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1.
Front Pediatr ; 8: 460, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33014917

RESUMO

Purpose: To analyze the influence of perioperative complications in the management of biliary atresia (BA). Methods: A retrospective study was performed using a total of 422 BA patients who underwent Kasai portoenterostomy (KPE) in a single institution between February 2016 and May 2017. Data on patients' clinical characteristics, laboratory examinations, perioperative complications, and outcomes were collected. Unpaired two-tailed t-test and χ2 test were employed for the comparison between BA patients with and without perioperative complications. Cox regression analysis was used to screen the risk factors for 2-years NLS in BA, and their influence on the 2-years NLS was analyzed using Kaplan-Meier survival analysis as well as the log-rank test. Results: The incidence of perioperative complications, 6-months jaundice clearance (JC) and 2-years native liver survival (NLS) rate were 60.4, 59.5, and 56.6%, respectively. Patients with perioperative complications had lower serum albumin (ALB) level, but higher aspartate aminotransferase-to-platelet ratio index (APRI) and international normalized ratio (INR) levels when compared with those without perioperative complications (ALB, P < 0.05; APRI, P < 0.01; INR, P < 0.05). Moreover, perioperative complications were correlated with glucocorticoid administration (P = 0.002). Univariate Cox regression analysis showed no relationship between perioperative complications and 2-years NLS (P > 0.05). However, multivariate Cox regression analysis indicated 6-months JC was an independent protective factor for 2-years NLS [P < 0.0001, hazard ratio (HR) = 0.074, 95% confidence interval = 0.05-0.11], and concordance index of this prediction model including age, weight, APRI, glucocorticoid, and 6-months JC was 0.811. Conclusion: Although perioperative complication is common during and after KPE, it had no influence on the prognosis of BA. However, assessment of the serum level of total bilirubin after KPE may serve as an important predictor for the outcome in BA.

2.
J Gastroenterol Hepatol ; 35(2): 334-342, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31271681

RESUMO

BACKGROUND AND AIM: Biliary atresia (BA) is a progressive fibro-inflammatory cholangiopathy with an unclear etiology. Various liver disorders are associated with an altered microbiome. However, gut microbiome in BA remains unknown. Here, we performed a case-control study to investigate the gut microbiota in BA. METHODS: A cross-sectional analysis was first conducted for 34 BA patients and 34 healthy controls. Then we investigated the shift in gut microbiota 2 weeks after the Kasai procedure in 16 BA patients. Gut microbiome was initially analyzed using 16S ribosome RNA gene sequencing and further validated by metagenomic sequencing. Fecal bile acids were determined using ultra-high performance liquid chromatography. RESULTS: Compared with healthy controls, BA showed lower diversity and significant structural segregation in the microbiome. At phylum level, Proteobacteria numbers increased, whereas those of Bacteroidetes decreased in BA. At genus level, several potential pathogens such as Streptococcus and Klebsiella thrived in BA, while numbers for Bifidobacterium and several butyrate-producing bacteria declined. The microbiome was also disturbed after the Kasai procedure. Operational taxonomic units responding to BA showed significant correlation with liver function. Furthermore, the abundance ratio of Streptococcus/Bacteroides showed great promise in distinguishing BA from healthy controls. Intestinal bile acids were dramatically decreased in BA, and Clostridium XIVa positively correlated with the ratio of primary/secondary bile acids. CONCLUSIONS: Gut microbial dysbiosis, may be caused by decreased bile acids, was associated with liver function and had a good diagnostic potential for BA. Therefore, further exploration of gut microbiota may provide important insights into their potential diagnostic and therapeutic benefits.


Assuntos
Atresia Biliar/microbiologia , Microbioma Gastrointestinal , Atresia Biliar/complicações , Atresia Biliar/diagnóstico , Estudos de Casos e Controles , Disbiose/etiologia , Humanos
3.
Gastroenterol Res Pract ; 2019: 4621372, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31781188

RESUMO

Objectives: To detail the effects of vitamin D (VD) deficiency and assess the relationships between VD deficiency and liver function and liver fibrosis in patients with biliary atresia (BA). Methods: In this study, BA patients confirmed by intraoperative cholangiography were enrolled between January 2017 and February 2019. Preoperative serum 25-(OH)D level, liver function, serum biomarker levels of liver fibrosis, and histopathologic features were recorded. Deficiency, insufficiency, and sufficiency of VD were defined as serum 25-(OH)D concentrations of <10, 10-20, and >20 ng/ml, respectively. Associations between serum 25-(OH)D level and liver function and liver fibrosis were analyzed. Results: A total of 161 BA infants were included. The median (interquartile range (IQR)) serum 25-(OH)D level in all patients was 7.56 (IQR: 4.48-11.40) ng/ml. The rates of 25-(OH)D deficiency, insufficiency, and sufficiency were 67.1% (108/161), 29.2% (47/161), and 3.7% (6/161), respectively. Serum 25-(OH)D level was negatively correlated with alkaline phosphatase (r = -0.232, P = 0.003). After adjusting for age, a decrease in serum 25-(OH)D level was correlated with the increase of the Batts-Ludwig stage score (odds ratio (OR): 0.94, 95% confidence interval (CI): 0.88-0.99; P = 0.028). Serum 25-(OH)D level was also correlated with the N-terminal propeptide of type III procollagen (PIIINP) (r = -0.246, P = 0.002). Additionally, PIIINP (P = 0.038) and ALP (P = 0.031) were independently associated with serum 25-(OH)D level. Conclusions: VD deficiency was common and inversely correlated with liver fibrosis in BA patients. Furthermore, VD was not correlated with liver function except alkaline phosphatase.

4.
Pediatrics ; 144(5)2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31604829

RESUMO

BACKGROUND: The overlapping features of biliary atresia (BA) and other neonatal cholestasis with alternative causes (non-BA) have posed challenges for diagnosis. Matrix metalloproteinase-7 (MMP-7) has been reported to be promising in diagnosing BA. We aimed to validate the diagnostic accuracy of MMP-7 for BA in a large population sample. METHODS: We enrolled 288 patients with neonatal obstructive jaundice from March 2017 to October 2018. Serum MMP-7 levels were measured by using an enzyme-linked immunosorbent assay. Receiver operating characteristic curves were constructed, and decision curve analysis was done. A Pearson correlation coefficient test was conducted to assess the correlation between MMP-7 levels and other characteristics. RESULTS: The median serum MMP-7 levels were 38.89 ng/mL (interquartile range: 22.96-56.46) for the BA group and 4.4 ng/mL (interquartile range: 2.73-6.56) for the non-BA group (P < .001). The area under the receiver operating characteristic curve value was 0.9829 for MMP-7, and the sensitivity, specificity, positive predictive value, and negative predictive value were 95.19%, 93.07%, 97.27%, and 91.43%, respectively, at a cutoff value of 10.37 ng/mL. When MMP-7 was combined with γ glutamyl transferase, the diagnostic accuracy was slightly improved without significance when compared with MMP-7 alone and had an area under the curve of 0.9880 (P = .08). Decision curve analysis also showed potential for MMP-7 to be used for clinical applications. A significant correlation was found with fibrosis stage from liver biopsy (R = 0.47; P < .001). CONCLUSIONS: MMP-7 demonstrated good accuracy in diagnosing BA and holds promise for future clinical application. Furthermore, its correlation with liver fibrosis indicated its potential use as a therapeutic target or prognostic biomarker.


Assuntos
Atresia Biliar/diagnóstico , Icterícia Neonatal/sangue , Metaloproteinase 7 da Matriz/sangue , Área Sob a Curva , Atresia Biliar/sangue , Atresia Biliar/complicações , Biomarcadores/sangue , Biópsia , Coleta de Amostras Sanguíneas/métodos , Coleta de Amostras Sanguíneas/normas , Técnicas de Apoio para a Decisão , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Lactente , Icterícia Neonatal/etiologia , Fígado/patologia , Masculino , Curva ROC , Sensibilidade e Especificidade
5.
Biomark Med ; 13(14): 1157-1173, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31559834

RESUMO

Aim: To develop a nomogram for differential diagnosis between advanced and early pediatric appendicitis (PA). Patients & methods: We retrospectively studied 669 PA patients. Patient characteristics and 24 serum markers were subjected to univariate and multivariate analysis, based on which the nomogram was constructed. Results: Fibrin degradation product, CRP and Na+ differed significantly between the advanced and early PA. The value of area under the receiver operating characteristic curve (AUC) was 0.8602, which was greater than that of a single serum marker. Furthermore, the nomogram showed better discriminative ability than the biomarker alone. Notably, validation indicated high stability and reproducibility. Conclusion: The nomogram is superior to the serum marker alone, and may hold promise in clinical application.


Assuntos
Apendicite/diagnóstico , Nomogramas , Criança , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Fatores de Tempo
6.
J Pediatr Surg ; 54(12): 2565-2569, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31526660

RESUMO

BACKGROUND: Intrahepatic cystic lesion (ICL) is a common complication for biliary atresia post-Kasai portoenterostomy. The purpose of this study was to review the cases in our hospital and assess the correlation between characteristics of ICL and clinical outcomes. METHODS: We retrospectively analyzed 787 cases of biliary atresia from 2012 to 2016. Demographics, clinical details, and postoperative outcomes were reviewed. RESULTS: A total of 76 patients were diagnosed with ICLs using ultrasound post-Kasai procedure, and the incidence was 9.7%. Preoperative characteristics showed no significant differences between ICL (+) and ICL (-) groups. Nearly 70% (53/76) of the patients with ICLs had a history of cholangitis. The 2-year native liver survival rate was 60.4% for those with a history of cholangitis and 87% for those without (P = 0.017). Further analysis showed that the 2-year native liver survival rate was 42.9% for those diagnosed within 3 months post-Kasai procedure, 54.2% for those diagnosed between 3 and 6 months, and 80.0% for those diagnosed beyond 6 months (P = 0.002), while no significance was observed for type (P = 0.094) or site (P = 0.406) of ICL. CONCLUSION: Patients with ICLs had a high incidence of cholangitis. The prognosis was closely related with the history of cholangitis and the onset time of ICLs. LEVEL OF EVIDENCE: Level II.


Assuntos
Atresia Biliar/cirurgia , Colangite/etiologia , Cistos/etiologia , Hepatopatias/etiologia , Fígado/fisiopatologia , Portoenterostomia Hepática/efeitos adversos , Criança , Pré-Escolar , Cistos/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Lactente , Hepatopatias/diagnóstico por imagem , Masculino , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Período Pós-Operatório , Prognóstico , Estudos Retrospectivos , Fatores de Tempo , Ultrassonografia
7.
EBioMedicine ; 34: 223-230, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30077722

RESUMO

BACKGROUND & AIMS: The overlapping features of biliary atresia (BA) and the other forms of neonatal cholestasis (NC) with different causes (non-BA) has posed challenges for the diagnosis of BA. This study aimed at developing new and better diagnostic models for BA. METHODS: We retrospectively analyzed data from 1728 newborn infants with neonatal obstructive jaundice (NOJ). New prediction models, including decision tree (DT), random forest (RF), and multivariate logistic regression-based nomogram for BA were created and externally validated in an independent set of 508 infant patients. RESULTS: Fiver predictors, including gender, weight, direct bilirubin (DB), alkaline phosphatase (ALP), and gamma-glutamyl transpeptidase (GGT) were significantly different between the BA and non-BA groups (P < .05), from which DT, RF, and nomogram models were developed. The area under the receiver operating characteristic (ROC) curve (AUC) value for the nomogram was 0.898, which was greater than that of a single biomarker in the prediction of BA. Performance comparison of the three diagnostic models showed that the nomogram displayed better discriminative ability (sensitivity, 85.7%; specificity, 80.3%; PPV, 0.969) at the optimal cut-off value compared with DT and RF, which had relatively similar high sensitivity and PPV (0.941 and 0.947, respectively), but low specificity in the modeling group. In sub-analysis of the discriminative capacity between the nomogram and GGT (<300 or ≥ 300), we found that the nomogram was superior to the GGT alone in the preoperative diagnosis of BA. CONCLUSIONS: The nomogram has demonstrated better performance for the prediction of BA, holding promise for future clinical application.


Assuntos
Atresia Biliar/diagnóstico , Fosfatase Alcalina/sangue , Grupo com Ancestrais do Continente Asiático , Atresia Biliar/sangue , Bilirrubina/sangue , Colestase/sangue , Colestase/diagnóstico , Estudos de Coortes , Árvores de Decisões , Feminino , Humanos , Lactente , Recém-Nascido , Icterícia Neonatal/sangue , Icterícia Neonatal/diagnóstico , Masculino , Nomogramas , gama-Glutamiltransferase/sangue
8.
Cell Death Dis ; 9(5): 545, 2018 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-29748604

RESUMO

Biliary atresia is one of the most common liver disease in infancy. The cause and pathogenesis remain largely unknown. This study aimed to investigate the potential regulatory effect of miR-29b/142-5p on IFN-γ gene methylation. miRNAs microarray was performed on four pairs of liver and blood specimens from biliary atresia and choledochal cysts. We found the overexpression of miR-142-5p and mRNA level of DNA methyltransferase (DNMT) 1, and miR-29b and DNMT3a/DNMT3b were significantly negatively correlated in biliary atresia livers. Meanwhile, the methylation of the LINE-1, ALU and SAT2 repetitive sequences and the IFN-γ promoter was lower, but the expression of IFN-γ was upregulated. After transfected with DNMTs siRNAs, downregulation of DNMTs exerted a significant hypomethylating effect on the repetitive sequences, which led to upregulation of IFN-γ in Jurkat cells. The direct interactions between miR-29b and DNMT3a/3b, and miR-142-5p and DNMT1 were identified using luciferase reporter assays. By transfecting mimics of miR-29b/142-5p into Jurkat cells, we found overexpression of miR-29b/142-5p markedly suppressed expression of DNMTs. Furthermore, the methylation of repetitive sequences and the IFN-γ promoter region were remarkably downregulated, and with elevated IFN-γ expression. After transfecting the miRNA inhibitors, the levels of DNMTs and the methylation of the IFN-γ gene promoter region was upregulated, while levels of IFN-γ were markedly suppressed. Our study suggested that miRNA-29b/142-5p overexpression and targeted inhibition of DNMTs expression resulted in decreased overall gene methylation and overexpression of the methylation-sensitive IFN-γ gene.


Assuntos
Atresia Biliar/metabolismo , Metilação de DNA , Regulação da Expressão Gênica , Interferon gama/biossíntese , MicroRNAs/biossíntese , Elementos Alu , Atresia Biliar/patologia , DNA (Citosina-5-)-Metiltransferases/metabolismo , Feminino , Humanos , Lactente , Células Jurkat , Elementos Nucleotídeos Longos e Dispersos , Masculino , Regiões Promotoras Genéticas
9.
Int J Clin Exp Pathol ; 11(5): 2846-2851, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-31938405

RESUMO

Biliary atresia (BA) is a devastating liver disease of complex pathogenesis in neonates, characterized by an inflammatory and fibrosing obstruction of extrahepatic bile ducts. Cytotoxic T lymphocyte-associated antigen-4 (CTLA4) is expressed on the surface of a subset of regulatory T cells (Treg) and down regulates the human immune response. To investigate the possible association between CTLA4 gene polymorphisms and BA susceptibility, we conducted a case-control study in the Chinese children. Three single nucleotide polymorphisms (SNPs) in the CLTA4 gene (rs231725, rs231775 and rs3087243) were genotyped in 113 BA patients and 133 healthy controls. The statistical analysis revealed no significant difference between BA patients and healthy controls in allele or genotype frequencies (rs231725, P = 0.2718, OR = 0.814, 95% CI = 0.564-1.175; rs231775, P = 0.1599, OR = 1.316, 95% CI = 0.897-1.931; rs3087243, P = 0.0572, OR = 1.582, 95% CI = 0.984-2.543), neither in the distribution of haplotypes of these CTLA4 gene SNPs. The result of our study is the first one to provide the evidence that there is no significant association between CLTA4 gene polymorphisms and BA susceptibility in Chinese children.

10.
Cell Death Dis ; 8(3): e2719, 2017 03 30.
Artigo em Inglês | MEDLINE | ID: mdl-28358366

RESUMO

Biliary atresia is a rare, devastating disease of infants where a fibroinflammatory process destroys the bile ducts, leading to fibrosis and biliary cirrhosis, and death if untreated. The cause and pathogenesis remain largely unknown. We tried to investigate factors involved in biliary atresia, especially forkhead box A3 (Foxa3), which might exert a role in the treatment of liver disease. We used RNA sequencing to sequence the whole transcriptomes of livers from six biliary atresia and six choledochal cysts patients. Then, we employed a rat disease model by bile duct ligation (BDL) and adenovirus transduction to address the function of Foxa3 in biliary atresia. We found that tight junction, adherence junction, cell cycle, apoptosis, chemokine singling, VEGF and MAPK signaling pathways were enriched in biliary atresia livers. We showed that Foxa3 expression was notably decreased in liver samples from biliary atresia patients. More importantly, we found that its lower expression predicted a poorer overall survival of biliary atresia patients. Rats that received BDL surgery and Foxa3 expression adenovirus resulted in a significant decrease in the deposition of collagen, and expression of profibrotic cytokines (transforming growth factor-ß and connective tissue growth factor) and fibrosis markers (α-smooth muscle actin, collagen I and collagen III), as compared with rats that received BDL surgery and control adenovirus. Our data suggested a protection role for Foxa3 during the progression of liver fibrosis in biliary atresia, and thereby supported increasing Foxa3 as a targeted treatment strategy.


Assuntos
Atresia Biliar/metabolismo , Fator 3-gama Nuclear de Hepatócito/metabolismo , Cirrose Hepática/metabolismo , Sistema de Sinalização das MAP Quinases , Animais , Atresia Biliar/complicações , Atresia Biliar/genética , Atresia Biliar/terapia , Modelos Animais de Doenças , Feminino , Fator 3-gama Nuclear de Hepatócito/genética , Humanos , Cirrose Hepática/etiologia , Cirrose Hepática/genética , Cirrose Hepática/terapia , Masculino , Camundongos Endogâmicos BALB C , Ratos , Fator A de Crescimento do Endotélio Vascular/genética , Fator A de Crescimento do Endotélio Vascular/metabolismo
11.
Medicine (Baltimore) ; 95(30): e4428, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27472740

RESUMO

BACKGROUND: A thoracoscopic approach for repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) has become a standard procedure in many pediatric surgical centers. However, whether thoracotomy or thoracoscopy offer advantages in terms of surgical outcomes is not known. METHODS: To evaluate the efficacy and safety of thoracoscopic repair (TR) versus conventional open repair (COR) for EA with TEF.PubMed, Cochrane Library, and EMBASE were searched to identify relevant literature until 2016.Studies comparing surgical outcomes of patients undergoing TR versus COR for EA with TEF were reviewed.The quality of each included study was assessed using the Newcastle-Ottawa scale score. A fixed or random-effect model was applied depending on heterogeneity tests. RESULTS: Eight observational clinical studies involving 452 patients were included in this meta-analysis. The meta-analysis of 2 major postoperative complications (leaks and strictures) did not show significant differences between TR and COR. Overall estimates of the odds ratio (OR) of TR versus COR for leaks and strictures were: 1.57 (95% confidence interval [CI], 0.77-3.20; P = 0.22) and 0.90 (95% CI, 0.27-2.97; P = 0.86), respectively. However, meta-analysis of operation time (OR = 19.59, 95% CI = 0.77-38.40, P = 0.04), timing of extubation (OR = -2.50, 95% CI = -3.39 to -1.62, P < 0.001), time to 1st oral feeding (OR = -2.58, 95% CI = -3.79 to -1.36, P < 0.001), and duration of hospital stay (OR = -10.76, 95% CI = -16.39 to -5.12, P < 0.001) showed significant differences.No randomized controlled trial was included, and most studies had small sample sizes and were based on retrospective analysis. CONCLUSION: TR and COR show a similar complication rates of leaks and strictures for EA/TEF repair. Although associated with a longer operative time, TR has the advantages of an earlier time to extubation and 1st oral feeding, and shorter hospital stay.


Assuntos
Atresia Esofágica/cirurgia , Toracoscopia , Toracotomia , Fístula Traqueoesofágica/cirurgia , Humanos , Recém-Nascido , Estudos Observacionais como Assunto , Complicações Pós-Operatórias/etiologia , Ensaios Clínicos Controlados Aleatórios como Assunto , Resultado do Tratamento
12.
J Pediatr Surg ; 49(9): 1400-4, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25148746

RESUMO

OBJECTIVE: This study sought to examine promoter methylation and expression of the identified sonic hedgehog (SHH) gene in terminal rectal tissues of children with congenital anorectal malformations (ARMs). METHODS: Tissue samples from the terminal rectum of pediatric patients with ARMs (five cases each of high and intermediate malformation - two cases of rectovesical fistula, two cases of rectourethral prostatic fistula, one case of cloaca with >3cm common channel, four cases of rectourethral bulbar fistula and one case of imperforate anus without fistula, respectively, and ten cases of low malformation - five cases of perineal fistula and five cases of vestibular fistula, respectively), and patients with non-gastrointestinal tract malformation (six cases, anal fistula) were collected and divided into three groups: high-intermediate ARM (ARMhi-int), low ARM (ARMlo), and control (Cont.). Real-time RT-PCR was used to detect mRNA expression levels of the verified differentially methylated gene SHH, and bisulfite genomic sequencing was performed to evaluate DNA methylation in the SHH promoter region. RESULTS: The average methylation levels of the SHH promoter were significantly higher in ARMhi-int (0.850±0.030, P=0.0036) and ARMlo (0.540±0.053, P=0.0087) groups than in Cont. group (0.280±0.032). SHH mRNA expression levels were lower in ARMhi-int (0.340±0.015, P=0.0065) and ARMlo (0.530±0.042, P=0.0156) groups than in Cont. group (0.870±0.046). The average methylation levels of the SHH promoter were higher in ARMhi-int group than in ARMlo group (0.850±0.030 vs. 0.540±0.053, P=0.0095), while SHH expression was significantly reduced in ARMhi-int group compared to ARMlo group (0.340±0.15 vs. 0.530±0.042, P=0.0252). The methylation levels of the SHH promoter in ARMhi-int group were negatively correlated with SHH gene expression (r=-0.89, P<0.01). CONCLUSIONS: The SHH gene, which plays a major role in the development of the anorectum and enteric nervous system, is hypermethylated at its promoter, and this is correlated with low levels of SHH gene expression. This epigenetic modification may therefore be responsible for the observed changes in SHH expression, which could in turn underlie the pathogenesis of congenital ARMs.


Assuntos
Canal Anal/anormalidades , Canal Anal/metabolismo , Metilação de DNA , Proteínas Hedgehog/genética , Reto/anormalidades , Reto/metabolismo , Feminino , Regulação da Expressão Gênica no Desenvolvimento , Proteínas Hedgehog/metabolismo , Humanos , Lactente , Masculino , Análise de Sequência com Séries de Oligonucleotídeos , Regiões Promotoras Genéticas/fisiologia , RNA Mensageiro/metabolismo , Reação em Cadeia da Polimerase em Tempo Real
13.
Int J Clin Exp Med ; 7(11): 4016-23, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25550910

RESUMO

OBJECTIVE: To analyze the etiology and clinical diagnostic method for chronic complex anal and rectal inflammation in children less than 3 years old. METHOD: Seven children (5 males and 2 females; 1 year 8 months to 3 years of age at the time of physician evaluation) with chronic complex anal and rectal inflammation were enrolled between May 2008 and May 2013 at our hospital. Clinical history, results of auxiliary examinations, and empirical treatment of the children were analyzed retrospectively combined with the etiologic diagnosis. RESULTS: Four patients were confirmed to have Crohn's disease and one patient was confirmed to have intestinal tuberculosis; two patients were suspected to have Crohn's disease. Anemia and low pre-albumin level were common (seven patients); serologic testing revealed four patients with elevated IgG levels and seven patients with elevated IgA levels; there were no patients with positive tuberculosis antibody titers and two patients were weakly positive for C-ANCA (one patient with Crohn's disease and one patient intestinal tuberculosis). Colonoscopies revealed that the entire colon was affected in one patient, the left hemicolon was affected in four patients, and the sigmoid colon and rectum were affected in two patients. Two patients with Crohn's disease and one patient with intestinal tuberculosis were diagnosed by colonoscopies in combination with histopathologic examinations. Two patients with Crohn's disease were confirmed after empirical drug treatment, and two other patients were not definitely diagnosed. CONCLUSION: The possibility of Crohn's disease or intestinal tuberculosis should be considered in the clinical diagnosis of complex chronic anal and rectal inflammation in younger children. Local surgery is sometimes unnecessary. Empirical drug treatment should be used if necessary.

14.
PLoS One ; 8(7): e68602, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23874684

RESUMO

Biliary atresia (BA) is a devastating cholestatic liver disease targeting infants. Current diagnosis depends on surgical exploration of the biliary tree. The aim of the present study was to identify potential biomarkers for the diagnosis of biliary atresia (BA). Two-dimensional electrophoresis was utilized for the identification of proteins that were differentially expressed in liver biopsies of 20 BA patients and 12 infants with non-BA neonatal cholestasis (NC) as controls. Using mass spectrometry, we identified 15 proteins with expressions significantly altered. Out of the 15 proteins identified, heat shock protein (HSP) 90 was the most significantly altered and was down-regulated in BA samples compared to NC samples using immunoblotting analysis. Our findings suggest that HSP90 might be a potential biomarker for the diagnosis of BA and may be used for monitoring further development and therapy for BA. This study demonstrated that a comprehensive strategy of proteomic identification combined with further validation should be adopted in biomarker discovery.


Assuntos
Atresia Biliar/diagnóstico , Proteínas de Choque Térmico HSP90/genética , Proteínas de Choque Térmico HSP90/metabolismo , Atresia Biliar/genética , Atresia Biliar/metabolismo , Biomarcadores/metabolismo , Biópsia/métodos , Colestase/diagnóstico , Colestase/genética , Colestase/metabolismo , Regulação para Baixo , Eletroforese em Gel Bidimensional/métodos , Feminino , Humanos , Lactente , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/genética , Doenças do Recém-Nascido/metabolismo , Fígado/metabolismo , Fígado/cirurgia , Masculino , Espectrometria de Massas/métodos , Proteômica/métodos
15.
Pediatr Surg Int ; 29(4): 375-9, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23334620

RESUMO

OBJECTIVE: The objective of this study is to analyze the effect of exogenous glial cell-derived neurotrophic factor (GDNF) in the development of the enteric nervous system (ENS) in the rectal end of fetal rats. MATERIALS AND METHODS: Thirty pregnant Sprague-Dawley rats were categorized randomly into three groups: ethylene thiourea (ETU), ETU + GDNF, and control. On day 10 of gestation, ETU was injected via a gastric tube in the ETU group and ETU + GDNF group. On day 11 of gestation, GDNF was administered through the tail vein in the ETU + GDNF group. On day 20 of gestation, fetal rats were harvested by cesarean section. The prevalence of anorectal malformations (ARMs) in the fetal rats was observed. GDNF expression in the rectal end of fetal rats was detected by immunohistochemical and Western blotting analyses. RESULTS: The prevalence of ARMs in the ETU group and ETU + GDNF group was 51.4 and 52.5 %, respectively, but the difference between the two groups was not significant (P > 0.05). In the rectal end of fetal rats with an anus, GDNF expressions in the three groups were not significantly different (P > 0.05). In the rectal end of fetal rats without an anus: GDNF expression in the ETU + GDNF group was significantly higher than that in the ETU group (P = 0.036); GDNF expression in the rectal end of fetal rats without an anus from the ETU group and ETU + GDNF group was significantly lower than that of fetal rats with an anus (ETU group P = 0.001; ETU + GDNF group P = 0.028). There was a significant difference in the gray level ratio of GDNF and actin between the ETU group and ETU + GDNF group (P < 0.0001), and the expression in the ETU + GDNF group was significantly up-regulated. CONCLUSION: GDNF could not totally prevent the occurrence of ETU-induced ARMs, but it up-regulated expression of the GDNF gene in the wall of the rectal end, thereby promoting the growth of a hypogenetic ENS.


Assuntos
Anus Imperfurado/fisiopatologia , Sistema Nervoso Entérico/fisiologia , Fator Neurotrófico Derivado de Linhagem de Célula Glial/fisiologia , Reto/inervação , Animais , Malformações Anorretais , Eletroforese , Feminino , Fator Neurotrófico Derivado de Linhagem de Célula Glial/metabolismo , Neurônios/metabolismo , Gravidez , Ratos , Ratos Sprague-Dawley , Regulação para Cima/fisiologia
16.
J Pediatr Surg ; 46(7): 1400-5, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21763842

RESUMO

OBJECTIVE: The aim of this study was to study the effect of vitamin A deficiency (VAD) on the embryological development of anorectal malformations (ARMs) and the enteric nervous system. MATERIALS AND METHODS: Female Sprague-Dawley rats were divided into 3 groups: VAD group, normal group (negative control), and ethylene thiourea (ETU) group (positive control) with a normal diet. On day 20 of pregnancy, cesarean section was performed on all rats. The incidence of ARMs in the fetal rats and Protein gene product 9.5 (PGP9.5) and S-100 protein expression by immunohistochemistry were determined. RESULTS: The incidence of ARMs in VAD and ETU groups was 64.8% (59/91) and 45.9% (61/133), respectively (P > .05). Anorectal malformations were not found in the normal group. Protein gene product 9.5 and S-100 protein expression in the non-ARM rectums of the VAD group was lower than the ETU (P = .0156 vs P = .0105) and normal groups (P = .0091 vs P = .0024). There was no significant difference in PGP9.5 and S-100 protein expression between ETU and normal groups. In the ARM rectums, PGP9.5 and S-100 protein expression in the VAD group was lower than the ETU group (P < .0001). Protein gene product 9.5 and S-100 protein expression was also lower in ARM than non-ARM rectums in the VAD and ETU groups (P < .0001, P = .0203, and P = .0122, respectively). CONCLUSION: Vitamin A deficiency during pregnancy may result in the embryological development of ARMs. Enteric nervous system development may be related to ARMs.


Assuntos
Anormalidades Múltiplas/etiologia , Canal Anal/anormalidades , Sistema Nervoso Entérico/anormalidades , Complicações na Gravidez/fisiopatologia , Reto/anormalidades , Deficiência de Vitamina A/fisiopatologia , Anormalidades Induzidas por Medicamentos/embriologia , Anormalidades Induzidas por Medicamentos/etiologia , Anormalidades Múltiplas/induzido quimicamente , Anormalidades Múltiplas/embriologia , Canal Anal/embriologia , Animais , Anus Imperfurado/embriologia , Anus Imperfurado/etiologia , Dieta , Sistema Nervoso Entérico/embriologia , Etilenotioureia/toxicidade , Feminino , Regulação da Expressão Gênica no Desenvolvimento/efeitos dos fármacos , Proteínas do Tecido Nervoso/biossíntese , Proteínas do Tecido Nervoso/genética , Neurônios/metabolismo , Gravidez , Complicações na Gravidez/metabolismo , Distribuição Aleatória , Ratos , Ratos Sprague-Dawley , Reto/embriologia , Proteínas S100/biossíntese , Proteínas S100/genética , Cauda/anormalidades , Cauda/embriologia , Teratogênios/toxicidade , Ubiquitina Tiolesterase/biossíntese , Ubiquitina Tiolesterase/genética , Vitamina A/sangue , Deficiência de Vitamina A/metabolismo
17.
Pediatr Surg Int ; 25(1): 41-5, 2009 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19039595

RESUMO

PURPOSE: The aim of this paper was to assess the clinical value of anorectal manometry (ARMM) in the diagnosing of Hirschsprung's disease (HD) in neonates. METHODS: From January 2003 to June 2005, 75 patients in whom HD was clinically suspected were analyzed. ARMM was performed using a desk, high rate gastrointestinal dynamic detection system and the results were compared with barium enema and rectal suction biopsy. RESULTS: Based on rectal suction biopsies in 52 of 75 patients, the positive, false positive, negative, and false negative rates of ARMM in the diagnosis of HD in neonates were found to be 92.3, 1.9, 1.9, and 3.8%, respectively. Forty-three of 75 patients were diagnosed with HD by both ARMM and barium enema and the diagnoses were validated by pathologic results. The diagnosis of HD was excluded in 18 patients in whom HD was clinically suspected, but in whom the results of ARMM and barium enema were normal. Twelve patients who had ARMM results consistent with HD and a negative barium enema, had serial ARMM performed; a rectoanal inhibitory reflex (RAIR) was elicited in four patients, thereby excluding HD and the remaining eight patients were diagnosed with HD by review of barium enema and pathologic results. One of two patients with a positive barium enema for HD, but an ARMM showing the presence of RAIR was excluded by pathologic results and the other patient was lost to follow-up. The diagnostic accuracies of ARMM and barium enema for HD in neonates were 93.3 and 86.7%, respectively. There was no difference in rectal resting pressure and anal rhythmic wave frequency between neonates with HD and healthy neonates, but neonates with HD had higher anal sphincter pressures than healthy neonates (P=0.0074). CONCLUSIONS: ARMM is a simple, safe, and non-invasive method with high specificity for the diagnosis of HD in neonates.


Assuntos
Doença de Hirschsprung/diagnóstico , Membrana Mucosa/fisiopatologia , Reto/fisiopatologia , Canal Anal/patologia , Canal Anal/fisiologia , Sulfato de Bário , Biópsia , Meios de Contraste , Enema , Reações Falso-Negativas , Reações Falso-Positivas , Feminino , Humanos , Recém-Nascido , Masculino , Manometria , Reto/patologia , Sucção
18.
J Pediatr Surg ; 43(9): 1691-5, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18779008

RESUMO

OBJECTIVE: The aim of the study is to appraise bowel movements in children with Hirschsprung's disease (HD) after a transanal Soave 1-stage endorectal pull-through (TAS) procedure. METHODS: From October 2000 to October 2004, 44 patients with HD underwent a TAS procedure, 35 had a Soave operation via a laparotomy, 29 underwent a Soave procedure via laparoscopy, and 39 had an Ikeda-Soper procedure; the patients were followed up from 1 to 5 years after surgery. Evaluation of the perioperative therapeutic effect and postoperative bowel movements between the 4 groups were analyzed by Kelly's score and anorectal manometry. RESULTS: The TAS procedure required less operative time and costs than the Soave procedure via laparotomy or laparoscopy (P < .05) and less than that of Ikeda-Soper procedure for the duration of the preoperative preparation, the duration of the surgical procedure, intraoperative blood transfusions, days of postoperative fasting, antibiotic use, and days and cost of hospitalization (P < .01). There were no differences in short-term and long-term complications between the same Soave procedures; the Soave procedure exceeded the Ikeda-Soper operation in the incidence of enterocolitis 3 months postoperatively (P < .05), but there was no difference between the TAS procedure and the Ikeda-Soper procedure. There was no difference in bowel movements 12 months postoperatively and the rectal anal inhibitory reflex, high-pressure zone length, resting anal canal pressure, and the sensation threshold 1 year postoperatively between the TAS procedure and the Ikeda-Soper procedure, but the active contractile pressure was lower after the TAS procedure than the Ikeda-Soper procedure. CONCLUSIONS: The TAS procedure is more suitable than the Soave operation via laparotomy or laparoscopy and Ikeda-Soper procedure and is feasible in infants with short segment type and common type HD.


Assuntos
Defecação , Doença de Hirschsprung/cirurgia , Laparoscopia , Laparotomia , Canal Anal , Criança , Pré-Escolar , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Recuperação de Função Fisiológica
19.
Pediatr Surg Int ; 24(6): 671-6, 2008 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18408936

RESUMO

The aim of this study was to evaluate the clinical advantages of a single-stage correction of imperforate anus with a rectourethral or a rectovestibula fistula in neonates by a semi-posterior sagittal anorectoplasty (PSARP). The medical records of 38 neonates (5 females and 33 males) who had imperforate anus with a rectourethral or a rectovestibula fistula were reviewed and analyzed in Children's Hospital of Fudan University between January 2004 and July 2007. All patients had an anorectal malformation but without obvious sacral dysplasia. The first group had a single-stage PSARP without a colostomy. All neonates were full-term without severe cardiac, renal anomalies or severe abdominal distension. The second group had a staged Peña procedure; the neonates were not suitable for a single-stage PSARP because of severe abdominal distention or cardiac, renal anomalies. A transverse colostomy was performed, followed 3-6 months later by the Peña procedure, and colostomy closure 2 months thereafter. The congenital anomalies, fetal age, birth weight, time and age of the definitive operation, complications, length of hospital stay, cost, and postoperative bowel movements were analyzed between these two groups. Among the 38 patients, 22 had a single-stage definitive operation and 16 had a staged pull-through procedure. There were significant differences in congenital anomalies between the two groups (P = 0.0314), but no statistically significant differences between the fetal ages, weight at birth, and time and age at the time of the definitive operation (P > 0.05). One patient in the first group had intestinal obstruction and intestinal perforation. For the second group, early complications were related to the colostomy. At after 3 years postoperatively, 9 patients in the first group and 12 patients in the second group were followed-up and all had positive voluntary bowel movements. There were no statistically significant differences in soiling and constipation grades between the two groups. The total length of hospital stay was 12.06 +/- 0.85 and 33.85 +/- 0.94 days and the cost was 10,681.1 +/- 1,759.5 and 27,355.9 +/- 1,952.0 RMB for the first and second groups, respectively. There was a statistically significant difference in the total length of hospital stay and cost between the two groups (P < 0.0001); however, there were no statistically significant differences in the length of hospital stay and cost during the definitive operation between the two groups (P > 0.05). This retrospective study shows that it is feasible for correction of imperforate anus with a rectourethral or a rectovestibula fistula in neonates using a modified PSARP without a colostomy.


Assuntos
Anus Imperfurado/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Análise Custo-Benefício , Procedimentos Cirúrgicos do Sistema Digestório/economia , Feminino , Humanos , Tempo de Internação , Masculino , Fístula Retal/cirurgia , Estudos Retrospectivos
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