The etiology of inflammatory bowel disease(IBD) is complex and multifactorial.Recent genetic association studies within large IBD cohorts have identified 163 genetic loci.But these 163 loci in total explain 13.6％ of Crohn's disease and 7.5％ of ulcerative colitis total disease variance.The classification defines very early-onset IBD as diagnosis before the age of 5 years old,and also before the age of 6 years old in some research.It is likely that genetic factors contribute more to the etiology of very early-onset IBD.Recently some cases have been reported,which have linked to very early-onset IBD,such as IL-10 and IL-10 receptor gene mutation,immune dysregulation,polyendocrinopathy,enteropathy,X-linked syndrome,X-linked lymphoproliferative syndrome,IL-21 deficiency,mevalonate kinase deficiency,and so on.And many of these diseases are classified as primary immunodeficiencies.This paper introduces these series of very early-onset IBD in brief.

Systemic lupus erythematosus(SLE) is an autoimmune inflammatory disease,virtually every system and organ can be affected.Gastrointestinal manifestations of SLE attract far less attention than the other major organ involvements.Gastrointestinal manifestations of these diseases often can't be diagnosed early and correctly,because the clinical manifestation varies and often nonspecific,and often confused with the side effect of medicine.But SLE related gastrointestinal involvement is clinically important because some cases can be life-threatening if not treated promptly.This paper reviews the common SLE gastrointestinal involvements,including the diagnosis and treatment of esophageal and gastric involvement,lupus mesenteric vasculitis,lupus pancreatitis,lupus peritonitis,pseudo-obstruction,proteinlosing gastroenteropathy,and hepatic involvement,to improve the recognition of gastrointestinal manifestations,and make an early and accurate diagnosis so that appropriate treatment along with prognosis can be made.

Children systemic rheumatic diseases are characterized by multisystem involvement,the underlying pathogenesis for gastrointestinal manifestations is deposition of immune complexes in blood vessel walls resulting in ischemia or thrombosis.Gastrointestinal manifestations of these diseases often can't be diagnosed early and correctly,because children can't demonstrate the clinical features accurately,the clinical manifestation varies and often nonspecific,and often confused with the side effect of medicine.Gastrointestinal manifestations are considered rare,comparing with other system involvement,but the consequence can be life-threatening.This makes early and immediate diagnosis and management very important.This paper reviews the various children systemic rheumatic diseases that affect the gastrointestinal tract,including systemic lupus erythematosus,Henoeh Schonlein Purpura,Bechet's disease,mixed connective tissue disease,scleroderma,polyarteritis nodosa,Wegener's granulomatosis,Sjogm's syndrome,dermatomyositis.

Objective To summarise the clinical data of neonatal early onset sepsis (EOS) and investigate the correlation factors, clinical manifestation, diagnosis, therapy and prognosis of EOS. Methods Data of 32 neonatal EOS patients admitted into the neonatal intensive care unit, Peking Union Medical College Hospital from January 2000 to June 2009 were collected and retrospectively analyzed. Results Among 32 EOS infants, there were 23 preterm infants (71.9%), nine term infants (28.1%); 21 low birth weight infants (65.6%), six very low birth weight infants (18.8%) and one macrosomia (3.1%). Among 32 mothers, 27 (84.4%) were accompanied with various kinds of complications during perinatal period, such as 15 perinatal infection (46.9%), six preeclampsia (18.8%), five gestational diabetes mellitus (15.6%) and one hypothyroidism (3.1%). EOS infants had various clinical manifestations, including 25 low response (78.1%), 20 respiration or temperature abnormity (62.5%), 18 pallor and clammy skin (56.3%), 18 feeding intolerance (56.3%), 18 fever (56.3%), 15 metabolic acidosis (46.9%), 8 infectious shock (25.0%), 20(62.5%) high white blood cell count (>25×109/L), 22 (68.8%) low blood platelets (<100×109/L) and 28 (87.5%) high C-reaction protein (>8 mg/L). Blood culture of 24 infants were positive (75.0%), among which nine infections were caused by gram-positive bacteria (9/24, 37.5%), including Listeria monocytogenes, group B Streptococcus, Staphylococcus, et al; 15 infections were caused by gram-negative bacteria (15/24, 62.5%), including Klebsiella Pneumoniae, Enterobacteria, Bacillus Smaragdinus, et al. Antibiotics were used in all infants when EOS was supposed to be or infectious symptoms were presented, and were adjusted under the results of culture. Twenty-two infants (68.8%) were cured, eight(25.0%) were given up from the therapy, two(6.3%) died. Conclusions The neonatal EOS correlates to various kinds of perinatal factors, its clinical manifestations are complicated and usually involves many systems. Multiple factors in perinatal period, clinical manifestation and laboratory examinations should be considered to make early diagnosis, assist management to improve the prognosis.

Liver cirrhosis is the end stage of various chronic liver diseases.Early diagnosis and staging of liver cirrhosis,as well as effective disease assessment,are of great clinical significance.The application of liver biopsy,serological testing,and radiological examination in the clinical diagnosis of liver cirrhosis and the research advances in evaluation of liver reserve function,complications,and prognosis are re-viewed.It is pointed out that with the development of molecular biological and imaging techniques,the accuracy and specificity of noninva-sive diagnosis will be increased,and the disease assessment system will be improved.

Objective To evaluate the long-term effects of postnatal dexamethasone administration in prevention or treatment of chronic lung disease (CLD) in preterm infants.Methods All randomized controlled clinical trials (RCT) on long-term effect of postnatal dexamethasone in prevention or treatment of chronic lung disease in preterm infants were searched in Medline,Embase database,Cochrane Library and Chinese Medical Citation Index database,China National Knowledge infrastructure,VIP Chinese Periodical Database and Chinese Digital Hospital Library and Wanfang Chinese periodical database.As a supplementation,the works of domestic and international pediatric conferences were collected by manual search.The follow-up time was defined as longer than two years.Revman 5.0 was used for Meta-analysis for the effects of dexamethasone on nervous system,respiratory system and mortality.Results Eight RCTs fulfilled the including criteria.Statistical analysis revealed that the incidence of recurrent respiratory disease decreased from 49.7％ to 38.9％ in the dexamethasone group compared to the control group with RR =0.57 (95％ CI:0.37-0.88,P ＜ 0.05).But the incidence of cerebral palsy in dexamethasone group increased significantly (RR =1.95,95％ CI:1.41-2.71,P ＜ 0.01).Stratified analysis revealed that the incidence of cerebral palsy in early-use group (within 1 week after birth) was higher than that in control group (RR =2.59,95％ CI:1.59-4.21,P ＜0.01) ; while the incidence in later-use group (1 week after birth) was not statistically different from that in control group (RR =1.5,95％ CI:0.95-2.36,P =0.08).There were no significant differences in the incidence of mental retardation (RR =0.77,95％ CI:0.28-0.14,P ＞ 0.05),deafness (RR =0.52,95％ CI:0.17-1.56,P =0.24) and severe visual impairment (RR =1.56,95％ CI:0.77-3.18,P =0.22) between dexamethasone group and control group,as well as in mortality rate (RR =0.91,95％ CI:0.71-1.16,P ＞ 0.05).Conclusions Intravenous administration of dexamethasone after birth for prevention or treatment of CLD in preterm infants can reduce the incidence of recurrent respiratory diseases in childhood; meanwhile the incidence of cerebral palsy may increase,it almost occurs in the early dexamethason administration group,not in the late group.Dexamethasone may not reduce the mortality of CLD.

AIM:To investigate the efftct of Gypenosides on contraction of isolated duodenum smooth mustle of rabbits.METHODS:The routine experimental method of isolated duodenal smooth muscle of rabbits were used.The contraction curves of isolated duodenum smooth mustle before and after administration of different concentrations of Gypenosides(0.01%-1.00%)were recorded by Medlab biological information collecting system.RESULTS:Different concentrations of Gypenosides significantly reduced the contraction amplitude of isolated duodenum smooth mustle of rabbits,and showed a dose-dependent relationship.CONCLUSION:Gypenosides can inhibit the contraction of isolated duodenum smooth muscle of rabbits.

The general data of 48.978 burn cases collected from 16 institutes and burn center in a period of 22 years (1958-1979) were analysed. It was found that the highest incidence of burns occurred in summer and before age of 30 (78.1%). particularly in the age group of 20-24 and under 5(22.97% and 22.95% respectively). Thermal burn was the commonest cause (89.57%). The ratio between female and male was 1:3. The ratio between cases with and without third degree burns was also 1:3. The burn extent was predominently under 30% TBSA (85.10%). cases with burn larger than 70% TBSA occupied less than 3% of the total. The exposed parts of the body. i.e. head, neck and limbs, were the prevalent areas affected. The mean hospitalization period was 26.9 days. The overall mortality rate was 4.93% and LA50 75.93%(Y = 7.5588-0.0337X). The incidences of shock and sepsis were 10.85% and 7.95% respectively. It was also found that the mortality rate (in terms of LA50). and incidences of both shock and sepsis have lowered remarkably (p

Objective To summarize and analyze the impact factors on enteral feeding in very low birth weight infants (VLBWI) and extremely low birth weight infants (ELBWI). Methods A retrospective study was carried out in VLBWI and ELBWI who had achieved full enteral feeding prior to discharge. The impact factors correlated to the time of achieving full enteral feeding were analyzed. If the data underwent bi-variable normal distribution, they were analyzed with Pearson correlation test; otherwise they would be analyzed with Spearman correlation test. T test was used for single factor analysis and multiple linear regression analysis was carried out to determine the significant risk factors associated with the time of achieving full enteral feeding. Results One hundred and forty-seven infants with mean gestational age of (31.0±2.0) weeks, mean birth weight of (1246±185) g and mean time of achieving full enteral feeding of (24. 4± 10. 5) days were admitted. With the single factor analysis, it was found that birth weight (r=- 0. 477, P = 0. 000), gestational age (r = - 0. 405, P= 0. 000), mechanical ventilation duration (r= 0. 393, P = 0. 000), the time began to enteral feeding (r = 0. 318, P = 0. 000), initial milk volume (r = - 0. 263, P = 0. 001 ), the milk volume on the third day (r= -0. 412, P=0. 000) and the seventh day (r= -0. 592, P=0. 000),neonatal respiratory distress syndrome (t = 3. 368, P = 0. 001), umbilical catheterization (t = 3. 571,P=0. 000), abnormal blood glucose level (t=3. 285, P=0. 001), aminophylline using (t=4. 341,P=0. 000), phototherapy (t=3. 054, P=0. 003) and sepsis (t=3. 244, P=0. 001) were correlated to the time of achieving full enteral feeding. Multiple linear regression showed that the birth weight (t=4. 175, P= 0. 000), the time began to enteral feeding (t= 2. 851, P = 0. 005), aminophylline using (t=2. 231, P=0. 027), sepsis (t=3. 895, P=0. 000), phototherapy (t=2. 852, P=0. 005)and the milk volume on the seventh day (t= 7. 332, P=0. 000) were significantly correlated with the time of achieving full enteral feeding. Conclusions The enteral feeding of VLBWI and ELBWI was not only influenced by maturity of gastrointestinal tract, but also by other parenteral correlation factors. Multiple factors associated with all around clinical conditions should be considered when providing enteral feeding for VLBWI and ELBWI.

A neonatal girl with overextended knees admitted to NICU of our hospital was diagnosed as cri du chat (cat cry) syndrome.We collected 34 cases of cri du chat reported in journals since 2000,the clinical features of total 35 cases were retrospectively analyzed.Among 35 cases 12 were boys and 23 girls.The most common clinical manifestations were characteristic face features(100％),difficult feeding(100％) and typical sound of cry(94％).The main complains at hospital visit were typical cry,difficult feeding and cyanosis in the neonatal period,while in childhood period were recurrent respiratory infection,developmental retardation and other abnormalities.Most cases were diagnosed in the neonatal phase,while 85.3％ were in the first year.The diagnosis was based on karyotype analysis; chromosome 5 short arm deletion (5P-) was the most significant genetic variation and clinical features were associated with the position of deletion.