Lisa R Young
Division of Pulmonary and Sleep Medicine, The Children’s Hospital of Philadelphia, Perlman School of Medicine at the University of Pennsylvania, 19104, Philadelphia, PA, USA | ...
KOL Resume for Lisa R Young
Division of Pulmonary and Sleep Medicine, The Children’s Hospital of Philadelphia, Perlman School of Medicine at the University of Pennsylvania, 19104, Philadelphia, PA, USA
Children's Hospital of Philadelphia, 6567, Philadelphia, Pennsylvania, United States.
Children's Hospital of Philadelphia Philadelphia, Pennsylvania and.
Department of Pediatrics, Division of Neonatology, Vanderbilt University Medical Center, Nashville, United States of America
Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
These authors contributed equally
Division of Pulmonary Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Children's Hospital of Philadelphia, 6567, Philadelphia, Pennsylvania, United States
University of Pennsylvania, Philadelphia, Pennsylvania, USA
Division of Pulmonary Medicine, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
Department of Cell and Developmental Biology, Vanderbilt University, Nashville, Tennessee
2 Division of Pediatric Pulmonary Medicine, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee.
Department of Medicine, Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee
Division of Pediatric Allergy, Immunology, and Pulmonary Medicine, Vanderbilt University School of Medicine, 2200 Children's Way, 11215 Doctors' Office Tower, Nashville, TN 37232-9500, USA
Division of Pulmonary Medicine, Department of Pediatrics, Vanderbilt University School of Medicine, 2200 Children’s Way, 37232-9500, Nashville, TN, USA
1 Vanderbilt University School of Medicine Nashville, Tennessee.
Division of Pediatric Pulmonary Medicine, Monroe Carell Jr Children's Hospital at Vanderbilt, Division of Allergy, Pulmonary, and Critical Care, Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA
Division of Allergy, Immunology, and Pulmonary Medicine, Department of Pediatrics,
Allergy, Pulmonary and Critical Care, Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee; and
Division of Pediatric Pulmonary Medicine, Department of Pediatrics, Vanderbilt University, Nashville, Tennessee
Division of Allergy, Immunology, and Pulmonary Medicine, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN, USA
From the University of Cincinnati (F.X.M., L.R.Y., B.W.K., R.T.M., B.C.T.) and Cincinnati Children's Hospital Medical Center (L.R.Y., L.K., B.C.T.) — both in Cincinnati
Division of Pulmonary Medicine, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH
Children's Interstitial Lung Disease Research Network (CHILDRN), .
Departments of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Division of Pulmonary Medicine, Cincinnati Children’s Hospital, Agricultural Research Service, Washington State University, Pullman, Washington
Division of Pulmonary and Critical Care, University of Cincinnati College of Medicine (F.X.M., L.R.Y., J.M.E.) — both in Cincinnati.
From Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH.
University of Cincinnati, Pulmonary, Critical Care, and Sleep Medicine, 231 Albert Sabin Way, Cincinnati, OH 45267, USA.
Lisa R Young: Influence Statistics
|mild radiologic abnormalities||#1|
|rare mortality absence||#1|
|hyperplasia child child||#1|
|suspected ild studies||#1|
|hpspf lung transplantation||#1|
|hps4 hps pulmonary||#1|
|hps pulmonary hps||#1|
|histologic resolution pig||#1|
|durable cell lines||#1|
|directed therapies understanding||#1|
|translational review insights||#1|
|infancy clinical score||#1|
|time unexplained exacerbation||#1|
|disorders lung development||#1|
|guideline diagnostic approach||#1|
|pnecs progenitor cells||#1|
|transient proliferative capacity||#1|
|child conditions basis||#1|
|hpspf hispanic americans||#1|
|frustrating parents clinicians||#1|
|agents lung fibrosis||#1|
|lung sensory organ||#1|
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Prominent publications by Lisa R Young
Sirolimus for Angiomyolipoma in Tuberous Sclerosis Complex or Lymphangioleiomyomatosis
[ PUBLICATION ]
BACKGROUND: Angiomyolipomas in patients with the tuberous sclerosis complex or sporadic lymphangioleiomyomatosis are associated with mutations in tuberous sclerosis genes resulting in constitutive activation of the mammalian target of rapamycin (mTOR). The drug sirolimus suppresses mTOR signaling.
METHODS: We conducted a 24-month, nonrandomized, open-label trial to determine whether sirolimus reduces the angiomyolipoma volume in patients with the tuberous sclerosis complex or sporadic ...
|Known for Tuberous Sclerosis | Patients Sirolimus | Angiomyolipoma Volume | 24 Months | Brain Lesions|
Efficacy and Safety of Sirolimus in Lymphangioleiomyomatosis
[ PUBLICATION ]
BACKGROUND: Lymphangioleiomyomatosis (LAM) is a progressive, cystic lung disease in women; it is associated with inappropriate activation of mammalian target of rapamycin (mTOR) signaling, which regulates cellular growth and lymphangiogenesis. Sirolimus (also called rapamycin) inhibits mTOR and has shown promise in phase 1-2 trials involving patients with LAM.
METHODS: We conducted a two-stage trial of sirolimus involving 89 patients with LAM who had moderate lung impairment--a 12-month ...
|Known for Lam Sirolimus | Quality Life | Treatment Period | Forced Expiratory Volume | Lung Function|
Serum Vascular Endothelial Growth Factor-D Prospectively Distinguishes Lymphangioleiomyomatosis From Other Diseases
[ PUBLICATION ]
OBJECTIVES: The majority of women with lymphangioleiomyomatosis (LAM) present with cystic lung disease, and most require lung biopsy for definitive diagnosis. The purpose of this study was to determine the prospective diagnostic usefulness of a serologic test for vascular endothelial growth factor-D (VEGF-D), a lymphangiogenic growth factor.
METHODS: We prospectively measured serum VEGF-D levels by enzyme-linked immunoassay in 48 women presenting with cystic lung disease. Diagnostic test ...
|Known for Lam Women | Growth Factor | Vascular Endothelial | Cystic Lung Disease | Serum Vegfd Levels|
OBJECTIVE: Neuroendocrine cell hyperplasia of infancy is a form of childhood interstitial lung disease originally reported as persistent tachypnea of infancy. Reports of small series of cases and anecdotal experience have suggested that this disorder may have a consistent CT pattern. The purpose of this study was to review the CT findings in children with neuroendocrine cell hyperplasia of infancy to determine the findings at high-resolution CT, the diagnostic accuracy of CT compared ...
|Known for Neuroendocrine Cell Hyperplasia | Infancy Diagnosis | Findings Children | Interstitial Lung | Persistent Tachypnea|
Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme
[ PUBLICATION ]
RATIONALE: Children's Interstitial and Diffuse Lung Disease (chILD) is a heterogeneous group of disorders that is challenging to categorize. In previous study, a classification scheme was successfully applied to children 0 to 2 years of age who underwent lung biopsies for chILD. This classification scheme has not been evaluated in children 2 to 18 years of age.
OBJECTIVES: This multicenter interdisciplinary study sought to describe the spectrum of biopsy-proven chILD in North America and ...
|Known for Diffuse Lung Disease | Lung Biopsies | 18 Years | Child Classification | Pulmonary Hypertension|
RATIONALE: Up to 20% of cases of idiopathic interstitial pneumonia cluster in families, comprising the syndrome of familial interstitial pneumonia (FIP); however, the genetic basis of FIP remains uncertain in most families.
OBJECTIVES: To determine if new disease-causing rare genetic variants could be identified using whole-exome sequencing of affected members from FIP families, providing additional insights into disease pathogenesis.
METHODS: Affected subjects from 25 kindreds were ...
|Known for Rare Variants | Familial Interstitial Pneumonia | Short Telomeres | Fip Families | Genetic Basis|
Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy
[ PUBLICATION ]
Mutations in the LRBA gene (encoding the lipopolysaccharide-responsive and beige-like anchor protein) cause a syndrome of autoimmunity, lymphoproliferation, and humoral immune deficiency. The biological role of LRBA in immunologic disease is unknown. We found that patients with LRBA deficiency manifested a dramatic and sustained improvement in response to abatacept, a CTLA4 (cytotoxic T lymphocyte antigen-4)-immunoglobulin fusion drug. Clinical responses and homology of LRBA to proteins ...
|Known for Lrba Deficiency | Immune Dysregulation | Lung Diseases | Ctla4 Trafficking | Abatacept Therapy|
Management of Pneumothorax in Lymphangioleiomyomatosis Effects on Recurrence and Lung Transplantation Complications
[ PUBLICATION ]
STUDY OBJECTIVES: Pneumothorax is a common complication of lymphangioleiomyomatosis (LAM), and the optimal approach to its treatment and prevention is unknown. Chemical or surgical pleurodesis are often required to prevent recurrence. However, their efficacy in LAM is unclear, and whether they contribute to perioperative complications during lung transplantation is uncertain.
SETTING: The LAM Foundation database of registered patients.
DESIGN: A questionnaire was sent to all registered ...
|Known for Lung Transplantation | Lam Patients | Recurrence Pneumothorax | Chemical Pleurodesis | Common Complication|
Serum VEGF-D concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Siro
[ PUBLICATION ]
BACKGROUND: VEGF-D is a lymphangiogenic growth factor that has a key role in tumour metastasis. Serum VEGF-D concentrations are increased in most patients with lymphangioleiomyomatosis, a rare neoplasm associated with mTOR-activating tuberous sclerosis gene mutations, lymphadenopathy, metastatic spread, and pulmonary cyst formation. We used data from the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial to assess the usefulness of serum VEGF-D ...
|Known for Serum Vegf | Patients Lymphangioleiomyomatosis | Sirolimus Placebo | Expiratory Volume | Miles Trial|
Localized hypoxia links ER stress to lung fibrosis through induction of C/EBP homologous protein
[ PUBLICATION ]
ER stress in type II alveolar epithelial cells (AECs) is common in idiopathic pulmonary fibrosis (IPF), but the contribution of ER stress to lung fibrosis is poorly understood. We found that mice deficient in C/EBP homologous protein (CHOP), an ER stress-regulated transcription factor, were protected from lung fibrosis and AEC apoptosis in 3 separate models where substantial ER stress was identified. In mice treated with repetitive intratracheal bleomycin, we identified localized hypoxia ...
|Known for Lung Fibrosis | Hypoxia Stress | Homologous Protein | Cultured Disease Models | Endoplasmic Reticulum|
Diffuse Lung Disease in Young Children
[ PUBLICATION ]
RATIONALE: Considerable confusion exists regarding nomenclature, classification, and management of pediatric diffuse lung diseases due to the relative rarity and differences in the spectrum of disease between adults and young children.
OBJECTIVES: A multidisciplinary working group was formed to: (1) apply consensus terminology and diagnostic criteria for disorders presenting with diffuse lung disease in infancy; and (2) describe the distribution of disease entities, clinical features, ...
|Known for Young Children | Diffuse Lung Disease | Lung Biopsy | Disorders Infancy | Neuroendocrine Cell Hyperplasia|
INTRODUCTION: A high prevalence of gastroesophageal reflux (GER) has been reported in lung transplant recipients and is possibly linked to the development of bronchiolitis obliterans syndrome. The etiology of posttransplant GER remains unknown but may occur due to the transplant operation or posttransplant medications, or represent preexisting GER disease. We evaluated these possibilities by studying the nature and severity of GER in a cohort of patients before and after lung ...
|Known for Lung Transplantation | Gastroesophageal Reflux | Gastric Motility | Esophageal Manometry | Abnormal Acid Contact|
Heterogeneous Pulmonary Phenotypes Associated With Mutations in the Thyroid Transcription Factor Gene NKX2-1
[ PUBLICATION ]
BACKGROUND: Mutations in the gene encoding thyroid transcription factor, NKX2-1, result in neurologic abnormalities, hypothyroidism, and neonatal respiratory distress syndrome (RDS) that together are known as the brain-thyroid-lung syndrome. To characterize the spectrum of associated pulmonary phenotypes, we identified individuals with mutations in NKX2-1 whose primary manifestation was respiratory disease.
METHODS: Retrospective and prospective approaches identified infants and children ...
|Known for Mutations Nkx2 | Factor Gene | Pulmonary Phenotype | Lung Disease | Thyroid Nuclear|
Idiopathic pulmonary fibrosis (IPF) is a progressive and often fatal lung disease for which there is no known treatment. Although the traditional paradigm of IPF pathogenesis emphasized chronic inflammation as the primary driver of fibrotic remodeling, more recent insights have challenged this view. Linkage analysis and candidate gene approaches have identified four genes that cause the inherited form of IPF, familial interstitial pneumonia (FIP). These four genes encode two surfactant ...
|Known for Idiopathic Pulmonary | Surfactant Protein | Genetic Studies | Lung Fibrosis | Pathogenesis Ipf|
An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy
[ PUBLICATION ]
BACKGROUND: There is growing recognition and understanding of the entities that cause interstitial lung disease (ILD) in infants. These entities are distinct from those that cause ILD in older children and adults.
METHODS: A multidisciplinary panel was convened to develop evidence-based guidelines on the classification, diagnosis, and management of ILD in children, focusing on neonates and infants under 2 years of age. Recommendations were formulated using a systematic approach. Outcomes ...
|Known for Childhood Interstitial | Ild Infants | Evaluation Management | Lung Diseases | Topic Societies|
Key People For Pulmonary Fibrosis
Lisa R Young:Expert Impact
Concepts for whichLisa R Younghas direct influence:Pulmonary fibrosis, Lung disease, Neuroendocrine cell hyperplasia, Lung diseases, Pudlak syndrome, Diffuse lung disease, Β1 integrin, Trial participation.
Lisa R Young:KOL impact
Concepts related to the work of other authors for whichfor which Lisa R Young has influence:Tuberous sclerosis, Pulmonary fibrosis, Lung transplantation, Pseudomonas aeruginosa, Mammalian target, Lymphangioleiomyomatosis lam, Lrba deficiency.
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