Chronic granulomatous disease is a primary immunodeficiency caused by abnormities in the NADPH oxidase.It is rare clinically and mortality is high.Most patients present with the repeated and fatal infections.Local granuloma can easily lead to local obstruction.At present,there are several specific diagnostic methods for chronic granulomatous disease,including p-nitro-blue tetrazolium chloride,dihydrorhodamine 123 and gene sequence analysis,etc.Hematopoietic stem cell transplantation is the only radical method.Appropriate timing of transplantation is critical to improve the success rate of transplantation.Gene therapy still has a long way to clinical use due to effectiveness and safety.This review describes recent progress in the study of pathogenesis,early diagnosis,treatment of chronic granulo matous disease.

OBJECTIVE: To study the effect of compound glycyrrhizin on hepatic function and hepatic fibrosis of patients with chronic hepatitis B. METHODS: 33 patients with chronic hepatitis B were injected i.v with 80ml compound glycyrrhizin q.d for 4 weeks. The hepatic function, hepatic fibrosis of patients and the clinic effects before and after treatment were observed. RESULTS: The total effective rates for patients with mild, moderate and severe degrees were 100%, 92.3% and 91.7%, respectively. There were significant differences in alanine aminotransferase, total bilirubin(P

Objective To discuss the nursing management for patients receiving percutaneous fluoroscopy - guided gastrostomy (PFG) for the treatment of dysphagia caused by head and neck cancer. Methods A total of 15 patients with dysphagia caused by head and neck cancer received PFG. Before PFG sufficient preparation and psychological nursing care were carried out for all the 15 patients. After the operation the patients were kept under close observation for any changes in his or her clinical conditions. Proper prevention measures against complications were adopted. Results PFG procedure was successfully accomplished in all 15 patients. Postoperative complications included gastric bleeding (n = 1), stoma infection (n = 1) and proctoptosis (n = 2), and the disorders were recovered after clinical management. After PFG intravenous nutrition was no longer employed in all patients, and enteral nutrition was conducted. The quality of life was significantly improved in all patients. Conclusion PFG is technically - simple and minimally- invasive with fewer complications. This technique provides a new way to give enteral nutrition for patients with dysphagia caused by head and neck cancer. Excellent psychological nursing, sufficient preoperative preparation, comprehensive postoperative nursing care and medical advices at the time of discharge are the key points to ensure a successful PFG.

A fungus that is capable of producing EPA and DHA was isolated from seawater. A fast extraction and methyl esterification of microbial lipid were established as well as their content determination by capillary gas chromatography using external calibration method. The results showed that the methyl ester prepared directly form the wet mycelia treated with CHCl3-KOH-CH3OH is less time-consuming and the method fits for the fast determination. To determine its EPA and DHA content,the wet mycelia was hydrolyzed according to Weibul 's method and the lipid obtained was methyl esteri.fied with KOH-MeOH,the results obtained by capillary gas chromatography using external calibration method were 7. 32% and 7. 58% respectively.

Objective To investigate the clinical effect of acellular allograft dermal tissue patch used in transvaginal rectovaginal fistula repair.Methods From Jan.2008 to Dec.2011,22 patients with rectovaginal fistula undergoing treatment in Chinese People's Liberation Army General Hospital were studied retrospectively.Twelve patients treated by tissue patch were classified into study group matched with 10 patients with general surgery as controls.Results In study group,11 patients were successfully repaired by their first surgery; one patient was successfully fixed by the second surgery.The successful rate of first operation was 11/12 in study group and 4/5 in recurrent transvaginal rectovaginal fistula.In control group,7 patients were fixed successfully in their first surgeries,the successful rate of first surgery was 7/10.Two primary patients and 1 recurrent patient were successfully fixed by their second surgeries.All of the patients were followed up for (9.0 ± 2.0) months,and no recurrence diseases were observed.Conclusion The transvaginal rectovaginal fistula fixed using acellular allografi dermal tissue patch could get less trauma and higher cure rate.

Objective To compare the clinical value of Heidelberg retinal tomography (HRT-Ⅱ) and Heidelberg optical coherence tomography (HRA OCT) in discriminating healthy eyes and pre-perimetric glaucoma eyes.Methods A total of 33 patients with pre-perimetric glaucoma and 37 healthy subjects were included in this study.All individuals underwent imaging tests with HRT-Ⅱ and HRA OCT within 1-month period.ANOVA and Kruskal-Wallis test were used to compare all of the parameters from HRT-Ⅱ and HRA-OCT between pre-perimetric glaucoma eyes and normal eyes.Receiver operating characteristic curves (ROC) were calculated for all of the parameters to seek the best discriminant point of each instrument between pre-perinmetric glaucoma and healthy eyes.Results Statistically significant differences were found in most of the HRT-Ⅱ parameters except for disc area and height variation contour between pre-perimetric glaucomatous eyes and normal eyes (all P＜0.05),which showed that most of the HRT-Ⅱ parameters were capable of diagnosing preperimetic glaucoma except for the disc area and height variation contour (all P＜0.05).66.7％ of the areas under the receiver operating characteristic curves (AUC) from HRT-Ⅱ parameters were larger than 0.800 and 58.3％ of the AUC were larger than 0.900.Statistically significant differences were found in most of the HRA-OCT parameters except for the nasal retinal nerve fiber layer (RNFL)thickness between pre-perimetric glaucoma eyes and normal eyes (all P＜0.05),which showed that most of the HRA-OCT parameters were capable of diagnosing pre-perimetic glaucoma except for the nasal RNFL thickness (all P＜0.05).66.7％ of the AUC from HRA-OCT parameters were larger than 0.800 and 33.3 ％ of the AUC were larger than 0.900.No statistically significant difference was found between the AUC for the best parameter diagnosing pre-perimetic glaucoma from HRT Ⅱ (cup/disk area ratio,AUC =0.984) and HRA OCT (average RNFL thickness,AUC =0.940).No Statistically significant differences were found in specificity and sensitivity of diagnosing pre-perimetic glaucoma between the two best parameters cup/disk area ratio and average RNFL thickness (both P＞0.05).Conclusions Both HRT-Ⅱ and HRA-OCT can well reflect the damage of optic disc and retinal nerve fiber layer in eyes with pre-perimetric glaucoma.HRT-Ⅱ and HRA-OCT have higher accuracy and similar sensitivity and specificity for the diagnosis of pre-perimetric glaucoma.

OBJECTIVE:To study the correlation of inorganic elements and active ingredients in Paris polyphylla. METH-ODS:HPLC was adopted for contents determination of polyphyllinⅠ,Ⅱ,Ⅵ and Ⅶ:the column was Phenomenex C18 with mo-bile phase of acetonitrile-water(gradient elution)at a flow rate of 1.0 ml/min,detection wavelength was 203 nm,and column tem-perature was 30 ℃. ICP-MS was adopted for contents determination of Na,Mg,K,Ca,Cr,Mn,Fe,Ni,Zn,Se,Sr,Mo,Pb, Cd,Hg,Cu and As:RF power was 1.55 kW,sampling depth was 10 mm,the plasma gas flow was 15.0 ml/min,carrier gas flow rate was 0.86 L/min,spray chamber temperature was 2 ℃,helium collision mode was He,gas flow was 4.5 ml/min,measurement points/peak was 6,sampling mode was hopping peak collection(repeated 3 times). And SPSS 21.0 software was adopted for step-wise regression analysis of correlation between the two above-mentioned. RESULTS:The linear range was 0.939-4.697 μg(r=0.999 8)for polyphyllinⅠ,1.124-5.620 μg(r=0.999 6)polyphyllinⅡ,0.784-3.918 μg for polyphyllin Ⅵ,0.976-4.880 μg for poly-phyllinⅦ.RSDs of precision,stability and reproducibility tests were lower than 2%,recoveries were 96.53%-100.71%(RSD=1.69%,n=6) for polyphyllinⅠ,98.19%-99.55%(RSD=0.58%,n=6) forⅡ,95.45%-100.83%(RSD=1.87%,n=6) for poly-phyllin Ⅵ,96.11%-102.01%(RSD=2.07%,n=6)for polyphyllin Ⅶ . The linear range was 0-195.984 3 mg/kg(r≥0.999 3),de-tection limit was no higher than 65.201 ng/kg. PolyphyllinⅠshowed positive correlation with Mg and negative correlation with Pb;polyphyllin Ⅱ showed positive correlation with Cu and negative correlation with As;polyphyllin Ⅵ showed positive correlation with Sr and Hg and negative correlation with Mn;polyphyllin Ⅶ showed positive correlation with Na and Ni and negative correla-tion with As;the total contents of the 4 polyphyllins showed positive correlation with Ni and Sr and negative correlation with Fe. CONCLUSIONS:The contents of inorganic elements and P. polyphylla have certain correlation with active ingredient.

Objective To identify high?risk factors of white matter damage ( WMD ) in premature infants,in order to reduce the incidence and improve the prognosis of brain injury?Methods Five hundred and seventy?seven cases of preterm infants treated from January 2012 to December 2013 in the Pediatrics Department of Affiliated Hospital of Qingdao University were slecteted?According to MRI of the brain at 2 weeks after birth, all the patients were divided into WMD group(133 cases) and the control group(444 cases)?Single?factor and multifactor logistic regression were used for the analyses of the high?risk factors of WMD?Results WMD incidence was 23?05%( 133/577) in 577 cases of premature infants,with 108 cases( 81?2%) of focal injury,5 cases(3?8%) of diffuse injury and 20 cases(15?0%) of periventricular leukomalacia(PVL) injury?There was significant statistically difference in different gestational age( P=0?017)?Multi factor Logistic regression analysis showed that metabolic acidosis(OR=4?489,P=0?000),asphyxia(OR=3?519,P=0?000),perinatal infection ( OR=2?650,P=0?003) were independent risk factors for WMD,while cesarean( OR=0?293,P=0?000) was protective factor for WMD?Conclusion WMD is a complex interaction outcome of multiple risk factors in premature infant?Perinatal hypoxia and infection are the main risk factors of WMD in premature infants.

Objective Bone loss due to estrogen deficiency can be prevented by estrogen replacement therapy. But it produces a number of side effects associated with the cancer of breast and uterus which limits its application. Selective estrogen receptor modulators(SERMs) are a new category of therapeutic agents, they could bind to estrogen receptors with high affinity and mimic the effects of estrogen in some tissue but act as estrogen antagonists in others. This study was designed to observe the effect of Raloxifene on osteoporosis of the OVX rat model and probe into its mechanism. Methods Thirty-two 3-month-old female SD rats were randomly divided into four groups: the basal control, the OVX, OVX with RLX,OVX with DES. All rats were killed at the end of the experiment five months later. Body weight, bone mineral density (BMD), bone histomorphometry, uterine weight were measured. Results BMD of total body, lumbar spine and femur in OVX rats was significantly lower than that of rats treated with RLX by decreasing 21%, 35%, 40%, respectively. The number of osteoclast was significantly decreased in RLX-treated-OVX rats compared to OVX rats (P

Objective To present the clinical features and to evaluate interventional therapy for Budd-Chiari syndrome in Chinese youth.Methods From January 1990 to April 2012,227 cases who hospitalized at the age ＜ 29 underwent color Doppler ultrasound scan and digital subtraction angiography (DSA).87 cases were with occlusive inferior vena cava (IVC type),105 cases with occlusive hepatic veins (HV type) and 35 cases with occlusive inferior vena cava and hepatic veins (MIX type).The occlusive veins were opened by percutaneous transluminal angioplasty (PTA),endovascular stent placement,intravenous catheter thrombolysis or combination.Postoperative anticoagulation was given to all patients.Results The symptoms and signs of portal hypertension disappeared or were alleviated in successful cases.Technical success was achieved in 210 patients.The success rate was 100％ in IVC type,85.7％ in HV type and 94.3％ in MIX type.IVC pressure decreased from (26.52 ± 8.16) cm H2O to (14.28 ±4.08) cmH2O(P ＜ 0.05) and HV pressure dropped from(35.70 ± 13.26) cm H2O to(18.36 ±8.16) cm H2O (P ＜0.05).Restenosis or occlusion was found in 21.4％ (45/210) patients after a follow-up of 1 month to 15 years.The rate was 13.8％ (12/87) in IVC type,31.1％ (28/90) in HV type and 15.2％ (5/33) in MIX type.These patients were managed by interventional procedures.Technical successwas achieved in 44 cases with restenosis.Conclusions Hepatic vein occlusion was the most common type of BCS in Chinese youth.The symptoms and signs of portal hypertension were the initial clinical manifestations.Postoperative recurrence rate in HV type was higher than that in the other two types.