Role of regional anesthesia in patients with acute sickle cell pain: A scoping review

Sickle cell disease is the most prevalent inherited blood disorder in the world, with significant morbidity and mortality. Patients often have recurrent painful vaso-occlusive episodes, and the American Society of Hematology gives a conditional recommendation for the use of regional anesthesia for acute sickle cell pain management. This scoping review summarizes the current evidence and identifies gaps for future research. Our screening process is outlined, and articles that mentioned the use of regional anesthesia for acute sickle cell crises were included. We present and interpret our results and highlight opportunities for future investigation.     

HRAS-related epidermal nevus syndromes: Expansion of the spectrum with first branchial arch defects

Epidermal nevus syndrome (ENS) comprises a heterogeneous group of neurocutaneous syndromes associated with the presence of epidermal nevi and variable extracutaneous manifestations. Postzygotic activating HRAS pathogenic variants were previously identified in nevus sebaceous (NS), keratinocytic epidermal nevus (KEN), and different ENS, including Schimmelpenning–Feuerstein–Mims and cutaneous-skeletal-hypophosphatasia syndrome (CSHS). Skeletal involvement in HRAS-related ENS ranges from localized bone dysplasia in association with KEN to fractures and limb deformities in CSHS. We describe the first association of HRAS-related ENS and auricular atresia, thereby expanding the disease spectrum with first branchial arch defects if affected by the mosaic variant. In addition, this report illustrates the first concurrent presence of verrucous EN, NS, and nevus comedonicus (NC), indicating the possibility of mosaic HRAS variation as an underlying cause of NC. Overall, this report extends the pleiotropy of conditions associated with mosaic pathogenic variants in HRAS affecting ectodermal and mesodermal progenitor cells.     

Young offenders in forensic institutions in the Netherlands after committing serious crimes: Contribution of mandatory treatment and reduction of reincarceration

Background

In the Netherlands, young offenders who have been convicted of a particularly serious offence may be subjected to a so-called ‘Placement in an Institution for Juveniles’ (PIJ) measure if they are considered to pose a high ongoing risk to public safety. They form a rarely studied distinct group. Treatment in specialist forensic custodial institutions for young people (FYCI) is an intervention of last resort and costly. The most serious young offenders tend to be the hardest to rehabilitate while preventing further offending. Treatment is focussed on reducing risk of harm as well as improving health and other protective factors.

Aims

To explore the contribution of treatment in an FYCI under a forensic treatment order—the PIJ-measure—to the reduction of risk of reoffending.

Methods

In a pre–post intervention study, the Juvenile Forensic Profile (JFP) was used to score complete case files of 178 young offenders at the start and end of their placement in an FYCI under the PIJ-measure, 59% of those serving between the years 2013 and 2016 inclusive. The JFP covers risk and protective factors in seven domains encompassing criminal behaviour, family, environment, risk factors, psychopathology, psychology and behaviour during incarceration. Change or stability in scores was tested against reincarceration within 2 years of PIJ-measure completion.

Results

Impulse control and alcohol and drug use problems showed the greatest improvements. Behaviour that deteriorates during the stay is primarily related to obtaining more autonomy during reintegration efforts, including furlough. Reincarceration in the 2 years of community follow-up was unusual (13.5%). The two main variables associated with reincarceration were problematic behaviour during the pre-discharge year and lack of behavioural improvement during treatment.

Conclusions

Outcomes of mandatory treatment in this group of serious young offenders have not previously been studied in a rigorous pre–post intervention study design. We found evidence of an overall tendency to improvement over time in mental state and social skills, reflected by risk assessment scale scores. Continued substance use problems while incarcerated and continuing social skills deficits were most strongly associated with reincarceration suggests a possible need for review of these areas in the PIJ-measure programme. Results contribute to knowledge about risk assessment, treatment and preventions of harms by serious young offenders and may inform evidence-based policies and practices in the Netherlands and beyond.