Extreme hyperferritinemia in the setting of acute myeloid leukaemia: a case report of hemophagocytic lymphohistiocytosis.

Introduction: Major hyperferritinemia is a rare feature in clinical laboratories associated with a wide variety of disorders, including hemophagocytic lymphohistiocytosis (HLH). The diagnosis of HLH is based on clinical and biological criteria, such as those proposed by the Histiocyte Society. However, several of these criteria are not relevant in the specific setting of hematologic malignancies. Materials and methods: A 69-year-old male was treated for an acute myeloid leukaemia. On day 15 after the start of chemotherapy, he developed severe sepsis with high fever, low blood pressure and hepatosplenomegaly. Results: Blood tests were marked by extreme hyperferritinemia (191,000 µg/L, reference range: 26-388 µg/L) with increased C-reactive protein (87.0 mg/L) and procalcitonin (1.94 µg/L) and aspartate aminotransferase (499 U/L 37 °C) in the setting of chemotherapy-induced aplasia. This unusual extreme ferritinemia led to suspect HLH triggered by an invasive infection. Under intensive treatment, the clinical status improved and ferritin levels significantly decreased. Conclusions: The diagnosis of HLH is usually based on clinical and biological criteria, mainly fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis and hyperferritinemia. In this patient, the diagnosis of HLH was challenging because several criteria, such as hypertriglyceridemia, hemophagocytosis and hypofibrinogenemia, were absent. In addition, some criteria of HLH are not relevant in the setting of hematologic malignancy, in which fever, splenomegaly, cytopenias and elevated lactate dehydrogenase are commonly observed independently of HLH. This unusual case of extremely high ferritinemia emphasizes the important weight of the ferritin level for the diagnosis of HLH in adult patients in the setting of hematologic malignancies

The construct validity of the Dutch personality inventory for DSM-5 personality disorders (PID-5) in a clinical sample

The factor structure and the convergent validity of the Personality Inventory for DSM-5 (PID-5), a self-report questionnaire designed to measure personality pathology as advocated in the fifth edition, Section III of Diagnostic and Statistical Manual of Mental Disorders (DSM-5), are already demonstrated in general population samples, but need replication in clinical samples. In 240 Flemish inpatients, we examined the factor structure of the PID-5 by means of exploratory structural equation modeling. Additionally, we investigated differences in PID-5 higher order domain scores according to gender, age and educational level, and explored convergent and discriminant validity by relating the PID-5 with the Dimensional Assessment of Personality PathologyBasic Questionnaire and by comparing PID-5 scores of inpatients with and without a DSM-IV categorical personality disorder diagnosis. Our results confirmed the original five-factor structure of the PID-5. The reliability and the convergent and discriminant validity of the PID-5 proved to be adequate. Implications for future research are discussed

The structure of a gene network reveals 7 biological sub-graphs underlying eQTLs in pig

International audienceIntegrative and system biology is a very promising tool for deciphering the biological and genetic mechanisms underlying complex traits. Transcriptomic analyses, in combination with genomic polymorphism, for instance, can give interesting insight on the genetic control of gene expression (eQTL studies). When hundreds of genes are detected with a link between their expression and some genetic polymorphisms (eQTL), the following question raises: what are the biological underlying functions? One tool is the use of a gene network, displaying interactions between genes with a genetic control (having at least an eQTL). There exist several possibilities for inferring a gene network: literature mining (using softwares such as Ingenuity) or inference from gene expression data. Although the first framework is a useful tool, it has some limitations: there is still a serious problem of lack of annotation in the pig genome, and a bias in information provided by Ingenuity (literature mainly devoted to Human, Mouse and Rat). We will hence explore in this work the inference of gene network from expression data. One simple method of inference was focused on, that has proven useful: Gaussian networks (Schäfer and Strimmer 2005). The following problem to be faced is the interpretation of such a "large" network (more than 100 genes). The aim of this study is to propose an adequate method for deciphering the structure of large gene networks. With the use of a good clustering of graph, the structure of one graph can be highlighted, and can reveal several sub graphs, each corresponding to particular biological functions

New Control Method For Fixed Speed Compressors With Side Stream

Case StudyProblem statement: Operability issues were experienced on flash gas back-to-back compressors with lower molecular weight side stream, driven by fixed speed electric motor: • Failed attempts of start-up/loading • During normal operation compressors went off-line due to process upset (e.g. inlet flow decrease) • Unable to operate parallel trains simultaneously. Consequent loss of production

Regulation of the CRL4(Cdt2) ubiquitin ligase and cell-cycle exit by the SCF(Fbxo11) ubiquitin ligase

F-box proteins and DCAF proteins are the substrate binding subunits of the Skp1-Cul1-F-box protein (SCF) and Cul4-RING protein ligase (CRL4) ubiquitin ligase complexes, respectively. Using affinity purification and mass spectrometry, we determined that the F-box protein FBXO11 interacts with CDT2, a DCAF protein that controls cell-cycle progression, and recruits CDT2 to the SCF(FBXO11)complex to promote its proteasomal degradation. In contrast to most SCF substrates, which exhibit phosphodegron-dependent binding to F-box proteins, CDK-mediated phosphorylation of Thr464 present in the CDT2 degron inhibits recognition by FBXO11. Finally, our results show that the functional interaction between FBXO11 and CDT2 is evolutionary conserved from worms to humans and plays an important role in regulating the timing of cell-cycle exit.Fil: Rossi, Mario. University Of New York; Estados Unidos. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigación en Biomedicina de Buenos Aires; ArgentinaFil: Duan, Shanshan. University Of New York; Estados Unidos. Howard Hughes Medical Institute; Estados UnidosFil: Jeong, Yeon Tae. University Of New York; Estados UnidosFil: Horn, Moritz. Max Planck Institute for Biology of Ageing; Alemania. University of Cologne; AlemaniaFil: Saraf, Anita. The Stowers Institute for Medical Research; Estados UnidosFil: Florens, Laurence. The Stowers Institute for Medical Research; Estados UnidosFil: Washburn, Michael P.. The Stowers Institute for Medical Research; Estados Unidos. University of Kansas; Estados UnidosFil: Antebi, Adam. Max Planck Institute for Biology of Ageing; Alemania. University of Cologne; AlemaniaFil: Pagano, Michele. University Of New York; Estados Unidos. Howard Hughes Medical Institute; Estados Unido

The Relationship between Non-Suicidal Self-Injury and the UPPS-P Impulsivity Facets in Eating Disorders and Healthy Controls

In the present study, we investigated the association between Non-Suicidal Self-Injury (NSSI) and the UPPS-P impulsivity facets in eating disorder patients and healthy controls. The prevalence of NSSI in eating disorder (ED) patients ranged from 17% in restrictive an- orexia nervosa (AN-R) patients to 43% in patients with bulimia nervosa (BN). In healthy con- trols (HC), the prevalence of NSSI was 19%. Eating disorder patients from the binge eating/ purging type showed significantly more NSSI compared to restrictive ED and HC partici- pants. Binge-eating/purging ED patients also scored significantly higher on Negative/Posi- tive Urgency, Lack of Premeditation and Lack of Perseverance compared to HC and restrictive ED patients. Comparable findings were found between ED patients and HC with and without NSSI; ED patients and HC with NSSI scored significantly higher in four of the five UPPS-P dimensions compared to participants without NSSI; Sensation Seeking was the exception. Finally, the presence of NSSI in HC/ED patients was particularly predicted by low levels of Perseverance. Therefore, the treatment of ED patients with NSSI certainly needs to focus on the training of effortful control

Space radiation parameters for EUI and the Sun Sensor of Solar Orbiter, ESIO and JUDE instruments

This paper presents predictions of space radiation parameters for four space instruments performed by the Centre Spatial de Liège (ULg – Belgium); EUI, the Extreme Ultra-violet Instrument, on-board the Solar Orbiter platform; ESIO, Extreme-UV solar Imager for Operations, and JUDE, the Jupiter system Ultraviolet Dynamics Experiment, which was proposed for the JUICE platform. For Solar Orbiter platform, the radiation environment is defined by ESA environmental specification and the determination of the parameters is done through ray-trace analyses inside the EUI instrument. For ESIO instrument, the radiation environment of the geostationary orbit is defined through simulations of the trapped particles flux, the energetic solar protons flux and the galactic cosmic rays flux, taking the ECSS standard for space environment as a guideline. Then ray-trace analyses inside the instrument are performed to predict the particles fluxes at the level of the most radiation-sensitive elements of the instrument. For JUICE, the spacecraft trajectory is built from ephemeris files provided by ESA and the radiation environment is modeled through simulations by JOSE (Jovian Specification Environment model) then ray-trace analyses inside the instrument are performed to predict the particles fluxes at the level of the most radiation-sensitive elements of the instrument

Modelling the Dynamics of an Aedes albopictus Population

We present a methodology for modelling population dynamics with formal means of computer science. This allows unambiguous description of systems and application of analysis tools such as simulators and model checkers. In particular, the dynamics of a population of Aedes albopictus (a species of mosquito) and its modelling with the Stochastic Calculus of Looping Sequences (Stochastic CLS) are considered. The use of Stochastic CLS to model population dynamics requires an extension which allows environmental events (such as changes in the temperature and rainfalls) to be taken into account. A simulator for the constructed model is developed via translation into the specification language Maude, and used to compare the dynamics obtained from the model with real data.Comment: In Proceedings AMCA-POP 2010, arXiv:1008.314