Effects of Notch signalling pathway on the relationship between vascular endothelial dysfunction and endothelial stromal transformation in atherosclerosis.

At present, with the improvement of living standards and population aging, the incidence of cardiovascular and cerebrovascular disease is on the rise and has been a serious threat to human health. Statistics show that the current death caused by cardiovascular and cerebrovascular disease has become the first cause of death has been increasing year by year. Therefore, studies on coronary heart disease and atherosclerosis (AS) have become a hot topic in clinical and basic research. In this study, the question of the effect of Notch signalling pathway on the relationship between endothelial dysfunction and endothelial stromal transformation in AS was studied in depth. Based on our results, we drew conclusions as follows. First, the Notch signalling pathway was activated in the atherosclerotic model; secondly, the Notch signalling pathway was demonstrated to enhance AS by promoting vascular endothelial dysfunction; thirdly, it was demonstrated that the Notch signalling pathway was mediated by promoting endothelial and to enhance AS; finally, we confirmed the endothelial function through the Notch signalling pathway to affect the transformation of endothelial stroma to achieve synergistic AS effect. The results of this study have a good guiding significance for the important role of Notch signalling in AS and indicate the ability to influence endothelial function and endothelial stromal transformation by intervening Notch signalling pathway and can affect the relationship between them, and thus eventually achieve the treatment of AS.

Duhamel operation vs. transanal endorectal pull-through procedure for Hirschsprung disease: A systematic review and meta-analysis.

OBJECTIVE: To compare treatment outcomes in children with Hirschsprung's disease who underwent treatment using the Duhamel or TERPT surgical procedures. METHODS: Medline, Cochrane, EMBASE, and Google Scholar databases were searched through December 26, 2016. Search strings included Hirschsprung's disease, fecal incontinence, transanal endorectal pull-through, and Duhamel operation. Randomized controlled studies (RCTs) and retrospective studies that compared the treatment of Hirschsprung's disease in with TERPT or Duhamel surgical procedures in neonates, infants, or children were included. RESULTS: The study included six studies with a total of 280 patients. The meta-analysis indicated that the Duhamel and TERPT interventions were similar with respect to rate of postoperative fecal incontinence (OR=0.85, 95% CI=0.37 to 1.92, P=0.692) and operation time (difference in means=46.68min, 95% CI=-26.96 to 114.31, P=0.226). The Duhamel procedure was associated with longer postoperative hospital stay (Difference in means=3.14days, 95% CI=1.46 to 4.82, P<.001) and a lower rate of enterocolitis (OR=0.21, 95%=0.07 to 0.68, P=0.009) compared with the TERPT procedure. CONCLUSIONS: The study found that Duhamel and TERPT procedures showed similar benefit in treating Hirschsprung's disease, although differences exist with respect to length of postoperative hospital stay and the incidence of enterocolitis. THE TYPE OF STUDY: Meta-analysis. LEVEL OF EVIDENCE: Level II.

Comparison of the Nuss versus Ravitch procedure for pectus excavatum repair: an updated meta-analysis.

BACKGROUND/PURPOSE: To evaluate surgical outcomes of Nuss versus Ravitch repair of pectus excavatum via a systematic review and meta-analysis. METHODS: Medline, PubMed, Cochrane, EMBASE, and Google Scholar databases were searched up to September 5, 2016 using the following search terms: pectus excavatum, funnel chest, Nuss; Ravitch, minimally invasive, and open surgery. Randomized controlled trials, two-arm prospective, and two-arm retrospective studies were eligible for inclusion. RESULTS: Nineteen studies were included with a total of 1731 patients: 989 treated with Nuss and 742 treated with Ravitch. The overall analysis revealed that patients in the Nuss group had significantly shorter operation time (pooled SMD=-2.83, 95% CI=-3.76 to -1.90, p<0.001) and less blood loss (pooled SMD=-1.68, 95% CI=-2.28 to -1.09, P<0.001) than the Ravitch group. However, the length of hospital stay was similar between groups (pooled SMD=-0.55, 95% CI=-1.44 to 0.35, p=0.230). These findings were similar in the subgroup analysis for randomized and non-randomized controlled studies. Complications were not assessed due to inconsistent reporting across the included studies. CONCLUSIONS: Our meta-analysis demonstrate that the Nuss procedure has a shorter operative time and less operative blood loss than the Ravitch procedure while the postoperative length of stay was similar. LEVELS OF EVIDENCE: Level III.

Foxp3 promoter methylation impairs suppressive function of regulatory T cells in biliary atresia.

Biliary atresia (BA) is characterized by progressive inflammation of the biliary system leading to liver cirrhosis, necessitating liver transplantation in pediatric patients. Various cell types have been reported to participate in the proinflammatory response in rhesus rotavirus (RRV)-induced BA mouse models, including T helper (Th) 1, Th2, Th17, CD8+ T cells, and natural killer cells. The immune suppressive regulatory T (Treg) cells, on the contrary, were reported not to function properly. The underlying mechanism is largely unknown. Focusing on the impaired suppressive function of Treg, we found methylation status of CpG islands within the Foxp3 promoter region of Treg cells in BA patients and murine models were both increased. Moreover, by injecting 5-aza-2'-deoxycytidine (Aza) as DNA-methylation inhibitor to RRV-infected mice, BA phenotypes were alleviated. Furthermore, Treg cells isolated from "RRV+Aza"-injected mice had better suppressive function than Treg cells from mice injected with RRV only, both in vivo and ex vivo. Thus we concluded that aberrant increased methylation status of "Foxp3 promoter" in Treg cells leads to impaired Treg suppressive function, exacerbating inflammatory injury in BA.

A Modified Approach for Inguinal Hernias in Children: Hybrid Single-Incision Laparoscopic Intraperitoneal Ligation.

PURPOSE: To report a laparoscopic approach for pediatric inguinal hernia repair using a hybrid single-incision laparoscopic (H-SIL) technique and its clinical outcomes. MATERIALS AND METHODS: A retrospective study was carried out in inguinal hernia cases treated with the new H-SIL approach using intracorporeal jumping purse-string sutures. The operative time, length of postoperative hospital stay, efficiency of the operation, and complications were analyzed. RESULTS: In total, 157 inguinal high ligations were performed in 106 children (89 boys, 17 girls). The median age was 1.5 years (range, 25 days-11.6 years). The mean operative time was 15.8±3.4 minutes for the single-side procedure and 20.3±2.5 minutes for bilateral procedures. The mean postoperative hospital stay was 0.99±0.52 (range, 0.25-3 days). No postoperative bleeding, abdominal wall emphysema, abdominal viscera injury, or scrotal edema was found, and there were no known cases of postoperative testicular atrophy or hypotrophy. Ninety-three percent of the patients became fully mobile on the first postsurgical day. The median follow-up period was 17 months (range, 9-21 months), with no recurrence, no visible scars on the abdominal wall, and no foreign body felt in the inguinal region. CONCLUSIONS: This H-SIL approach is a safe and efficient method for pediatric inguinal hernia repair. The maneuverability is the same as that in the triport laparoscopic technique, and the cosmetic results are similar to those of single-port laparoscopic surgery.

Elevated Th17 cells accompanied by decreased regulatory T cells and cytokine environment in infants with biliary atresia.

PURPOSE: The aim of this study was to investigate the role of Th17 and T reg cells in biliary atresia (BA) and to assess the liver cytokine environment in BA patients. METHODS: The percentages of Th17 and T reg cells in peripheral blood mononuclear cells (PBMCs) of BA patients and healthy controls (HC) were evaluated. The serum concentrations of IL-17a and IL-23 as well as Foxp3, IL-17a, ROR-γt, IL-6, IL-1ß and TGF-ß1 m-RNA and protein expressions in liver tissues and the number of Foxp3, IL-17a, ROR-γt, CD4 expressing cells which infiltrated the hepatic tissues were determined. RESULTS: The Th17/T reg cell ratio (P < 0.001) and blood concentrations of IL-17a and IL-23 (P < 0.05) were increased in the BA as compared to the HC group. Expressions of Foxp3, ROR-γt, IL-17a, IL-1ß, IL-6 as well as TGF-ß1 mRNA and proteins were significantly increased in BA as compared to HC livers (P < 0.01, P < 0.05). High levels of IL-17a/ROR-γt-positive and moderate levels of Foxp3-positive cells infiltrated damaged BA bile ducts and the ratio of FoxP3+ T to CD4+ T cells was significantly lower in BA than in HC samples (P < 0.01). CONCLUSION: Cytokine-induced imbalance between Th17 and T reg cells in BA livers may be involved in bile duct damage.

10 years of experience with laparoscopic-assisted endorectal Soave pull-through procedure for Hirschsprung's disease in China.

OBJECTIVE: To report early and late results of laparoscopic-assisted endorectal Soave pull-through (LAEPT) with a short and V-shaped partial resection muscular cuff for Hirschsprung's disease (HD) over a 10-year period. METHODS: The clinical courses of 218 patients who underwent modified LAEPT for HD were reviewed. LAEPT was described by the Georgeson technique. The main modifications included less dissection of the bottom of the pelvis, rectal mucosa dissection with a long cuff, coloanal anastomosis with a short cuff, and a V-shaped partial resection in the posterior wall of the muscular cuff. RESULTS: From September 1999 to July 2009, 218 patients were operated on by the same surgeon. Ages ranged from 15 days to 12 years old. The aganglionic segment was located in the rectum or sigmoid colon in 176 patients, in the left colon in 38 children, and in the transverse colon in 4 patients. The median operating time was 176 minutes. Conversion to open surgery was required in 2 patients. Bleeding of the left iliac vein occurred in 1 patient, and a 180° twist of the neorectum occurred in 2 cases. Median first bowel movement time was 23 hours, and median postoperative hospital stay was 10.4 days. Median daily defecation frequency was 4.6 within 2 weeks and 2.3 at 3 months postoperatively. The immediate postoperative complications included intestine herniation from the trocar site in 2 patients, perianal excoriation in 32 patients, and anastomotic leakage in 3 patients. Follow-up ranging from 6 to 120 months was obtained for 182 patients. Late postoperative complications included postoperative adhesive bowel obstruction (1.1%), enterocolitis (7.7%), anasomostic stenosis (2.2%), constipation (1.6%), and soiling (3.6%). Eighty-seven percent had excellent and good bowel function. CONCLUSION: LAEPT with a short muscular cuff with a V-shaped partial resection in the posterior wall is a safe and effective procedure for HD.

Transanal endorectal pull-through for Hirschsprung's disease using long cuff dissection and short V-shaped partially resected cuff anastomosis: early and late outcomes.

BACKGROUND: Transanal endorectal pull-through was described by De la Torre-Mondragon's technique. In the original transanal pull-through procedure, a long rectal muscular cuff was dissected and left for anocolic anastomosis, which would sometimes lead to postoperative obstructive symptoms and enterocolitis. While a modified short mucosal dissection may increase the risk of injury to pelvic and perirectal nerves and other structures when dissected on the outside of the rectum deep in the pelvis. We report early and late results of the modified transanal procedure for Hirschsprung's disease (HD) over 8 years. METHODS: The clinical course of all children with aganglionic rectum or sigmoid colon receiving the modified transanal pull-through between May 2003 and April 2011 were reviewed. The main modifications were rectal mucosa dissection with a long cuff, coloanal anastomosis with a short cuff and a V-shaped partial resection in the posterior wall of the muscular cuff. Children with preliminary stoma or total colonic disease were excluded. RESULTS: Short- and long-term follow-up was obtained in 137 patients with HD operated upon by the same surgeon. The aganglionic segment was located in the rectum or sigmoid colon in all patients. The mean age at surgery was 165 ± 74 days. The mean operating time was 108 ± 38 min. Mean intra-operative blood loss was estimated to be 15 ± 10 ml. No patient required a blood transfusion. Mean postoperative hospital stay was 7 ± 2 days. Early postoperative complications included perianal excoriation in 38 patients (27.7 %), enterocolitis in two patients (1.4 %), and anastomotic leak in two patients (1.4 %). Late postoperative complications included perianal excoriation in 16 patients (11.7 %), anal stricture in two patients (1.4 %), constipation in four patients (2.8 %), enterocolitis in 10 patients (7.3 %), and soiling problems in six patients (4.4 %). Mean follow-up was 56 months (6 months-9 years). In patients older than 4 years, 85.4 % of them had excellent/good bowel function, 9.4 % had fair bowel function, and 5.2 % of patients had bad bowel function. CONCLUSION: Transanal endorectal pull-through with a long cuff dissection and a short V-shaped resected cuff anastomosis is a safe and effective procedure for HD. It reduced incidence of anastomotic stricture and constipation without an increased soiling incidence.

Laparoscopically assisted anorectal pull-through for high imperforate anus in infants: intermediate results.

PURPOSE: The aim of this study was to evaluate the clinical outcomes and postoperative anal function in infants with congenital high imperforate anus treated with laparoscopically assisted anorectal pull-through (LAARP). METHODS: From January 2004 to July 2007, 33 patients (28 boys and 5 girls, age ranging from 3 to 10 months) with high imperforate anus underwent LAARP. Clinical data of the LAARP group were retrospectively compared with those treated by posterior sagittal anorectoplasty (PSARP; n = 28) during the same time period. Anorectal function of these patients was evaluated using the following 3 methods: the Kelly score, anorectal vector volume manometry, and magnetic resonance imaging between the ages of 3.1 and 4.4 years. RESULTS: The mean operative time in LAARP and PSARP groups was 112.5 ± 12.4 and 120.4 ± 18.5 minutes (P > .05), respectively. The mean length of hospital stay in the LAARP group was shorter than that of PSARP group (11.3 ± 2.1 vs 14.6 ± 2.3 days, P < .01). No significant difference was observed between LAARP and PSARP groups regarding the Kelly score (3.52 ± 1.42 vs 3.49 ± 0.82). Although magnetic resonance imaging revealed lower malposition rates of rectum in the LAARP group than those of the PSARP group at both I-line (3.0% vs 14.3%) and M-line (3.0% vs 10.7%) levels, this was not statistically different (P > .05). Compared with the PSARP group, lower asymmetric index, larger vector volume, and higher anal canal pressure at rest and during voluntary squeeze were observed in LAARP group (P < .05). However, there were no significant differences in the length of high-pressure zone (15.2 ± 5.8 vs 15.1 ± 6.2 mm) and the presence of rectoanal relaxation reflex (84.8% vs 85.7%). CONCLUSIONS: Satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectal pull-through has advantages over PSARP, including shorter hospital stay and better position of rectum. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.

Laparoscopic choledochal cyst excision, hepaticojejunostomy, and extracorporeal Roux-en-Y anastomosis: a technical skill and intermediate-term report in 62 cases.

BACKGROUND: This study aimed to present the authors' technique and the intermediate-term outcome for laparoscopic choledochal cyst excision with Roux-en-Y hepatoenterostomy. METHODS: This retrospective study investigated 62 children (39 girls and 23 boys) who had undergone laparoscopic resection of choledochal cyst. The average age of the children was 2.3 years. The retrospective data and the following investigations about type of choledochal cyst, surgical technique, conversion rate, morbidity, and mortality were analyzed. RESULTS: Of the 62 patients, 43 (69.4%) showed type 1a choledochal cysts, 16 (25.8%) showed type 1c, 2 (3.2%) showed type 4a, and 1 (1.6%) showed type 4b. Total cyst excision could be performed for 51 of the patients (82.3%). The large cysts were opened on the front wall, then divided circumferentially in 29 cases. The small cysts did not need to be opened before excision in 22 cases. For 11 patients (17.7%), Lilly's (Surg Gynecol Obstet 146:254-256, 1978) technique was adopted, and for 5 patients with a huge cyst, the duodenum together with the head of the pancreas had to be mobilized for visualization of the cyst's lower limit. The hepatic duct was excised, and plastic operation of bile duct was performed for two patients. The mean operative time was 226±41.2 min. Eight patients needed blood transfusion, and conversion was required for one patient. The mean hospital stay was 8±1.5 days, and the mean follow-up period was 38 months. The overall morbidity rate was 8.2% (5/61) including bile leakage (n=1), adhesive small bowel obstruction (n=1), intestinal necrosis (n=1), and cholangitis (n=1). Inflammatory edema anastomotic narrowing occurred in one patient. None of the patients needed surgery due to anastomotic stricture. CONCLUSIONS: Laparoscopic choledochal cyst excision, hepaticojejunostomy, and extracorporeal Roux-en-Y anastomosis can be safely and quickly performed for children, with satisfactory intermediate-term results. Extracorporeal Roux-en-Y anastomosis could shorten the operative time.

Endoscopic transaxillary approach for congenital muscular torticollis.

BACKGROUND: Surgical correction of the congenital muscular torticollis (CMT) is recommended for patients with unsuccessful conservative treatment. Open operative techniques all leave noticeable scars. We proposed a modified endoscopic 1-trocar transaxillary and neck microincision approach for the treatment of CMT. METHODS: Endoscopic release of the sternocleidomastoid (SCM) muscle was performed in 45 infants and children aged 6 months to 15 years. One 5-mm incision was made in the anterior axillary fold, and a subcutaneous tunnel over the clavicular and sternal heads of the SCM muscle was made. A subcutaneous space was established by CO2 inflation at a pressure of 8 mm Hg and then endoscopically using a 5-mm endoscope. Two additional 1.5- to 2-mm supraclavicular mini-incisions were made beside the SCM muscle for the introduction of miniforceps and electrocautery, respectively. The sternal and clavicular attachments were dissected and divided by electrocautery. Clinical evaluation was performed using the Lee scoring system. RESULTS: The operation was successfully completed endoscopically in all 45 children. The mean operative time was 40 minutes. No injuries of major blood vessels or nerves were encountered. A small bleed was noted in 1 child owing to reoperation. Follow-up for 6 months to 3 years in 42 patients showed complete muscular release and satisfactory cosmetic appearance with no recurrence. The results were classified as excellent in 88.1% (37/42), good in 9.5% (4/42), fair in 2.4% (1/42), and poor in 0 using the Lee scoring system. The neck scars were not visible 1 month after the procedure. CONCLUSIONS: The subcutaneous endoscopic transaxillary and micro-neck incision approach for the treatment of CMT is a safe, practical procedure that provides good functional and cosmetic outcomes without vascular or neural injury.

Laparoscopic extensive colectomy with transanal Soave pull-through for intestinal neuronal dysplasia in 17 children.

BACKGROUND: Open colectomy has been preferred for intestinal neuronal dysplasia type B (IND) due to its low morbidity rate and good functional results. The aim of this study was to investigate the feasibility and results of laparoscopic colectomy with transanal Soave pull-through for the treatment of IND in children. METHODS: Seventeen infants and children suffering from IND were treated by laparoscopic extensive colectomy with transanal Soave pull-through. The diagnosis of IND was made via anorectal manometry, X-ray contrast enema, suction biopsies, and laparoscopic full-thickness biopsies with hematoxylin-eosin staining. The technique used four or five abdominal ports. The sigmoid, transverse, and right colon up to the last ileal cove were mobilized laparoscopically in the extended form of IND. A modified Soave's anastomosis was performed. The patients' data, surgical procedures, operative data, postoperative complications and clinical outcomes were analyzed. RESULTS: Five patients underwent laparoscopic left colectomy with modified transanal Soave procedures, and the other 12 were treated by laparoscopic subtotal colectomy and required a Deloyers' maneuver for the Soave pull-through. The proximal margin of barium stagnation in patients with left colectomy was restricted to the distal end of the descending colon, sigmoid colon, and that in patients with subtotal colectomy was restricted to the proximal end of the descending colon, transverse colon, hepatic flexure, and ascending colon. Postoperative complications included anastomotic leakage, severe perianal erosions, postoperative enterocolitis, and soiling. During a mean follow-up of 4 years, bowel frequency was 4-10 times per day in 3 months postoperatively in patients with subtotal colectomy. The clinical results were good, with no stool incontinence or constipation. CONCLUSIONS: Laparoscopic procedure for left colectomy and subtotal colectomy with transanal Soave pull-through in infants and children with IND is safe, feasible, and effective. The location of barium stagnation in proximal margin may be used as a method to predict initially the proximal margin of the resected bowel segment.

The evaluation of laparoscopy-assisted cholangiography in the diagnosis of prolonged jaundice in infants.

BACKGROUND: Biliary atresia (BA) is the progressive inflammatory obstruction and fibro-obliteration of all or part of the extrahepatic biliary tree and the intrahepatic bile ducts and has its onset exclusively within the first several months of life. This study was undertaken to present the value of diagnostic laparoscopy in infants with prolonged jaundice and technique for laparoscopic cholangiography. METHODS: A 5-mm umbilical trocar was introduced to create a port for a 30-degree laparoscope. If the gallbladder was of good size, the fundus was exteriorized through the right subcostal trocar site and a catheter was inserted into the gallbladder for cholangiography, following partial dissection from the liver bed, if required. If the gallbladder was atretic, the fundus was not exteriorized and a laparotomy was performed and cholangiography was abandoned, because the lumen of an atretic gallbladder was usually not fully patent. RESULTS: At laparoscopy, 12 patients had good-sized gallbladders and minimal-to-mild liver fibrosis. They underwent cholangiography via the exteriorized fundus, and infantile hepatitis syndrome (HIS) or cholestatic syndrome (CS) in 8 cases, BA in 2 cases, and biliary hypoplasia (CBDH) in 2 cases were identified. Five patients' gallbladders dissected from the liver bed underwent cholangiography, and BA in 3 cases and CBDH in 2 cases were identified. The remaining 21 had atretic gallbladders and varying degrees of liver fibrosis, so cholangiography via the exteriorized fundus was abandoned and converted to open Kasai portoenterostomy. CONCLUSIONS: Laparoscopy-assisted cholangiography is a simple, accurate, and safe method in the diagnosis of prolonged jaundice in infants and allows the anatomic structure of the biliary tree to be obtained accurately with minimal surgical intervention.

Clinical value of pelvic 3-dimensional magnetic resonance image reconstruction in anorectal malformations.

OBJECTIVE: The study aimed to build a 3-dimensional (3D) reconstruction of pelvic magnetic resonance images and evaluate the clinical value in anorectal malformations (ARMs). METHODS: Magnetic resonance imaging (MRI) examinations were performed on a 1.5-T magnet. Sagittal, coronal, and transverse turbo spin-echo T1-weighted and fast spin-echo T2-weighted images of the pelvic region were obtained in 22 children. A 3D reconstruction was made on a computer and assisted by the 3D-Doctor software (Trial Version, Able Software Corp). The level and type of ARM and the developmental state of the striated muscle complex (SMC) were analyzed with 3D reconstruction image. RESULTS: The 3D images of the pelvic were confirmed in 22 cases. Three-dimensional reconstructed images perfectly displayed the anatomical relationships of the SMC and the rectal atresia in these spaces. The 3D configuration of the SMC was different in each of the high- and low-type cases. The high-type malformation of SMCs differed particularly from the descriptions. CONCLUSIONS: Pelvic magnetic resonance 3D reconstructed images were able to show the dimensional anatomical relations of pelvis, bladder, urethra, rectum, and SMC. Both a 3D image and positional information with MRI offers the surgeon a simulated operative profile of the SMC superior to MRI slices alone, which will help in providing morphological data for image diagnosis and operation of the ARM.

Nuss operation for pectus excavatum: a single-institution experience.

BACKGROUND: The Nuss procedure for repair of pectus excavatum (PE) has been accepted worldwide because of minimal invasiveness and excellent cosmetic results. We summarized our experience with the treatment of 115 patients aged 2.7-18 years. METHODS: All the 115 patients underwent the Nuss procedure successfully from July 2003 to February 2008. They were divided into two groups: children group (below 12 years) and adolescents group (aged 12-18 years). RESULTS: The rate of complications was 14.7% and 37.5% in the children and adolescents groups, respectively (P<0.05). There was significant difference in operation time, length of hospital stay, and analgesic time between the two groups (P<0.05). The initial results of Nuss procedure were excellent. CONCLUSIONS: The Nuss procedure can be performed with excellent early results in children. We suggest that children with PE should accept the Nuss procedure as early as possible when they are over 5 years old.

Expression of matrix metalloproteinase 1 and 2 in congenital choledochal cyst and its clinical significance.

BACKGROUND: The pathogenesis of congenital choledochal cyst (CCC) is still not well elucidated. This study was conducted to detect the expression of matrix metalloproteinase-1 and 2 (MMP-1 and MMP-2) in CCC. The roles of MMP-1 and MMP-2 in CCC formation were also discussed. METHODS: Specimens of CCC and gallbladders were taken from 53 patients with CCC, including 21 boys and 32 girls aged from 4 months to 17 years (mean: 6.5 years old). Twenty-eight normal gallbladders served as controls. Immunohistochemical method was used to detect the expressions of MMP-1 and MMP-2. Image analysis was made to quantitatively analyze the protein expression. RESULTS: The expressions of MMP-1 and MMP-2 were located in epithelial cells, matrix cells, and inflammatory cells of the CCC wall. Various expressions were also found in the mucosa membrane cells of the CCC gallbladder. The expressions of MMP-1 and MMP-2 were low in the control group. Quantitatively, the expressions of MMP-1 and MMP-2 in the CCC wall were markedly higher than in the CCC gallbladder and the control group (P<0.01). There was no significant difference between the CCC gallbladder and the control group (P>0.05). CONCLUSIONS: The expression of MMP-1 and MMP -2 in the CCC wall is higher than in the CCC gallbladder and controls. The over-expression of MMP-1 and MMP-2 may be involved in the pathogenesis of CCC.

Diagnosis and treatment of biliary atresia: a retrospective study.

BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrhosis. Early diagnosis of BA is difficult, and there is no specific therapy for BA at present. The purpose of this study was to investigate the diagnosis, treatment and postoperative outcome of BA and to explore new diagnostic and therapeutic strategies. METHODS: Thirty-one children with biliary atresia from our hospital and 201 children with such disease from other hospitals in China were reviewed retrospectively in terms of diagnostic modalities, operative age, operative methods and long-term survival rates after operation. RESULTS: The operative age of the 31 patients varied from 13 to 270 days. It was <60 days in 6 children (19%), 61-90 days in 14 (45%), 91-120 days in 8 (26%), and >120 days in 3 (10%). No children underwent transplantation for BA. Their 1-6 year survival rate on average was 32.3% (10/31). The early operative rate in China was 12.9% (30/232). One child received liver transplantation with a long-term survival rate of 31.9%. CONCLUSIONS: Early recognition of babies with BA is critical for optimal intervention for preventing progressive fibrosis. Clinical presentation, imaging and laparoscopy are helpful in enhancing the early diagnostic rate of BA patients. The long-term survival rates can be obtained after the improvement of operative methods, liver transplantation for children with advanced BA, and development of other medical strategies.