A 4-year-old girl presented with generalized papules, erythema, desquamation, subcuta-neousnodules around large joints, as well as a 3-year-history of progressive eye damage. Three months after her birth, papules developed on the lower limbs, and gradually spread to involve the whole body. Eye damage appeared after a high fever and she was diagnosed with keratoleukoma, old iritis, and posterior synechia. Physical examination revealed sharply demarcated corneal opacity, shallow anterior chamber, obscure irides, and posterior synechia; pupils were not round. No superficial lymphadenectasis was observed. Dermatological examination showed ichthyosis-like coarse skin with generalized papules, erythema and desquamation, increased wrinkles and absence of sweat. Skin temperature was higher than normal. There were soft, irregu-larly sized, non-tender, movable subcutaneous masses at the wrist, ankle and knee joints without inflamma-tion or warmth. Type-B uitrasonography of both eyes disclosed vitreous opacification, posterior detachment of vitreous, extravasation at the posterior vitreous membrane, and decreased intraocular pressure. Histopathology of lesions on the back, waist, and back of hands as well as subcutaneous nodules on the wrists showed epidermal acanthosis and infiltration with a large number of histiocytes, foam cells and multi-nucleated giant cells in the dermis. No abnormal findings were observed from other examinations. This case was diagnosed as Blau syndrome, which is an autosomal dominant inherited disease; currently, there is no effective therapy for it.

Objective To explore the application of Propensity Score Matching Methods in medical scientific research,and demonstrate the operation process of SPSS.Methods This article summarized and discussed the principle and application of PSM through the literature,a real example was adopted to introduce the operation procedures of SPSS.Results We can use PSM process to control confounding factors between the retrospective study groups effectively through SPSS.Conclusions PSM is an efficient tool to conduct retrospective study.It can provide necessary help for clinical data mining.

Adult ; Aged ; Aged, 80 and over ; Cardia ; Enteral Nutrition ; Esophageal Neoplasms ; surgery ; Female ; Humans ; Intubation, Gastrointestinal ; Male ; Middle Aged ; Postoperative Period ; Stomach Neoplasms ; surgery ; Time Factors

Objective To investigate the risk factors on children with coronary artery lesions (CAL) secondary to Kawasaki di-sease(KD). Methods One hundred and forty - five patients with KD from January 1999 to December 2001 were collected. Among them, 93 cases without CAL and 52 cases with CAL. The test results, therapeutic methods and prognosis were analyzed in two groups. Results The duration of fever was longer and mean value of erythrocyte sedimentation rate (ESR) higher in group with CAL than those m the group without CAL(P0.05).The incidence of CAL was 18.8% (18/96) in patients of IVIG treatment within 10 days from onset, and 69.4% (34/49) in patients without IVIG treatment (P

Objective To investigate the inhibitory effect of earthworm fibrinolytic enzyme (EFE) on the metastasis of human hepatoma cells.Methods A metastatic model of human hepatocellular carcinoma in nude mice via orthotopic implantation was established.After the modeling for 7 days,the mice were divided randomly into the control group,low-dose EFE group (800 uku/kg?d-1),and high-dose EFE group (1600 uku/kg?d-1).After administration for 30 days,the implanted tumors were weighted,and the intrahepatic transmission rate and abdominal cavity seeding rate were calculated after examination by naked eyes.Then the pulmonary metastasis were examined under microscope,and the expression of focus adhesion kinase (FAK) and ?1-intigrin were detected through RT-PCR and western blotting method.Results Compared with the model control group,the mean weight of the orthotopic tumor was reduced,and the intrahepatic transmission rate and abdominal cavity seeding rate were decreased in FEF groups (P

Female ; Hemifacial Spasm ; etiology ; Humans ; Male ; Mastoiditis ; complications ; Middle Aged

Adult ; Aged ; Disulfiram ; adverse effects ; Drug-Related Side Effects and Adverse Reactions ; Female ; Humans ; Male

Objective To study the mechanism of effects of estrogen receptor (ER) on T and B lymphocytes in patients with SLE and synergistic effect of T and B lymphocytes in the pathogenesis of SLE.Methods Real-time fluorescence quantitative PCR was performed to detect the mRNA expressions of ER-α,interleukin 10 (IL-10) and B lymphocyte stimulator (BLyS) in peripheral blood mononuclear cells (PBMCs)from 40 SLE patients and 40 normal human controls. The clinical and laboratory correlation with the levels of these parameters was analyzed. Results A significant increase was observed in the relative expression levels of ER-α, IL-10 and BLyS mRNA in SLE patients compared with the normal human controls (P ＜ 0.05 or 0.01 ), in active SLE patients compared with inactive SLE patients (P ＜ 0.05 or 0.01 ). Additionally, the mRNA expression levels of the 3 parameters were significantly correlated with the presence of renal damage, proteinuria, arthritis, etc. No statistical difference was observed in the mRNA expression levels of these parameters between female and male patients or between female and male normal controls. Conclusions IL-10 and BLyS appear to be correlated with the disease activity and severity of SLE, and ER-α may play an important role in the action mechanism of T and B lymphocytes in SLE.

Objective To evaluate the regulatory role of mTOR signaling in activation of renal interstitial fibroblasts and the potential effect on interstitial fibrosis. Methods 8-week old female C57BL/6 mice (n=30) underwent unilateral ureteral obstruction (UUO) to induce renal interstitial fibrosis. Animals were randomly divided into rapamycin (2 mg·kg-1· d-1) group and UUO group (vehicle-treated) (n=15 each group). Daily intraperitoneal injection of rapamycin or saline was applied to animals from day 1 before operation to the end of experiment.Three mice were sacrificed at day 1,3,7,14 respectively and kidneys were harvested for further analysis.NIH3T3 cells were stimulated by TGF-β for 12 hours with the presence or bsence of rapamycin (100 nmol/L). Results Immunofluorescent co-staining revealed that active fibroblasts highly expressed pS6K and α-SMA in kidney interstitium.Administation of rapamycin significantly inhibited activation of mTOR signaling in fibroblasts and ameliorated interstitial fibrosis of obstructed kidneys.Real-time PCR confirmed that rapamycin decreased the mRNA expression of FSP1,TGF-β,CTGF and Col4A1 in fibrotic kidneys. In vitro experiment revealed that TGF-β induced highly expression of pS6K and αSMA in cultured NIH3T3 cells,which could be markedly inhibited by rapamycin. Conclusions mTOR signaling highly activates in interstitial fibroblasts during kidney fibrosis.Inhibition of mTOR signaling by rapamycin decreases the activation of fibroblasts and ameliorates interstitial fibrosis.