PKHD1 ( polycystic kidney and hepatic disease 1), the causal gene of human autosomal recessive polycystic kidney disease(ARPKD), is located on chromosome 6p12.2 and covers a genomic region of ～500 kb. PKHD1 is among the largest human genes, with a minimum of 86 exons from which multiple transcripts may be generated by alternative splicing. The longest continuous open reading frame consists of 12 222 bp, encoding a 4 074 amino acid protein, designated as fibrocystin/polyductin (FPC). FPC is predicted to be a receptor like protein, with single transmembrane domain and a short cytoplasmic tail. The expression of mouse FPC can be detected in various duct-containing organs. In mouse embryogenesis, FPC appears in developing neural tube, bronchi, and the primordial gut as early as the day of E9.5. In fetal human kidneys, high level of FPC expression is present in ureteric bud and its expression continues throughout the process of renal tubuobranching. In adult human kidneys, FPC mainly expresses in the epithelia of renal collecting ducts. FPC is subcellularly localized to the primary cilia and concentrated on the basal bodies in renal epithelial cells. The detail function of FPC is still unrevealed, most recent studies demonstrate that FPC, as a receptor, may transduce cell signal by interacting with a trp superfamily TRPP2 (PKD2), which is a causal gene for ADPKD, and mediate intracellular calcium homeostasis to regulate differentiation, proliferation, migration and polarity of various duct/tubular epithelia, in turn, to modulate the formation of all physiologic ducts, tubules and tracts.

Objective To explore the role of choledochofiberscopy in the diagnosis and treatment of biliary complication after orthotopic liver transplantation.Methods The patients with continuous biliary infection and obstructive jaundice in 6 cases subject to orthotopic liver transplantation were treated by choledochofiberscopy through T tube sinus tract. The findings from choledochofiberscope at different periods after operation were recorded.Results Three to five months after operation, there were a lot of flocs in the bile duct cavity under the choledochofiberscopy, and mucous membrane of bile duct was pale or coarse. On the postoperative month 6, 11 and 18, choledochofiberscopy revealed there were funicular, columned and brown biliary stones in bile duct cavity which mostly located in porta hepatis, and there existed stenosis in anastomotic stoma of bile duct to varying degrees; the wall of bile duct was coarse, with congestion of the mucous membrane. The flocs and stones could be extracted conveniently by the choledochofiberscope.Conclusion We can observe the transplanted liver’s bile duct through the T tube sinus tract by the choledochofiberscopy and treat the biliary complications conveniently.

Using method of orthogonal design we observed that low K+,high Ca2+ & low Mg2+ in perfusate produced the peak incidence of reperf-usion-indaced ventricular fibrillation & introduction of K+ & Ca2+ significantly affected the incidence & pnset of it, The appropriate prop-ortion of K+, ea2+ & Mg2+ in perfusate is the important effecting factor of reperfusion-induced arrhythmias in Langendorff heart of rats.

BACKGROUND: Complications, caused by bile duct injury after liver transplantation, are difficult for diagnosis and treatmerit and the bottlenecks for the development of liver transplantation. OBJECTIVE: To observe and record bile duct injury and do biopsy in parallel with treating biliary complications following liver transplantation successfully with choledochoscope, and to analyze the relationships between various bile duct injuries, histopathological types and biliary complications following liver transplantation. DESIGN, TIME AND SETTING: Case analysis was carried out at Dalian Institute of Hepatobiliary Surgery, Department of Hepatobiliary Surgery. Dalian Friendship Hospital between July 2001 and October 2005. PARTICIPANTS: Nineteen patients after liver transplantation were divided into three groups according to the occurrence of biliary complications: four cases for normal group, twelve cases for bile duct injury group, three cases for hepatic artery miury group. METHODS: They were observed. diagnosed and recorded respectively and take biopsy for pathological analysis through the choledochoscope. With regard to the cases without T-tube. Choledochoscope combined duodenoscope were used to take biopsies. MAIN OUTCOME MEASURES: Choledochoscope was used to observe T-tube cholangiography, the appearances and pathological changes of intemal and external bile duct mucous membrane. healing of the donor-receptor bile duct stoma. Patients in the bile duct injury group were done examinations described above after endoscopic stone extraction and stenosis expansion. RESULTS: The intemal and extemal bile duct anatomy of the patients in the normal group were normal, without bile ductstenosis and scar, their bile duct mucous membrane lookcd good, and the anastomosis of the donor-receptor bile duct healed well. Restored mucous tissue coating with intact epithelium was found by pathological examination. Various kinds of bile duct stones, simple, multiple and casting mould stones, were found in patients of the bile duct injury group. Bile duct mucous membranes were injured at different degrees and repaired after removing stones and relieving obstruction by endoscope. Contrast examination demonstrated that bile duct tree regained normal. In hepatic artery injury group, patients had bile duct wall ischemic necrosis and lost bile duct normal structure, congestion was obvious, biliary sludge and stones completely filled in the bile duct tree. Interrupted bile duct structure were found in Ⅲ grade bile ducts.Pathological examination revealed extensive bile duct wall necrosis,indistinct strcture,more bile infiltration,proliferative granulation tissue and suppuration focus.CONCLUSION:Bile ducts are injured at different degrees in orthotopic liver transplantation;cold preservation/repeffusion injury is the most important initiating agent leading to bile duct tree injuries;the vessel plexus damage and microcirculatory disturbance surrounding the bile ducts maybe one of the mechanisms of the bile duct injury.

Objective To investigate the diagnostic value of two-dimensional and color Doppler ultrasonngraphy on diagnosis and judgement of prednisone withdraw in patients with subacute thyroiditis.Methods Sixty-six patients with clinically proved subacute thyroiditis underwent two-dimensional and color Doppler ultrasonography before treatment of prednisone. The ultmsonographic features and bloodtlow distribution in thyroid of these patients were analyzed retrospectively. Target of prednisone withdraw was judged according to ultrasonographic images (36 eases) and clinical symptom (30 cases). Results The ultmsonographic features of subacute thyroiditis mainly presented as bilateral or unilateral diffussion or focal low echogenicity in the affected thyroid, and color Doppler ultrasonography showed the increased vascularity around the lesions. Intraarterial bloodflow feature was low velocity and low resistance pattern, prednisone withdraw was performed according to ultrasonographic images after the treatment of prednisone, then relapse rate was decreased compared with the group of clinical symptom [8.33% (3/36) vs 26.67% (8/30)] (P <0.05). Conclusion Two-dimensional and color Doppler ultrasonography is highly valuable for diagnosing and treating subacute thyroiditis.

Objective To evaluate the application of Herniamesh in repairing adult umbilical hernia.Methods The data of 12 aduh patients with umbilical hernia were treated with Hemiamesh from January 2007 to March 2009 was analyzed retrospectively,included characteristics,methods and effects of Hemiamesh Oval Patch 0812 on the repair of hernia.Results All cases were cured.The average stay in hospital was (5±2)d.No postoperative complication,no recurrence follow-up visited 1 to 25 months.Conclusion Repairing umbilical hernia in adult with Hemiamesh Oval Patch 0812 is a kind of simple,safe,effective operation and the ring of hernia no more than 4cm.

Objective To discuss the effectiveness of anterolateral thigh propeller flaps for treatment of inguinal skin and soft tissue defects.Methods From June,2009 to October,2014,12 patients with inguinal skin and soft tissue defects were treated with anterolateral thigh propeller flaps pedicled with perforator of descending branch of lateral circumflex femoral artery.Of them there were 8 males and 4 females,aged from 22 to 51 years.The left side was involved in 3 cases and right side 9 cases.Defects were caused by traffic accident injury in 5 cases,crash injury of heavy object in 7 cases.There were mere skin and soft tissue in 2 cases,combined with bone fractures,nerves,vessels and muscles injury in 10 cases.The area of defects ranged from 9.0 cm×5.0 cm to 22.0 cm×9.0 cm.During operation,anterolateral thigh propeller flaps ranging from 11.0 cm×7.0 cm to 24.0 cm×1 1.0 cm were used to repair the wounds.Results All flaps and skingrafts survived after operation and the wounds obtained primary healing.After 8-24 months follow-up,all flaps were characterized by soft texture,good color,and satisfactory appearance.According to the Britain's Medical Research Council at 8 months after operation,the sensation of the flaps were recovered to S2 ～ S3+,No obvious scar contracture and chromatosis were observed at donor site.Conclusion Anterolateral thigh propeller flaps pedicled with artery descending branch of lateral circumflex femoral perforator is an ideal choice for the reconstruction for inguinal skin and soft tissue defects.

Objective To find out related proteins that could possibly predict curative effects of neoadjuvant chemotherapy(NACT) by applying surface-enhanced laser desorption/ionization time-of-flight mass spectrometry(SELDI-TOF MS)to detect the difference in protein spectrum of breast cancer tissues.Methods Cancer tissue samples from 30 cases of breast cancer patients were detected by SELDI-TOF MS before NACT to get protein spectrum of breast cancer tissues.After NACT,the curative effects were evaluated according to RECIST standard and patients were divided into effective group(19 cases with complete response or partial response)and invalid group (11 cases with stable disease or progressive disease).Results 11 different protein peaks (P ＜ 0.05) were screened out:3491 Da(t =3.189 P =0.004),5158 Da(t =3.897 P =0.001),5360 Da(t =2.157 P =0.04),7549 Da(t =2.173 P=0.038),8451 Da(t =2.258 P =0.032),8694 Da(t =3.234 P =0.003),9089 Da (t =2.653 P =0.013),10528 Da(t =3.127 P =0.004),13445 Da(t =2.231 P =0.034),15118 Da(t =3.255 P =0.003),and 44065 Da(t =2.554 P =0.017).They were all expressed higher in effective group than in invalid group.The difference had statistical significance(P ＜ 0.05).Conclusion SELDI-TOF MS technology can screen out protein spectrum sensitive to NACT,providing evidences for breast cancer patients whether or not taking NACT.

Objective To explore ideal surgical axillary management of early breast cancer,and to determine the feasibility of reducing false negative rate of sentinel lymph node biopsy(SLNB)by combination of axillary suspicious lymph node biopsy and SLNB.Methods From Jan.2008 to Oct.2009,42 consecutive cases with early breast cancer were enrolled.All patients underwent suspicious node hook-wire location by doppler ultrasonography before operation.SLNB and suspicious lymph node biopsy were performed during operation.Complete axillary nodes dissection(ALND)or level II dissection would be conducted according to the biopsy result.The difference of node status prediction between SLNB and SLNB with axillary suspicious lymph node biopsy and was compared.Results All the 42 cases successfully underwent SLNB (100%).There were 2 false negatives occurred in SLNB,resulting in false negative rate of 11%,sensitivity of 88.9%and accuracy of 95.2%in predicting axillary nodes status.By contrast,SLNB with axillary suspicious node biopsy showed a false-negative rate of 0%,sensitivity of 100%,and accuracy of 100%.Conclusions Compared to SLNB in early breast cancer,combination of suspicious node biopsy and SLAB has a tendency of reducing false negative rate.However,Because of the limited samples,the difference has no statistical significance(P=0.2500).