cuses tumors. Survivin seems to be a promising marker for analyzing clinical stages and predicting the prognosis of TCC.

Objective: To analyze the clinical characteristics of X-linked inhibitor of apoptosis (XIAP) deficient patients with clinical manifestation of Crohn's disease. Methods: Clinical manifestations, laboratory investigations, genetic testing and therapeutic interventions of one case of XIAP deficiency who was admitted to Department of Gastroenterology in Children's Hospital, Zhejiang University School of Medicine in May 2016 were summarized. PubMed and Chinese database for articles published from January 2016 to June 2017 were searched using the key words of'Crohn's disease’and'XIAP’, and the relevant literature was reviewed. Results: The case we reported was a 6-year-1-month-old boy with recurrent bloody stool for 2 months, and abdominal pain with fever for 2 weeks. The patient had a past history of hemophagocytic lymphohistiocytosis (HLH) and epilepsy in the past one year. Complete blood cell count showed mild anemia (Hb108 g/L). The patient had an elevated high-sensitivity C reactive protein (86 mg/L) and erythrocyte sedimentation rate (46 mm/1h) . White blood cells, pus cells and red blood cells were found on routine stool examination. Biochemical panel showed hypoalbuminemia (25.2 g/L) , elevated transaminase (alanine aminotransferase 175 U/L, aspartate transaminase 229 U/L) , hypertriglyceridemia (4.41 mmol/L) , and hyperferritinemia (>1 650.0 μg/L) . Magnetic resonance enterography revealed the intestinal wall thickening and increased enhancement in parts of illeum and colon. Capsule endoscopy revealed multiple ulcers in jejunum. Colonoscopy showed multiple ulcers in colon and the pathological examination revealed chronic inflammation in mucosa of terminal ileum and colon, which was combined with partial necrosis and ulceration. Some phagocytes were seen in bone marrow smears. The patient was given multiple diagnoses, including hemophagocytic lymphohistiocytosis, Crohn's disease, sepsis, epilepsy, severe malnutrition, and hypoproteinemia. The pediatric Crohn's disease activity index (PCDAI) was 37.5. Genetic testing identified a hemizygotic mutation of c.910G>T chrX:123022501 p.G304X in XIAP. The parents had no such mutation. The patient showed response to infliximab with oral intake of mercaptopurine and corticosteroids, and had remission with PCDAI of 0. There were 9 relevant articles (Chinese 0 English 9), which showed 33.3% XIAP deficient patients manifested with inflammatory bowel disease(IBD), who might have other manifestations such as hemophagocytic lymphohistiocytosis or splenomegaly simultaneously or sequentially. Those patients showed poor response to monotherapy. Conclusion XIAP deficient patients have various clinical manifestations. Genetic testing is important to those male pediatric IBD patients who have the complicated symptoms or little response to standard therapy.

Objective: To explore the clinical features and complications of foreign bodies in the upper gastrointestinal tract in children and to investigate the effectiveness of endoscopic management. Methods: Data of patients with foreign bodies in upper gastrointestinal tract were collected retrospectively at Endoscopy Center, the Children's Hospital, Zhejiang University School of Medicine, from January 2011 to December 2016. Clinical characteristics, the types of foreign bodies, the location and duration of foreign body impaction were summarized. The risk factors of complications and endoscopic removal failure were analyzed by using Logistic regression analysis. Results: A total of 1 334 patients (825 males and 509 females) were enrolled. The median age was 2.5 years, with a range from 0.25 to 15 years and peak age 1-3 years. Twenty patients had esophageal diseases. The most common foreign body ingested was coin (n=964, 72.3%). Foreign bodies were most commonly located in the esophagus (n=1 002, 75.1%), especially in the upper esophagus (n=857, 85.5%). The duration of foreign body impaction ranged from 3 hours to 5 years. Among 1 334 patients, 252 patients (18.9%) developed complications, including ulcers (n=101, 40.0%) and perforations(n=13, 5.2%). The success rate of endoscopic removal was 96.6% (n=1 288). By Logistic regression analysis, sharp foreign body ingestion (OR=6.893, 95%CI: 4.421-10.746) , esophageal impaction (OR=5.253, 95%CI:3.352-8.233) and foreign body impaction longer than 24 hours (OR=4.336, 95%CI:3.091-6.082) were risk factors of complications. Sharp foreign body ingestion was the risk factor of endoscopic failure (OR=5.372, 95%CI:2.773-10.406) . Conclusions Coin is the most common foreign body in upper gastrointestinal tract. Sharp foreign bodies impacted in the esophagus over 24 hours increase the risk of complications. Endoscopic removal of foreign bodies from the upper gastrointestinal tract in children has a high success rate. Sharp foreign body ingestion increases the risk of failure in endoscopic removing.

OBJECTIVETo investigate the efficacy of infliximab versus corticosteroids in achieving clinical remission in pediatric patients with Crohn's disease in China.

METHODData of all newly diagnosed active Crohn's disease pediatric cases seen from June 2009 to December 2013 in Children's Hospital, Zhejiang University School of Medicine were retrospectively recorded and reviewed.

INCLUSION CRITERIAthe age of the children was less than 18 years; pediatric Crohn's disease activity index (PCDAI) was more than 10; infliximab or corticosteroids were used for inducing remission; infliximab, immunosuppressive medications or mesalamine was prescribed for maintaining remission. Patients in steroids group were followed up for more than 1 year. The enrolled patients were divided into two groups: infliximab group and steroids group. Clinical data, laboratory findings and side effects of the medications were collected at week 2, 4, 12, 24 and 48. PCDAI and Crohn's disease endoscopic index score (CDEIS) were calculated. Clinical response rate, clinical remission rate, relapse rate, mucosal healing and growth were evaluated.

RESULTEleven children received infliximab therapy and 11 subjects received corticosteroids. In Infliximab group, 6, 5 and 7 patients were in clinical remission at week 2, 4, and 8, while so were 6, 9, and 9 patients in steroids group. The difference was not statistically significant (χ² = 0.00, 3.14, 0.92, P > 0.05). In infliximab group, 8, 8, and 11 patients were in clinical remission at week 2, 4, and 8, so were 8, 9, and 9 patients in steroids group. The difference was not statistically significant (χ² = 0.00,0.26, 2.20, P > 0.05). When compared with data at baseline, significant decreases were observed in the median PCDAI between the two groups at week 2, 4, and 8 (all P < 0.05). But there were no significant differences between two groups at week 2, 4, and 8 (all P > 0.05). At week 12, 24 and 48, 8/11, 7/8, 3/5 cases on infliximab versus 7/11, 9/11, 8/11 cases on steroids maintained remission. There was no significant differences between the two groups (all P > 0.05). In 7 patients and 9 patients remission was successfully induced at week 8. The relapse rate was similar at week 12, 24, and 48 (χ² = 0.83, 0.09, 1.00, all P > 0.05). Height for age Z score in infliximab group was significantly higher than that in steroids group at week 24 (P < 0.05). Body mass index Z score between the two groups at week 8, 24, and 48 were not statistically significant (all P > 0.05). Of the children treated with infliximab, 3 developed side effects. All the children treated with steroids got Cushing's syndrome.

CONCLUSIONIn children with Crohn's disease, infliximab therapy is as effective as corticosteroids to induce remission.Less side effects were observed with infliximab therapy compared with immunosuppressive medication and mesalamine.

Adrenal Cortex Hormones ; therapeutic use ; Antibodies, Monoclonal ; therapeutic use ; Child ; China ; Crohn Disease ; drug therapy ; Humans ; Infliximab ; Remission Induction ; methods ; Retrospective Studies ; Treatment Outcome