Cerebral small vessel disease (SVD) is an important subtype of cerebrovascular disease. It is also one of the major reasons for resulting in vascular cognitive impairment or dementia in the elderly. SVD is a small arteriovenous lesion in subcortex caused by a variety of causes, mainly causing subcortical lacunar infarction, white matter damage, microblecds and other pathological changes. There is evidence that vascular endothelial function and blood-brain barrier damage may result in small vessel structures and perivascular changes, which may be the initial factors.of causing SVD. Genetic susceptibility is also one of the risk factors that can not be ignored.

Adult ; Factor XIII ; antagonists & inhibitors ; Factor XIII Deficiency ; complications ; etiology ; Female ; Humans ; Sjogren's Syndrome ; complications

OBJECTIVE To suvey the mastering of knowledge on prevention and treatment of AIDS among medical staff and evaluate the effect of health education on them.METHODS Suvey among medical staff was performed by self-designed questionnaires before and after education,then evaluated the effect of health education.RESULTS The mean score before education was 20.12?2.28;The mean score after education was 20.77?4.04.There were significant differences between them(t=6.417,P

OBJECTIVESince glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary hemolytic erythrocyte enzyme deficiency, most cases have single nucleotide mutations in the coding region, and current test methods for gene mutation have some missed detections, this study aimed to investigate the feasibility of RT-PCR sequencing in the detection of gene mutation in G6PD deficiency.

METHODSAccording to the G6PD/6GPD ratio, 195 children with anemia of unknown cause or who underwent physical examination between August 2013 and July 2014 were classified into G6PD-deficiency group with 130 children (G6PD/6GPD ratio <1.00) and control group with 65 children (G6PD/6GPD ratio≥1.00). The primer design and PCR amplification conditions were optimized, and RT-PCR sequencing was used to analyze the complete coding sequence and verify the genomic DNA sequence in the two groups.

RESULTSIn the G6PD-deficiency group, the detection rate of gene mutation was 100% and 13 missense mutations were detected, including one new mutation. In the control group, no missense mutation was detected in 28 boys; 13 heterozygous missense mutations, 1 homozygous same-sense mutation (C1191T) which had not been reported in China and abroad, and 14 single nucleotide polymorphisms of C1311T were detected in 37 girls. The control group showed a high rate of missed detection of G6PD deficiency (carriers) in the specimens from girls (35%, 13/37).

CONCLUSIONSRT-PCR sequencing has a high detection rate of G6PD gene mutation and a certain value in clinical diagnosis of G6PD deficiency.

Adolescent ; Child ; Child, Preschool ; Female ; Glucosephosphate Dehydrogenase ; genetics ; Glucosephosphate Dehydrogenase Deficiency ; diagnosis ; genetics ; Humans ; Infant ; Male ; Mutation ; Reverse Transcriptase Polymerase Chain Reaction ; methods ; Sequence Analysis, DNA

OBJECTIVETo investigate the effect of Xianxiong decoction on the mice with acute lung injury induced by lipopolysaccharide.

METHODEighty female ICR mice were randomly divided into 8 groups: model group, Xianxiong decoction group, Daxianxiong decoction group, Xianxiong decoction group without Kansui Radix group, Xianxiong decoction group without Glycyrrhizae Radix et Rhizoma group, Glycyrrhizae Radix et Rhizoma and Kansui Radix group, normal group and control group. Animals of each group, except normal group, were undertaken intraperitoneal injection and intranasal inhalation of lipopolysaccharide (LPS) on day 1, 2, 3 to establish acute lung injury (ALI) model. 30 min after modeling, 0.2 mL corresponding drugs were administrated to each mice, dexam ethasone and normal saline were given to the mice of control group and normal group respectively. White blood cell in blood, neutrophil percentage of blood and bronchoalveolar lavage fluid (BALF) supernatant, the ratio of wet and dry lung tissue ( W/D), histopathological changes of lung tissue were estimated. Sixty ICR mice were randomly divided into normal, model, control, high, middle and low dose Xianxiong decoction groups and were modeled in the same way. ELISA was applied to detect the level of NF-kappaB, TNF-alpha and IL-6 in BALF, PCR for NF-kappaB and TNF-alpha mRNA in lung tissue, and Western blot for NF-kappaB and TNF-alpha. Half of 20 ICR mice were administrated with Xianxiong decoction of its maximum tolerant normal saline.

RESULTCompared with model group, the number of WBC in blood of Xianxiong decoction group mice decreased (P < 0.01), percentage of neutrophils in both blood and BALF decreased as well (P < 0.01, P < 0.05); it also significantly reduced the ratio of W/D (P < 0.01); and found the alveolar wall, the number of inflammatory cells infiltrating improved, compared with model group. Xianxiong decoction reduced the level of NF-kappaB, TNF-alpha and IL-6 in BALF (P < 0.01, P < 0.01, P < 0.05); its high and low dose groups only found TNF-alpha level declined. Five mice died 24 h after administration of Xianxiong decoction which indicated its toxicity when other influential factors were considered.

CONCLUSIONXianxiong decoction is effective on the ALI mice induced by LPS, but it is of toxicity at 3 g x mL(-1).

Acute Lung Injury ; drug therapy ; genetics ; metabolism ; pathology ; Animals ; Bronchoalveolar Lavage Fluid ; chemistry ; Drugs, Chinese Herbal ; administration & dosage ; Female ; Humans ; Interleukin-6 ; genetics ; metabolism ; Lipopolysaccharides ; adverse effects ; Lung ; drug effects ; metabolism ; pathology ; Mice ; Mice, Inbred ICR ; NF-kappa B ; genetics ; metabolism ; Tumor Necrosis Factor-alpha ; genetics ; metabolism

OBJECTIVETo observe the curative effect of porous tantalum rod and Gugutou Huaisiyu Capsule (GHC) for steroid-induced osteonecrosis of femoral head (SONFH).

METHODSA total 60 hips of 50 SONFH patients were randomly assigned to the treatment group and the control group according to grouping time, 25 in each group (30 hips). Patients in the control group were implanted with porous tantalum rod, while those in the treatment group additionally took GHC (5 pills each time, three time per day for 2 successive months; and then twice per day for 4 successive months). Then all patients were followed-up to observe Harris hip score. The curative effect and the femoral head survival time were assessed.

RESULTSA total of 49 patients (59 hips) were followed-up. The Harris hip score of the two groups at the final follow-up was significantly improved after treatment, with statistical difference when compared with before treatment (P < 0.01). Besides, it was higher in the treatment group than in the control group. The curative effect and the survival time were superior in the treatment group, with statistical difference when compared with the control group (P < 0.05).

CONCLUSIONSPorous tantalum rod combined GHC got better effect in treating SONFH. It could significantly improve the function of affected hips and prolong the survival time of femoral head.

Capsules ; Drugs, Chinese Herbal ; administration & dosage ; pharmacology ; therapeutic use ; Femur Head Necrosis ; drug therapy ; Humans ; Prostheses and Implants ; Steroids ; adverse effects ; Tantalum

OBJECTIVESevere Epstein-Barr (EB) virus infection is potentially a devastating process that often leads to death encountered in pediatrics recently. Inappropriate control of EB virus replication may cause severe infection resulting in multiple organ dysfunction. However, little information is available on pulmonary complications associated with EB virus infection. The aim of the present study was to investigate severe EB virus (EBV) infection complicated with lung injury in pediatric intensive care unit (PICU), including clinical characteristics, laboratory or imaging feature and outcomes.

METHODA total of 45 children with severe EBV infection seen in PICU of Shanghai Children's Hospital between January 2011 and December 2014 were retrospectively reviewed. According to clinical characteristics and imaging feature, 45 children were divided into non-lung injury group (n =27), lung injury without pulmonary fibrosis group(n = 12) and pulmonary fibrosis group (n = 6).

RESULTIn totally 45 cases of severe EBV infection, 21 (46.7%) were male and 24 (53. 3%) were female, mean age was 2. 4 years; 18 cases were complicated with lung injury, including 8 male and 10 female, median age was 31. 2 months. All of 18 cases presented with fever and cough, 15 of them exhibited dyspnea,12 cases were complicated with gasping, and 6 cases with ARDS. Eight cases accepted mechanical ventilation for acute respiratory distress; 6 cases who developed pulmonary fibrosis had tachypnea, refractory hypoxemia and hypercapnia, severe pulmonary air leak. The average EBV-DNA level in peripheral blood was 4. 42 x 10(6) copies/ml (range: 3. 25 x 10(3) - 6.59 x 10(7) copies/ml). Anti-EBV antibodies were positive in 41 cases, 18 cases were positive (+) for VCA-IgM, 15 cases were VCA-IgG and EA-IgG (+), 8 cases VCA-IgM and VCA-IgG (+). The radiographic findings revealed pulmonary interstitial infiltrates in all 18 cases with lung injury, 4 cases with segmental consolidation and 2 cases showed pleural effusions. HRCT scanning found EBV associated fibrosis including multifocal patches and diffuse ground-glass attenuation in both lungs, reticular opacities and honeycombing changes were observed 4 weeks after illness onset. There were significant differences in respiratory failure, PICU stay (days), Pediatric risk of mortality III (PRISM III) and pediatric clinical illness score(PCIS), serum TNF-α, EBV-DNA levels, percentage of NK cells and CD4+/CD8+ T cell ratio among non-lung injury group, lung injury without pulmonary fibrosis group and pulmonary fibrosis group (X2 =27. 12, F = 85. 23, 78. 23, 88. 68, 323. 80, 7. 35, χ2 = 6. 71, 12. 15; all P < 0. 05). COX regression analysis revealed that EBV-DNA and serum TNF-α levels were correlated with pulmonary fibrosis significantly (OR = 3. 92, P = 0. 04; OR = 5. 95, P = 0. 01). The patients with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) had higher incidence of pulmonary fibrosis compared with non-EB-HLH (70% vs. 13%, χ2 = 4. 82, P = 0. 03). Of 18 cases, 8 cases died, including 3 cases with pulmonary fibrosis. The surviving cases with pulmonary fibrosis needed longer additional oxygen. Chest HRCT imaging of 3 cases with pulmonary fibrosis was improved at 6 months and oxygen therapy was discontinued at 18 months after discharge.

CONCLUSIONEB virus infection complicated with lung injury had higher incidence of respiratory failure, pulmonary fibrosis with a fatal outcome. EBV-DNA and serum TNF-α level were found to be statistically significant indicators of pulmonary fibrosis. Pulmonary fibrosis associated with severe EB virus infection may be reversible.

Antibodies, Viral ; blood ; CD4-CD8 Ratio ; Child, Preschool ; China ; DNA, Viral ; blood ; Epstein-Barr Virus Infections ; pathology ; Female ; Herpesvirus 4, Human ; Humans ; Intensive Care Units, Pediatric ; Killer Cells, Natural ; Lung Injury ; virology ; Lymphohistiocytosis, Hemophagocytic ; pathology ; virology ; Male ; Pulmonary Fibrosis ; pathology ; virology ; Retrospective Studies ; Tumor Necrosis Factor-alpha ; blood

Objective To investigate the impact of compliance and quality of life of psychological intervention for cancer patients and their primary caregivers,as well as the correlation between the psychological issues of patients and their primary caregivers.Methods The enrolled patients were randomly divided into intervention group and control group.The patients in intervention group were given to standardize anti-tumor therapy,while the patients and their primary caregivers were given psychological intervention once a week.The patients in the control group only received standard anti-tumor therapy.By TDL determination of quality of life,anxiety rating scale (SAS) and self-rating depression scale (SDS),in front of the psychological intervention after 8 weeks of intervention,the two groups of patients and their primary caregivers were questionnaired,and recorded the completion of the treatment plan.By SPSS 12.0 software,the statistics were completed.Results 51 cases in intervention group and 38 cases in control group were able to complete the number of people expected to treat there was a statistically significant (P ＜ 0.05).TDL determination and quality of life scores in intervention group patients and their primary caregivers were significantly higher (P ＜ 0.05).SAS and SDS score in intervention group patients and their primary caregivers were significantly lower than control group (P ＜ 0.05).Conclusion The effective psychological intervention to cancer patients and their primary caregivers during the treatment of patients could improve the compliance of cancer patients,the quality of life of cancer patients and their primary caregivers.The psychological problems between the patients and primary caregivers are positive correlation.

Objective To study critical hemophagocytic syndrome (HPS) or macrophage activation syndrome (MAS) presented with multiple organ dysfunction syndrome (MODS) in pediatric intensive care unit (PICU),including clinical features and outcomes In order to explore the effect of bedside continuous hemodialysis/hemofiltration (CBP) as adjuvant treatment for severe HPS/MAS.Methods A total of 19 children with HPS/MAS were hospitalized met the diagnostic criteria for HPS from January,2009 to December,2012.Twelve cases were treated with CBP by continuous venin-venin hemodialysis/hemofiltration (CVVHDF) or high-volume hemofiltration (HVHF) following conventional anti-inflammatory therapy.The replacement liquid dose was 50-75 ml/ (kg · h).The organs function were evaluated and laboratory biomarkers including blood 、electrolytes,ferritin changes were measured before and after CBP treatment.Results Ninteen cases of HPS were acute onset and developed to MODS rapidiy after admission to PICU.The main clinical features were the irregular fever or high fever,hepatosplenomegaly and significant liver damage,nervous system dysfunction and disseminated intravascular coagulation (DIC).Eight cases were death and mortality rate was 42.1％,and all death occurred in those aged less than 3 years old.The mortality rate were 25％ (3/12) and 71.4％ (5/7) in CBP group and non-CBP group respectively.After CBP for 6-24 hours,the fever returned to normal range and blood electrolytes improved.The serum ferritin,serum alanine aminotransferase (ALT) and lactate dehydrogenase (LDH) reduced significantly.Serum creatinine (sCr),blood urea nitrogen (BUN) level improved.Four cases with acute respiratory distress syndrome (ARDS) improved and the ventilator parameters were downregulated.Conclusions Our findings indicate that HPS/MAS complicated with MODS is life threatening with high mortality rate.CBP therapy can lower the fever within a short time,correct electrolyte imbalance,stable circulatory function,improve the lung,liver,and brain function.It is suggested that CBP may be the potential effective therapy in severe HPS/MAS with MODS in children.

Objective To explore the clinical features and diagnostic methods of tuberculosis infection in PICU,and improve the understanding of tuberculosis.Methods We analysed the clinical features and diagnostic methods of severe tubercle bacillus infectious diseases in PICU from Jan 2009 to Dec 2012.Severity of disease was graded by pediatric critical illness score.The diagnosis of the pulmonary tuberculosis was in accord with the diagnostic criteria of paediatric pulmonary tuberculosis established by Chinese Medical Association paediatrics branch.And the diagnosis of tuberculosis meningitis and tuberculosis peritonitis based on the clinical physical examination,laboratory examination and pathologic finding.Results Among 22 cases enrolled in this study,totally 16 cases were pulmonary tuberculosis,6 cases were extrapulmonary tuberculosis,and 3 cases were tuberculosis meningitis.The clinical feature of severe tuberculosis infection in PICU was accompanying with one or multiple organ dysfunction besides tuberculosis infection symptom,among them,respiratory dysfunction occurred in 16 cases,cardiovascular dysfunction was observed in 2 cases,and central nervous system dysfunction was found in 3 cases,even 1 patient experienced cardiovascular system dysfunction,respiratory disorder as well as gastrointestinal system dysfunction simultaneously.Sixteen cases of pulmonary tubercle bacillus infection manifested respiratory failure besides fever,cough,shortness of breath and tuberculosis toxicosis symptom,2 cases of them developed into acute respiratory distress syndrome,8 cases needed mechanical ventilation.Two cases of pericardial effusion presented cardiac tamponade.The level of adenosine deaminase elevated in 12 cases,and the positive result of enzyme-linked immunospot assay for tubercle bacillius was observed in 14 cases.Conclusion It is very important to be aware of that severe tubercle bacillus infection exist in critically ill patients admitted in PICU,measuring the level of adenosine deaminase and taking enzyme-linked immunospot assay for tubercle bacillius test are important accessory examination for tuberculosis diagnosis in children.