1.Effect of femoral rotation on hip bone mineral density measurement.
Hai TANG ; Su-mei REN ; Xian-zheng LUO
Acta Academiae Medicinae Sinicae 2003;25(3):267-270
OBJECTIVETo measure hip bone mineral density (BMD) on different femoral rotations and to identify the influence of femoral rotation during hip densitometry.
METHODSForty women were recruited at the out-department of orthopedic, with the average age of 60.7 years (45-85 years), the average height of 161.8 cm (150-175 cm), and the average weight of 67.4 kg (48-80 kg). BMD measurement of hip was done in neutral position (0 degrees), 15 degrees and 30 degrees of internal rotation under quality control of DXA machine. All the subjects were asked to get down scan table and reposition after each measuring in different rotation.
RESULTSHip BMD of proximal femur (femoral neck, Ward's area and trochanter region) have significant variations in measurement (P < 0.01) among neutral position, 15 degrees and 30 degrees of internal rotation. There were more BMD change at the cancellous region (Ward's triangle and trochanter region) than at cortical bone area (femoral neck region). Statistical analysis showed a significant positive correlation for hip BMD change between neutral degrees to 15 degrees of internal rotation and 15 degrees to 30 degrees of internal rotation. But no correlation between neutral degrees to 30 degrees of internal rotation for hip BMD change.
CONCLUSIONSFemoral rotation was shown to have a significant effect on hip BMD measurements. Cortical bone region of hip have a less BMD change than cancellous bone region. Proper positioning of the femur during scan can improve precision significantly.
Absorptiometry, Photon ; methods ; Aged ; Aged, 80 and over ; Bone Density ; Female ; Femur ; Hip ; Humans ; Middle Aged ; Posture ; Rotation
2.Effect and mechanism of Danhong injection on isolated mesenteric arterial rings in rats.
Xiao-wen ZHI ; Xian-ming SU ; Wei-yi FENG ; Hong-mei ZHANG
China Journal of Chinese Materia Medica 2012;37(17):2607-2611
OBJECTIVETo study the effect and mechanism of Danhong injection on isolated mesenteric arterial rings in rats.
METHODAn isolated vascular ring experiment was conducted to determine the changes in tension of vascular rings with a biological signal collection and analytical system.
RESULTDanhong injection had no impact on the tension of vascular rings. Danhong injection showed a significant vasodilatation effect on treated arteria rings of norepinephrine, and no remarkable impact was made on the effect without endothium. It showed notable effect on blood vessels treated with Ca(2+) and no significant impact on those treated with caffeine. It could inhibit NE-induced intracellular calcium from releasing and external calcium from inflowing. No effects of potassium channel blockers on aorta ring tensile force were found.
CONCLUSIONDanhong injection shows significant vasodilation effect, which mainly works through vascular smooth muscle. Its vasodilation effect may be related to inhibitory receptor, voltage-dependent Ca(2+)-release and IP3 receptor-mediated Ca(2 +)-influx.
Animals ; Calcium ; metabolism ; Drugs, Chinese Herbal ; administration & dosage ; In Vitro Techniques ; Injections ; Mesenteric Arteries ; drug effects ; physiology ; Rats ; Rats, Sprague-Dawley ; Vasodilation ; drug effects
3.Security evaluation of bupivacaine, ropivacaine combined with fentanyl in postoperative continuous epidural analgesia.
Xiao-hong WAN ; Qing-qing HUANG ; Mei-xian SU ; Lin-jun WAN ; Hai-qiong HUANG
Chinese Journal of Surgery 2006;44(17):1200-1202
OBJECTIVETo investigate the effects, side-effects and security of bupivacaine, ropivacaine combined with fentanyl in postoperative continuous epidural analgesia.
METHODSA total of 1 600 postoperative continuous epidural analgesia patients receiving different agents in SICU were divided into two groups: 0.1% bupivacaine +5 microg/ml fentanyl group (group B, n = 920) and 0.2% ropivacaine +2 microg/ml fentanyl group (group R, n = 680). The effects (visual analog-scale score and content to analgesia), side effects were analyzed retrospectively in the two groups.
RESULTSCompared with group B, patients in group R had higher analgesia contentment (P < 0.05), but no difference in visual analog-scale score was found in the two groups. The incidences of urinary retention, nausea and vomiting, skin itching in group B were significantly higher than those in group R (P < 0.05). In each group, patients over sixty had higher ratio of hypotension than those under sixty (P < 0.05); The female patients had a higher incidence of nausea and vomiting than male patients (P < 0.05); The incidence of debility and numbness of lower limbs in patients with lumbar segments epidural analgesia was higher than those with thoracic analgesia (P < 0.05).
CONCLUSIONS0.1% bupivacaine +5 microg/ml fentanyl and 0.2% ropivacaine +2 microg/ml fentanyl can provide adequate pain relief in postoperative continuous epidural analgesia, and 0.2% ropivacaine +2 microg/ml fentanyl comes with less side effects. The incidence of complication is related with analgesics, age, gender and the position of epidural puncture.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Amides ; administration & dosage ; adverse effects ; Analgesia, Epidural ; adverse effects ; methods ; Analgesia, Obstetrical ; adverse effects ; methods ; Analgesics, Opioid ; administration & dosage ; adverse effects ; Anesthetics, Local ; administration & dosage ; adverse effects ; Bupivacaine ; administration & dosage ; adverse effects ; Female ; Fentanyl ; administration & dosage ; adverse effects ; Humans ; Male ; Middle Aged ; Pain, Postoperative ; prevention & control ; Retrospective Studies
4.The nutrition status of special population living in the areas affected by Wenchuan Earthquake after 3 months.
Li-yun ZHAO ; Dong-mei YU ; Jian HUANG ; Xian-feng ZHAO ; Jian-wen LI ; Wen-wen DU ; Wen-tao YU ; Chang SU ; Shi-an YIN
Chinese Journal of Preventive Medicine 2010;44(8):701-705
OBJECTIVETo investigate and analyze the nutritional status of infants, preschool and primary school students, pregnant and lactating women in Wenchuan earthquake disaster area after 3 months.
METHODSIn August 2008, the nutritional and health status information of special population were collected and evaluated using the questionnaires, anthropometric and hemoglobin concentration measurements from four settlements of villages and towns in Mao and Mianzhu Country of Sichuan and Kang Country and Wudu District in Gansu provinces. A total of 236 infants aged below 36 months, 48 preschool children, 368 primary students, 32 pregnant women and 72 lactating women were investigated. Principal investigator indexes included the low body weight, growth retardation, anemia prevalence, two-week prevalence of diarrhea and respiratory disease, food intake and nutrition-related diseases, the percentage of patients morbidity of 36 months infants, preschool and primary school students; the prevalence of anemia, the prevalence of nutrition-related diseases of pregnant and lactating women.
RESULTSThe stunting prevalence was 14.6% (34/236) and the anemia prevalence was up to 40.1% (61/236) among infants younger than 36 months. Besides, the percentages of infants and young children suffered from the respiratory-infected disease and diarrhea within recent two weeks were 40.4% (95/236) and 30.2% (71/236) respectively. The percentage of low body weight of preschool children was 14.6% (7/48) and the growth retardation and anemia prevalence was 14.6% (7/48) and 39.6% (19/48), respectively. Among primary students, 6.3% (23/368) showed growth retardation and 12.2% (45/368) were anemia. The prevalence of anemia status of pregnant women and lactating mothers were 53.9% (17/32) and 24.4% (18/72) respectively. The main food composition of 45.8% (33/72) lactating women were grain and vegetables, 29 (40.3%), 32 (44.4%), 28 (38.9%) and 53 (73.6%) lactating women did not have animal originated (including meat, aquatic, livestock and poultry products) food, eggs, beans and their products, milk and dairy products, respectively.
CONCLUSIONSpecial population lived in disaster area have suffered different degree of malnutrition. The main dietary pattern was vegetable food. The consumption of meat, eggs, milk and milk products was relatively insufficient.
Adolescent ; Adult ; Child ; Child, Preschool ; China ; epidemiology ; Disasters ; Earthquakes ; Female ; Humans ; Infant ; Lactation ; Male ; Nutrition Disorders ; epidemiology ; Nutritional Status ; Pregnancy ; Students ; Surveys and Questionnaires ; Young Adult
5.Hematopoiesis support of mesenchymal stem cells in children with aplastic anemia.
Yan WU ; Jie YU ; Lei ZHANG ; Qing LUO ; Jian-Wen XIAO ; Xiao-Mei LIU ; Ying XIAN ; Bi-Tao DAI ; You-Hua XU ; Yong-Chun SU
Chinese Journal of Contemporary Pediatrics 2008;10(4):455-459
OBJECTIVEThe abnormality of hemopoietic inductive microenvironment (HIM) is involved in the pathophysiology of aplastic anemia (AA). Mesenchymal stem cells (MSC) are main source of bone marrow stromal cells which constitute the bone marrow HIM. Thus, the bone marrow failure in AA may be related to the function of MSC. The aim of the study was to investigate the hematopoiesis support function of MSC in children with AA in vitro.
METHODSBone marrow samples were collected from 24 children with AA at diagnosis and 19 children with idiopathic thrombocytopenic purpura (ITP), infectious mononucleosis or lymphadenitis (controls). MSCs from bone marrow samples were isolated, cultured and expanded. Morphology, proliferation activity and colony forming unit-fibroblast (CFU-F) were measured. The ability of bone marrow MSC to adhere hemopoietic cells was assayed by MTT. The concentration of stem cell factor (SCF) released from MSC was tested using ELISA. Mononuclear cells (MNC) of bone marrow were plated onto a feeder layer formed by MSC. Cells count and BFU-E, CFU-GM, CFU-GMME productions were measured.
RESULTSThe first and third passage time of MSC in children with AA was longer than that in the controls. The number of CFU-F in children with AA (15.70+/-5.78) was less than that in the controls (21.73+/-5.74) (P<0.05). The concentration of SCF in MSC supernatants in children with AA (30.69+/-16.82 pg/mL) was significantly lower than the controls (50.74+/-14.83 pg/mL) (P<0.01). The total MNC count and the number of BFU-E, CFU-GM and CFU-GMME colonies in the support of MSC in children with AA were significantly lower than those in the controls (P<0.01).
CONCLUSIONSThe hematopoiesis support function of MSC was significantly reduced in children with AA in vitro. The decreased hematopoiesis support function of MSC may be related its decreased proliferation capacity and SCF release activity.
Adolescent ; Anemia, Aplastic ; physiopathology ; Cell Adhesion ; Child ; Child, Preschool ; Female ; Hematopoiesis ; Humans ; Leukocytes, Mononuclear ; physiology ; Male ; Mesenchymal Stromal Cells ; physiology ; Stem Cell Factor ; physiology
6.Effects of rice peptide YGEGSSEEG on improving endothelial cell injury induced by tumor necrosis factor
Zhi-Yuan JU ; Li-Li WANG ; Li-Ya LIU ; Xian-Rong ZHOU ; Su-Mei ZHOU ; Li-Tao TONG
Chinese Pharmacological Bulletin 2018;34(4):501-507
Aim To investigate the peptides and its protection for vascular endothelial cells, derived from the absorbed components of rice α-globulin,which was shown to be effective in anti-atherosclerosis. Methods The amino acid sequence was purified by gel chro-matography and RP-HPLC, and determined by ESI/MS. Then the peptide was chemically synthesized. Hu-man umbilical vein endothelial cell injury model was induced by tumor necrosis factor-α. The cell viability was measured by cell counting kit to screen the appro-priate peptide intervention concentration. The apoptotic rate was detected by flow cytometry. Bcl-2, Bax, p-p38, vascular cell adhesion molecule and the protein expression level of NF-κB signaling pathway were de-tected by Western blot and immunofluorescent stai-ning. Results Apoptosis of HUVECs induced by TNF-α was significantly increased by YGEGSSEEG, which also regulated expression of Bcl-2/Bax proteins and inhibited phosphorylation of p38 protein. Besides, the peptide suppressed the production of VCAM-1, ICAM-1 and activation of NF-κB pathway. While it did not significantly improve the oxidative stress response in HUVECs. Conclusion Peptide YGEGSSEEG pro-tects vascular endothelial cells through suppressing ap-optosis and expression of adhesion molecules.
7.Establishment of a complex alcoholic liver fibrosis mouse model and investigation of OPN and TGF-beta1 hepatic expression.
Ling-bo KONG ; Wei-guang REN ; Hong-mei MI ; Su-xian ZHAO ; Yu-guo ZHANG ; Yue-min NAN
Chinese Journal of Hepatology 2013;21(3):207-212
OBJECTIVETo create a convenient method to establish an alcoholic liver fibrosis model in mice and use it to explore the putative pathogenic mechanisms involving the immunomodulatory proteins osteopontin (OPN) and transforming growth factor-betal (TGF-beta1).
METHODSForty C57BLI6J mice were fed the Lieber-DeCarli 4% ethanol-containing liquid diet for four weeks, followed by an additional four weeks of the 4% ethanol diet combined with intraperitoneal injection of carbon tetrachloride (CC14 5% solution in olive oil; 2ml/ kg body weight, 2 times/week) to induce alcoholic liver fibrosis. Control groups (n = 6 each) included: normal diet; normal diet plus CCl4 injections; ethanol diet alone; ethanol diet plus solvent (olive oil) injections. Model establishment was monitored by sacrificing six mice at model inception (week 0), and weeks 4, 5, 6, 7, and 8 of modeling to collect liver tissues and blood for histological and biochemical analyses. Extent of hepatic steatosis, inflammation, and fibrosis was assessed by hematoxylin-eosin and Masson staining. Liver function markers, serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels, were tested by automated enzymatic assays. Alpha-smooth muscle actin (alpha-SMA) expression was detected by immunohistochemistry. The mRNA and protein expression of OPN and TGF-beta1 was detected by real-time quantitative reverse transcription-PCR and western blotting, respectively. Significance of differences between multiple groups was assessed by one-way ANOVA analysis followed by least significant difference t-test or Kruskal-Wallis H test followed by the Mann-Whitney U test.
RESULTSCompared to the control groups, the group of mice administrated ethanol and CCl4 developed mild to moderate hepatic steatosis at week 4 of modeling, progressive necroinflammation and perisinusoidal and portal fibrosis from weeks 5-8, and irregular necrosis and bridging fibrosis at week 8. In addition, the model group showed progressive up-regulation of a-SMA expression in the activated hepatic stellate cells (HSCs) and fibrotic areas from weeks 5-8. Both hepatic OPN and TGF-beta1 showed significantly increasing trends in mRNA and protein expressions from weeks 5-8 (OPN mRNA: 1.83 +/- 0.25, 2.94 +/- 0.19, 3.45 +/- 0.31, and 5.99 +/- 0.17 (F= 476.27, P < 0.001); OPN protein: 0.52 +/- 0.06, 1.02 +/- 0.10, 1.52 +/- 0.11 and 1.50 +/- 0.08 (F= 298.03, P< 0.001); TGF-beta1 mRNA: 13.19 +/- 0.40, 3.31 +/- 0.28, 1.58 +/- 0.18 and 2.08 +/- 0.26 (F= 85.55, P < 0.001); TGF-P31 protein: 1.26 +/- 0.16, 0.96 +/- 0.12, 1.09 +/- 0.25 and 1.10 +/- 0.20 (F = 43.64, P < 0.001).
CONCLUSIONFeeding C57BL/6J mice the Lieber-DeCarli ethanol-containing liquid diet combined with CCl4 intraperitoneal injection is a convenient method to establish a model of alcoholic liver fibrosis within a relatively short amount of time (eight weeks). Progression of alcoholic liver fibrosis is accompanied by increased hepatic expression of OPN and TGF-beta1, which may contribute to the pathogenic mechanism of this disease and may be targets of future molecular therapies.
Actins ; metabolism ; Animals ; Disease Models, Animal ; Liver ; metabolism ; Liver Cirrhosis, Alcoholic ; metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Osteopontin ; metabolism ; Transforming Growth Factor beta1 ; metabolism
8.Activation of Fas/FasL and its downstream signaling pathway promotes development of alcoholic steatohepatitis and liver fibrosis in mice.
Wei-guang REN ; Ling-bo KONG ; Hong-mei MI ; Su-xian ZHAO ; Yu-guo ZHANG ; Rong-qi WANG ; Yue-min NAN
Chinese Journal of Hepatology 2013;21(2):129-133
OBJECTIVETo explore the role and mechanism of the Fas/Fas ligand (FasL) system and its downstream signaling pathway related to the progression of alcoholic steatohepatitis and liver fibrosis.
METHODSEighteen C57BL/6J mice were randomly divided into three groups: controls; alcoholic steatohepatitis model, given four-weeks of a 4% ethanol-containing Lieber-DeCarli liquid diet; alcoholic steatohepatitis and liver fibrosis model, given the four-week alcohol diet followed by twice weekly intraperitoneal injections of carbon tetrachloride (5% olive oil solution; 2 mL/kg dose) during the fifth to eighth weeks. Mice in the model groups were sacrificed at the end of week 4 and 8, respectively, along with control mice for comparative analyses. Liver tissue sections were evaluated for hepatocellular apoptosis by terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL) assay. The mRNA expression of Fas, FasL, cysteine aspartate-specific proteases 3 (caspase 3), and cytochrome P450 2E1 (CYP 2E1) in liver tissues was detected by reverse transcription (RT)-PCR, visualized by ethidium bromide staining, and normalized to the gray-value of GAPDH expression. The protein expression of Fas and caspase 3 were detected by western blotting (b-actin normalized), and of FasL and CYP 2E1 by immunohistochemistry staining. Intergroup differences and statistical significance were evaluated by single factor analysis of variance and the least squares difference-t test or the Kruskal-Wallis H test and the Mann-Whitney U test.
RESULTSThe number of apoptotic cells in the liver sections was significantly higher in both model groups with alcoholic steatohepatitis (vs. controls) and the amount in the alcoholic steatohepatitis plus liver fibrosis model was significantly higher than that in the model with only alcoholic steatohepatitis. In addition, activation of Fas, FasL and its downstream signaling pathway showed an increasing trend with extent of liver injury. The hepatic mRNA (by RT-PCR) and protein (by western blotting) normalized expression levels in the controls, alcoholic steatohepatitis models, and alcoholic steatohepatitis plus liver fibrosis models were, respectively: Fas mRNA: 0.50+/-0.05, 0.61+/-0.10, 0.76+/-0.03 (H=12.137, P less than 0.05), protein: 0.52+/-0.14, 0.86+/-0.10, 0.99+/-0.09 (F=12.758, P less than 0.01); FasL mRNA: 0.31+/-0.03, 0.53+/-0.02, 1.02+/-0.04 (F=153.260, P less than 0.01); caspase 3 mRNA: 0.86+/-0.11, 0.85+/-0.05, 1.33+/-0.16 (F=8.740, P less than 0.01), protein: 0.40+/-0.03, 0.69+/-0.06, 1.02+/-0.10 (F=90.785, P less than 0.01); CYP 2E1 mRNA: 0.72+/-0.14, 1.00+/-0.15, 1.30+/-0.20 (H=4.713, P less than 0.01). The changes in hepatic FasL and CYP 2E1 expression detected by immunohistochemistry were consistent with the mRNA expression.
CONCLUSIONActivation of Fas/FasL and its downstream signaling pathway, which induces hepatocellular apoptosis, contributes to the development of alcoholic steatohepatitis and liver fibrosis.
Animals ; Apoptosis ; Cytochrome P-450 CYP2E1 ; metabolism ; Fas Ligand Protein ; metabolism ; Fatty Liver, Alcoholic ; metabolism ; pathology ; Liver Cirrhosis ; metabolism ; pathology ; Male ; Mice ; Mice, Inbred C57BL ; Signal Transduction ; fas Receptor ; metabolism
9.Long-term stable expression of antisense cDNA of cyclin B1 profoundly inhibits the proliferation of tumor cells and suppresses tumorigenicity in implanted mice.
Tao ZHANG ; Ling ZHANG ; Ji-Cheng LI ; Dong WEI ; Yu-Quan WEI ; Ru ZHANG ; Peng CHENG ; Xian-Cheng CHEN ; Huan-Yi LIU ; Xiao-Mei SU
Chinese Medical Journal 2008;121(15):1433-1438
BACKGROUNDCyclin B1 (CLB1) is necessary for mitotic initiation in mammalian cells and plays important roles in cancer development. Therefore, a potential strategy in cancer therapy is to suppress the activity of CLB1 by delivering antisense constructs of CLB1 into tumor cells. In previous CLB1 studies, antisense constructs with a short half life were often used and these constructs might not persistently inhibit CLB1.
METHODSWe successfully created a recombinant plasmid encoding the full-length antisense cDNA of mouse cyclin B1 (AS-mCLB1) and transfected this construct to the murine Lewis lung carcinoma (LL/2) and CT-26 colon carcinoma (CT-26) cells. We isolated clones of LL/2 and CT-26 transfectants with stable expression of AS-mCLB1. Reverse transcriptional polymerase chain reaction (RT-PCR) and Western blot were applied to detect the expression of the mRNA and protein levels of CLB1. To further test the efficacy of this strategy in vivo, AS-mCLB1-expressing LL/2 and CT-26 transfectants were implanted into mice.
RESULTSWe found the expression of the mRNA and protein levels of CLB1 decrease in these transfectants. The inhibition of CLB1 caused prominent G1 arrest, abnormal morphology, retarded cell growth and an increase in apoptosis. In AS-mCLB1-expressing LL/2 and CT-26 transfectants implanted mice, tumorigenicity was effectively suppressed compared with the controls. In addition, the expression of AS-mCLB1 also significantly increases the survival duration of implanted animals.
CONCLUSIONAS-mCLB1 is likely to be useful in future cancer therapy, which may be associated with its ability to down-regulate the expression of CLB1 and then induce G1arrest and apoptosis in tumor cells.
Animals ; Apoptosis ; Cell Proliferation ; Cell Survival ; Cyclin B ; antagonists & inhibitors ; genetics ; Cyclin B1 ; DNA, Antisense ; pharmacology ; DNA, Complementary ; pharmacology ; G1 Phase ; Mice ; Mice, Inbred C57BL ; Neoplasm Transplantation ; Neoplasms, Experimental ; pathology ; therapy
10.Successful treatment of a patient with Wiskott-Aldrich syndrome using hematopoietic stem cell transplantation--case report and literature review.
Jie YU ; Xian-min GUAN ; Bi-tao DAI ; Li-ping JIANG ; Yong-chun SU ; Jian-wen XIAO ; Xiao-mei LIU ; Ying XIAN ; You-hua XU ; Xi-qiang YANG ; Xiao-dong ZHAO
Chinese Journal of Pediatrics 2009;47(3):183-188
OBJECTIVEWiskott-Aldrich syndrome (WAS) is a primary immunodeficiency diseases. The patients with classical WAS have poor prognosis. The hematopoietic stem cell transplantation is the most effective method to cure WAS at present. In this report, a patient with WAS was cured with HLA identical sibling bone marrow transplantation (BMT).
METHODSWiskott-Aldrich syndrome protein (WASP) was detected using flow cytometry and WASP were analyzed for the diagnosis. The bone marrow was collected from the elder sister who was the HLA identical sibling donor. A total of 4.38x10(8)/kg mononuclear cell (MNC) and 3.78x10(6)/kg CD34+ cells were collected and transfused into the patient after the conditioning regimen with busulfan/cyclophosphamide. Cyclosporine only was used for graft-versus-host disease prophylaxis. WASP and short tandem repeats (STR) were detected as the evidence of engraftment.
RESULTSThe diagnosis was WAS: WASP (-IVS9+2T>C, WASP-negative). The patient received busulfan/cyclophosphamide 9 days before the transplantation. WBC decreased to 0.1x10(9)/L in d+4; The absolute number of neutrophils (ANC) was 0.8x10(9)/L in d+13, and exceeded 1.0x10(9)/L later on. From d(-9)-d+14 the patient was dependent on platelet transfusion. From d+15 the patient's PLT>50x10(9)/L and returned to normal after d+30. In d+9-d+10 mild GVHD (I degree) occurred but subsided after the steroid treatment. From d+50, WASP was detected positive and STR showed full donor DNA chimera. Follow-up for 510 d post-transplant, the patient suffered only from mild cold twice, no eczema, no bleeding occurred. The PLT is normal and no chronic GVHD occurred. The levels of IgG, IgM and IgA of the patient were approximately normal.
CONCLUSIONThe HLA-identical sibling's BMT seems to be the periorit treatment of choice for the WAS patient.
Child, Preschool ; Hematopoietic Stem Cell Transplantation ; Humans ; Male ; Treatment Outcome ; Wiskott-Aldrich Syndrome ; surgery