Inherited bone marrow failure syndromes are a group of congenital diseases.Their pathogenesis are not entirely clear.The causative genes have been found in part of the diseases, and we have preliminarily mastered their diagnosis and treatment.The diagnosis is mostly based on typical clinical manifestations and related laboratory tests.Along with the genetic testing developmenff,genetic testing has become the main method to diagnose IBMFS.Hematopoietic stem cell transplantation is currently the only way to cure these diseases.Support and symptomatic treatment also play an important role in terms of delaying and improving the condition.Gene therapy is still not mature, and other treatments are constantly being explored.

Objective To investigate the possible role of orexin in reproduction and examine the changes in the expression of orexin and its receptors(OX1R,OX2R) in the rat hypothalamus during pregnancy,parturition and lactation. Methods The expressions of prepro-orexin(prepro-OX),orexin-A,OX1R,and OX2R in the rat hypothalamus during pregnancy,parturition,and lactation were evaluated by competitive reverse transcription-polymerase chain reaction(RT-PCR) and immunohistochemistry assay. Results Orexin-A immunoreactive(ir) neurons and the OX1R subtype in neuronal cell bodies were mainly located in the lateral hypothalamic area(LHA) as well as in the magnocellular neurons of the paraventricular and supraoptic nuclei respectively in pregnant and lactating rats.The prepro-OX and OX1R mRNA levels on the 1st day of lactation were significantly higher than that during late pregnancy and lactation.No significant changes of OX2R expression were observed during the various reproductive phases.Conclusion Orexin might be involved in regulating reproductive function in early lactation through their binding sites in hypothalamic PVN and SON.

Objective To investigate a possible role of orexin in the regulation of estrous cycle by examining the expression of orexin and orexin receptors(OX1R,OX2R) in the rat hypothalamus during the estrous cycle. Methods The levels of prepro-orexin(prepro-OX),orexin-A,OX1R,and OX2R in the rat hypothalamus during the estrous cycle were evaluated by competitive reverse transcription-polymerase china reaction(RT-PCR) assay. Results Only expression OX1R mRNA during late proestrus was significantly higher than that at metestrus.No significant changes of prepro-OX and OX2R mRNA expression were observed during the various estrous cycle phases.Conclusion Orexin might regulate the secretion of GnRH and/or LH by binding OX1R,contributing to the occurrence of ovulation.

The impulsive electroencephalograph (EEG) noises in evoked potential (EP) signals is very strong, usually with a heavy tail and infinite variance characteristics like the acceleration noise impact, hypoxia and etc., as shown in other special tests. The noises can be described by a stable distribution model. In this paper, Wigner-Ville distribution (WVD) and pseudo Wigner-Ville distribution (PWVD) time-frequency distribution based on the fractional lower order moment are presented to be improved. We got fractional lower order WVD (FLO-WVD) and fractional lower order PWVD (FLO-PWVD) time-frequency distribution which could be suitable for a stable distribution process. We also proposed the fractional lower order spatial time-frequency distribution matrix (FLO-STFM) concept. Therefore, combining with time-frequency underdetermined blind source separation (TF-UBSS), we proposed a new fractional lower order spatial time-frequency underdetermined blind source separation (FLO-TF-UBSS) which can work in a stable distribution environment. We used the FLO-TF-UBSS algorithm to extract EPs. Simulations showed that the proposed method could effectively extract EPs in EEG noises, and the separated EPs and EEG signals based on FLO-TF-UBSS were almost the same as the original signal, but blind separation based on TF-UBSS had certain deviation. The correlation coefficient of the FLO-TF-UBSS algorithm was higher than the TF-UBSS algorithm when generalized signal-to-noise ratio (GSNR) changed from 10 dB to 30 dB and a varied from 1. 06 to 1. 94, and was approximately e- qual to 1. Hence, the proposed FLO-TF-UBSS method might be better than the TF-UBSS algorithm based on second order for extracting EP signal under an EEG noise environment.
Algorithms ; Electroencephalography ; Evoked Potentials ; Humans ; Models, Theoretical ; Signal Processing, Computer-Assisted

Objective To investigate the effects of emodin on the migration and proliferation of vascular smooth muscle cell (VSMC) and the metabolism of emodin in VSMC. Methods The effects of emodin on the migration or proliferation of vascular smooth muscle cell were measured by “transwell" migration system and MTT assay. Results The migration of VSMC could be significantly inhibited by emodin and the inhibitory ratio was 83.8% in 5 ?g/mL emodin group. The antiproliferative effect of emodin was in a dose- and time- dependent manner. However, the supernatant concentration of emodin insignificantly dereased after culture for 24 h. The cytotoxicity of emodin and cellular ROS was not influenced by CYP inducer or inhibitor. The mRNA expression of emodin's primary metabolic enzyme (cytochrome p450 oxidase, CYP) up-regulated insignificantly after treatment of emodin for 24 h. Conclusion Emodin can inhibit the migration and proliferation of VSMC and can not be metabolized by VSMC. Emodin may be a choice for the medication of drug-eluting stent.

Objective To investigate the effect of insulin resistance(IR) on the persons with abnormal blood glucose who underwent dietary interventions.Methods 194 individuals with abnormal blood glucose were selected from hyperlipidemia population taking the community intervention program in districts of Beijing.The insulin resistance and insulin action index(IAI) were calculated with fasting blood glucose(FBG) and fasting blood insulin(FINS),and analyzed the relationship of variety on their blood glucose,serum lipids and body weight,body mass index(BMI),waist circumference,waist hip ratio(WHR).Results 1) Blood glucose(FBG),triglyceride(TG),waist circumference and WHR had significant positive relationship with IR but negative with IAI;2) In the intervention group,FBG and TG were reduced significantly in the subgroup with highest IAI.Conclusions IR was related with blood glucose,serum lipids,waist circumference and WHR,but no significant relation with body weight and BMI;It was possible that IR had some effect on dietary intervention.

Objective To study ST-segment deviation on predicting culprit artery or culprit lesion in acute myocardial infarction(AM/).Methods The study population included patients with AMI who subsequently underwent coronary angiography during hospitalization.Culprit artery and culprit parts were evaluated,and ST-segment characteristics were analyzed.Results A higher ST-segment elevation in leadⅢthan in leadⅡand ST-segment depression＞1 mm in lead aVL or lead I were highly sensitive(75%)and specific(100%)markers for right coronary artery-related AMI.ST-segment depression＞1 mm in leadⅡ,Ⅲand aVF was lowly sensitive(39%)and highly specific (93%)marker for the left anterior descending artery-related AMI.Conclusion The culprit artery or culprit lesion coutd be predicted by using electrocardiogram at admission.

This study aimed to explore the cognitive dysfunction of and hippocampal neuron damage to Wistar rats with STZ-induced diabetes at different morbidity time. All Wistar rats in the tests received intraperitoneal injections of streptozotocin (STZ; 60 mg/kg) to induce type 1 diabetes. The concentration of blood glucose and the body weight were investigated, the cognitive ability of rats was assessed using a standardized Y-maze, and the apoptotic neurons in the CA1 of the hippocampus were also examined by using the HE staining. While the sickening time was prolonged, the blood glucose concentration of the experimental rats increased continuously and the body weight decreased. On the 70th day after STZ administration, the neuronal loss in the hippocampal CA1 region increased and the working errors increased in rats with the diabetes. The results showed that Wistar rats could complicate with diabetic encephalopathy in 70 days after injection of STZ for inducing the diabetes.
Animals ; Blood Glucose ; Body Weight ; CA1 Region, Hippocampal ; cytology ; pathology ; Cognition ; Cognition Disorders ; physiopathology ; Diabetes Mellitus, Experimental ; physiopathology ; Diabetes Mellitus, Type 1 ; Neurons ; pathology ; Rats ; Rats, Wistar ; Streptozocin

Objective To study clinically the feasibility of early diagnosis of cardiac allograft vascularopathy (CAV) and chronic rejection. Methods A 13 year old female patient with dilated cardiomyopathy received orthotopic heart transplantation for advanced heart failure, and subsequent immunosuppressive therapy including cyclosporine, prednisone and mofetil, and a monthly close follow up. Coronary angiography and left ventricular endomyocardial biopsy (EMB) was performed 9 months after the operation. Results The clinical and follow up data of the case showed that cardiac or systemic nonspecific symptoms such as exertional chest discomfort, palpitation, fatigue or fever of unknown reasons were the first and ignorable clinical symptoms, and found disappeared after dosage addition of cyclinsporine, which indicated a early clinical manifestations of rejection or vasculopathy. While persistent sinus tachycardia on electrocardiogram, decreased left ventricular ejection fraction (from 64?% ～68?% down to 47?%～50?%), enlarged right atrial (from 32～ 41?mm up to 44?mm in diameter), thickened intraventricular septal ( 13?mm ), repetitive tricuspid regurgitation on echocardiography, increased white blood cells without infection were observed respectively. Coronary angiography showed a typical (type B) vasculopathy with diffuse concentric stenosis of mid and distal left anterior descending artery and its small braches, the distal diagonal, circumflex, and right coronary artery were also involved to some extent. Furthermore, early mild chronic rejection of grade I A was proved by pathologic study. Conclusion Close clinical observation and follow up, serial echocardiography and electrocardiogram, reaction to enhanced anti rejection, and other related laboratory methods could be analyzed and colligated for early noninvasive diagnosis of cardiac allograft vasculopathy and chronic rejection, while EMB and coronary angiography are still the precise diagnostic ways.

Objective To evaluate the detection rate of multi-slice spiral CT (MSCT)signs and the clinical value of multi-planar reconstruction (MPR)in T1a and T1b peripheral lung cancer patients.Methods Eighty-seven cases with peripheral lung cancer proved by pathology were collected.The cases were divided into T1a and T1b group based on the TNM classification.The MSCT and MPR images were compared between the two groups.Results (1)Detection rate of the deep sublobe sign,spinous process sign, short spiculated sign,pleural indentation sign,vascular convergence sign,multi-nodule accumulation sign,vacuole sign and air bron-chogram sign,were 1 1.4%,20.0%,31.4%,60.0%,25.7%,45.7%,42.9% in T1a group and 42.3%,36.5%,57.7%, 80.8%,5 1.9%,25.0%,21.2% in T1b group,respectively.The difference were all statistically significant (P < 0.05)between T1a and T1b group except that of the spiculated sign (P = 0.098).(2)The detection rate of the sublobe sign,spinous process sign, spiculated sign,pleural indentation sign and vascular convergence sign were higher on MPR images than on axial thin-slice images in both T1a and T1b group.Conclusion The detection rate of the tumor-lung interface’s signs are lower in T1a than in T1b,the detec-tion rate of internal structure signs of the tumor are higher in T1a than in T1b in peripheral lung cancer patients.MPR has important value in early diagnosis of peripheral lung cancer.