Multi-disciplinary hybrid therapy for tracheoesophageal fistula in children: analysis of 4 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore an innovative technique that is aided by multi-disciplinary hybrid approach in identification and treatment of tracheoesophageal fistula (TEF) in children intraoperatively.</p><p><b>METHOD</b>From April 2008 to October 2011, 4 patients with isolated TEF were presented with 2 H-type fistulas and 2 recurrent TEF. For all the four cases, with the cooperation of the gastroenterologists, respiratory physician and surgeon, methylene blue was first injected into the trachea for detecting the dye in the esophagus by the gastroscopy. Bronchoscopy was performed where the fistula tract was shown by the methylene blue and a guide wire was passed through the fistula. The patients underwent rigid gastroscopy and the guide wire was identified and brought out through the mouth by biopsy pliers. This created a wire loop through the fistula. X-ray was then used to identify the level of the fistula. According to the level of the fistula it was determined whether surgical incision and approach should be used. The fistula was then repaired successfully by surgery.</p><p><b>RESULT</b>In the 4 patients, with the aid of gastroscopy and bronchoscopy, identification of the fistula intraoperatively was then facilitated by traction on the loop. The fistula was identified and repaired. There were no fistula recurrences.</p><p><b>CONCLUSION</b>Multi-disciplinary hybrid therapy for tracheoesophageal fistula in children is beneficial for the precise localization of the fistula. This new technique is an effective and definitive method in identification and treatment of TEF in children.</p>

Effects of vitamin A on the immune function of intestinal mucosa lymphocytes in mice / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the effects of vitamin A on the development of T lymphocytes in peripheral blood and small intestine and on the cytokine response of intestinal mucosa in mice.</p><p><b>METHODS</b>Twenty young mice were randomly fed with forage containing vitamin A 250 or 4 IU/g (n=10 each). Three weeks later, the levels of CD4+ CD25+ T subsets in peripheral blood and intestinal mucosa were measured by flow cytometry. The levels of cytokines IFN-γ, IL-4, IL-17 and IL-23 in stool were measured using ELISA.</p><p><b>RESULTS</b>The levels of CD4+ CD25+ T subsets in peripheral blood and intestinal mucosa in the 250 IU/g vitamin A group were significantly higher than those in the 4 IU/g vitamin A group (P<0.05). The IL-4 level in stool increased, in contrast, the IL-23 level in stool decreased significantly in the 250 IU/g vitamin A group when compared with the 4 IU/g vitamin A group (P<0.05).</p><p><b>CONCLUSIONS</b>vitamin A may promote the development of CD4+ CD25+ T lymphocytes in peripheral blood and small intestine. Moreover, it may be involved in intestinal mucosa-associated immune response by regulating cytokines IL-4 and IL-23.</p>

Molecular genetic analysis of 10 Chinese patients with glycogen storage disease type III / 中华儿科杂志

<p><b>OBJECTIVE</b>Glycogen debranching enzyme (AGL) plays an important role in complete degradation of the glycogen, and has two independent catalytic activities, i.e., those of alpha-1, 4-glucanotransferase (EC 2.4. 1.25) and amylo-1,6-glucosidase (EC 3.2. 1.33). A deficiency in activities of AGL causes excessive accumulation of glycogen with short branched outer chains and results in glycogen storage disease type III (GSD III; MIM #232 400), an autosomal recessive inborn disorder of glycogen metabolism. The present study aimed to investigate the mutation of AGL in 10 Chinese patients with GSD III.</p><p><b>METHOD</b>Clinical and laboratory data of 10 patients with typical clinical manifestations of GSD III suggesting hypoglycemia, hyperlipidemia, increased creatine-phosphokinase and its isozyme were collected. The coding regions and their flanking introns of AGL gene of the 10 patients were amplified by PCR and analyzed by direct DNA sequencing. All the mutated alleles were confirmed by bidirectional DNA sequencing. The 3 novel splicing mutations were analyzed by restriction fragment length polymorphism (RFLP) in 50 healthy children (control). The 2 small deletions (c.408-411delTTTG, c.2717-2721delAGATC) were analyzed by fluorescent polymerase chain reaction and gene scan analysis to confirm the number of deleted bases.</p><p><b>RESULT</b>Thirteen different mutations were identified, including 4 splicing mutations (IVS6 + 1G > A, IVS6-1G > A, IVS14 + 1G > T, IVS26-2A > C), 5 nonsense mutations (R469X, R864X, S929X, R977X, Y1428X), 3 small deletions (c.408-411delTTTG, c.2717-2721delAGATC, c.2823delT) and 1 insert mutation (c.4234insT). Except for IVS14 + 1G > T, R864X, and R977X, the other 10 mutations are novel; 18 mutated alleles were identified in the 20 alleles (90%). IVS14 + 1G > T was the most frequently seen mutation, accounting for 5 of 20 (25%) alleles examined. None of homozygote and heterozygote of the 3 novel splicing mutations was found in the 50 healthy controls by RFLP analysis. With the fluorescent polymerase chain reaction and gene scan analysis, c.408411deTTTG mutation and c.2717-2721delAGATC mutation were confirmed to have 4 and 5 bases deletion respectively.</p><p><b>CONCLUSION</b>Thirteen mutations were identified in the 10 cases with GSD III, with 10 novel mutations. IVS14 + 1G > T was a relatively common mutation. This study revealed the heterozygosity of AGL gene in Chinese patients with GSD III.</p>

Value of the liver function test in differential diagnosis of infantile hepatitis syndrome and biliary atresia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>This study aimed to investigate the value of the liver function test in the differential diagnosis of infantile hepatitis syndrome (IHS) and biliary atresia (BA) by analyzing seven conventional serological markers in this test using receiver operating characteristic (ROC) curves.</p><p><b>METHODS</b>Serum levels of seven conventional serological markers: alanine aminotransferase (ALT), aspartate aminotransferase (AST), gamma-glutamyl transpeptidase (gamma-GT), alkaline phosphatase (ALP), total bilirubin (TB), conjugated bilirubin (CB) and serum albumin (ALB) were measured in 103 children with IHS and 60 children with BA. ROC curves were used to evaluate the sensitivity, specificity, positive and predictive values and optimal cut-off. The united tests (parallel test and serial test) of gamma-GT, TB and CB were performed to elevate diagnostic efficiency.</p><p><b>RESULTS</b>Compared with the IHS group, the BA group had significantly increased serum ALT, AST, gamma-GT, TB and CB levels (p<0.01). The area under ROC (AUCROC) of AST, gamma-GT, CB and TB was 0.77, 0.881, 0.841 and 0.87, respectively. gamma-GT showed the highest AUCROC, specificity, positive predictive value and positive likelihood ratio in the diagnosis of BA, followed by CB, TB and AST in turn. The negative predictive value of CB was the highest, followed by TB. The negative likelihood ratio of CB was the lowest but its Youden index was the highest. The Youden index of gamma-GT and TB was lower than that of CB. After the parallel tests, the sensitivity and negative predictive value of gamma-GT, CB and TB increased to 100%. After the serial tests, the specificity of gamma-GT, CB and TB increased to 90.4% and the positive predictive value increased to 87.5%.</p><p><b>CONCLUSIONS</b>The measurement of gamma-GT, TB and CB levels are valuable in the differential diagnosis of BA and IHS. An imaging examination is required in the parallel test positive patients.</p>

Relationship between the types of Helicobacter pylori strains and chronic upper gastrointestinal diseases in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the relationship of the types of Helicobacter pylori (H. pylori) strains with the classification and the severity of chronic gastro-duodenal diseases in children.</p><p><b>METHODS</b>One hundred and fifteen children with chronic upper gastrointestinal symptoms who were diagnosed as H. pylori infection by gastroscopy were enrolled in this study. H. pylori strains were serotyped by immunoblot technique. The gastric biopsy specimens of all patients were studied histologically.</p><p><b>RESULTS</b>Type I H. pylori strains were confirmed in 84 cases (73.0%), intermediate type strains in 21 cases (18.3%), and type II strains in 10 cases (8.7%). Type I H. pylori strains infection caused a moderate gastric mucosal inflammation in 83 cases and a severe inflammation in 1 case. Intermediate type H. pylori strains infection caused a moderate gastric mucosal inflammation in 21 cases. Type II H. pylori strains infection caused a mild gastric mucosal inflammation in 2 cases and a moderate inflammation in 8 cases. Different types of H. pylori strains resulted in different severity of gastric mucosal inflammation (x2=15.444, P < 0.01). The gastric mucosal inflammation due to type I H. pylori strains was the most severe, while the inflammation due to type II H. pylori strains was relatively mild. The incidence of nodulus lymphaticus of gastric mucosa due to type I, type II and intermediate type H. pylori strains infection was 76.2%, 47.6% and 40.0%, respectively (x2=10.171, P < 0.01). The classification of chronic gastro-duodenal diseases was not associated with the types of H. pylori strains.</p><p><b>CONCLUSIONS</b>Type I strains were the leading cause of H. pylori infection in children. All of types of H. pylori strains can cause pathohistologic changes of gastric mucosa. Type I H. pylori strains infection can result in the most severe gastric mucosal inflammation and the highest incidence of nodulus lymphaticus. The immunoblot serotyping of H.pylori strains may be useless for the classification of chronic upper gastrointestinal diseases but it is helpful for the evaluation of the severity of the diseases in children.</p>

Endoscopic and histopathological characteristics of chronic gastritis,duodenitis and peptic ulcer in children / 上海第二医科大学学报

Objective To investigate the relationship between the endoscopic characteristics of chronic gastritis,duodenitis,peptic ulcer and their histopathologic findings in children,and explore the relationship between Helicobacter pylori(Hp) infection and the severity of histopathologic changes of gastroduodenal mucosa in children. Methods Three hundreds and sixty-six children with chronic upper gastrointestinal symptoms who were examined by gastroscopy were enrolled.The gastric and duodenal mucosal biopsy specimens of all the patients were studied histopathologically. ResultsTypes of chronic gastroduodenal diseases in all these patients were: chronic gastritis(n=206,56.3%),chronic gastritis combined with duodenitis(n=112,30.6%),chronic gastritis combined with peptic ulcer(n=48,13.1%).There was chronic inflammation in gastric mucosa and duodenal bulb mucosa in all the cases examined by histopathologic examination.Hp infection was found in 106 cases(28.96%).The gastric mucosal inflammation was more severe in those with Hp infection than those without(P0.05).The were significant differences in the incidences of inflammation activity,atrophia and nodulus lymphaticus of gastric mucosa between those with Hp infection and those without(P