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PurposeThe Audio-Visual Assisted Therapeutic Ambience in Radiotherapy (AVATAR) system was the first published radiation therapy (RT)-compatible system to reduce the need for pediatric anesthesia through video-based distraction. We evaluated the feasibility of AVATAR implementation and effects on anesthesia use, quality of life, and anxiety in a multicenter pediatric trial.Methods and materialsPediatric patients 3 to 10 years of age preparing to undergo RT at 10 institutions were prospectively enrolled. Children able to undergo at least 1 fraction of RT using AVATAR without anesthesia were considered successful (S). Patients requiring anesthesia for their entire treatment course were nonsuccessful (NS). The PedsQL3.0 Cancer Module (PedsQL) survey assessed quality of life and was administered to the patient and guardian at RT simulation, midway through RT, and at final treatment. The modified Yale Preoperative Anxiety Scale (mYPAS) assessed anxiety and was performed at the same 3 time points. Success was evaluated using the χ2 test. PedsQL and mYPAS scores were assessed using mixed effects models with time points evaluated as fixed effects and a random intercept on the subject.ResultsEighty-one children were included; median age was 7 years. AVATAR was successful at all 10 institutions and with photon and proton RT. There were 63 (78%) S patients; anesthesia was avoided for a median of 20 fractions per patient. Success differed by age (P = .04) and private versus public insurance (P < .001). Both patient (P = .008) and parent (P = .006) PedsQL scores significantly improved over the course of RT for patients aged 5 to 7. Anxiety in the treatment room decreased for both S and NS patients over RT course (P < .001), by age (P < .001), and by S versus NS patients (P < .001).ConclusionsIn this 10-center prospective trial, anesthesia avoidance with AVATAR was 78% in children aged 3 to 10 years, higher than among age-matched historical controls (49%; P < .001). AVATAR implementation is feasible across multiple institutions and should be further studied and made available to patients who may benefit from video-based distraction.

Objective To investigate the effectiveness and safety of hybrid surgery (microsurgery combined with intraoperative DSA) in the treatment of acute hemorrhagic cerebral vascular disease. Methods The clinical data, imaging data, treatment and prognosis of 11 patients with acute hemorrhagic cerebral vascular disease treated by hybrid operation from April 2020 to February 2021 in the Anqing First People's Hospital of Anhui Medical University were analyzed. The results showed 3 cases of cerebral arteriovenous malformation (CAVM), including 2 cases located in temporoparietal lobe and one case in right lateral fissure; 2 cases of dural arteriovenous fistula (DAVF) at the base of anterior cranial fossa while fistulaes located in sieve plate; 6 cases of intracranial aneurysms, including 3 cases in the middle cerebral artery (MCA) bifurcation, one case in the pericallosal artery, and 2 cases in the communicating segment of internal carotid artery (ICA). Results All 11 patients completed the surgery successfully, and the success rate was 100%. CAVM resection combined with hematoma removal were performed in 3 cases of CAVM, including one case that intraoperative DSA suggested a little residual malformed mass after initial resection, while DSA reexamination suggested total resection after supplementary resection. Dural arteriovenous fistula resection combined with hematoma removal were performed in 2 cases of anterior fossa, after the fistula was closed, DSA reexamination suggested the arteriovenous fistula disappeared completely. Intracranial aneurysm clipping was performed in 6 cases, DSA showed complete clipping in 4 cases, and complete clipping in 2 cases of aneurysm neck residual after adjustment or aneurysm clip supplement. DynaCT indicated satisfactory final removal of intracranial hematoma in all patients with intracranial hematoma. No patient had serious complications such as rebleeding and cerebral infarction. After a mean follow-up of 8.45 months, modified Rankin Scale (mRS) was 1 in 4 cases and 1 in 2 cases, and 0 in the other 9 cases. All patients underwent at least one CTA or DSA reexamination, and no residual or recurrent vascular lesions were found. Conclusions Hybrid operation as a new treatment mode for acute hemorrhagic cerebral vascular disease is safe and effective, and further accumulation of cases and experience are needed.

Plant inflorescence architecture is determined by inflorescence meristem (IM) activity and controlled by genetic mechanisms associated with environmental factors. In Arabidopsis (Arabidopsis thaliana), TERMINAL FLOWER1 (TFL1) is expressed in the IM and is required to maintain indeterminate growth, whereas LEAFY (LFY) is expressed in the floral meristems (FMs) formed at the periphery of the IM and is required to activate determinate floral development. Here, we address how Arabidopsis indeterminate inflorescence growth is determined. We show that the 26S proteasome subunit REGULATORY PARTICLE AAA-ATPASE 2a (RPT2a) is required to maintain the indeterminate inflorescence architecture in Arabidopsis. rpt2a mutants display reduced TFL1 expression levels and ectopic LFY expression in the IM and develop a determinate zigzag-shaped inflorescence. We further found that RPT2a promotes DNA METHYLTRANSFERASE1 degradation, leading to DNA hypomethylation upstream of TFL1 and high TFL1 expression levels in the wild-type IM. Overall, our work reveals that proteolytic input into the epigenetic regulation of TFL1 expression directs inflorescence architecture in Arabidopsis, adding an additional layer to stem cell regulation. The proteasome subunit RPT2a promotes DNA METHYLTRANSFERASE1 degradation, causing DNA hypomethylation upstream of TERMINAL FLOWER1 (TFL1) and high TFL1 levels in the Arabidopsis inflorescence meristem. [ABSTRACT FROM AUTHOR]

Introduction: 123I‐MIBG scan is used in neuroblastoma (NB) to monitor treatment response. Time to resolution of 123I‐MIBG avidity after radiation therapy (RT) is unknown. We sought to determine time to resolution of 123I‐MIBG avidity after RT and local failure (LF) rate. Methods: We performed a retrospective review of children with high‐risk NB who underwent 123I‐MIBG scans pre‐ and post‐RT from 2003 to 2019. Time from RT to resolution of 123I‐MIBG activity was analysed. LF and cumulative incidence of local progression (CILP) after RT stratified by site, presence of residual disease and use of boost RT were determined. Results: Forty‐two patients with median age 3.9 years (1.9–4.7 years) were included, with median follow‐up time 3.9 years (1.4–6.9). Eighty‐six lesions were treated with RT to median dose of 21.6 Gy. Eighteen of 86 lesions were evaluable for time to resolution of MIBG avidity after RT, with median resolution time of 78 days (36–208). No LF occurred among 26 patients who received RT to primary sites after GTR, versus 4/12 (25%) patients treated with residual primary disease. 2‐year CILP was 19% (12% primary disease 25% metastatic disease (P = 0.18)). 2‐year CILP for non‐residual primary, residual primary, non‐residual metastatic and residual metastatic lesions was 0%, 42%, 11% and 30% respectively (P = 0.01) and for boosted and non‐boosted residual lesions was 29% and 35% (P = 0.44). Conclusion: Median time to MIBG resolution after RT was 78 days. Primary lesions without residual disease had excellent local control. LF rate was higher after RT for residual disease, with no benefit for boost RT. [ABSTRACT FROM AUTHOR]

Dystrophic epidermolysis bullosa (DEB) is a series of severe genetic conditions affecting skin and nails caused by mutations in the COL7A1 gene. DEB has a strong phenotypic variability. In the present study, we recruited a case with a boy exhibiting typical DEB indication, and performed a clinical, genetic, and experimental investigation, followed by a prenatal diagnosis on their current pregnancy. Whole exome sequencing identified a novel compound heterozygous variation in COL7A1, consisting of two variants, namely c.191T>C (p.Leu64Pro) and c.5124G>A (p.Leu1708=) in the proband. In vitro study by minigene system indicated that c.5124G>A would result in an increased ratio of a transcript with exon‐skipping, which supported its pathogenicity. Further prenatal detection confirmed the genotype–phenotye co‐separation in this family. In conclusion, the findings in our study expanded the mutation spectrum of DEB, and emphasized the importance of paying attention to specific synonymous variants in the filtering process. [ABSTRACT FROM AUTHOR]