Langerhans cell histiocytosis in children with refractory diarrhoea and hypoalbuminaemia as the initial presentation: two case reports and a literature review.

Langerhans cell histiocytosis (LCH) involving the gastrointestinal tract is a rare condition for which clinical experience is limited. We describe the cases of two patients who initially presented with chronic diarrhoea, hypoproteinaemia, and intermittent fever. These findings suggest that in cases of refractory diarrhoea accompanied by recurrent hypoalbuminaemia, especially with abdominal rash, LCH should be considered. Gastrointestinal endoscopy, biopsy, and imaging studies are essential for obtaining a definitive diagnosis. This approach might be helpful for the early recognition of gastrointestinal tract involvement in LCH.

Catheter-related bloodstream infections in children with intestinal failure: a 6-year review from an intestinal rehabilitation center in China.

BACKGROUND: Children with intestinal failure (IF) have frequent catheter-related bloodstream infections (CRBSIs). This study aimed to analyze the clinical presentation and laboratory parameters of CRBSIs in children with IF. METHODS: This 6-year retrospective study was conducted among IF children with CRBSIs at an intestinal rehabilitation center in China. Clinical data were collected, including data of temperature and gastrointestinal symptoms. Blood/catheter culture, fecal tests, and calculation of inflammatory index were performed, which were obtained within 1 week since CRBSI onset. RESULTS: Fifty children with 87 CRBSIs were identified, of which there were 17 suspected and 70 confirmed cases. Seventy-two pathogens were cultured from 70 positive blood cultures: 63% were Gram-positive organisms, 23% were Gram-negative organisms, and 11% were fungal organisms. Overall, 48.6% were enteric organisms; 47.2% of bacterial pathogens were consistent between fecal and blood cultures. Moreover, 46.3% fecal routines showed abnormalities including increased white blood cells, occult blood positive and the presence of fat droplets. The consistent symptom at onset of CRBSIs was fever and gastrointestinal symptoms including increased stool output, abdominal distension, or both. C-reactive protein (CRP) and procalcitonin (PCT) were elevated, i.e., 16.5 mg/L [interquartile range (IQR) 8.7-44.7] and 0.48 ng/mL (IQR 0.2-1.76), respectively. CONCLUSIONS: IF children had a high rate of CRBSIs, of which larger proportions were due to Gram-positive and enteric organisms. Fever and/or gastrointestinal symptoms, combined with elevated CRP and PCT, is conducive to the early diagnosis of CRBSIs in IF patients.

Alterations in thymocyte populations under conditions of endotoxin tolerance.

BACKGROUND: Endotoxin tolerance (ET) is a protective phenomenon in which pre-treatment with a tolerance dose of lipopolysaccharide (LPS) leads to dramatically elevated survival. Accumulating evidence has shown that peripheral T cells contribute to the induction of ET. However, what happens to T cell development in the thymus under ET conditions remains unclear. The purpose of this study was to analyze the alterations in thymocyte populations (double-positive [DP] and single-positive [SP] cells) under ET conditions. METHODS: Mice were intraperitoneally injected with LPS at a concentration of 5 mg/kg to establish an LPS tolerance model and were divided into two groups: a group examined 72 h after LPS injection (72-h group) and a group examined 8 days after LPS injection (8-day group). Injection of phosphate-buffered saline was used as a control (control group). Changes in thymus weight, cell counts, and morphology were detected in the three groups. Moreover, surface molecules such as CD4, CD8, CD44, CD69, and CD62L were analyzed using flow cytometry. Furthermore, proliferation, apoptosis, cytokine production, and extracellular signal-regulated kinase (ERK) pathway signaling were analyzed in thymocyte populations. The polymorphism and length of the T-cell receptor (TCR) ß chain complementarity-determining region 3 (CDR3) were analyzed using capillary electrophoresis DNA laser scanning analysis (ABI 3730). RESULTS: Thymus weight and cell counts were decreased in the early stage but recovered by the late stage in a murine model of LPS-induced ET. Moreover, the proportions of DP cells (control: 72.130 ±â€Š4.074, 72-h: 10.600 ±â€Š3.517, 8-day: 84.770 ±â€Š2.228), CD4+ SP cells (control: 15.770 ±â€Š4.419, 72-h: 44.670 ±â€Š3.089, 8-day: 6.367 ±â€Š0.513), and CD8+ SP cells (control: 7.000 ±â€Š1.916, 72-h: 34.030 ±â€Š3.850, 8-day: 5.133 ±â€Š0.647) were obviously different at different stages of ET. The polymorphism and length of TCR ß chain CDR3 also changed obviously, indicating the occurrence of TCR rearrangement and thymocyte diversification. Further analysis showed that the expression of surface molecules, including CD44, CD69, and CD62L, on thymocyte populations (DP and SP cells) were changed to different degrees. Finally, the proliferation, apoptosis, cytokine production, and ERK pathway signaling of thymocyte populations were changed significantly. CONCLUSION: These data reveal that alterations in thymocyte populations might contribute to the establishment of ET.

Hereditary pancreatitis of 3 Chinese children: Case report and literature review.

BACKGROUND: Hereditary pancreatitis (HP) is quite rare and is distinguished by incomplete penetrance presentation as early-onset relapsing pancreatitis, usually beginning in childhood. HP is now known to be commonly relevant to mutations in the PRSS1 (gene-encoding cationic trypsinogen), SPINK1 (serine protease inhibitor, Kazal type 1), CFTR (cystic fibrosis), carboxypeptidase A1 (CPA1), and chymotrypsin C (CTRC) genes as reported in some Caucasian studies. HP has a variable spectrum of severity and may develop complications. METHODS & RESULTS: We describe the clinical course of 3 preschool children, hospitalized with postprandial abdominal pain, whose laboratory tests showed high serum amylase. Similar episodes of abdominal pain led to readmission, and the patients recovered quickly after using symptomatic therapy. The condition of the first boy, who developed a pancreatic tail pseudocyst and splenic infarction, was especially complicated. The boy underwent 2 endoscopic retrograde cholangiopancreatographies and stenting, along with a surgical procedure that completely relieved his symptoms for 3 months. The 3 patients and their parents were given genetic testing. All of the patients carried 1 or more gene mutations inherited from their mothers, fathers, or both parents; however, none of the parents were affected. CONCLUSION: For children with repeated pancreatitis, clinicians should consider HP in the differential diagnosis. It is reliable to perform gene sequencing on suspicious patients and their parents. Multidisciplinary and comprehensive treatment should be recommended to manage HP and its complications. Cholangiopancreatography and stenting is a relatively minimally invasive approach when compared with surgery and can be tried as an early intervention. Surgical procedures should be reserved for patients with complications.

Use of the Conventional Side-viewing Duodenoscope for Successful Endoscopic Retrograde Cholangiopancreatography in Postgastrectomy Patients.

OBJECTIVES: The aim of this study was to evaluate the usefulness of the conventional side-viewing duodenoscope for successful endoscopic retrograde cholangiopancreatography (ERCP) in postgastrectomy patients. METHODS: A total of 220 consecutive patients with bile duct stones or a distal common bile duct stricture who had previously undergone gastrectomy and were referred for ERCP were analyzed for the outcome of their ERCP. All ERCP procedures were performed using a conventional side-viewing duodenoscope. In patients who had undergone a Billroth II gastroenterostomy and total gastrectomy with Roux-en-Y reconstruction, we also used the procedure of retrieval balloon-assisted enterography. RESULTS: The study group included 220 patients who had previously undergone gastrectomy (77 women and 143 men; mean age, 72.2 y; range, 11 to 93 y). The overall enterography success rate was 90.5% (199/220), and the diagnostic and ERCP success rates were both 88.6% (195/220). Endoscopy was unsuccessful in 21 patients who received Billroth II gastroenterostomy and Roux-en-Y reconstruction. After successful endoscopy, diagnostic and ERCP success was not achieved in 4 patients with Billroth II gastroenterostomy, with or without Braun anastomosis, due to cannulation failure. The procedure-related complication rate was 5.5% (12/220), including immediate bleeding (0.9%, 2/220), pancreatitis (4.1%, 9/220), and perforation (0.5%, 1/220). There were no procedure-related deaths. CONCLUSIONS: The side-viewing duodenoscope is a useful instrument for performing successful ERCP in patients postgastrectomy. In addition, retrieval balloon-assisted enterography may improve the enterography success rate in postgastrectomy patients with Billroth II and Roux-en-Y reconstruction.

Antisense oligonucleotides against microRNA-21 reduced the proliferation and migration of human colon carcinoma cells.

BACKGROUND: Colon carcinoma is one of the commonly tumors that threaten human beings as its highly morbidity and mortality. Recent evidences suggested that microRNA-21 (miR-21) played an important role in the development of colon carcinoma and might be a potential biological marker for the diagnosis and prognosis of colon carcinoma. However, the potential effect of miR-21 based therapeutic studies in colon carcinoma remains to be fully elucidated. METHODS: In present study, we constructed an eukaryotic expression vector encoding antisense oligonucleotides against miR-21 (termed as p-miR-21-ASO) and the expression of miRNA-21 in human colon cancer was detected by Real-time PCR. To assess its possible effect on the proliferation and migration capacity of human colon carcinoma cells in vitro, CCK-8 assay, colony formation assay and cell invasion, as well as migration assay, were performed respectively. Moreover, PTEN, one of target molecules of miRNA-21, was analyzed by Western blot and Fluorescence activated cell sorter assay. Finally, the transduction of AKT and ERK pathways in human colon carcinoma cells was determined by Western blot. RESULTS: We found that transiently transfection of p-miR-21-ASO could efficiently decrease the relative expression of miR-21 in human colon carcinoma HCT116 cells, accompanied by impaired proliferation and clone formation. Furthermore, we found that down-regulation of miR-21 also could significantly abrogate the invasion and migration capacity in vitro, as well as the expression of vascular endothelial growth factor which is critical for the metastatic capacity of colon carcinoma cells. Mechanistic evidence showed that down-regulation of miR-21 increased the expression of its target molecule PTEN in HCT116 cells. Finally, we revealed that the expression level of both phosphor-ERK1/2 and phosphor-AKT also were altered. CONCLUSIONS: Therefore, our data suggested miR-21 ASO against miR-21 might be a useful strategy to alter the expression of miR-21 in colon carcinoma cells, which was helpful for the development of miR-21-based therapeutic strategies against clinical colon carcinoma.

Retrieval-balloon-assisted enterography for ERCP after Billroth II gastroenterostomy and Braun anastomosis.

AIM: To describe an optimal route to the Braun anastomosis including the use of retrieval-balloon-assisted enterography. METHODS: Patients who received a Billroth II gastroenterostomy (n = 109) and a Billroth II gastroenterostomy with Braun anastomosis (n = 20) between January 2009 and May 2013 were analyzed in this study. Endoscopic retrograde cholangiopancreatography (ERCP) was performed under fluoroscopic control using a total length of 120 cm oblique-viewing duodenoscope with a 3.7-mm diameter working channel. For this procedure, we used a triple-lumen retrieval balloon catheter in which a 0.035-inch guidewire could be inserted into the "open-channel" guidewire lumen while the balloon could be simultaneously injected and inflated through the other 2 lumens. RESULTS: For the patients with Billroth II gastroenterostomy and Braun anastomosis, successful access to the papilla was gained in 17 patients (85%) and there was therapeutic success in 16 patients (80%). One patient had afferent loop perforation, but postoperative bleeding did not occur. For Billroth II gastroenterostomy, there was failure in accessing the papilla in 15 patients (13.8%). ERCP was unsuccessful because of tumor infiltration (6 patients), a long afferent loop (9 patients), and cannulation failure (4 patients). The papilla was successfully accessed in 94 patients (86.2%), and there was therapeutic success in 90 patients (82.6%). Afferent loop perforation did not occur in any of these patients. One patient had hemorrhage 2 h after ERCP, which was successfully managed with conservative treatment. CONCLUSION: Retrieval-balloon-assisted enterography along an optimal route may improve the ERCP success rate after Billroth II gastroenterostomy and Braun anastomosis.

ERCP for patients who have undergone Billroth II gastroenterostomy and Braun anastomosis.

Endoscopic retrograde cholangiopancreatography (ERCP) is efficacious in patients who have undergone Billroth II gastroenterostomies, but the success rate decreases in patients who also have experienced Braun anastomoses. There are currently no reports describing the preferred enterography route for cannulation in these patients. We first review the patient's previous surgery records, which most often indicate that the efferent loop is at the greater curvature of the stomach. We recommend extending the duodenoscope along the greater curvature of the stomach and then advancing it through the "lower entrance" at the site of the gastrojejunal anastomosis, along the efferent loop, and through the "middle entrance" at the site of the Braun anastomosis to reach the papilla of Vater. Ten patients who had each undergone Billroth II gastroenterostomy and Braun anastomosis between January 2009 and December 2011 were included in our study. The overall success rate of enterography was 90% for the patients who had undergone Billroth II gastroenterostomy and Braun anastomosis, and the therapeutic success rate was 80%. We believe that this enterography route for ERCP is optimal for a patient who has had Billroth II gastroenterostomy and Braun anastomosis and helps to increase the success rate of the procedure.

Entering the duodenal diverticulum: a method for cannulation of the intradiverticular papilla.

Successful cannulation of the common bile duct may be difficult in patients in whom the papilla is located entirely within a diverticulum. In this study, we report successful biliary cannulation in three patients following intubation of the distal tip of the duodenoscope into the duodenal diverticulum and locating the major papilla. No complications occurred during the operation or during the postoperative period. This method didn't need second incubation an endoscope and might lower the burden of patients. So this skill is useful to deal with the papilla hidden inside the large diverticulum because of its safety and convenience.