Mucopolysaccharidosis type I(MPS Ⅰ), a rare autosomal recessive metabolic disease with complex clinical manifestations, often involves multiple organs and results in a heavy disease burden, for which low diagnosis rate and delayed diagnosis are common. Enzyme replacement therapy and hematopoietic stem cell transplantation are the main treatments for MPS Ⅰ, and MPS Ⅰ patients can benefit from the two therapies as shown by a large amount of research data. There is a lack of awareness of MPS Ⅰ and little research has been done on that at present in China. This article will review the epidemiological characteristics, gene mutations and clinical phenotypes, clinical manifestations, diagnosis and treatment of MPS Ⅰ for understanding the diseases comprehensively as well as promoting early diagnosis and treatment, and earlier treatment may be beneficial to patients.

Objective To investigate the methods of isolation,culture,identification and labeling of mesenchymal stem cells(MSCs) in vitro and lay a foundation for further study on intervention of MSCs on immunologic rejection of organ transplantation. Methods MSCs were isolated and cultivated by adherent methods . The expressions of CD90 and CD45 of cells were analyzed by using flow cytometry in order to identify MSCs.The third generation of MSCs were labeled by DAPI,the labeling efficiency was detected.Results Primary cultured MSCs adhered to plastic surface within 48 h and reached 90% confluence within 7-10 d .Flow cytometry showed that the positive rates of CD90 and CD45 of MSCs at third generation were 99.8% and 6.8%. MSCs expressed CD90 but no CD45.All of the MSCs after labeling by DAPI showed blue fluorescence by immunofluoroscope. DAPI labeling was sensitive and highly efficient to MSCs.Conclusion Adherent method is simple and easy to isolate and cultivate MSCs and it can serve as a routine method.DAPI labeling can be used as a efficient method to label MSCs.

Objective To investigate the effects of inhaled low dose nitric oxide(NO)on lung ischemia-reperfusion injury during flush and delayed 10 min after reperfusion.Methods Sixty health a- dult male Sprague-Dawley rats were randomly allocated to the control and the NO group.Before the donor lung was harvested,the right hilus was clipped for 5 min(clipping test),then blood sample was collected from carotid artery for arterial blood gas analysis as baseline.Lung transplantation was per- formed in a“cuff-like”vessel anastomosis technique.Dynamic compliance(Cdyn)and resistance of airway(Raw)were monitored before operation(baseline)and after 2-h reperfusion.The graft's gas exchange and oxygenation were assessed by“clipping test”after 2-h reperfusion.The lung graft was harvested for measuring wet/dry weight ratio(W/D),the activity of myeloperoxidase(MPO)and in- ducible nitric oxide synthase(iNOS),the content of malonyldialdehyde(MDA),and the expression of iNOS gene and protein.Results After 2-h reperfusion,compared to the control group,PaO_2/FiO_2, OI,and Qs/Qt were improved significantly in the NO group(277?91 vs.157?47,P＜0.01;2.67?0.89 vs.4.72?1.48,P＜0.01;21.1?4.57 vs.27.1?2.37,P＜0.01,respectively).The activi- ties of MPO were significantly reduced in NO group(1.80?0.46 vs 3.08?0.65 U/g tissue,P＜0.01).The content of MDA in the lung tissue of NO group was significantly higher than that of the control group(34.8?7.9 vs.20.0?11.2 nmol/mg protein,P＜0.05).Inflammatory cell infiltration was also significantly reduced(P＜0.05).The expression of iNOS gene and protein in the lung tissue of NO group was significantly lower than that of the control group.The activities of iNOS were also significantly reduced in NO group(10.6?10.2 vs 97.8?82.2 nmol?g~(-1)?min~(-1),P＜0.05).The im- munohistochemical positive staining of iNOS was localized in the alveolar epithelial cells and the in- flammatory cells infiltrated in the alveolar spaces and mesenchymal tissue.But there were no signifi- cant differences between two groups in Cdyn,Raw and W/D ratio.Conclusion Inhaled low dose NO might mitigate the intrapulmonary shunt,prevent neutrophil sequestration,inhibit the expression of iNOS gene and protein in isograft,thereby ameliorate ischemia-reperfusion injury and improve the ox- ygenation of the graft.

Objective　To report the experience in diagnosis and treatment of inferior vena cava stenosis (IVCS) after orthotopic liver transplantation (OLT). Methods　The clnical data of 3 patients with IVCS out of 51 OLT patients were analysed retrospectively. Results　The incidence of IVCS after OLT was 5.8% (3/51) in our hospital. In the 3 cases, IVCS of the posteriorhepatic IVC segment occurred within the first postoperative month. IVCS was identified by color duplex ultrasonography and confirmed by angiography. Percutaenous transluminal angioplasty (PTA) or metallic stent replacement were used in the 3 cases resulting in restoration of normal venous flow and elimination of legs edema. The first patient died of cerebral hemorrhage 14 days after transplantation during anti-coagulative therapy. The other two recovered smoothly with good liver function, abdominal ultrasonography demonstrated patency of the IVC. The 2 cases were alive for 18 and 4 months respectively. Conclusions　The venacavographic balloon angioplasty and metallic stent replacement are safe and useful for post-OLT IVCS. The short-term result is excellent.

Objective To compare the ultrafine chest drainage tube with traditional thick hose as lower tube after uniport thoracoscope pulmonary nodules resection(segmentectomy,lobectomy) and evaluate its efficacy and safety.Methods Ultrafine chest drainage tube was applied in 55 patients underwent uniport thoracoscopy pulmonary resection,and traditional thick closed drainage hose was applied in another 55 patients in the same period as the control group.Postoperative drainage quantity,drainage time,pain score and incision healing between two group were compared.Results No statistically difference was found in two groups at thoracic drainage quantity on 1 day,2 days postoperative and total drainage time.But statistically difference was found in thoracic drainage quantity on 3 days postoperative,pain score and incision healing(P ＜0.05).Conclusion Ultrafine chest drainage tube has little trauma,definite clinical effect and can be easily accepted by patients.

Currently, multiple difficulties exist in clinical liver transplantation, such as shortage of donor liver, increasing quantity of patients waiting for liver transplantation and lack of matching donors, etc. Some children and adult patients have little chance of undergoing liver transplantation, which also limits the development of liver transplantation. In this context, split liver transplantation emerges, in which 1 donor liver can be applied to 2 or even more recipients. It may effectively increase the utilization rate of donor liver and alleviate the shortage of donor liver. With the development of split liver transplantation, the survival rate of split liver transplantation is comparable to that of total liver transplantation. Multiple transplantation centers have routinely adopted split liver transplantation. In this article, the development of split liver transplantation, the selection and matching of donors and recipients, the split and reconstruction techniques of donor liver and postoperative complications were reviewed, aiming to provide reference for subsequent development of split liver transplantation in clinical practice and increase the chance of liver transplantation for more patients diagnosed with end-stage liver diseases.

To report the clinical, imaging, and pathological feature of a rare case of central precocious puberty with primary pigmented nodular adrenocortical disease(PPNAD), and to conduct a retrospective analysis of PPNAD with relevant literatures. The pubic hair was found in the child for more than one year. Physical examination showed Cushing′s syndrome. ACTH in blood decreased, cortisol rhythm was disordered, 24-hour urine free cortisol increased and the paradoxical increase of urine free cortisol after high dose dexamethasone suppression test. Adrenal enhancement computed tomography(CT)showed multiple small nodular shadows in bilateral adrenal glands. Gonadotropin releasing hormone(GnRH)stimulation test supported central precocious puberty and GnRH analogue was used to control the sexual development. PPNAD was supported by pathology result. The symptoms of Cushing′s syndrome were relieved partially after left adrenalectomy.

Objective To evaluate the value of postoperative prophylactic radiotherapy for N0 e-sophageal squamous carcinoma. Methods From January 1993 to December 2006,859 patients with patho-logically staged N0 and M0 esophageal squamous cell carcinoma were included in this study. Among them, 760 received surgery alone, and 99 received surgery followed by postoperative radiotherapy. Radiotherapy started within 3 to 4 weeks after surgery. The median total dose was 50 Gy(2 Gy/F,5 F/w). Results In surgery alone group and postoperative radiotherapy group,the 5-yeur overall survival rotes were 72.2% vs 77.4% (X2 =0. 13,P >0.05) for all patients,34.6% vs67.1% (X2 =7.72,P <0.05) forpT4 disease,and 70.2% vs 81.3% (X2 =4.01 ,P <0.05) for tumor length >5 cm. Postoperative radiotherapy could lower the recurrence rate for pT4 patients. Conclusions For patients with NO esophageal squamous carcinoma, postoperative radiotherapy can significantly improve the survival for pT4 and tumor length > 5 cm,and also re-duce the recurrence for pT4 patients.

Objective To retrospectively analyze the treatment results and side effects of postoperative chemoradiotherapy and postoperative radiotherapy of thoracic esophageal squamous cell carcinoma (EPC) with positive lymph nodes.Methods Three hundred and four patients of thoracic EPC were initially treated with three-field lymphadenectomy, with pathological positive lymph nodes and without distant metastases;and randomly divided into two groups:140 patients of the simple postoperative radiotherapy (RT) and 164 patients of postoperative chemoradiotherapy (CRT).The median total dose was 50 Gy.The regimen of concurrent chemotherapy included cisplatin and taxol, and the intermission was 21 days;the median cycles were 2.35.Results The 3-year overall survival rates were 65.7% of CRT and 52.8% of RT (χ2=6.90,P=0.009), and The 3-year disease-free survival rates were 62.5% of CRT and 52.8% of RT (χ2=4.75,P=0.029).The incidences of the supraclavicular lymph-node metastases were 1.8% and 7.1%(χ2=5.21,P=0.022), respectively;and the incidences of distant metastases were 18.3% and 27.9%(χ2=3.94,P=0.047), respectively;and the incidences of overall metastases and recurrences were 27.4% and 39.3%(χ2=4.80,P=0.028), respectively.Early side effects included granulopenia (96.3% and 32.1%,χ2=140.31,P=0.000), radiation-induced esophagitis (37.2% and 26.4%,χ2=4.01,P=0.045),and gastrointestinal toxicity (23.2% and 5.0%,χ2=19.77,P=0.000).Late side effects were 6.1% and 5.0%(χ2=0.17,P=0.678), respectively.Conclusions Postoperative chemoradiotherapy of thoracic EPC with positive lymph nodes can improve the overall survival and disease-free survival, decrease the incidence of the supraclavicular metastases, the distant metastases, the total metastases and recurrences.More severe early side effects were observed in chemoradiotherapy than in the radiotherapy group,but well tolerated.

Objective To analyze the relationship between the number as well as distributions of positive lymph nodes, and the clinical outcomes of postoperative radiotherapy for thoracic esophageal squamous cell carcinoma (TESCC) , And to evaluate the efficacy of postoperative radiotherapy . Methods From January 1993 to March 2007,945 patients with TESCC treated with three-field lymphadenectomy were involved in this study. All patients were with lymphoid metastasis but without distant metastasis. Among them, 590 patients received surgery alone and the other 355 received surgery plus postoperative radiotherapy. The radiotherapy were begun in the third or fourth week after operation and the median total radiation dose was 50 Gy in 25 fractions of 2 Gy, 5 fractions per week. Results The follow-up rate was 94.5%. 189 patients finished minimal follow-up of five years. The 5-year survival rates in the surgery alone group and in the postoperative radiotherapy group were 29.6% and 38.0%, respectively (χ~2 = 10.44 ,P = 0.001). In stratification analysis, compared with the surgery alone, postoperative radiotherapy could increase the 5-year survival rate of the patients with 3 to 5 (30.5% : 23.1%, χ~2 = 4.11, P = 0.043) or > 5 positive nodes (16.7% : 8.9%, χ~2= 6.87, P= 0.009) , or metastastatic node in the region of supraclavicular or upper mediastinum (45.5% : 34.9%, χ~2= 5.37, P = 0.020). In patients with positive nodes number less than 3, or with medium mediastinum or lower mediastinum lymph nodes metastasis, postoperative radiotherapy could not increase the 5-year survival rates (50.7% : 41.2%, χ~2 = 3.30, P = 0.069 ; 32.0% : 27.7% , χ~2= 2.22 , P = 0. 137) , Though could decrease lymph nodes metastasis in supraclavicular and medium-upper mediastinum (15 : 76, χ~2 = 18.10, P = 0.000; 18 : 97, χ~2= 26.81, P = 0.000). Conclusions In TESCC patients with positive lymph nodes number ≥3 or nodes in the region of supraclavicular and upper mediastinum, postoperative radiotherapy can improve the survival after three field lymphadenectomy. And the rate of metastastatic lymph nodes in the supraclavicular region or upper-medium mediastinum can also be decreased.