Objective To investigate the toxicities of methotrexate in the treatment of the children with acute lymphoblastic leukemia Method 28 children with acute lymphoblastic leukemia were randomly divided into two groups(A and B), they were treated with methotrexate 3.0 g/m 2 (n=16) and 5.0 g/m 2(n=12). The toxicities of two doses of methotrexate were observed. Results The main toxicities of methotrexate were marrow suppression,gastrointestinal reaction and liver damage. They were mostly mild.No statistically significant difference was found between group A and group B .Conclusion The toxicities of methotrexate are common and can be tolerated.

Objective To summerize our experience in the diagnosis and treatment of pancreatic cystic neoplasms.Methods A retrospective analysis was conducted on the clinical data of 126 patients with pancreatic cystic neoplasms seen between January 2003 and December 2012 in the Fuzhou General Hospital.Results There was no special clinical manifestation in this series of 126 patients with pancreatic cystic neoplasms.The diagnostic accuracies of ultrasound,CT and MRI were 90.8％ (109/120),93.4％ (114/122) and 96.3％ (103/107) respectively.Eighteen and ten patients were observed to have elevated serum CA19 9 and CEA respectively.One hundred and five patients received conventional open surgery,while 21 patients underwent laparoscopic operations.The operations in cluded enucleation of tumor (n=11),pancreatic segment resection (n=5),spleen preserving distal pancreatectomy (n=30),distal pancreatectomy (n=26),duodenum-preserving pancreatic head resection (n=4),pylorus-preserving Whipple resection (n=5),Whipple procedure (n=38),total pancre atectomy (n=2) and exploratory laparotomy and biopsy (n=5).The perioperative mortality rate was 0.8％ (1/126).Pancreatic fistula (B and C) occurred in 16 patients (12.7％,16/126),and postoperative hemorrhage (B and C) occurred in 9 patients (7.1％,9/126).One hundred and fourteen patients were followed up from 6 to 72 months (median 52.4 months).The five-year survival rates for all the pancreatic cystic neoplasms,non-invasive and invasive neoplasms were 80.5％,96.4％ and 40.7％ respectively.Conclusions Pancreatic cystic neoplasms are a rare subset of pancreatic tumor,being increasingly detected due to the widespread use of abdominal imaging and improved imaging techniques.Most of them are benign,but some are malignant or they have malignant transformation and metastatic potential.Patients with asymptomatic benign pancreatic cystic neoplasms,especially small size,are candidates for observation.Patients with clinical symptoms or having a high suspicion of malignancy should be offered surgical resection.Non-invasive neoplasms should receive pancreatic preserving operations.Pancreatectomy plus regional lymph nodes dissection with or without combined resection of adjacent multi organs should be adopted for invasive neoplasms.

Objective To study the anatomy of the caudate lobe and to apply the knowledge in caudate lobe resection and piggyback liver transplantation. Methods Dissection of the caudate lobe was carried out in 35 fresh adult livers. Before dissection, the portal venous system was injected with barium sulfate emulsion to show the portal branches of the caudate lobe. Results The caudate lobe was shown to consiste of 3 portions: (1) the Spigelian lobe, the paracaval portion and the caudate process. The Spigelian lobe was pyramidal in shape with 3 faces, a peak and a base; (2) the paracaval portion was situated to the right and to the front of the vena cava; (3) the caudal process was the liver parenchyma between the hepatic hilum and the anterior aspect of the vena cava. Conclusions The entire caudate lobe is situated at the posterior part of the liver and it surrounds the vena cava. The key point in resecting the entire caudate lobe or in performing a piggyback liver transplantation is adequate mobilization and division of all the outflowing veins of the caudate lobe.

Objective:To elucidate the role of ERK1/2 MAPKs signal transduction in the protective effects of ischemic preconditioning during liver transplantation. Methods: Twenty-four rats were equally randomized into 4 groups: sham control (group A); liver transplantation (group B); ischemic preconditioning+liver transplantation (group C), and MEK inhibitor intervention+ischemic preconditioning+liver transplantation (group D). Serum AST and ALT were detected after operation and cellular ultrastructures were observed by transmission electron microscopy. Liver tissue ERK1/2 MAPKs phosphorylation activities were evaluated through determining ERK-1 protein phosphorylation by Western blotting. Results: Serum ALT and AST activity in group B and group D was significantly higher than those in group A, and remained normal in group C. Liver tissue ERK1/2 MAPKs phosphorylation activity increased significantly in group C, and the activation effect was inhibited in group B and D. Cellular ultrastructure was obviously damaged in group B and group D, and the damage was prevented in group C.Conclusion: Ischemic preconditioning has a protective effect on transplanted liver cells in which ERK1/2 MAPKs pathway plays a pivotal role.

A 33-year-old male patient complained of presenting goiter on the low back area for 2 months. Pathological examinations of resected goiter suggested non-Hodgkin lymphoma and showed that T cells, immunoblasts, and hemogram were roughly normal, and 2% sarcoma cells could be found in bone marrow. Stage Ⅳ T-cell non-Hodgkin's lymphoma was diagnosed. Following 4 months of chemotherapy using CHOP protocol (cyclophosphamide, adriamycin, vincristine, and prednisone included), the patient underwent bone marrow activation and autologous peripheral blood stem cell transplantation in combination with recombinant interleukin-2 application in April 1998. The preprocessing was performed under MACC protocol (L-sarcolysinum, cytarabine, cyclophosphamide, and Iomustine included). Ten days after autologous stem cell transplantation, neutrophil concentration was > 0.5×109/L and sixteen days after transplantation, blood platelet concentration was > 50×109/L. Six days after transplantation, the patient exhibited fever, and E. Coli infection was confirmed through blood culture. After antibiotic treatment, body temperature recovered to normal, and fever disappeared. The patient had been followed-up for 10 years and 10 months. During the follow-up period, he lived a normal life and work.

Objective To identify the types of pulmonary complications after pancreaticoduodenectomy,and to discuss the prevention and management of these complications.Methods Clinical data of 165 cases of pancreaticoduodenectomy in our hospital were retrospectively analyzed.Pulmonary complications were identified,therapeutic effects were observed.Results The incidence rate of pulmonary complications was 19.4％ ( 32/165),case-fatality rate was 6.25％ (2/32),two patients died from pneumonia,respiratory failure and ARDS.Complications mainly included pneumonia 13.9％ (23/165),pleural effusion 4.2％ (7/165),atelectasis 3.6％ (6/165),pneumothorax 1.8％ ( 3/165 ),respiratory failure 2.4％ (4/165) and ARDS 1.2％ (2/165).Conclusion Pulmonary complications after pancreaticoduodenectomy are not rare,especially for pulmonary infection and most are hospital acquired pneumonia.To understand rules and particularity of respiratory physiopathological changes after pancreaticoduodenectomy is very important for patients to safely pass over the perioperative period.

Objective To investigate the clinical characteristics, diagnosis, treatment and prognosis of pancreatic gastrointestinal stromal tumor (GIST). Methods We reported a case and reviewed the medical literature on pancreatic malignant GIST. We searched the Pubmed and main domestic database. The clinical data of the reported cases were studied, and their predictive factors for postoperative recurrence and metastasis were analyzed. Results Between January 1980 and July 2010, 16 cases of pancreatic GIST were reported. There were 7 males and 9 females, with a median age pf 56.5 (31-72)years. The clinical symptoms were nonspecific. The main presentation was upper abdominal pain or discomfort. A preoperative diagnosis was suspected on radiological examination. The tumor mainly appeared as a well-defined solid-cystic mass. Irregular enhancement appeared in the circumferential and solid portion of the tumor on enhanced CT scan sequences. The pancreatic and biliary ducts were rarely dilated. Endoscopic ultrasound guided fine needle aspiration cytology (EUS-FNA) was helpful in preoperative diagnosis. Of the 15 surgical patients, 14 underwent complete resection, while the remaining received cyst-jejunostomy. A correct diagnosis was made on histopathology and immunohisto-chemistry. On a mean follow up of 21 months (range, 1-60) in 14 patients, all patients were alive.Recurrence or metastasis occurred in 4 patients with tumors of high malignant potential. On univariate analysis, the only significant predictor for adverse outcome was mitoses≥10/50 HPF. Conclusions Pancreatic GIST is a rare tumor of relatively low malignant potential. It has a better prognosis than ductal adenocarcinoma. It is important to arrive at a correct diagnosis and treat the tumor with radical resection. Aggressive surgical resection is potentially curative. Imatinib is recommended in the treatment of patients with tumors with high malignant potential.

Objective To evaluate the efficacy of preoperative regional intra-arterial chemotherapy (RIAC) in the treatment of resectable pancreatic head carcinoma. Methods The clinical data of 50 patients with resectable pancreatic head carcinoma who had been admitted to the Research Institute of Pancreatic Diseases of Fudan University from December 2006 to July 2007 were retrospectively analyzed. Patients were randomly divided into2 groups (n =25 in each group): patients in group A were treated with preoperative RIAC followed by regional pancreaticoduodenectomy, and patients in group B were treated with surgical procedure routinely. The lymphatic metastases in the 50 specimens of pancreatic head carcinoma were detected by histological examination with hematoxylin and eosin (HE) staining, and lymphatic micrometastases were detected by immunohistochemical method with staining of cytokeratin AE1/AE3 in 10 specimens with negative HE staining of the lymph nodes in each group. Results There was no significant difference in the incidence of complications, the length of hospital stay and the 1-, 2-year survival rates between the 2 groups (χ2 = 0.12, 2.88, P > 0.05). The incidence of positive lymph node metastasis in group A was 7.1% (52/734), which was significantly higher than 22.1% (118/532) in group B (χ2 = 60.01, P < 0.05). The incidence of lymphatic micrometastasis was 9.4% (30/319) in group A, and 9.1% (23/252) in group B, with no statistical difference between the 2 groups (χ2= 0.01, P > 0.05). Conclusions Preoperative RIAC is helpful in improving the prognosis of patients with resectable pancreatic head carcinoma by reducing the incidence of lymphatic metastasis and decreasing tumor stage.

BACKGROUND: Triggering receptor expressed on myeloid cells-1 (TREM-1) is a cell surface receptor expressed on neutrophils and monocytes. TREM-1 acts to amplify inflammation and serves as a critical mediator of inflammatory response in the context of sepsis. To date, the predisposition of TREM-1 gene polymorphisms to septic shock has not been reported. This study was designed to investigate whether TREM-1 genomic variations are associated with the development of septic shock. METHODS: We genotyped two TREM-1 single nucleotide polymorphisms (SNPs, rs2234237 and rs2234246) and evaluated the relationships between these SNPs and septic shock on susceptibility and prognosis. RESULTS: TREM-1 rs2234246 A allele in the promoter region was significantly associated with the susceptibility of septic shock in recessive model (AA, OR=3.10, 95％Cl 1.15 to 8.32, P=0.02), and in codominant model (AG, OR=0.72, 95％Cl 0.43–1.19, P=0.02; AA, OR=2.71, 95％Cl 1.00–7.42;P=0.03). However, in three inherited models (dominant model, recessive model, and codominant model), none of the assayed loci was significantly associated with the prognosis of septic shock. The non-survivor group demonstrated higher plasma IL-6 levels (99.7±34.7 pg/mL vs. 61.2±26.5 pg/mL, P<0.01) than the survivor group. Plasma concentrations of IL-6 among the three genotypes of rs2234246 were AA 99.4±48.9 pg/mL, AG 85.4±43 pg/mL, and GG 65.3±30.7 pg/mL (P<0.01). The plasma concentrations of IL-6 in patients with AA genotypes were significantly higher than those in patients with GG genotypes (P<0.01). CONCLUSION: TREM-1 genetic polymorphisms rs2234246 may be significantly correlated only with susceptibility to septic shock in the Chinese Han population.

OBJECTIVETo explore the clinical feature, imaging and their diagnostic value for Joubert syndrome (JS).

METHODThe clinical data, imaging feature, and 31 references from China Biomedical literature database (CBMdise) were reviewed and analyzed.

RESULTThe age of onset of 32 patients including male 20 and female 12 ranged from 3 days to 6 years (mean 2.2 years). All the 32 patients with Joubert syndrome showed "slow growth" and "reduced muscle tension", 26 cases (81.3%) showed "gasp for breath", 26 cases (81.3%) showed "unusual motion of eyeball", 2 cases (6.3%) showed additional fingers (toes), 6 cases (18.8%) showed stretching tongue with agape. The typical imaging features of Joubert syndrome included "molar tooth sign", "midline cleavage" between cerebellar hemispheres and "bat-wing" like fourth ventricle, all the 32 patients with Joubert syndrome showed "midline cleavage", "molar tooth sign" was present in 29 cases (90.1%), and "bat-wing" like fourth ventricle in 30 cases (93.8%).

CONCLUSIONJoubert syndrome is a rare congenital brain malformation. The typical clinical manifestations included "gasp for breath", "reduced tension of muscle", "slow growth" and "unusual motion of eyeball", and at the same time the patients had the following typical imaging features of brain: "molar tooth sign", "midline cleavage" and "bat-wing" like fourth ventricle.

Abnormalities, Multiple ; Cerebellar Diseases ; diagnosis ; physiopathology ; Cerebellum ; abnormalities ; Child ; Eye Abnormalities ; diagnosis ; physiopathology ; Female ; Humans ; Kidney Diseases, Cystic ; diagnosis ; physiopathology ; Male ; Retina ; abnormalities ; physiopathology