Background Researches showed that multifocal electroretinogram (mfERG) is able to assess the retinal function in the eyes with acute Vogt-Koyanagi-Harada ( VKH ) syndrome. But the mfERG characteristics of convalescence stage of VKH are still below clear. Objective Present study was to compare and follow up the variation process of visual acuity and mfERG in acute and recovery stages of VKH syndrome. Methods This was a clinic-based retrospective study. Visual acuity, mfERG and fundus fluorescence angiography ( FFA ) were recorded from 35 eyes of 18 acute VKH cases. The period of follow-up in recovery stage lasted about 18 months with the repetitive recording results for 4 times. Results In this study, the visual acuity range in acute stage VKH was 0. 01 to 1.0, and 91.4% (32/35 eyes) was below 0.6. Compared with normal control group, the visual acuity was significantly decreased (P＜0.01). The response densities (amplitudes) of N1 ,P1 waves of the first-order kernel were significantly lowed in all the 6 rings,and the implicit times of 1-4 rings of both waves were significantly prolonged in acute VKH eyes(P＜0. 05). The abnormalities of retinal function showed a regional difference at the posterior pole retina with the dominant change in the first ring,showing a cutting off78% in the P1 amplitude. The abnormal degree of mfERG was more serious as the the increase of retinal eccentricity. In 2 months of convalescence after glucocorticosteroids therapy,the range of visual acuity were 0. 1-1.2 ,and the amplitudes of N1, P1 of 1-2 rings were greatly elevated in comparison with acute on-set (P＜0. 05 ). However, there was still a remarkable difference in the amplitudes of from 1 through 6 rings,comparing with normal. The response density of P1 wave from whole recording region was only 44% of normal. Though the visual acuity was stable during the follow-up duration, a decreasing tendency in N1 and P1 amplitudes were seen. The implicit times of both wave shortened only in 1-3 rings in recovery stages of VKH (P＜0.05). Conclusion VKH syndrome cause serious damage of posterior retinal function.Macular region is the site with greater retinal functional lesion and restore before and after medication. This hardly recovery of retinal function can last over one and half year,even satisfied visual acuity is stable after proper treatment.

Objective:To observe the protective effect of acupuncture plus mild hypothermia on brain tissues in rats with cerebral ischemia-reperfusion injury (CIRI), and the influence on protein expression levels of phosphorylated Raf-1, MEK-2 and ERK1/2 in the mitogen-activated protein kinase (MAPK)/extracellular regulated protein kinases (ERK) pathway, and to explore the mechanism of acupuncture plus mild hypothermia therapy for the ischemic stroke. Methods:Ninety Sprague-Dawley (SD) rats were randomly divided into a blank control group, a sham operation group, a model group, an acupuncture group, a mild hypothermia group and an acupuncture plus mild hypothermia group, 15 rats in each group. Except the rats in the blank control group, the remaining rats were used to prepare the middle cerebral artery occlusion (MCAO) models according to the modified occlusion method using lines, while only the occlusion lines were inserted without blocking the brain arteries of rats in the sham operation group. When the vital signs of rats were stable, rats in the blank control group did not receive any intervention; rats in the sham operation group and the model group received fastening without treatment; rats in the acupuncture group, the mild hypothermia group, and the acupuncture plus mild hypothermia group were treated with the corresponding therapeutic methods. 72 h later, observed neurologic injury score, evaluated infarction area ratio by 2,3,5-tripheyl tetrazolium chloride (TTC) staining, determined apoptosis by TUNEL assay, and measured the phosphorylated Raf-1, MEK-2 and ERK1/2 protein expression levels in rat ischemic hippocampal tissues by Western blot assay. Results: Compared with the blank control group and the sham operation group, after modeling, the neurologic injury score, infarction area ratio and apoptotic cells were increased, and phosphorylated Raf-1, MEK-2 and ERK1/2 protein expression levels were significantly increased in the model group; the differences were statistically significant (P<0.05 orP<0.01). Compared with the model group, after acupuncture or mild hypothermia therapy, neurologic injury score and infarction area ratio were decreased; apoptotic cells and phosphorylated Raf-1, MEK-2 and ERK1/2 protein expression levels were significantly decreased; the differences were statistically significant (P<0.05 orP<0.01). Compared with the acupuncture group, neurologic injury score and phosphorylated Raf-1, MEK-2 and ERK1/2 protein expression levels were decreased in the acupuncture plus mild hypothermia group; differences between the groups were statistically significant (P<0.05 or P<0.01). Compared with the mild hypothermia group, phosphorylated Raf-1, MEK-2 and ERK1/2 protein expression levels decreased in the acupuncture plus mild hypothermia group, and differences were statistically significant (P<0.01). Conclusion:Acupuncture or mild hypothermia therapy can improve neurologic injury, reduce infarction area and apoptosis, which brought about protective effect on the brain tissues, in the MCAO model. The protective effect of acupuncture plus mild hypothermia group is the strongest. The mechanism may involve the MAPK/ERK pathway, by reducing the phosphorylated Raf-1, MEK-2 and ERK1/2 protein expression levels.

Objective To investigate the clinical and pathological features of Fabry disease in Chinese patients. Methods This retrospective study included all in-patients Fabry disease patients at PUMC Hospital from 2004 to 2014.Results The 12 patients included 10 males and 2 females.The middle age at onset was 10.The most fre-quent symptoms in our group were pain(10/12), angiokeratoma(9/12), renal abnormalities (7/12), hearing loss ( 6/12 ) , hypohidrosis ( 5/12 ) , cardiac abnormalities ( 5/12 ) , cerebrovascular abnormalities ( 3/12 ) and ophthalmological abnormalities ( 3/12 ) .The severe clinical manifestations were more frequently found in males . Electron microscopy plays a very important role in pathological diagnosis of Fabry disease .7 patients were suspec-ted to have family history .5 patients were examined regularly in which 4 cases were stable , and 1 patient re-ceived hemodialysis after 3 years'follow-up.Conclusions Onset of Fabry disease is early, many organs could be involved and patients may have different clinical manifestations.There are great differences in clinical features be-tween male and female patients .

Objective: To investigate the effect of acupuncture plus mild hypothermia on neurological function impairment score, cerebral infarct size and apoptosis-related factors in cerebral ischemia reperfusion injury (CIRI) rats.Methods: Sixty healthy male Sprague-Dawley (SD) rats were routinely reared for 1 week. Ten rats were randomly selected as the sham operation group and 10 rats as the blank control group, while the remaining 40 rats were subjected to preparing the middle cerebral artery occlusion (MCAO) model by modified filament occlusion method. The 40 MCAO rats were further randomly divided into a model group, an acupuncture group, a mild hypothermia group and an acupuncture plus mild hypothermia group, with 10 rats in each group. Rats in the sham operation group, the blank control group and the model group did not accept treatment except binding; rats in the acupuncture group received acupuncture treatment; rats in the mild hypothermia group received mild hypothermia treatment; rats in the acupuncture plus mild hypothermia group received acupuncture and mild hypothermia treatment. 72 h after the treatment, neurological function impairment score was performed; the infarct area ratio was determined by 2,3,5-tripheyl tetrazolium chloride (TTC) staining; apoptosis of brain cells was observed by TUNEL method; the expressions of Bcl-2, Bax and Caspase-3 were detected by immunohistochemistry.Results: Compared with the blank control group and the sham operation group, the neurological function impairment score, cerebral infarct area ratio, apoptosis, and the expressions of Bax and Caspase-3 in the model group were significantly increased, while the expression of Bcl-2 was significantly decreased, and there were significant between-group differences (allP<0.05). After the treatment, there were statistically significant differences among the treatment groups in the neurological function impairment score, cerebral infarct area ratio and apoptosis in the ischemic side of rats, as well as the expressions of Bcl-2, Bax and Caspase-3 (allP<0.05), and from the figures, tables and statistical analysis, it was found that a better tendency in the acupuncture plus mild hypothermia group than the acupuncture group or mild hypothermia group.Conclusion: Acupuncture plus mild hypothermia can protect the brain cells by improving neurological function impairment, decreasing cerebral infarct area ratio, reducing the number of apoptotic cells in the ischemic area and regulating the expressions of apoptosis related proteins to inhibit apoptosis.

Objective:To observe effect of acupuncture combined with hypothermia therapy on MAPK/ERK pathway and apoptosis related factorsin rats suffered cerebral ischemia reperfusion and to explore underlying mechanisms.Methods:Middle cerebral artery ischemia model were established.Ninety SD rats were randomly assigned into a blank group,a control group,a model group,an acupuncture group,a mild hypothermia group,and an acupuncture with hypothermia group.After 72 h treatment,nervefunction defect scores were observed,and infarction area percent was detected by 2,3,5-triphenyl-2H-tetrazolium chloride (TTC) staining;expressions of Bcl-2 and Bax were examined by immunohistochemistry;apoptotic cells were detected by TUNEL assay;and expression levels of phospho-mitogen-activated protein kinase(p-MEK2) and phospho-extracellular signal regulated kinase 1/2 (p-ERK1/2) in the rats' hippocampus ischemic side were determined by Western blot.Results:In the rats of the model group,the neural function defect scores,the infarction area percent,the expression level of Bax,and apoptotic cells increased,while the level of Bcl-2 decreased significantly.The level of p-MEK2 and p-ERK1/2 increased obviously compared with the blank and control groups (P＜0.05 or P＜0.01).After treatment with acupuncture and hypothermia,the neural function defect scores,infarction area percent,and the level ofBax,apoptotic cells and the levels of p-MEK2 and p-ERK1/2 were significantly decreased,while the level of Bcl-2 in the treatment group was significantly elevated (P＜0.05 or P＜0.01) compared with the model group.Compared with the acupuncture group or the hypothermia group,the neural function defect scores and the levels of p-MEK2 and p-ERK1/2 in the acupuncture combined with hypothermia group were significantly reduced (P＜0.05 or P＜0.01).Conclusion:Acupuncture and hypothermia therapy can improve cerebral function,and reduce the cerebral injury through down-regulation of Bax level,and up-regulation of Bcl-2 level,which is related to reducing the levels of p-MEK2 and p-ERK1/2.The therapeutic effects on cerebral ischemia reperfusion injury for combination of acupuncture with hypothermia are better than those with single application of acupuncture or hypothermia.

Objective To investigate the presence of occult brain tissue damage in patients with relapsing neuromyelitis optica(RNMO)and its possible mechanism by using diffusion tensor imaging (DTI).Methods DTI scans were performed in 16 patients with RNMO and 16 sex-and age-matched healthy controls.Histogram analysis of mean diffusivity (MD)and fractional anisotropy (FA)was performed in brain tissue (BT),white matter (WM)and gray matter (GM)to detect the presence of occult brain tissue damage in RNMO patients.Region of interest(ROI )analysis of MD and FA was also performed in 6 dedicated regions with or without direct connection with spinal cord or optic nerve to determine the relationship between occult brain tissue damage and the damage of spinal cord and optic nerve.Results Patients with RNMO had a significantly higher average MD of the BT[RNMO(0.95?0.02)? 10~(-3)mm~2/s,controls (0.91?0.03)?10~(-3)mm~2/s,t = 3.940,P

Objectives To summarize the characteristics,differential diagnosis and management of incomplete 17 alpha-hydroxylase/17,20-1yase deficiency(17 OHD)of Chinese patients.Methods Six cases of incomplete 17 OHD from Peking Union Medical College Hospital were studied retrospectively through analyzing their clinical data,and the molecular pathogenic mechanism was discussed after literature review.Results Four cases of 46,XX incomplete 17 OHD were reported.The clinical characteristics included female phenotype,various degrees of breast development and absent or sparse axillary/pubic hair, oligomenorrhea or secondary amenorrhea,recurrent luteinized ovarian cysts,hypogonadism with persistent hyperprogesteronemia or high serum 17 alpha-hydroxyprogesterone level,with or without hypokalemic hypertension.There were also 2 cases of 46,XY incomplete 17 OHD,in which ambiguous genitalia were present besides hypokalemic hypertension.Conclusions Incomplete 17 OHD is a very rare form of congenital enzymatic deficiencies of steroid synthesis,which should be included in the differential diagnosis when there are menstrual disorders,sexual infantilism,recurrent ovarian cysts or ambiguous genitalia.Under such circumstances,hyperprogesteronemia offers a valuable clue for further investigation.

OBJECTIVETo summarize clinical outcomes of locking compression plate (LCP) combined with minimally invasive percutaneous plate osteosynthesis (MIPPO) for the treatment of Pilon fracture.

METHODSFrom January 2009 to December 2012, Pilon fracture patients treated by LCP with MIPPO were retrospectively analyzed. All open fractures, pathologic fractures and those who had limb vascular disease or nerve injury were excluded. Thirty-eight patients were enrolled, including 29 males and 9 females aged from 21 to 78 years old with an average of 48 years old. According to AO classification, 20 cases were type B, 18 cases were type C. Operative time, blood loss, reduction quality, time of fracture healing complications and postoperative ankle joint function were applied for evaluating clinical outcomes, AOFAS scoring were used for assessing postoperative clinical effects.

RESULTSAll patients were followed up from 13 to 24 months (averaged 18 months). All patients obtained bone union without any plate failures or loss of fixation/reduction. One patient occurred superficial wound infection, and resolved with antibiotics and local wound care. Postoperative average AOFAS score was 81 (ranged 65 to 97).

CONCLUSIONLCP with MIPPO for Pilon fratcure has advantages of less invasion, fewer complications and satisfactory ankle function.

Adult ; Aged ; Bone Plates ; Female ; Fracture Fixation, Internal ; methods ; Humans ; Male ; Middle Aged ; Minimally Invasive Surgical Procedures ; methods ; Retrospective Studies ; Tibial Fractures ; surgery

OBJECTIVETo study the effect of Fuzheng Sanjie recipe in regulating tumor-associated macrophages (TAMs) in Lewis lung cancer mice.

METHODEfforts were made to establish the Lewis lung cancer mouse model, weigh tumors and calculate the anti-tumor rate. The immunohistochemical method was used to examine the infiltration degree of CD68 + in tumor tissues in each group. ELISA was used to examine the content of IFN-γ, TGF-β, IL-4, IL-13, IL-6, IL-10, IL-12, TNF-α in mice serum.

RESULTCompared with the tumor-bearing model group, all of the other groups showed higher tumor inhibition rates, i. e. 50.28% for the DDP group, 34.37% for the TCM-preventing group and 66.76% for the Chinese and western medicine group, with statistical difference (P < 0.05), but without statistical difference in the infiltration degree of CD68+. The expressions of the IFN-γ, IL-6, IL-12 in tumor-bearing groups were lower than that in the blank control group, but with higher contents of IL-4, IL-13, TGF-β. Intervened with different drugs, there were significant differences in content among some relevant cytokines (P < 0.05), as well as statistical differences among the TCM prevention group, the Chinese and western medicine group and the tumor-bearing control group (P <0. 05) , but without statistical difference in TNF-α and IL-10 content from the tumor-bearing control group (P < 0.05).

CONCLUSIONFuzheng Sanjie recipe could reverse the immune remodeling effect and control the tumor growth by down-regulating the expressions of IL-4, IL-13, TGF-α in lung cancer immune microenvironment and up-regulating the expression of IFN-γ.

Animals ; Cell Line, Tumor ; Disease Models, Animal ; Drugs, Chinese Herbal ; administration & dosage ; Humans ; Interleukin-10 ; blood ; Interleukin-12 ; blood ; Interleukin-13 ; blood ; Lung Neoplasms ; blood ; drug therapy ; immunology ; Macrophages ; drug effects ; immunology ; Male ; Mice ; Mice, Inbred C57BL ; Transforming Growth Factor beta ; blood ; Tumor Necrosis Factor-alpha ; blood

OBJECTIVETo observe and compare the quality of life (QOL) and survival time in patients with advanced primary hepatic cancer (PHC) after they have been treated by the combination of ganji recipe and interventional therapy with Fructus Bruceae Oil Emulsion (FBE) or by the trans-hepatic arterial chemical embolization (TACE) adopting Seldinger's technique.

METHODSSeventy-seven patients with advanced PHC were randomly assigned to two groups, 37 patients in the control group treated with TACE alone, and 40 in the treatment group with the combined therapy. One therapeutic course was 4 weeks, and the intervention therapy was repeated generally after an interval of 4-6 weeks according to patients'condition ,the average intervention frequency was 2.3 for one case. The QOL and the survival time in patients were evaluated after treatment.

RESULTSSignificant difference was shown in the changes of the Karnofsky score between the two groups (P < 0.05). The scores suggested that the improvements in the treatment group, including patients'physical energy enhancing, symptoms alleviating and overall QOL improving, were superior to those in the control group (P < 0.05). No significant difference of the 3-month survival rate between the two groups was found (P > 0.05), but the 0.5- and 1-year survival rate were significantly superior in the treatment group to those in the control group (67.6% vs. 42.4% and 38.2% vs. 16.1%, respectively). The median survival time in the two groups was 8.9 and 5.3 months respectively.

CONCLUSIONCombination of ganji recipe and interventional therapy with FBE in treating PHC could improve patients' QOL, raise the 0.5- and 1-year survival rate, and prolong the survival time, suggesting TCM treatment has an affirmative significance for enhancing the therapeutic efficacy and improving the prognosis in patients.

Adult ; Aged ; Brucea ; chemistry ; Carcinoma, Hepatocellular ; therapy ; Drugs, Chinese Herbal ; therapeutic use ; Embolization, Therapeutic ; methods ; Female ; Humans ; Liver Neoplasms ; therapy ; Male ; Middle Aged ; Phytotherapy ; Plant Oils ; administration & dosage ; Quality of Life ; Seeds ; chemistry