Accessing the soot-related radiative heat feedback in a flame spreading in microgravity: optical designs and associated limitations

Novel, high-fidelity results related to soot from microgravity flames were obtained by an international topical team on fire safety in space. More specifically, embedded optical techniques for evaluation of the soot-related radiative feedback to the base material from a spreading non-premixed flame in microgravity were developed. The configuration used a non-buoyant axisymmetric flame propagating in an opposed laminar stream overa Low Density PolyEthylene coating of an electrical wire. Within this context, both the standard Broadband Two Color Pyrometry (B2CP) and its recent extension Broadband Modulated Absorption/Emission (BMAE) technique can be deployed to measure the spatial distribution of soot temperature and volume fraction within the flame. Both fields are then processed to establish the field of local radiative balance attributed to soot within the flame, and ultimately the soot contribution to the radiative flux to the wire. The present study first assesses the consistency of the methodology contrasting an experimental frame and a synthetic one, the latter being produced by a signal modeling that processes fields delivered by a numerical simulation of the configuration as inputs. Using the synthetic signals obtained, the fields of local radiative balance within the flame are then computed and significant discrepancies were disclosed locally between the fields originating from the synthetic BMAE and B2CP inputs. Nevertheless, the subsequent evaluation of the soot-related radiative heat feedback to the wire shows that a weak deviation among the techniques implemented is expected. This finding is corroborated by similar evaluations conducted with experimental BMAE and B2CP measurements obtained in parabolic flights. As BMAE is implemented in an ISS configuration within the SCEM rig, BMAE and B2CP will soon provide long-duration soot observations in microgravity. In order to contrast the upcoming results, this current study quantifies discrepancies originating from the post-processing regarding soot temperature and volume fraction, and shows that the radiative feedback evaluation from both methods should be consistent

Kasabach-Merritt phenomenon and prenatal counseling: a case series.

Kasabach-Merritt phenomenon can be encountered in the perinatal period. No consensus exists regarding prenatal management. We report one prenatal case leading to therapeutic abortion and one neonatal case, successfully treated by a multimodal therapy. Prenatal counseling should include the possibility of neonatal multimodal treatment that can lead to favorable outcomes

Accessing the soot-related radiative heat feedback in a flame spreading in microgravity: Optical designs and associated limitations

Novel, high-fidelity results related to soot from microgravity flames were obtained by an international topical team on fire safety in space. More specifically, embedded optical techniques for evaluation of the soot-related radiative feedback to the base material from a spreading non-premixed flame in microgravity were developed. The configuration used a non-buoyant axisymmetric flame propagating in an opposed laminar stream over a Low Density PolyEthylene coating of an electrical wire. Within this context, both the standard Broadband Two Color Pyrometry (B2CP) and its recent extension Broadband Modulated Absorption/Emission (BMAE) technique can be deployed to measure the spatial distribution of soot temperature and volume fraction within the flame. Both fields are then processed to establish the field of local radiative balance attributed to soot within the flame, and ultimately the soot contribution to the radiative flux to the wire. The present study first assesses the consistency of the methodology contrasting an experimental frame and a synthetic one, the latter being produced by a signal modeling that processes fields delivered by a numerical simulation of the configuration as inputs. Using the synthetic signals obtained, the fields of local radiative balance within the flame are then computed and significant discrepancies were disclosed locally between the fields originating from the synthetic BMAE and B2CP inputs. Nevertheless, the subsequent evaluation of the soot-related radiative heat feedback to the wire shows that a weak deviation among the techniques implemented is expected. This finding is corroborated by similar evaluations conducted with experimental BMAE and B2CP measurements obtained in parabolic flights. As BMAE is implemented in an ISS configuration within the SCEM rig, BMAE and B2CP will soon provide long-duration soot observations in microgravity. In order to contrast the upcoming results, this current study quantifies discrepancies originating from the post-processing regarding soot temperature and volume fraction, and shows that the radiative feedback evaluation from both methods should be consistent

Bacterial Deposition of Gold on Hair: Archeological, Forensic and Toxicological Implications

Trace metal analyses in hair are used in archeological, forensic and toxicological investigations as proxies for metabolic processes. We show metallophilic bacteria mediating the deposition of gold (Au), used as tracer for microbial activity in hair post mortem after burial, affecting results of such analyses. Methodology/Principal Findings Human hair was incubated for up to six months in auriferous soils, in natural soil columns (Experiment 1), soils amended with mobile Au(III)-complexes (Experiment 2) and the Au-precipitating bacterium Cupriavidus metallidurans (Experiment 3), in peptone-meat-extract (PME) medium in a culture of C. metallidurans amended with Au(III)-complexes (Experiment 4), and in non-auriferous soil (Experiment 5). Hair samples were analyzed using scanning electron microscopy, confocal microscopy and inductively coupled plasma-mass spectrometry. In Experiments 1–4 the Au content increased with time (P = 0.038). The largest increase was observed in Experiment 4 vs. Experiment 1 (mean = 1188 vs. 161 µg Kg−1, Fisher's least significance 0.001). The sulfur content, a proxy for hair metabolism, remained unchanged. Notably, the ratios of Au-to-S increased with time (linear trend P = 0.02) and with added Au and bacteria (linear trend, P = 0.005), demonstrating that larger populations of Au-precipitating bacteria and increased availability of Au increased the deposition of Au on the hair. Conclusion/Significance Interactions of soil biota with hair post mortem may distort results of hair analyses, implying that metal content, microbial activities and the duration of burial must be considered in the interpretation of results of archeological, forensic and toxicological hair analyses, which have hitherto been proxies for pre-mortem metabolic processesGenevieve Phillips, Frank Reith, Clifford Qualls, Abdul-Mehdi Ali, Mike Spilde and Otto Appenzelle

Phosphofructo-1-Kinase Deficiency Leads to a Severe Cardiac and Hematological Disorder in Addition to Skeletal Muscle Glycogenosis

Mutations in the gene for muscle phosphofructo-1-kinase (PFKM), a key regulatory enzyme of glycolysis, cause Type VII glycogen storage disease (GSDVII). Clinical manifestations of the disease span from the severe infantile form, leading to death during childhood, to the classical form, which presents mainly with exercise intolerance. PFKM deficiency is considered as a skeletal muscle glycogenosis, but the relative contribution of altered glucose metabolism in other tissues to the pathogenesis of the disease is not fully understood. To elucidate this issue, we have generated mice deficient for PFKM (Pfkm−/−). Here, we show that Pfkm−/− mice had high lethality around weaning and reduced lifespan, because of the metabolic alterations. In skeletal muscle, including respiratory muscles, the lack of PFK activity blocked glycolysis and resulted in considerable glycogen storage and low ATP content. Although erythrocytes of Pfkm−/− mice preserved 50% of PFK activity, they showed strong reduction of 2,3-biphosphoglycerate concentrations and hemolysis, which was associated with compensatory reticulocytosis and splenomegaly. As a consequence of these haematological alterations, and of reduced PFK activity in the heart, Pfkm−/− mice developed cardiac hypertrophy with age. Taken together, these alterations resulted in muscle hypoxia and hypervascularization, impaired oxidative metabolism, fiber necrosis, and exercise intolerance. These results indicate that, in GSDVII, marked alterations in muscle bioenergetics and erythrocyte metabolism interact to produce a complex systemic disorder. Therefore, GSDVII is not simply a muscle glycogenosis, and Pfkm−/− mice constitute a unique model of GSDVII which may be useful for the design and assessment of new therapies

Clinical spectrum of MTOR-related hypomelanosis of Ito with neurodevelopmental abnormalities

PURPOSE: Hypomelanosis of Ito (HI) is a skin marker of somatic mosaicism. Mosaic MTOR pathogenic variants have been reported in HI with brain overgrowth. We sought to delineate further the pigmentary skin phenotype and clinical spectrum of neurodevelopmental manifestations of MTOR-related HI. METHODS: From two cohorts totaling 71 patients with pigmentary mosaicism, we identified 14 patients with Blaschko-linear and one with flag-like pigmentation abnormalities, psychomotor impairment or seizures, and a postzygotic MTOR variant in skin. Patient records, including brain magnetic resonance image (MRI) were reviewed. Immunostaining (n = 3) for melanocyte markers and ultrastructural studies (n = 2) were performed on skin biopsies. RESULTS: MTOR variants were present in skin, but absent from blood in half of cases. In a patient (p.[Glu2419Lys] variant), phosphorylation of p70S6K was constitutively increased. In hypopigmented skin of two patients, we found a decrease in stage 4 melanosomes in melanocytes and keratinocytes. Most patients (80%) had macrocephaly or (hemi)megalencephaly on MRI. CONCLUSION: MTOR-related HI is a recognizable neurocutaneous phenotype of patterned dyspigmentation, epilepsy, intellectual deficiency, and brain overgrowth, and a distinct subtype of hypomelanosis related to somatic mosaicism. Hypopigmentation may be due to a defect in melanogenesis, through mTORC1 activation, similar to hypochromic patches in tuberous sclerosis complex

Histologic assessment of biliary obstruction with different percutaneous endoluminal techniques

BACKGROUND: Despite the sophisticated cross sectional image techniques currently available, a number of biliary stenosis or obstructions remain of an uncertain nature. In these pathological conditions, an "intrinsic" parietal alteration is the cause of biliary obstruction and it is very difficult to differentiate benign from malignant lesions using cross-sectional imaging procedures alone. We evaluated the efficacy of different endoluminal techniques to achieve a definitive pathological diagnosis in these situations. METHODS: Eighty patients underwent brushing, and or biopsy of the biliary tree through an existing transhepatic biliary drainage route. A subcoort of 12 patients needed balloon-dilatation of the bile duct and the material covering the balloon surface was also sent for pathological examination (balloon surface sampling). Pathological results were compared with surgical findings or with long-term clinical and instrumental follow-ups. Success rates, sensitivity, specificity, accuracy, confidential intervals, positive predictive value and negative predictive value of the three percutaneous techniques in differentiating benign from malignant disease were assessed. The agreement coefficient of biopsy and brushing with final diagnosis was calculated using the Cohen's "K" value. RESULTS: Fifty-six patients had malignant strictures confirmed by surgery, histology, and by clinical follow-ups. Success rates of brushing, balloon surface sampling, and biopsy were 90.7, 100, and 100%, respectively. The comparative efficacy of brushing, balloon-surface sampling, and biopsy resulted as follows: sensitivity of 47.8, 87.5, and 92.1%, respectively; specificity of 100% for all the techniques; accuracy of 69.2, 91.7 and 93.6%, Positive Predictive Value of 100% for all the procedures and Negative Predictive Value of 55, 80, and 75%, respectively. CONCLUSIONS: Percutaneous endoluminal biopsy is more accurate and sensitive than percutaneous bile duct brushing in the detection of malignant diseases (p < 0.01)

Influence of metals and metalloids on the composition and fluorescence quenching of the extracellular polymeric substances produced by the polymorphic fungus <i>Aureobasidium pullulans</i>

Aureobasidium pullulansis a ubiquitous and widely distributed fungus in the environment, and exhibits substantial tolerance against toxic metals. However, the interactions between metals and metalloids with the copious extracellular polymeric substances (EPS) produced byA. pullulansand possible relationships to tolerance are not well understood. In this study, it was found that mercury (Hg) and selenium (Se), as selenite, not only significantly inhibited growth ofA. pullulansbut also affected the composition of produced EPS. Lead (Pb) showed little influence on EPS yield or composition. The interactions of EPS fromA. pullulanswith the tested metals and metalloids depended on the specific element and their concentration. Fluorescence intensity measurements of the EPS showed that the presence of metal(loid)s stimulated the production of extracellular tryptophan-like and aromatic protein-like substances. Examination of fluorescence quenching and calculation of binding constants revealed that the fluorescence quenching process for Hg; arsenic (As), as arsenite; and Pb to EPS were mainly governed by static quenching which resulted in the formation of a stable non-fluorescent complexes between the EPS and metal(loid)s. Se showed no significant interaction with the EPS according to fluorescence quenching. These results provide further understanding of the interactions between metals and metalloids and EPS produced by fungi and their contribution to metal(loid) tolerance