Objective To investigate the clinical efficacy of laparoscopic hepatectomy.Methods The clinical data of 258 patients who received laparoscopic hepatectomy at the First Affiliated Hospital of Fujian Medical University from March 2010 to January 2013 were retrospectively analyzed.There were 196 patients with primary hepatic cancer,45 with hepatic hemangioma,13 with hepatic focal nodular hyperplasia,2 with hepatic metastatic cancer,1 with carcinoma of gallbladder and 1 with hepatic hamartoma.All patients were followed up via phone call or out-patient examination till March 2013.Results A total of 142 patients received single hepatic segmentectomy,98 received multiple hepatic segmentectomy,18 received multiple lesions resection.Fifty-one patients received hepatic tumorectomy + cholecystectomy.All the operations were successfully done under laparoscope without conversion to the open surgery.The mean tumor diameter and the operation time were (5 ± 3) cm (range,1.0-11.5 cm) and (113 ± 56) minutes (range,50-310 minutes),respectively.Intraoperative hepatic portal occlusion was performed on 122 patients,and the time for hepatic portal occlusion was (15 ± 7)minutes.The volume of intraoperative blood loss was (211 ± 195)mL (range,10-650 mL),and blood transfusion was not needed.The capsule of the tumor was complete.The distance between the resection margin and the malignant tumor was above 1.5 cm,and there was no residual tumor in the resection margin.The hepatic function was back to the normal level in 1 week after the operation,and no patient had hepatic failure.The duration of postoperative hospital stay was (7.2 ± 1.3)days (range,5-10 days).One patient was complicated with bile leakage,6 with slight peritoneal effusion,and other patients had no postoperative complications.The rate of follow-up was 91.47％ (236/258),and the time of follow-up was (16 ± 10) months.A total of 199 patients with malignant hepatic tumors were followed up.During the follow-up,180 patients had tumor-free survival; 18 patients had postoperative tumor recurrence; 1 patient had omental metastasis and received surgical resection.Thirty-seven patients with benign hepatic tumor survived without complication during the follow-up.Conclusion Laparoscopic hepatectomy is effective for the treatment of hepatic tumors.Multiple hepatic inflow occlusion under laparoscope in a short time may improve the safety of surgery,without prolonging the recovery time of patients.

Objective To calculate the residoal liver volume using a virtaal liver surgery planning system,and to investigate the value of standardized estimated liver remnant volume ratio (STELR) in prcdicting hepatic dysfunction after hepatectomy.MethodsThe clinical data of 76 patients with primary liver cancer who were admitted to the First Affiliated Hospital of Fujian Medical University from April 2007 to October 2011 were retrospectivcly analyzed.The virtual resection and residual liver volume measurements were carried out using Liv 1.0 software.The value of STELR in predicting hepatic dysfunction was assessed using receiver operator characteristic (ROC) curves.Effects of different risk factors on postoperative hepatic dysfunction were analyzed using univariate analysis of variance and multivariate Logistic regression models. Results The mean residual liver volumes predicted by the software and resected during operation were (489 ± 206)ml and (459 ± 199 )ml,respectively,with a positive correlation between predicted and actual resection volumes (r =0.916,P ＜ 0.05).Of the 76 patients,48 had mild hepatic dysfunction,19 had moderate hepatic dysfunction and 9 had severe hepatic dysfunction.A critical STELR of 53％ was associated with severe hepatic dysfunction.Severe hepatic dysfunction was detected in 2 out of 59 (3％) patients with STELR ≥ 53％ and 7 out of 17 (41％) patients with STELR ＜ 53％,which represented a significant difference ( x2 =5.085,P ＜ 0.05 ).The result of univariate analysis revealed that STEL,R,operating time,intraoperative blood loss were significant prognostic indicators for severe hepatic dysfunction ( F =7.227,8.630,13.809,P ＜0.05).Multivariate Logistic regession revealed that STELR was a significant independent predictor of severe hepatic dysfunction ( Wald =6.675,P ＜ 0.05 ).Conclusion The likelihood of severe hepatic dysfunction after hepatectomy can be predicted by STELR.

AIM:To generate thalassemia-specific integration-free induced pluripotent stem cells ( iPSC) and to detect their ability of differentiation into hematopoietic precursors .METHODS:The plasmids pEB-C5 and pEB-Tg were transfected into the fibroblast cells from hemoglobin Bart ’ s hydrops fetalis ’ s skin by the method of nuclear transfection to reprogramm the cells into iPSC .The ability of the iPSC to differentiate into 3-germ layer cells was determined .The iPSC were cocultured with mouse OP 9 cells to differentiate into hematopoietic precursors and the hematopoietic precursor specific antigens were detected .RESULTS:The integration-free iPSC from hemoglobin Bart ’ s hydrops fetalis ’ s skin fibroblasts were successfully derived, and had the ability to differentiate into 3 germ layers.When cocultured with OP9 cells for 9 d, the positive rate of hematopoietic progenitor cell marker CD 34 was 18.7%, and the CD34 and CD45 double positive rate was 12.2%.CONCLUSION:Hemoglobin Bart ’ s hydrops fetalis ’ s skin fibroblasts can be successfully induced into “in-tegration-free” iPSC.This cell line has the ability to differentiate into 3 germ layers , and can be differentiated into hemato-poietic precursors when cocultured with OP 9 cells.

Objective To investigate the acid α glucosidase(GAA)gene mutations and clinical features of a Chinese patient exhibiting signs and symptoms of infantile glycogen storage disease type Ⅱ(GSD Ⅱ). Methods Clinical features of the patient were reviewed,and GAA activity in the patient＇s and her parents＇ whole leukocytes were measured. GAA coding regions were amplified by polymerase chain reaction(PCR),and analyzed by direct DNA sequencing. Results The patient showed feeding difficulties,generalized hypotonia and weakness starting at 2 months of age. Cardiomegaly and cardiomyopathy were found at 4 months. She died of cardiorespiratory failure at the age of 6 months. GAA activity in leukocytes was low in the patient(17.3% of the median normal range). Genotyping revealed the patient was a heterozygote for a novel nonsense mutation p.W738X and a previously reported nonsense mutation p.E888X. The reported pseudodeficiency allele c.1726G ＞ A;2065G ＞ Awas found in the patient and her mother. Conclusions Correct diagnosis was made for this patient by combination of GAA activity assay and genetic analysis. From the clinical course,this patient should be classified as infantile type of GSD Ⅱ,suggesting that the novel mutation p.W738X may have a damaging effect on the function of GAA. Pseudodeficiency allele found in this family highlights the importance of genetic analysis of GAA when performing diagnosis and prenatal diagnosis for the affected families,as this allele causes low GAA activity in normal individuals.

Objective To study the factors influencing prognosis in the patients with recurrent glioblastoma muhiforme (GBM) and to investigate the effect of retreatemt.Methods The retrospective analysis method was adopted to collect the clinical and follow up data in 36 cases of recurrent GBM retreatment in the neurosurgery department of this hospital from March 2008 to March 2013.The prognosis influencing factors were analyzed.Results The univariate analysis results showed that the gender,resection degree,treatment mode and initial scheme had the influence on the progression free survival(P＜0.05).The resection degree had an impact on the overall survival(P＜0.05).The multivariate analysis results showed that KPS score,resection degree and treatment mode had effect on the progression free survival(P＜0.05).The resection degree had an influence on the overall survival (P＜0.05).Conclusion If the patients with recurrent GBM still hasthe chance of operation whole excision,the re-treatment can reach the effect for relieving the symptoms,improving the quality of life and prolonging the survival period.

Langerhans cell histiocytosis (LCH) is a group of unexplainable abnormal proliferation and aggregation of Langerhans cell. LCH can be classified into four clinical variants: Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma, and congenital self-healing LCH. LCH is most prevalent in children. Lesions can be localized in a single system or multiple organs, and clinical manifestations vary depending on the affected organs. The skin and mucocutaneous tissues are the starting point of the affected tissue. This study presents a LCH case characterized by transient self-healing. This case can further provide references for the clinical diagnosis and treatment of LCH.
Child ; Humans ; Histiocytosis, Langerhans-Cell/therapy* ; Diagnosis, Differential