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 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.

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 matrix metalloproteinase-9 (MMP-9) gene polymorphism (-1562C ＞ T/R279Q) and acute coronary syndrome (ACS) in Uygur nationality of Xinjiang Autonomous Region of China. Methods A total of 352 patients with ACS including 213 patients with unstable angina pectoris and 139 patients with acute myocardial infarction evidenced by using coronary arteriography and 421 control subjects were recruited in this study. The MMP-9-1562C ＞ T and R279Q genotypes were detemined by using PCR-RFLP method. The relationship between the polymorphism in the MMP-9 gene and the severity of coronary arterial stenosis was analyzed. All polymorphisms were determined for confimation with Hardy-Weinberg expectations in both groups separately. Differences in distributions of genotypes and alleles between two groups were analyzed with x2 test. The association between the MMP-9 polymorphisms and the risk of ACS was estimated by odds ratio(Ors) and their 95% confidence intervals (CIs), and the comprehensive evaluation of the factors associated with ACS was determined by using multifactor logistic regression. P ＜ 0. 05 was considered to be statistically significant. Results The genotype frequencies for CT + TT genotypes and T allele were 25.9 and14.5 percent in ACS subjects and 15.7 and 8.4 percent in control subjects, respectively. The genotype frequencies were different significantly between the two groups (x2 = 12.26,P ＜ 0.01;x2 = 14.15,P ＜ 0.01, respectively). No relationship between R279Q polymorphism and ACS was found in this study ( P ＞ 0.05). The multifactor logistic regression analysis showed that the T allele carrier (CT + TT) significantly increased the risk of ACS compared with the CC genotype ( OR = 1.791,95 % CI: 1. 088 - 2.951, P = 0.022) after adjustment for tradition risk factors. The frequencies for CT + TT and CC genotypes of the -1562C ＞ T polymorphism were not statistically different among ACS patients with one, two and three or more significantly diseased vessels ( x2 = 1.15, P = 0.56). Conclusions The findings suggest that the polymorphism in MMP-9 gene promoter (-1562C ＞ T) is associated with the susceptibility to the ACS. The T allele might be an independent risk factor for the ACS. But the -1562C ＞ T polymorphism may not be useful as a predictor of the severity of coronary arterial stenosis. The R279Q polymorphism of MMP-9 gene was not significantly associated with ACS in this studied population.

Objective To explore the effects of aging on ventricular remodeling and cardiac rupture after acute myocardial infarction in mice. Methods　Male C57BL/6 mice of 3 months and 12 months old were randomly divided into sham operation group and myocardial infarction(MI)group.Following acute myocardial infarction(AMI)modeling induced by open-chest surgery,the events of cardiac rupture were monitored and the echocardiography and hemodynamics were performed on the 7th day after surgery.Zymography,immunohistochemical method and pathological staining were used to measure the activity of matrix metalloproteinases(MMPs),the content of collagen and the degree of inflammatory cell infiltration on the 3rd and 7th days after surgery,respectively. Results　The incidence of cardiac rupture was higher in elderly group than that in young group(38.0% vs.16.0%,X2=6.139,P＜0.05).Compared with young group,significant infarct expansion,left ventricular (LV)remodeling and hemodynamic deterioration were showed in elderly group on the 7th day after surgery(t=5.754,P＜0.05).The degree of inflammatory cell infiltration and the expression of MMP-9 were significantly increased in elderly group on the 3rd day following AMI modeling(P＜0.05),and the collagen content and the expression of type Ⅲ collagen were significantly increased (P＜0.05)compared with young group. Conclusions　Aging is a risk factor for post-infarct cardiac rupture in the mice model.The mechanisms which are responsible for this age-related difference of cardiac rupture are related to increasing degree of inflammatory cell infiltration, overexpression of MMP-9 and type Ⅲ collagen and aggravated early LV remodeling.