance of religion and spirituality in medical practice, as well as research relating to the Church and its clergy, and finally the lingering effects of COVID-19.

Methionine supplementation for multi-organ dysfunction in MetRS-related pulmonary alveolar proteinosis.

INTRODUCTION: Pulmonary alveolar proteinosis related to mutations in the methionine tRNA synthetase (MARS1) gene is a severe, early-onset disease that results in death before the age of 2 years in one-third of patients. It is associated with a liver disease, growth failure and systemic inflammation. As methionine supplementation in yeast models restored normal enzymatic activity of the synthetase, we studied the tolerance, safety and efficacy of daily oral methionine supplementation in patients with severe and early disease. METHODS: Four patients received methionine supplementation and were followed for respiratory, hepatic, growth and inflammation-related outcomes. Their course was compared to those of historical controls. Reactive oxygen species production by patient monocytes before and after methionine supplementation was also studied. RESULTS: Methionine supplementation was associated with respiratory improvement, clearance of the extracellular lipoproteinaceous material and discontinuation of whole-lung lavage in all patients. The three patients who required oxygen or noninvasive ventilation could be weaned off within 60 days. In addition, liver dysfunction, inflammation and growth delay improved or resolved. At a cellular level, methionine supplementation normalised the production of reactive oxygen species by peripheral monocytes. CONCLUSION: Methionine supplementation was associated with important improvements in children with pulmonary alveolar proteinosis related to mutations in the MARS1 gene. This study paves the way for similar strategies for other tRNA synthetase deficiencies.

Systemic inflammatory syndrome in children with FARSA deficiency.

Variants in aminoacyl-tRNA synthetases (ARSs) genes are associated to a broad spectrum of human inherited diseases. Patients with defective PheRS, encoded by FARSA and FARSB, display brain abnormalities, interstitial lung disease and facial dysmorphism. We investigated four children from two unrelated consanguineous families carrying two missense homozygous variants in FARSA with significantly reduced PheRS-mediated aminoacylation activity. In addition to the core ARS-phenotype, all patients showed an inflammatory profile associated with autoimmunity and interferon score, a clinical feature not ascribed to PheRS-deficient patients to date. JAK inhibition improved lung disease in one patient. Our findings expand the genetic and clinical spectrum of FARSA-related disease.

Strategies for recognizing pneumonia look-alikes.

Community-acquired pneumonia is a common diagnosis in children. Among the many children whose symptoms and/or chest X-ray is consistent with community-acquired pneumonia, it can be difficult to distinguish the rare cases of differential diagnoses that require specific management. The aim of this educational article is to provide clinicians with a series of questions to ask themselves in order to detect a possible differential diagnosis of pneumonia in children. The value of this approach is illustrated by 13 real clinical cases in which a child was misdiagnosed as having lobar pneumonia. What is Known: • When a lobar pneumonia is diagnosed, an appropriate antibiotic treatment leads to the resolution of the clinical signs in most cases. • However, several diseases can be look-alikes for pneumonia and mislead the practitioner. What is New: • This article provides a new approach to identify differential diagnoses of pneumonia in children. • It is illustrated by 13 real-life situations of children misdiagnosed as having pneumonia.

Technical, Ethical, Legal, and Societal Challenges With Digital Twin Systems for the Management of Chronic Diseases in Children and Young People.

Advances in digital medicine now make it possible to use digital twin systems (DTS), which combine (1) extensive patient monitoring through the use of multiple sensors and (2) personalized adaptation of patient care through the use of software. After the artificial pancreas system already operational in children with type 1 diabetes, new DTS could be developed for real-time monitoring and management of children with chronic diseases. Just as providing care for children is a specific discipline-pediatrics-because of their particular characteristics and needs, providing digital care for children also presents particular challenges. This article reviews the technical challenges, mainly related to the problem of data collection in children; the ethical challenges, including the need to preserve the child's place in their care when using DTS; the legal challenges and the dual need to guarantee the safety of DTS for children and to ensure their access to DTS; and the societal challenges, including the needs to maintain human contact and trust between the child and the pediatrician and to limit DTS to specific uses to avoid contributing to a surveillance society and, at another level, to climate change. .

Mental Health, Islam, Clinical Issues and COVID-19.

In this fifth issue of the Journal of Religion and Health for 2022, four key themes are revisited: (1) mental health, (2) Islam, (3) various clinical issues relating to religiosity and/or spirituality and (4) the ongoing concerns of COVID-19.

China, Gender Issues, Medical Philosophy and Measurement Scales.

In this fourth issue of the Journal of Religion and Health for 2022, four key themes are explored: (1) religious and spiritual issues in China, (2) gender-related issues affecting communities, couples, women and men, (3) a multitude of philosophical perspectives regarding medicine, science, health and religion, (4) and an array of new or adapted religion/spirituality measurements and scales. Finally, we also recall and celebrate the life of former JORH Editorial Board member, Professor John S. Peale.

Cancer, Religious Philosophy, Death and Dying.

This final issue of JORH for 2022 revisits the topics of (1) cancer, (2) religious philosophy, and (3) uniquely collates a number of papers discussing the theme of death and dying-which seems an appropriate topic given the conclusion and celebration of life for one of the most internationally admired monarchs, Queen Elizabeth II (1926-2022). Lastly a call for papers is issued regarding religion, spirituality, suicide, and its prevention: https://www.springer.com/journal/10943/updates/23471166 .