Publications by authors named "Ofer Schiller"

26 Publications

  • Page 1 of 1

Fluid Responsiveness Predictability in Immediate Postoperative Pediatric Cardiac Surgery. Is the Old Slandered Central Venous Pressure Back Again?

Shock 2021 Apr 20. Epub 2021 Apr 20.

Pediatric Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, 4920235 Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel, 6997801.

Objective: Acute low cardiac output (CO) is a frequent scenario in pediatric cardiac intensive care units (PCICU). While fluid responsiveness has been studied extensively, literature is scarce for the immediate postoperative congenital heart surgery population admitted to PCICUs. This study analyzed the utility of hemodynamic, bedside ultrasound and Doppler parameters for prediction of fluid responsiveness in infants and neonates in the immediate postoperative cardiac surgery period.

Design: A prospective observational study.

Setting: University affiliated, tertiary care hospital, PCICU.

Participants: Immediate postoperative pediatric patients displaying a presumed hypovolemic low CO state were included. A clinical, arterial derived, hemodynamic, sonographic, Doppler-based, and echocardiographic parameter assessment was performed, followed by a fluid bolus therapy.

Interventions: 15-20cc/kg Crystalloid fluid bolus.

Main Outcome Measures: Fluid responsiveness was defined as an increase in cardiac index > 10% by echocardiography.

Results: Of 52 patients, 34 (65%) were fluid responsive. Arterial systolic pressure variation (SPV), continuous-Doppler preload parameters, and inferior vena-cava distensibility index (IVCDI) by bedside ultrasound all failed to predict fluid responsiveness. Dynamic central venous pressure (CVP) change yielded a significant but modest fluid responsiveness predictability of AUC 0.654 (p = 0.0375).

Conclusions: In a distinct population of mechanically ventilated, young, pediatric cardiac patients in the immediate postoperative period, SPV, USCOM preload parameters, as well as IVC-based parameters by bedside ultrasound failed to predict fluid responsiveness. Dynamic CVP change over several hours was the only parameter that yielded significant but modest fluid responsiveness predictability.
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http://dx.doi.org/10.1097/SHK.0000000000001786DOI Listing
April 2021

Extracorporeal Membrane Oxygenation as a Rescue Therapy for Postoperative Diastolic Dysfunction and Refractory Chylothorax.

ASAIO J 2021 May;67(5):e99-e101

Pediatric Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

This is the first published case, as far as we know, of a term neonate with refractory chylothorax secondary to diastolic dysfunction in the cardiac postoperative period, where extracorporeal membrane oxygenation (ECMO) was used to improve the physiologic derangements, thus allowing resolution of the chylous effusion. The infant was prenatally diagnosed with d-transposition of the great arteries. He was started on prostaglandin infusion and underwent balloon atrial septostomy followed by arterial switch operation. After surgery, he developed anasarca and high-volume chylothorax that did not respond to medical management and fasting. Cardiac catheterization demonstrated severe diastolic dysfunction and pulmonary hypertension. On postoperative day 19, he was placed on veno-arterial (VA) ECMO and had gradual regression of the chylothorax and edema. After 13 days on ECMO support, he was decannulated with small, self-limiting, reaccumulation of chylous effusion. He was discharged home on postoperative day 57, and has since been thriving with no evidence of reaccumulation of the chylous effusion. In summary, VA ECMO support could be considered as a rescue modality for patients with uncontrollable refractory high-volume chylous effusion, after other treatment options have been pursued.
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http://dx.doi.org/10.1097/MAT.0000000000001279DOI Listing
May 2021

Transposition of the Great Arteries-Are We Doing Better? Correlating Outcome to Change in Renal Function Over 2 Decades of Arterial Switch Operation.

Pediatr Crit Care Med 2020 09;21(9):e782-e788

Pediatric Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.

Objectives: It is believed that management of neonates with dextro-transposition of the great arteries is constantly improving. Renal function may play a role in the prognosis of patients after congenital heart surgery. The aim of this study was to describe the outcome of neonates who underwent arterial switch operation during the past 2 decades using renal function as a surrogate marker for morbidity and mortality.

Design: Retrospective cohort study.

Setting: Dedicated cardiac ICU of a university-affiliated pediatric medical center.

Patients: Infants who underwent arterial switch surgery in 1993-2015.

Interventions: None.

Measurements And Main Results: The cohort included 336 infants who underwent arterial switch operation for dextro-transposition of the great arteries (n = 169, 50%), transposition of the great arteries/ventricular septal defect (n = 133, 40%), or Taussig-Bing anomaly (n = 34, 10%). Between 1993-1998 and 2012-2015, the mean minimal postoperative estimated glomerular filtration rate rose from 30 mL/min/1.73 m to 40 mL/min/1.73 m (p < 0.05), and the proportion of patients with estimated glomerular filtration rate less than 30 mL/min/1.73 m decreased from 56% to 23% (p < 0.05). The daily furosemide dosage decreased from 4 mg/kg/d to 0.5 mg/kg/d (p < 0.05). Urinary output on operative day 0 decreased over time, but urinary output on operative day 2 significantly increased. Maximal lactate levels and time to lactate normalization decreased steadily. Dialysis was performed in only a few patients in the early periods, and in none in the last 6 years. The mean mortality rate of patients with dextro-transposition of the great arteries and transposition of the great arteries/ventricular septal defect decreased to 2.7% in the last 6 years. The odds ratio of a prolonged hospital stay (≥ 28 d) in a patient with estimated glomerular filtration rate less than 30 mL/min/1.73 m was 18.79, and in a patient with transposition of the great arteries/ventricular septal defect, 3.39. The odds ratio of dying after Rashkind atrial septostomy was 4.42.

Conclusions: During the past 2 decades, there has been significant improvement in outcome of patients undergoing transposition of the great arteries repair. Renal function was found to be a good prognostic marker of morbidity and mortality.
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http://dx.doi.org/10.1097/PCC.0000000000002387DOI Listing
September 2020

Variability of Care of Infants With Severe Respiratory Syncytial Virus Bronchiolitis: A Multicenter Study.

Pediatr Infect Dis J 2020 09;39(9):808-813

The Pediatric Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petah Tikva, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.

Background: Viral bronchiolitis caused by respiratory syncytial virus (RSV) is a common childhood disease accounting for many hospitalizations worldwide. Some infants may clinically deteriorate, requiring admission to an intensive care unit. We aimed to describe diagnostic and therapeutic measures of bronchiolitis in Israeli pediatric intensive care units (PICUs) and evaluate intercenter variability of care.

Methods: Medical records of all RSV-infected infants admitted to 5 Israeli PICUs over 4 RSV seasons were retrospectively reviewed.

Results: Data on 276 infants with RSV-positive bronchiolitis, admitted to the participating PICUs were analyzed. Most of the infants were males with a mean admission age of 4.7 months. Approximately half of the infants had pre-existing conditions such as prematurity, cardiac disease or chronic lung disease. Respiratory distress was the most common symptom at presentation followed by hypoxemia and fever. There was significant variation in the methods used for RSV diagnosis, medical management and respiratory support of the infants. Furthermore, utilization of inhalational therapy and transfusion of blood products differed significantly between the centers. Although a bacterial pathogen was isolated in only 13.4% of the infants, 82.6% of the cohort was treated with antibiotics.

Conclusions: Significant variation was found between the different PICUs regarding RSV bronchiolitis diagnosis, medical management and respiratory support, which may not be accounted for by the differences in baseline and clinical characteristics of the infants. Some of these differences may be explained by uneven resource allocations. This diversity and the documented routine use of medications with weak evidence of efficacy calls for national guidelines for bronchiolitis management.
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http://dx.doi.org/10.1097/INF.0000000000002707DOI Listing
September 2020

The Utility of Albumin Level as a Marker of Postoperative Course in Infants Undergoing Repair of Congenital Heart Disease.

Pediatr Cardiol 2020 Jun 14;41(5):939-946. Epub 2020 Mar 14.

Pediatric Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 4920235, Petach Tikva, Israel.

We sought to examine the role of preoperative and 2nd postoperative day albumin levels as predictors for postoperative course in infants undergoing repair of congenital heart disease. This retrospective, single-center, observational study comprised consecutive infants younger than 1 year who had undergone repair of tetralogy of Fallot, ventricular septal defect, complete atrioventricular canal or transposition of the great arteries over a 25 months period. We correlated preoperative and postoperative day (POD) #2 albumin level to vaso-inotropic score (VIS) and intensive care unit (ICU) length of stay (LOS) as markers for degree and duration of postoperative cardiac support. A composite outcome was defined as maximal vaso-inotropic score of > 10 and ICU LOS > 96 h. Preoperative albumin level negatively correlated with VIS and ICU LOS. Compared to preoperative albumin level of > 4 g/dL, the relative risk of meeting composite criteria was 1.5 for preoperative albumin of 3.1-4 g/dL and 2.6 for preoperative albumin ≤ 3 g/dL. Compared to POD#2 albumin level > 3 g/dL, the relative risk of meeting composite criteria was 1.8 for albumin of 2.6-3 g/dL, and 2.5 for albumin ≤ 2.5 g/dL. In summary, we found that preoperative and POD#2 albumin levels predicted prolonged and complicated postoperative course. These finding may help clinicians to inform the patient's parents, early in the ICU hospitalization, as to the predicted risks and difficulties of their infant's postoperative course.
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http://dx.doi.org/10.1007/s00246-020-02339-6DOI Listing
June 2020

Pediatric Extracorporeal Membrane Oxygenation Reach-Out Program: Successes and Insights.

ASAIO J 2020 Sep/Oct;66(9):1036-1041

Pediatric Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.

The shortage of dedicated pediatric extracorporeal membrane oxygenation (ECMO) centers and the expanding indications for pediatric ECMO necessitate a regional program for transport of ECMO-supported patients. Data about feasibly and safety of pediatric ECMO transport are scarce. Our aim is to describe our experience with a pediatric ECMO reach-out program and review pertinent literature. Demographic, clinical, and outcome data were collected retrospectively from the charts of all patients cannulated onto ECMO at referring centers and transported to our center from 2003 to 2018. Similar data were recorded for patients who were referred for ECMO support from within the hospital. The cohort included 80 patients cannulated at 17 referring centers. The transport team included a senior pediatric cardiac surgeon and an ECMO specialist. All transfers but one were done by special emergency medical service ambulance. No major complications or deaths occurred during transport, and all patients were stable upon arrival to our unit. Mortality was lower in the ECMO reach-out cohort than in-house patients referred for ECMO support. This is the first study from Israel and one of the largest to date describing a dedicated pediatric ECMO transport program. Extracorporeal membrane oxygenation transport appears to be feasible and safe when conducted by a small, highly skilled mobile team. Successful reach-out program requires open communication between the referring physician and the accepting center. As survival correlates with ECMO volume, maintaining a large ECMO center with 24/7 retrieval capabilities may be the best strategy for pediatric mechanical circulatory support program.
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http://dx.doi.org/10.1097/MAT.0000000000001110DOI Listing
March 2021

Alveolar Dead-Space Fraction and Arterial Saturation Predict Postoperative Course in Fontan Patients.

Pediatr Crit Care Med 2020 04;21(4):e200-e206

Pediatric Cardiac Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Objectives: Fontan surgery, the final surgical stage in single ventricle palliation, redirects systemic venous blood into the pulmonary circulation for gas exchange. A decrease in pulmonary blood flow can lead to major complications and grave outcomes. Alveolar dead-space fraction represents the portion of inhaled air that does not participate in gas exchange and hence quantifies ventilation-perfusion abnormalities in the lung. Increased alveolar dead-space fraction has been associated with prolonged mechanical ventilation and worse outcome after congenital heart surgery. The association of alveolar dead-space fraction with clinical outcomes in patients undergoing Fontan operation has not been reported.

Interventions: None.

Design, Setting, And Patients: A retrospective charts review of all pediatric patients who underwent Fontan surgery during June 2010-November 2018 in a tertiary-care pediatric hospital. Associations between alveolar dead-space fraction and arterial oxyhemoglobin saturation to a composite outcome (surgical or catheter-based intervention, extracorporeal membrane oxygenation use, prolonged ventilation, prolonged hospital length of stay, or death) were explored. Secondary endpoints were parameters of severity of illness, chest drainage duration, and length of stay.

Measurements And Main Results: Of 128 patients undergoing Fontan operation, 34 met criteria for composite outcome. Alveolar dead-space fraction was significantly higher in the composite (0.33 ± 0.14) versus control (0.25 ± 0.26; p = 0.016) group. Alveolar dead-space fraction greater than or equal to 0.29 indicated a 37% increase in risk to meet composite criteria. Admission arterial oxygen saturation was significantly lower in composite versus control group (93.4% vs 97.1%; p = 0.005). Alveolar dead-space fraction was significantly associated with increased durations of mechanical ventilation, ICU length of stay, duration of thoracic drainage, and parameters of severity of illness.

Conclusions: Alveolar dead-space fraction and arterial saturation may predict complicated postoperative course in patients undergoing the Fontan operation.
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http://dx.doi.org/10.1097/PCC.0000000000002205DOI Listing
April 2020

Blood Cultures Drawn From Arterial Catheters Are Reliable for the Detection of Bloodstream Infection in Critically Ill Children.

Pediatr Crit Care Med 2018 05;19(5):e213-e218

Department of Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Objectives: Arterial catheters may serve as an additional source for blood cultures in children when peripheral venipuncture is challenging. The aim of the study was to evaluate the accuracy of cultures obtained through indwelling arterial catheters for the diagnosis of bloodstream infections in critically ill pediatric patients.

Design: Observational and comparative.

Setting: General and cardiac ICUs of a tertiary, university-affiliated pediatric medical center.

Patients: The study group consisted of 138 patients admitted to the general or cardiac PICU in 2014-2015 who met the following criteria: presence of an indwelling arterial catheter and indication for blood culture.

Interventions: Blood was drawn by peripheral venipuncture and through the arterial catheter for each patient and sent for culture (total 276 culture pairs).

Measurements And Main Results: Two specialists blinded to the blood source evaluated each positive culture to determine if the result represented true bloodstream infection or contamination. The sensitivity, specificity, and positive and negative predictive values of the arterial catheter and peripheral cultures for the diagnosis of bloodstream infection were calculated. Of the 56 positive cultures, 41 (15% of total samples) were considered diagnostic of true bloodstream infection. In the other 15 (5%), the results were attributed to contamination. The rate of false-positive results was higher for arterial catheter than for peripheral venipuncture cultures (4% vs 1.5%) but did not lead to prolonged unnecessary antibiotic treatment. On statistical analysis, arterial catheter blood cultures had high sensitivity (85%) and specificity (95%) for the diagnosis of true bloodstream infection, with comparable performance to peripheral blood cultures.

Conclusion: Cultures of arterial catheter-drawn blood are reliable for the detection of bloodstream infection in PICUs.
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http://dx.doi.org/10.1097/PCC.0000000000001462DOI Listing
May 2018

Neonatal Cardiac Surgery in the New Era: Lessons learned from 1000 Consecutive Cases.

Isr Med Assoc J 2016 Nov;18(11):645-648

Division of Pediatric Heart Institute, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.

Background: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success.

Objectives: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years.

Methods: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel.

Results: A total of 1003 neonates aged < 30 days underwent surgery for congenital heart defects at our center. Neonatal surgery accounted for 22.5% of all cardiac surgeries. Neonatal operative mortality was 7.3%, Operative mortality for individual lesions were: simple aortic coarctation (CoA) (198 patients, 2.5%), CoA with hypoplastic arch (24, 4%), CoA with ventricular septal defect (VSD) (84, 2.3%), transposition of the great arteries (TGA, simple and complex, 185, 6.3%), TGA with VSD (37, 0%), truncus arteriosus (26, 3.8%), interrupted aortic arch (25, 4%), Norwood Sano (71, 19.7%), neonatal tetralogy of Fallot (41, 0%), and shunt (131 patients, 12%).

Conclusions: Neonatal surgical capabilities have improved substantially over the last decades. Excellent results can be expected for lesions that can be repaired to create biventricular circulation. Improved results can be attributed in part to the evolution of surgical strategies and assistive technologies, but essential is the collaborative effort of surgeons, cardiologists, anesthesiologists, and intensive care specialists acting as a cohesive team whose performance far exceeds the sum of its individual members' contributions.
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November 2016

Pulmonary hypertension specific treatment in infants with bronchopulmonary dysplasia.

Pediatr Pulmonol 2017 01 22;52(1):77-83. Epub 2016 Jun 22.

Pulmonary Outpatient Clinic, Schneider Children's Medical Center of Israel, Petach Tikva, Affiliated With Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Objective: When bronchopulmonary dysplasia (BPD) is complicated by pulmonary hypertension (PH), morbidity and mortality are significantly increased. BPD-associated PH is not included in the current indications for PH medications. However, limited data demonstrate hemodynamic improvement and decreased mortality with PH-specific treatment. This report describes our 6-year experience treating BPD-associated PH with PH medications, mainly sildenafil.

Study Design: The medical records of 20 infants diagnosed with BPD-associated PH at a tertiary pediatric pulmonary hypertension clinic in 2008-2014 were reviewed. Clinical improvement was defined as a decrease in Ross functional class by at least one degree. PH severity was classified by echocardiography as mild, moderate, or severe. Hemodynamic improvement was defined as a decrease in PH severity by at least one level.

Results: Eighteen out of 20 patients were treated with PH medications: 12 sildenafil, 5 sildenafil and bosentan, and 1 bosentan. Median follow-up time was 2 years. Mean functional class significantly decreased from 3.2 ± 0.9 at diagnosis to 1.7 ± 0.9 at the last follow-up. Improvement in functional class was observed in 15/16 children (94%). Moderate or severe PH was found in 13/18 children (72%) at diagnosis, and in three (17%, all moderate PH) at the last follow-up. Improvement in PH class by echocardiography was demonstrated in 14/18 children (78%). The survival rate was 95%.

Conclusion: Treatment of BPD complicated by PH with PH-specific medications, mainly sildenafil, is associated with improvement in both clinical and hemodynamic parameters and a low mortality rate. Pediatr Pulmonol. 2017;52:77-83. © 2016 Wiley Periodicals, Inc.
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http://dx.doi.org/10.1002/ppul.23508DOI Listing
January 2017

Pediatric Dilated Cardiomyopathy Patients Do Not Meet Traditional Cardiac Resynchronization Criteria.

J Cardiovasc Electrophysiol 2015 Aug 25;26(8):885-889. Epub 2015 May 25.

Division of Cardiology, Children's National Medical Center and the Department of Pediatrics, George Washington University School of Medicine, Washington, District of Columbia, USA.

Introduction: Cardiac resynchronization therapy (CRT) is an effective device-based intervention for adults with heart failure (HF) with specific indications, based on large, multicenter randomized clinical trials. The criteria for CRT in adult HF include significant symptoms, ventricular systolic dysfunction, prolonged QRS duration, and left bundle branch block (LBBB) pattern on electrocardiogram (ECG). Despite having less data, CRT is also being widely utilized in children with HF. The shortage of evidence-based CRT criteria in pediatrics prompted us to review a cohort of children with dilated cardiomyopathy and evaluate their potential eligibility for CRT using the traditional adult criteria.

Methods: Single-center data of all pediatric patients with dilated cardiomyopathy were extracted from the heart failure registry and retrospectively reviewed. Patients who had at least 2 separate visits that included HF scoring, electrocardiogram, and echocardiogram were included. Patients who were ventricular paced were excluded.

Results: Data for 52 patients meeting inclusion criteria were analyzed. The mean ejection fraction was 25% on the first clinical evaluation and 27% on the second visit. No patient and 2 patients met the adult criteria for prolonged QRS on the first and second encounters, respectively. No patients had an LBBB pattern on ECG.

Conclusions: None of the pediatric HF patients in our study met the published Class I criteria for CRT device therapy in adults. These findings suggest that extrapolation of adult HF data to pediatrics is not sufficient for CRT criteria. Specific guidelines for device implantation in children must be based on scientific investigation including pediatric clinical trials.
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http://dx.doi.org/10.1111/jce.12690DOI Listing
August 2015

Noncoronary inflammation in Kawasaki disease is associated with abnormal myocardial deformation in the acute phase.

J Am Soc Echocardiogr 2014 Dec;27(12):1329-35

Department of Cardiology, Children's National Medical Center, Washington, District of Columbia.

Background: Patients with Kawasaki disease (KD) are at risk for developing coronary artery lesions, but the association of noncoronary changes such as mitral regurgitation (MR) and/or pericardial effusion (PE) with cardiac mechanics in the acute phase of KD has not been previously described. The aim of this study was to test the hypothesis that these noncoronary markers for carditis are associated with abnormalities in strain (ε) and strain rate (SR) in patients with MR or PE not appreciated by conventional echocardiography.

Methods: Longitudinal and circumferential ε and SR analyses were retrospectively performed on patients with KD. Patients with and without MR or PE were compared. Strain values were also compared between patients with and without coronary artery lesions. Values for ejection fraction, shortening fraction, and clinical laboratory parameters were correlated with MR or PE. Follow-up echocardiographic outcomes were recorded at the first encounter after initial diagnosis. Follow-up ε and SR data were also obtained in the group with MR or PE and altered ventricular mechanics at diagnosis.

Results: Of the 110 patients reviewed, 92 had appropriate image quality for either longitudinal ε and SR or circumferential ε and SR analysis. Twenty-eight patients (30%) had either MR or PE. Longitudinal ε and SR were significantly decreased in patients with MR or PE compared with patients without MR or PE (ε: -16.4 ± 4.0% vs -19.0 ± 3.7%, P = .004; SR: -1.3 ± 0.7 vs -1.6 ± 0.4 sec(-1), P = .03). No significant difference in longitudinal ε or SR was noted between patients with and without coronary artery lesions (ε: -17.9 ± 4.1% vs -17.8 ± 3.8%, P = .50; SR: -1.5 ± 0.3 vs -1.6 ± 0.8 sec(-1), P = .50). In the group with abnormal coronary arteries, presence of MR or PE was correlated with decreased longitudinal ε (-16.1 ± 3.6% vs -18.9 ± 3.4%, P = .02), without a significant difference in longitudinal SR (-1.6 ± 0.4 vs -1.5 ± 0.4 sec(-1), P = .20). At approximately 3-week follow-up (21.3 ± 15.8 days), longitudinal ε and SR for the group with MR or PE had increased significantly compared with diagnosis (ε: -16.4 ± 4.3% vs -18.6 ± 0.5%, P = .03; SR: -1.3 ± 0.6 vs -1.8 ± 0.4 sec(-1), P = .008), coincident with resolution of MR or PE. In both groups, erythrocyte sedimentation rate and C-reactive protein were elevated (85.3 ± 36.2 mm/h vs 75.1 ± 33.1 mm/h [P = .34] and 12.3 ± 6.7 vs 11.7 ± 8.2 mg/dL [P = .83]), but only modest correlations were noted between longitudinal ε and elevated erythrocyte sedimentation rate (r = 0.52, P = .01; confidence interval, 0.10-0.80) and C-reactive protein (r = 0.50, P = .02; confidence interval, 0.10-0.80) in patients with MR or PE. Shortening fraction and ejection fraction were within the normal range in both groups.

Conclusions: Patients presenting with KD with MR or PE at diagnosis are likely to have altered ventricular mechanics compared with patients with KD without MR or PE despite normal conventional echocardiographic measures of function. There is no significant difference in ventricular mechanics when comparing patients with KD with coronary ectasia or aneurysms and those without coronary lesions. Presence of abnormal ε in patients with KD with altered ventricular mechanics correlates modestly with laboratory inflammatory markers. Peak systolic longitudinal ε and SR increased significantly at 3-week follow-up compared with initial diagnosis, coincident with resolution of MR or PE.
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http://dx.doi.org/10.1016/j.echo.2014.09.014DOI Listing
December 2014

Reconstruction of right ventricular outflow tract in neonates and infants using valved cryopreserved femoral vein homografts.

J Thorac Cardiovasc Surg 2014 Mar 15;147(3):874-9. Epub 2013 Dec 15.

Division of Cardiac Surgery, Children's National Medical Center, Washington, DC. Electronic address:

Objectives: Aortic or pulmonary homografts (A/PHs) are common biomaterials used for restoration of right ventricle to pulmonary artery continuity for repair of various congenital heart defects. The smaller sized homografts required for early primary repair in neonates and infants are prone to early failure and are in short supply. Due to these limitations, since 2008 it has been our preference to use valved segments of cryopreserved femoral vein homograft (cFVH) for right ventricle to pulmonary artery reconstruction. This study was undertaken to assess the performance of cFVH compared with A/PH in neonates and infants.

Methods: A retrospective review of all infants and neonates who underwent biventricular early primary repair with right ventricle to pulmonary artery reconstruction using homograft conduits at a single center was conducted. Patients who received cFVH constituted the study group, whereas all other patients received A/PH and formed the control group. Patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals who had conduits placed to promote pulmonary artery growth or to unifocalized pulmonary vasculature were excluded from the study because they have different clinical indications for reoperation and reintervention. Demographic, anatomical, perioperative, and follow-up variables were compared between the groups using univariate and multivariable Cox regression analyses. Kaplan-Meier analysis and log-rank tests were used to identify intergroup differences in freedom from catheter intervention, reoperation, or overall freedom from reintervention (catheter and/or surgical).

Results: A total of 36 patients (20 cFVH and 16 A/PH) were included in the study. There were no intergroup differences in the demographic, anatomic, and perioperative variables, except for significantly shorter aortic crossclamp time in the cFVH group. Univariate analysis revealed a higher catheter reintervention rate as well as higher reoperation rate in the A/PH group. Multivariate Cox regression correcting for the intergroup differences in the length of follow-up revealed comparable freedom from catheter intervention, freedom from reoperation, or freedom from either intervention in the cFVH and the A/PH groups.

Conclusions: Valved femoral vein homografts have comparable short- and intermediate-term performance to A/PHs for right ventricular outflow tract reconstruction and are an attractive alternative to other small conduits for use in neonates and infants.
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http://dx.doi.org/10.1016/j.jtcvs.2013.11.006DOI Listing
March 2014

Adrenal insufficiency in children undergoing heart surgery does not correlate with more complex postoperative course.

Pediatr Cardiol 2013 23;34(8):1860-7. Epub 2013 Jun 23.

Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202, Petach Tikva, Israel,

Although some evidence suggests benefit of steroid supplementation after pediatric cardiac surgery, data correlating adrenal function with the postoperative course is scarce. This study sought to determine if adrenal insufficiency (AI) after cardiac surgery is associated with a more complicated postoperative course in children. A prospective study was performed during a 6-month period at a pediatric medical center. Included were 119 children, 3 months and older, who underwent heart surgery with cardiopulmonary bypass. Cortisol levels were measured before and 18 h after surgery. Patients were divided into two groups by procedure complexity (low or high), and clinical and laboratory parameters were compared between patients with and without AI within each complexity group. In the low-complexity group, 45 of the 65 patients had AI. The normal adrenal function (NAF) subgroup had greater inotropic support at 12, 24, and 36 h after surgery and a higher lactate level at 12 and 24 h after surgery. There were no significant differences between subgroups in duration of ventilation, sedation, intensive care unit (ICU) stay, or urine output. In the high-complexity group, 27 patients had AI, and 27 did not. There were no significant differences between subgroups in inotropic support or urine output during the first 36 h or in mechanical ventilation, sedation, or ICU stay duration. Children with AI after heart surgery do not have a more complex postoperative course than children with NAF. The adrenal response of individual patients seems to be appropriate for their cardiovascular status.
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http://dx.doi.org/10.1007/s00246-013-0728-yDOI Listing
July 2014

The poor performance of RSR' pattern on electrocardiogram lead V1 for detection of secundum atrial septal defects in children.

J Pediatr 2013 Feb 19;162(2):308-12. Epub 2012 Aug 19.

Division of Cardiology, Children's National Medical Center, Washington, DC 20010-2970, USA.

Objective: To assess the accuracy of RSR' patterns in lead V1 (RSR'-V1) in diagnosing atrial septal defects (ASDs) in children.

Study Design: Children who underwent an electrocardiogram (ECG) during 2010 were divided into 2 ECG groups: RSR'-V1 and normal (no RSR'-V1). Children who underwent an echocardiogram during 2010 were also divided into an ASD group and a normal echocardiogram group. The 4 groups were matched in a 2 × 2 table format where the RSR'-V1 was the "test" and ASD was the "disease." Sensitivity, specificity, positive/negative predictive values, and pre/post-test probabilities were calculated.

Results: There were 4658 ECG studies included in the analysis: 836 had RSR'-V1 and 3822 were normal without RSR'-V1. Of 4935 echocardiographic studies analyzed, 329 had an ASD and 4606 were normal; 1363 patients had both studies done during the study period. The ECG sensitivity for diagnosing an ASD was 36.1%, specificity was 80%, positive predictive value was 14.7%, and negative predictive value was 92.9% with an overall accuracy of 76.2%. Patients with ASD and RSR'-V1 were significantly older than patients with ASD and no RSR'-V1 pattern.

Conclusion: RSR'-V1 is a poor screening test for the detection of ASD. It should not change the clinical suspicion or the decision to obtain an echocardiogram. Older children without RSR'-V1 on ECG are unlikely to have an ASD.
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http://dx.doi.org/10.1016/j.jpeds.2012.07.017DOI Listing
February 2013

Polymerase-chain-reaction-based diagnosis of invasive fungal pulmonary infections in immunocompromised children.

Pediatr Pulmonol 2012 Oct 10;47(10):994-1000. Epub 2012 Feb 10.

Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Objective: Fungal pneumonia is a serious complication in immunocompromised children. It is difficult to diagnose because of the low sensitivity of clinical and standard laboratory tests. The aim of this study was to investigate the diagnostic impact of polymerase chain reaction (PCR) assays for fungal pathogens in bronchoalveolar lavage (BAL) fluid.

Study Design: BAL samples obtained from hospitalized immunocompromised patients with clinical pneumonia between January 2007 and June 2009 were processed for microscopy and cultures in addition to PCR-based fungal assays. The results were compared between the standard and PCR methods.

Results: Seventy-seven children with 100 episodes of pneumonia were included in the study. Fungal pathogens were detected by standard microbiological investigations in 10 episodes (10%) and by PCR-based assays alone in 20 episodes (20%). There was no significant difference in clinical improvement or mortality rate between patients diagnosed by the different methods. In 61 episodes, no fungal pathogen was identified by either method. Prolonged antifungal therapy was avoided in 43 episodes.

Conclusion: PCR-based assay for the diagnosis of fungal pulmonary infections may be a useful adjunct to clinical and standard microbiological techniques. The use of PCR may decrease the time to diagnosis, increase the rate of detection of fungal pathogens, and spare patients unnecessary antifungal treatment.
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http://dx.doi.org/10.1002/ppul.22523DOI Listing
October 2012

Cor triatriatum with partial anomalous pulmonary venous return: a rare case of parallel obstruction and successful staged treatment.

Pediatr Cardiol 2012 Feb 25;33(2):363-5. Epub 2011 Nov 25.

Children's National Medical Center, Washington, DC 20010, USA.

Cor triatriatum sinister is an uncommon congenital cardiac defect that has rarely been described in association with left-sided partial anomalous pulmonary venous return. We present a case of such rare anatomy with multilevel obstruction that presented in infancy as cardiogenic shock. The patient underwent staged treatment with extracorporeal membrane oxygenation stabilization, catheter-based balloon dilatation of the cor triatriatum and atrial septostomy, followed by definitive surgical repair, with excellent result.
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http://dx.doi.org/10.1007/s00246-011-0148-9DOI Listing
February 2012

Procalcitonin level as an aid for the diagnosis of bacterial infections following pediatric cardiac surgery.

J Crit Care 2012 Apr 29;27(2):220.e11-6. Epub 2011 Sep 29.

Pediatric Critical Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 49202, Israel.

Purpose: The aim of the present study was to determine if blood procalcitonin can serve as an aid to differentiate between bacterial and nonbacterial cause of fever in children after cardiac surgery.

Materials And Methods: A nested case-control study of children who underwent open cardiac surgery in critical care units of fourth-level pediatric hospital was performed. Blood samples for procalcitonin level were collected 1 day before operation; 1 hour postoperation; on postoperative days 1, 2, and 5; and on the day of fever, when it occurred.

Results: Of 665 children who underwent cardiac bypass surgery, 126 had a febrile episode postoperatively, 47 children with a proven bacterial infection and 79 without bacterial infection. Among the 68 children in whom fever developed within the first 5 postoperative days, procalcitonin level at fever day was significantly higher in those with bacterial infection (n = 16) than in those without infection (n = 52). Similarly, among the 58 children in whom fever developed after day 5 postoperation, a significant difference was found in procalcitonin level at fever day between those with (n = 31) and without (n = 27) bacterial infection.

Conclusion: During the critical early and late periods after cardiac surgery in children, procalcitonin level may help to differentiate patients with bacterial infection from patients in whom the fever is secondary to nonbacterial infectious causes.
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http://dx.doi.org/10.1016/j.jcrc.2011.07.076DOI Listing
April 2012

Role of C-reactive protein velocity in the diagnosis of early bacterial infections in children after cardiac surgery.

J Intensive Care Med 2012 May-Jun;27(3):191-6. Epub 2011 May 11.

Pediatric Critical Care Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.

Fever after cardiac surgery in children may be due to bacterial infection or noninfectious origin like systemic inflammatory response syndrome (SIRS) secondary to bypass procedure. A marker to distinguish bacterial from nonbacterial fever in these conditions is clinically important. The purpose of our study was to evaluate, in the early postcardiac surgery period, whether serial measurement of C-reactive protein (CRP) and its change over time (CRP velocity) can assist in detecting bacterial infection. A series of consecutive children who underwent cardiac surgery with bypass were tested for serum levels of CRP at several points up to 5 days postoperatively and during febrile episodes (>38.0°C). Findings were compared among febrile patients with proven bacterial infection (FWI group; sepsis, pneumonia, urinary tract infection, deep wound infection), febrile patients without bacterial infection (FNI group), and patients without fever (NF group). In all, 121 children were enrolled in the study, 31 in the FWI group, 42 in the FNI group, and 48 patients in the NF group. Ages ranged from 4 days to 17.8 years (median 19.0, mean 46 ± 56 months). There was no significant difference among the groups in mean CRP level before surgery, 1 hour, and 18 hours after. A highly significant interaction was found in the change in CRP over time by FWI group compared with FNI group (P < .001). Mean CRP velocity ([fCRP - 18hCRP]/[fever time (days) - 0.75 day]) was significantly higher in the infectious group (4.0 ± 4.2 mg/dL per d) than in the fever-only group (0.60 ± 1.6 mg/dL per d; P < .001). A CRP velocity of 4 mg/dL per d had a positive predictive value (PPV) of 85.7% for bacterial infection with 95.2% specificity. Serial measurements of CRP/CRP velocity after cardiac surgery in children may assist clinicians in differentiating postoperative fever due to bacterial infection from fever due to noninfectious origin.
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http://dx.doi.org/10.1177/0885066610396642DOI Listing
October 2012

Postoperative thrombotic thrombocytopenic purpura in an infant: case report and literature review.

J Pediatr Surg 2011 Apr;46(4):764-766

Institute of Pediatric Nephrology, Schneider Children's Medical Center of Israel, Petach Tikva 49202, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Thrombotic thrombocytopenic purpura is caused by an imbalance of von Willebrand factor and its cleaving protease, which leads to the formation of microthrombi in end-organs. It rarely occurs in the pediatric population. Plasma exchange can significantly reduce mortality and morbidity. We present a 14-month-old infant in whom clinical and laboratory abnormalities compatible with thrombotic thrombocytopenic purpura were noted several days after resection of a large pelvic tumor. Treatment with double volume plasma exchange on postoperative day 5 led to complete resolution of the renal failure, thrombocytopenia, anemia, and neurological manifestations. ADAMTS13 inhibitors were negative and no mutations were found in factor H, factor I, membrane cofactor protein, and thrombomodulin to account for genetic predisposition to thrombotic thrombocytopenic purpura or atypical hemolytic uremic syndrome. Postoperative anemia, thrombocytopenia, fever, and neurological deficits in children should raise the suspicion of thrombotic thrombocytopenic purpura. Early diagnosis is important because the disorder is readily and efficiently treated with plasma exchange.
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http://dx.doi.org/10.1016/j.jpedsurg.2011.01.009DOI Listing
April 2011

Central apnoeas in infants with bronchiolitis admitted to the paediatric intensive care unit.

Acta Paediatr 2011 Feb 29;100(2):216-9. Epub 2010 Sep 29.

Schneider Children's Medical Center of Israel, Petah Tiqwa, Israel.

Aim: To further characterize apnoea(s) complicating bronchiolitis because of respiratory syncytial virus (RSV), to describe the incidence of this complication and identify possible risk factors for apnoea(s) and its development.

Methods: The files of infants admitted to the paediatric intensive care unit (PICU) for RSV bronchiolitis during three bronchiolitis seasons (2004-2007) were reviewed for demographic, clinical and laboratory parameters. Parameters were compared between patients with and without apnoeas.

Results: Seventy-nine patients met the study criteria: 43 were admitted to the PICU for central apnoeas and the remainder for respiratory distress or failure. The percentage of infants admitted for apnoea increased during the study period (28.6 to 77.1%, p = 0.004). The overall prevalence of apnoea in this population was 4.3%. Possible risk factors for apnoea(s) were younger age (1.3 vs. 4.3 months, p = 0.002), lower admission weight (3.3 vs. 5 kg, p < 0.001), lower gestational age (35.8 vs. 37.8 weeks, p = 0.01), admission from the emergency room (50% vs. 9.1%, p < 0.001) and lack of hyperthermia (p < 0.001). Respiratory acidosis was found to be a protective factor on logistic regression analysis.

Conclusion: The prevalence of apnoea in infants admitted to the PICU for RSV bronchiolitis in our centre may be increasing. Preterm, younger infants with no fever are at relatively high risk of apnoea at presentation, while older infants with fever are at lower risk.
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http://dx.doi.org/10.1111/j.1651-2227.2010.02004.xDOI Listing
February 2011

Nitric oxide for post-liver-transplantation hypoxemia in pediatric hepatopulmonary syndrome: case report and review.

Pediatr Transplant 2011 Nov 8;15(7):E130-4. Epub 2010 Apr 8.

Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petah Tiqwa 49202, Israel.

HPS is rare in the pediatric population. Liver transplantation is the ultimate treatment for severe HPS. There are only a few case reports and one series of children in whom HPS was the main indication for liver transplantation. Outcome was good in most of them, with full regression of the pulmonary process. However, hypoxemia in the early post-operative course can have severe consequences, and effective treatment modalities are needed. There are rare instances of the use of iNO for the treatment of post-operative hypoxemia. We describe a 10.5-yr-old boy with severe HPS owing to chronic liver disease after bone marrow transplantation. Liver transplantation from a living related donor (the same sister who donated the bone marrow) was complicated by severe hypoxemia on POD 2. iNO was administered via the ventilator circuit and, after extubation, through nasal prongs. It was slowly tapered down and stopped on POD 10. The child had an otherwise uneventful course and was discharged home on POD 21 with normal oxygen saturation. Liver transplantation should be offered to children with severe HPS. iNO can reverse the hypoxemia that may occur after the operation.
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http://dx.doi.org/10.1111/j.1399-3046.2010.01313.xDOI Listing
November 2011

Bi-level positive airway pressure ventilation in pediatric oncology patients with acute respiratory failure.

J Intensive Care Med 2009 Nov-Dec;24(6):383-8. Epub 2009 Oct 29.

Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petah Tiqva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

The aim of the study was to describe our experience with bi-level positive airway pressure (BiPAP) ventilation in oncology children with acute respiratory failure, hospitalized in a single tertiary pediatric tertiary center. This was a retrospective cohort study of all pediatric oncology patients in our center admitted to the intensive care unit with acute hypoxemic or hypercarbic respiratory failure from January 1999 through May 2006, who required mechanical ventilation with BiPAP. Fourteen patients met the inclusion criteria with a total of 16 events of respiratory failure or impending failure: 12 events were hypoxemic, 1 was combined hypercarbic and hypoxemic, and 3 had severe respiratory distress. Shortly after BiPAP ventilation initiation, there was a statistically significant improvement in the respiratory rate (40.4 +/- 9.3 to 32.5 +/- 10.1, P < .05] and a trend toward improvement in arterial partial pressure of oxygen (PaO(2); 71.3 +/- 32.7 to 104.6 +/- 45.6, P = .055). The improvement in the respiratory status was sustained for at least 12 hours. In 12 (75%) events there was a need for sedation during ventilation; 12 children needed inotropic support during the BiPAP ventilation. Bi-level positive airway pressure ventilation failed in 3 (21%) children who were switched to conventional ventilation. All of them have died during the following days. One child was recategorized to receive palliative care while on BiPAP ventilator and was not intubated. In 12 of 16 BiPAP interventions (75%; 11 patients), the children survived to pediatric intensive care unit (PICU) discharge without invasive ventilation. No major complications were noted during BiPAP ventilation. Bi-level positive airway pressure ventilation is well tolerated in pediatric oncology patients suffering from acute respiratory failure and may offer noninferior outcomes compared with those previously described for conventional invasive ventilation. It appears to be a feasible initial option in children with malignancy experiencing acute respiratory failure.
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http://dx.doi.org/10.1177/0885066609344956DOI Listing
February 2010

Computerized order entry with limited decision support to prevent prescription errors in a PICU.

Pediatrics 2009 Sep 10;124(3):935-40. Epub 2009 Aug 10.

Schneider Children's Medical Center of Israel, Pediatric Intensive Care Unit, Petach Tikva, Israel.

Objective: The value of computerized physician order entry (CPOE) and clinical decision support systems (CDSSs) in preventing prescription errors in pediatrics is unclear. We investigated the change in prescription error rates with the introduction of CPOE with and without a CDSS limited to weight-based dosing in a PICU.

Methods: In a PICU of a major tertiary-care pediatric medical center, 5000 orders were reviewed, that is, 1250 orders from each of 4 periods: period 1, before CPOE implementation; period 2, 1 year after CPOE implementation; period 3, after CDSS implementation; and period 4, after a change in prescription authorization. Prescription errors were identified and classified into: potential adverse drug events (ADEs), medication prescription errors (MPEs), and rule violations (RVs).

Results: We identified 273 errors (5.5%). The rate of potential ADEs decreased slightly between periods 1 and 2 (from 2.5% to 2.4%) and significantly in periods 3 and 4 (to 0.8% and 0.7%, respectively; P < .005). The rate of MPEs decreased slightly between periods 1 and 2 (from 5.5% to 5.3%), but new types of MPEs appeared. There was a significant decrease in period 3 (to 3.8%; P < .05) and a dramatically significant decrease in period 4 (to 0.7%; P < .0005). Only 3 RVs were found. Interrater agreement (kappa statistic) was 0.788 between evaluators.

Conclusions: CPOE implementation decreased prescription errors only to a small extent. However, the addition of a CDSS that limits doses by weight significantly reduced prescription error rates and, most importantly, potential ADEs. This finding emphasizes the major impact of weight-based calculation errors in pediatrics.
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http://dx.doi.org/10.1542/peds.2008-2737DOI Listing
September 2009

Pulmonary hypertension in an infant with achondroplasia.

Pediatr Neurosurg 2008 13;44(4):341-3. Epub 2008 Jun 13.

Department of Pediatrics B, Schneider Children's Medical Center of Israel, Petah Tiqva, Israel.

An 18-month-old achondroplastic child presented with respiratory distress and severe pulmonary hypertension which was considered to be due to an atrial septal defect. The septal defect was closed via catheterization with Amplatzer occluder device, but the patient showed only mild to moderate clinical improvement. In addition, sleep monitoring study revealed apneas, oxygen desaturation and CO(2) retention; therefore, magnetic resonance imaging of the brain was performed, showing medullary compression by a stenotic foramen magnum. Surgical craniocervical decompression led to an improvement in sleep disturbances and pulmonary hypertension. In conclusion, several factors, among which medullary compression, may be a cause of pulmonary hypertension in achondroplasia patients.
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http://dx.doi.org/10.1159/000138374DOI Listing
September 2008

Yunis-Varon syndrome: further delineation of the phenotype.

Am J Med Genet A 2008 Feb;146A(4):532-7

Department of Medical Genetics, Schneider Children's Medical Center of Israel and Rabin Medical Center, Beilinson Campus, Petah Tikva, Israel.

Yunis-Varon syndrome (YVS) is a rare autosomal recessive condition characterized by limb defects, ossification defects, generalized hypotrichosis and, frequently, a severe neonatal course. The molecular basis is unknown. We report on a newborn infant with previously undescribed findings, including hydrops fetalis, primary pulmonary hypertension and unusually severe abnormalities of toes. We review clinical data on 22 published cases in order to delineate the phenotype of this condition. Clinical recommendations for prenatal and postnatal evaluation of patients and fetuses at risk are discussed.
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http://dx.doi.org/10.1002/ajmg.a.32135DOI Listing
February 2008