Perceived risk of infection, ethical challenges and motivational factors among frontline nurses in Covid-19 pandemic: prerequisites and lessons for future pandemic.

BACKGROUND: Infection risk was significant for front-line nurses during the Covid-19 outbreak. The pandemic presented several ethical difficulties and sapped nurses' drive to labor harder for longer periods. This study evaluates registered nurses' perceptions of Covid-19 infection risk, ethical dilemmas, and motivating factors. MATERIALS AND METHODS: During March and April 2022, 400 registered nurses from a newly established tertiary care hospital participated in this cross-sectional exploratory survey. The risk assessment scale, motivation to work scale, and ethical dilemma scale were used to assess the perceived risk of infection, motivational factors and ethical challenges experienced by the nurses. Appropriate descriptive and inferential statistics were applied to compute the results. RESULTS: 76.4% of nurses feared working as a nurse put them at higher risk of infection. Besides the fear of contracting infection, nurses believed they were the source of infection to family members (70.8%) and people around (67.5%). 63.3% of nurses agree that they do not have the right to refuse treatment and every patient has the right to receive optimal care, regardless of age, gender, and medical conditions. Professional obligation to treat patients (72.3%) and sound professional knowledge and experience (83.5%) are important motivating factors to work during the pandemic. Multilinear regression analysis revealed that professional education (95% CI, 3.845 - 0.694, p = 0.005), Covid-19 positive status (95% CI,0.455-2.756, p = 0.006), and post-Covid-19 hospitalization (95% CI, 1.395-6.978, p = 0.003) and duration of hospitalization (95% CI, 0.754-0.058, p = 0.022) are independent predictors of higher perceived risk of infection among nurses. CONCLUSIONS: During the pandemic, nurses were afraid to work and faced personal and family risks of contracting the virus. Despite these challenges, they still feel a strong sense of commitment and dedication to providing the best possible care. Nurse administrators need to create a supportive environment that follows ethical principles and meets the needs of nurses to boost their motivation and encourage them to continue working for longer periods.

Inborn Errors of Ketogenesis: Novel Variants, Clinical Presentation, and Follow-Up in a Series of Four Patients.

Inborn errors of ketogenesis are rare disorders that result in acute and fulminant decompensation during lipolytic stress, particularly in infants and children. These include mitochondrial 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) synthase (HMGCS) deficiency and HMG-CoA lyase (HMGCL) deficiency. In this series, we describe the clinical, biochemical, and molecular profiles of four patients along with dietary interventions and their outcomes on a long-term follow-up. Two patients each of HMGCS and HMGCL deficiency were evaluated with clinical history, biochemical investigations, including tandem mass spectrometry (TMS) and urine gas chromatography-mass spectrometry (GCMS). Molecular analysis was performed by whole-exome sequencing, as well as exon array validated by long-range polymerase chain reaction. All individuals were diagnosed with acute metabolic decompensation in the early infancy period except one with HMGCL deficiency who had the first presentation at 5 years of age. Central nervous system manifestations, severe metabolic acidosis, hyperammonemia, hypoglycemia with a normal lactate, and absence of urinary ketones were observed in all the affected individuals. The disorder was life-threatening in three individuals and one succumbed to the illness. TMS was nonspecific and urine GCMS revealed dicarboxylic aciduria in HMGCS deficiency. Both the patients with HMGCL deficiency demonstrated elevated 3 hydroxyisovaleryl carnitine levels in TMS and metabolites of leucine degradation in urine GCMS. We identified five novel variants that included a large deletion involving exon 2 in HMGCL gene. There was no evidence of long-term neurological sequelae in the living individuals. Diet with moderation of fat intake was followed in two individuals with HMGCS deficiency. Low leucine and protein diet with moderation of fat intake was followed in the individual with HMGCL deficiency. All affected individuals are thriving well with no further major metabolic decompensation.