Objective To explore the diagnosis and curative effect of phenylketonuria(PKU) in Gansu.Methods Fifty-nine thousand nine hundred infants and 118 borderline cases were screened from Oct.1999 to Dec.2006.Three drops of venous blood was collected from the exterior or interior part in the heel,and trickled the venous blood onto the specialized filter paper,which could be formed into 3 bloodstains with the diameter of 0.8-1.0 cm.Phenylalanine(phe) was detected by chemiluminescence method,and PKU was screened by the PKU standard of diagnosis.Infants with PKU had been given low phe food.During the treatment,phe level,physical development and intellectual development were measured regularly.Patients′ body weight,height and head circumference were measured once 1 month.Patients′ intellectual quotient(IQ) were measured every half year(IQ was analyzed by Gesell test).Results were analyzed by SPSS 11.0 software for t test.Results In total,93 patients with PKU were diagnosed and treated.Thirty-six cases were identified by neonatal screening before 3 mouths old.Mental retardation was found in 57 cases before 6 years old.After treatment with low-phe diet,the follow-up for early-treated patients revealed that their physical and mental development were normal,and height,weight and head circumference were in the normal amplitude,and the results of IQ was(92?12)scores.In later treated patients,abnormal behaviors were significantly improved and their developmental quotients were elevated.Before treatment,Gesell value was(57?20)scores;after 12-18 months treatment Gesell value was(70?20)scores.The difference was significant(t=1.705 P

Objective To investigate the effects of suramin application in acute angle closure glaucoma surgery patients.Methods A prospective study method was used.From February 2012 to January 2016,178 acute angle closure glaucoma surgeries in our hospital for treatment were selected,and were equally divided into observation and control groups (89 cases in each group) according to the order of admission,and two groups were given filtration surgery.The observation group was treated with suramin adjuvant therapy after surgery.The duration were 1 ～ 14 d after operation.The prognosis of two groups was observed.Results All patients were successfully completed surgery.The intraoperative and postoperative complications were no seriously occurred;the postoperative intraocular pressure in the observation group and the control group were (11.52 ± 3.24) mmHg and (16.98 ± 5.33) mmHg that compared preoperative [(31.98 ± 5.22) mmHg,(31.87 ± 5.11) mmHg] with significantly different (P ＜ 0.05),while the observation group was significantly lower than that of the control group (P ＜ 0.05).The postoperative peak systolic velocity (PSV) and end diastolic velocity (EDV) values of the central retinal artery of the observation group and the control group were significantly higher than that of preoperation (P ＜ 0.05),and the postoperative PSV and EDV values of the central retinal artery of the observation group were significantly higher than that in the control group (P ＜ 0.05).The postoperative ratios of functional filtering bleb in the observation group and control group were 80.9％ and 60.7％,respectively.The observation group was significantly higher than that of the control group (P ＜ 0.05).Conclusions The application of suramin in acute close angle glaucoma filtration surgery can promote the function of filtering bleb and intraocular pressure reduction,and improve the ocular blood flow speed that has good application effects.

· AIM: To explore the relationship between the expression of Fas/FasL and the apoptosis in retinal ischemia/reperfusion injury of rats, as well as the therapeutic effects of basic fibroblast growth factor (bFGF)on the ischemic retina.injury were made by transiently elevating introcular pressure. A total of 28 rats were divided into Normal Group and Operative Group. The latter were subdivided into 1, 6, 12, 24, 48 and 72h after reperfusion, in which the left eyes of the rats were in the ischemia/reperfusion groups and the right ones were in the treatment groups (bFGF intracameral injection). Apoptosis was assessed by the terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick-end labelling (TUNEL) method, and theexpression of Fas/FasL ligand was studied by strept avidin-biotin complex (SABC) immunohistochemistry.mai rats' retinae, but there were a significant number of TUNEL positive cells in 6-24h after transient ischemia followed by a decrease at 48h. The number of TUNEL positive cells reached a maximum at 24h after ischemia.The expression of Fas gradually increased as early as at 6h, reached a peak at 24h, then decreased at 48h. Similarly, the expression of Fas ligand was at peak in 24-48h in GCL and INL of retina. bFGF ministered before reperfusion inhibited apoptotsis and ameliorated the tissue damage. It also diminished Fas and FasL expression in ischemic/reperfused retina.siently elevated IOP induced apoptosis of cells in the retina. Fas/FasL may have an important role in the early events of the apoptotic pathways. bFGF can rescue RGCs from retinal ischemia/reperfusion injury through down-regulation of Fas and Fas ligand expression and may represent an important mechanism for therapeutic neuroprotection.

Stress cardiomyopathy is an atypical myocardial disease induced by emotional or physical stress, with the characteristic of left ventricular systolic dysfunction, transient imaging and electrocardiogram (ECG) changes. Sudden cardiac death can occur in severe cases. Clinical symptoms are likely to appear on acute myocardial infarction, but the exact pathological mechanism is unclear. In the present study, we perform a systematic review of the literature on the clinical manifestations, epidemiological characteristics, ECG, imaging and laboratory tests of stress cardiomyopathy, in order to provide the values for forensic pathology diagnosis.
Death, Sudden, Cardiac ; Diagnostic Imaging ; Electrocardiography ; Humans ; Myocardial Infarction ; Stress, Psychological ; Takotsubo Cardiomyopathy/physiopathology*

Objective To evaluate the plasma levels of adrenomedullin (ADM) and urotensin Ⅱ ( U-Ⅱ ) in children with capillary bronchiolitis, and their clinical significance. Methods One hundred and fifty three children with capillary bronchiolitis and 36 healthy children were recruited. Plasma levels of ADM and U- Ⅱ were measured at acute stage ( <7days) and convalescent stage (>14days) of airway inflammation. The relationship of plasma ADM and U-Ⅱ levels with symptom scores was evaluated. Results Plasma levels of ADM and U-Ⅱ in acute stage of capillary bronchiolitis were significantly higher than those in convalescent stage and healthy controls ( ADM: t = 20. 57 and 26. 26, P < 0. 01; U-Ⅱ: t = 14. 27 and 7. 61, P < 0. 01 ) , and there were significant differences among mild, moderate and severe capillary bronohiolitis (F = 245. 94 and 304. 79, P <0. 01). Plasma level of U-Ⅱ in convalescent stage of capillary bronchiolitis was lower than that of healthy controls (t = 6. 99, P <0. 01 ) , but ADM was still in a higher level ( t = 8. 98, P < 0. 01 ). In the convalescent stage, there was significant difference on U-Ⅱ levels among mild, moderate and severe capillary bronchiolitis ( F = 25.69, P < 0. 01 ) , but no significant difference was observed for ADM levels (F = 2. 25 , P > 0. 05 ) . Plasma levels of ADM and U- Ⅱ in acute stage showed positive correlation with symptom scores, and the regression coefficients were 0.884 (P =0. 000) for ADM and 0. 943 (P = 0. 000) for U-Ⅱ . Conclusion Plasma ADM and U-Ⅱ levels in children with capillary bronchiolitis are increased in acute stage and correlated with the symptom scores, which may serve as laboratory indicators for assessing the severity of the disease.

Objective To study the efficacy of rituximab in the treatment of pemphigus vulgaris (PV) and its underlying mechanism. Methods A 38-year-old female with PV presented with refractory, painful oral ulcers and erosions. Since she was poorly responsive to prednisone 80 mg daily, intravenous ritu-ximab of 500 mg once a week was given for successive 3 weeks followed by 5 successive days of intra-venous gamma globulin at a dose of 400 mg/kg per day, and a total of two treatment sessions were conducted. ELISA was used to detect the serum titer of anti-Dsg3 antibodies and their IgG subtypes (IgG1 and IgG4) as well as serum level of interferon-gamma (IFN-gamma), interleukin-4 (IL-4), interleukin-10 (IL-10). Results Three months after the end of two treatment sessions, the symptom was obviously improved, and lesions subsided; alter another 1 month, clinical symptom fully disappeared. During the 1-year follow-up, no lesions recurred. The anti-Dsg3 antibody titer was 253.33 U/mL before treatment, plateaued at 250 U/mL within 4 weeks after the initial infusion, decreased till 3 months after the withdrawal, and reached 26.06 U/mL 7 months after the withdrawal, and remained within normal range till the time of this writing. The serum titer of IgGl subclass of anti-Dsg3 antibodies dropped dramatically fight after the first infusion, but that of IgG4 subclass remained at a high level at early stage of medication, began to decline until 3 months after the with-drawal, and finally reached the normal range following clinical remission. Also, serum level of IFN-garnma and IL-10 correlated with the severity of PV. Conclusions Combined rituximab is effective for the control of PV, likely by eliminating Dsg3-specific antibodies, especially IgG4 subclass of them.

Objective To evaluate the effect of dexmedetomidine on spinal purinergic receptor 2X-4 (P2X4)/nucleotide-binding oligomerization domain-like receptor family pyrin domain-containing 3 (NLRP3) pathway in a rat model of diabetic neuropathic pain (DNP).Methods Twenty-four pathogenfree adult male Sprague-Dawley rats,weighing 200-220 g,aged 8 weeks,were allocated into 3 groups (n =8 each) using a random number table:control group (C group),DNP group and DNP plus dexmedetomidine group (DNP+D group).Diabetes mellitus was induced by intraperitoneal 1％ streptozotocin 60 mg/kg and confirmed by blood glucose ≥ 16.7 mmol/L 3 days later.In group DNP+D,dexmedetomidine 50 μg/kg was intraperitoneally injected once a day for 6 consecutive weeks starting from 3 days after the model was successfully established.The mechanical paw withdrawal threshold (MWT) and sciatic nerve conduction velocity (SNCV) were measured at 2,4 and 6 weeks after injection of dexmedetomidine.The rats were sacrificed at 6 weeks after injection of dexmedetomidine,and the L4-6 segments of the spinal cord were removed for examination of the pathological changes (with a light microscope) and for determination of P2X4,NLRP3 and interleukin-lbeta (IL-1β3) expression (by Western blot).The sural nerve was obtained for examination of the ultrastructure by electron microscopy.Results Compared with group C,the MWT was significantly decreased at 2,4 and 6 weeks after injection of dexmedetomidine,the SNCV was decreased at 6 weeks after injection of dexmedetomidine,the expression of P2X4,NLRP3 and IL-1β in the spinal cord was up-regulated (P＜0.05),and the pathological changes of the spinal cord and sural nerve were marked in DNP and DNP+D groups.Compared with group DNP,the MWT was significantly increased at 2,4 and 6 weeks after injection of dexmedetomidine,the SNCV was increased at 6 weeks after injection of dexmedetomidine,the expression of P2X4,NLRP3 and IL-1β in the spinal cord was down-regulated (P＜0.05),and the pathological changes of the spinal cord and sural nerve were significantly attenuated in group DNP+D.Conclusion The mechanism by which dexmedetomidine mitigates DNP is probably related to inhibition of P2X4/NLRP3 pathway in rats.

Objective To detect levels of serum platelet activating factor(PAF),thrombomodulin(TM) and white blood cell(WBC),platelet count(PLT) in neonates with meconium aspiration syndrome(MAS),and observe changes of mediators of inflammation and function of endotheliocute.Methods All cases were taken vein blood in 24 h and 72-96 h after birth.Surm PAF and TM were detected by EILSA technique,at the same time,blood cell counts were determined.Results PAF and WBC in neonates with MAS increased,which were relevant to the patients′ condition.TM of neonates with MAS increased significantly,especially in 72-96 h after birth and(aggrava)-ted with the patients′ condition.Conclusion Neonates with MAS have inflammatory reaction and injured endotheliocyte,which are(inte)-raction.

Objective:To evaluate the reliability and validity of the Chinese version of behavioral activation for depression scale (C-BADS) in patients in methadone maintenance treatment (MMT).Methods:A total of 162 opioid addicts were recruited from the MMT programs in Wuhan city for the C-BADS investigation.Items analysis and exploratory factors analysis (EFA) were conducted with SPSS 21.0 and confirmatory factors analysis (CFA) was conducted with AMOS 24.0.Results:The correlation coefficients between subscales and total score were 0.228-0.813 (all P<0.01). Except for activation subscale, the correlation coefficients among subscales were lower than those between subscale dimensions and total score.The item-total score correlations coefficient were 0.060-0.716.Four factors were extracted by EFA, named avoidance/rumination(AR), activation(AC), social impairment(SI) and work/school impairment(WS) respectively, which the cumulative variance was 51.153%, and the factor loading of each item ranged from 0.328 to 0.797.The fitting indexes of CFA model were as following: χ 2/ df=1.594, CFI=0.839, TLI=0.820, IFI=0.844, RMSEA=0.069, SRMR=0.089.The Cronbach's α of the scale was 0.790, and the split-half reliability coefficient was 0.827. Conclusion:C-BADS has good internal consistency reliability and construct validity, but it still needs to be modified for the measurement of behavior activation of patients in MMT.

Chrysanthemum coronarium is an economically important plant in Asia, and used medicinally, ornamentally and as a vegetable. In April 2017, leaf spot disease on C. coronarium was observed in Shiyan, Hubei, China. A single-spore isolate was obtained and identified based on morphology and sequence analysis using four regions (rDNA ITS, GAPDH, EF-1α, and RPB2). The results indicated that the fungus is Alternaria argyranthemi. The pathogenicity tests revealed that the species could cause severe leaf spot and blight disease on the host. This is the first report of leaf spot disease on C. coronarium caused by A. argyranthemi in the world, which is also a new record of Alternaria species in China.
Alternaria* ; Asia ; Asteraceae ; China* ; Chrysanthemum* ; Fungi ; Plants ; Sequence Analysis ; Vegetables ; Virulence