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Chronic pancreatitis is a focal or diffuse chronic inflammation and fibrosis of pancreatic tissue, which is progressive, persistent and irreversible.Chronic pancreatitis in children has its own characteristics of early onset and frequent occurrence, which greatly affects the nutritional status and quality of life of children.Recently, diagnosis and treatment of this severe inflammation gradually improved with a burgeoning technique: endoscopic retrograde cholangiopancreatography (ERCP), especially in the treatment of pediatric chronic pancreatitis.The detection rate of the disease has been increasing year by year.As an effective and safe procedure for the treatment of chronic pancreatitis in children, ERCP has replaced traditional surgery and become the first-line treatment method for pediatric chronic pancreatitis.
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Objective To investigate the effects of Helicobacter pylori( HP) on gut microbiota in children by comparing the difference of gastric microbiota between HP-positive and HP-negative individuals. Methods Genome was extracted from excrements of 8 HP-positive cases and 8 HP-negative cases. After genomic extraction,the hypervariable region of 16S rRNA gene were amplified and a small fragment library was constructed,and high-throughput sequencing was carried out, then the data of the lower machine was effectively sequenced by biological information processing. We could seek for the species that have changed significantly due to HP infection by comparing the differences in the composition of intestinal flora between the two groups. Results Compared with HP-negative group,HP-positive group showed less OTUs. The dif-fenece of biodiversity between them was conspicuous. The Caproiciproducens,Enterobacteriaceae,Enterobac-teriales,Blautia-obeum,Esherichia-albertli,human-gut-metagenome and Dorea in HP-positive group were sig-nificantly higher than HP-negative group,while the Bacteroides-uniformis, Bacteroidaceae and Bacteroides in HP-negative group were significantly higher than HP-positive group. Conclusion HP could significantly affect the structure and composition of gut microbiota in children.
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Objective To investigate the clinical value of the polymerase chain reaction (PCR) technique in detection of pediatric helicobacter pylori(HP) infection.Methods A total of 130 children with different digestive tract symptoms received esophagogastroduodenoscopy,and 120 children between 3 and 17 years old were enrolled.The gastric antrum mucosa was taken under the gastroscope for 2 blocks,and the gastric juice was absorbed as the specimen.One block of gastric antrum mucosa was examined histopathologi-cally,and the other block of gastric antrum mucosa and gastric juice were examined by PCR.We used the primers UreC,HP-16s,CSTP to detect HP,and then used the primers Cag750 and Cag595 to detect CagA. Results A total of 28 cases(23.33%) of upper gastrointestinal ulcer were detected by gastroscopy,and HP was detected by histopathological method in 26 cases(21.67%),and 41 cases(34.17%) were detected by PCR method.The detection rate of HP by PCR was significantly higher than that of HP in pathological method (χ2= 4.659,P = 0.031). By pathological examination of HP,14 cases (50%) and 12 cases (13.04%) with peptic ulcers and no peptic ulcers were detectd,and the difference in detection rate was statistically significant(χ2=17.275,P<0.001).Samples of children with peptic ulcers and no peptic ulcers were detected in 16 cases(57.14%) and 25 cases (27.17%) by PCR,and the difference in detection rate was statistically significant (χ2=8.572,P=0.003).The CagA were detected in 7 cases of peptic ulcers and 7 cases of non peptic ulcers by PCR,and the difference in detection rate was statistically significant(25.00%vs 7.61%,χ2=6.300,P=0.012).Conclusion The PCR method could quickly and sensitively detect the HP and its CagA gene,and the detection of gastric mucosa and gastric juice by PCR could improve the detection rate of HP.A combination of PCR and pathological method is suggested as the detection method for children′s HP infection.
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Objective To explore the clinical characteristics and diagnosis of hepatolenticular degeneration (WD) in children. Method The clinical data of 38 children with WD were analyzed retrospectively. Results In the 38 cases (15 males and 23 females), the median age at diagnosis was 6 years, and the average interval between onset and confirmed diagnosis was 5.7 months. The median course of disease was 2 months and the longest was 3 years. Hepatic dysfunction was the most common initial symptom (71.1%), and 27 cases had glutamic acid aminotransferase > 2 ULT (71.1%); Serum ceruloplasmin decreased obviously in 3 cases (94.7%), copper oxidase was significantly reduced in 37 cases (97.4%); 24 h urine copper increased in 33 cases, in which 32 cases(84.2%)had>150 μg/24 h.The K-F rings were presented in 10 cases(26.3%).ATP7B gene sequencing was performed in 19 cases, and the positive rate was 83.3%. Conclusions Onset with liver lesions was common in children with WD, The combination of the results of serum ceruloplasmin, copper oxidase, and 24 h urine copper may made a clinical diagnosis.For a highly suspected case with inadequate evidence,the ATP7B gene detected is helpful.
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Objective To explore the clinical characteristics and diagnosis of hepatolenticular degeneration (WD) in children. Method The clinical data of 38 children with WD were analyzed retrospectively. Results In the 38 cases (15 males and 23 females), the median age at diagnosis was 6 years, and the average interval between onset and confirmed diagnosis was 5.7 months. The median course of disease was 2 months and the longest was 3 years. Hepatic dysfunction was the most common initial symptom (71.1%), and 27 cases had glutamic acid aminotransferase > 2 ULT (71.1%); Serum ceruloplasmin decreased obviously in 3 cases (94.7%), copper oxidase was significantly reduced in 37 cases (97.4%); 24 h urine copper increased in 33 cases, in which 32 cases(84.2%)had>150 μg/24 h.The K-F rings were presented in 10 cases(26.3%).ATP7B gene sequencing was performed in 19 cases, and the positive rate was 83.3%. Conclusions Onset with liver lesions was common in children with WD, The combination of the results of serum ceruloplasmin, copper oxidase, and 24 h urine copper may made a clinical diagnosis.For a highly suspected case with inadequate evidence,the ATP7B gene detected is helpful.
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A 13-year-old girl initially complained of thrombocytopenia 3 years ago and had positive antinuclear antibody(ANA),weakly positive anti-double-stranded (anti-ds) DNA antibodies and decrease of C3 1 month ago.She was admitted to the ward following dyspnea and intermittent cough for three days this time.The initial diagnosis was systemic lupus erythematosus (SLE).The clinical manifestation after admission involved multiple organs such as liver,kidney and spleen.However,cirrhosis could not be explained by SLE.Besides,anti-dsDNA as a specific index for SLE was only weakly positive in this case and the decrease of C3 was independent to activity of the disease.Abnormal signals in bilateral basal ganglia confirmed by MRI could not be explained by SLE,either.Therefore,the initial diagnosis as SLE was suspicious.Further laboratory test showed low ceruloplasmin and increased 24 urine Cu to correct the diagnosis as hepatolenticular degeneration (HLD) and pulmonary infection.Gene sequence analysis revealed heterozygous mutation in ATP7B gene (a splice site mutation:c.1708-5T ＞ G;a missense mutation:c.2333G ＞ T,p.Arg778Leu).HLD should be suspected in any patient with liver abnormalities of uncertain causes along with involvement of multiple systems.Gene sequence analysis is helpful to early diagnosis of HLD.
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Objective To explore the pathogenesis and diagnosis of chronic granulomatous disease. Methods Clinical features and laboratory examination results of a child with chronic granulomatous disease were retrospectively analyzed. Genome DNA was extracted from peripheral blood of the child and his parents. The high-throughput sequencing was performed by Illumina sequencing platform, using the Agilent SureSelect exome capture method. Results The child had recurrent infections along with liver enlargement and dysfunction. The anti-infection and symptomatic treatment were unsatisfactory. Gene sequencing analysis revealed a homozygous point mutation (c.7C?>?T, p.Gln3*) in CYBA gene. His mother had the same heterozygous mutation in this locus, and his father had a large fragment heterozygous deletions. No other candidate gene mutations were identiifed. Conclusions The diagnosis of chronic granulomatous disease is conifrmed in this child. It is caused by CYBA gene mutation.
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<p><b>OBJECTIVE</b>To evaluate the growth of children in weight and height before and after liver transplantation and the relation between malnutrition and postoperative time.</p><p><b>METHOD</b>Growth was assessed for children who received liver transplantation from July 2007 to December 2012 after operation during follow-up. Weight and height were measured for 51 children in May 30, 2013. Weight and height percentiles of each child were calculated in accordance with data surveying on physical development of children in nine provinces/municipalities. Underweight was defined as weight less than the third percentile of same age and sex groups. Growth retardation was defined as height less than the third percentile of same age and sex groups. Children were set into 2 groups (before liver transplantation group, after liver transplantation group). The incidence of underweight and growth retardation were analyzed by chi-square test. Children were divided into 4 groups according to the length of time from operation time to May 30, 2013: 1 year after liver transplantation (1-365 days); 2 years after liver transplantation (366-730 days); 3 years after liver transplantation (731-1 095 days) ; 4 years or more after liver transplantation (1 096-2 133 days) . The underweight and growth retardation were analyzed by hierarchical log linear model.</p><p><b>RESULT</b>The mean age of 51 children was 44.78 months (range 13 months to 13 years old), 26 of them were male and 25 female. The number of children with underweight and growth retardation were 20 (39%) and 35 (69%) respectively before transplantation and were 5 (10%) and 14 (27%) respectively after transplantation. There was a significant difference between underweight incidence before and after operation (χ(2) = 10.385, P = 0.001). There was significant difference between growth retardation incidence before and after operation (χ(2) = 15.710, P = 0.000). The subjects included 10 patients at 1 year after operation (underweight n = 3, growth retardation n = 3), 19 patients at 2 years (underweight n = 1, growth retardation n = 9), 10 patients at 3 years (underweight n = 1, growth retardation n = 2), 12 patients at 4 years and above (underweight n = 0, growth retardation n = 0). Parameter analysis of hierarchical log linear estimates: underweight at 1 year = 0.661, underweight at 2 years = -0.214, underweight at 3 years = 0.119, underweight at 4 years and above = -0.566. Growth retardation at 1 year = 0.282, at 2 years = 0.613, at 3 years = 0.051, at 4 years and above = -0.946.</p><p><b>CONCLUSION</b>Compared with after liver transplantation, obvious malnutrition existed in patients before transplantation. Patients have the ability to catch-up growth after liver transplantation. Reduced effect of underweight occurred in second year after liver transplantation. Reduced effect of growth retardation occurred in third year after liver transplantation.</p>
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Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Biliary Atresia , Therapeutics , Body Height , Body Weight , Child Development , Growth Disorders , Epidemiology , Liver Cirrhosis , Therapeutics , Liver Transplantation , Malnutrition , Epidemiology , Postoperative Period , Retrospective Studies , Thinness , Epidemiology , Time FactorsABSTRACT
<p><b>OBJECTIVE</b>To summarize the clinical characteristics, early diagnosis, comprehensive treatment and prognosis of 6 cases of children with post-transplantation lymphoproliferative disorder (PTLD) after liver transplantation.</p><p><b>METHOD</b>Data of 6 cases with PTLD seen between January 2011 and December 2013 were retrospectively analyzed. The anti-rejection drug dose adjustments, the effect of rituximab, antiviral therapy and comprehensive treatment program after surgery were explored.</p><p><b>RESULT</b>(1) The diagnosis of PTLD was confirmed by histologic findings. Six cases of PTLD including 3 males and 3 females were diagnosed as congenital biliary atresia and underwent split liver transplantation. The occurrence rate of PTLD was 2.9%. (2) The median time to the development of PTLD was less than 6 months. The initial symptom of PTLD in all patients was fever and clinical manifestations of PTLD were non-specific, depending on the involving organs. Five cases of PTLD developed gastrointestinal symptoms, including diarrhea, abdominal pain, and abdominal distension. One case developed respiratory symptoms, including cough and tachypnea. Three cases had lymph node involvement. In 2 cases pathophysiology involved polymorphic lymphocyte proliferation and in 4 cases B lymphocyte proliferation. (3) Two cases died, in whom EBV DNA was not detected and were diagnosed as PTLD by surgical pathology before death. Four survived cases had high EBV-DNA load and then were diagnosed as PTLD by biopsy pathology. (4) Of the 6 cases of PTLD, 2 cases died and 4 cases survived. The overall mortality was 33%. The dead cases were only treated with laparotomy because of intestinal obstruction or perforation and the survived cases were treated with tacrolimus at reduced doses or discontinuation and rituximab. In 2 cases antiviral therapy (acyclovir) was continued, including 1 cases of intestinal obstruction treated with surgical repair. All the survived patients were followed up for 4 months to 1 year and no evidence has been found.</p><p><b>CONCLUSION</b>EBV infection is the high risk factor for PTLD after liver transplantation. Close clinical surveillance of EBV DNA for pediatric liver transplantation was important for the early diagnosis of PTLD. Reducing doses of immunosuppressive agents and rituximab is the initial therapy for PTLD. A reduction in the dose of tacrolimus is suggested. Operation therapy can also play a role in the management of local complications.</p>
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Female , Humans , Infant , Male , Antiviral Agents , Biliary Atresia , Therapeutics , DNA, Viral , Drug Therapy, Combination , Early Diagnosis , Epstein-Barr Virus Infections , Diagnosis , Therapeutics , Immunosuppressive Agents , Liver Transplantation , Lymphoproliferative Disorders , Diagnosis , Mortality , Therapeutics , Pediatrics , Postoperative Complications , Retrospective Studies , Survival Rate , TacrolimusABSTRACT
Objectives To explore the clinical features, diagnosis and treatment of Crohn’s disease complicated by erythromelalgia (EM) in a pediatric case. Methods The clinical manifestation, results of laboratory testing and endoscopy, mutational analysis of the SCN9A gene, and the follow-up record were collected and analyzed based on review of literature to a thirteen-year-old girl with Crohn’s disease complicated by erythromelalgia. Results The patient experienced symptoms of anorexia, fatigue, diarrhea, dark red and swelling skin, increased skin temperature and burning pain in her both lower extremities during the course of disease. The endoscopic ifndings included multiple ulcerations and polypoid protrusion lesion in colon, and the pathological examination showed the local abscess formation in colonic mucosa. The mutation in SCN9A gene of the child was excluded by gene analysis. The symptoms were gradually ameliorated after treatment using prednisone and mesalazine combined with dipyridamole and low-molecular-weight heparin calcium. Conclusions Crohn’s disease complicated by erythromelalgia is rare. The pathogenesis may relate to immune factors, thrombocytosis, and hyper-coagulable states, etc. The combination of glucocorticoids, 5-aminosalicylic acid and anticoagulants may lead to a better therapeutic effect.
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Objective To retrospectively study the results of endoscopic manifestation pathology,and treatment of colonic polyps. Methods Clinical data of 121 patients diagnosed with colonic polyps and treated by endoscopy between November 2002 and September 2009 were studied. Complete colonic investigation was done by Olympus PCF240I electron endoscopy. Colonic polyp excision was done by high frequency electric coagulation. Results All patients had bloody stool with mucus for 1 week to 8 years. Ninty four(77.7%)patients had a single polyp,6(5.0%)had two polyps,21(17.3%)with multiple polyps,and 5 patients had familial polyposis coli. The size of the polyps ranged from 0.3 cm to 4 cm and the morphology of Yamada Ⅰ to Ⅳ. The pathological changes were juvenile polyps,inflammatory polyps,polypoid formation,and canalicular adenoma. Most common pathology types were Yamada Ⅳ and juvenile polyps,107 of the 121 patients(88.4%)had juvenile polyps,and one was found to have malignant transformation. 115 patients accepted high frequency electric coagulation excision under general anesthesia,without complication. Conclusions Colonic polyp is one of the common diseases in children. Children should be checked by total colonoscopy in time when they present with bloody stool,anemia,rectal polyps prolapse or intussusception. Polypectomy under endoscope is a most safe and reliable treatment method when the diagnosis is confirmed. For isolated juvenile polyp,regular recheck is not necessary after polypectomy except when bloody stool recurs. Regular follow up by colonoscope is necessary for juvenile polyp combined with adenoma and adenomatous polyps. Colonoscope is necessary at any time when bloody stool occurs. Familial polyposis coli is difficult to be treated by endoscopy,thus surgical procedure is suggested.