Objective To evaluate the efficacy and safety of sildenafil (viagra ) on men with ED of various etiologies in China. Methods This study was a double blind, randomized (1∶3, placebo∶ sildenafil), placebo controlled, parallel group, multi center, flexible dose escalation study of sildenafil oral tablets (25, 50 and 100mg) taken over an 8 weeks period. A total of 628 subjects were screened and randomized. Results The primary efficacy variables (questions 3 and 4 from IIEF) revealed a statistically significant ( P

Objective To investigate the feasibility of achieving consistent image quality with dose reduction technology in lumber spine MSCT examination with Z-axis automatic tube current modulation (ATCM). Methods Forty-eight patients diagnosed as lumber intervertebral disc protrution scanned twice by MSCT before and after interventional operations with the same coverage from third lumbar vertebra to first sacral vertebra. The first scan (FM) was with fixed tube current of 320 mAs. The follow-up scan was with ATCM with noise index (NI) of 12.0 HU. At the levels of L3-4, L4-5 and 15-S1, image quality, image noise and radiation dose were measured and analyzed. Image quality and radiation dose were compared by paired t-test and the image noise was compared by ANOVA test. Results The dosage of the ATCM had a 31.3% reduction compared with FM, the average DLP was(187.9±66.4)mGy·cm and(273.4±45.4) mGy·cm respectively, where t = 8.205, P < 0.05. The average noise and their deviations for the FM group were (9.8±2.4) HU,(9.9±2.4) HU, and (11.5±3.2) HU at level of L3-4, L4-5, 15-S1, respectively. With ATCM, the average noise was(12.0±0.8) HU, (11.7±0.6) HU, and (11.7±1.4) HU, respectively. There was statistical difference between the two groups (F = 23.31, P < 0.05). The image quality scores for the FM group were (4.7±0.3), (4.5±0.2), (4.5±0.2) and showed no statistical difference to ATCM group (4.6±0.3), (4.5±0.2), (4.5±0.2) at level of L3-4, L4-5, L5-S1, respectively, where t = 1.000, P > 0.050 Conclusion ATCM technique with the noise index setting at 12.0 HU can achieved a 31.3% dose reduction while keep the consistent image quality for lumbar spine MSCT study.

Objective To evaluate diagnostic value of CT coronal scan to foreign body in bronchus of children.Methods 30 cases appearances of CT coronal scan to foreign body in bronchus of children were analysed.The appearances between CT coronal scan and axial scan,X-ray examination of chest were contrasted in some cases.Results Foreign body in bronchus or granuloma around part of foreign body were shown clearly by CT coronal scan in 30 cases,28 cases obstructive emphysema,13 cases obstructive pneumonia and 3 cases obstructive atelectasis were also shown clearly.Another,CT coronal scan showed the double margin sign of 16 cases diaphragm and 11 cases mediastinum.Conclusion CT coronal scan could show direct and indirect signs of the most foreign body in bronchus of children and it may be acte as primary examintion.

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.

Objective To analyze the clinical characteristics and prognosis of disseminated cryptococcosis in children.Methods The data of disseminated cryptococcosis inpatients were reviewed retrospectively at Beijing Children's Hospital,Capital Medical University,from January 2002 to September 2014.The demographic data,clinical manifestations,laboratory findings,imaging,antifungal treatments and outcomes of all the patients were analyzed.Results Overall 25 children with disseminated cryptococcosis were enrolled including 17 boys(68.0％).The average age was 7 years old.Four cases (16.0％) had underlying conditions,among them 1 case had human immunodeficiency virus(HIV)-positive.The median time to diagnosis was 32 (23-47) days,44.0％ of the patients (11 cases) were misdiagnosed,and 8 cases misdiagnosed as tuberculosis.All patients had fever.Other common clinical manifestations included cough (16 cases),headache (10 cases),vomiting (10 cases),altered mental status (6 cases) and stomachache (6 cases).Respiratory system involvement was seen in all cases,central nervous system was involved in 18 cases,other organ involvement included lymph nodes,spleen,liver,kidney,skin,skeleton and costicartilage.Amphotericin B (AmB) + Fluconazole (Flu) ± 5-flucytosine (5-FC) was the most common therapy (15 cases),Flu ± 5-FC for 6 cases,AmB ± 5-FC for 2 cases,Voricanazole (VOR) for 1 case.Sixteen cases (66.7％) got recovery/improved on discharge,8 cases (33.3％) rejected to the advise and discharged with treatment failure,and the HIV-infected patient transferred to a special hospital.Fifteen patients (60.0％) were followed up,and 13 cases (87.0％) showed recovery,but 2 died in the long-term prognosis.Conclusions Disseminated cryptococcosis in children is more common in school-age boys.Most patients are without underlying conditions.Disseminated cryptococcosis can cause multiple organ damage.Patients with prolonged fever,cough,headache,with or without underlying diseases,can be suspected as disseminated cryptococcosis.Blood and cerebrospinal fluid culture for fungus,cryptococcal antigen should be performed for early diagnosis and treatment.

Objective To investigate the expressions of Occludin in brain after bacterial meningitis and to discuss possible molecular mechanism of bacterial meningitis when brain edema occurs. Methods The models of bacterial meningitis and normal control were constructed via inoculating intracisternally with strain Ⅲ streptococcus pneumoniae and the same volume of normal saline solution, respectively. The expression of Occludin in brain was detected by immunohistochemistry and Western blot methods respectively and 24 h, 48 h and 5 days after inoculation. Results (1) Loeffler neurologic deficit score (NDS) in 24 h, 48 h and 5 d decreased significantly when compared with that of control group (P < 0.05). (2) After the brain received streptococcus pneumoniae injection, expression of Occludin began to decrease at 24 h and touch the bottom at 48 h,then increase at the 5th day, but still remained lower than that in control group, which indicated statistical difference (P < 0.05). Conclusions Expression of Occludin in the models of bacterial meningitis decreased firstly and then increased regularly. It suggests that Occludin plays a protective role during the development of infectious brain edema.

Objective To summarize the clinical features of histiocytic necrotizing lymphadenitis (HNL) and the lesion of central neural system in children.Methods Data of the 4 cases in Beijing Children's Hospital from January 2009 to December 2014 were reviewed and analyzed,as well as other files of 12 patients from PubMed,CNKI,etc.Results All 4 patients mentioned above started from fever and lymphadenopathy,3 cases had headache,2 cases of them experienced altered mental status,and 1 case had cclampsia.The findings of cerebrospinal fluid (CSF) test was similar to viral encephalitis;2 cases had normal magnetic resonance imaging (MRI) result;1 case with MRI test indicated brain white matter diseases with extensive intracerebral hemorrhage,and 1 case had small range of encephalomalacia in left cerebral hemisphere and cerebral dura mater.Through CSF examination,2 cases were positive to Epstein-Barr viral capsid antigen (EBV-VCA)-IgM,while the 2 other cases had no positive trace of EBV-VCA-IgM.Lymph node biopsy was in accordance with HNL typical changes,1 case was positive for Epstein-Barr virus encoded RNA (EBER).Twelve cases in previous literatures were diagnosed as HNL by lymph node biopsy,and their clinical features of central nervous system were headache and altered mental status.There was no change in pathogen.It is also found that the cases in which CSF was performed had shown being negative.CT/MRI image descriptions involved in temporosphenoid lobe,hippocampus,caudate lobe,leptomeninges,etc.Conclusions HNL can be complicated with lesions of the central nervous system,characterized by aseptic meningitis,encephalitis,etc.The prognosis of most cases is good,a few can have neurological sequelae.HNL may relapse,so long-term follow-up is ne-cessary.Epstein-Barr virus in HNL and lesion of central nervous system mechanism still needs further research.

Objective To compare the efficacy of adrenocorticotrophic hormone (ACTH) and methylprednisolone on the rat models of infantile spasms (IS).Methods The SD rats on postnatal 10 day (P10) were divided into blank group (n =18),control group (n =18) and model group (n =110) according to the random number table method.The rats of model group were prepared by adopting prenatal stress exposure and N-methyl-D aspartate (NMDA) injection.In the model group,after inducing epileptic seizures,the rats were divided into different groups (18 rats in each group) according to the random number table method as following:model group Ⅰ (subcutaneous injection ofACTH,50 IU/kg,at P10:14:00,21:00;P11,P12:7:00,14:00,21:00;P13:7:00),model group Ⅱ (subcutaneous injection of 9 g/L saline),model group Ⅲ (intraperitoneal injection of methylprednisolone,60 mg/kg,at P11,P12,P13:9:00,once per day),model group Ⅳ (intraperitoneal injection of 9 g/L saline) and model group Ⅴ (positive control group,with no drug or saline injection).Three days later,epilepsy was induced again,and the rats of model group were intraperitoneally injected with NMDA (12 mg/kg) at P13 (10:00).The rats of control group were injected intraperitoneally with same volume of 9 g/L saline,but the rats of blank group were not treated.Behaviors of rats with epilepsy seizures were observed and epilepsy scores were given.The expression of corticotropin-releasing hormone (CRH) in the hypothalamus of each group was detected by using immunohistochemistry and fluorescence quantitative polymerase chain reaction.The learning and memorizing capacity of the rats were measured by Y-maze experiment.Results There was no death in the model group after the onset of seizure.In the model group Ⅰ,13 cases were attacked(72.22％),and 14 cases were attacked in the model group Ⅲ (78.78％).The level of attack was decreased.The buckling state was not observed in model group and Ⅲ,but the latency period of epilepsy was prolonged and the epilepsy scores were significantly decreased.There were no significant differences of onset latency [(2 369.38 ± 628.70) s vs.(1 922.93 ± 462.36) s] and epilepsy score [(2.15 ± 1.14) scores vs.(2.07 ± 0.83) scores] between the 2 groups (all P ＞ 0.005).The rats of model group Ⅱ,Ⅳ,Ⅴ were all attacked completely and presented buckling state.There was no onset or death in blank group and control group.The number of CRH positive cells and CRH mRNA relative expression of each model group were higher than those in the blank group and control group.The number of CRH positive cells and CRH mRNA expression of model group Ⅰ and Ⅲ were lower than those in model group Ⅱ,Ⅳand Ⅴ,and the differences were significant (all P ＜ 0.002 4).There was no significant difference in the number of CRH-positive cells(39.12 ± 5.98 vs.41.48 ± 7.61) and CRH mRNA relative expression (1.92 ± 0.16 vs.2.06 ± 0.39) between model group Ⅰ and Ⅲ (all P ＞ 0.002 4).No significant difference was found between blank group and control group,or among model group Ⅱ,Ⅳ and Ⅴ (all P ＞ 0.002 4).There were no significant differences in the learning capacity among all groups (F =2.196,P ＞ 0.002 4).The correct response rate after 24 hours of the model group was lower than the blank group and control group,and ACTH and methylprednisolone pretreatment did not influence the memorizing capacity (P ＞ 0.002 4).Conclusion The effect of pretreatment of ACTH is similar to that of methylprednisolone in the rat model of IS.

OBJECTIVE: To explore the genetic basis for three children with Menkes disease. METHODS: The patients were subjected to next-generation sequencing (NGS) to detect potential variants of the ATP7A gene. Suspected variants were verified by Sanger sequencing of their family members and 200 healthy individuals. Multiplex ligation-dependent probe amplification (MLPA) was also carried out to detect potential deletions in their family members and 20 healthy individuals. RESULTS: Variants of the ATP7A gene were detected in all of the three families, including a novel c.1465A>T nonsense variant in family 1, a novel c.3039_3043del frame-shifting variant in family 2, and deletion of exons 3 to 23 in family 3, which was reported previously. Based on the standards and guidelines of American College of Medical Genetics and Genomics, the c.1465A>T and c.3039_3043del variants of ATP7A gene were predicted to be likely pathogenic (PVS1+PM2). CONCLUSION Variants of the ATP7A gene may underlay the Menkes disease in the three children. Above findings have facilitated clinical diagnosis and enriched the spectrum of genetic variants of Menkes disease.
Case-Control Studies ; Child ; Copper-Transporting ATPases/genetics* ; Exons ; Family Health ; High-Throughput Nucleotide Sequencing ; Humans ; Menkes Kinky Hair Syndrome/genetics* ; Mutation ; Pedigree

Objective To evaluate the clinical application value of multi-slice helical CT volumetric (VH) scanning in lumber spine. Methods One thousand of patients with back and leg pain who underwent CT examinations were selected as subjects. We simulated the traditional protocol of single-slice(SS) discrete scanning for L3/4, L4/5, and LS/S1 intervertebral discs. The VH scanning mode was performed with 120 kV, 210 mAs,pitch of 1.5 and coverage of 97. 5 mm. The simulated SS scanning mode was performed with 120 kV, 240 mAs and coverage of 45.0 mm. The diagnostic outcomes and the radiation doses were compared between the two scanning modes. Two groups doctors observed ten terms, including the osseous spinal stenosis,narrowed intervertebral space and so on in two scanning modes respectively. Then consistency analysis of the data was carried out. Results The VH scanning mode showed far more features than the SS mode. The detection rates of the VH mode in the osseous spinal stenosis, narrowed intervertebral space,herniated nucleus pulposus, narrowed lateral recess, vertebral lesion, hypertrophy of L5 transverse process,abnormal direction of facet, facet degeneration, lumbar spondyloschisis, and paraspinal soft tissue were 11.8% (n =118), 38. 5% (n =385), 9. 3% (n =93), 46. 8% (n =468), 31.4% (n =314), 5.7% (n =57), 25.4% (n = 254), 49. 7% (n = 497), 9.9% (n = 99), and 0. 6% (n = 6) respectively, while the detection rates of the SS mode in ten terms were 5.6% (n = 56), 0, 0. 6% (n = 6), 27. 9% (n = 279),22.4% (n =224), 1.2% (n = 12), 16.7% (n = 167), 37.2% (n =372), 0.5% (n =5), and 0.2%(n = 2) respectively. The difference between the two groups had statistically significance (average P <0.05), except the paraspinal soft tissue abnormal (P > 0.05). The detection rates of the VH mode were higher than the SS mode in the osseous spinal stenosis, narrowed intervertebral space, herniated nucleus pulposus, lumbar spondyloschisis, being 6.2% (n = 62) , 38. 5% (n = 385) , 8.7% (n = 87), and 9.4%(n =94), respectively. In addition, VH mode only partially showed the articular facets, narrowed lateral recess, hypertrophy of L.5 transverse process, and paraspinal soft tissue. We could not acquire the imaging slices paralleling to intervertebral discs in SS mode in 467 patients (46.7%) with lumbosacral angle greater than 35°. The radiation dose of VH mode (164.9 mGy/em) was slightly higher than SS mode (147.0 mGy/cm) Conclusion MSCT VH scanning mode can significantly improve the diagnostic rate of lumbar spine diseases compared with SS mode, and was not restricted by the lumbosacral angle with slightly increasing radiation dosage.