Miracle-dz/UniFAR-FTUs huggingface dataset:
Head and Neck MRI Findings in CHARGE Syndrome

SUMMARY: Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and deafness (CHARGE) syndrome is a disorder with multiple congenital anomalies seen on imaging. A retrospective review of 10 patients with CHARGE syndrome who underwent MR imaging of the brain as part of a preoperative evaluation for cochlear implantation was conducted. Structural abnormalities of the entire MR imaging of the head were evaluated, including the auditory system, olfactory system, face, skull base, and central nervous system. The most frequent MR imaging findings included dysplasias of the semicircular canals and hypoplasia of the frontal lobe olfactory sulci. Less frequent findings included cleft lip/palate and coloboma. Our study uncovered new findings of a J-shaped sella, dorsal angulation of the clivus, and absent/atrophic parotid glands, not previously described in patients with CHARGE. Our results emphasize the utility of MR imaging in the diagnosis and management of patients with CHARGE syndrome.
The variable P5 proteins of typeable and non‐typeable Haemophilus influenzae target human CEACAM1

Haemophilus influenzae, a commensal of the human respiratory mucosa, is an important cause of localized and systemic infections. We have recently shown that numerous strains of capsulate (typeable) and acapsulate (non‐typeable) H. influenzae target the carcinoembryonic antigen (CEA) family of cell adhesion molecules (CEACAMs). Moreover, the ligands appeared to be antigenically variable and, when using viable typeable bacteria, their adhesive functions were inhibited by the presence of capsule. In this report, we show that the antigenically variable outer membrane protein, P5, expressed by typeable and non‐typeable H. influenzae targets human CEACAM1. Variants and mutants lacking the expression of P5 of all strains tested were unable to target purified soluble receptors. A non‐typeable strain that did not interact with CEACAM1 was made adherent to both the soluble receptors and CEACAM1‐transfected Chinese hamster ovary cells by transformation with the P5 gene derived from the adherent typeable strain Rd. However, several H. influenzae mutants lacking P5 expression continued to bind the cell‐bound CEACAM1 receptors. These observations suggest that (i) CEACAM1 alone can support P5 interactions and (ii) some strains contain additional ligands with the property to target CEACAM1 but require the receptor in the cellular context. The identification of a common ligand in diverse strains of H. influenzae and the presence of multiple ligands for the same receptor suggests that targeting of members of the CEACAM family of receptors may be of primary significance in colonization and pathogenesis of H. influenzae strains.
Modelling non-systemic pesticide residues in fruits with initial deposit variability and weather effects

A flexible and generic model was developed to predict the decline of residues of a non-systemic pesticide for both single and multi-spray situations as well as for different tree canopy zones. The model predicts not only the average residue levels, but also the confidence interval of the residues through either a deterministic or a stochastic approach. This generic model includes several key aspects of residue fates in the environment: initial deposit, physical loss and growth dilution. The model considers a tree canopy in three distinct zones for which initial deposition of pesticides may differ. In addition to predicting the average residue within each zone, it also estimates the 95 and 99% confidence intervals of residues on individual fruit within each zone. For the purpose of evaluation, this model was parameterized specifically for captan, one of the most important non-systemic fungicides used to control disease in horticultural crops. The observed average initial deposit for each zone was used in the evaluation. The overall correlation between predicted average residues and those observed on apple fruit in two applications was 0.93. Confidence intervals were also predicted accurately.
Drug-Induced Pseudotumor Cerebri

Pseudotumor Cerebri (PTC) is an uncommon disorder whose etiology is largely unknown, although its association with steroid withdrawal, hypervitaminosis A, and the use of the tetracycline group of drugs has been well documented. We report here a case in which a patient on chronic divalproex therapy for a seizure disorder developed PTC. Changing his antiepileptic medication from divalproate to topiramate effected a remission of PTC symptoms while maintaining his seizure-free status. It is recommended that physicians treating epilepsy, vascular headaches, or mood disorders with divalproate consider the diagnosis of PTC when their patients complain of new onset of headaches or an increase in frequency or severity of existing headaches—especially those associated with a visual disturbance—to prevent permanent visual loss.
Chronic lymphocytic leukaemia (CLL) in Central Africans.

OBJECTIVE To document the clinical and haematological features of chronic lymphocytic leukaemia (CLL) in Central Africans. DESIGN Prospective descriptive analysis. SETTING Tertiary referral teaching hospitals. SUBJECTS 48 Zimbabweans and 27 Malawians formed the basis of this analysis. RESULTS There were 75 patients (40 males and 35 females) studied and their ages ranged from 32 to 78 years with a mean +/- s.d. of 56.8 +/- 10.1 years. The peak age incidence of 26.7% occurred between 60 to 64 year old and 21.3% were below 50 years. The major clinical findings included: splenomegaly (68%); hepatomegaly (37.3%); anaemia (34.7%); lymphadenopathy (33.3%) and nine (12%) patients were diagnosed incidentally. The majority of patients (78.7%) had Rai stage III and IV and only seven (9.3%) patients were in stage 0. Of the 32 patients treated with chemotherapy, 25.9% and 59.3% achieved complete or partial remissions respectively. Six patients were still alive after a follow up period of a mean +/- s.d of 39.3 +/- 24.4 months; five were lost to follow up after a mean +/- s.d period of 28.6 +/- 18.8 months and 16 were dead after a mean +/- s.d. period of 25.7 +/- 19.1 months. The main causes of death in the treated group were septicaemia in six, pneumonia in four and tuberculosis in three. In the untreated group of 43 patients, two refused therapy, four died shortly after diagnosis and 37 were lost to follow up. CONCLUSIONS AND RECOMMENDATIONS Although the study has disclosed that CLL is not rare in central Africans and its presentations are similar to cases reported in the literature, the majority of patients seek medical treatment late. Optimal therapy is impossible due to lack of chemotherapy and supportive services.Therefore, it is recommended that tertiary referral centers in African health systems should be equipped for better management of CLL patients.
A CMOS Backgate-Coupled QVCO Based on Back-to-Back Series Varactor Configuration for Minimal AM-to-PM Noise Conversion

A 3.3 GHz CMOS quadrature voltage-controlled oscillator (QVCO) with very low phase noise is presented. The back-to-back series varactor configuration is employed in the LC tank for minimizing the AM-to-PM noise conversion. The backgate coupling for quadrature phase inter-locking further eliminates the noise contribution from coupling transistors and also reduces power consumption. The implemented QVCO in 0.18 mum CMOS technology achieved very low phase noise of -133 dBc/Hz at 1 MHz offset, where the total power consumption is 4.4 mW from a 1.0 V supply. The chip has a very high FOM of -196.6 dBc/Hz.
The Role of Microorganisms and Productions in Biodesulfurization ofFossiel Fuels

Desulfurization of fossil fuels demands a high technology as the crude oil is very complex mixture of molecules with a largest hydrocarbons form in composition. Hydrogenic method for desulfurization, takes higher cost and energy and also sulfur is not separated completely from heterocyclic poly-aromatic compounds. Researchers are focused on the use of microbial desulfurization [Biodesulfurization] where sulfur removal reaction acts under mild condition and with lower costs by bacteria. The method could be fixed the flaws in the hydrogenic method through microorganisms and their production role in desulfurization and in fact replace, full or partial of the hydrogenic method as a supplement.
Prolactin effects on the dietary regulation of mouse mammary tumor virus proviral DNA expression.

Chronic energy-intake restriction inhibits mouse mammary tumor virus (MMTV)-induced mammary tumors in C3H/Ou mice by greater than 90%. We have shown that associated with suppression of mammary tumorigenesis there is a reduction or inhibition of circulating prolactin, MMTV particles expressed, and MMTV mRNA transcription in mammary glands (and in most organs tested). To understand the concerted action of prolactin, energy-consumption level, and MMTV on inducing mammary tumors, experiments were designed to control prolactin and energy levels in order to evaluate their effects on MMTV mRNA expression. Mice on restricted diets were grafted with adenohypophyses, and mice fed ad libitum were treated with the dopaminomimetic agent octahydrobenzo [g]quinoline. Adenohypophyseal grafting significantly increased prolactin in dietary (energy)-restricted mice, and this effect was associated with an increase in MMTV mRNA expression within the mammary gland; a linear correlation between prolactin levels and MMTV mRNA expression in the mammary gland was found. Conversely, elimination of the nocturnal peak of circulating prolactin by i.p. injection of dopaminomimetic octahydrobenzo [g]quinoline to mice fed ad libitum delayed (by 8 weeks) and reduced (even as long as 25 weeks) mammary gland MMTV mRNA expression. These findings associate prolactin influences with MMTV mRNA production in mice and help explain the link between chronic energy-intake restriction and reduced MMTV gene expression.
High performance controller for voltage-controlled current source inverter with nonlinear loads

This paper presents an advanced voltage control method for voltage-controlled current source inverter(VC-CSI) with nonlinear loads. The proposed technique adopts the proportional sinusoidal signal integrator (PSSI) controller to achieve a rather good voltage tracking ability under nonlinear load conditions, which can be implemented directly in the stationary αϟ frame with no need of the rotating coordinate transformation. This method can selectively eliminate the harmonics of the output voltage and does not require accurate circuit parameters, which can improve the control system's flexibility and robustness. Both the control principle of the proposed technique for the whole system and the influence of the PSSI controller's parameters on the control performance have been discussed in detail. Finally, all these analyses and the effectiveness of the proposed control method are firstly validated by simulation, and then are verified further in a 5kVA laboratory prototype.
WIDE COOL AND ULTRACOOL COMPANIONS TO NEARBY STARS FROM Pan-STARRS 1

We present the discovery of 57 wide (>5″) separation, low-mass (stellar and substellar) companions to stars in the solar neighborhood identified from Pan-STARRS 1 (PS1) data and the spectral classification of 31 previously known companions. Our companions represent a selective subsample of promising candidates and span a range in spectral type of K7–L9 with the addition of one DA white dwarf. These were identified primarily from a dedicated common proper motion search around nearby stars, along with a few as serendipitous discoveries from our Pan-STARRS 1 brown dwarf search. Our discoveries include 23 new L dwarf companions and one known L dwarf not previously identified as a companion. The primary stars around which we searched for companions come from a list of bright stars with well-measured parallaxes and large proper motions from the Hipparcos catalog (8583 stars, mostly A–K dwarfs) and fainter stars from other proper motion catalogs (79170 stars, mostly M dwarfs). We examine the likelihood that our companions are chance alignments between unrelated stars and conclude that this is unlikely for the majority of the objects that we have followed-up spectroscopically. We also examine the entire population of ultracool (>M7) dwarf companions and conclude that while some are loosely bound, most are unlikely to be disrupted over the course of ∼10 Gyr. Our search increases the number of ultracool M dwarf companions wider than 300 AU by 88% and increases the number of L dwarf companions in the same separation range by 82%. Finally, we resolve our new L dwarf companion to HIP 6407 into a tight (0.″13, 7.4 AU) L1+T3 binary, making the system a hierarchical triple. Our search for these key benchmarks against which brown dwarf and exoplanet atmosphere models are tested has yielded the largest number of discoveries to date.
Mechanisms of Performance Degradation of (La,Sr)(Co,Fe)O3-δ Solid Oxide Fuel Cell Cathodes

Symmetric cells with porous La 0.6 Sr 0.4 Co 0.2 Fe 0.8 O 3- δ (LSCF) electrodes on Gd 0.1 Ce 0.9 O 1.95 (GDC) electrolytes were aged at 800 ◦ C for 800 hours in ambient air. Electrochemical impedance spectroscopy (EIS) measurements performed periodically at 700 ◦ C showed a continuous increase of the polarization resistance from 0.15 to 0.34 (cid:2) · cm 2 . Three-dimensional (3D) tomographic analysis using focused ion beam–scanning electron microscopy (FIB-SEM) showed negligible changes due to the ageing, suggesting that the observed resistance increase was not caused by electrode morphological evolution. However, an increased amount, by a factor of 3, of a water-soluble Sr rich surface phase on the aged LSCF electrode was detected by an etching procedure coupled with inductively coupled plasma-optical emission spectrometry (ICP-OES). The electrochemical analysis in combination with the microstructural parameters determined by FIB-SEM was used to examine the effect of Sr segregation on the rate of oxygen surface exchange, based on the Adler-Lane-Steele (ALS) model. © and three-dimensional The chemical LSCF LSCF microstructure, out an could be affecting use effect of surface
Primary nerve grafting: A study of revascularization

It was the purpose of this study to evaluate the revascularization of primary nerve repair and grafts using orthogonal polarization spectral (OPS) (Cytometrix, Inc.) imaging, a novel method for real‐time evaluation of microcirculatory blood flow. Twenty male Sprague Dawley rats (250 g) were anesthetized with vaporized halothane and surgically prepared for common peroneal nerve resection. Group I animals (n = 10) underwent primary neurorraphy following transection, utilizing a microsurgical technique with 10‐0 nylon suture. Group II (n = 10) animals had a 7‐mm segment of nerve excised, reversed, and subsequently replaced as a nerve graft under similar techniques. All animals were evaluated using the OPS imaging system on three portions (proximal, transection site/graft, and distal) of the nerve following repair or grafting. Reevaluation of 5 animals randomly selected from each group using the OPS imaging system was again performed on days 14 and 28 following microsurgical repair/grafting. Values were determined by percent change in vascularity of the common peroneal nerve at 0 hr following surgery. Real‐time evaluation of blood flow was utilized as an additional objective criterion. Percent vascularity in group I and II animals increased from baseline in all segments at day 14. By day 28, vascularity in nerves of group I rats decreased in all segments to values below baseline, with the exception of the transection site, which remained at a higher value than obtained directly after surgical repair. In group II animals, vascularity remained above baseline in all segments except the distal segment, which returned to vascularity levels similar to those at 0 hr. Further, occlusion of the vessels demonstrated in the graft and distal segments following initial transection appeared to be corrected. This study suggests that revascularization may occur via bidirectional inosculation with favored proximal vascular growth advancement. The use of real‐time imaging offers a unique evaluation of tissues through emerging technologies. © 2003 Wiley‐Liss, Inc. MICROSURGERY 23:60–65 2003
Temperature-and pH-Induced Structural Changes in the Membrane of the Hyperthermophilic Archaeon Aeropyrum pernix K 1

The influence of pH and temperature on the structural organization, fluidity and permeability of the hyperthermophilic archaeon membrane was investigated in situ by a combination of electron paramagnetic resonance (EPR) and fluorescence emission spectroscopy. For EPR measurements, Aeropyrum pernix cells, after growing at different pHs, were spin-labeled with the doxyl derivative of palmitic acid methylester (MeFASL[10,3]). From the EPR spectra maximal hyperfine splitting (2Amax) and empirical correlation time (semp), which are related to mean membrane fluidity, were determined. The mean membrane fluidity increases with temperature and depends on the pH of the growth medium. Computer simulation of the EPR spectra shows that membrane of A. pernix is heterogeneous and consists of the regions characterized with three different types of motional characteristics, which define three types of membrane domains. Order parameter and proportion of the spin probes in the three types of domains define mean membrane fluidity. The fluidity changes of the membrane with pH and temperature correlate well with the ratio between the fluorescence emission intensity of the first and third bands in the vibronic spectra of pyrene, I1/I3. At pH 7.0 a decrease of I1/I3 from 2.0 to 1.2, due to the penetration of pyrene into the nonpolar membrane region, is achieved at temperatures above 65 C, the lower temperature limit of A. pernix growth.
Phase II Metabolism of Hesperetin by Individual UDP-Glucuronosyltransferases and Sulfotransferases and Rat and Human Tissue Samples

Phase II metabolism by UDP-glucuronosyltransferases (UGTs) and sulfotransferases (SULTs) is the predominant metabolic pathway during the first-pass metabolism of hesperetin (4′-methoxy-3′,5,7-trihydroxyflavanone). In the present study, we have determined the kinetics for glucuronidation and sulfonation of hesperetin by 12 individual UGT and 12 individual SULT enzymes as well as by human or rat small intestinal, colonic, and hepatic microsomal and cytosolic fractions. Results demonstrate that hesperetin is conjugated at positions 7 and 3′ and that major enzyme-specific differences in kinetics and regioselectivity for the UGT and SULT catalyzed conjugations exist. UGT1A9, UGT1A1, UGT1A7, UGT1A8, and UGT1A3 are the major enzymes catalyzing hesperetin glucuronidation, the latter only producing 7-O-glucuronide, whereas UGT1A7 produced mainly 3′-O-glucuronide. Furthermore, UGT1A6 and UGT2B4 only produce hesperetin 7-O-glucuronide, whereas UGT1A1, UGT1A8, UGT1A9, UGT1A10, UGT2B7, and UGT2B15 conjugate both positions. SULT1A2 and SULT1A1 catalyze preferably and most efficiently the formation of hesperetin 3′-O-sulfate, and SULT1C4 catalyzes preferably and most efficiently the formation of hesperetin 7-O-sulfate. Based on expression levels SULT1A3 and SULT1B1 also will probably play a role in the sulfo-conjugation of hesperetin in vivo. The results help to explain discrepancies in metabolite patterns determined in tissues or systems with different expression of UGTs and SULTs, e.g., hepatic and intestinal fractions or Caco-2 cells. The incubations with rat and human tissue samples support an important role for intestinal cells during first-pass metabolism in the formation of hesperetin 3′-O-glucuronide and 7-O-glucuronide, which appear to be the major hesperetin metabolites found in vivo.
Isomaltulose Synthase (PalI) of Klebsiella sp. LX3

Isomaltulose synthase from Klebsiella sp. LX3 (PalI, EC 5.4.99.11) catalyzes the isomerization of sucrose to produce isomaltulose (α-d-glucosylpyranosyl-1,6-d-fructofuranose) and trehalulose (α-d-glucosylpyranosyl-1,1-d-fructofuranose). The PalI structure, solved at 2.2-Å resolution with an R-factor of 19.4% and Rfree of 24.2%, consists of three domains: an N-terminal catalytic (β/α)8 domain, a subdomain between Nβ3 and Nα3, and a C-terminal domain having seven β-strands. The active site architecture of PalI is identical to that of other glycoside hydrolase family 13 members, suggesting a similar mechanism in substrate binding and hydrolysis. However, a unique RLDRD motif in the proximity of the active site has been identified and shown biochemically to be responsible for sucrose isomerization. A two-step reaction mechanism for hydrolysis and isomerization, which occurs in the same pocket is proposed based on both the structural and biochemical data. Selected C-terminal truncations have been shown to reduce and even abolish the enzyme activity, consistent with the predicted role of the C-terminal residues in the maintenance of enzyme conformation and active site topology.
Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy.

BACKGROUND Polyarteritis nodosa is a systemic necrotizing vasculitis with a pathogenesis that is poorly understood. We identified six families with multiple cases of systemic and cutaneous polyarteritis nodosa, consistent with autosomal recessive inheritance. In most cases, onset of the disease occurred during childhood. METHODS We carried out exome sequencing in persons from multiply affected families of Georgian Jewish or German ancestry. We performed targeted sequencing in additional family members and in unrelated affected persons, 3 of Georgian Jewish ancestry and 14 of Turkish ancestry. Mutations were assessed by testing their effect on enzymatic activity in serum specimens from patients, analysis of protein structure, expression in mammalian cells, and biophysical analysis of purified protein. RESULTS In all the families, vasculitis was caused by recessive mutations in CECR1, the gene encoding adenosine deaminase 2 (ADA2). All the Georgian Jewish patients were homozygous for a mutation encoding a Gly47Arg substitution, the German patients were compound heterozygous for Arg169Gln and Pro251Leu mutations, and one Turkish patient was compound heterozygous for Gly47Val and Trp264Ser mutations. In the endogamous Georgian Jewish population, the Gly47Arg carrier frequency was 0.102, which is consistent with the high prevalence of disease. The other mutations either were found in only one family member or patient or were extremely rare. ADA2 activity was significantly reduced in serum specimens from patients. Expression in human embryonic kidney 293T cells revealed low amounts of mutant secreted protein. CONCLUSIONS Recessive loss-of-function mutations of ADA2, a growth factor that is the major extracellular adenosine deaminase, can cause polyarteritis nodosa vasculopathy with highly varied clinical expression. (Funded by the Shaare Zedek Medical Center and others.).