Alan Flake

Summary

Affiliation: The Children's Hospital of Philadelphia
Country: USA

Publications

  1. ncbi request reprint In utero hematopoietic stem cell transplantation. A status report
    A W Flake
    Department of Surgery, Children s Hospital of Philadelphia, PA 19104, USA
    JAMA 278:932-7. 1997
  2. ncbi request reprint Chorioamniotic membrane separation following open fetal surgery: pregnancy outcome
    R Douglas Wilson
    Department of Surgery, Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA
    Fetal Diagn Ther 18:314-20. 2003
  3. ncbi request reprint In utero stem cell transplantation
    Alan W Flake
    Surgery and Obstetrics and Gynecology, University of Pennsylvania, USA
    Best Pract Res Clin Obstet Gynaecol 18:941-58. 2004
  4. pmc Surgery in the human fetus: the future
    Alan W Flake
    Department of Surgery and the Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, PA 19104, USA
    J Physiol 547:45-51. 2003
  5. ncbi request reprint The conceptual application of systems theory to stem cell biology: a matter of context
    Alan W Flake
    The Children s Institute for Surgical Science, Children s Hospital of Philadelphia, Philadelphia, PA 19104 4318, USA
    Blood Cells Mol Dis 32:58-64. 2004
  6. ncbi request reprint Genetic therapies for the fetus
    Alan W Flake
    Center for Fetal Diagnosis and Therapy, The Children s Institute for Surgical Science, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Clin Obstet Gynecol 45:684-96; discussion 730-2. 2002
  7. ncbi request reprint In utero stem cell transplantation for the treatment of genetic diseases
    A W Flake
    Department of Surgery, Children s Hospital of Philadelphia, PA 19104, USA
    Schweiz Med Wochenschr 129:1733-9. 1999
  8. ncbi request reprint The current status and future potential of fetal intervention: image is everything
    A W Flake
    The Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, PA 19104 4399, USA
    Comput Med Imaging Graph 23:51-7. 1999
  9. ncbi request reprint In utero transplantation for thalassemia
    A W Flake
    Children s Institute of Surgical Science, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Ann N Y Acad Sci 850:300-11. 1998
  10. ncbi request reprint Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome
    Holly L Hedrick
    Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104 4399, USA
    J Pediatr Surg 39:430-8; discussion 430-8. 2004

Research Grants

Detail Information

Publications80

  1. ncbi request reprint In utero hematopoietic stem cell transplantation. A status report
    A W Flake
    Department of Surgery, Children s Hospital of Philadelphia, PA 19104, USA
    JAMA 278:932-7. 1997
    ....
  2. ncbi request reprint Chorioamniotic membrane separation following open fetal surgery: pregnancy outcome
    R Douglas Wilson
    Department of Surgery, Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA
    Fetal Diagn Ther 18:314-20. 2003
    ....
  3. ncbi request reprint In utero stem cell transplantation
    Alan W Flake
    Surgery and Obstetrics and Gynecology, University of Pennsylvania, USA
    Best Pract Res Clin Obstet Gynaecol 18:941-58. 2004
    ..This chapter presents the recognized barriers to engraftment in the fetus and discusses promising experimental strategies to overcome these barriers...
  4. pmc Surgery in the human fetus: the future
    Alan W Flake
    Department of Surgery and the Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, PA 19104, USA
    J Physiol 547:45-51. 2003
    ....
  5. ncbi request reprint The conceptual application of systems theory to stem cell biology: a matter of context
    Alan W Flake
    The Children s Institute for Surgical Science, Children s Hospital of Philadelphia, Philadelphia, PA 19104 4318, USA
    Blood Cells Mol Dis 32:58-64. 2004
    ..Although the complexity of the system is beyond current mathematical modeling, the conceptual framework of systems theory can provide context to multiple controversies in stem cell biology...
  6. ncbi request reprint Genetic therapies for the fetus
    Alan W Flake
    Center for Fetal Diagnosis and Therapy, The Children s Institute for Surgical Science, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Clin Obstet Gynecol 45:684-96; discussion 730-2. 2002
  7. ncbi request reprint In utero stem cell transplantation for the treatment of genetic diseases
    A W Flake
    Department of Surgery, Children s Hospital of Philadelphia, PA 19104, USA
    Schweiz Med Wochenschr 129:1733-9. 1999
    ..In the near future, advances in our understanding of stem cell biology, developmental ontogeny, and gene therapy may allow prenatal stem cell and gene therapy to achieve their full potential...
  8. ncbi request reprint The current status and future potential of fetal intervention: image is everything
    A W Flake
    The Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, PA 19104 4399, USA
    Comput Med Imaging Graph 23:51-7. 1999
    ..In the future improvements in imaging and non-invasive technology will drive further expansion of indications and therapeutic options for fetal intervention...
  9. ncbi request reprint In utero transplantation for thalassemia
    A W Flake
    Children s Institute of Surgical Science, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Ann N Y Acad Sci 850:300-11. 1998
    ..Prenatal strategies involving multiple transplants, donor-specific tolerance induction, and postnatal same-donor transplants should be considered...
  10. ncbi request reprint Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome
    Holly L Hedrick
    Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104 4399, USA
    J Pediatr Surg 39:430-8; discussion 430-8. 2004
    ..To understand the natural history and define indications for fetal intervention in sacrococcygeal teratoma (SCT), the authors reviewed all cases of fetal SCT presenting for evaluation...
  11. ncbi request reprint Reproductive outcomes after pregnancy complicated by maternal-fetal surgery
    R Douglas Wilson
    Pediatric Surgery, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Am J Obstet Gynecol 191:1430-6. 2004
    ..The reproductive outcomes for women after the pregnancy complicated by maternal-fetal surgery were evaluated to obtain evidence-based information for prenatal risk counseling...
  12. ncbi request reprint The EXIT procedure: experience and outcome in 31 cases
    Sarah Bouchard
    Division of Pediatric General, The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    J Pediatr Surg 37:418-26. 2002
    ..The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions...
  13. ncbi request reprint Transduction of satellite cells after prenatal intramuscular administration of lentiviral vectors
    Tippi C MacKenzie
    The Children s Institute for Surgical Science, Children s Hospital of Philadelphia, Philadelphia, 3615 Civic Center Blvd, Philadelphia, PA 19104 4318, USA
    J Gene Med 7:50-8. 2005
    ..In this study we performed experiments to further define the morphology and function of these cells...
  14. ncbi request reprint Management of congenital lung lesions
    N Scott Adzick
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Semin Pediatr Surg 12:10-6. 2003
    ..The finding that fetuses with hydrops are at very high risk for fetal or neonatal death has led to successful fetal thoracoamniotic shunt placement or fetal surgical resection...
  15. ncbi request reprint Persistent expression of hF.IX After tolerance induction by in utero or neonatal administration of AAV-1-F.IX in hemophilia B mice
    Denise E Sabatino
    Department of Hematology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Mol Ther 15:1677-85. 2007
    ..IX expression. This supports the concept of a narrow "window of opportunity" for tolerance induction...
  16. doi request reprint Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome
    Andrew Mong
    Department of Radiology, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Pediatr Radiol 38:1171-9. 2008
    ..Prompt airway intervention at delivery after accurate prenatal diagnosis may allow survival of this otherwise fatal condition...
  17. ncbi request reprint Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia
    Holly L Hedrick
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Am J Obstet Gynecol 197:422.e1-4. 2007
    ..The purpose of this study was to determine the ability of liver position and lung-to-head ratio to predict outcome in isolated left congenital diaphragmatic hernia...
  18. ncbi request reprint Abnormal umbilical cord Doppler sonograms may predict impending demise in fetuses with sacrococcygeal teratoma. A report of two cases
    Oluyinka O Olutoye
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Fetal Diagn Ther 19:35-9. 2004
    ..To identify factors predictive of fetal demise in fetuses with sacrococcygeal teratoma (SCT)...
  19. ncbi request reprint Epinephrine inhibits tracheal occlusion induced lung growth in fetal sheep
    Yoshihiro Kitano
    The Children s Institute for Surgical Science, The Children s Hospital of Philadelphia, Philadelphia, PA 19104 4399, USA
    Fetal Diagn Ther 18:333-7. 2003
    ..We hypothesized that continuous infusion of Epi would have a detrimental effect on lung growth following TO in the fetal sheep model...
  20. ncbi request reprint Severe pulmonary hypoplasia associated with giant cervical teratomas
    Kenneth W Liechty
    Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 41:230-3. 2006
    ..The use of the ex utero intrapartum treatment (EXIT) procedure has salvaged many fetuses with giant neck masses. Despite an adequate airway, a subset of these patients die from an inability to achieve adequate gas exchange...
  21. ncbi request reprint Abnormal umbilical cord Dopplers may predict impending demise in fetuses with sacrococcygeal teratoma. A report of 2 cases
    Oluyinka O Olutoye
    The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Fetal Diagn Ther 18:428-31. 2003
    ..To identify factors predictive of fetal demise in fetuses with sacrococcygeal teratoma (SCT)...
  22. ncbi request reprint Congenital high airway obstruction syndrome: natural history and management
    Foong Yen Lim
    Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 38:940-5. 2003
    ..Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition with a poorly understood natural history...
  23. doi request reprint Fetal tracheal occlusion for severe congenital diaphragmatic hernia in humans: a morphometric study of lung parenchyma and muscularization of pulmonary arterioles
    Enrico Danzer
    The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104 4318, USA
    J Pediatr Surg 43:1767-75. 2008
    ..The aim of this study is to examine structure of lung parenchyma (Pp) and muscularization of pulmonary arterioles (PAs) in human fetuses that underwent tracheal occlusion (TO) therapy for severe congenital diaphragmatic hernia (CDH)...
  24. doi request reprint Emergent excision of a prenatally diagnosed sacrococcygeal teratoma
    Kha M Tran
    Department of Anesthesiology and Critical Care Medicine, The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Paediatr Anaesth 18:431-4. 2008
    ..We present a case of prenatally diagnosed SCT and address the evaluation, anesthetic considerations, and mechanisms needed to care for this high risk population...
  25. doi request reprint Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome
    R Douglas Wilson
    Fetal Heart Program, The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Fetal Diagn Ther 25:15-20. 2009
    ..Prenatal surveillance and growth characteristics are evaluated in a cohort of fetuses with sacrococcygeal teratomas (SCT) as part of risk assessment...
  26. ncbi request reprint Successful revision of portoenterostomy in an infant with biliary atresia
    Barbara A Haber
    Division of Gastroenterology and Nutrition, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 41:e1-3. 2006
    ..At 14 months of age, the child is anicteric and growing well. In this case, successful revision of hepatoportoenterostomy averted the need for liver transplantation...
  27. doi request reprint Predicting the severity of congenital high airway obstruction syndrome
    Jessica L Roybal
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, PA 19104 4318, USA
    J Pediatr Surg 45:1633-9. 2010
    ..In our relatively large series of CHAOS patients, we have observed a spectrum of clinical severity. The aim of this study was to identify the prenatal characteristics of CHAOS predictive of a milder postnatal course...
  28. ncbi request reprint Apoptotic cell-mediated immunoregulation of dendritic cells does not require iC3b opsonization
    Edward M Behrens
    Laboratory of Dendritic Cell Biology, Division of Rheumatology, Joseph Stokes, Jr Research Institute, Children s Hospital of Philadelphia, Philadelphia, PA 19104 4318, USA
    J Immunol 181:3018-26. 2008
    ..We conclude that the apoptotic cell receptor system on DCs is sufficiently redundant such that the absence of CR3 engagement does not significantly affect the normal anti-inflammatory processing of apoptotic cells...
  29. ncbi request reprint Prenatal glucocorticoids and exogenous surfactant therapy improve respiratory function in lambs with severe diaphragmatic hernia following fetal tracheal occlusion
    Marcus G Davey
    The Children s Institute for Surgical Science and the Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, PA 19104, USA
    Pediatr Res 60:131-5. 2006
    ..GC or surfactant therapy alone significantly improved respiratory gas exchange, Cl, and ventilatory efficiency index. Total lung capacity was normalized only in DH/TO lambs that received both GC and S...
  30. ncbi request reprint Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion
    R Douglas Wilson
    The Center for Fetal Diagnosis and Treatment, Department of Surgery at The Children s Hospital of Philadelphia University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4399, USA
    Prenat Diagn 26:1058-61. 2006
    ..Autopsy-identified CPL...
  31. ncbi request reprint Does a myelomeningocele sac compared to no sac result in decreased postnatal leg function following maternal fetal surgery for spina bifida aperta?
    R Douglas Wilson
    Department of Surgery and Obstetrics and Gynecology, Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4399, USA
    Fetal Diagn Ther 22:348-51. 2007
    ..A fetus with large sac S1 myelomeningocele (MMC) but bilateral talipes prompted the question, 'Does the presence or size of an MMC sac affect postnatal leg function?'..
  32. pmc Maternal alloantibodies induce a postnatal immune response that limits engraftment following in utero hematopoietic cell transplantation in mice
    Demetri J Merianos
    Children s Center for Fetal Research, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    J Clin Invest 119:2590-600. 2009
    ..This study resolves the apparent contradiction of induction of an adaptive immune response in the pre-immune fetus and confirms the potential of actively acquired tolerance to facilitate prenatal therapeutic applications...
  33. ncbi request reprint Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival
    William H Peranteau
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Fetal Diagn Ther 22:365-71. 2007
    ..To evaluate the effect of prenatal steroid treatment on the growth of congenital cystic adenomatoid malformations (CCAM) and survival in affected fetuses not amenable to other percutaneous ultrasound-guided prenatal interventions...
  34. doi request reprint In utero meconium passage in fetuses and newborns with myelomeningocele
    Enrico Danzer
    The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, and the University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4318, USA
    J Neurosurg Pediatr 3:141-6. 2009
    ..The authors retrospectively investigated whether midgestational fetal myelomeningocele (fMMC) repair alters intrauterine meconium exposure...
  35. doi request reprint Repair of indirect inguinal hernias by "laparoscopic joining of the edges"
    Pablo Laje
    Department of Pediatric Surgery, Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Surg Laparosc Endosc Percutan Tech 18:280-2. 2008
    ..To evaluate a new laparoscopic technique for the treatment of indirect inguinal hernias...
  36. doi request reprint Stem cell and genetic therapies for the fetus
    Jessica L Roybal
    Children s Center for Fetal Research, Children s Hospital of Philadelphia, PA, USA
    Semin Fetal Neonatal Med 15:46-51. 2010
    ..Here we review the current status and future potential of prenatal cellular and genetic therapy...
  37. doi request reprint In utero hematopoietic stem cell transplantation: progress toward clinical application
    Demetri Merianos
    Children s Center for Fetal Research, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Biol Blood Marrow Transplant 14:729-40. 2008
    ..Because donor specific tolerance induction requires relatively minimal engraftment, this strategy may hold the key to broad clinical application of IUHCT in the near future...
  38. ncbi request reprint Pulmonary vascular malformations
    Kenneth W Liechty
    Center for Fetal Research, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Semin Pediatr Surg 17:9-16. 2008
    ..This paper will review the components of these lesions, their associated anomalies, the diagnosis and natural history, and finally, current concepts in the management of pulmonary vascular malformations...
  39. ncbi request reprint Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment
    R Douglas Wilson
    Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4399, USA
    Am J Med Genet A 140:151-5. 2006
    ..In the appropriate situation, this maternal fetal surgery approach for CCAM is life-saving for the affected fetus with acceptable maternal morbidity risks in the present and future pregnancies...
  40. ncbi request reprint AAV-mediated factor IX gene transfer to skeletal muscle in patients with severe hemophilia B
    Catherine S Manno
    Department of Pediatrics, University of Pennsylvania and The Children s Hospital of Philadelphia, PA, 19104, USA
    Blood 101:2963-72. 2003
    ....
  41. ncbi request reprint Prenatal glucocorticoids improve lung morphology and partially restores surfactant mRNA expression in lambs with diaphragmatic hernia undergoing fetal tracheal occlusion
    Marcus G Davey
    The Children s Institute for Surgical Science and the Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Pediatr Pulmonol 41:1188-96. 2006
    ..We hypothesize that a GC-induced reduction in lung liquid volume during TO contributes, in part, to the observed increase in AE2 cell density and SPmRNA expression...
  42. ncbi request reprint Temporary tracheal occlusion in fetal sheep with lung hypoplasia does not improve postnatal lung function
    Marcus G Davey
    Children s Institute for Surgical Science and Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104 4399, USA
    J Appl Physiol 94:1054-62. 2003
    ..Lung compliance remained low in DH/TO+R lambs, most likely as a consequence of the persistent increase in alveolar wall thickness in these animals...
  43. ncbi request reprint High-level allogeneic chimerism achieved by prenatal tolerance induction and postnatal nonmyeloablative bone marrow transplantation
    William H Peranteau
    Children s Institute for Surgical Science, The Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Blood 100:2225-34. 2002
    ..This study supports the concept of prenatal tolerance induction to facilitate nonmyeloablative postnatal strategies for cellular therapy. If clinically applicable, such an approach could dramatically expand the application of IUHSCT...
  44. ncbi request reprint Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations
    R Douglas Wilson
    Department of Surgery, Center for Fetal Diagnosis and Treatment at the Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA 19104 4399, USA
    Fetal Diagn Ther 19:413-20. 2004
    ....
  45. ncbi request reprint Right congenital diaphragmatic hernia: Prenatal assessment and outcome
    Holly L Hedrick
    Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104 4399, USA
    J Pediatr Surg 39:319-23; discussion 319-23. 2004
    ..To understand the natural history of right congenital diaphragmatic hernia (CDH), the authors retrospectively reviewed 27 cases of right CDH that presented for prenatal evaluation or postnatal treatment...
  46. ncbi request reprint Fetal myelomeningocele repair: short-term clinical outcomes
    Mark P Johnson
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadephia, PA 19104, USA
    Am J Obstet Gynecol 189:482-7. 2003
    ..The study was undertaken to evaluate short-term clinical outcomes with antenatal myelomeningocele (MMC) repair...
  47. ncbi request reprint Acute cardiovascular effects of fetal surgery in the human
    Jack Rychik
    Center for Fetal Diagnosis and Treatment and the Fetal Heart Program, The Children s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104, USA
    Circulation 110:1549-56. 2004
    ..The indications for fetal surgery continue to expand, yet little is known about the acute sequelae of fetal surgery on the human cardiovascular system...
  48. ncbi request reprint Surfactant protein expression is increased in the ipsilateral but not contralateral lungs of fetal sheep with left-sided diaphragmatic hernia
    Marcus G Davey
    Children s Institute for Surgical Science and Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, Abramson 1112 D, 3615 Civic Center Blvd, Philadelphia, PA 19104, USA
    Pediatr Pulmonol 39:359-67. 2005
    ..Furthermore, AE2 cell density is increased in DH, suggesting that the increase in SP mRNA and protein levels is due to increases AE2 cell number. Our data further support the premise that fetal lung hypoplasia favors an AE2 phenotype...
  49. ncbi request reprint The ex utero intrapartum therapy procedure for high-risk fetal lung lesions
    Holly L Hedrick
    The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104 4399, USA
    J Pediatr Surg 40:1038-43; discussion 1044. 2005
    ..Indications for the ex utero intrapartum therapy (EXIT) procedure have expanded to include any fetal anomaly in which resuscitation of the neonate may be compromised...
  50. pmc A randomized trial of prenatal versus postnatal repair of myelomeningocele
    N Scott Adzick
    Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    N Engl J Med 364:993-1004. 2011
    ..Prenatal repair of myelomeningocele, the most common form of spina bifida, may result in better neurologic function than repair deferred until after delivery. We compared outcomes of in utero repair with standard postnatal repair...
  51. doi request reprint Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions
    ANTHONY Y TSAI
    The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 43:513-7. 2008
    ..On the other hand, malignant degeneration, pneumonia, and pneumothorax are known consequences of cystic lung lesions. This study aims to assess the safety of resection for asymptomatic lung lesions that were diagnosed before birth...
  52. doi request reprint Main bronchus occlusion for treatment of congenital diaphragmatic hernia in fetal lambs
    Jean Marc Biard
    The Center for Fetal Diagnosis and Treatment at the Children s Hospital of Philadelphia, PA 19104 4399, USA
    J Pediatr Surg 43:620-6. 2008
    ....
  53. doi request reprint Cystic adenomatoid malformations are induced by localized FGF10 overexpression in fetal rat lung
    Silvia Gonzaga
    The Children s Center for Fetal Research, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104 4318, USA
    Am J Respir Cell Mol Biol 39:346-55. 2008
    ..These findings support a role for FGF10 in the induction of human CCAM and provide further mechanistic insight into the role of FGF10 in normal and abnormal lung development...
  54. ncbi request reprint Mixed chimerism following in utero hematopoietic stem cell transplantation in murine models of hemoglobinopathy
    Satoshi Hayashi
    The Childrens Institute for Surgical Science, Department of Surgery, The Children s Hospital of Philadelphia, 3516 Civic Center Boulevard, Philadelphia, PA 19104 4399, USA
    Exp Hematol 31:176-84. 2003
    ..To investigate the biology of IUHSCTx in hemoglobinopathies, we performed fully allogeneic IUHSCTx in murine models of beta-thalassemia (Thal) and sickle cell disease (SCD)...
  55. doi request reprint Reproductive outcomes in subsequent pregnancies after a pregnancy complicated by open maternal-fetal surgery (1996-2007)
    R Douglas Wilson
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Am J Obstet Gynecol 203:209.e1-6. 2010
    ..The reproductive and gynecologic outcomes for women after the pregnancy complicated by open maternal-fetal surgery (OMFS) were evaluated...
  56. pmc Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy
    Pablo Laje
    Department of General Surgery, The Children s Hospital of Philadelphia and The University of Pennsylvania Medical Center, Philadelphia, PA 19104, USA
    Blood 113:2172-80. 2009
    ..This success may provide the basis for development of a novel therapeutic strategy to cure hereditary TTP in humans...
  57. pmc Haploidentical in utero hematopoietic cell transplantation improves phenotype and can induce tolerance for postnatal same-donor transplants in the canine leukocyte adhesion deficiency model
    William H Peranteau
    Center for Fetal Research, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Biol Blood Marrow Transplant 15:293-305. 2009
    ....
  58. pmc Prenatal transplantation of cytokine-stimulated marrow improves early chimerism in a resistant strain combination but results in poor long-term engraftment
    Aimen F Shaaban
    Laboratory for Fetal Cellular Therapy, Department of Surgery, University of Wisconsin Medical School, Madison, WI 53792 7375, USA
    Exp Hematol 34:1278-87. 2006
    ..To evaluate this hypothesis, we stimulated the donor cells with vascular endothelial growth factor (VEGF) and stem cell factor (SCF) prior to IUHCT in a chimerism-resistant murine strain combination...
  59. pmc CD26 inhibition enhances allogeneic donor-cell homing and engraftment after in utero hematopoietic-cell transplantation
    William H Peranteau
    The Center for Fetal Research, Children s Hospital of Philadelphia, Abramson Research Bldg, Rm 1116B, 3615 Civic Center Blvd, Philadelphia, PA 19104 4318, USA
    Blood 108:4268-74. 2006
    ..The resulting increased donor chimerism suggests that CD26 inhibition may in the future be used as a method of increasing donor-specific tolerance following IUHCT...
  60. ncbi request reprint Structural and functional characterization of bladder smooth muscle in fetal rats with retinoic acid-induced myelomeningocele
    Enrico Danzer
    The Center for Fetal Research, The Children s Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 4318, USA
    Am J Physiol Renal Physiol 292:F197-206. 2007
    ....
  61. pmc Evidence for an immune barrier after in utero hematopoietic-cell transplantation
    William H Peranteau
    Center for Fetal Research, Children s Hospital of Philadelphia, PA 19104, USA
    Blood 109:1331-3. 2007
    ..In contrast, all congenic recipients maintain stable, long-term, multilineage chimerism. These results strongly support an immune barrier to allogeneic engraftment after IUHCT...
  62. ncbi request reprint Diagnosis and characterization of fetal sacrococcygeal teratoma with prenatal MRI
    Enrico Danzer
    The Center for Fetal Diagnosis and Treatment, Department of Radiology, The Children s Hospital of Philadelphia, Philadelphia, PA 19104 4399, USA
    AJR Am J Roentgenol 187:W350-6. 2006
    ..The purpose of this study was to determine whether prenatal MRI provides additional information about fetal sacrococcygeal teratoma compared with prenatal sonography...
  63. ncbi request reprint Fetal liver as a source of autologous progenitor cells for perinatal tissue engineering
    Alexander S Krupnick
    Harrison s Department of Surgical Research, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, USA
    Tissue Eng 10:723-35. 2004
    ..We have demonstrated that mesenchymal progenitor cells with multilineage potential can be isolated from the fetal liver and have potential utility for autologous tissue engineering...
  64. ncbi request reprint Targeted gene transfer to fetal rat lung interstitium by ultrasound-guided intrapulmonary injection
    Tiago Henriques-Coelho
    The Center for Fetal Research, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Mol Ther 15:340-7. 2007
    ..This model system may facilitate in vivo studies of dynamic lung morphogenesis and could provide insight into the efficacy of prenatal gene transfer strategies for treatment of specific lung disorders...
  65. ncbi request reprint Gene transfer to ocular stem cells by early gestational intraamniotic injection of lentiviral vector
    Masayuki Endo
    The Children s Center for Fetal Research, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Mol Ther 15:579-87. 2007
    ..This model may be useful for the investigation of mechanisms of genetic and/or developmental ocular disease and for the development of prenatal gene therapy for specific ocular disorders...
  66. ncbi request reprint Long-term transgene expression in cardiac and skeletal muscle following fetal administration of adenoviral or adeno-associated viral vectors in mice
    Sarah Bouchard
    Division of Pediatric General, Thoracic, and Fetal Surgery, The Children s Institute for Surgical Science, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    J Gene Med 5:941-50. 2003
    ....
  67. ncbi request reprint Postnatal chest wall deformities after fetal thoracoamniotic shunting for congenital cystic adenomatoid malformation
    Aziz M Merchant
    Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, and Department of Surgery and Obstetrics Gynecology, University of Pennsylvania, Philadelphia, PA 19104 4399, USA
    Fetal Diagn Ther 22:435-9. 2007
    ..Large macrocystic congenital cystic adenomatoid malformations (CCAMs) can be treated with thoracoamniotic (TA) shunting to reduce CCAM volume. Two CCAM fetuses treated with TA shunt had postnatal radiographic rib deformities...
  68. doi request reprint Intradural inclusion cysts following in utero closure of myelomeningocele: clinical implications and follow-up findings
    Enrico Danzer
    The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Neurosurg Pediatr 2:406-13. 2008
    ..The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure...
  69. ncbi request reprint Preliminary investigations into a new method of functional assessment of the fetal heart using a novel application of 'real-time' cardiac magnetic resonance imaging
    Mark A Fogel
    Division of Cardiology, Department of Pediatrics, University of Pennsylvania School of Medicine, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Fetal Diagn Ther 20:475-80. 2005
    ..Because of quantitative echocardiographic limitations of fetal ventricular volumes as well as poor windows, we sought to determine if real-time magnetic resonance imaging (MRI) could be used...
  70. doi request reprint A tissue engineering approach for prenatal closure of myelomeningocele: comparison of gelatin sponge and microsphere scaffolds and bioactive protein coatings
    Miho Watanabe
    Department of Surgery and Children s Center for Fetal Research, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104 4318, USA
    Tissue Eng Part A 17:1099-110. 2011
    ..These findings support the potential for the development of a tissue-engineered injectable scaffold that could be applied by ultrasound-guided injection, much earlier and less invasively than sponge or sheet-based composites...
  71. ncbi request reprint The natural history of prenatally diagnosed conjoined twins
    Tippi C MacKenzie
    Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    J Pediatr Surg 37:303-9. 2002
    ..To assess the impact of improved antenatal diagnosis in the management and outcome of conjoined twins, the authors reviewed their experience with 14 cases...
  72. ncbi request reprint Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung
    Timothy M Crombleholme
    Division of General, The Center for Fetal Diagnosis and Treatment at the Children s Hospital of Philadelphia, USA
    J Pediatr Surg 37:331-8. 2002
    ..The authors now report the results of prospective use of the CVR both to track tumor growth and regression during gestation and confirm its predictive value in fetuses with CAM...
  73. ncbi request reprint Influence of congenital heart disease on survival in children with congenital diaphragmatic hernia
    Meryl S Cohen
    Cardiac Center and The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    J Pediatr 141:25-30. 2002
    ..We sought to assess outcome in patients with CDH and HD to determine if LHR is also predictive of outcome in this subset of patients...
  74. ncbi request reprint Antenatal vitamin A administration attenuates lung hypoplasia by interfering with early instead of late determinants of lung underdevelopment in congenital diaphragmatic hernia
    Maria J Baptista
    Development Unit, Health and Life Research Institute, Health Sciences School, University of Minho, 4710 057 Braga, Portugal
    J Pediatr Surg 40:658-65. 2005
    ..The authors investigated if vitamin A enhances lung growth because of effects on both early and late determinants of lung hypoplasia...
  75. ncbi request reprint Complete allogeneic hematopoietic chimerism achieved by a combined strategy of in utero hematopoietic stem cell transplantation and postnatal donor lymphocyte infusion
    Satoshi Hayashi
    Children s Institute for Surgical Science, The Children s Hospital of Philadelphia, PA 19104 4399, USA
    Blood 100:804-12. 2002
    ....
  76. ncbi request reprint Fetal heart development in the nitrofen-induced CDH rat model: the role of mechanical and nonmechanical factors
    Jorge Correia-Pinto
    Department of Physiology, Faculty of Medicine, University of Porto, Porto, Portugal
    J Pediatr Surg 38:1444-51. 2003
    ..The authors analyzed whether nonmechanical and mechanical factors affect cardiac growth and correlations between lung and heart weights during gestation...
  77. ncbi request reprint Fetal therapy
    Mark I Evans
    Columbia University College of Physicians and Surgeons, St Luke s Roosevelt Hospital Center, 1000 10th Avenue, New York, NY 10019, USA
    Best Pract Res Clin Obstet Gynaecol 16:671-83. 2002
    ....
  78. ncbi request reprint Gastroschisis: growth patterns and a proposed prenatal surveillance protocol
    Denise A Netta
    Division of Maternal Fetal Medicine, Department of Obstetrics, Gynecology, and Reproductive Sciences, University of Medicine and Dentistry of New Jersey Robert Wood Johnson Medical School, New Brunswick, NJ, USA
    Fetal Diagn Ther 22:352-7. 2007
    ..The growth assessment could be available as a prospective tool to direct an antepartum fetal surveillance protocol...
  79. ncbi request reprint Stem cell and genetic therapies for the fetus
    Alan W Flake
    The Center for Fetal Diagnosis and Treatment, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Semin Pediatr Surg 12:202-8. 2003
    ..Although clinical application is currently limited to a few highly selected disorders that are amenable to cellular therapy, there is reason to believe that a dramatic increase in application will occur in the near future...
  80. ncbi request reprint Engraftment of bone marrow and fetal liver cells after in utero transplantation in MDX mice
    Tippi C MacKenzie
    Children s Institute for Surgical Science, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 37:1058-64. 2002
    ....

Research Grants18

  1. Fetal Biology and Therapy Training Program
    Alan Flake; Fiscal Year: 2007
    ..Penn Graduate School and IHGT. This request is made for 2 Trainee slots in year 1, 3 Trainee slots in year 2, and 4 Trainee slots each year thereafter. ..
  2. Strategies for prenatal stem cell transplantation
    Alan Flake; Fiscal Year: 2007
    ..The ultimate goal of these studies is to develop safe and effective strategies that can be translated to the in utero treatment of a broad range of human genetic hematologic disorders including Sickle Cell Disease and Thalassemia. ..
  3. Fetal Stromal Progenitor Cells
    Alan Flake; Fiscal Year: 2006
    ..abstract_text> ..
  4. Strategies for Prenatal Stem Cell Tranplantation
    Alan Flake; Fiscal Year: 2004
    ..The studies in this proposal are designed to identify and test strategies to increase engraftment after IUHSCTx. This would allow application of this approach to a broad range of prenatally diagnosed genetic disorders. ..
  5. PRENATAL STRATEGIES FOR TREATMENT OF HEMOGLOBINOPATHIES
    Alan Flake; Fiscal Year: 2002
    ..abstract_text> ..
  6. Strategies for prenatal stem cell transplantation
    Alan W Flake; Fiscal Year: 2010
    ..The ultimate goal of these studies is to develop safe and effective strategies that can be translated to the in utero treatment of a broad range of human genetic hematologic disorders including Sickle Cell Disease and Thalassemia. ..