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1.

Background

In children with extrahepatic portal vein obstruction (EHPVO), formation of a mesentericoportal bypass (Rex shunt) restores hepatopetal flow, relieves portal hypertension, and reduces variceal bleeding and hypersplenism. The Rex shunt is created by inserting a vein graft between the superior mesenteric vein and the umbilical segment (Rex) of the left portal vein within the Rex recess of the liver. The preoperative evaluation of a patient with EHPVO includes an accurate assessment of the venous inflow and outflow. The inflow portal vein is readily assessed by ultrasound and magnetic resonance imaging. The outflow intrahepatic portal vein is harder to assess. We report our experience of patients evaluated with wedged hepatic vein carbon dioxide portography (WHVCP).

Method

All children referred for venography from October 2001 to October 2007 were prospectively identified, and clinical and radiologic data were reviewed retrospectively. The imaging findings were correlated to findings at surgery.

Results

Eleven children (range, 3-14 years, median, 6 years) were referred for preoperative wedged hepatic venography. The left portal vein at the Rex recess was clearly identified in 9 patients (82%). In the other 2 patients (18%), the Rex segment was not identified despite opacification of left and right intrahepatic portal veins; this was taken to indicate an occluded segment. Wedged venography was performed with carbon dioxide in 10 patients (91%). Carbon dioxide was contraindicated in the final patient because of the presence of a ventricular septal defect.

Conclusion

Our series demonstrates the use of WHVCP as a diagnostic tool in preoperative assessment of the Rex segment of left portal vein in children with extrahepatic portal vein obstruction.  相似文献   

2.

Purpose

The mesenteric to left portal vein bypass (MLPVB) has been successfully used to treat extrahepatic portal vein obstruction (EHPVO) in children. We examined the effect of failed prior surgical or radiological procedures intended to treat complications of portal hypertension on the success rate of subsequent MLPVB surgery.

Methods

Sixty-two patients younger than 18 years with EHPVO underwent MLPVB between 1997 and 2006. Children were divided into 3 groups: those with no prior surgery related to portal hypertension, those with prior portosystemic shunts, and those with either splenectomy or mesenteric vascular embolization procedures. The effect of prior procedures on the patency rate of the MLPVB was then examined.

Results

Of 62 children, 11 (17.7%) had significant procedures to treat symptoms of portal hypertension: 6 had at least 1 portosystemic shunt attempt, 3 had isolated splenectomy, and 2 had embolization of the splenic artery or coronary and peripancreatic varices. Patients with previous portal hypertension surgery were significantly older and larger than those with no surgery. Patients with no prior interventions had a significantly higher MLPVB patency rate (88.2%, 45/51) than those with no prior interventions (63.6%, 7/11). Prior splenectomy alone was not found to adversely affect MLPVB. Patients with prior embolization procedures or unsuccessful shunts had significantly poorer successful outcomes (0% and 66.7%) than those with no prior interventions (88.2%; P < .005).

Conclusions

The results demonstrate that prior portosystemic shunts or mesenteric embolizations have a deleterious effect on outcome after MLPVB and should be avoided whenever possible. This study suggests that patients with symptomatic EHPVO should undergo MLPVB as a primary intervention rather than as a rescue procedure to optimize MLPVB patency.  相似文献   

3.

Purpose

The purpose of this study was to evaluate the feasibility, safety, and efficacy of portal vein recanalization (PVR) and propose a new classification for better selecting candidates with portal vein occlusion (PVO) in whom PVR could be feasible.

Materials and methods

The charts of 15 non-cirrhotic patients in whom stent placement using a trans-hepatic approach was attempted for the treatment of PVO with cavernous transformation were reviewed. There were 12 men and 5 women with a mean age of 47 ± 12 years (range: 22–60 years). Intrahepatic involvement was classified into 3 groups according to the intrahepatic extent of PVO: type 1 included occlusions limited to the origin of the main portal vein and/or the right or left portal branches, type 2 included type 1 plus extension to the origin of segmental branches, type 3 included type 2 plus extension to distal branches.

Results

There were 6 patients with PVO type 1, 7 patients with PVO type 2, and 2 patients with PVO type 3. Indications for PVR were gastrointestinal bleeding (n = 6), portal biliopathy (n = 2), reduce portal pressure before surgery (n = 4), or other (n = 3). PVR was successful in 13 patients (87%) with no severe side effects. Failure of PVR or early stent thrombosis occurred in 100% of type 3 vs. 8% of type 1 and 2 patients (P = 0.03). During a mean follow-up of 42 ± 28 months (range: 6–112 months), patients with a permeable stent had resolution of portal hypertension-related manifestations. In 13 patients in whom PVR was feasible, stent permeability was 77% at 2 years (87% vs. 60% in patients who received anticoagulation or not, respectively; P = 0.3).

Conclusion

PVR is feasible in most patients with non-cirrhotic, non-tumoral portal vein occlusion when there is no extension of the occlusion to distal branches.  相似文献   

4.

Background/Purpose

Extrahepatic portal vein obstruction (EHPVO) is an important cause of chronic portal hypertension in children. Although usually idiopathic in etiology, genetic and acquired thrombophilia have been implicated in EHPVO. Meso-Rex bypass is increasingly used to treat EHPVO in children.

Objective

The objective of this study is to assess the relationship of postoperative anticoagulation strategies and thrombophilic risk factors to the development of bypass thrombosis following the meso-Rex bypass.

Methods

Records of children who underwent meso-Rex bypass for EHPVO at a single institution from 1999 to 2009 were reviewed, and preoperative thrombophilia testing, perioperative anticoagulation strategies, and postoperative bypass patency based on imaging at last follow-up were examined.

Results

Sixty-five children with EHPVO underwent a first time meso-Rex bypass during the study period, and 9 of 65 (14 %) developed bypass thrombosis. The use of warfarin in the postoperative period was more common among children with thrombosed shunts than among those with open shunts [63 % vs. 20 %; OR, 6.5 (95 % CI, 1.3–31.5), p?=?0.022]. The contribution of genetic or acquired thrombophilia to shunt thrombosis was inconclusive given variability in testing.

Conclusions

Choice of anticoagulation following meso-Rex bypass may affect postoperative incidence of bypass thrombosis. Role of thrombophilic risk factors in the development of shunt thrombosis remains unclear.  相似文献   

5.

Introduction

Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear.

Methods

Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography.

Results

Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time.

Conclusions

These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.  相似文献   

6.

Background

Extrahepatic portal vein obstruction (EHPVO) has been associated with growth impairment in children. We hypothesized that growth parameters improve after reversal of portal hypertension and restoration of mesenteric venous blood flow to the liver by the mesenterico-left portal vein bypass (MLPVB).

Methods

A retrospective review of 45 children with idiopathic EHPVO who underwent MLPVB between 1997 and 2007 and had follow-up data for analysis was carried out. Growth was assessed using SD scores (z scores) for height, weight, and body mass index (BMI) at the time of operation and at early (5-12 months) and late (13-24 months) follow-up.

Results

The mean height and weight of children with EHPVO was significantly lower than the general population before surgery. Mean BMI was also lower, although statistically insignificant. All parameters increased significantly after MLPVB as follows: height from −0.42 before surgery to −0.12 (P = .027) at 5 to 12 months and −0.14 (P = .026) at 13 to 24 months; weight from −0.49 before surgery to 0.03 (P < .001) at 5 to 12 months and 0.35 (P < .001) at 13 to 24 months; and BMI from −0.22 before surgery to 0.17 (P = .001) at 5 to 12 months and 0.48 (P < .001) at 13 to 24 months.

Conclusion

Restoration of portal blood flow to the liver by MLPVB improves growth in children with EHPVO.  相似文献   

7.

Background/Purpose

Portosystemic shunt operations are indicated in patients with extrahepatic portal hypertension owing to portal vein thrombosis (EPH-PVT) suffering from recurrent variceal bleeding despite endoscopic sclerotherapy. Mesenterico left portal bypass procedure (MLPB) is an alternative procedure to the portosystemic shunt operations in patients with EPH-PVT. MLPB operation reestablishes hepatopetal portal blood flow. We herein present our experience with MLPB in children with EPH-PVT.

Methods

Six patients were treated for EPH-PVT with recurrent bleeding despite endoscopic sclerotherapy (2 boys and 4 girls) in our unit. All patients were evaluated preoperatively with complete blood count, portal duplex system Doppler ultrasonography, magnetic resonance angiography, and upper gastrointestinal (GI) endoscopy. MLPB operation was performed as described by de Ville de Goyet. During the postoperative period, patients were evaluated with complete blood count, portal duplex system Doppler ultrasonography, upper GI endoscopy, and magnetic resonance angiography.

Results

Six patients were assessed to be candidates for MLPB procedure and were operated to perform the MLPB procedure. Left portal veins were found to be patent during the operation in 4 patients, and the MLPB procedure was performed. Internal jugular vein was used in 3 patients and enlarged inferior mesenteric vein in 1 patient. Left portal veins of the remaining 2 patients were found to be obliterated; therefore, mesocaval shunt was performed. The postoperative course of the patients was uneventful except for 1 patient. During the following period, the leukocyte and the platelet counts were significantly increased in 3 of the 4 patients after the MLPB procedure. Upper GI bleeding occurred in the early postoperative period in 1 patient with MLPB procedure because of prepyloric ulcer that was successfully treated by endoscopic sclerotherapy. Internal jugular vein graft thrombosis was detected on the 10th postoperative day. This patient underwent a second laparotomy, the distal half of the graft was found to be sclerosed and narrowed that the graft was revised with a synthetic allograft.

Conclusions

Based on a review of the literature, the MLPB functions well in patients with portal hypertension caused by portal vein thrombosis and appears to have a physiologic advance over shunts that decompress but do not return blood directly to the liver. Because intra-abdominal veins appear to function well as a conduit in this operation, it may be favored by eliminating additional incision and increased risk in such patients.  相似文献   

8.

Background

Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up.

Method

We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction.

Results

Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count.

Conclusion

Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence.  相似文献   

9.

Background

Liver nodules have been reported after portal systemic shunt surgery (PSSS) in animal experiments or in humans with liver cirrhosis. The aim of our study was to assess the incidence of liver nodules after surgery for extrahepatic portal vein obstruction (EHPVO) in children without associated liver disease.

Methods

We retrospectively reviewed the charts of 45 children who had surgery from 1979 to 2005 for EHPVO in our institution, consisting of 38 PSSS and 7 portal reperfusion procedures (PRPs). We assessed the presence of liver nodules on ultrasonography.

Results

Of 45 patients, 7 (15%) had liver nodules during a median of 80 months of follow-up. All the nodules occurred after PSSS. Five nodules were subjected to biopsy; we found 2 liver cell adenomas and 3 focal nodular hyperplasias.

Conclusions

In this study, liver nodules occurred in 18% of cases after PSSS for EHPVO in children and not after PRP. As many children have undergone PSSS throughout the world, the presence of liver nodules should be considered during the follow-up of those patients.  相似文献   

10.

Purpose

To correlate point-shear wave elastography (SWE) with liver hypertrophy after right portal vein embolization (RPVE) and to determine its usefulness in predicting postoperative liver failure in patients undergoing partial liver resection.

Patients and methods

Point-SWE was performed the day before RPVE in 56 patients (41 men) with a median age of 66 years. The percentage (%) of future remnant liver (FRL) volume increase was defined as: %FRLpost?%FRLpre%FRLpre×100 and assessed on computed tomography performed 4 weeks after RPVE.

Results

Median (range) %FRLpre and %FRLpost was respectively, 31.5% (12–48%) and 41% (23–61%) (P < 0.001), with a median %FRL volume increase of 25.6% (?8; 123%). SWE correlated with %FRL volume increase (P = ?0.510; P < 0.001). SWV (P = 0.003) and %FRLpre (P < 0.001) were associated with %FRL volume increase at multivariate regression analysis. Forty-three patients (77%) were operated. Postoperative liver failure occurred in 14 patients (32.5%). Median SWE was different between the group with (1.68 m/s) and without liver failure (1.07 m/s) (P = 0.018). The AUROC of SWE predicting liver failure was 0.724 with a best cut-off of 1.31 m/s, corresponding to a sensitivity of 21%, specificity of 96%, positive predictive value 75% and negative predictive value of 72%. SWE was the single independent preoperative variable associated with liver failure.

Conclusions

SWE assessed by point-SWE is a simple and useful tool to predict the FRL volume increase and postoperative liver failure in a population of patients with liver tumor.  相似文献   

11.

Purpose

The aim of this study was to determine whether US reduces number of puncture attempts, procedure time, and complication rate during IJV access in children.

Methods

A prospective study was performed in children (age ≤ 18 years) admitted to our institution, from September 2013 to July 2014, with indications for central venous access. Patients meeting the inclusion criteria were randomized to the US-guided or control groups. The same physician performed all IJV cannulations in both groups. The end-points for comparison were: length of time to venous access, number of attempts, and rate of complications.

Results

Fifty-one patients were included: 23 in the US-guided group and 28 in the control group. There were no between-group differences in weight, age, or sex. In the US-guided group, the number of punctures needed to achieve IJV access (median [interquartile range], 3 [2–5] vs. 1 [1, 2]; P < 0.001), time to achievement of venous access, and complication rate (39% vs. 4.3%, P < 0.009) were significantly lower.

Conclusion

US guidance is a useful adjunct to central venous access in children, facilitating the procedure, decreasing time to cannulation, and increasing safety.

Type of study

Prospective randomized study.

Level of evidence

1.  相似文献   

12.

Background

Children with congenital heart disease (CHD) often require noncardiac surgery. We compared outcomes following open and laparoscopic intraabdominal surgery among children with and without CHD.

Methods

We performed a retrospective cohort study using the 2013–2015 National Surgical Quality Improvement Project-Pediatrics. We matched 45,012 children < 18 years old who underwent laparoscopic surgery to 45,012 children who underwent open surgery. We determined the associations between laparoscopic (versus open) surgery and 30-day mortality, in-hospital mortality, 30-day morbidity, and postoperative length-of-stay.

Results

Among children with minor CHD, laparoscopic surgery was associated with lower 30-day mortality (Odds Ratio [OR] 0.34 [95% Confidence Interval 0.15–0.79]), inhospital mortality (OR 0.42 [0.22–0.81]) and 30-day morbidity (OR 0.61 [0.50–0.73]). As CHD severity increased, this benefit of laparoscopic surgery decreased for 30-day morbidity (ptrend = 0.01) and in-hospital mortality (ptrend = 0.05), but not for 30-day mortality (ptrend = 0.27). Length-of-stay was shorter for laparoscopic approaches for children at cost of higher readmissions. On subgroup analysis, laparoscopy was associated with lower odds of postoperative blood transfusion in all children.

Conclusions

Intraabdominal laparoscopic surgery compared to open surgery is associated with decreased morbidity in patients with no CHD and lower morbidity and mortality in patients with minor CHD, but not in those with more severe CHD.

Level-of-evidence

Level III: Treatment Study.  相似文献   

13.

Background/Purpose

The effect of portal flow deprivation to the liver on bile composition and the biliary system remains undefined in children. This report catalogues the authors’ experience with biliary tract problems in children with extrahepatic portal vein thrombosis (EHPVT).

Methods

Twenty-nine children with symptomatic idiopathic EHPVT were evaluated for the Rex shunt procedure (mesenterico-left portal bypass) over a 4-year period. The authors retrospectively reviewed all operative reports and pre- and postoperative abdominal ultrasound findings with regard to associated congenital anomalies and abnormal biliary tract findings.

Results

Seven of the 29 patients with EHPVT (24%) had associated nonbiliary congenital abnormalities. Twenty-four of 29 (83%) patients had detectable biliary tract pathology by ultrasound examination. Biliary symptoms developed in 3 of the 9 (33%) patients with either stones or sludge (10.3% of all patients). Two patients were treated by cholecystectomy. There was no statistical correlation between biliary tract pathology and the age of presentation, symptoms of portal hypertension, gender, or underlying medical condition.

Conclusions

The authors have noted a high incidence of biliary tract pathology in patients with EHPVT compared with the normal population and a 10% incidence of symptomatic biliary pathology in this series.  相似文献   

14.

Purpose

This study examines non-accidental trauma (NAT) fatalities as a percentage of all injury fatalities and identifies injury patterns in NAT admissions to two level 1 pediatric trauma centers.

Methods

We reviewed all children (< 5 years old) treated for NAT from 2011 to 2015. Patient demographics, injury sites, and survival were obtained from both institutional trauma registries.

Results

Of 4623 trauma admissions, 557 (12%) were due to NAT. However, 43 (46%) of 93 overall trauma fatalities were due to NAT. Head injuries were the most common injuries sustained (60%) and led to the greatest increased risk of death (RR 5.1, 95% CI 2.0–12.7). Less common injuries that increased the risk of death were facial injuries (14%, RR 2.9, 95% CI 1.6–5.3), abdominal injuries (8%, RR 2.8, 95% CI 1.4–5.6), and spinal injuries (3%, RR 3.9, 95% CI 1.8–8.8). Although 76% of head injuries occurred in infants < 1 year, children ages 1–4 years old with head injuries had a significantly higher case fatality rate (27% vs. 6%, p < 0.001).

Conclusion

Child abuse accounts for a large proportion of trauma fatalities in children under 5 years of age. Intracranial injuries are common in child abuse and increase the risk of death substantially. Preventing NAT in infants and young children should be a public health priority.

Type of study

Retrospective Review.

Level of evidence

II  相似文献   

15.

Introduction

Contrast-enhanced CT remains the first-line imaging for evaluating postoperative abscess (POA) after appendicitis. Given concerns of ionizing radiation use in children, we began utilizing quick MRI to evaluate POA and summarize our findings in this study.

Materials and Methods

Children imaged with quick MRI from 2015 to 2017 were compared to children evaluated with CT from 2012 to 2014 using an age and weight matched case–control model. Radiation exposure, size and number of abscesses, length of exam, drain placement, and patient outcomes were compared.

Results

There was no difference in age or weight (p > 0.60) between children evaluated with quick MRI (n = 16) and CT (n = 16). Mean imaging time was longer (18.2 ± 8.5 min) for MRI (p < 0.001), but there was no difference in time from imaging order to drain placement (p = 0.969). No children required sedation or had non-diagnostic imaging. There were no differences in abscess volume (p = 0.346) or drain placement (p = 0.332). Thirty-day follow-up showed no difference in readmissions (p = 0.551) and no missed abscesses. Quick MRI reduced imaging charges to $1871 from $5650 with CT.

Conclusion

Quick MRI demonstrated equivalent outcomes to CT in terms of POA detection, drain placement, and 30-day complications suggesting that MRI provides an equally effective, less expensive, and non-radiation modality for the identification of POA.

Type of Study

Retrospective Case–Control Study.

Level of Evidence

Level III.  相似文献   

16.

Introduction

Physiologic compromise in children with acute appendicitis has heretofore been difficult to measure. We hypothesized that the Compensatory Reserve Index (CRI), a novel adjunctive cardiovascular status indicator, would be low for children presenting with acute appendicitis in proportion to their physiological compromise, and that CRI would rise with fluid resuscitation and surgical management of their disease.

Methods

Ninety-four children diagnosed with acute appendicitis were monitored with a CipherOx CRI? M1 pulse oximeter (Flashback Technologies Inc., Boulder, CO). For clarity, CRI = 1 indicates supine normovolemia, CRI = 0 indicates hemodynamic decompensation (systolic blood pressure < 80 mmHg), and CRI values between 1 and 0 indicate the proportion of volume reserve remaining before collapse. Results are presented as counts with proportion (%), or mean with 95% confidence interval (CI).

Results

Mean age was 11 years old (95% CI: 10–12), and 49 (52%) of the children were male. Fifty-four (57%) had nonperforated appendicitis and 40 (43%) had perforated appendicitis. Mean initial CRI was significantly higher in those with nonperforated appendicitis compared to those with perforated appendicitis (0.57, 95% CI: 0.52–0.63 vs. 0.36, 95% CI: 0.29–0.43; P < 0.001). The significant differences in mean CRI values between the two groups remained throughout the course of treatment, but lost its significance at 2 h after surgery (0.63, 95% CI: 0.57–0.70 vs. 0.53, 95% CI: 0.46–0.61; P = 0.05).

Conclusion

Low CRI values in children with perforated appendicitis are indicative of their lower reserve capacity owing to peritonitis and hypovolemia. CRI offers a real-time, noninvasive adjunctive tool to monitor tolerance to volume loss in children.

Level of evidence

Study of diagnostic test; Level of evidence: Level III.  相似文献   

17.

Introduction

Anthropometric measurements can be used to define pediatric malnutrition. Our study aims to: (1) characterize the preoperative nutritional status of children undergoing abdominal or thoracic surgery, and (2) describe the associations between WHO-defined acute (stunting) and chronic (wasting) undernutrition (Z-scores <?2) and obesity (BMI Z-scores > + 2) with 30-day postoperative outcomes.

Methods

We queried the Pediatric NSQIP Participant Use File and extracted data on patients’ age 29 days to 18 years who underwent abdominal or thoracic procedures. Normalized anthropometric measures were calculated, including weight-for-height for < 2 years, BMI for ages ≥ 2 years, and height for age. Logistic regression models were developed to assess nutritional outlier status as an independent predictor of postoperative outcome.

Results

23,714 children (88% ≥ 2y) were evaluated. 4272 (18%) were obese, while 2640 (11.1%) and 904 (3.8%) were stunted and wasted, respectively, after controlling for gender, ASA/procedure/wound classification, preoperative steroid use, need for preoperative nutritional support, and obese children had higher odds of SSIs (OR 1.29, 95% CI 1.1–1.5, p = 0.001), while stunted children were at increased risk of any 30-day postoperative complication (OR 1.16, 95% CI 1.0–1.3, p = 0.036).

Conclusion

Children who are stunted or obese are at increased risk of adverse outcome after abdominal or thoracic surgery.

Level of Evidence

III  相似文献   

18.

Purpose

The purpose of this study was to determine the usefulness of point shear wave elastography (p-SWE) of the liver and spleen for the detection of portal hypertension in pediatric patients.

Materials and methods

The study consisted of 38 healthy children and 56 pediatric patients with biopsy-proven liver disease who underwent splenic and liver p-SWE. The diagnostic performance of p-SWE in detecting clinically significant portal hypertension was assessed using receiver operating characteristic (ROC) curves.

Results

Reliable measurements of splenic and liver stiffness with p-SWE were obtained in 76/94 (81%) and 80/94 patients (85%), respectively. The splenic stiffness was highest in the portal hypertension group (P < 0.01). At ROC curve analysis, the area under the curve in the detection of portal hypertension was lower for splenic p-SWE than for liver p-SWE (0.906 vs. 0.746; P = 0.0239). The cut-off value of splenic p-SWE for portal hypertension was 3.14 m/s, with a specificity of 98.59% and a sensitivity of 68.18%. The cut-off value of liver p-SWE for portal hypertension was 2.09 m/s, with a specificity of 80.28% and a sensitivity of 77.27%.

Conclusion

In pediatric patients, p-SWE is a reliable method for detecting portal hypertension. However, splenic p-SWE is less accurate than liver p-SWE for the diagnosis of portal hypertension.  相似文献   

19.

Background

A recent single-centre study demonstrated that MRI is sensitive to detect early abnormalities in the lung and response to therapy in infants and preschool children with cystic fibrosis (CF) supporting MRI as an outcome measure of early CF lung disease. However, the feasibility of multicentre standardisation remains unknown.

Objective

To determine the feasibility of multicentre standardisation of chest MRI in infants and preschool children with CF.

Methods

A standardised chest 1.5 T MRI protocol was implemented across four specialised CF centres. Following training and initiation visits, 42 infants and preschool children (mean age 3.2 ± 1.5 years, range 0–6 years) with clinically stable CF underwent MRI and chest X-ray (CXR). Image quality and lung abnormalities were assessed using a standardised questionnaire and an established CF MRI and CXR score.

Results

MRI was successfully performed with diagnostic quality in all patients (100%). Incomplete lung coverage was observed in 6% and artefacts also in 6% of sequence acquisitions, but these were compensated by remaining sequences in all patients. The range of the MRI score in CF patients was similar across centres with a mean global MRI score of 13.3 ± 5.8. Cross-validation of the MRI against the CXR score revealed a moderate correlation (r = 0.43–0.50, p < 0.01).

Conclusion

Our results demonstrate that multicentre standardisation of chest MRI is feasible and support its use as radiation-free outcome measure of lung disease in infants and preschool children with CF.  相似文献   

20.

Background

The pathogenesis of gut inflammation, bacterial dysbiosis and increased rates of malignancy in CF is unclear. Fecal M2-pyruvate kinase (M2-PK) is a biomarker indicative of cellular proliferation that may be raised in intestinal malignancy and inflammation. Biomarkers, including M2-PK, may be useful in assessing effects of novel therapies on the gastrointestinal tract.

Methods

M2-PK was measured in stools collected from patients with CF and HC (0–10 years). Linear mixed model analysis was used.

Results

M2-PK levels did not significantly change in children with CF (36 patients, 77 samples) (P = 0.998) or HC (45 patients, 45 samples) (P = 0.21), over the age range 0–10 years. Patients with CF had elevated M2-PK compared to HC (median [IQR; range]: 10.7 [5.7–28.6; 1.0–239.1] (n = 77) vs. 1.0 [1.0–1.0; 1.0–50.0] (n = 45) U/mL, respectively; P = 0.001).

Conclusions

Fecal M2-PK was elevated in children with CF compared with HC during infancy and throughout childhood suggesting abnormalities in the CF gut exist in early life.  相似文献   

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