Objective To understand the clinical features of children with acute pancreatitis.Method Clinical data of 106 in patients in Children′s Hospital with acute pancreatitis from Jul.1991 to Jan.2005 were analyzed retrospectively.Results The most frequent(etiologic) factors of childhood acute pancreatitis were idiopathic(35.8%),mumps virus infection(33.9%)and biliary tract diseases(15%).Abdominal pain(96.2%),vomiting(72.6%)and fever(45.3%) were the most common clinical symptoms.And the most common signs were abdominal tenderness(73.6%),abdominal wall rigidity(17.9%)and abdominal distention(10.6%).Multisystem damage usually occurred in childhood acute pancreatitis.Serum amylase,urinary amylase and serum lipase elevated 84.8%,58.5% and 55%in patients,respectively.Fifty-one percont patients had abnormal ultrasound imaging and 81% had abnormal computed tomographic imaging.In 5 patients who were subjected to endoscopic retrograde cholangiopancreatography(ERCP) and magnetic resonance cholangiopancreatography(MRCP),the pancreaticobiliary system was visualized clearly.Nonsurgical therapy was done in most patients,only 6 cases received surgical therapy.Sixty-five cases were cured and 40 cases straightened up,only 1 case died.The efficiency rate was 99%.Pseudocysts occurred in 2 patients.Conclusions Idiopathic pancreatitis and mumps virus infection are the most frequent etiologic factors for childhood acute pancreatitis.Symptoms and signs such as abdominal pain and abdominal tenderness are the important diagnostic bases of childhood acute pancreatitis.Childhood acute pancreatitis was always accompanied by multisystem damage.Pancreatic enzyme test and radiography test were helpful in the diagnosis of childhood acute pancreatitis.Nonsurgical therapy is the leading therapeutic means of childhood acute pancreatitis.Timely diagnosis and allopathy help to improve the prognosis of childhood acute pancreatitis.Pseudocyst is the primary complication of childhood acute pancreatitis.

Rhinovirus

Protein fulfilling the their roles, one of important ways is through protein-protein interaction. In functional genomic era, identifying all of protein-protein interaction in proteome and mapping the protein interactions that have been attracting many scientists' attention , of which large-scale yeast two-hybrid system is one strategy of most widely used. In recent two years, ambitious projects have launched to examine all of the protein-protein interaction in Saccharomyces cer-evisiae using large-scale yeast two-hybrid system. Nevertheless, huge protein network is larger than that we predict and single yeast two-hybrid system cannot solve all the problems, which need be complemented by other wags.

0.05). In stratified studies, risk of asthma in individuals with null genotype of GSTM1 is 2.667 times of that with wild genotype after exposure to light air pollution. Risk of asthma in individuals living in heavy air pollution area is 2.125 time of that in light pollution area for all wild genotype of GSTM1 individuals, but without statistical significance. Conclusion It was not found that the relationship between GSTM1, GSTT1 polymorphism and asthma. Synergism of genotype of GSTM1, GSTT1 and air pollution was not also seen in this study.

Objective To investigate the changes of monocyte chemoattractant protein-1(MCP-1)in serum and urine of lupus nephritis(LN)patients in active phase and remission phase.Methods The levels of MCP-1 in serum and urine of 58 LN patients(27 of active phase and 31 of remission phase)were measured by ELISA.The correlation between the levels of MCP-1 in variant phase of LN and other relevant factors were analyzed.Results The MCP-1 levels in sera of both active phase and remission phase of LN patients were markedly higher than those in controls(548.5?347.2 ng/L and 469.1?298.4 ng/L vs 273.3?146.7 ng/L,P0.05).Conclusion The MCP-1 levels in urine of LN patients is more suitable to evaluate the activity of disease as a sensitive marker.

Objective To identify the nutritional risks of the hospitalized children with congenital heart disease (CHD) during perioperative period and the relationship between nutrition risks and the clinical outcomes.Methods Totally 780 CHD children (522 cases of ventricular septal defect,133 cases of atrial septal defect,70 cases of tetralogy of fallot,and 55 of patent ductus arteriosus) were recruited.The STRONGkids (Screening Tool for Risk on Nutritional Status and Growth) was used to analyze the nutritional risks of CHD children and to identify the nutritional risks of these children at different ages.Also,the potential effect of pulmonary hypertension on malnutrition and effects of the possible nutritional risks on the clinical outcomes were analyzed.Results Of these 780 CHD children,260 (33.3％) were found to be with high and moderate nutritional risks.The incidences of these risks were 49.2％ (187/380) in the 0-1-year-old group,which was significantly higher than that in the 1-3-year-old group (26.2％,60/229) (x2 =25.174,P ＜0.001) and the ＞ 3-year-old group (7.6％,13/171) (x2 =36.029,P ＜ 0.001).Both pulmonary hypertension and malnutrition showed synergic effects on the clinical nutritional risk,and higher nutrition risks were associated with poorer clinical outcomes.Conclusions STRONGkids can effective evaluate the nutritional risks of CHD children during the perioperative period and thus can inform the standardized nutrition support.

Background Stickler syndrome is a genetic connective tissue disorder that affects the ocular,skeletal,orofacial and auditory systems.To determine the gene mutation loci can offer a basis for genetic diagnosis and management of Stickler syndrome.Objective The aim of this study was to research the clinical characteristics of a pedigree with Stickler syndrome and identify the disease-causing gene mutation.Methods This study was approved by Ethic Committee of Peking Union Medical College Hospital.The clinical study and pedigree analysis were performed in one family with Stickler syndrome type Ⅰ (STL Ⅰ).Nine family members were examined with informed consent.The entire coding regions of COL2A1 gene with flanking intronic regions were amplified by PCR and directly sequenced.The detected sequence change was confirmed to be mutationloci by examining whether they existed in normal control individuals.Mutant proteins were predicted with online software.Results There were 4 generations and 11 members in this family,and 2 members died,including 1 patient.Three patients were found in 9living families.Inheritance of this family complicd with an autosomal dominant inheritance mode.All affected individuals showed the consistent phenotypes with STL Ⅰ,including high myopia,membranous vitreous anomaly and surface central flat,short nose,palatoschisis,etc.Mutation screening of COL2A1 gene revealed that the first base of intron 12 was deleted(IVS12+1G del).Nucleotide sequence analysis showed that this mutation led to the functional abnormal of this gene by forming termination cordon in advance.This mutation occurred in all affected individuals,however,no mutation was observed in any unaffected member or 100 normal unrelated individuals.Conclusions This study identifies a novel splice-site mutation(IVS12+ 1G del)in COL2A1 gene in a Chinese STL Ⅰ pedigree.This is the first report on a mutation in a Chinese STL Ⅰ family.

Objective To study the clinical characteristics and effects of surgical treatment of children with thalamic tumors.Methods The clinical data of 22 cases were retrospectively studied and followed-up 6 months to 9 years.Results There were 13 boys and 9 girls,their ages ranging from 3 to 13 years.The average duration of symptoms before diagnosis about 2 months.Headache and papilledem were the most symptoms and signs,respectively.Most children′s thalamic tumors were low grade tumors with clear verge.In this group,good results were obtained that total remove 9 cases,subtotal remove 8 cases,partial remove 3 cases,biopsy 2 cases and no surgical death.Conclusions Clinical character of children thalamic tumors is distinct and good surgical results in the nearly future.The long results are determined by type of pathology.

ABCB11 gene encodes bile salt export pump (BSEP).It is almost exclusively expressed in the canalicular microvilli of liver.It is the principal conveyor of bile acids from hepatocyte cytoplasm into bile canaliculus.It is clearly that BSEP defects can induce progressive familial intrahepatic cholestasis type 2 and benign recurrent intrahepatic cholestasis type 2.ABCB11 gene variation are also responsible for intrahepatic cholestasis of pregnancy,drug-induced cholestasis,primary sclerosis cholangitis and primary bile cirrhosis.This paper reviewed the association of ABCB11 gene variation and these diseases.

Objective To understand human rhinovirus (HRV) etiology of acute lower respiratory tract infection (ALRTI) in children in Shanghai area and establish a nested reverse transcription- polymerase chain reaction (nested RT-PCR) assay.Methods Three hundred and forty-two naso- pharyngeal secretion (NPS) samples from ALRTI cases who were hospitalized were collected during January 2005—December 2005.Nested RT-PCR techniques were used to detect HRV-specific RNA.The PCR products were sequenced and data of nucleotides were analyzed.The proportion of HRV infection in children with ALRTI,the distribution of gender,age and season,and clinical char- acteristics were also investigated.Results Forty-six (13.5%) of 342 samples were HRV positive detected by nested RT-PCR.The sequences of 15 positive samples shared high homology of 83%- 97% with HRV sequence in GenBank.Within the 15 positive samples,nucleotide homology varied from 64.4% to 98.4%,and the ratio of genetic variation was from 1.6% to 48.3%./00.These 15 ampli- cons attribute to the two branches of HRV cladogram.The sequences of 15 amplieons were highly varied,in which single nucleotide mutation and several nearby nueleotides mutations were found. Ribonucleotide deletion and insertion in the nucleotide sequence was also found.HRV positive sam- ples were detected in 33 boys and 13 girls,respectively.The ratio of infection cases between boys and girls was 2.5:1.Of 46 HRV infected cases,27 (58.7%) were less than 12 months of age and 38 (82.6%) were less than 3 years old.HRV infected ALRTI occured all the year round and peaked from March to May.Of the patients whose NPS samples were HRV positive detected by nested RT-PCR,45 patients were diagnosed with bronchopneumonia and 1 was diagnosed with asthmatic bronchitis.Fever of most patients was moderate.The peripheral blood leukocyte counts in thirty-nine (84.8%) patients were less than 10?10~9/L.Neutrophil percentages in thirty-seven (80.4%) patients were less than 0.50.C-reactive protein of thirty-six (78.3%) patients were less than 8 mg/L. All of these features were the characteristics of viral pneumonia.The complications were not common and conditions of most patients were not severe.All the children were cured.Conclusions This nes- ted RT-PCR technique is highly specific,rapid and convenient for the detection of HRV RNA in NPS of patients with ALRTI and the genome of HRV viruses is highly variable.The incidence of HRV infection predominates in children in Shanghai area.ALRTI of HRV is short of specificity and condi- tions of most patients are not severe and their prognoses are fine.