Database : MEDLINE
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[PMID]: 29520463
[Au] Autor:Mercer-Rosa L; Elci OU; Pinto NM; Tanel RE; Goldmuntz E
[Ad] Address:Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, 34th and Civic Center Blvd, Suite 8NW35, Philadelphia, PA, 19104, USA. mercerrosal@email.chop.edu.
[Ti] Title:22q11.2 Deletion Status and Perioperative Outcomes for Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collateral Vessels.
[So] Source:Pediatr Cardiol;, 2018 Mar 08.
[Is] ISSN:1432-1971
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Deletion of 22q11.2 (del22q11) is associated with adverse outcomes in patients with tetralogy of Fallot (TOF). We sought to investigate its contribution to perioperative outcome in patients with a severe form of TOF characterized by pulmonary atresia (PA) or severe pulmonary stenosis (PS) and major aortopulmonary collateral arteries (MAPCAS). We conducted a retrospective review of patients with TOF/MAPCAS who underwent staged surgical reconstruction between 1995 and 2006. Groups were compared according to 22q11.2 deletion status using t-tests or the Wilcoxon Rank sum test. We included 26 subjects, 24 of whom survived the initial operation. Of those, 21 subjects had known deletion status and constitute the group for this analysis [15 with no deletion present (ND) and 6 del22q11 subjects]. There was no difference with respect to occurrence of palliative procedure prior to initial operation, or to timing of closure of the ventricular septal defect (VSD). Other than higher prevalence of prematurity (50%) in the del22q11 group versus no prematurity in the ND, the groups were comparable in terms of pre-operative characteristics. The intra- and post-operative course outcomes (length of cardiopulmonary bypass, use of vasopressors, duration of intensive care and length of hospital stay, tube-feeding) were also comparable. Although the del22q11 had longer mechanical ventilation than the ND, this difference was not significant [68 h (range 4-251) vs. 45 h (range 3-1005), p = 0.81]. In this detailed comparison of a small patient cohort, 22q11.2 deletion syndrome was not associated with adverse perioperative outcomes in patients with TOF, PA, and MAPCAS when compared to those without 22q11.2 deletion syndrome. These results are relevant to prenatal and neonatal pre-operative counseling and planning.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1007/s00246-018-1840-9

  2 / 20793 MEDLINE  
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[PMID]: 29417607
[Au] Autor:Wang Q; Wu YR; Jiao XT; Wu PF; Zhao LQ; Chen S; Sun K
[Ad] Address:Department of Pediatric Cardiology, Xinhua Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
[Ti] Title:Fetal pulmonary valve stenosis or atresia with intact ventricular septum: Predictors of need for neonatal intervention.
[So] Source:Prenat Diagn;, 2018 Feb 07.
[Is] ISSN:1097-0223
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:OBJECTIVES: To compare the fetal echocardiographic measurements and neonatal outcome of fetuses with diagnosis of critical pulmonary stenosis (CPS/IVS) and pulmonary atresia with intact ventricular septum (PA/IVS) to identify the predictors of neonatal ductus dependence and the need for neonatal intervention. METHODS: Forty-four fetuses with a diagnosis of membranous PA/IVS or CPS/IVS referred to Shanghai Xinhua Hospital Affiliated to Shanghai Jiaotong University between June 2009 and November 2014 were respectively analyzed. We analyzed their fetal and pediatric echocardiographic features and clinical features after birth. RESULTS: Among the 29 infants in the final analysis, 19 were ductal dependent and were diagnosed as CPS/IVS or PA/IVS after birth. Ten patients with fetal CSP/IVS were ductal independent and were diagnosed as pulmonary stenosis after birth. In midtrimester, significance was only detected in pulmonary valve (PV) regurgitation between 2 groups (P = .009). The fetuses with PV regurgitation in midtrimester were more likely to be ductal independent (odds ratio = 6.67; P = .010) than those with absence of PV regurgitation in the midtrimester scan. In late trimester, the infants in ductus-independent group had better fetal right ventricular development than those in ductus dependent group. The right ventricle over left ventricle length ratio in last scan over 0.86 had the best capability in predicting ductus independence during neonatal period with help of receiver-operating characteristic curve and the logistic regression analysis. CONCLUSION: Among fetuses with pulmonary valve stenosis and right ventricle dysplasia at the time of first fetal echo in midtrimester, use of pulmonary valve regurgitation can identify who will be ductal dependent at births. In late trimester, right ventricle over left ventricle length ratio less than 0.86 had the best capability of predicting neonatal ductal dependence. Fetuses with reversed ductus arteriosus flow direction in late trimester were more likely to be ductus dependent after birth.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.1002/pd.5224

  3 / 20793 MEDLINE  
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[PMID]: 29516127
[Au] Autor:Peterson RE; Freire G; Marino CJ; Jureidini SB
[Ad] Address:Division of Pediatric Cardiology, Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children's Hospital, 1465 S. Grand Blvd, Saint Louis, MO, 63104, USA. renuka.peterson@health.slu.edu.
[Ti] Title:Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.
[So] Source:Pediatr Cardiol;, 2018 Mar 08.
[Is] ISSN:1432-1971
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis. Patients were divided into two groups, those with RVDCC (determined by angiography or pathology evaluation) and those without. Echocardiographic evaluation of the coronary arteries included 2-dimensional measurements and pulse wave Doppler flow pattern in 3 epicardial coronary arteries. Velocity-time integral (VTI) and maximal velocity (V ) were measured and compared between the two groups. Three coronary flow patterns were demonstrated: (1) all antegrade flow, (2) antegrade to retrograde VTI flow ratio > 1, and (3) antegrade to retrograde VTI flow ratio ≤ 1. Of the 7 patients with RVDCC, 6 (86%) had evidence of flow pattern 3 in ≥ 2 of the 3 coronary arteries in contrast to 0 (0%) of the non-RVDCC patients (p = 0.001). Higher retrograde V was associated with RVDCC (p < 0.001) and coronary artery dilatation with Z-score ≥ + 3 was also associated with RVDCC (p = 0.02). Echocardiographic evaluation of the coronaries can be useful in identifying RVDCC. More retrograde flow in at least two coronary arteries is strongly suggestive of RVDCC. Dilatation of the coronary arteries is also supportive evidence.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1007/s00246-018-1846-3

  4 / 20793 MEDLINE  
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[PMID]: 29447337
[Au] Autor:Mainwaring RD; Patrick WL; Ma M; Hanley FL
[Ad] Address:Division of Pediatric Cardiac Surgery, Department of Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children's Hospital at Stanford, Stanford, CA, USA.
[Ti] Title:An analysis of patients requiring unifocalization revision following midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.
[So] Source:Eur J Cardiothorac Surg;, 2018 Feb 13.
[Is] ISSN:1873-734X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:OBJECTIVES: Midline unifocalization has been developed for the surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. All patients will eventually require reoperation because of the presence of a conduit, and some patients may also require revision of the distal unifocalized bed. The purpose of this study was to analyse the need for unifocalization revision following midline unifocalization. METHODS: This was a retrospective review of 241 patients who underwent midline unifocalization for the treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Two hundred and four (85.4%) patients had a single-stage complete repair, whereas 37 patients had a unifocalization and placement of a central shunt. Seventy-eight patients have subsequently undergone reoperations at our institution, including 44 operations that required unifocalization revision. These 44 patients were compared with the 191 operative survivors who did not require revision. RESULTS: An analysis of risk factors for requiring unifocalization revision included the following: (i) single-stage complete repair versus unifocalization and shunt (14.7% vs 37.8%, P < 0.001), (ii) right ventricle to aortic pressure ratio at the initial repair (0.33 ± 0.07 vs 0.44 ± 0.08, P < 0.001) and (iii) absence of central pulmonary arteries (32.8% vs 13.4%, P < 0.001). CONCLUSIONS: Data demonstrate that 44 of 241 (18%) patients who underwent midline unifocalization have subsequently required revision of their unifocalization. The need for unifocalization revision was associated with 3 factors, all of which were known at the time of discharge from the initial unifocalization. These data suggest that potentially higher risk patients should be monitored more closely than their lower risk counterparts.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1093/ejcts/ezy017

  5 / 20793 MEDLINE  
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[PMID]: 29444216
[Au] Autor:Elias P; Poh CL; du Plessis K; Zannino D; Rice K; Radford DJ; Bullock A; Wheaton GR; Celermajer DS; d'Udekem Y
[Ad] Address:Department of Cardiac Surgery, The Royal Children's Hospital Melbourne, Melbourne, VIC, Australia.
[Ti] Title:Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death.
[So] Source:Eur J Cardiothorac Surg;, 2018 Feb 12.
[Is] ISSN:1873-734X
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. RESULTS: Late follow-up data were available for 120 patients. The median length of follow-up after the Fontan procedure was 9.1 years (interquartile range 4.2-15.4 years). Late death occurred in 9% of patients (11/120). Six were sudden, unexpected deaths; 4 of those occurred in patients known to have right ventricle-dependent coronary circulation (RVDCC). Those with RVDCC had a higher incidence of sudden death (4/20 vs 2/100; P = 0.007). RVDCC was associated with late death (P = 0.01) and the development of myocardial ischaemia after Fontan completion (P < 0.001). The 10-year survival rate was 77% (95% confidence interval 56-100%) for patients with RVDCC vs 96% (95% confidence interval 92-100%) for patients without RVDCC. CONCLUSIONS: Long-term survival of patients with pulmonary atresia with intact ventricular septum after the Fontan procedure is excellent, but patients with RVDCC remain susceptible to coronary ischaemia and sudden death. Closer surveillance and investigation for exercise-induced ischaemia may be necessary.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.1093/ejcts/ezy038

  6 / 20793 MEDLINE  
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[PMID]: 28453807
[Au] Autor:Polimenakos AC; Subramanian S; ElZein C; Ilbawi MN
[Ad] Address:Division of Pediatric Cardiovascular Surgery, Advocate Children's Hospital, Oak Lawn, IL, USA.
[Ti] Title:Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection.
[So] Source:Interact Cardiovasc Thorac Surg;24(5):747-754, 2017 05 01.
[Is] ISSN:1569-9285
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27.6 ± 12.1 months. RESULTS: The initial procedure was pulmonary artery banding in 9 patients, systemic-to-pulmonary shunt in 2 and Damus-Kaye-Stansel/Norwood procedure in 2. Median follow-up was 69 months (interquartile range 25-75, 21-99). There was 1 death after a Damus-Kaye-Stansel/Norwood procedure and one interstage death after a bidirectional Glenn procedure. Nine (of 11) survivors underwent a Fontan operation. A fenestrated Fontan procedure was the predominate operation in 78%. One patient was deemed unsuitable for a Fontan operation. There was 1 takedown and 1 late death after the Fontan operation. Heterotaxy syndrome did not affect outcome ( P > 0.05). There was no statistical difference in the pre-Fontan McGoon ratio, hospital length of stay, duration of pleural drainage and Fontan-related adverse events between patients with a dominant right ventricle and those with a left ( P > 0.05). CONCLUSIONS: A TCPC in patients with T21 and an FSV is associated with reproducible, satisfactory outcomes. An assisted-Glenn procedure with pulsatile pulmonary blood flow and a fenestrated Fontan may be associated with attenuated perioperative morbidity and late attrition.
[Mh] MeSH terms primary: Abnormalities, Multiple
Down Syndrome/diagnosis
Heart Bypass, Right/methods
Heart Defects, Congenital/surgery
Heart Ventricles/abnormalities
Postoperative Complications/epidemiology
[Mh] MeSH terms secundary: Female
Follow-Up Studies
Heart Defects, Congenital/diagnosis
Heart Ventricles/surgery
Humans
Infant
Infant, Newborn
Male
Retrospective Studies
Survival Rate/trends
Time Factors
Treatment Outcome
United States/epidemiology
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1709
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[Js] Journal subset:IM
[Da] Date of entry for processing:170429
[St] Status:MEDLINE
[do] DOI:10.1093/icvts/ivw413

  7 / 20793 MEDLINE  
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[PMID]: 29465587
[Au] Autor:Shi YP; Li YD; Lv XZ; Yang YH
[Ad] Address:Department of Echocardiography.
[Ti] Title:Systemic-pulmonary arteriovenous fistulae with pulmonary hypertension: A case report.
[So] Source:Medicine (Baltimore);97(8):e9959, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Arteriovenous (AV) fistulae is an extremely rare disease of vascular malformation that involves fistulae formation between the systemic and pulmonary AV systems. CASE REPRESENTATION: This case report describes a rare systemic-pulmonary AV fistulae of congenital origin, accompanied by pulmonary hypertension, as determined by aortic angiography and echocardiography. CONCLUSION: Characteristics, diagnosis, and therapeutic approaches of this rare abnormality are explored.
[Mh] MeSH terms primary: Arteriovenous Fistula/complications
Hypertension, Pulmonary/congenital
Pulmonary Artery/abnormalities
Pulmonary Veins/abnormalities
[Mh] MeSH terms secundary: Adult
Humans
Male
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180302
[Lr] Last revision date:180302
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180222
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009959

  8 / 20793 MEDLINE  
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[PMID]: 29289271
[Au] Autor:Wu T; Yu Y; Zhang Y; Deng Z
[Ad] Address:Department of Respiratory Medicine, Affiliated Hospital of Ningbo University Medical College, Ningbo, China.
[Ti] Title:Congenital Descending Aorta-Pulmonary Vein Fistula.
[So] Source:Am J Med Sci;355(1):97-98, 2018 01.
[Is] ISSN:1538-2990
[Cp] Country of publication:United States
[La] Language:eng
[Mh] MeSH terms primary: Aorta, Thoracic/abnormalities
Aorta, Thoracic/diagnostic imaging
Arteriovenous Fistula/diagnostic imaging
Pulmonary Veins/abnormalities
Pulmonary Veins/diagnostic imaging
[Mh] MeSH terms secundary: Aorta, Thoracic/surgery
Arteriovenous Fistula/surgery
Humans
Male
Pulmonary Veins/surgery
Young Adult
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Em] Entry month:1803
[Cu] Class update date: 180301
[Lr] Last revision date:180301
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180101
[St] Status:MEDLINE

  9 / 20793 MEDLINE  
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[PMID]: 28744668
[Au] Autor:Sano A; Yotsumoto T
[Ad] Address:Department of Thoracic Surgery, Chigasaki Municipal Hospital, Honson 5-15-1, Chigasaki, Kanagawa, 253-0042, Japan. sanoa-tky@umin.ac.jp.
[Ti] Title:Single-port thoracoscopic lung wedge resection using the Endo GIA Radial Reload.
[So] Source:Surg Today;48(2):248-251, 2018 Feb.
[Is] ISSN:1436-2813
[Cp] Country of publication:Japan
[La] Language:eng
[Ab] Abstract:The GIA Radial Reload is a surgical stapler with a curved cut line that is perpendicular to the direction of instrument insertion. We used the GIA Radial Reload in three cases of single-port thoracoscopic lung wedge resection. The operations were performed through a 3.0-4.5-cm incision. For the first stapler, we selected the GIA Radial Reload. The orientation of this device's cut line enabled us to easily cut the lung behind the lesion during single-port thoracoscopic surgery.
[Mh] MeSH terms primary: Pneumonectomy/instrumentation
Surgical Staplers
Thoracoscopy/instrumentation
[Mh] MeSH terms secundary: Aged
Arteriovenous Fistula/surgery
Female
Humans
Leiomyosarcoma/secondary
Leiomyosarcoma/surgery
Lung Neoplasms/secondary
Lung Neoplasms/surgery
Middle Aged
Pneumonectomy/methods
Pulmonary Artery/abnormalities
Pulmonary Veins/abnormalities
Thoracoscopy/methods
Uterine Neoplasms/pathology
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180228
[Lr] Last revision date:180228
[Js] Journal subset:IM
[Da] Date of entry for processing:170727
[St] Status:MEDLINE
[do] DOI:10.1007/s00595-017-1572-0

  10 / 20793 MEDLINE  
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[PMID]: 29298459
[Au] Autor:Hsu CC; Kwan GN; Evans-Barns H; van Driel ML
[Ad] Address:Department of Medical Imaging, University of Toronto, 4th Floor, 263 McCaul Street, Toronto, Ontario, Canada, M5T 1W7.
[Ti] Title:Embolisation for pulmonary arteriovenous malformation.
[So] Source:Cochrane Database Syst Rev;1:CD008017, 2018 01 04.
[Is] ISSN:1469-493X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Pulmonary arteriovenous malformations are abnormal direct connections between the pulmonary artery and pulmonary vein which result in a right-to-left shunt. They are associated with substantial morbidity and mortality mainly from the effects of paradoxical emboli. Potential complications include stroke, cerebral abscess, pulmonary haemorrhage and hypoxaemia. Embolisation is an endovascular intervention based on the occlusion of the feeding arteries the pulmonary arteriovenous malformations thus eliminating the abnormal right-to-left-shunting. This is an update of a previously published review. OBJECTIVES: To determine the efficacy and safety of embolisation in patients with pulmonary arteriovenous malformations including a comparison with surgical resection and different embolisation devices. SEARCH METHODS: We searched the Cystic Fibrosis and Genetic Disorders Group's Trials Register; date of last search: 10 April 2017.We also searched the following databases: the Australian New Zealand Clinical Trials Registry; ClinicalTrials.gov; International Standard Randomised Controlled Trial Number Register; International Clinical Trials Registry Platform Search Portal (last searched 27 August 2017). to be updatedWe checked cross-references and searched references from review articles. SELECTION CRITERIA: Trials in which individuals with pulmonary arteriovenous malformations were randomly allocated to embolisation compared to no treatment, surgical resection or embolisation using a different embolisation device. DATA COLLECTION AND ANALYSIS: Studies identified for potential inclusion were independently assessed for eligibility by two authors, with excluded studies further checked by a third author. No trials were identified for inclusion in the review and hence no analysis was performed. MAIN RESULTS: There were no randomised controlled trials included in the review; one ongoing trial has been identified which may be eligible for inclusion in the future. AUTHORS' CONCLUSIONS: There is no evidence from randomised controlled trials for embolisation of pulmonary arteriovenous malformations. However, randomised controlled trials are not always feasible on ethical grounds. Accumulated data from observational studies suggest that embolisation is a safe procedure which reduces morbidity and mortality. A standardised approach to reporting with long-term follow-up through registry studies can help to strengthen the evidence for embolisation in the absence of randomised controlled trials.
[Mh] MeSH terms primary: Arteriovenous Malformations/therapy
Embolization, Therapeutic/methods
Pulmonary Artery/abnormalities
Pulmonary Veins/abnormalities
[Mh] MeSH terms secundary: Embolization, Therapeutic/adverse effects
Humans
[Pt] Publication type:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T; REVIEW
[Em] Entry month:1802
[Cu] Class update date: 180226
[Lr] Last revision date:180226
[Js] Journal subset:IM
[Da] Date of entry for processing:180104
[St] Status:MEDLINE
[do] DOI:10.1002/14651858.CD008017.pub5


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