ABCB11 gene encodes bile salt export pump (BSEP).It is almost exclusively expressed in the canalicular microvilli of liver.It is the principal conveyor of bile acids from hepatocyte cytoplasm into bile canaliculus.It is clearly that BSEP defects can induce progressive familial intrahepatic cholestasis type 2 and benign recurrent intrahepatic cholestasis type 2.ABCB11 gene variation are also responsible for intrahepatic cholestasis of pregnancy,drug-induced cholestasis,primary sclerosis cholangitis and primary bile cirrhosis.This paper reviewed the association of ABCB11 gene variation and these diseases.

CD36 Antigens ; metabolism ; Cell Aggregation ; Cholesterol ; blood ; Foam Cells ; pathology ; Glomerulonephritis, IGA ; blood ; metabolism ; pathology ; Glomerulonephritis, Membranoproliferative ; blood ; metabolism ; pathology ; Glomerulonephritis, Membranous ; blood ; metabolism ; pathology ; Glomerulosclerosis, Focal Segmental ; blood ; metabolism ; pathology ; Humans ; Nephritis ; blood ; metabolism ; pathology ; Nephritis, Hereditary ; blood ; metabolism ; pathology

Objective To observe the effects of thin recipe ofBuyang Huanwu Decoction on expressions of glutathione (GSH) antioxidant system including GSH, GSH-Px andγ-GCS in the brain of focal cerebral ischemia rats;To study its mechanism of antioxidant effect.Methods Totally 120 SD rats were randomly divided into four groups according to random number table method:sham-operation group, model group,Buyang Huanwu Decoction group and thin recipe ofBuyang Huanwu Decoction group. Except for the sham-operation group, the rest groups established focal cerebral ischemia rat model by middle cerebral arterial occlusion. The treatment groups were given corresponding medicine by gavage, while the sham-operation group and model group were given the same amount of normal saline 2 h after modeling. Each group was detected after cerebral ischemia for 1 day, 3 days, and 7 days respectively. The content of GSH and the activity of GSH-Px were detected. At the same time,γ-GCS mRNA and protein levels were determined by using real time-PCR and Western blot.Results The content of GSH and the activity of GSH-Px at each time point decreased in the model group (P<0.05), compared with the corresponding sham-operation group. But the expression ofγ-GCS mRNA and protein increased, with statistical significance on day 1 (P<0.05). Compared with model group, content of GSH and GSH-Px activity levels increased differently in each treatment group, whileγ-GCS mRNA and protein expression raised, with statistical significance on day 3 (P<0.05).Conclusion The thin recipe ofBuyang Huanwu Decoction plays antioxidant effect by regulating the expression of glutathione antioxidant system GSH, GSH-Px andγ-GCS after cerebral ischemia, which may be one of its therapeutic mechanisms for cerebral ischemia.

BACKGROUND:Correlation between subchondral bone and articular cartilage in the process of osteoarthritis has not been fuly elucidated. Degeneration of cartilage is the focus of attention, and the subchondral bone also plays an important role in the process of osteoarthritis. OBJECTIVE: To observe the differences between experimental osteoarthritis models in rabbit knees established by two kinds of surgical methods and two kinds of proteases inducing methods, and to explore the correlation between subchondral bone mass and degeneration of cartilage. METHODS:Thirty-two New Zealand rabbits were randomly and averagely divided into four groups: Hulth group (group A), anterior cruciate ligament transaction group (group B), colagenase type II group (group C) and papain group (group D). The right knees of rabbits were established as osteoarthritis models, and the left knees served as controls. Bone mineral density of the knee joint was evaluated by dual-energy X-ray absorptiometry scanning at 0, 4 and 8 weeks after modeling. The rabbits were sacrificed at 8 weeks after MRI scanning, bilateral knee joints were harvested for general and histological observation. Quantitative analysis was done according to Mankin scores. RESULTS AND CONCLUSION: Bone mineral density of the right knees decreased at 0, 4 and 8 weeks after modeling, and the rank was as folows: group A > group B > group C > group D. MRI scanning showed that the articular cartilage thickness of the medial and lateral femoral condyle on the right knees became thinner compared with the left side, and the rank was as folows: group A < group B < group C < group D. Observation by specimens and pathological slices showed that the articular cartilage degeneration of the surgery groups worsened, group A was the most serious one, and group 1D was the lightest. Both surgery and proteases inducing methods can successfuly establish osteoarthritis models in rabbit knees. Surgery inducing models resemble the advanced or intermediate stage of osteoarthritis, while the proteases inducing models resemble the early stage of osteoarthritis. Degeneration of the articular cartilage and changes of subchondral bone are related in progressive development.

Objective To observe the effect of a traditional Chinese medicine, BuyangHuanwu Decoction, on the ethology in rat models of post-stroke depression ( PSD) .Methods Sixty male SD rats, after open-field ethology scoring, were randomly divided into five groups ( 12 rats in each group):sham operation group, cerebral ischemic stroke group, post-stroke depression ( PSD) group, BuyangHuanwu Decoction group, and fluoxetine group.The rat PSD models were established with separately breeding and chronic unpredictable moderate stress ( CUMS ) after middle cerebral artery occlusion. Behavior changes of the rats were detected by body weight measurement, neurological scoring, sugar consumption test and open-field test.Results Compared with the sham operation group and cerebral ischemic stroke group, the body weight,sugar consumption and open-field spontaneous activity in the PSD model group were decreased, and the neurological function deficits became more severe ( P <0.01 or P <0.05 ) .Compared with the PSD group, Both BuyangHuanwu Decoction group and fluoxetine group got significantly higher scores of body weight,sugar consumption and spontaneous activity,and improved neurological function deficits(P<0.01 or P<0.05).Conclusions PSD rats exhibit abnormal behavior.BuyangHuanwu Decoction can significantly improve the behavioral impairment of PSD rats. The antidepressant effect of BuyangHuanwu Decoction is comparable to that of fluoxetine in the treatment of PSD rats.

Objective:To observe the clinical effect of wheat grain-sized cone moxibustion combined with fire needle therapy in the treatment of dorsal wrist ganglion (DWG). Methods:A total of 63 patients with DWG were randomized into a treatment group (n=32) and a control group (n=31). Cases in the control group were treated with fire needle therapy. Based on the therapy given to the control group, cases in the treatment group were additionally given wheat grain-sized cone moxibustion therapy. Then the therapeutic efficacies and recurrence rate were evaluated. Results:After treatment, the overall effective rate was 93.8% in the treatment group, versus 77.4% in the control group, and the between-group comparison showed a statistical significance (P<0.05); 6 months after the treatment, the recurrence rate in the treatment group was obviously lower than that in the control group (P<0.05). Conclusion:The long-term and short-term effects of wheat grain-sized cone moxibustion combined with fire needle therapy in the treatment of DWG are better than those of fire needle therapy alone, and therefore, worth popularizing.

OBJECTIVETo reassess the diagnostic value of 24 hour urinary copper excretion in children with Wilson disease (WD).

METHODSFrom July 2005 to June 2007, inpatients over three years old in a pediatric liver center were assigned into WD and non-WD group.

RESULTS94 patients, including 26 cases in WD and 68 in non-WD group, were enrolled in this study. The median of 24 h urinary copper excretion was 98.5 microg in WD group and 25.8 microg in the non-WD group (Z = -6.111, P equal to 0.000). The area under receiver operator curve (ROC) was 0.909 (95% CI: 0.839-0.979, P equal to 0.000). The sensitivity, specificity, accuracy, positive predictive value and negative predictive value were 84.6%, 91.2%, 89.4%, 78.6% and 93.9% respectively using 52.0 ug as a cutoff value, and 50.0%, 97.1%, 84.0%, 86.7% and 83.5% using 100 microg as a cutoff value. The goodness of fitness of 52 microg criteria was significantly higher than 100 microg criteria (kappacoefficient 0.760, 0.541 respectively, P equal to 0.000).

CONCLUSIONComparing to 100, 52 microg of 24 h urinary copper excretion as a cutoff value significantly improves the sensitivity and accuracy for diagnosing WD in children.

Adolescent ; Age Factors ; Ceruloplasmin ; Child ; Child, Preschool ; Copper ; urine ; Female ; Hepatitis ; diagnosis ; pathology ; urine ; Hepatitis A ; diagnosis ; pathology ; urine ; Hepatolenticular Degeneration ; diagnosis ; pathology ; urine ; Humans ; Liver ; pathology ; Male ; Penicillamine ; Predictive Value of Tests ; ROC Curve ; Sensitivity and Specificity ; Time Factors

BACKGROUNDWe undertook a randomized controlled trial to ascertain if single-incision laparoscopic cholecystectomy (SILC) was more beneficial for reducing postoperative pain than traditional laparoscopic cholecystectomy (TLC). Moreover, the influencing factors of SILC were analyzed.

METHODSA total of 552 patients with symptomatic gallstones or polyps were allocated randomly to undergo SILC (n = 138) or TLC (n = 414). Data on postoperative pain score, operative time, complications, procedure conversion, and hospital costs were collected. After a 6-month follow-up, all data were analyzed using the intention-to-treat principle.

RESULTSAmong SILC group, 4 (2.9%) cases required conversion to TLC. Mean operative time of SILC was significantly longer than that of TLC (58.97 ± 21.56 vs. 43.38 ± 19.02 min, P < 0.001). The two groups showed no significant differences in analgesic dose, duration of hospital stay, or cost. Median pain scores were similar between the two groups 7 days after surgery, but SILC-treated patients had a significantly lower median pain score 6 h after surgery (10-point scale: 3 [2, 4] vs. 4 [3, 5], P = 0.009). Importantly, subgroup analyses of operative time for SILC showed that a longer operative time was associated with greater prevalence of pain score >5 (≥100 min: 5/7 patients vs. <40 min, 3/16 patients, P = 0.015).

CONCLUSIONSThe primary benefit of SILC appears to be slightly less pain immediately after surgery. Surgeon training seems to be important because the shorter operative time for SILC may elicit less pain immediately after surgery.

Adolescent ; Adult ; Aged ; Cholecystectomy, Laparoscopic ; adverse effects ; Female ; Gallstones ; surgery ; Humans ; Male ; Middle Aged ; Operative Time ; Pain Measurement ; Pain, Postoperative ; diagnosis ; Polyps ; surgery ; Prospective Studies ; Treatment Outcome ; Young Adult

Bias ; Humans ; Pain Measurement ; Pain, Postoperative

OBJECTIVETo explore whether SLC25A13 gene mutation exists and what is its mutation spectrum in mainland Chinese infants with intrahepatic cholestasis and abnormal blood amino acids.

METHODSBlood amino acids were analyzed by mass chromatographic analysis in infants referred to Fudan University Children's Hospital from June 2003 to June 2007 for investigations of intrahepatic cholestasis of unknown origin. SLC25A13 gene mutations were studied in 14 children whose serum levels of citrulline and/or methionine were at least two times above the upper normal range. In patients in whom only one mutation was detected, all other exons and their neighboring sequences were then analyzed.

RESULTSEight patients with SLC25A13 gene mutations, including 2 with compound heterozygous mutation 851del4/1638ins23, one with homozygous mutation 851del4/851del4, one with compound heterozygous mutation 851del4/R184X, one with homozygous mutation IVS6+1G more than A/IVS6+1G more than A, and 3 with heterozygous mutation 851del4 were found.

CONCLUSIONSSLC25A13 gene mutations exist in Chinese infants with intrahepatic cholestasis and abnormal blood amino acids. Their mutation spectrum is different from that in Japan. 851del4 is the most common mutation in our study. IVS6+1G more than A is a mutation that has not been reported before.

Amino Acids ; blood ; Cholestasis, Intrahepatic ; blood ; genetics ; DNA Mutational Analysis ; Humans ; Infant ; Infant, Newborn ; Mitochondrial Membrane Transport Proteins ; genetics ; Mutation