Dried Blood Spot Postmortem Metabolic Autopsy With Genotype Validation for Sudden Unexpected Deaths in Infancy and Childhood in Hong Kong.

Background Inborn errors of metabolism (IEM) are collectively rare but potentially preventable causes of sudden unexpected death (SUD) in infancy or childhood, and metabolic autopsy serves as the final tool for establishing the diagnosis. We conducted a retrospective review of the metabolic and molecular autopsy on SUD and characterized the biochemical and genetic findings. Methodology A retrospective review of postmortem metabolic investigations (dried blood spot acylcarnitines and amino acid analysis, urine metabolic profiling where available, and next-generation sequencing on a panel of 75 IEM genes) performed for infants and children who presented with SUD between October 2016 and December 2021 with inconclusive autopsy findings or autopsy features suspicious of underlying IEM in our locality was conducted. Clinical and autopsy findings were reviewed for each case. Results A total of 43 infants and children aged between zero days to 10 years at the time of death were referred to the authors' laboratories throughout the study period. One positive case of multiple acyl-CoA dehydrogenase deficiency was diagnosed. Postmortem reference intervals for dried blood spot amino acids and acylcarnitines profile were established based on the results from the remaining patients. Conclusions Our study confirmed the importance of metabolic autopsy and the advantages of incorporating biochemical and genetic testing in this setting.

Factors Influencing Nursing Students' Knowledge of and Attitudes Toward Urinary Incontinence.

PURPOSE: This study aimed to determine nursing students' knowledge about and attitudes toward patients with urinary incontinence. SUBJECTS AND SETTING: The sample comprised 392 nursing students from 5 educational institutions in Hong Kong; all participants were enrolled in year 4 or 5 of their undergraduate nursing program, and all had completed formal education on urinary incontinence and clinical experience caring for patients with urinary incontinence. METHODS: A cross-sectional survey was conducted in February 2017. Participants completed a 55-item questionnaire that included items querying demographic and pertinent professional background information, along with 2 validated instruments: the Urinary Incontinence Knowledge Scale (UIKS) and the Urinary Incontinence Attitude Scale (UIAS). Analysis of variance was performed to compare the differences in scores among nursing students based on demographic or educational background. Pearson's correlation coefficient or χ2 was used to examine the relationships between variables and multivariate regression analyses were performed to identify the predictors of attitude toward urinary incontinence. RESULTS: Urinary incontinence knowledge was moderate (mean 22.0/30, SD 4.4) and attitudes about urinary incontinence were positive (mean 41.6/60, SD 4.5). There was a significant correlation between attitudes and knowledge (r = 0.175, P = .001), institution at which the students received training (χ2 = 161.790, P = .000), and the experience of having taken a course that included instruction about urinary incontinence (χ2 = 37.707, P = .014). Regression analysis revealed that knowledge and institution were predictors of attitudes. Participants reported high level of interest in learning more about urinary incontinence (71.2%). CONCLUSIONS: Nursing students residing in Hong Kong have a moderate level of knowledge and positive attitude toward urinary incontinence. This study suggests that educational institution and specific instruction about urinary incontinence play key roles in developing positive attitudes toward caring for patients with urinary incontinence.

Are psychiatric comorbidities and associated cognitive functions related to treatment response to methylphenidate in boys with attention-deficit/hyperactivity disorder?

BACKGROUND: Methylphenidate (MPH) has been found to be an effective medication for attention-deficit/hyperactivity disorder (ADHD). However, there are neither consistent nor sufficient findings on whether psychiatric comorbidities and associated cognitive functions of ADHD are related to treatment response to MPH in ADHD children. OBJECTIVES: This study investigated whether psychiatric comorbidities, IQ, and neurocognitive deficits are related to treatment response to MPH in ADHD children. In some ways, it is preferable to have a drug that the effectiveness of which to a disorder is not affected by its associated cognitive functions and psychiatric comorbidities. On the other hand, it is likely that the baseline symptom severity of ADHD is associated with the effectiveness of MPH treatment on the symptoms post treatment. METHODS: A total of 149 Chinese boys (aged 6-12 years) with ADHD, combined type, and normal IQ participated in this study. Assessment of ADHD symptom severity was conducted pre and post MPH treatment, while assessment of psychiatric comorbidities, IQ, and neurocognitive deficits was performed in a non-medicated condition. Treatment response was defined as the ADHD symptom severity post MPH treatment. RESULTS: Results indicated that MPH treatment was effective, significantly improving the ADHD condition. Yet, comorbid disorders, IQ, and neurocognitive deficits were not related to MPH treatment response on ADHD symptoms. These findings indicated that the effectiveness of MPH was not affected by psychiatric comorbidities and associated cognitive functions of ADHD. Instead, as expected, it was the baseline symptom severity that was mainly related to the treatment response, ie, the milder the baseline condition, the better the treatment response. CONCLUSION: The current findings positively endorse the widespread clinical use of MPH for treating ADHD. It improves the behavioral symptoms of ADHD regardless of varying psychiatric comorbidities, IQ, and neurocognitive deficits.