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Objective:To investigate the gastrointestinal characteristics of children with glycogen storage disease (GSD) type Ⅰ.Methods:From June to December 2020, clinical data of children aged 0-18 years with GSD type Ⅰ diagnosed by genetic testing from all provinces and cities in China, including Beijing, Shanghai, Guangdong, Guangxi, Hunan, Sichuan, Yunnan, Guizhou, Henan, Hebei, Zhejiang, Jiangsu, Shaanxi, Anhui and Heilongjiang, were collected.A cross-sectional questionnaire survey was used for data analysis.Results:A total of 52 questionnaires were obtained, and 43 eligible patients aged 1-18 years were recruited, involving 30 males (69.8%) and 13 females (30.2%). Among them, 9 patients were GSD type Ⅰa and 34 patients were type Ⅰb.Seven patients (16.3%) had siblings who were also diagnosed as GSD type Ⅰb.The gastrointestinal manifestations included recurrent diarrhea in 26 patients (60.5%), perianal lesions (erythema, ulcer, abscess) in 25 patients (58.1%), abdominal pain/distension in 24 patients (55.8%), nausea/vomiting in 22 patients (51.1%), mucus/bloody stool in 14 patients (32.6%). Thirty-three patients (76.7%) had recurrent stomatitis and oral ulcer, and 38 patients (88.0%) had at least two gastrointestinal symptoms.White blood cell (WBC) count was <4.0×10 9/L in 24 patients (55.8%), and absolute neutrophils count was <1.5×10 9/L in 19 patients (44.2%), which was <0.5×10 9/L in 10 patients (23.3%). WBC count and absolute neutrophils count both decreased in children with GSD type Ⅰb.Platelets were >300×10 9/L in 30 patients (69.8%). Eighteen patients with GSD type Ⅰb underwent gastroscopy and colonoscopy, and 16 patients were diagnosed with GSD-related inflammatory bowel disease.Thirty-nine patients (90.7%) were fed with raw corn starch, 3 patients (6.9%) with maltodextrin and 19 patients (44.2%) with special enteral formula.Twenty patients with type Ⅰb GSD needed repeated antibiotic treatment due to neutropenia and neutrophil dysfunction.Fifteen patients were treated with granulocyte colony-stimulating factor (G-CSF). Among them, 11 patients were diagnosed as GSD-related bowel disease. Conclusions:Children with GSD type Ⅰ commonly have gastrointestinal symptoms, especially those with GSD type Ⅰb.The incidence of GSD-related inflammatory bowel disease is high in those children.G-CSF treatment cannot prevent the development of GSD-associated inflammatory bowel disease and its pathogenesis needs further research.Diet therapy is the first-line treatment of GSD type Ⅰ.Multidisciplinary management is helpful to reduce the complications and improve the quality of life in children with GSD type Ⅰ.
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Objective:To discover the epidemiological characteristics and clinical manifestations of Nontyphoidal Salmonella(NTS) infection in children suffering from diarrhea in Guangzhou, and to provide references for the prevention and treatment of salmonella infection. Methods:A total of 570 diarrhea children and 296 non-diarrhea controls were collected with stratified sampling from three districts of Guangzhou Women and Children′s Medical Center from January 2019 to December 2019. Through bacterial culture, corresponding colonies were selected and Salmonella diagnostic serum was applied for preliminary serum diagnosis, and then systemic biochemical method was adopted for diagnosis. A structured questionnaire was conducted to record the demographic information and clinical symptoms from each subject. Results:The detection rate of NTS was 6.67% (38/570 cases, 95% CI: 4.90%-9.02%) in children with diarrhea, and 1.01% (3/296 cases, 95% CI: 0.34%-2.93%) in children without diarrhea. There were statistically differences in NTS detection rate between children with diarrhea and the control ( χ2=13.805, P<0.05, OR=6.976, 95% CI: 2.135-22.796). There were no significant differences in NTS detection rate between male and female children with diarrhea ( χ2=0.395, P>0.05, OR=1.254, 95% CI: 0.619-2.541). The detection rate of NTS was 5.30% (22/416 cases, 95% CI: 3.52%-7.88%) in children younger than 2 years old, and was 10.40% (16/154 cases, 95% CI: 6.50%-15.21%) in children over 2 years old. There were statistically differences ( χ2=4.700, P<0.05, OR=2.076, 95% CI: 1.060-4.068) between the younger and the older groups.The detection rate of NTS was 5.40% (25/460 cases, 95% CI: 3.70%-7.89%) for children with diarrhea in outpatient department and 11.80% (13/110 cases, 95% CI: 7.04 %-19.18%) for children with diarrhea in inpatient department, with statistically significant differences ( χ2=5.813, P<0.05, OR=2.332, 95% CI: 1.152-4.721). The detection rate of NTS diarrhea in children was 4.60% (10/217 cases, 95% CI: 2.52%-8.28%) in spring, 8.50% (12/141 cases, 95% CI: 4.93%-14.29%) in summer, 9.60% (15/144 cases, 95% CI: 6.41%-16.48%) in autumn and 1.50% (1/168 cases, 95% CI: 0.11%-3.30%) in winter, respectively, with statistically differences among the four seasons( χ2=9.404, P<0.05). There were significant differences in most common clinical symptoms of fever, vomiting, abdominal pain, bloody stool and pasty stool between NTS positive and negative children(all P<0.05). Salmonella enteritis is the main type, and Salmonella typhimurium is the second type. Conclusions:NTS is one of the most important bacterial pathogens and leads to diarrhea in children in Guangzhou city, without differences in gender.Children over 2 years old are more likely to suffer from NTS infection.High incidence is in autumn.The most common clinical symptoms include fever, vomiting, abdominal pain, bloody stool and pasty stool. Salmonella enteritis is the main type, and Salmonella typhimurium is the second type.Laboratory tests can provide references for the diagnosis and treatment of NTS-related diarrhea.
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Objective To predict and verify the upstream regulatory microRNA (miRNA)of protein kinase D1 (PKD1),and to investigate its role in cerulein induced acute pancreatitis (AP)in rats. Methods Potential up-stream regulatory miRNA of PKD1 was predicted by using bioinformatics software. Dual luciferase reporter gene system and Western blot were applied to verify the regulation of PKD1 by the selected miRNA. Experimental AP was induced by 6 intraperitoneal injection of cerulein (20 μg/ kg)at hourly intervals after administration of the CY5 - labeled notar-get control (AP group,n = 20)or selected miRNA (treatment group,n = 20),respectively by intraperitoneal injection into rats. Other rats were divided randomly into a normal control group (n = 10)without any treatment. Besides 10 rats in either AP or treatment group were sacrificed 6 hours after the first injection of cerulein,and the rats were all sacri-ficed 24 hours after the first injection. The blood samples and pancreatic tissues of each rat were collected to test serum amylase and lipase activities,or to make hematoxylin - eosin stain for AP pathological scores as well as PKD1 immuno-histochemical staining,respectively. Results TargetScan 7. 1 software analysis showed that miR - 128 - 3p was the po-tential upstream regulatory miRNA of PKD1,which was verified by dual luciferase reporter gene system and Western blot detection. Compared to the normal control group,serum amylase and lipase activities after 6 h exposure to cerulein increased in both AP group and the treatment group[13313. 00(9424. 00 - 15995. 00)U/ L,13552. 00(10399. 50 -18408. 25)U/ L vs. 1430. 50(1214. 25 - 1543. 25)U/ L;547. 00 (515. 00 - 627. 00)U/ L,857. 50(522. 00 -1222. 25)U/ L vs. 34. 00(32. 50 - 34. 75)U/ L],and the differences were significant(χ2 = 8. 715,P < 0. 05;χ2 =9. 115,P < 0. 05),which indicated that the rat models of AP were successfully established. The immunohistochemical scores of PKD1 after 24 h exposure to cerulein decreased in the treatment group[0. 50(0 - 2. 75)scores],compared with the normal control group [4. 00(4. 00 - 8. 00)scores]and the AP group [4. 00(3. 75 - 8. 00)scores],and difference was significant(χ2 = 18. 302,P < 0. 05). Accordingly,the total pathological scores of HE staining decreased significantly in the treatment group,as compared to the AP group (3. 80 ± 0. 85 vs. 6. 90 ± 1. 15,t = 4. 481,P < 0. 01). The results showed that the inflammatory cell infiltration and tissue necrosis were significantly improved after miR -128 - 3p treatment. Conclusions miR - 128 - 3p is the upstream regulatory microRNA of PKD1 which protects pan-creata from necrotic injury and inflammatory cell infiltration in PKD1 - mediated acute pancreatitis.
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Objective To analyze the clinical, endoscopic and histopathological features of eosinophilic gastroenteritis ( EG) in children. Methods A retrospective study of 76 children with EG was performed to analyze clinical symptoms, laboratory and imaging results, endoscopic and pathological features, status of Helicobacter pylori ( H. pylori) infection, treatment and outcomes. Results The main clinical symptoms were abdominal pain in 55. 3%(42/76) cases, vomiting in 39. 5% (30/76) cases and hematochezia in 38. 2% cases( 29/76) . The hemoglobin level decreased significantly in 34 cases ( 44. 7%, 34/76). Peripheral blood eosinophil (EOS) count increased significantly in 9 cases (11. 8%,9/76) and EOS percentage increased significantly in 13 cases(17. 1%,13/76). Total serum IgE elevated in 32 cases ( 54. 2%, 32/59 ) . There were also 18 cases ( 36. 7%, 18/49 ) positive in serum allergen?specific immunoglobulin E ( sIgE) test and 25 cases ( 32. 9%,25/76) positive in fecal occult blood test. Among 51 cases of abdominal ultrasound examination, there were 7 cases of ascites, 4 cases of pelvis fluid and 3 cases of intestinal wall change. Endoscopic examination in 76 cases showed 63 cases ( 82. 9%) of mucosal hyperemia/edema,20 cases ( 26. 3%) of ulceration, 17 cases ( 22. 4%) of erosion, 11 cases ( 14. 5%) of nodularity or hyperplasia and 9 cases ( 11. 8%) of normal mucosa. The pathological examination showed mucosal inflammation with a large number of EOS infiltration(≥20 per HPF).There were 12 cases(15. 8%, 12/76) of H. pylori infection. Among the 76 cases, clinical symptoms improved significantly in 74 patients after treatment with dietary allergen avoidance, anti?allergy medications, antacids, montelukast and corticosteroid, and the total efficacy was 97. 4%. The efficacy of dietary allergen avoidance, anti?allergy medications, antacids and montelukast was 93. 8%( 61/65 ) . The efficacy of corticosteroid was 86. 7%(13/15). Conclusion The clinical manifestations and endoscopic characteristics of EG in children lack specificity. In terms of diagnosis, the elevated total serum IgE and the positive sIgE test may be taken as reference for the diagnosis of EG. The definite diagnosis is based on pathological examination ( EOS infiltration≥20 per HPF).While in terms of treatment, dietary allergen exclusion, anti?allergy medications, antacids and montelukast are highly effective, which can be taken as the first option. There is no need of corticosteroid as routine therapy.
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<p><b>OBJECTIVE</b>To analyze the efficacy of percutaneous endoscopic gastrostomy (PEG) in pediatric patients.</p><p><b>METHOD</b>From October 2011 to October 2014, children in the gastrointestinal ward of Guangzhou Women and Children's Medical Center received PEG or jejunal tube PEG(JET-PEG). The success rate, operation time were recorded. The changes of their weight, enteral nutrition calories and the incidence of pneumonia before and after the first 6 months of operation were compared. Follow-up was conducted until October 2014, the recent and long term complications, the length of indwelling time, the replacement or removal of the tube were recorded, the patients swallowing function or the primary disease's outcomes were observed.</p><p><b>RESULT</b>Of the 13 cases, 10 were male, 3 were female, their average age was 2 years (range 1.8 months-9 years). We performed PEG for 12 of the patients who had congenital craniofacial problems that led to feeding difficulties or recurrent cough and pneumonia (6/12), or neurological disorders (6/12) with inability to swallow, and in one case JET-PEG was performed, this child suffered from chronic intestinal pseudo-obstruction with vomiting and abdominal distension. The gastrostomy was successful in all the patients through one operation, the average operation time of PEG was (25 ± 3) minutes, JET-PEG was 60 minutes. One local skin infection was noted, no long-term complication occurred. In the first 6 months after operation, all the patients gained weight((5.5-30.5) kg postoperation vs. (3.0-30.0) kg preoperation), and 12 cases' enteral nutrition calories increased (from (209-502) to(272-543) kJ/(kg·d)), the incidence of pneumonia decreased in the children who had recurrent pneumonia before the operation (from (0-1.5) to (0-0.16) per month). Until October 2014, their average length of gastric tube indwelling time was 17.8 months (range 4-36 months). In 4 cases PEG tube was removed when they could eat completely independently, the other 9 needed enteral vein nutrition via PEG tube or jejunal tube, in 3 of them balloon type gastric fistula tube was applied. Two of the 13 cases who had cleft palate received stomatological operations when their weight grew to meet the standard.</p><p><b>CONCLUSION</b>PEG and JET-PEG are safe and effective method for enteric nutrition feeding in pediatrics, the technique causes minimal trauma and has rapid postoperative recovery, few complications, good aesthetic appearances and simple nursing, it can significantly improve their nutritional status and quality of life.</p>
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Child , Child, Preschool , Female , Humans , Infant , Male , Enteral Nutrition , Methods , Gastrostomy , Incidence , Nervous System Diseases , Therapeutics , Pneumonia , TherapeuticsABSTRACT
<p><b>OBJECTIVE</b>To evaluate the efficacy of endoscopic balloon dilatation, stenting and surgery in treating corrosive esophageal stricture of children.</p><p><b>METHOD</b>We retrospectively analyzed the management of 31 children who were treated with endoscopic balloon dilatation, stenting or gastric tube esophagoplasty because of corrosive esophageal stricture between August 2005 and December 2012. Dysphagia was graded into 4 grades according to Stooler grading methods. The efficacy was divided into 3 grades which were very effective, effective and ineffective according to the improvement of dysphagia.</p><p><b>RESULT</b>Among 31 children with corrosive esophageal stricture, in 22 children the problem was caused by alkali, in 9 by acid; 23 children had long-segment stricture and 8 had short-segment stricture. The number (rates) of cases in whom the endoscopic balloon dilatation was very effective, effective, and ineffective were 12 (38.7%) , 7 (22.6%) , 12 (38.7%) , respectively. The number of cases in whom stenting was effective or ineffective was 1 and 3, respectively. The gastric tube esophagoplasty was very effective and effective in 8 and 2 cases, respectively. Total rates of very effective, effective, and ineffective of 3 treatments were 64.5%, 29%, 6.5% respectively. The efficacy of endoscopic balloon dilatation was better in short-segment stricture and bigger caliber stricture, the efficacy was not so good for other types of strictures. The efficacy of balloon dilatation was not related with the nature of substances (either alkaline or acidic). Compared with very effective group, the ineffective group with longer course of disease before dilatation (t = -2.091, P = 0.048) , smaller stricture calibre (t = 2.855, P = 0.009) , longer stricture segment (t = -3.405, P = 0.003) and longer dilatation time (t = -2.103, P = 0.047) , had statistical significance.</p><p><b>CONCLUSION</b>Endoscopic balloon dilatation was the preferred treatment for corrosive esophageal stricture of children. The efficacy was better in shorter course of disease, short-segment stricture and bigger caliber stricture, for other situations the efficacy was not so good. The efficacy of balloon dilatation was not related with the nature of substances.Stent or surgery can be considered if efficacy of balloon dilatation was not so good.Gastric tube esophagoplasty is an effective treatment for complicated corrosive esophageal stricture.</p>
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Burns, Chemical , Therapeutics , Deglutition Disorders , General Surgery , Therapeutics , Dilatation , Methods , Esophageal Stenosis , General Surgery , Therapeutics , Gastroscopy , Retrospective Studies , Stents , Treatment OutcomeABSTRACT
To discuss clinical diagnosis and treatment of cow’s milk protein allergy and cow’s milk protein-induced FPIES (food protein induced enterocolitis syndrome). Methods We retrospectively analyzed clinical data of one infant with milk protein allergy-induced FPIES. Results A 67 days old female on mixed breast and formula feeding developed recurrent diarrhea, abdominal distension, vomiting, mucousy and bloody stools, feeding dififculty, anemia, and failure to thrive since 2 weeks after birth. Laboratory studies showed anemia, increased CRP level and elevation of peripheral white blood count and eosinophil proportion. Milk-speciifc IgE was negative. She was previously hospitalized 4 times, all with admitting diagnosis of“necrotizing enterocolitis”. We treated her with milk protein elimination for 4 weeks and all symptoms were resolved. Milk protein re-challenge test was positive, consistent with clinical features of cow’s milk protein allergy-induced infant FPIES. Conclusions Cow’s milk protein allergy and cow’s milk protein-induced FPIES can present with non-speciifc and variable clinical symptoms and signs, and should be considered in the differential diagnosis.
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Objective To evaluate physiologic ability and surgical stress (E-PASS) for predicting postoperative complications in patients undergoing elective colonic surgery. Methods The clinical data of 158 patients with colonic cancer who were admitted to the West China Hospital from August to October, 2009 were retrospectively analyzed. E-PASS was applied to evaluate the surgical risk. Three indexes of the E-PASS system,including preoperative risk score (PRS), surgical stress score (SSS) and comprehensive risk score (CRS) were compared with actual postoperative outcomes. Correlation between PRS, SSS, CRS and postoperative risks was analyzed using multiple linear regression analysis. Results Of the 158 patients, 27( 17.1% ) had postoperative complications, and the predictive value was 19.4% ± 2.0% according to the E-PASS. Dukes stages, physical performance indexes, severe heart disease, severe pneumonia disease, length of operation time were correlated with the incidence of complications (r = 0. 193, 0. 410, 0. 183, 0. 174, 0. 198, P ＜ 0.05). PRS, CRS and SSS had good predictive effect on postoperative risks (r = 0. 299, 0. 349, 0. 183, P ＜ 0. 05 ). Conclusions E-PASS system is a relatively simple, fast and user friendly tool for predicting the risk of short-term postoperative complications.
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Objective To analyze the features of intracranial epidermoid cyst on CT and MRI imaging.Methods 26 patients with intracranial epidermoid cysts proven pathologically were examined with pre-and post-contrst enhanced CT and MR imging,and 6 of 26 cases undergone CT plain scan.CT and MR imaging of 20cases were retrospectivelly reviewed.Results Among 26 cases,16 were located in the cisterns of cerebell opontine angle,7 in saddle area.2 inside of the skull;1in the brain essence.On CT,25 cases were homogeneous hypodense,one case was promiscuous high density,16 cases demonstrted inhomogeneous hypointensityimaging on T1WI,and inhomogenous hyperintensity on T2WI.4 cases display inhomogeneous signal.No contrast enhancement was noted except 2 cases with slightly periphery enhancement.One case brain organization receives invades,There was edema around tumor.17 cases demonstrated slightly inhomogeneous hyperintensity on FLAIR MR images,3 cases obviously advance.Conclusion The CT and MRI features of intracranial epidermoid cysts were certain characteristic,and MRI played an important role.as to uncharacteristic epidermoid cyst,there is still space for further research.