BackgroundProliferative vitreo-retinal disease (PVD)is one group of ocular complications marked by the enhanced proliferation of various cells included retinal pigment epithelial (RPE) cells.Insulin-like growth factor-1 (IGF-1)and vascular endothelial growth factor(VEGF) are implicated in the aberrant cell proliferation and pathological neovascularization that characterizes PVD,but the signaling mechanism is unclear now. Objective This study was to explore the effect of IGF-1 on VEGF in cultured human RPE cells under the small hairpin loop RNA (shRNA) keeping hypoxia-inducible factor-1 α ( HIF-1 α) silencing. Methods Human retinas were isolated from 4 healthy male donors,and the RPE cells were harvested and cultured.The ceils were identified using anti-human keratin antibody.The third to fifth generation of human RPE cells were used in the experiment.One target site of HIF-1α mRNA was chosen by certain design principle,and shRNA was designed and synthesized by the target site and transferred into the cells in vitro,and then the cells were cultivated with 50 μg/L IGF-1 for 24 hours.The mRNA and protein expressions of HIF-1α and VEGF were detected by RT-PCR and Western blot respectively. Results Cultured human RPE cells showed the flat irregularly multangular shape,and 97％cells appeared the positive response for keratin.HIF-1α mRNA expression in human RPE cells was significantly lower in 50 μg/L IGF-1 group than the 0 pg/L IGF-1 group ( 1.49±0.18 vs 1.46±0.17 ) ( t =0.335,P =0.743 ),however,the expressing levels of HIF-1α protein( 1049.86±172.54 vs 0.00±0.00) and VEGF mRNA(0.95±0.15 vs 0.35±0.07) and VEGF protein (391.98±56.77 vs 214.36±37.15)were raised in the 50 μg/L IGF-I group compared with 0 μg/L IGF-1 group (t=16.098,9.935,6.928,P＜0.05).After the HIF-1α-specific shRNA was transferred into cultured RPE cells,the expressions of both HIF-1α mRNA and its protein significantly decreased in RPE cells under 50 μg/L IGF-1 concentration condition( F=68.679,89.904,P=0.000),moreover,the expression of VEGF mRNA and its protein were significantly lowed(F=21.770,6.205,P＜0.05). ConclusionsIGF-1 promotes the accumulation of HIF-1α protein and induce the expression of VEGF in human RPE cells,which probably play a pivotal role in the development of PVD.

Objective To investigate the clinical manifestations of patients with pulmonary artery hypertension (PAH) associated with hereditary hemorrhagic telangiectasia (HHT). Methods This retrospective analysis summarized the clinical features of 6 patients with PAH associated with HHT hospitalized at department of cardiology in Cardiovascular Institute and Fuwai Hospital between January 2006 and May 2009. Results The mean age of the 6 patients (3 male) was 34 years (8 -67years). Recurrent epistaxis were present in all patients, there were 4 patients with severe PAH and 2 patients with moderate PAH. All of the six patients with PAH associated with HHT were misdiagnosed at the first hospital visit.Clinical symptoms were significantly improved in 4 patients and remained unchanged in 2 patients combined hepatic venous malformation post medical therapy. Conclusions Misdiagnosis for patients with PAH associated with HHT is a common phenomenon in daily clinical practice. Patients could benefit from the corresponding medical therapy after the establishment of the correct diagnosis.

Objective To report a new approach,endoscopic transoral approach for the resection of jugular foramen schwannoma.Methods Nine patients with jugular foramen schwannoma ( three males and six females,ranging in age from 15 to 61 years old ) were treated by direct surgery via a pure endoscopic transoral approach to the jugular foramen. Eight patients complained of hypoglossal nerve palsy with hemiatrophy of the tongue; six cases complained of vagus nerve palsy. Three cases complained of glossopharyngeal nerve palsy,one case complained of facial nerve palsy and hearing loss.Results The nerves in this area were preserved and radical intracapsular removal of the tumor was performed via endoscopic transoral approach in the nine cases.Tumor removal,as assessed by intraoperative endoscopic inspection,postoperative magnetic resonance imaging and clinical evaluation,revealed all tumors were completely removed.One patient suffered from temporary swallowing difficulties and temporary right vagus palsy Ⅰ day after surgery.There were no others intraoperative and postoperative complications.All patients were followed up for 4 -29 months,no recurrences were occured in all these patients and the muscle bulk,motor and the pre-postoperative swallowing fuction,the vagus palsy,the facial nerve palsy and hearing loss had improved in these patients.Conclusion The endoscopic transoral approach and intracapsular removal of the tumor provided for successful minimally invasive surgery in the jugular foramen schwannomas.

Objective To analyze the diagnostic feature, treatment and prognosis of patients with Cantrell syndrome. Methods Clinical manifestation, diagnosis, operation and follow-up data of 5 patients with Cantrell syndrome were summarized in this retrospective analysis. Results The age of the 5 patients was 7 days-76 years, definite diagnosis was made in 3 cases and 2 cases presented feature of incomplete Cantrell syndrome. Three patients with full Cantrell syndrome were correctly diagnosed before operation and confirmed by operation. One patient with incomplete Cantrell syndrome (two-vessel stenosis ) received bypass surgery. Another asymptomatic patient with incomplete Cantrell syndrome ( apical diverticulum of the left ventricle) does not need operation and is under observation. During follow-up, 1 patient died at 60months after operation and the remaining 4 patients are alive and well. Conclusions With the development of modern imaging technology, it becomes easy to make correct diagnose Cantrell syndrome before operation.Prognosis is fine post timely operation and related intervention.

Objective To analyze the status of quality indicators(QI) on specimen acceptability and establish preliminary qual ity specification.Methods Web based External Quality Assessment system was used to collect data of laboratories partici pated in "Medical quality control indicators in clinical laboratory" from 2015 to 2017,including once in 2015 and 2017 and twice in 2016.Rate and sigma scales were used to evaluate incorrect sample type,incorrect sample container,incorrect fill level and anticoagulant sample clotted.The 25th percentile (P25) and 75th percentile (P75) of the distribution of each QI were employed to establish the high,medium and low specification.Results 5 346,7 593,5 950 and 6 874 laboratories sub mitted the survey results respectively.The P50 of biochemistry (except incorrect fill level),immunology and microbiology reach to 6σ.The P50 of clinical laboratory is 4 to 6σ except for incorrect sample container.There is no significant change of the continuous survey results.Based on results in 2017 to establish the quality specification,the P25 and P75 of the four QIs is 0 and 0.084 4 ％,0 and 0.047 6 ％,0 and 0.114 2 ％,0 and 0.078 4 ％,respectively.Conclusion According to the results of the survey,most laboratories had a faire performance in biochemistry,immunology and microbiology,and clinical laboratory needs to be strengthened.Laboratories should strengthen the laboratory information system construction to ensure the actual and reliable data collection,and make a long time monitoring to achieve a better quality.