Objective To assess the clinical features and treatment of children with long QT syndrome (LQTS)and syncope.Methods Eleven cases of children with LQTS and syncope between January 2009 and July 2014 in Hunan Children′s Hospital were retrospectively analyzed for clinical features,treatment and long term follow -up.Results There were 11 cases of children with LQTS aged 4.0 -14.5(9.16 ±2.71)years,8 male and 3 female, with syncope more than once.The range of QTc was 460 -521(483.72 ±22.90)ms.For 3 cases of acquired LQTS,1 case was parathyroid hypothyroidism causing hypocalcemia,1 case was myocarditis complicated with third degree atrio-ventricular block,and 1 case showed atrial flutter receiving amiodarone post congenital cardiac surgery.All patients re-covered after the inducement removed and primary illness cured.For 8 cases of congenital LQTS,3 cases of LQTS un-derwent genetic test (1 case of KCNQ1 gene mutation,2 cases of KCNH2 gene mutation).One case died after frequent torsade de pointes (Tdp)and ventricular fibrillation during hospitalization,the remaining 7 patients were given oral pro-pranolol,potassium chloride sustained -release tablets after discharge.Follow -up time was 8 to 75 months,an average of (45.73 ±24.42)months.One case died suddenly at home after 25 months of follow -up.The remaining 6 cases of children with congenital LQTS could withstand general activities without syncope,in which 4 cases had normal QTc by electrocardiography(ECG),and the findings in 2 cases did not change compared with those previously.The QTc re-turned to normal in children with acquired LQTS in the follow -up review.Conclusions Children with congenital LQTS should receive early genetic screening and genotyping for rational use of drugs.For children with higher risk of sudden death,drug therapy combined with implantable cardioverter defibrillator should be considered.For acquired LQTS,it should be better to remove the inducement and treat primary disease actively.

Objective To explore the clinical and pathologic features of Langerhans cell histiocytosis(LCH).Method Thirty-eight cases of LCH from both in hospital and outpatient department in dermatology and pediatry department from Jan.1991 to Jun.2006 were analyzed about the features of clinic,pathology and immunohistochemistry.Results The mean age of onset was 2.18 years old.The male /female ratio was 1.71.Skin lesions occurred in 78.9% of the patients.Among them,68.5% were as the first manifestation.The eruptions mainly distributed on trunk,90% of them presented as hemorrhagic maculopapules,nevertheless,3.3% of the eruptions showed as crust with hilar depression,which was similar to pityriasis lichenoides etvarioliformis acuta.Fever,hepatomegalia and splenomegia occurred in patients at a rate of 60.5%,68.4%,55.3% respectively.Thirty-one point six percent of the patients had got lymphadenectasis,the neck and inguinal lymph nodes were the common site to be affected.Ossature involvement occurred in 31.6% of the patients,which 8.3% got multiple injuries,howe-ver,91.7% got a solitary bone involved.Skull was the main site to be injured,else were lumbar,humerus,hipbone,an so on.Respiratory tract,auditory canal,mucosa were also the sites involved in this disease,but the incidence rate was lower than 10%,respectively.The laboratory data showed that 81.3% of the patients were anaemia,60.7% with abnormal subgroup of T-cell,and 32.1% positive for EBV-IgG.The skin histopathology data of 26/30 cases revealed that lichenoid infiltrates of Langerhans cells confined to the upper dermis.Cytologic features were cells with abundant eosinophilic cytoplasms,and longitudinally grooved or reniform nuclei.Lymphocytes and a few eosinophils also could be seen.Four cases of thirty showed that the proliferative Langerhans cells were with pale cytoplasms,besides,there were numerous eosinophils,and sometimes a few multinucleate cells were scattered.The immunity test of 20 cases of thirty displayed that CD1a(+)S100(+)KP-1(-).Biopsy of lymphaden and tumor of the skull of the rest 8 patients were all diagnosed as eosinophilic granuloma through both hematoxylin and eosin-stained section and immune marks.Conclusions Multiple systems can be involved in LCH.Hemorrhagic maculopapules,fever and splenohepatomegalia are common presentations in this disease.The morphous of nucles of histiocytes is particular,and to diagnose definitely,both CD1a(+) and S-100(+) are needed.

OBJECTIVE: To analyze postmortem chemical changes in Landrace costal cartilages and ribs using attenuated total reflection-Fourier transform infrared (ATR-FTIR) spectroscopy, and to provide a novel technique for estimation of postmortem interval (PMI). METHODS: The swines were sacrificed by hemorrhage and their costal cartilages and ribs were kept in 20 degrees C. The chemical analysis of the costal cartilages and ribs were performed using ATR-FTIR every 72 h. The correlation between the certain spectral parameters and PMI was also analyzed. The time-dependent changes of costal cartilages were more significant than ribs. RESULTS: There were no obvious changes for the main absorbance bands position, and some absorbance band ratios showed time-dependent changes and significant correlations with the PMI. CONCLUSION ATR-FTIR has the ability to analyze postmortem chemical changes of the swine costal cartilages and ribs, and it can be a new method to estimate PMI based on spectroscopy.
Animals ; Autopsy ; Costal Cartilage ; Forensic Pathology/methods* ; Hemorrhage ; Models, Animal ; Postmortem Changes ; Rats ; Rats, Sprague-Dawley ; Regression Analysis ; Ribs ; Spectroscopy, Fourier Transform Infrared ; Swine ; Time Factors

Objective To evaluate the value of endoscopic ultrasonography (EUS) in the diagnosis of Crohn's Disease (CD).Methods A total of 436 patients with endoscopically suspected CD underwent EUS and the clinical data of these patients were analyzed retrospectively.Changes of gastrointestinal wall stratification and perienteric complications detected by EUS were documented systematically.The consistency between the diagnosis of EUS and the results of pathology were recorded.Results A total of 297 cases of CD and 139 cases of non-CD were clinically diagnosed,while 277 CDs (including 17 non-CDs clinically diagnosed) and 159 non-CDs (including 37 CDs clinically diagnosed) were diagnosed by EUS.The sensitivity,specificity and accuracy rate of EUS in diagnosing CD were 87.5％ (260/297),87.8％ (122/139) and 87.6％ (382/436),respectively.Dilated vessels in submucosa were detected in 40 patients,fistulae in 13,abscesses in 5 and enlarged lymph nodes in 75.Conclusion EUS can show gastrointestinal wall stratification of CD clearly with high diagnostic accuracy.Meanwhile,EUS can detect extraluminal complications well to help in providing useful information for surgery.

Adult ; Eccrine Glands ; pathology ; Female ; Hamartoma ; pathology ; Humans ; Sweat Gland Diseases ; pathology

Adult ; Humans ; Male ; Plasma Cells ; pathology ; Skin Diseases ; diagnosis

OBJECTIVETo study the effects of Volatile Oil of Radix Angelicae Sinensis (VOA) on experimental asthma in rat model based on abnormal immune functions of Treg cells.

METHODSAfter grouping, the asthmatic rats were developed through injecting OVA and AI(OH)3 for sensitization and then administering OVA aerosol for challenge, and the respiratory functions, asthmatic behaviors, IL-10 levels in bronchoalveolar lavage fluid (BALF) (ELISA) and Foxp3 expression (immunohistochemistry) in lung of asthmatic rats were observed.

RESULTSVOA at the doses of 40-160 mg/kg could improve the respiratory functions and the asthmatic behaviors, and upgrade IL-10 levels in BALF and Foxp3 expression in lung of asthmatic rats.

CONCLUSIONVOA has some effects of anti-asthma and one of the mechanisms is to improving the lower immune functions of Treg cells.

Angelica sinensis ; chemistry ; Animals ; Asthma ; drug therapy ; Bronchoalveolar Lavage Fluid ; chemistry ; Disease Models, Animal ; Forkhead Transcription Factors ; metabolism ; Interleukin-10 ; chemistry ; Lung ; metabolism ; Oils, Volatile ; pharmacology ; Plant Oils ; pharmacology ; Rats ; T-Lymphocytes, Regulatory ; cytology

OBJECTIVETo compare clinical results of two methods for the treatment of femoral neck fracture, which are cannulated screw fixation combined with percutaneous autogenous bone marrow grafting, and simple cannulated screw fixation. To investigate the curative effects of cannulated screw fixation combined with percutaneous autogenous bone marrow garfting to promote fracture healing and reducing femoral neck necrosis.

METHODSThe clinical data of 60 cases, which were enrolled from December 2000 to December 2008 consecutively in our hospital, were analyzed retrospectively. Thirty patients with femoral neck fractures were treated with cannulated screw fixation and percutaneous autogenous bone marrow grafting. There were 20 males and 10 females, ranging in ages from 18 to 89 years,with an average of (52.3 +/- 0.2) years. There were 13 patients with traffic accident, 3 patients with falling injuries and 14 patients with tumble. Based on the Garden classification for femoral neck fractures, 1 patient was type I, 6 patients were type II, 12 patients were type III and 11 patients were type IV. Among 30 patients in the control group, 16 patients were male and 14 patients were female, ranging in age from 18 to 91 years, with an average of (51.9 +/- 0.1) years. Twelve patients injured with traffic accident, 1 patient with falling injuries and 17 patients with tumble. Based on the Garden classification for femoral neck fractures, 5 patients were type I, 2 patients were type II,15 patients were type III, and 8 patients were type IV. Patients in the control group were treated with cannulated screw fixation only. All the patients were followed up for 2 years after operation. The fracture healing and complications were evaluated and compared between the two groups.

RESULTSThe average healing time was (7.1 +/- 1.2) months in the observing group and (8.0 +/- 1.4) months in the control group. The healing of femoral neck fracture occurred in 29 cases in observing group while in 24 cases in the control group contrast to femoral head necrosis occurred in 1 case in the observing group while in 6 cases in the control group. According to Harris scoring system, the good and excellent rate of the two groups had statistical difference (P < 0.05).

CONCLUSIONCannulated screw fixation and percutaneous autogenous bone marrow grafting is a more efficient method for accelerating healing of femoral neck fractures and reducing femoral head necrosis.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Bone Marrow Transplantation ; Bone Screws ; Female ; Femoral Neck Fractures ; physiopathology ; surgery ; Fracture Fixation, Internal ; methods ; Humans ; Male ; Middle Aged ; Transplantation, Autologous

BACKGROUND: The association of genetic variation in the IRAK-1 gene with sepsis outcome has been proved. However, few studies have addressed the impact of the IRAK-4 gene variants on sepsis risk. This study aimed to determine whether the polymorphisms in the IRAK-4 gene are associated with susceptibility to and prognosis of severe sepsis in the Chinese Han ethnic population.METHODS: In this case-control study, 192 patients with severe sepsis hospitalized in the emergency department of Zhongshan Hospital from February 2006 to December 2009 and 192 healthy volunteers were enrolled. Exclusion criteria included metastatic tumors, autoimmune diseases, AIDS or treatment with immunosuppressive drugs. This study was approved by the ethical committee of Zhongshan Hospital, Fudan University. Sepsis patients were divided into a survival group (n=124) and a non-survival group (n=68) according to the 30-day mortality. Primer 3 software was used to design PCR and sequencing primers. Genomic DNA was extracted from peripheral blood mononuclear cells. Seven tagSNPs in IRAK-4 were selected according to the data of the Chinese Han population in Beijing from the Hapmap project and genotyped by direct sequencing. The chi-square test was used to evaluate the differences in genotype and allele frequencies between the two groups.RESULTS: The distributions of all tagSNPs were consistent with Hardy-Weinberg equilibrium. The allele and genotype frequencies of rs4251545 (G/A) were significantly different between the severe sepsis and healthy control groups (P=0.015, P=0.035, respectively). Carriers of the rs4251545A had a higher risk for severe sepsis compared with carriers of the rs4251545G (OR=1.69, 95％ CI: 1.10-2.58). The allele and genotype frequencies of all SNPs were not significantly different between the survival group and non-survival group.CONCLUSION: These findings indicate that the variants in IRAK-4 are significantly associated with susceptibility to severe sepsis in the Chinese Han ethnic population.

ObjectiveTo study an application of a method of improving the quality of sampling in review to determine the light areas of endemic fluorosis(referred to as endemic fluorosis) in quality control. Methods Of 15 light endemic fluorosis township(town), six were randomly sampled, and the prevalence of dental fluorosis in 22 village primary school children aged 8 to 12 were reviewed to determine the improved quality of sampling in Xuyong county Sichuan province. ResultsSix townships(towns) were selected by simple random sampling from 15 endemic fluorosis townships(towns) for review inspection in Xuyong country. A total of 22 villages were verified, accounting for 22.7％ of the total 97 villages verified. Of the 416 children for review inspection of dental fluorosis, 383 children were positive. The consistent rate of children' s dental fluorosis was 92.07％, and the verification to be slight villages was up to 21 endemic villages, accounting for 95.45％. ConclusionsThe application of a method of improving the quality of sampling can improve the efficiency of quality control, and improve the accuracy. It is a novel quality control method.