bluuebunny/crossref_metadata_2025_split huggingface dataset:
Haemostatic, Endothelial and Lipoprotein Parameters and Blood Pressure Levels in Women with a History of Preeclampsia
Authors: He Shu , Silveira Angela , Hamsten Anders , Blombäck Margareta , Bremme Katarina 
Published: June 2019

SummaryTo determine whether perturbations of haemostatic function and lipoprotein metabolism prevail long after preeclampsia and increase the risk of future coronary heart disease (CHD), we conducted a follow-up study in women with (cases, n = 25) or without (controls, n = 24) a history of preeclampsia. Blood samples were taken in the follicular and in the luteal phases of a menstrual cycle. Levels of blood pressure (BP) and proteinuria measured during the index pregnancy were included in the evaluation. Compared to control women who had undergone a normal pregnancy, the formerly preeclamptic patients had higher systolic (p <0.01) and diastolic (p <0.05) BPs and increased plasma levels of von Willebrand factor (vWF), fibrinogen, cholesterol, triglycerides and very low density lipoprotein (VLDL) (all p <0.05). The lipid, vWF, and fibrinogen levels were positively related to the degree of BP elevation but not to the degree of proteinuria during the index pregnancy. Except for the increase in vWF level, all biochemical perturbations were only present in the luteal but not in the follicular phase samples. In conclusion, persistent endothelial dysfunction with ensuing dysregulation of blood pressure, haemostatic perturbation and dyslipoproteinemia after preeclampsia may indicate a proneness to future CHD.
DNA-Polymorphisms and Plasma Levels of Vascular Disease Risk Factors in Greenland Inuit
Authors: de Maat Moniek P. M. , Bladbjerg Else Marie , Johansen Lars G. , de Knijff Peter , Gram Jørgen , Kluft Cornelis , Jespersen Jørgen 
Published: June 2019

SummaryGreenland Inuit are a population with a low risk of cardiovascular disease. Recently, we stated that frequencies of potentially high risk alleles of the apolipoproteins, fibrinogen, factor V, glycoprotein IIIa and factor VII (FVII) genes have different allele frequencies in the Inuit when compared with Caucasian populations. We have extended this study and evaluated whether or not this was also true for the genetic polymorphisms of tissue-type plasminogen activator (t-PA), plasminogen activator inhibitor-1 (PAI-1), angiotensin-converting enzyme (ACE) and angiotensinogen in a group of 133 Greenland Inuit, aged 30-34 y. In addition, we compared the plasma levels of these factors and those of C-reactive protein (CRP) and D-Dimer in Inuit and in Danes, comparable for age and gender. Frequencies (f) were assessed of the alleles that are known as the potential high risk alleles in Caucasians.In the Inuit, the f(insertion allele) of the t-PA intron8ins311 polymorphism was 0.37 (CI 0.32-0.43), the f(4G allele) of the PAI-1 promoter polymorphism was 0.88 (CI 0.83-0.91), the f(deletion allele) of the ACE intron16ins287 polymorphism was 0.40 (CI 0.33-0.47) and the f(M-allele) of the angiotensinogen M/T353 polymorphism was 0.30 (CI 0.25-0.38). As for fibrinogen and FVII polymorphisms, these frequencies are all significantly different from what is reported for Caucasian populations. In the Inuit, plasma levels of fibrinogen and D-Dimer were higher than in the Danes, the PAI-1 levels were lower and FVII, t-PA and CRP levels were comparable. The observed allele frequencies of the polymorphisms of t-PA, fibrinogen, FVII, ACE, angiotensinogen and the plasma levels of PAI-1 and D-Dimer were in accordance with the low CVD risk in the Inuit, considering the observed associations between these measures and CVD risk in Caucasian populations, but for other measures this was not the case (allele frequencies of the PAI-1 polymorphism, and plasma levels of fibrinogen, FVII and t-PA).In conclusion there are clear differences in genetic background and plasma levels of risk factors in Greenland Inuit compared with Caucasian populations, and these differences were sometimes, but not always, in accordance with the observed low cardiovascular disease risk of the Inuit population.
Born Again: Recurrent Hepatic Encephalopathy in an Adult without Cirrhosis
Authors: George Tom , Rajesh Sasidharan , Philips Cyriac Abby 
Published: June 2019

AbstractCongenital extrahepatic portosystemic shunt (CEPS) develops between the por-to-mesenteric and systemic veins. The splanchnic venous system drains directly to systemic circulation in this condition. We present a case of an adult onset Type II CEPS presenting with hepatic encephalopathy. The patient underwent PARTO (plug-assisted retrograde transvenous obliteration) and had a significant clinical improvement on subsequent follow-up. Our case highlights the importance of imaging and the role of an interventional radiologist in the management of the condition.
High fusion performance in Super H-mode experiments on Alcator C-Mod and DIII-D
Authors: Snyder P.B. , Hughes J.W. , Osborne T.H. , Paz-Soldan C. , Solomon W.M. , Knolker M. , Eldon D. , Evans T. , Golfinopoulos T. , Grierson B.A. , Groebner R.J. , Hubbard A.E. , Kolemen E. , LaBombard B. , Laggner F.M. , Meneghini O. , Mordijck S. , Petrie T. , Scott S. , Wang H.Q. , Wilson H.R. , Zhu Y.B. 
Published: June 2019

Abstract
The ‘Super H-Mode’ regime is predicted to enable pedestal height and fusion performance substantially higher than standard H-Mode operation. This regime exists due to a bifurcation of the pedestal pressure, as a function of density, that is predicted by the EPED model to occur in strongly shaped plasmas above a critical pedestal density. Experiments on Alcator C-Mod and DIII-D have achieved access to the Super H-Mode (and Near Super H) regime, and obtained very high pedestal pressure, including the highest achieved on a tokamak (p
ped ~ 80 kPa) in C-Mod experiments operating near the ITER magnetic field. DIII-D Super H experiments have demonstrated strong performance, including the highest stored energy in the present configuration of DIII-D (W ~ 2.2–3.2 MJ), while utilizing only about half of the available heating power (P
heat ~ 7–12 MW). These DIII-D experiments have obtained the highest value of peak fusion gain, Q
DT,equiv ~ 0.5, achieved on a medium scale (R  <  2 m) tokamak. Sustained high performance operation (β
N ~ 2.9, H98 ~ 1.6) has been achieved utilizing n  =  3 magnetic perturbations for density and impurity control. Pedestal and global confinement has been maintained in the presence of deuterium and nitrogen gas puffing, which enables a more radiative divertor condition. A pair of simple performance metrics is developed to assess and compare regimes. Super H-Mode access is predicted for ITER and expected, based on both theoretical prediction and observed normalized performance, to allow ITER to achieve its goals (Q  =  10) at I
p  <  15 MA, and to potentially enable more compact, cost effective pilot plant and reactor designs.
DOZ047.03: The functional chewing training for chewing dysfunction in children with repaired esophageal atresia–tracheoesophageal fistula
Authors: Arslan S S , Demir N , Karaduman A , Tanyel F C , Soyer T 
Published: June 2019

Abstract

Introduction
Chewing disorders (CD) may cause restrictions in solid food intake and can be seen in 37% of children with esophageal atresia–tracheoesophageal fistula (EA-TEF).1 The Functional Chewing Training (FuCT) is a holistic approach to improve chewing function (CF) in children. The study aimed to evaluate the effects of FuCT on CF in children with EA-TEF.

Materials and Methods
Twenty children with CD were included. Patients received 12 weeks of FuCT, including impairment-based and adaptive components. Chewing performance level was scored with the Karaduman Chewing Performance Scale (KCPS), and tolerated food texture was determined by the International Dysphagia Diet Standardization Initiative (IDDSI). The baseline and final levels of KCPS and IDDSI were compared to evaluate the effects of FuCT on CF.

Results
45% of cases were isolated-EA and 55% were EA-distal TEF with a median age of 31 (min = 25, max = 84) months, of which 65% (n = 13) were male. Baseline evaluation showed that 12 cases were in level-1, 6 cases in level-3 and 2 cases in level-4 according to the KCPS. Eight children with CD (40%) had IDDSI level-3 and 12 (60%) had level-7. There was a significant improvement in KCPS scores and IDDSI scores after 12 weeks of training (P < 0.01, P = 0.005, respectively). KCPS scores showed level-0 in 15 cases, and level-1 in 5 cases. All children had IDSSI level-7.

Conclusions
The FuCT is an effective method to improve chewing function in children EA-TEF who had CD.
DOZ047.10: Acquired tracheoesophageal fistulas after esophageal atresia repair
Authors: Boybeyi-Turer O , Soyer T , Tanyel F C 
Published: June 2019

Abstract

Aim
Tracheoesophageal fistula (TEF) recurrence is a frequent but challenging complication after esophageal atresia (EA) repair. Although most recurrent TEFs are commonly seen at the original fistula site, long new fistulas localized differently from the congenital TEF sites are called acquired TEFs (acq-TEF). Acq-TEFs are long new fistulas with unusual locations, including fistulas from the esophagus to anywhere on the airway such as the bronchus, trachea, or lung parenchyma. Herein, we aimed to discuss diagnostic and management challenges in different localizations of acquired TEF.

Methods
The medical records of patients admitted with acq-TEF in the last 5 years were retrospectively evaluated. The demographic features, admission complaints, physical and radiological findings, TEF localization and management were recorded.

Results
From 16 TEF recurrences, 4 TEFs were acquired fistulas. Admission age ranged from 3 months to 8 years. The female/male ratio was 2/2. The complaints were recurrent respiratory tract infections, choking, and coughing in all cases. Three of the cases had proximal EA + distal TEF; the other case was isolated EA. Primary repair was performed in 3 cases and colon interposition was performed in 1 case. Anastomotic leak and mediastinitis after initial operation were seen in 3 cases. Three acq-TEFs were to the cervical part of the trachea, one was from the colon conduit to the trachea, one was to right bronchus by passing through the intrathoracic abscess cavity, and one was directly to the right bronchus. In all cases the TEFs were shown in sine-esophagography and confirmed with bronchoscopy during operation. TEF was repaired by thoracotomy incision in 3 cases and callor incision in 1 case. The second acq-TEF of Case 1 was closed spontaneously. Muscle flap or pleura was placed between suture lines in all cases. All TEFs were confirmed to be closed with esophagography in all cases at postoperative period.

Conclusion
Acq-TEF is mostly seen secondary to local or diffuse mediastinitis. Besides its classical location of TEF, acq-TEFs may be seen at unusual rare localizations such as esophagus to right bronchus, esophagus to abscess cavity, and conduit to trachea. They cause both diagnostic and surgical challenge. Clinicians should be aware of these different localizations of Acq-TEFs in order to evaluate and manage these patients more comprehensively.
DOZ047.113: Outcome of routine esophagogastroduodenoscopy, high-resolution manometry (HRM), and pH-(impedance) measurement in esophageal atresia patients according to international guidelines
Authors: van Lennep M , Krishnan U , van Wijk M P 
Published: June 2019

Abstract

Background
Evidence-based guideline recommendations for follow-up of patients with esophageal atresia (EA) include regular surveillance esophagogastroduodenoscopy (EGD) with biopsies and a pH(-impedance) measurement (pH-MII) to evaluate gastroesophageal reflux disease (GERD) in asymptomatic patients. In addition, high-resolution manometry (HRM) is considered useful to assess esophageal dysmotility. Additional testing is recommended when symptoms are present. Our aim was to evaluate the diagnostic and clinical yield of these tests.

Methods
All EA patients who underwent EGD, pH-MII, and HRM according to EA guideline recommendations in 2017 and 2018 were included.

Results
Seventy-eight patients (53% male, 78% type C, median age 5 (0–17) years) were included. Thirty-one patients had EGD (n = 30), pH-MII (n = 11), and/or HRM (n = 7) as part of a routine surveillance program. Forty-seven patients had EGD (n = 47), pH-MII (n = 26), and HRM (n = 9) for symptom evaluation. Symptoms at the time of testing included dysphagia (51%), regurgitation (41%), and heartburn (24%). Results and consequences of the surveillance and clinically indicated tests are shown in Tables 1 and 2, respectively. Macroscopic EGD findings included stricture (21%), signs of eosinophilic esophagitis (EoE, 3%), and signs of reflux esophagitis (3%). Histological findings included EoE (13%) and reflux esophagitis (17%). Clinical implications of EGD results included medication alteration (GERD/EoE), esophageal dilation, antireflux surgery, or diet change. MII-pH was performed in 37 patients; 15 (41%) were on acid suppressive medication during measurement. Clinical implications of MII-pH results were: start or adjustment of acid suppressive therapy (30%), start or increase of other medication (22%), ceasing of acid suppressive therapy (14%), and referral for fundoplication (3%). HRM was performed in 16 patients (21%, n = 2 uninterpretable due to continuous crying) and showed absent peristalsis in 8/14 (50%), ineffective peristalsis in 4/14 (25%), and fragmented peristalsis in 2/14 patients (12.5%). Clinical implications of manometry results included referral for contrast esophagram (n = 1), increased gastrostomy feeding (n = 1), and gastrostomy placement (n = 1).

Conclusion
EGD, MII-pH, and HRM reveal multiple abnormalities that have clinical consequences when performed as per recommended surveillance guidelines, which highlights their importance. In most symptomatic patients, additional testing demonstrates underlying pathology. Abnormal diagnostic tests can help guide clinical management in asymptomatic and symptomatic EA patients.
DOZ047.119: Five year experience of a tertiary-level referral center in children with anastomotic strictures after esophageal atresia repair
Authors: Contini A C I , Rea F , Guerra L , Caldaro T , Torroni F , Tambucci R , Angelino G , De Angelis P , Balassone V , Romeo E , Faraci S , Federici di Abriola G , Dall’Oglio L 
Published: June 2019

Abstract

Background
Esophageal atresia (EA) is the most common congenital anomaly of the esophagus. Anastomotic strictures (AS) frequently occur in patients surgically treated for esophageal atresia (EA). The primary aim of this study is to determine the role of esophageal dilations in the management of AS in childhood after reconstruction of EA.

Methods
A retrospective chart review of patients treated with esophageal dilation for EA was conducted at our tertiary referral center from 2013 to 2017. We included patients treated at our Institution since diagnosis and patients referred from other Institutions. All dilations were performed with Savary-Gilliard polyvinyl bougies. Dilation was performed on an ‘as needed’ basis.

Results
Eighty-nine patients (68.5% males, 31.5% female) underwent 433 dilations overall in the study period (median: 3, range: 1–36). Type I of EA was 26.1%, Type II was 9.1%, Type III was 64.8%. Associated malformations in 52.8% cases. Six patients received 33 local applications of mitomycin C (MMC) and two of triamcinolone acetate for recurrent stenosis. Four patients developed a preanastomotic diverticulum-treated endoscopically. Nine patients (10.1%) underwent dynamic esophageal stent placement after a median of six dilations.
Antireflux surgery was performed in 28 patients (31.5%), Nissen fundoplication in 20 patients, Toupet in 6, and both in 2. Patients treated with antireflux surgery received a mean of 7.1 dilations before surgery, versus 3.9 in patients without antireflux surgery (P = 0.0285, unpaired t test).
Seven patients (7.9%), 5 referral, needed major esophageal surgery (4 esophageal reanastomosis and 3 esophagocolonplasty). Patients underwent major surgery received a mean of 19.3 dilations versus 3.6 dilations in no-surgery patients (P = 0.0002, Mann-Whitney U test). Six of 9 stented patients did not require surgery.
Perforation was present in 0.4% of 433 dilatations.

Conclusions
Esophageal dilatation remains the mainstay of treatment for AS after EA repair. Stricture resection with end-to-end anastomosis is the reconstructive option of choice; esophageal substitution is reserved only in cases of intractable stricture. The use of esophageal dynamic stent, MMC, or steroids could help in avoiding or delaying the need of operative stricture resection. Antireflux surgery is mandatory in case of severe pathological gastroesophageal reflux to prevent intractable strictures from developing.
DOZ047.121: Usefulness of conservative treatment in recurrent tracheoesophageal fistula in children
Authors: Isoldi S , Torroni F , Faraci S , Balassone V , Rea F , Tambucci R , Angelino G , Romeo E F , Guerra L , Caldaro T , Contini A C I , di Abriola G F , De Angelis P , Bottero S , Bagolan P , Trozzi M , Dall’Oglio L 
Published: June 2019

Abstract

Introduction
Recurrent tracheoesophageal fistula (TEF) is a challenging complication in children with repaired esophageal atresia (EA). Thoracotomy, with surgical repair, is the traditional therapeutic approach, however it is often associated with a significant morbidity and technical difficulty. Endoscopic techniques, from the tracheal and/or esophageal side, have recently been proposed as a primary treatment, in order to prevent reoperation especially in previously operated fields. Aim of this retrospective study is to present our experience as Tertiary Care Center and Referral Center for EA, on the endoscopic management of recurrent TEF in children with repaired EA.

Method
This is a retrospective study conducted on children with repaired EA referred at Bambino Gesù Children's Hospital from January 2009 to January 2019. Demographic and clinical details, including type of EA, presence of fistula, concomitant malformations, endoscopic evaluation, number and kind of treatments, performed were collected.

Results
We identified 171 patients with EA, 15 type 1 (9%), 7 type 2 (4%), 140 type 3 (82%), 6 type 4 (3%), 3 type 5 (2%). Among patients with TEF, 11/156 (7%- M:F/6:5) presented with a TEF recurrence. Among the latter, 5 (45.4%) were patients with concomitant malformation (2 VACTER, 2 anorectal malformation, 1 Down syndrome, cardiac abnormalities, and hypothyroidism). All patients with TEF recurrence presented with symptoms, such as chronic cough, vomit, bronchitis, recurrent pneumonia, and failure to thrive. All patients underwent endoscopic evaluation both from digestive and tracheal side. Six (6/11–54.5%) patients underwent surgery, 5 patients (5/11–45.4%) were treated with endoscopic transtracheal fibrin glue injection after brushing that was successful in two patients (2/5–40%), one patient needed a retreatment. These patients maintained successful closure after treatment confirmed at endoscopic follow-up. Remaining three patients needed surgical approach during follow-up.

Conclusion
Endoscopic repair of recurrent TEF has proved safe and effective in the literature as an alternative to a second open thoracotomy/surgical repair. In our series, although in two cases, it represented a valid alternative to surgery. We suggest that it is worthy to try a conservative approach before surgery.
DOZ047.22: FEED-EASY: feeding disorders in children with esophageal atresia study
Authors: Pham A , Dugelay E , Bonnard A , Gelas T , Rousseau V , Thomassin N , Cabon-Boudard I , Michaud L , Guinot A , Rebeuh J , Mandat A L E , Djeddi D , Fouquet V , Bourchany A , Irtan S , Comte A , Bridoux-Henno L , Dupont C , Dimitrov G , Turquet A , Borderon C , Pelatan C , Legault E C , Jung C , Willot S , Gottrand F , Bellaiche M 
Published: June 2019

Abstract

Introduction
With advances in surgical and neonatal care, survival of patients with esophageal atresia (EA) has improved over time. While a number of conditions associated with EA may have an impact on feeding development (delayed primary anastomosis, anastomotic leaks, recurrent tracheoesophageal fistula, anastomotic stricture, gastroesophageal reflux, esophageal dysmotility, etc.) and although children with EA experience a number of oral aversive events in their first year of life, feeding disorders (FD) are poorly described and frequently unrecognized. The primary aim of this study was to describe FD in children born with EA, with a standardized scale. The secondary aim was to describe conditions associated with FD.

Methods
FEED-EASY is a multicentric French study. Parents of children born with EA between 2013 and 2016 in one of the 22 participating centers were asked to participate and received the French version of the standardized and reproductive ‘Montreal Children's Hospital Feeding Scale (MCH-FS)’.

Results
One hundred and forty-five children were included; 61 (42%) had FD according to the MCH-FS. These children were characterized by disinterest in food, oral hypersensitivity, difficulty in touching some textures and food avoidance, with an influence in quality of life. Nineteen (13%) were tube-fed between 1 and 4 years of age. Birth weight and chronic respiratory difficulties were associated with FD in children with EA. Anastomotic stricture (present in 31% of the included children) was not associated with FD.

Conclusions
FD is frequent and unrecognized in children with EA, and can influence growth and quality of life. MCH-FS allows pediatricians to identify FD in children with EA within a couple of minutes.
DOZ047.23: How esophageal atresia affects the family life
Authors: Rozensztrauch A , Patkowski D , Śmigiel R 
Published: June 2019

Abstract

Background
Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is seen in about 35,000 births. The impact of caring for children after surgical repair of EA is poorly understood and the literature regarding this area is scarce. Mortalities are now mostly attributable to any associated anomalies or prematurity, but morbidity and quality of life (QoL) are important to address.

Objective
This study aimed to investigate whether syndromic EA, feeding problems, and prematurity have a significant influence on the family of a child after surgical repair of EA.

Methods
Ethical approval was obtained. This cross-sectional study consisted of 73 participants (mean mothers’ age = 28, SD = 5 years; mean fathers’ age = 31, SD = 6 years) who were parents of children after surgery of EA. The impact of EA on families was assessed using an author-designed questionnaire (ADQ) to collect medical and sociodemographic background data as well as a standardized questionnaire, the PedsQLTM Family Impact Scale (PedsQL-FIM).

Results
Cardiac malformations associated with EA significantly (P = 0.037) affect the functioning of the family in the emotional domain. Skeletal malformations have the greatest impact on the family functioning. Three statistically significant correlations have been demonstrated: (P = 0.021) in the social domain; (P = 0.009) in the cognitive domain; and (P = 0.023) in the domain of communication. The families of patients with TEF had the statistically lower (P < 0.05) score of functioning in the emotional domain than those without TEF. Nonhealth factors frequently included changes in daily activities and deterioration of social position affected caregivers.

Conclusions
Difficulties with feeding and the presence of associated anomalies significantly affect the functioning of the family of the child with EA.