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Introduction The objective of this study is to summarize the clinical characteristics and management of rare diseases of colorectal vascular malformation (CRVM) in children. Methods We retrospectively analyzed the clinical data of CRVM patients admitted to the Children's Hospital of Fudan University from 2004 to 2019. Results A total of 23 cases (16 males, 7 females) were enrolled. The median age of symptom onset was 1.4 years. Hematochezia and anemia were cardinal symptoms. Fourteen patients (60.9%) were misdiagnosed as anal fissures (n = 4), internal hemorrhoids (n = 3), rectal polyps (n = 2), inflammatory bowel disease (n = 2), portal hypertension (n = 2), and Meckel's diverticulum (n = 1), respectively. The average time from symptom onset to diagnosis was 4.5 ± 4.4 years. Other vascular malformations were detected in eight patients (34.8%). All patients showed a positive anomalous vascular image on contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI). The sensitivity of colonoscopy in the diagnosis of CRVM was 82.6% (19/23). A total of 21 patients underwent a modified Soave procedure. The lesions were mostly restricted to the colorectum and showed transmural diffuse distribution, with an average length of 20 ± 5.4 cm. Two patients (9.5%) experienced surgical complications. Bloody stools reappeared in two patients (9.5%), and colonoscopy showed abnormal angiogenesis at the anastomotic site, which were cured by sclerotherapy and/or electrocautery. The median follow-up time was 78 months. Bloody stools were absent at the last time of follow-up, and hemoglobin was in the normal range for all patients. Conclusion The identification of CRVM in children often is delayed. Colonoscopy, CT, and MRI are essential in making the correct diagnosis. The modified Soave procedure is safe and feasible to treat CRVM in children. Endoscopic sclerotherapy and/or electrocautery can be used for residual lesions.

BackgroundIt has been proposed that the dose of rescue opioids should be individually titrated to the severity of obstructive sleep apnea after adenotonsillectomy. However, a sleep study is not always available before adenotonsillectomy. This randomized, controlled and blinded trial evaluated a strategy of pain control individualized to the results of a fentanyl test, rather than the results of polysomnography, in children after adenotonsillectomy.MethodsA total of 280 children (3–10 years old) undergoing elective adenotonsillectomy were randomized into an individualized protocol (IP) group or a conservative protocol (CP) group. All patients received a fentanyl test before extubation. Pain was assessed every 10 min in the recovery room, and rescue morphine was given when the Children’s Hospital of Eastern Ontario Pain Scale (CHEOPS) score was > 6. In the IP group, the dose of rescue morphine was individualized to the results of the fentanyl test (10 μg/kg in the case of a positive result and 50 μg/kg in the case of a negative result). In the CP group, the dose was fixed (25 μg/kg). The primary outcome was the percentage of patients requiring at least one medical intervention. The secondary outcome was the median duration of CHEOPS scores > 6.ResultsFewer patients in the IP group than in the CP group required medical interventions [11.9% (16/134) vs 22.3% (29/130),P = 0.025]. The median duration of CHEOPS scores > 6 was shorter in the IP group than in the CP group [20 (95% CI: 17 to 23) min vs 30 (95% CI: 28 to 32) min,P ConclusionsCompared with a conservative dosing approach, this individualized protocol may improve analgesia without a significant increase in respiratory adverse events.Trial registrationClinicalTrials.govNCT02990910, registered on 13/12/2016.

Background Children with severe obstructive sleep apnea syndrome (OSAS) are more sensitive to opioids. Identifying such children and reducing or even eliminating opioids are necessary but difficult. We have previously shown that patients sensitive to intraoperative fentanyl require less opioids postoperatively. Aim The objective of this study was to evaluate the performance of a postinduction fentanyl test in identifying severe obstructive sleep apnea syndrome. Methods A prospective, observational, assessor-blinded study was carried out with 104 sleep study assessed children undergoing elective adenotonsillectomy. Intravenous fentanyl (1 µg/kg) was administered as a test in nonpremedicated, spontaneously breathing, sevoflurane-induced patients before endotracheal intubation. The respiratory rates before and after fentanyl administration were studied. The primary outcome was the sensitivity and specificity of the postinduction fentanyl test in identifying severe OSAS compared with polysomnography. Results A postinduction fentanyl test had a likelihood ratio of 7.2 (95% CI: 3.6-14.6) and an area under the curve value of 0.896 (95% CI: 0.821-0.947) to identify severe obstructive sleep apnea syndrome. The pragmatic cut-off value for the postinduction fentanyl test was found to be 50%. Using a reduction in respiratory rate of >50%, the postinduction fentanyl test detected severe OSAS with a sensitivity of 87%, a specificity of 88%, a positive predictive value of 85%, and a negative predictive value of 89%. Conclusion Our study showed that a postinduction fentanyl test had good predictive value in identifying severe obstructive sleep apnea syndrome and early postoperative adverse respiratory events and could provide a reference for postoperative analgesia in children undergoing adenotonsillectomy.

To investigate the mechanism of 25 hydroxyvitamin D (25(OH)D) deficiency in children with biliary atresia (BA) and its effect on liver fibrosis. The serum vitamin D and 25(OH)D, and expression of 25 hydroxylase (CYP2R1 and CYP27A1) in the liver of BA patients were detected and compared with those in the control group. We investigated the effect of differential expression of CYP2R1 in hepatocytes on the expression of genes related to liver fibrosis in primary hepatic stellate cells (HSCs) of BA and animal models of cholestasis. The ratio of 25(OH)D/vitamin D in the BA group was significantly lower than that in the control group. The mRNA and protein expression of CYP2R1 and CYP27A1 in liver tissue of the BA group was significantly lower than that in the control group. Exogenous active vitamin D (calcitriol) inhibited the proliferation and migration of primary HSCs isolated from BA patients, and reduced the expression of fibrosis-related genes in vitro. Downregulation of expression of CYP2R1 in hepatocytes increased expression of transforming growth factor (TGF)-β1, collagen (Col)-1α1 and tissue inhibitor of metalloproteinase (TIMP)-1, and decreased the expression of matrix metalloproteinase (MMP)-2 in cocultured primary HSCs of BA. Upregulation of expression of CYP2R1 in mice with bile duct ligation significantly increased the level of 25(OH)D, decreased the expression of TGF-β1, Col-1α1 and TIMP-1, and increased the expression of MMP-2. Children with BA have impaired vitamin D activation due to CYP2R1 deficiency. The dysactivation of vitamin D can promote the proliferation and activation of HSCs and participate in the development of hepatic fibrosis in BA.

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 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.

The cross-resistance and biochemical mechanism of the beet armyworm, Spodoptera exigua (Hubner), to spinosad was studied in the laboratory. S. exigua population were collected from Shanghai suburb. After five generations of selection, the resistance of S. exigua to spinosad increased 345.4 times compared with the susceptible strain. There was no cross-resistance between spinosad and fenvalerate, phoxim, methomyl, abamectin, and cyfluthrin. When the inhibitors, PBO, TPP, DEF, and DEM were used as synergist in the susceptible strain and resistant strain, the synergistic ratio was 0.7-, 0.5-, 1.0-, and 0.6- fold for the susceptible strain, and 9.8-, 1.5-, 2.6-, and 1.5-fold for the resistant strain, respectively. The results revealed that PBO had significant synergistic effect on the resistant strain. The activity in vitro of microsomal-O-demethylase and glutathione S-transferase in the resistant strain was 5.2- and 1.0-fold of the susceptible strain, respectively. The results implied that microsomal-O-demethylase might be important in conferring spinosad resistance in the S. exigua population.

The relationship between the cytochrome P-450 (cytP450 monooxygenase and insecticide resistance mechanisms in Helicoverpa armigera was studied. The level of cytP450 and activity of p -ni-troanisole (p -NA) O-demethylase in resistant strain were 1. 71 times and 2. 21 times respectively higher as compared with those of the susceptible strain. Aldrin epoxidase activity showed 1. 35 fold strain difference. Therefore aldrin epoxidase might not be important for H. armigera resistance mechanism, the level of cytP450 and activity of p -NA O-demethylase might play a vital role in H. armigera resistance mechanisms. The reason of different resistance mechanisms among different H. armigera resistant strains is discussed.

This paper explains the economic rationale for adopting Hub-and-Spoke structure in transport networks. Hub-and-spoke and Fully-Connected are two fundamental types of transport network structure. The reason for adopting one structure instead of the other in some cases is that it has certain economic benefits over the other. On the basis of a discussion on the concept of economies of scale/density/scope in transport, a comparative analysis has been made on the transport costs and the economies of scale/density/scope indexes between the Hub-and-Spoke and the Fully-Connected structure in transport network. We argue that the stimulation for adopting the network structure of Hub-and-Spoke is to exploit the economies of traffic density and the precondition for such exploitation is there exist the economies of scope.