Distinct attentional and executive profiles in neurofibromatosis type 1: Is there difference with primary attention deficit-hyperactivity disorder?

PURPOSE: Attentional and executive dysfunctions are the most frequent cognitive disorders in neurofibromatosis type 1 (NF1), with a high prevalence of attention deficit-hyperactivity disorder (ADHD). We (i) compared attentional profiles between NF1 children with and without ADHD and children with primary ADHD criteria and (ii) investigated the possible relationship between attentional disorders and "unidentified bright objects" (UBOs) in NF1. METHODS: This retrospective study included 47 NF1 children, 25 with ADHD criteria (NF1+adhd group), matched for age, sex, and cognitive level with 47 children with primary ADHD (ADHD group). We collected computer task (sustained-attention, visuomotor-decision, inhibition, and cognitive-flexibility tasks) scores normalized for age and sex, and brain magnetic resonance imaging data. RESULTS: (i) Working memory was impaired in all groups. (ii) Omissions (p < 0.002) and response-time variability (p < 0.05) in sustained-attention and visuomotor-decision tasks and errors (p < 0.02) in the cognitive-flexibility task were lower for the NFI+adhd and ADHD groups than for the NF1-no-adhd group. (iii) The NF1+adhd group had slower response times (p ≤ 0.02) for inhibition and visuomotor-decision tasks than the other groups. (iv) We found no relevant association between cognitive performance and UBOs. CONCLUSIONS: NF1 children with ADHD have an attentional and executive functions deficit profile similar to that of children with primary ADHD, but with a slower response-time, increasing learning difficulties. The atypical connectivity of fronto-striatal pathways, poorer dopamine homeostasis, and increased GABA inhibition observed in NF1 renders vulnerable the development of the widely distributed neural networks that support attentional, working-memory, and executive functions.

Leukocyte cell population data in patients with cardiac surgery and cardiopulmonary bypass: A potential readily available tool to monitor immunity.

Purpose: Cardiac surgery with cardiopulmonary bypass triggers sterile inflammation that is responsible for post-operative morbidity. Automated flow cytometry devices used for leucocyte count provide cell population data (CPD) regarding fluorescence intensity, size and granularity of leukocytes that have never been studied in the context of sterile inflammation. Our objective was to explore leukocyte cell population data in patients undergoing cardiac surgery with cardiopulmonary bypass in order to determine whether CPD could be used to monitor immune cell activation. Methods: This is an ancillary study of a cohort of patients undergoing cardiac surgery with cardiopulmonary bypass. Cell population data (CPD) extracted from a routine automated flow cytometer were analyzed (Fluorescence targeted to nucleic acids). The time points of interest were: pre-operative, postoperative and 5 days after surgery. The variations in those parameters were studied. Data were then compared between patients according to the occurrence of a composite criteria (supra-ventricular arrythmia, stroke, acute renal failure, and/or death). Results: Data from 1453 patients were analyzed. The neutrophil count, fluorescence granularity (NE-SCC), intensity (NE-SFL) and size (NE-FSC) increased with surgery. Heterogeneity of neutrophils decreased in terms of fluorescence granularity (NE-WX) and size (NE-WZ) but increased in terms of intensity (NE-WY). The lymphocyte count decreased with surgery. While fluorescence granularity (LY-X) and size increased (LY-Z), Lymphocyte intensity decreased (LY-Y). Lymphocytes were less heterogeneous in terms of their granularity, size and intensity after surgery (LY-WX, LY-WY, LY-WZ). Patients who developed the composite complication criteria had a higher pre-operative neutrophil count (5.08 [3.89;6.95] vs 4.76 [3.60;6.13], p = 0.02; AUC = 0.56 [0.51;0.60]), and more heterogeneous neutrophils in terms of fluorescence granularity (NE-WX, AUC = 0.57 [0.52;0.62]) and intensity (NE-WY, AUC 0.61 [0.56;0.65]). Those patients also had lower pre-operative lymphocyte count (1.49 [1.10;1.14] vs 1.81 [1.39;2.39], p<0.01, AUC = 0.61 [0.57;0.66]) and fluorescence granularity (LY-X, AUC = 0.57 [0.53;0.62]). NE-WX, NE-WY and LY-X were associated with post-operative complications after adjustment on the EuroSCORE 2 (adjusted odd ratio of 1.01 [1.00;1.02]; 1.01 [1.00;1.01] and 1.08 [1.02;1.15] respectively). Conclusion: Cardiac surgery with cardiopulmonary bypass was associated with substantial alterations of CPD probably reflecting leukocytes activation in sterile inflammation. Pre-operative NE-WX, NE-WY and LY-X biomarkers levels were associated with post-operative complications, independently of the EuroSCORE 2. Such routine, unexploited and low cost parameters might represent useful tools likely to monitor immune function and predict outcomes for patients undergoing cardiac surgery. Our findings requires validation on a larger external cohort.

In a tertiary maternity hospital, when should a paediatrician be present in the delivery room?

OBJECTIVE: 10% of newborns require positive pressure ventilation (PPV) at birth. There are few data on prenatal or early postnatal factors that are predictive of the need for a paediatrician in the delivery room. The study analysed prenatal obstetric and early postnatal factors associated with the requirement for paediatrician assistance in this setting. METHODS: Over a three-month period, all consecutive births in a tertiary hospital's maternity unit were prospectively evaluated with regard to the need for paediatrician assistance (requested either before or after the delivery), the requirement for resuscitation, and transfer to a neonatal intensive care unit (NICU). RESULTS: For a total of 584 consecutive births, paediatrician assistance was requested before delivery in 170 cases (30.5%) and after in 78 cases (13.3%). 78% of the newborns requiring PPV, 95.8% of those requiring endotracheal intubation and 86.3% of those requiring transfer to the NICU matched recently published prenatal criteria for paediatrician assistance. Along with a low Apgar score and a cord blood pH <7.20, these criteria covered 95% of the prenatal and early postnatal requests for paediatrician assistance. CONCLUSIONS: These criteria for neonatal resuscitation in the delivery room would enable medical staff to anticipate the need for paediatrician assistance.

The IL-4 rs2070874 polymorphism may be associated with the severity of recurrent viral-induced wheeze.

BACKGROUND: Childhood recurrent wheezing and consequently asthma corresponds to various phenotypes. Our aim was to link genetic variants of asthma candidate genes to the phenotypes of early onset wheezing. STUDY DESIGN: We included very young consecutive children presenting with recurrent wheezing who had been evaluated for the severity of wheezing, associated atopic comorbidities, and tested for biomarkers of atopy and inflammation. All were genotyped for 16 single nucleotide polymorphisms (SNPs) linked with asthma or atopy. An unsupervised hierarchical bottom-up method was used for clustering the phenotypes and a multinomial logistic regression was performed for each individual SNP. RESULTS: We replicated the three phenotypes previously described Trousseau Asthma Program in 317 children aged 21.5 ± 7.9 months: cluster 1 (nonatopic uncontrolled severe wheeze), n = 207, a severe viral-induced wheeze, cluster 2 (atopic multiple trigger wheeze), n = 61, with multiple allergic comorbidities, and cluster 3 (episodic viral wheeze), n = 49, a mild viral-induced wheeze. The TT-genotype of the IL-4 rs2070874 polymorphism was significantly associated with the nonatopic uncontrolled severe wheeze compared to the episodic viral wheeze (OR 7.9; CI95% [2.5-25.3]; P = 0.001). CONCLUSION: Association between the TT-genotype of IL-4 rs2070874 polymorphism and a severe phenotype of viral-induced wheeze further underlines the role IL-4 plays in the inflammation pathway leading to viral respiratory infections.

Polyethylene bag wrapping to prevent hypothermia during percutaneous central venous catheter insertion in the preterm newborn under 32 weeks of gestation.

AIM: In preterm neonates, during nursing procedures, body temperature decreases. This study evaluates the interest of polyethylene bag wrapping to prevent this decrease during percutaneous central venous catheter (PCVC) insertion procedure, in preterm neonates under 32 weeks of gestation nursed in closed incubators. METHODS: This prospective observational study compared two periods: [May 2009-September 2009]: "without polyethylene bag wrapping" and [October 2009-March 2010]: "with polyethylene bag wrapping". The main criterion was newborn skin temperature at the end of the procedure. RESULTS: There was no difference between the two groups for skin temperature before the procedure (36.9 ± 0.3 °C versus 36.9 ± 0.3 °C; p = NS). The skin temperature at the end of the procedure was lower in the "without bag wrapping" group (36.0 ± 0.5 °C) compared to the "bag wrapping" group (36.4 C ± 0.5 °C; p = 0.01). Furthermore, no skin temperature at the end was higher than 37.4 °C in the bag wrapping group. CONCLUSION: The use of a polyethylene bag was effective in decreasing skin temperature fall during a PCVC insertion procedure in our population. No side effects were observed. The benefit of prolonged wrapping or for shorter procedure should be evaluated.

Ex vivo MRI of axillary lymph nodes in breast cancer.

PURPOSE: To provide a strategy for precise co-localization of lymph nodes on axillary lymph-node dissection (ALND) specimens both on pathology and MR. To identify nodal features suggestive of metastatic involvement on a node-to-node basis. MATERIALS AND METHODS: National Institutional review-board approved this prospective study of 18 patients with breast cancer referred for ALND. Ex vivo T1 and inversion recovery (IR) T2 WI of ALND specimens tightly positioned within scaled plastic cranes was performed immediately after surgery. The correspondence of MR-based or pathologically based nodes location was assessed. The MR size and morphological presentation of metastatic and normal nodes were compared (Student's t-test or Mann-Whitney test). Quantitative variables were compared using Pearson coefficient. RESULTS: 207 nodes were retrieved on pathology and 165 on MR. MR-pathological correlation of nodes location was high regarding MR-identified nodes (r=0.755). An MR short axis threshold of 4mm yielded the best predictive value for metastatic nodal involvement (Se=78.6%; Sp=62.3%). Irregular contours (Se=35.7%; Sp=96.7%), central nodal hyper-intensity on IR T2 WI (Se=57.1%; Sp=91.4%), and a cortical thickness above 3mm (Se=63.6%; Sp=83.2%) were significantly associated with metastatic involvement. CONCLUSION: Ex vivo MR allows node-to-node correlation with pathology. Morphological MR criteria can suggest metastatic involvement.