Alpha-1 antitrypsin deficiency liver disease.

The clinical presentation of liver disease is highly variable in homozygous ZZ alpha-1 antitrypsin (AAT) deficiency, and not all patients with the homozygous ZZ genotype develop liver disease. Although not fully identified, there is likely a strong influence of genetic and environmental modifiers of the intracellular injury cascade and fibrotic response. Most ZZ children are well and remain undiagnosed. Of those who come to medical attention, the most common pediatric presentation is neonatal cholestatic hepatitis, sometimes referred to as "neonatal hepatitis syndrome". The gold standard for diagnosis of AAT deficiency is analysis of the AAT protein phenotype in the patient serum or the genotype of their DNA genome. Careful follow up of all diagnosed children is important. Heterozygotes for S and Z alleles of AAT (SZ) may develop progressive liver disease similar to ZZ patients and also require close monitoring. There is no specific treatment for AAT deficiency induced liver disease and current therapy remains supportive with management of complications. Rarely, patients require liver transplant and typically the patient outcomes are excellent. With improved understanding of liver injury mechanisms, new strategies for treatment are now being explored, including siRNA technology, molecules to modulate secretion, and enhancers of proteolysis.

The impact of neurosurgical technique on the short- and long-term outcomes of adult patients with Chiari I malformation.

OBJECTIVE: This study is aimed to compare and evaluate any differences in clinical and radiological outcomes of different operative techniques of cranio-cervical decompression (CCD) performed in adults with symptomatic Chiari malformation type I (CM-1) within a single tertiary neurosurgical center. METHODS: A retrospective review using the Hospital theatre management system (ORSOS) and records of patients who underwent CCD for CM-1 between January 2011 and October 2019 was performed. Patients were divided in three cohorts according to the operative technique used: an extradural osteo-ligamentous decompression (BD), BD followed by dural opening either without duraplasty (DOWD) or plus duroplasty (DOPD). The primary clinical outcome was measured by utilizing the Chicago Chiari Outcome Scale (CCOS). Syrinx outcome was measured on post-op MRI. Statistical analysis was performed using IBM SPSS 24 with α = 0.05. RESULTS: 67 adults underwent 69 CCD: 10 BD, 29 DOWD and 30 DOPD. Median follow-up was 47.3 months (Interquartile Range (IQR) 26.3-73.7). Patients who underwent DOPD had a shorter median hospital stay (p-value 0.001), fewer unplanned readmissions (p-value 0.015), a higher median CCOS (p-value 0.001) and a lower post-operative complications rate (p-value 0.001) compared to patients who underwent DOWD. BD revealed a 40 % failure rate and was ineffective in cases with syringomyelia. CONCLUSION: Better clinical outcomes, lower complication risk, and short duration of hospital stay were associated with patients who underwent dural opening with augmentative watertight duraplasty. Bony decompression alone despite being a very safe technique, does not appear to be reliable and effective in controlling and relieving the clinical symptoms and the syringomyelia of adult patients with CM-1.

Integration of 3-Dimensional/4-Dimensional Ultrasound Technology into Undergraduate Medical Education: Student Perception of Innovative Obstetrics Education.

The current study assessed level of comfort of medical students using 3-dimensional/4-dimensional (3D/4D) ultrasound technology after participating in an obstetrical ultrasound educational activity. The majority of students agreed that they were capable of performing and interpreting 2-dimensional and 3D/4D obstetrical ultrasounds and that the training benefited their clinical decision-making skills. Additionally, students agreed that access to 3D/4D ultrasound technology would encourage patients to seek physician care and act as a useful tool for patient education.

A prospective observational study of emergency department presentations following novel psychoactive substance use.

Background and aims Novel psychoactive substances are compounds intended to mimic the effects of illicit drugs. They provide a unique challenge to healthcare, as complications of their use and their impact on services are relatively unknown. This study aims to determine nature of presentations, patient demographics and impact on healthcare. Methods Novel psychoactive substances users who presented to a large urban emergency department over 4 weeks were prospectively identified and followed for 1 year. Patients over 13 years old were eligible for inclusion. Information regarding patient demographics and presentations was collected. Results During the study period, 53 patients (39 male), mean age 32.6 ± 8.9 (±standard deviation), presented 148 times with complaints relating to novel psychoactive substances use. Study population characteristics included history of illicit drug use (83.0%), intravenous drug use (64.2%), psychiatric diagnosis or symptoms (56.6%), methadone prescription (52.8%) and having no fixed abode (37.7%). Injection was the most common method of use (72.3%), Burst the most commonly named agent (19.6%) and behavioural change the most common presenting complaint (31.1%). Patients collectively spent 10,620 h in hospital over 1 year. Conclusion This study highlights differences between the young population targeted by government campaigns regarding novel psychoactive substances use and the presenting population to hospital, and the associated burden on the National Health Service.

Dry bedding provides cost-effective enrichment for group-housed rhesus macaques (Macaca mulatta).

Dry bedding has been shown to be an effective enrichment strategy for small groups of captive nonhuman primates housed in cages or in small enclosures with concrete flooring. However, dry bedding is used infrequently for large groups because of the perception that its use is time- and resource-intensive. We investigated the cost-effectiveness of this enrichment strategy in large groups (30 to 50 subjects) of rhesus macaques. Macaques were housed under 3 comparison conditions for 4 wk: pine shavings (n = 4), aspen and pine shaving mixture (n = 4), and nonbedded control (n = 4). As measures of resource consumption, husbandry tasks were documented by using time-in-motion methodology, and water usage was determined. In addition, groups underwent behavioral observations to assess the effect of dry bedding. The time required to care for units did not differ between bedded and nonbedded units. However, significantly less water was used for sanitization of bedded compared with nonbedded units. Monkeys housed in bedded units showed more foraging (13.8% ± 1.6% of time in bedded compared with 4.0% ± 0.3% of time in nonbedded units) and less aggression and self-grooming. Dry bedding benefited the macaques, reduced water usage and costs, and did not affect human resources.