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Background/aims: Genetic alterations, including changes in the expression of spastic paraplegia 20 (SPG20) and serine/threonine protein kinase 31 (STK31), may play an important role in the carcinogenesis of colorectal cancer (CRC). Identification of such changes is suitable for the recognition of tumors at an early stage, which would significantly improve patient survival. While recent studies have identified that SPG20 and STK31 expression levels increase in CRC tissues, their use as a biomarker is yet to be investigated. Our aim was to determine whether circulating SPG20 and STK31 mRNAlevels could help distinguish between patients with CRC and healthy individuals. Additionally, we aimed to analyze the correlation between SPG20 and STK31 expression patterns and the tumor stage in patients with CRC., Methods: Venous blood samples from 50 patients with CRC and 50 healthy controls were used. RNA extraction was performed, and the mRNA expression of SPG20 and STK31 was determined using RT-qPCR., Results: STK31 and SPG20 mRNA levels were significantly upregulated in patients compared to those in controls. There was a strong positive correlation between the expression of the two potential tumor biomarkers, STK31 and SPG20 (R=0.636, p=0.000). However, there was no significant relationship between the expression of STK31 or SPG20 and patient data, including demographic, clinical, pathological, and laboratory data. Additionally, there was a significant correlation between the expression level of STK31, but not SPG20, and patient disease-free survival (DFS) and overall survival (OS)., Conclusion: Circulating mRNA levels of SPG20 and STK31 could be used as ideal noninvasive biomarkers for early diagnosis of CRC. They could assist the oncologist in recommending appropriate management strategies for individual patients., Competing Interests: The authors declare that no conflicts of interest exist., (© Copyright by the Author(s). Published by Cell Physiol Biochem Press.)

Multi-institutional databases and registries have proliferated over the last decade in all specialties of medicine. They may be especially helpful in low-frequency/high-acuity fields such as pediatric and congenital heart diseases. The Society of Thoracic Surgeon's Congenital Heart Surgery Database (STSCHSD) is the largest single data set for the congenital heart disease population and includes contemporaneous data from over 120 programs in the United States (and several outside of the United States), capturing greater than 98% of the congenital cardiac surgical procedures in the United States. In 2010, the Congenital Cardiac Anesthesia Society partnered with the STSCHSD to incorporate anesthesia-related elements into the data set. Voluntary site participation in the anesthesia data has grown steadily. Currently, over 60 sites performing more than 60% of cardiac bypass procedures in the STSCHSD are submitting anesthesia data annually into the STSCHSD. Anesthesia data include perioperative medication usage, modalities for hemodynamic and neurologic monitoring, blood product, antifibrinolytic and procoagulant use, and anesthesia-related adverse events. This special article provides a descriptive summary of relevant findings to date, reflecting the wide variety in anesthesia practice patterns present among institutions and illustrates the functionality of a multisite registry in pediatric cardiac anesthesia which can be utilized both locally and nationally.

Objectives: To determine the incidence and degree of acute lung reperfusion injury (ARI) in patients undergoing balloon angioplasty of branch pulmonary artery stenosis and to evaluate the correlation and efficacy of an oxygenation index in confirming the clinical diagnosis., Design: Retrospective, single-center observational study., Setting: Cardiac catheterization laboratory at a tertiary care children's hospital., Patients: Patients with congenital heart disease undergoing pulmonary artery balloon angioplasty., Intervention: Review of patient medical and catheterization records., Measurements and Main Results: The records of all patients with biventricular physiology undergoing balloon angioplasty of branch pulmonary artery stenosis over a period of 2 years (12/2006-12/2008) were reviewed. Data collection included demographics, details of pulmonary artery intervention, right ventricle/femoral artery systolic pressure (RV/FA) ratio, and post-procedure recovery condition. Markers of ARI, including clinical, radiographic, and blood gas analysis, were examined. Criteria for ARI were based on the International Society of Heart and Lung Transplantation (ISHLT) grading system, in which a PaO2/FIO2 of 200 to 300 indicates ARI. The distribution of PaO2/FIO2 after pulmonary artery intervention, the relation of clinical to laboratory manifestation of ARI, and the correlation among different oxygenation indices were examined. During the study period, 46 patients with congenital heart disease and branch pulmonary artery stenosis were identified. Patient age ranged from 2 months to 25 years (mean 6.2±6 years) and weight ranged from 5 to 86 kg (mean 23±18 kg). ARI was identified in 10 of 46 patients (22%) using clinical criteria and correlated with ISHLT gas exchange criteria. Analysis of RV/FA ratio before (0.82±0.34) and after (0.71±0.22) balloon angioplasty revealed statistically significant decrease (p<0.004). The degree of ARI was graded using ISHLT criteria and correlated with the presence of clinical symptoms (p<0.002). As anticipated, the PaO2/FIO2 ratio had a strong correlation with A-aDO2 (r = 0.75) and SpO2/FIO2 (r = 0.7) and a strong specificity (0.78) to identify patients with clinical ARI., Conclusion: ARI often can occur after pulmonary artery interventions. The PaO2/FIO2 is a valuable test for identifying patients at risk of developing ARI and can help guide the care of these patients in the postintervention period., (© 2014 Elsevier Inc. All rights reserved.)

Objectives: To examine the incidence of subglottic stenosis in children with congenital heart disease undergoing surgical repair and the contributing factors to such a complication., Design: A retrospective chart review., Setting: Tertiary care children's hospital heart center., Patients: Children with congenital heart disease undergoing cardiac surgical repair and presenting with subglottic stenosis in the postoperative period., Measurements and Main Results: The authors reviewed their practice for a 4-year period (2002-2005) and identified 17 of 809 children <2 years old with congenital heart disease diagnosed with significant subglottic stenosis in the postoperative period. The incidence was 2.3% in children <1 year old and 2.1% in children <2 years old; the overall incidence was 1.08% in all children <18 years old (17/1,572) undergoing cardiac surgical repair in the same time period. Intubation duration of <24 hours had an incidence of 0.58% compared with 16% in those intubated for >96 hours. The common use of cuffed endotracheal tubes (783/809 children <2 years) did not result in an increased risk of subglottic stenosis in the authors' practice., Conclusion: Subglottic stenosis is an uncommon complication in children undergoing surgical repair of congenital heart defects. The most important risk factors are younger age and prolonged postoperative ventilation. Early diagnosis, referral, and intervention resulted in a favorable outcome in most of the authors' patients.

Study Objectives: To evaluate the hemodynamic effects of the long-acting, alpha-adrenergic blocker, phenoxybenzamine, in children of different age groups., Design: Retrospective chart review., Setting: Tertiary-care, congenital cardiac surgery center., Measurements: The data of 75 pediatric patients who received phenoxybenzamine while undergoing surgical repair of congenital heart defects on cardiopulmonary bypass (CPB) were studied. Patients were selected in three age groups: younger than one month (n = 25), one to 12 months (n = 25), and one to 5 years (n = 25). All patients received a full dose of phenoxybenzamine 1 mg/kg. Demographics, CPB duration, mean arterial pressure on CPB, mean flow on CPB (normalized to body surface area), and central-to-peripheral temperature gradients were recorded. Systemic vascular resistance index (SVRI) was calculated., Main Results: Cardiopulmonary bypass duration was significantly longer in the age group of younger than 1 mo than in the older groups. Mean CPB flow/body surface area was similar in all children (3.45 +/- 0.9, 3.74 +/- 0.69, and 3.48 +/- 0.59 L/min/m2, respectively; P < 0.28). However, mean SVRI was significantly lower in children younger than 1 mo (997.3 +/- 233, 1196.9 +/- 394, and 1168.83 +/- 227 dynes/cm2m5; P < 0.04). Temperature gradient was significantly narrower in patients younger than one month than those who were one to 12 months and one to 5 years at the end of cooling (0.90 degrees C +/- 0.1 degrees C, 1.04 degrees C +/- 3.61 degrees C, 1.4 degrees C +/- 3.07 degrees C; P < 0.001) at end-rewarming and termination of CPB (4.58 degrees C +/- 2.36 degrees C, 6.23 degrees C +/- 4.17 degrees C, 7.32 degrees C +/- 3.46 degrees C; P < 0.02). Multivariate analysis showed that patient age was a significant variable affecting response to phenoxybenzamine, after adjusting for duration of CPB (P = 0.31), mean hematocrit on CPB (P = 0.86), and core cooling temperature (P = 0.34)., Conclusion: The effect of phenoxybenzamine on SVRI, cooling, and rewarming on CPB varies with age as shown by more profound vasodilatation and narrower temperature gradients.

Objective: The primary study objective was to examine the impact of diagnosis on the inflammatory response in neonates with congenital heart disease undergoing cardiac surgery. The secondary objective was to study the impact of the inflammatory response on postoperative outcome in these neonates., Design: Observational study., Setting: Tertiary care children's hospital heart center., Patients: Neonates with hypoplastic left-heart syndrome (HLHS) undergoing stage I repair and patients with transposition of the great arteries (TGA) undergoing arterial switch operation., Measurements and Main Results: There were 24 neonates with HLHS and 21 neonates with TGA. Serum samples to measure interleukin (IL)-6 and -10 were obtained before and after CPB at 1, 3, 6, and 24 hours postoperatively. Time to extubation, intensive care unit (ICU) length of stay, and peritoneal fluid drainage were compared between the groups. Serum IL-6 and IL-10 concentrations increased after CPB when compared to the preoperative concentration. Preoperative concentrations of IL-6 were significantly elevated in the HLHS group (HLHS: 32 [21.1, 69.6] pg/mL v TGA: 7.2 [3.6, 22.5] pg/mL [median, 25th, and 75th percentile], p = 0.003) and remained elevated immediately after CPB, and at 3 and 6 hours postoperatively. The IL-10 to IL-6 ratio was lower in the HLHS group preoperatively and immediately after CPB. ICU length of stay was significantly longer in the HLHS group (TGA 4 [3-6] days v HLHS 6 [5-8] days, p = 0.031). Mortality in the HLHS group (4/24) was associated with significantly higher IL-6 postoperatively (IL-6 immediately postoperatively: HLHS survivors 59.9 [34.3, 65.7] pg/mL v nonsurvivors 98.7 [94.4, 104.5] pg/mL, p < 0.011)., Conclusions: All neonates with TGA or HLHS have a significant inflammatory response after CPB. Neonates with HLHS have evidence of an activated inflammatory response before CPB, which remains significant in the postoperative period. Accelerated interleukin expression and an abnormal cytokine balance correlate with longer time to extubation, longer ICU length of stay, and increased peritoneal fluid volume.

Regional cerebral oxygenation can be monitored using near-infrared spectroscopy (NIRS). Inadequacy of collateral cerebral circulation and regional cerebral ischemia during cardiac and vascular surgery may be detected by the use of NIRS monitoring. We report a 2-year-old child who underwent surgical repair of vascular ring and subclavian reimplantation, where use of NIRS helped in early detection and timely intervention to prevent prolonged cerebral ischemia.

To allow for growth in our anesthesiology residency, we assumed control of the clinical base year (postgraduate year 1[PGY-1]) and adjusted the curriculum to accommodate the expanded size. With this opportunity to change the curriculum, we created a clinical base year to prepare PGY-1 for clinical anesthesia training in PGY-2 to PGY-4 using, for this purpose, the best resources of our clinical site. We describe the process and preliminary results of the change.

Objectives: The purpose of this study was to compare the effects of a direct-acting arterial dilator, sodium nitroprusside, to an alpha-adrenergic receptor blocker, phenoxybenzamine, in infants with congenital heart defects undergoing cardiac repairs on cardiopulmonary bypass., Design: A prospective, multicenter, observational study., Setting: Tertiary care center., Participants: Sixty infants scheduled for elective congenital cardiac surgery repair requiring cardiopulmonary bypass., Interventions: Patients received either sodium nitroprusside 2 to 5 microg/kg/min infusion intraoperatively and in the intensive care unit (n=30 patients) or received phenoxybenzamine 1 mg/kg slowly intravenously at the onset of cardiopulmonary bypass (n=30 patients)., Measurement and Main Results: Despite similar mean arterial pressures during cardiopulmonary bypass in both groups, infants who received phenoxybenzamine had a significantly higher flow compared with those who received sodium nitroprusside (180+/-4.8 v 73+/-5.12 mL/kg/min, p<0.0001). Base deficit was significantly larger in the sodium nitroprusside group compared with the phenoxybenzamine group intraoperatively and postoperatively (3.4+/-0.5 v 1.3+/-0.5 mEq/L, p<0.05). The core-to-peripheral temperature gradient was significantly larger in the sodium nitroprusside group compared with the phenoxybenzamine group intra- and postoperatively at all points studied. In the intensive care unit, the left atrial pressure was significantly higher in the sodium nitroprusside group compared with the phenoxybenzamine group (9+/-0.4 v 7+/-0.4 mmHg, p

In children, myocardial ischemic changes during anesthesia are a rare event unless there is underlying pathology. The patient in this case report was an apparently healthy child scheduled for adenoidectomy and bilateral tympanostomy. Occurrence of significant ST changes as well as intraoperative and postoperative hypoxemia required further diagnostic work-up. Postoperative echocardiographic findings were suspicious of intrapulmonary right to left shunting. The pulmonary arteriovenous fistula is probably the major pathophysiological factor for the development of hypoxemia and paradoxical air embolism especially during positive pressure ventilation in our patient. Unexpected ST segment changes might also occur in patients with anomalous origin of coronary arteries. Although diagnostic work-up was inconclusive, it is necessary to rule out any underlying pathological process. Further follow-up is also important in order to learn more about these disease states that often have subclinical, but potentially fatal presentation.

Objective: To evaluate the effectiveness and safety of the ALLON 2001 microprocessor-based thermoregulation system in pediatric patients undergoing cardiac surgery requiring hypothermic cardiopulmonary bypass compared with the routine thermal care., Design: Prospective randomized clinical study., Setting: Single tertiary academic medical center., Participants: Infants (0-1 year) who underwent congenital heart surgery requiring hypothermic cardiopulmonary bypass (n = 18). Patients with open wounds and/or patients treated with an investigational drug or device within 30 days of surgery were excluded., Interventions: Randomized use of thermoregulation system (warming garment, n = 9) or routine thermal care (control, n = 9) after separating from cardiopulmonary bypass until the arrival to the pediatric intensive care unit (PICU)., Measurements and Main Results: There were no statistically significant differences in the demographic data, cardiopulmonary bypass time, operating room time, incidence of deep hypothermic circulatory arrest, and cooling temperature between the groups. The nasopharyngeal temperature was significantly higher in the warming garment group after separation from cardiopulmonary bypass. Nasopharyngeal temperature at 20 minutes was 36.5 degrees C versus 35.01 degrees C (p = 0.0047), at 40 minutes was 36.98 degrees C versus 35.30 degrees C (p = 0.034), and at admission to the PICU was 36.09 degrees C versus 35.31 degrees C (p = not significant). There was no difference in the core-to-peripheral temperature gradient (nasopharyngeal-to-skin temperature) between the 2 study groups at any time point. No adverse events related to the use of the warming garment thermoregulation system were observed., Conclusion: The investigated thermoregulation system was effective in preventing the after-drop of temperature that occurs after cardiopulmonary bypass in small infants compared with routine warming methods.

Implications: The authors describe the management of a patient with arrhythmogenic right ventricular dysplasia treated with right ventricular exclusion surgery.

Unlabelled: The glial-derived protein S100B is a serum marker of cerebral ischemia and correlates with negative neurological outcome after cardiopulmonary bypass (CPB) in adults. We sought to characterize the S100B release pattern before and after CPB in neonates and infants with congenital heart disease and correlate it with surgical mortality. Serum was collected before surgery and at 24 postoperative h from 109 neonates and infants with congenital heart disease. All patients had presurgical transthoracic echocardiograms and CPB with or without hypothermic circulatory arrest. S100B concentrations were determined using a two-site immunoluminometric assay (Sangtec 100). Thirty-day surgical mortality was observed. All neonates had significantly increased S100B concentrations before surgery that decreased by 24 postoperative h. Preoperative S100B concentrations in 32 neonates with hypoplastic left heart syndrome correlated inversely with the forward flow and size of the ascending aorta and postoperative mortality (r(2) = -0.63; P = 0.03). Among infants, increased pulmonary blood flow was associated with higher S100B levels before surgery than cyanosis. There was no correlation with postoperative S100B and time on CPB, hypothermic circulatory arrest, or 30-day surgical mortality. In conclusion, preoperative S100B concentrations correlate inversely with the size of the ascending aorta in hypoplastic left heart syndrome and may serve as a marker for preexisting brain injury and mortality., Implications: Neonates with hypoplastic left heart syndrome and no forward flow in the ascending aorta may have brain injury at birth before heart surgery.

Implications: Birth defects can affect the great vessels of the aorta and its branches, compressing the upper airway and the swallowing track. Surgical repair of these defects requires manipulating the airway to improve surgical exposure. This is a report of a series of cases with compromised airway resulting from such a birth defect and methods of its management.

Background: Necrotizing enterocolitis (NEC) is a serious gastrointestinal disease of unknown cause that predominantly affects premature infants, but it has been reported in term infants with congenital heart disease., Methods: In August and September of 1997, 4 cases of NEC in term infants with congenital heart disease were identified among 24 infants undergoing cardiac surgical procedures at our institution. Because nosocomial NEC had not previously been identified among infants in our pediatric intensive care unit, a case-control study was conducted to identify risk factors for NEC. All infants less than 4 months old undergoing cardiac surgery without development of NEC during the epidemic period were selected as controls., Results: The only significant risk factor associated with case-infants was a higher mean temperature gradient (rectal/toe) during the immediate 12-hour postoperative period (4.0 degrees C vs 2.0 degrees C, P <.01). Urine output was higher in control-infants in this postoperative period, although not statistically significant (11.9 mL/h vs 6.1 mL/h, P =.15)., Conclusion: Although an infectious etiology cannot be ruled out, the cases of NEC in infants with congenital heart disease after cardiac procedures may have resulted from mesenteric ischemia associated with a low perfusion state in the perioperative period.

The use of blood and blood products in cardiac surgery has undergone marked changes over the past three decades. This revolution occurred primarily through the study and understanding of hemodilution. This review marks the changes in the practice of blood transfusion in cardiac surgery. We review the methods of blood conservation through preoperative screening and preparation, intraoperative drug therapy and modification of surgical techniques, and postoperative use of shed mediastinal blood. We also examine the physiologic effects and limitations of hemodilution in adult and pediatric cardiac surgery. Finally, we review the recent trends in the use of blood component therapy and the future of blood substitutes.