Objective To study the clinical manifestations,diagnosis and surgical treatment of tubular adenoma in the extrahepatic bile duct.Methods The clinical data of 3 patients with tubular adenoma in the extrahepatic bile duct who were admitted to the Affiliated Hospital of Inner Mongolia Medical University from July 2010 to February 2012 were retrospectively analyzed.Results The tumors were located at the middle and lower part of the common bile duct.All the 3 patients received resection of the common bile duct and Roux-en-Y cholangioenterostomy,and recovered well.The results of pathological examination showed that all the patients were with tubular adenoma,and 2 patients had moderate atypical hyperplasia.Conclusions Tubular adenoma in the extrahepatic bile duct could be diagnosed by pathological examination.Radical resection and rerouting of the bile duct can get a better outcome.

The clinical data, laboratory testing, genetic testing results, diagnosis and treatment process of a child with PERCHING syndrome diagnosed and treated in the Department of Neonatology, the Third Affiliated Hospital of Zhengzhou University in June 2022 were retrospectively analyzed, and the relevant literatures were reviewed.The proband mainly presented with dyspnea and feeding difficulties after delivery, facial nevus flammeus, protrusion of eyes, small fissure of eyes, wide nasal root, limited opening of mouth, slightly high palatal arch, special posture, cryptorchid, hypospadias, and high muscle tone of limbs.Magnetic resonance imaging of the brain suggested possible agenesis of corpus callosum.Genetic testing showed complex heterozygous variations in the KLHL7 gene, and the two mutation sites have not been previously reported.A case of PERCHING syndrome caused by the KLHL7 gene mutation in China was reported for the first time, which provided new ideas for the diagnosis and treatment of children with PERCHING syndrome and reliable genetic evidence for family reproduction.

Objective: To investigate the protective effect of vitamin D3 on alcoholic liver injury in mice. Methods: Forty mice were randomly divided into 4 groups : normal Control (Control) group , vitamin D3 (VitD3 ) group , alcohol model (EtOH) group and alcohol + vitamin D3 (EtOH + VitD3 ) group. The mice were fed with the DeCarlialcohol liquid diet to establish alcoholic liver injury model. Serum levels of alanine aminotransferase (ALT) , aspartate aminotransferase (AST) and liver index were detected. HE staining was used to observe the pathological changes of liver. The relative expression levels of tumor necrosis factor α (TNF⁃α ) , transforming growth factor β (TGF⁃β) , interleukin⁃6 (IL⁃6) and interleukin⁃1β (IL⁃1β) mRNA were detected by quantitative real⁃time PCR (qRT⁃PCR) . The expressions of nuclear factor⁃kappa B (NF⁃κB) p65 and NF⁃κB p50 in liver were detected by Western blot. Results: The serum ALT , AST vitality , liver index and hepatic TNF⁃α , TGF⁃ β , IL⁃6 and IL⁃1β mRNA in EtOH group were significantly higher than those in Control group. EtOH group disorganized hepatocyte and hepatic lobules boundary was not clear, and the hepatocytes showed apparent inflammatory cells infiltration of liver cells and fat cavitation. NF⁃κB p65 and NF⁃κB p50 protein expression increased significantly. Compared with EtOH group ,the serum ALT , AST vitality , liver index and hepatic TNF⁃α , TGF⁃ β , IL⁃6 and IL⁃1β mRNA in EtOH + VitD3 group decreased significantly. The pathological staining results showed that inflammatory cells infiltration and decrease in the number of fat vacuoles , and the liver cells returned to normal liver cell structure. At the same time the NF⁃κB p65 and NF⁃κB p50 protein expression level decreased significantly. Conclusion Vitamin D3 has a certain protective effect on alcohol⁃induced liver injury in mice , and its main mechanisms are related to the inhibition of NF⁃κB pathway and the reduction of inflammatory response.

OBJECTIVETo observe the biological characteristic and the prognoses in patients with acute erythroleukemia (AEL).

METHODSThe results of 167 patients with newly diagnosed AEL, from January 2003 and June 2013 in the First Affiliated Hospital of Soochow University, were reviewed by MICM.

RESULTSFlow cytometry analysis indicated that CD13(96.1%), CD33(95.1%), CD117(87.4%) and CD34 (79.4%) were highly expressed in AEL. 56 of 148 (37.8%) AEL patients had a variety of cytogenetic abnormalities, 27 of 148(18.2%) patients were complex karyotype (abnormalities involving 3 or more chromosomes), the abnormalities of chromosomes 3, 5, 7 and 8 were more frequently involved and the most common one was ＋8, accounting for 35.7% of all abnormal karyotype, followed by 5q- (17.9%). Mutation analysis showed CEBPA mutation ratio of AEL patients was 44.0% (11/25), that of NPM1 as 15.4% (4/26). Initial induced remission rate of AEL was 56.6% (30/53), compared by 33.3% (4/12) of MDSM6. Survival analysis showed that the overall survival in female was better than that in male (P=0.047). The overall survival time of transplantation group is significantly longer than chemotherapy group (P=0.000). The OS of 13-39 years old group was the best, 40-49 years old group took second place, >50 years old group appeared to be the worst.

CONCLUSIONAEL had its own unique biological features, and allogeneic hematopoietic stem cell transplantation could significantly improve its poor prognosis.

Adolescent ; Adult ; Female ; Humans ; Leukemia, Erythroblastic, Acute ; diagnosis ; Male ; Middle Aged ; Prognosis ; Remission Induction ; Young Adult

【Objective】 To reduce the incidence of postoperative intestinal obstruction, we tried to improve surgical techniques by closing the cavity formed during radical cystectomy + ileal passage (Bricker) via laparoscopy to prevent the formation of abdominal hernia. 【Methods】 During Oct.2018 and Feb.2022, 41 patients were involved (conventional group). After standard laparoscopic radical cystectomy + pelvic lymphadenectomy, the ileum channel was established. The right medial retroperitoneum was sutured to cover the mesothelium and end of the ileum channel under open operation or endoscope. The space between the ureter and mesothelium of the ileum channel was sealed, and the end of the ileum channel and both ureters were externalized. During Feb.2022 and Dec.2022, 15 patients were involved (modified group). The right inner and outer lateral peritoneums below the ileal conduit were sutured to "bottom out" the gap between the ileal conduit and the right abdominal wall in addition to standard procedures. The recovery of intestinal function and incidence of bowel obstruction were compared between the two groups. 【Results】 In the conventional group, the intestinal function recovered within 2 to 6 days after surgery, with a median ventilation time of 3 days. Intestinal obstruction occurred in 3 patients, 2 of whom improved after conservative treatment while 1 underwent surgical exploration after ineffective conservative therapy. There were no significant differences in the time of discharge and ventilation between the two groups, but no intestinal obstruction occurred in the modified group. 【Conclusion】 Peritoneal externalization at the end of ileal passage can reduce the incidence of intra-abdominal hernia and postoperative intestinal obstruction, which is worthy of clinical application.