General anesthesia safety in progressive leukodystrophies: A retrospective study of patients with Krabbe disease and metachromatic leukodystrophy.

BACKGROUND: Krabbe disease and metachromatic leukodystrophy are leukodystrophies characterized by neurologic degeneration and early death. Patients often require general anesthesia for diagnostic and therapeutic interventions. METHODS: A retrospective review of medical records was conducted for patients with Krabbe disease and metachromatic leukodystrophy receiving general anesthesia at a large children's hospital between 2012 and 2017. Patient complications and American Society of Anesthesiologists Physical Status were recorded for all procedures. The Neurodevelopment in Rare Disorders classification system was created to categorize the severity of the patient's disease progression based on clinical markers. Descriptive and inferential statistics were used to compare: (a) complication rate of affected patients vs the general hospital population; (b) the accuracy of the novel Neurodevelopment in Rare Disorders classification system vs American Society of Anesthesiologists Physical Status regarding the assessment of complication risk; (c) complication rate in patients with hematopoietic stem cell transplantation vs those without transplantation; (d) complication rate in immunosuppressed patients vs nonimmunosuppressed patients; and (e) complication rate of the three most commonly performed procedures. RESULTS: A total of 96 patients underwent 287 procedures. Of these, 11 cases had complications, yielding a rate of 3.8%. This is significantly higher than the overall complication rate at our institution of 0.246%. Statistical analysis showed better correlation between the Neurodevelopment in Rare Disorders classification system and complication rate than American Society of Anesthesiologists Physical Status and complication rate. The system also showed better accuracy in differentiating low-risk and high-risk patients. No statistically significant difference in complication rate was found for patients with transplantation vs those without transplantation or for immunosuppressed vs nonimmunosuppressed patients. Of the three most common procedures, central catheter placement/removal exhibited the highest complication rate. CONCLUSIONS: Although the complication rate for patients with Krabbe disease and metachromatic leukodystrophy is higher than the general population, most complications were mild and self-limiting. These results suggest that, in experienced hands, general anesthesia is well tolerated in most children. Findings show that the Neurodevelopment in Rare Disorders classification system is a better indicator for assessing complication risk in patients with Krabbe and metachromatic leukodystrophy than American Society of Anesthesiologists Physical Status.

Substance use disorder risk assessment: positive emotional experiences with first time use and substance use disorder risk.

Introduction: Substance Use Disorder (SUD) screening tools used in current practice are designed to identify SUD once patients have begun regular dangerous drug use. While these screening tools are valuable, prevention and avoidance of SUD would save countless lives. The climbing number of deaths due to drug overdose make screening for and prevention of SUD imperative. This study addresses this care gap. The aim was to develop a simple screening tool for patients who may be prone to develop Alcohol Use Disorder (AUD) and/or SUD prior to addiction. It was hypothesized that participants with initially positive emotional experiences would be correlated with a future SUD diagnosis. Methods: The study involved a self-administered survey using a cross-sectional design and was carried out over one-month in the spring of 2021. Those patients who presented to the MAT clinic (SUD group) were seen in a separate area than the patients presenting for urgent care (Comparison group). Participants (N = 259) were voluntarily recruited from MAT and Urgent care: Patients receiving acute care were assigned to the Comparison (N = 126, 50.8% female, 5.7% non-white, 27.2% age < 34) and those receiving treatment for SUD were assigned to the MAT group (N =133, 40.8% female, 4.8% non-white, 36.8% ≤34). The survey questioned demographics (4 items), risk factors for AUD/SUD (6 items), information about first alcohol/opioid experiences (16 items), and factors for seeking AUD/SUD treatment and recovery (2 items). Feelings were categorized as positive (e.g., euphoria, happiness, self-confident), neutral (e.g., nothing, normal), or negative (e.g., depressed, sad, sick). Results: The MAT group felt more positive feelings with first usage of alcohol and opioids compared to the comparison group (p<.001). With first usage of opioids specifically, MAT (0.13 ± 0.04) and comparison (0.29 ± 0.07) groups differed (p <.001). Over half (55.3%), of the MAT participants reported feeling self-confident with first use of alcohol while only 29.7% of the comparison reported this (p<.001). Over three-fifths (63.7%) of the MAT group reported feeling of euphoria with the first usage of opioids compared to one-tenth (9.8%) in the comparison group (p<.001). Discussion: This retrospective cross-sectional report shows the first affective responses to substances may predict risk for future SUD and could be a prevention screening tool. Asking patients about positive feelings with first usage of alcohol/opioids could be a simple screening tool employed for prevention.