Objective With the development of children 's health care policy, mild Wilson Disease as the first symptom of pa-tients is increasing.In this study, we assessed the reliability of the 2001 international general standards of the Wilson Disease diagnos -tic score.Methods Twenty -one cases of Wilson Disease in children (11 cases were boys, 10 cases were girls, average age was 3 ~10.8 years) included in the study, other 21 cases with liver disease were age-matched and gender-matched.There were no clinical symptoms, only with abnormal liver function in laboratory tests .All the patients with Wilson Disease were all confirmed through genetic diagnosis.Results Receiver operating characteristic (ROC) curve analysis showed that at 20 mg /L threshold, the sensitivity of cer-uloplasmin was 93.4% (95% CI : 81.2% ~98.8%), the specificity was 81.8 (95% CI : 71.6% ~93.6%); urinary copper diag-nostic threshold was 40μg/L/24h, the sensitivity was 75.9% (95% CI :61.7% ~92.4%); the specificity was 89.9% (95% CI :73.7% ~96.6%).WD scoring diagnostic system of the positive and negative predictive values as high as 93% and 91.6%.Penicil-lamine load test in Wilson Disease and control groups , no significant difference between the ROC curve showed a sensitivity of only 10%.Conclusions WD scoring diagnostic system in the mild Wilson Disease patients still has a high diagnostic specificity , but peni-cillamine load test does not have diagnostic significance in some patients .

Diagnostic Errors ; legislation & jurisprudence ; Humans

It has been fully studied about interstitial cells of Cajal in gastrointestinal tract,and in recent years these cells are discovered in other tissues and organs,such as urinary system,cardiovascular system,reproductive system,liver,pancreas and mammary gland.The criterion and process reported by some authors are reliable for invesgating these cells,and can be helped to study the distribution and function of these cells outside gastrointestinal tract.It is showed that the fuction of interstitial cells of Cajal exsited in urinary organs seems to be related with the spontaneous rhythmic contraction of smooth muscle,while in heart seems to cause atrial fibrillation in humans,but the poteinal fuctions of interstitial cells of Cajal in other tissues and organs have not been revealed.

Objective To analyse the differences between idiopathic and symptomatic childhood epilepsy with occipital patoxysms. Methods The medical records of 38 patients with interictal spikes and/or sharp waves over occipital areas,posterior temporal-parietal areas,or both were retrospectively analysed.Epidemiological data,ictal symptoms,electroencephalogram,neuroimaging studies,treatment response and prognosis were compared using chi-square test or Mann-Whitney U test. Results Four patients of idiopathic group had positive family history related to nervous system.Seizures were more frequent in symptomatic group than in idiopathic group.Both groups showed autonomic symptoms,while ictal vomiting and deviation of eyes were more common in the presence of Panayiotopoulos type.The prognosis of idiopathic group was much better than that of symptomatic group. Conclusion Panayiotopoulos syndrome is more common.Neurologic deficits and electroencephalographic characteristics are major factors to predict seizure remission in children with occipital lobe epilepsy.Idiopathic childhood epilepsy with occipital paroxysms may result in better prognosis.

Objective To develop a stable and sensitive microemulsion liquid chromatography method by using oil-in-water microemulsion mobile phase;To simultaneously determine five major active lignans, namely, schizandrin, schisandrol B, schisantherin A, deoxyschizandrin, and schisandrin B, in Schisandrae Sphenantherae Fructus and Schisandrae Chinensis Fructus. Methods The quantitative determination was conducted by using Kromasil C18 column (250 mm × 4.6 mm, 5μm). The influence of the composition of the microemulsion system on separation effect and retention time was studied. The method was optimized to sodium dodecyl sulfonate 3.3%(SDS, W/V), n-octanol 1.2% (V/V) and n-butanol 6.6% (V/V) in 20 mmol/L phosphate buffer (adjusted to pH 7.0 with orthophosphoric acid and sodium hydroxide). The flow rate was set at 0.7 mL/min. The compound peaks were detected by UV at 254 nm. The column temperature was 25 ℃. Results Under the optimized conditions, baseline separation of 5 lignans was achieved in 20 min, and the separation was satisfying. Schizandrin, schisandrol B, schisantherin A, deoxyschizandrin, and schisandrin B were in linear relation within the ranges of 2.0-155.0, 8.3-166.7, 8.8-175.8, 8.3-166.7, 8.9-177.1μg/mL (r>0.999). The limit of detection ranged from 0.06μg/mL to 0.18μg/mL, and the limit of quantitation ranged from 0.17μg/mL to 0.55μg/mL. The average recovery values were between 97.22%and 100.8%. Conclusion This method is simple, accurate and reliable, and can be successfully applied to simultaneous determination of five major active lignans in 13 batches of Schisandrae Sphenantherae Fructus and Schisandrae Chinensis Fructus from different producing areas in China.

Objective To explore the clinical and electroencephalography features of children's frontal lobe epilepsy(FLE)and improve the diagnosis and therapy.Methods Medical records were reviewed and neuropsychological evaluations of patients with FLE diagnosed from 2000 to 2006.Children were examined by 24 h EEG and imaging examinations.Their charts for family medical history,seizure,aura,simultaneous phenomenon and anti-epilepic drug therapeutic effects were reviewed.Results Seizures were clonus in 24 cases,tonic in 4 cases,adversive,atonic,vomit in 2 cases,respectively,paresthesia,cephalic,visul,sensory in 1 case,Imaging finding were in 9 of 40.All the 40 cases were bought into 1 to 6 years' follow-up.Long-term control was achieved in 35 of 40.Partial control was achieved in 3 of 40.The left 2 cases had no response to the anti-epileptic drugs.Conclusions The common features of FLE included high seizure frequency,short duration and nocturnal preponderance.There is high positive rate of active EEG in detecting epileptic discharges,and active EEG shoud be necessary.Carbamazepine is recommended to be the first choice to FLE.

Objective To report the management of single allograft lung transplantation in 4 cases of COPD. Methods Four COPD cases receiving lung transplantation were analyzed respectively. There was diffuse emphysema in double lung in one case and in the remaining 3 cases severe diffuse emphysema that mixed together partially to be bulla but not to the 1/3 of the thoracic cavity. All the cases received the corpse donor. One case with diffuse emphysema received the single-right lung transplantation, one case received simultaneous single-lung transplantation and lung volume reduction and the remaining 2 cases received LVRS 47 days and 28 days after single-left lung transplantation respectively. The inductive treatment with the daclizumab or antithymocyte globulin (ATG) was done and the rejection was prevented with the Tacrolimus, mycophenolate and prednisone. Results The operations were successful in the 4 cases. One case had the acute rejection 5 days after operation and reco- vered after intensive treatment with Methylprednisolone. Two cases discharged successfully and lived to today and the first case lived more than 2 years. Two cases died 74 days and 77 days after lung transplantation respectively. Conclusion In late stage COPD, single allograft lung transplantation is an effective treatment to the bilateral emphysema patients and the lung volume reduction should be done simultaneously to the patients with the bullae that may be ruptured.

OBJECTIVETo observe the clinical effectiveness of Qilin Pills combined with sertraline in the treatment of secondary non-consolidated kidney qi premature ejaculation (PE).

METHODSA total of 120 patients with secondary non-consolidated kidney qi PE were randomly assigned to groups A (aged [35.5 ± 5.4] yr), B (aged [36.2 ± 5.7] yr), and C (aged [35.2 ± 5.3] yr) in the ratio of 1:1:1 to receive Qilin Pills (once 6 g, bid), sertraline (once 50 mg, qd), and Qilin Pills plus sertraline, respectively, all for 4 weeks. The intravaginal ejaculatory latency time (IELT) and PE diagnostic tool (PEDT) scores were obtained before and after medication and at 1 month after drug withdrawal, and comparative analyses were made among the three groups of patients.

RESULTSThe IELT was dramatically prolonged in groups A, B, and C after treatment ([3.23 ± 1.84], [3.87 ± 2.43], and [5.92 ± 3.11] min) and at 1 month after drug withdrawal ([1.85 ± 1.27], [1.52 ± 1.06], and [ 4.26 ± 1.88 ] min) as compared with the baseline ([0.88 ± 0.45], [0.84 ± 0.47], and [0.85 ± 0.50] min) (P < 0.01), even longer in group C than in A and B (P < 0.01). The PEDT scores of the three groups were 5.1 ± 1.8, 4.9 ± 1.7, and 3.8 ± 1.2 after treatment and 8.2 ± 2.4, 8.1 ± 2.4, and 6.5 ± 2.1 at 1 month after drug withdrawal, significantly improved in comparison with 13.2 ± 3.2, 12.8 ± 3.1, and 13.1 ± 3.4 before treatment (P < 0.01), even more significantly in group C than in A and B (P < 0.01).

CONCLUSIONQilin Pills combined with sertraline has a definite efficacy in the treatment of secondary non-consolidated kidney qi PE and therefore deserves wide clinical application.

Adult ; Drug Therapy, Combination ; methods ; Drugs, Chinese Herbal ; therapeutic use ; Ejaculation ; drug effects ; physiology ; Humans ; Male ; Premature Ejaculation ; drug therapy ; Qi ; Sertraline ; therapeutic use

Based on the function of nourishing blood and activating blood circulation,Naornaishu(NMS)composed of Rhizome Chuanxiong,Radix Salvice Miltiorrhizae,etc.has been used to treat cerebral arteriosclerosis and infarction.65 Patients with cerebral arteriosclerosis were treated with NMS for about two months and got a total effective rate of 98.5%.50 cases with eerebral infarction were also observed.It was shown that its effect on impro- ving myodynamies,alleviating aphasia,relieving dizziness and turning patholo- gic reflex negative was 86.4%,76.9%,100% and 38.0% respectively.No side effects were obser(?)ed during the period of more than two years of clinical applications.Therefore,NMS is regarded as an ideal medicine for preventing and treating cerebrovascular diseases.

Objective To study the effect of new type antiseptic gallium nitrate on the clearance of Staphylococcus aureus (S .aureus)biofilm,and explore new methods for suppressing bacterial biofilm formation.Methods Biofilm positive strains were screened among 14 clinically isolated S .aureus strains by crystal violet staining method,mini-mal inhibitory concentration (MIC)of gallium nitrate for biofilm positive strains and effect of gallium nitrate on the clearance of biofilm were measured.Results Of 14 S .aureus isolates,9 were biofilm positive strains;gallium ni-trate MICs for S .aureus ATCC 25923,biofilm-negative strain,and 9 biofilm positive strains were all 16 μg/mL;the clearance rate of gallium nitrate for early biofilm of S .aureus was significantly higher than mature biofilm ([86.53±0.96]% vs [62.54±1 .53]%,t=35.699,P <0.001 ).Conclusion Gallium nitrate can inhibit growth of S .aureus strains and clear biofilm,it can be applied in the prevention and control of S .aureus infection.