Recommendations for the Management of Initial and Refractory Pediatric Status Dystonicus.

Status dystonicus is the most severe form of dystonia with life-threatening complications if not treated promptly. We present consensus recommendations for the initial management of acutely worsening dystonia (including pre-status dystonicus and status dystonicus), as well as refractory status dystonicus in children. This guideline provides a stepwise approach to assessment, triage, interdisciplinary treatment, and monitoring of status dystonicus. The clinical pathways aim to: (1) facilitate timely recognition/triage of worsening dystonia, (2) standardize supportive and dystonia-directed therapies, (3) provide structure for interdisciplinary cooperation, (4) integrate advances in genomics and neuromodulation, (5) enable multicenter quality improvement and research, and (6) improve outcomes. © 2024 International Parkinson and Movement Disorder Society.

Strong tissue glue with tunable elasticity.

Many bio-adhesive materials adhere weakly to tissue due to their high water content and weak structural integrity. Others provide desirable adhesive strength but suffer from rigid structure and lack of elasticity after administration. We have developed two water-free, liquid four-armed PEG pre-polymers modified with NHS or with NH2 end groups which upon mixing changed from liquids to an elastic solid. The sealant and adhesive properties increased with the amount of the %v/v PEG4-NHS pre-polymer, and achieved adhesive properties comparable to those of cyanoacrylate glues. All mixtures showed minimal cytotoxicity in vitro. Mixtures of 90%v/v PEG4-NHS were retained in the subcutaneous space in vivo for up to 14days with minimal inflammation. This material's combination of desirable mechanical properties and biocompatibility has potential in numerous biomedical applications. STATEMENT OF SIGNIFICANCE: Many bio-adhesive materials adhere weakly to tissue (e.g. hydrogels) due to their high water content and weak structural integrity. Others provide desirable mechanical properties but suffer from poor biocompatibility (e.g. cyanoacrylates). This study proposes a new concept for the formation of super strong and tunable tissue glues. Our bio-materials' enhanced performance is the product of new neat (without water or other solvents) liquid polymers that solidify after administration while allowing interactions with the tissue. Moreover, the elastic modulus of these materials could easily be tuned without compromising biocompatibility. This system could be an attractive alternative to sutures and staples since it can be applied more quickly, causes less pain and may require less equipment while maintaining the desired adhesion strength.

Adenocarcinoid of the appendix: is right hemicolectomy necessary? A meta-analysis of retrospective chart reviews.

The purpose of this study was to determine the efficacy of simple appendectomy versus right hemicolectomy in the treatment of localized adenocarcinoid of the appendix. A literature review of retrospective chart reviews from 1966 to March 1, 2003, was performed. Outcomes of retrospective chart reviews were assessed on the basis of treatment modality. Meta-analysis of studies by determining odds ratios for appendectomy versus extended resection using the Hunter-Schmidt meta-analytic method was performed. One hundred patients from 13 studies met inclusion criteria. Seven per cent failure rate with appendectomy alone and 10 per cent with extended resection were observed [OR 1.9 (0.6-5.8); association chi2 1.15, 1 df, P = 0.28]. Our data supports the use of appendectomy alone in localized cases of adenocarcinoid of the appendix provided there is no cecal involvement and the tumor's histology is low grade.

Routine immunizations and adverse events in infants with single-ventricle physiology.

BACKGROUND: Infants with single-ventricle congenital heart defects are at risk of sudden unexpected death. In an effort to decrease the risk of sudden death, some centers have advocated that routine immunizations be deferred in this population. However, it is not known if an association exists between immunizations and adverse events. METHODS: The present study examined the relationship of routine immunizations with adverse events, which were defined as sudden death or hospital readmission. The diphtheria-tetanus-acellular pertussis (DTaP) vaccine was considered in the analysis. The patient population consisted of infants younger than 9 months old who resided locally and had not yet undergone bidirectional cavopulmonary anastomosis (BCPA). Immunization data were obtained from a mandatory statewide database. RESULTS: During a 35-month period, 137 patients with single-ventricle physiology were discharged home after neonatal surgery or directly from the newborn nursery. Hypoplastic left heart syndrome (HLHS) was the diagnosis in 58 patients (42%) and was the most common. In the entire cohort, there were four sudden deaths (3%), and 53 patients (38%) had at least one interval hospital admission. Immunization within 48 hours was not associated with adverse events (odds ratio, 1.48; 95% confidence interval, 0.73 to 2.90; p = 0.31). No sudden death events occurred within 48 hours of immunization. CONCLUSIONS: No association could be identified between routine immunizations and adverse events in infants with single-ventricle physiology. As such, the proposal to alter the immunization regimen in this population does not appear justified.