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Neurofibromatosis-Noonan syndrome (NFNS), a rare autosomal-dominant hereditary disease, is characterized by clinical manifestations of both neurofibromatosis type 1 (NF1) and NS. We present a case of NFNS with short stature caused by a heterozygous nonsense variant of the NF1 gene. A 12-year-old boy was admitted because of short stature, numerous café-au-lait spots, low-set and posteriorly rotated ears, sparse eyebrows, broad forehead, and inverted triangular face. Cranial and spinal magnetic resonance imaging showed abnormal nodular lesions. Molecular analysis revealed a novel heterozygous c.6189 C > G (p.(Tyr2063*)) variant in the NF1 gene. The patient was not prescribed recombinant growth hormone (GH) therapy because exogenous GH may have enlarged the abnormal skeletal lesions. During follow-up, Lisch nodules were found in the ophthalmologic examination. NFNS, a variant form of NF1, is caused by heterozygous mutations in the NF1 gene. The mechanism of GH deficiency caused by NF1 is still unclear. Whether NFNS patients should be treated with exogenous GH remains controversial. [ABSTRACT FROM AUTHOR]

Background: Endothelial dysfunction is a major pathophysiology observed in hypertension. Ghrelin, a key regulator of metabolism, has been shown to play protective roles in cardiovascular system. However, whether it has the effect of improving endothelial function and lowering blood pressure in Ang II-induced hypertensive mice remains unclear. Methods: In this study, hypertension was induced by continuous infusion of Ang II with a subcutaneous osmotic pumps and ghrelin (30 µg/kg/day) was intraperitoneal injection for 4 weeks. Acetylcholineinduced endothelium-dependent relaxation in aortae was measured on wire myograph and superoxide production in mouse aortae was assessed by fluorescence imaging. Results: We found that ghrelin had protective effects on Ang II-induced hypertension by inhibiting oxidative stress, increasing NO production, improving endothelial function, and lowering blood pressure. Furthermore, ghrelin activated AMPK signaling in Ang II-induced hypertension, leading to inhibition of oxidative stress. Compound C, a specific inhibitor of AMPK, reversed the protective effects of ghrelin on the reduction of oxidative stress, the improvement of endothelial function and the reduction of blood pressure. Conclusions: our findings indicated that ghrelin protected against Ang II-induced hypertension by improving endothelial function and lowering blood pressure partly through activating AMPK signaling. Thus, ghrelin may be a valuable therapeutic strategy for hypertension. [ABSTRACT FROM AUTHOR]

Oxygen-enriched combustion technology has an important application prospect, in which Oxy-steam combustion is a new generation of potential carbon capture technology. In this paper, the effects of O 2 /CO 2 and O 2 /H 2 O atmosphere on soot formation in laminar diffusion flame were studied. The flame image was taken by a color (CCD) camera in the visible band, the directional emission power in R and G bands was calibrated, and the two-dimensional distribution of temperature and soot volume fraction in the flame was reconstructed. The experimental results are compared with the numerical results, and the numerical results well predict the temperature of oxygen-enriched flame in O 2 /N 2 /CO 2 atmosphere, but overestimate the soot volume fraction. The laminar diffusion flame of ethylene in two kinds of oxygen-rich atmosphere was numerically studied by using a step-by-step decoupling method to separate the density, transport, thermal and chemical effects of the diluent by introducing four virtual substances. The calculation results show that combustion in O 2 /H 2 O and O 2 /CO 2 atmosphere reduces the flame peak temperature, but the effect on the flame centerline temperature distribution is different. The addition of H 2 O enhanced the concentration of OH through the reverse reaction rate of OH + H 2 =H + H 2 O, while the addition of CO 2 decreased the concentration of OH.O 2 /CO 2 combustion leads to the increase of flame height and radius, while in O 2 /H 2 O atmosphere, the flame height is shortened. Both CO 2 and H 2 O have a good inhibitory effect on soot formation. CO 2 in the oxidant affects soot nucleation and surface growth rate mainly by reducing flame temperature and H mole fraction, but not by promoting soot oxidation process. Water vapor also inhibits soot formation by increasing OH concentration through chemical effect. • The laminar diffusion flame of ethylene in oxygen-rich atmosphere was optically detected, and the temperature and soot volume fraction were reconstructed. • Using the ethylene laminar coaxial jet flame modeling method, the model can capture the various effects of CO2 and H2O on soot formation during ethylene combustion. • In the numerical calculation, the method of gradually decoupling the multiple effects of the diluent (CO2, H2O) is adopted. • The effects of CO2 and H2O diluents on soot formation were compared. [ABSTRACT FROM AUTHOR]

Kimura disease (KD) is a rare chronic granulomatous inflammation disease with unknown causes, which iscommonin young Asian males. Local lymph node drainages are characteristic of the disease, the most common of which occur in the salivary glands. Other reported drainages occur in the axilla, popliteal fossa, groin, and upper extremities. Unfortunately, there not many characteristic manifestations that typically present in patients with KD. Sometimes only increased peripheral blood eosinophils and serum IgE concentration are the only positive presentations. Therefore, it is very difficult for physicians to diagnose KD. We report herein on two cases of KD. Both of them presented in the soft tissues of the upper extremities. Special attention was given to the MRI findings of the two patients with a view to disclosing specific characteristics that should help in the realization of clinical diagnosis. [ABSTRACT FROM AUTHOR]

A new series of Ba-Co-O perovskite-type oxygen carriers has been successfully synthesized by the microwave-assisted sol-gel method and further applied for producing an O2/CO2 mixture gas. The oxygen adsorption/desorption performance of synthesized samples was studied in a fixed-bed reactor system. Effects of A/B-site substitution on the oxygen desorption performance of Ba-Co-O–based perovskites are also included. Furthermore, the effects of operating conditions including the adsorption time and temperature as well as the desorption temperature on oxygen production performance were investigated in detail. The results indicated that BaCoO3-δ exhibited an excellent oxygen desorption performance among the synthesized A/B-site–substituted ACoO3-δ and BaBO3-δ samples, and that the optimal adsorption time, adsorption temperature and desorption temperature for BaCoO3-δ were determined to be 20 min, 850 °C and 850 °C, respectively, in this study. [ABSTRACT FROM AUTHOR]

A sensitive high-performance liquid chromatography–electrospray ionization-mass spectrometry (HPLC–ESI-MS) assay is established for the determination of tiotropium in human plasma using benzyltriethylammonium chloride as the internal standard (IS). After being treated with C18 cartridges, plasma samples are separated by HPLC on a reversed-phase C18 column with a mobile phase of 40mM ammonium acetate buffer–methanol (56:44, v/v). Tiotropium is determined in a single-quadrupole MS. HPLC–ESI-MS is performed in the selected ion monitoring mode using target ions at m/z 392.0 for tiotropium and m/z 192.3 for the IS. The calibration curve is linear over the range 1.5–30 pg/mL. The intra- and inter-assay variability values are less than 10.1% and 13.6%, respectively. The mean plasma extraction recovery of tiotropium is 92.3 ± 5.0%. The method has been successfully applied to studying the pharmacokinetics of tiotropium in healthy Chinese volunteers. [ABSTRACT FROM AUTHOR]

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A sensitive liquid chromatography-electrospray ionization-mass spectrometry (LC-ESI-MS) method for determination of eperisone in human plasma using buflomedil as the internal standard (IS) is established. After being made alkaline with saturated sodium bicarbonate solution, plasma samples are extracted with a mixture of diethyl ether-cyclohexane (1:1, v/v) and separated by high-performance liquid chromatography on a reversed-phase C18 column with a mobile phase of 10mM ammonium acetate buffer (adjusted the pH to 3.9 with acetic acid)-methanol (20:80, v/v). Eperisone is determined using ESI in a single-quadrupole MS. LC-ESI-MS is performed in the selected ion monitoring mode using target ions at m/z 260 for eperisone and m/z 308 for the IS. Calibration curves are linear over the ranges 0.02-20 ng/mL for eperisone. The intra- and interassay variability values are less than 9.0% and 11.5%, respectively. The mean plasma extraction recovery of eperisone is 91.7 ± 6.6%. The method has been successfully applied to study the pharmacokinetics of eperisone in healthy, male, Chinese volunteers. Pharmacokinetic parameters of the reference and test tablets have been compared. [ABSTRACT FROM AUTHOR]

A sensitive liquid chromatography-electrospray ionization-mass spectrometry (LC-ESI-MS) method for determination of eperisone in human plasma using buflomedil as the internal standard (IS) is established. After being made alkaline with saturated sodium bicarbonate solution, plasma samples are extracted with a mixture of diethyl ether-cyclohexane (1:1, v/v) and separated by high-performance liquid chromatography on a reversed-phase C18 column with a mobile phase of 10mM ammonium acetate buffer (adjusted the pH to 3.9 with acetic acid)-methanol (20:80, v/v). Eperisone is determined using ESI in a single-quadrupole MS. LC-ESI-MS is performed in the selected ion monitoring mode using target ions at m/z 260 for eperisone and m/z 308 for the IS. Calibration curves are linear over the ranges 0.02-20 ng/mL for eperisone. The intra- and interassay variability values are less than 9.0% and 11.5%, respectively. The mean plasma extraction recovery of eperisone is 91.7 ± 6.6%. The method has been successfully applied to study the pharmacokinetics of eperisone in healthy, male, Chinese volunteers. Pharmacokinetic parameters of the reference and test tablets have been compared. [ABSTRACT FROM AUTHOR]