Our goals were to (1) validate the parental Ages and Stages Questionnaires (ASQ) as a screening tool for psychomotor development among a cohort of ex-premature infants reaching 2 years, and (2) ...analyse the influence of parental socio-economic status and maternal education on the efficacy of the questionnaire. A regional population of 703 very preterm infants (<35 weeks gestational age) born between 2003 and 2006 were evaluated at 2 years by their parents who completed the ASQ, by a pediatric clinical examination, and by the revised Brunet Lezine psychometric test with establishment of a DQ score. Detailed information regarding parental socio-economic status was available for 419 infants. At 2 years corrected age, 630 infants (89.6%) had an optimal neuromotor examination. Overall ASQ scores for predicting a DQ score ≤85 produced an area under the receiver operator curve value of 0.85 (95% Confidence Interval:0.82-0.87). An ASQ cut-off score of ≤220 had optimal discriminatory power for identifying a DQ score ≤85 with a sensitivity of 0.85 (95%CI:0.75-0.91), a specificity of 0.72 (95%CI:0.69-0.75), a positive likelihood ratio of 3, and a negative likelihood ratio of 0.21. The median value for ASQ was not significantly associated with socio-economic level or maternal education. ASQ is an easy and reliable tool regardless of the socio-economic status of the family to predict normal neurologic outcome in ex-premature infants at 2 years of age. ASQ may be beneficial with a low-cost impact to some follow-up programs, and helps to establish a genuine sense of parental involvement.
We investigated the challenging diagnostic case of a ventricular cystic glioneuronal tumor with papillary features, by RNA sequencing using the Illumina TruSight RNA Fusion panel. We did not retrieve ...the SLC44A1‐PRKCA fusion gene specific for papillary glioneuronal tumor, but an EWSR1‐PATZ1 fusion transcript. RT‐PCR followed by Sanger sequencing confirmed the EWSR1‐PATZ1 fusion. It matched with canonic EWSR1 fusion oncogene, juxtaposing the entire N‐terminal transcriptional activation domain of EWSR1 gene and the C‐terminal DNA binding domain of a transcription factor gene, PATZ1. PATZ1 protein belongs to the BTB‐ZF (broad‐complex, tramtrack and bric‐à‐brac ‐zinc finger) family. It directly regulates Pou5f1 and Nanog and is essential to maintaining stemness by inhibiting neural differentiation. EWSR1‐PATZ1 fusion is a rare event in tumors: it was only reported in six round cell sarcomas and in three gliomas of three exclusively molecular studies. The first reported glioma was a BRAFV600E negative ganglioglioma, the second a BRAFV600E negative glioneuronal tumor, not otherwise specified and the third, very recently reported, a high grade glioma, not otherwise specified. In our study, forty BRAFV600E negative gangliogliomas were screened by FISH using EWSR1 break‐apart probes. We performed methylation profiling for the index case and for seven out of the ten FISH positive cases. The index case clustered apart from other pediatric low grade glioneuronal entities, and specifically from the well‐defined ganglioglioma methylation group. An additional pediatric intraventricular ganglioglioma clustered slightly more closely with ganglioglioma, but showed differences from the main ganglioglioma group and similarities with the index case. Both cases harbored copy number variations at the PATZ1 locus. EWSR1‐PATZ1 gene fusion might define a new type of glioneuronal tumors, distinct from gangliogliomas.
ContextSupplementation of breast milk is difficult once infants suckle the breast and is often discontinued at end of hospitalisation and after discharge. Thus, breastfed preterm infants are exposed ...to an increased risk of nutritional deficit with a possible consequence on neurodevelopmental outcome.ObjectiveTo assess the relationship between breast feeding at time of discharge, weight gain during hospitalisation and neurodevelopmental outcome.DesignObservational cohort study.SettingTwo large, independent population-based cohorts of very preterm infants: the Loire Infant Follow-up Team (LIFT) and the EPIPAGE cohorts.Patients2925 very preterm infants alive at discharge.Main outcome measureSuboptimal neurodevelopmental outcome, defined as a score in the lower tercile, using Age and Stages Questionnaire at 2 years in LIFT and Kaufman Assessment Battery for Children Test at 5 years in EPIPAGE. Two propensity scores for breast feeding at discharge, one for each cohort, were used to reduce bias.ResultsBreast feeding at time of discharge concerned only 278/1733 (16%) infants in LIFT and 409/2163 (19%) infants in EPIPAGE cohort. Breast feeding is significantly associated with an increased risk of losing one weight Z-score during hospitalisation (LIFT: n=1463, adjusted odd ratio (aOR)=2.51 (95% CI 1.87 to 3.36); EPIPAGE: n=1417, aOR=1.55 (95% CI 1.14 to 2.12)) and with a decreased risk for a suboptimal neurodevelopmental assessment (LIFT: n=1463, aOR=0.63 (95% CI 0.45 to 0.87); EPIPAGE: n=1441, aOR=0.65 (95% CI 0.47 to 0.89) and an increased chance of having a head circumference Z-score higher than 0.5 at 2 years in LIFT cohort (n=1276, aOR=1.43 (95% CI 1.02 to 2.02)) and at 5 years in EPIPAGE cohort (n=1412, aOR=1.47 (95% CI 1.10 to 1.95)).ConclusionsThe observed better neurodevelopment in spite of suboptimal initial weight gain could be termed the ‘apparent breastfeeding paradox’ in very preterm infants. Regardless of the mechanisms involved, the current data provide encouragement for the use of breast feeding in preterm infants.
At the time of the 1889 world premiere of Massenet's Esclarmonde, Emmanuel Chabrier copiously annotated a copy of the piano-vocal score of this opera, which is considered, despite its unique ...features, one of the most Wagnerian works of the author. This remarkable and little-known document allows us once again to investigate the challenges of musical creation in a period when all dramatic composers were obliged to position themselves in relation to Wagner, particularly in France where lively debates were taking place between supporters and detractors of the master of Bayreuth. This new source also enriches our knowledge of two composers who, each in his own way, sought to respond to Wagnerian theater, all the while being inspired by it: from Wagner's example both composers drew subjects based on legends, used reminiscence motifs or enriched their orchestral and harmonic palettes with Wagnerian techniques; but Massenet remained faithful to closed vocal forms, which Chabrier rejected in favor of more continuous composition. Chabrier, however, shows himself captivated by Esclarmonde, for Massenet's score attests to the desire, very clear in both composers, to avoid blind submission to Wagner's influence, especially to the point of losing one's own identity.
The oncological impact of positive surgical margins (PSM) after robot-assisted partial nephrectomy (RAPN) is still under debate. We compared PSM and Negative Surgical Margins (NSM) in terms of ...recurrence-free survival (RFS), metastasis-free survival (MFS) and overall survival (OS) after RAPN, and we identified predictive factors of PSM. Multi-institutional study using the UroCCR database, which prospectively included 2166 RAPN between April 2010 and February 2021 (CNIL DR 2013-206; NCT03293563). Two groups were retrospectively compared: PSM versus NSM. Prognostic factors were assessed using Kaplan-Meyer curves with log-Rank test, cox hazard proportional risk model and logistic regression after univariate comparison. 136 patients had PSM (6.3%) and 2030 (93.7%) had NSM. During a median follow-up of 19 (9-36) months after RAPN, 160 (7.4%) recurrences were reported. Kaplan-Meier curves and analysis suggested that RFS, MFS and OS were not affected by a PSM (p = 0.68; 0.71; 0.88, respectively). In multivariate analysis predictors of PSM were a lower RENAL score (p = 0.001), longer warm ischemia time (WIT) (p = 0.003) and Chromophobe Renal Cell Carcinoma (chrRCC) (p = 0.043). This study found no impact of PSM on RFS, MFS or OS, and predictors of PSM were the RENAL score, WIT and chrRCC.
Preterm children are at greater risk of developmental impairment and require close follow-up for early and optimal medical care. Our goal was to examine use of the parent-completed Ages and Stages ...Questionnaire (ASQ) as a screening tool for neurodevelopmental disabilities in preterm infants at five years of age.
A total of 648 preterm children (<35 weeks gestational age) born between 2003 and 2004 and included in the regional Loire Infant Follow-up network were evaluated at five years of age. ASQ was compared with two validated tools (Intelligence Quotient and Global School Adaptation Score) and the impact of maternal education on the accuracy of this questionnaire was assessed.
Overall ASQ scores for predicting full-scale IQ<85 and GSA score produced an area under the receiver operating characteristic curve of 0.73±0.03 and 0.77±0.03, respectively. An ASQ cut-off value of 285 had optimal discriminatory power for identifying children with IQ scores<85 and GSA scores in the first quintile. ASQ values<285 were significantly associated with a higher risk of non-optimal neurologic outcomes (sensitivity of 0.80, specificity of 0.54 for IQ<85). ASQ values>285 were not distinctive for mild delay or normal development. In children with developmental delay, no difference was found when ASQ scores according to maternal education levels were analyzed.
ASQ at five years is a simple and cost-effective tool that can detect severe developmental delay in preterm children regardless of maternal education level, while its capacity to identify children with mild delay appears to be more limited.
The French Critical Zone research infrastructure, OZCAR-RI, gathers 20 observatories sampling various compartments of the critical zone, each having developed their own data management and ...distribution systems. A common information system (Theia/OZCAR IS) was built to make their in situ observation FAIR (findable, accessible, interoperable, reusable). The IS architecture was designed after consultation of the users, data producers and IT teams involved in data management. A common data model based on various metadata standards was defined to create information fluxes between observatories' ISs and the Theia/OZCAR IS. Controlled vocabularies were defined to develop a data discovery web portal offering a faceted search with various criteria, including variables names and categories that were harmonized in a thesaurus published on the web. This paper describes the IS architecture, the pivot data model and open-source solutions used to implement data discovery, and future steps to implement data downloading and interoperability services.
Purpose
To compare the oncological and perioperative outcomes of robot-assisted partial nephrectomy (RPN) and percutaneous thermal ablation (PTA) for treatment of T1 renal cell cancer (RCC) in ...patients older than 75 years.
Materials and methods
Retrospective national multicenter study included all patients older than 75 years treated for a T1 RCC by RPN or PTA between January 2010 and January 2021. Patients’ characteristics, tumor data, and perioperative and oncological outcomes were compared.
Results
A total of 205 patients for 209 procedures (143 RPN and 66 PTA) were included. In the PTA group, patients were older (80.4 ± 3.7 vs. 79 ± 3.7 years (
p
= 0.01)); frailer (ASA score (2.43 ± 0.6 vs. 2.17 ± 0.6 (
p
< 0.01)); and more frequently had a history of kidney surgery (16.7% 11/66 vs. 5.6% 8/143 (
p
= 0.01)) than in the RPN group. Tumors were larger in the RPN group (2.7 ± 0.7 vs. 3.2 ± 0.9 cm (
p
< 0.01)). Operation time, length of hospital stay, and increase of creatinine serum level were higher in RPN (respectively 92.1 ± 42.7 vs. 150.7 ± 61.3 min (
p
< 0.01); 1.7 ± 1.4 vs. 4.2 ± 3.4 days (
p
< 0.01); 1.9 ± 19.3% vs. 10.1 ± 23.7 (
p
= 0.03)). Disease-free survival and time to progression were similar (respectively, HR 2.2; 95% CI 0.88–5.5;
p
= 0.09; HR 2.1; 95% CI 0.86–5.2;
p
= 0.1). Overall survival was shorter for PTA that disappeared after Cox adjusting model (HR 3.3; 95% CI 0.87–12.72;
p
= 0.08).
Conclusion
Similar oncological outcomes are observed after PTA and RPN for T1 RCC in elderly patients.
Clinical relevance statement
Robot-assisted partial nephrectomy and percutaneous thermal ablation have similar oncological outcomes for T1a kidney cancer in patients over 75 years; however, operative time, decrease in renal function, and length of hospital stay were lower with ablation.
Key Points
•
After adjusting model for age and ASA score, similar oncological outcomes are observed after percutaneous thermal ablation and robot-assisted partial nephrectomy for T1 renal cell cancer in elderly patients
.
•
Operation time, length of hospital stay, and increase of creatinine serum level were higher in the robot-assisted partial nephrectomy group.
Background
The prognostic impact of renal cell carcinoma (RCC) morphotype remains unclear in patients who undergo partial nephrectomy (PN). Our objective was to determine the risk factors for ...recurrence after PN, including RCC morphotype.
Methods
Patients with RCC who had undergone PN were extracted from the prospective, national French database, UroCCR. Patients with genetic predisposition, bilateral or multiple tumours, and those who had undergone secondary totalization were excluded. Primary endpoint was 5-year, recurrence-free survival (RFS), and secondary endpoint was overall survival (OS). Risk factors for recurrence were assessed by multivariable Cox regression analysis.
Results
Overall, 2,767 patients were included (70% male; median age: 61 years interquartile range (IQR) 51-69). Most (71.5%) of the PN procedures were robot-assisted. Overall, 2,573 (93.0%) patients were recurrence free, and 74 died (2.7%). Five-year RFS was 84.9% (IQR 82.4-87.4). A significant difference in RFS was observed between RCC morphotypes (
p
< 0.001). Surgical margins (hazard ratio HR = 2.0 95% confidence interval (CI): 1.3-3.2,
p
< 0.01), pT stage >1 (HR = 2.6 95% CI: 1.8-3.7,
p
< 0.01) and Fuhrmann grade >2 (HR = 1.9 95% CI: 1.4-2.6,
p
< 0.001) were risk factors for recurrence, whereas chromophobe subtype was a protective factor (HR = 0.08 95% CI: 0.01-0.6,
p
= 0.02). Five-year OS was 94.0% 92.4-95.7, and there were no significant differences between RCC subgroups (
p
= 0.06). The main study limitation was its design (multicentre national database), which may be responsible for declarative bias.
Conclusions
Chromophobe morphotype was significantly associated with better RFS in RCC patients who underwent PN. Conversely, pT stage, Fuhrman group and positive surgical margins were risk factors for recurrence.
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