Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder. Here, we report a rare case of multi-system LCH in a 20-month-old children presenting nasal congestion, fever, abnormal liver function, anemia, and skin damage. The radiograph computed tomography showed an osteolytic lesion in the lateral skull base with tumor extension. Pathological biopsy was performed, and the histopathologic diagnosis was LCH. A general review of LCH, including clinical manifestations, diagnosis, treatment, and prgognosis, is presented.
Histiocytosis, Langerhans-Cell ; diagnosis ; Humans ; Infant ; Rare Diseases

BACKGROUND: Electrochemically deposited coating, as an alternative method of plasma spraying coating, has arose widely attention, however, few reports concerning its in vivo biological features, especially the effect of coating on early osseointegration and duration of bone healing is poorly understood. OBJECTIVE: To investigate the early osseointegration of electrodepositied calcium phosphate and calcium phosphate/chitosan coatings on rabbits' femurs. DESIGN, TIME AND SETTING: An open experiment. The experiment was performed at the Key Laboratory for Oral Biomedical Engineering of Ministry of Education, Stomatology School of Wuhan University from April 2008 to May 2009. MATERIALS: Eighteen male Japanese rabbits were supplied by the animals' center of Wuhan University. Ti_6Al_4V alloy was purchased from Baoji Titanium Nickel Co., Ltd. Chitosan with over 75% degree of deacetylation was produced by American Sigma Company.METHODS: ① Cylindrical titanium alloy implants (3.3×8.0 mm) were designed with a gap (0.3×4.0 mm) in the middle part by using precision machine tool. The implants were prepared electrodeposited calcium phosphate coating surface (ELD group) and electrodeposited calcium phosphate/chitosan coating surface (ELDC group) with 2.5 mA/cm~2 electric current and 52 V temperature. Meantime, the implants with sandblasted surface were prepared as the control group. Totally 36 implants were randomly inserted into the distal femur condyles of 18 rabbits, and the new bone formation was labeled by fluorescence staining. MAIN OUTCOME MEASURES: At weeks 2 and 4 after operation, the implants were subjected to histological and histomorphometric analysis. RESULTS: All groups were observed different-quantity new bone formation. However, only ELDC group was seen fibrous tissues intervened at bone-to-implant interface. The ELD coatings were heterogeneously degraded, and the majority of the ELDC coatings were degraded. The new bone within gaps of ELD and control groups could be seen under the confocal laser scanning microscope with a continuous process of bone apposition. Percentages of bone-to-implant contact within and outside of the gaps of ELD group at weeks 2 and 4 were significantly higher than those of ELDC and control groups (P < 0.05). Bone formation rates of ELD group were significantly higher than those of ELDC group at weeks 2 and 4 after operation (P < 0.05). CONCLUSION: ELD implants can promote early osseointegration and induce bone tissue growth into the gaps; however, ELDC implants go against osseointegration of rabbit femurs.

Objective To investigate the relation between homeostasis model assessment of insulin resistance(HOMA-IR) and the results of coronary angiography in coronary artery disease(CAD) patients complicated with hypertension.Methods One hundred and two CAD patients complicated with hypertension were enrolled into the investigation group and another 80 CAD patients without hypertension were considered as the control group.The HOMA-IR and the results of coronary angiography were compared between the 2 groups and their correlation was further analyzed.Results The HOMA-IR of the investigation group was higher than that of the control group(8.10?1.25 vs 4.70?2.13,P

BACKGROUND:Platelet-derived growth factor-B (PDGF-B) is an effective pro-angiogenic growth factor, and adeno-associated virus type 9 (rAAV9) has a strong cardiomyocyte targeting affinity, which is an ideal vehicle for ischemic heart disease gene therapy.
OBJECTIVE:To explore the PDGF-B gene transfection of in vitro neonatal rat myocardial cells mediated by rAAV9 against ischemia and hypoxia-induced cardiomyocytes apoptosis.
METHODRat neonatal myocardial cells were isolated and cultured, and then transfected by rAAV9-PDGF-B and empty virus, rAAV9 with enhance green fluorescent protein (eGFP), under multiplicity of infection (MOI) of 105, 106 and 107, respectively. We observed the expression of eGFP under fluorescence microscopy every day, and used flow cytometry to measure transfection efficiency of vector rAAV9. Western blot and immunofluorescence were used to evaluate protein expression of PDGF-B. Myocardial ischemia and hypoxia injury model was established in vitro on the 5th day of transfection of rAAV9-eGFP and rAAV9-PDGF-B with 107 MOI. The number of myocardial apoptosis was measured by TUNEL assay. Western blot was employed to detect the protein expression of Bax and Caspase-3 which were related apoptosis, and the effect and its possible mechanism of PDGF-B gene overexpression against myocardial apoptosis were explored.
RESULTS AND CONCLUSION:rAAV9 vector can efficiently transfect neonatal rat myocardial cells. eGFP and PDGF-B protein expressed in myocardial cells correctly and efficiently, and the expression intensity increased gradual y with the increasing of time course and MOI. The expression became stable on the 5th day, and the transfection efficiency showed significant difference among these groups (P<0.01). Myocardial apoptosis rate was significantly reduced in the rAAV9-PDGF-B group than the rAAV9-eGFP group (P<0.05), and protein levels of Bax and Caspase-3 in the rAAV9-PDGF-B group were significantly lower than those of the rAAV9-eGFP group (P<0.05). These data indicate that overexpression of PDGF-B gene can effectively reduce ischemia and hypoxia-induced myocardial apoptosis, and the possible mechanism might be by inhibiting Bax and Caspase-3 protein expression, which can provide evidence of rAAV9-PDGF-B vector in the gene therapy of ischemic heart diseases.

BACKGROUND:Recombinant adeno-associated virus serotype 9 has a high affinity in myocardial tissue, and the expression of recombinant adeno-associated virus serotype 9-enhanced green fluorescent protein (rAAV9-eGFP) in the aorta of atherosclerosis mice is not clear. OBJECTIVE:To explore the optimal time point of rAAV9-eGFP expression in the aorta of atherosclerosis mice. METHODS:Atherosclerosis model was established with high-fat diet in 30 ApoE-/-mice for 16 weeks. Among them, 25 mice were injected with 5.0×1011 vg (virus genomes) rAAV9-eGFP through the tail vein, while the remaining 5 mice were injected with saline, serving as the control group. The virus-transfected mice were kil ed at 14, 21, 28, 35 and 60 days after transfection, and aortic tissue was harvested. The expression of enhanced green fluorescent protein was detected with laser scanning confocal microscope. Western blot assays were used to detect the expression of enhanced green fluorescent protein in aorta. The expression of enhanced green fluorescent protein in vivo was observed and the optimal expression time point was determined. RESULTS AND CONCLUSION:rAAV9-eGFP effectively transfected the aorta of atherosclerosis mice, enhanced green fluorescent protein was expressed in aortic tissue, and the expression intensity increased gradual y with the increasing transfection time. The highest expression level was found at 35 days after transfection and then maintained stable at 60 days. There were significant differences at different time points after transfection (P<0.001). These data indicate that rAAV9-eGFP can be effectively expressed in the aorta of atherosclerosis ApoE-/-mice and rAAV9-eGFP can be regarded as the optimal vector in the treatment of atherosclerosis.

Objective: To develop the Risk Assessment Scale of Severe Psychiatric Patients in Community and test its reliability and validity. Methods: A random sample of 860 severe psychiatric patients, which was selected from 8 communities in Chaoyang District of Beijing, completed the Risk Assessment Scale of Severe Psychiatric Patients in Community. The internal consistency reliability, the observer reliability, and the correlative coefficients between the total and items of the scale were analyzed, and the exploratory factor analysis was conducted. Results: (1) The Cronbach's coefficient of the scale was 0. 86, and the observer coefficient was 0.92. (2) The Spearman correlative coefficients between the total and items ranged from 0.40 ～ 0.56. (3) Exploratory factor analysis showed that the scale had 4 main factors, all of which could account for 68.14 percent of the whole variance, and the ten item loadings ranged from 0. 60 ～0.91. (4) The patient who scored higher than 35 was called high risk patient Causing trouble behavior of high risk patient was obviously higher than others. Conclusion: The results indicate that the Risk Assessment Scale of Severe Psychiatric Patients in Community has good reliability and validity. It can be used to assess the risk of severe psychiatric patients in community.

AIM: To explore the changes in extracellular regulated protein kinase (ERK1/2) in the hypertrophic myocardium induced by pressure overload at the different time courses and to determine the molecular mechanism in the myocardium from hypertrophy to heart failure. METHODS: C57/BL mice, aged 12 week old, were subjected to sham-operation (SH) or transversing aortic constriction (TAC) to establish left ventricular hypertrophy. Echocardiographic assessments, hemodynamic determination, organ weight measurement, morphological and histological examination were performed at 1, 4, 8, 12 and 16 weeks after surgery. Meanwhile mRNA levels of atrial natriuretic peptide (ANP), α-myosin heavy chain (α-MHC), bcl-2 and bax were measured by RT-PCR, and ERK1/2 levels were detected by Western blotting. The animals in SH group were performed the same tests then sacrificed at 16 weeks. RESULTS: (1) Compared to SH group, LVESd, LVEDd, Awsth, Awdth, Pwsth and Pwdth progressively increased after TAC. Meanwhile, ejection fraction (EF%) significantly decreased at 16th week (P<0.05). LVSP, dp/dt_(max) and dp/dt_(min) in TAC group were progressively increased after 4 weeks. From 8-12 weeks these parameters maintained stable and then sharply decreased at 16th week (all P<0.05). However, LVEDP was statistically increased at 8th week. These echocardiographic and hemodynamic changes indicated a development of LVH and eventually progressing towards to heart failure. (2) Histologically, cardiac collagen measured by percentage of Sirius red positive stained area and apoptosis index showed progressive increases from 4 to 16 weeks. (3) Compared to SH group, mRNA levels of ANP was time-dependently increased while α-MHC and Bcl-2 were time-dependently decreased. The ratio of Bcl-2 /Bax was decreased. Phosphorylation of ERK1/2 was increased at 4th week, then decreased with age of TAC (all P<0.05). CONCLUSION: Pressure-overload induced by TAC results in a development of LVH from early concentric hypertrophy to late eccentric hypertrophy, and eventually toward cardiac dysfunction or heart failure. Those changes are associated with increase in cell size and cardiac fibrosis. ERK1/2 signaling pathway may involve in the regulation of myocardial cell apoptosis in hypertrophic and failure heart.

Objective To investigate the association between acute myocardial infarction (AMI) and the (GT)n repeat sequence polymorphism in promoter region of heme oxygenase-1 (HO-1) , and to study the influence of serum bilirubin on AMI as well for HO-1 as a rate-limiting enzyme of bilirubin production in patients from Uighur national minority. Method Totally 287 patients with AMI evidenced by coronary arteriography admitted from January 2006 to June 2008 were eligible for being studied, and another 190 healthy subjects without anomaly in coronary arteriography, and with normal findings in physical examination and in variety of biochemical assays were enrolled as controls. Serum total cholesterol (TC), triglyceride (TG), high density lipoprotein cholesterol (HDLC), low-density lipoprotein cholesterol (LDL-C), serum bilirubin were detected. Polymerase chain reaction-nondenaturing polyacrylamide gel electrophoresis was used to detect HO-1 promoter (GT)n repeat polymorphism, and at the same time the serum bilirubin was determined. The group representation of samples was tested with HardyWeinberg balance test. Differences in distributions of genotypes and alleles between AMI patients and control subjects were analyzed using Chi-square test. Comprehensive evaluation of the factors associated with myocardial infarction using multi-factor Logistic regression analysis. P < 0.05 was considered as significantly different. Results Body mass index, triglyceride, high density lipoprotein cholesterol and the proportion with hypertension in myocardial infarction group was significantly higher than those in control group ( P < 0.01) . The X~2 values of HO-1genotype distribution in the myocardial infarction group and the control group were 2.09 and 0.05, respectively (P > 0.05), consist with the results of Hardy-Weinberg balance test. The HO-1 genotype was classified into three groups, L/L, L/S and S/S. The L/L genotype frequency (35.5%) and L-allele frequency (57.8%) in AMI group and in control group showed statistically significant differences, respectively (X~2 = 11.65, P = 0.001; X~2= 11.32, P = 0.003). The bilirubin level of L/L genotype significantly decreased compared with that of S/S, L/S genotype ( P all < 0. 001) . Logistic regression analysis showed that body mass index, high blood pressure,triglycerides, blood bilirubin and HO-1 gene polymorphism are risk factors of myocardial infarction. Conclusions To the Xinjiang Uighur ethics, HO-1 promoter ( GT) n repeat polymorphism and the occurrence of myocardial infarction are relevant. People with L allele genotype have lower serum bilirubin and higher risk of myocardial infarction.

Objective To investigate the cerebral vascular autoregulation in patients with mitochondrial encephalomyopathy with lactic acidosis and strokc-like episodes (MELAS) during the remission of stroke-like episodes,including cerebrovascular CO2 reactivity and vascular endothelial function.Methods Twenty-nine MELAS patients confirmed by genetic analysis were recruited in this study. They underwent the examination at least 2 weeks after the onset of last stroke-like episode.Twenty-eight healthy people were collcctcd as healthy controls. Carotid ultrasound and brain magnetic resonance angiogram (MRA) were done to evaluate the cervical and intracranial appearance of large arteries. Evaluation of vascular autoregulation included: (1) the cerebrovascular CO2 reactivity with breath-holding test by transcranial Doppler and calculating breath holding index (BHI),and ( 2 ) flow-mediatcd dilation ( FMD )and nitroglycerin-mediated dilation with ultrasound assessment of humeral artery.Independent-samples t test was done between the results of two groups.Results Carotid ultrasound and cranial MRA revealed no abnormalities in both MELAS patients and healthy controls.The BHI of MELAS patients was significantly decreased than that of normal controls ( 1.36 ± 0.52 vs 1.81 ±0.26,t =- 3.693,P ＜ 0.01 ),and the FMD of MELAS patients was also significantly lower than that of normal controls (11.0％ ±4.8％ vs 15.8％ ±5.8％,t =-3.390,P ＜0.01).Conclusion The function of vascular autoregulation,including cerebrovascular CO2 reactivity and FMD,is impaired in MELAS patients.

Here we report the clinical,radiological and neuropathological findings of a patient with tumor-like inflammatory demyelinating diseases of the central nervous system.The patient was a 51-year-old man with a four-month history of inflammatory pseudotumor and no other significant medical history,who presented to our hospital recurrent relapse numbness and weakness of his right extremities,dysarthria and memory deterioration.Brain magnetic resonance imaging(MRI) showed mass focal lesion in white matter of left parietal lobes.The biopsy showed numerous infiltrating macrophages and lymphocytes within the perivascular.The patient responded clinically to corticosteroid and intravenous immunoglobulin(IVIG) therapy.According to the results of the biopsy and the MRI,a diagnosis of inflammatory pseu-dotumor of the central nervous system was made.The vascular dysfunction may act in the pathogenesis of inflammatory pseudotumor of the central nervous system.