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  1. Article ; Online: GJ Express: an improvement initiative to decrease sedation and anesthesia for gastrojejunostomy tube exchanges.

    Cheng, Qianqian Ellie / Schriefer, Jan / Sosa, Tina / Haen, Sarah / Ferguson, Samantha J / Clark, Alexander / Boerman, Christine / Hochreiter, Carly / Gabel, Megan E / Yung, Arvid / Lee, David E / Ackerman, Kate G

    Pediatric research

    2024  

    Abstract: Background: Overuse of sedation and anesthesia causes delays in gastrojejunostomy tube (GJ) exchanges, increased risk of complications, unnecessary use of resources, preventable hospital admissions, and an adverse impact on patient and family experience. ...

    Abstract Background: Overuse of sedation and anesthesia causes delays in gastrojejunostomy tube (GJ) exchanges, increased risk of complications, unnecessary use of resources, preventable hospital admissions, and an adverse impact on patient and family experience. Our hospital was over-utilizing sedation and anesthesia, and we aimed to decrease this use from 78% to 20% within two years.
    Methods: An interdisciplinary quality improvement team comprehensively evaluated current processes for GJ tube exchanges through a retrospective chart review for baseline data with prospective time series analysis after improvement implementation. The primary outcome measure was the percentage of pediatric patients that utilized sedation or anesthesia for routine GJ tube exchanges.
    Results: A statistical process control p-chart was used to calculate and show changes over time for patients (n = 45 patients average). The median percent of pediatric GJ tube exchanges performed with sedation or anesthesia decreased from 77.8% to 11.3%. Most patients (76%) were covered by Medicaid programs; with low reimbursement rates, decreased anesthesiologist billing revenue does not have a negative financial impact.
    Conclusions: An interprofessional improvement initiative that engaged patients and families, incorporated pediatric-specific staff services, and developed systematic weaning was associated with a significant decrease in the overuse of sedation and anesthesia for GJ tube exchanges.
    Impact: We believe that this work is highly relevant and impactful for medical centers caring for children who require gastrojejunostomy tubes, an increasingly common approach to management of children with feeding issues. There is very little literature available on the use of sedation or anesthesia for changing these tubes. While large children's medical centers in the USA usually do not utilize sedation or anesthesia, there are likely many serious outliers, especially when children receive care outside of a pediatric specific institution. This paper brings awareness to this serious issue and provides information about how we changed care to achieve higher patient safety and lower medical costs.
    Language English
    Publishing date 2024-02-22
    Publishing country United States
    Document type Journal Article
    ZDB-ID 4411-8
    ISSN 1530-0447 ; 0031-3998
    ISSN (online) 1530-0447
    ISSN 0031-3998
    DOI 10.1038/s41390-024-03070-1
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Kif7 is required for the patterning and differentiation of the diaphragm in a model of syndromic congenital diaphragmatic hernia.

    Coles, Garry L / Ackerman, Kate G

    Proceedings of the National Academy of Sciences of the United States of America

    2013  Volume 110, Issue 21, Page(s) E1898–905

    Abstract: Congenital diaphragmatic hernia (CDH) is a common birth defect that results in a high degree of neonatal morbidity and mortality, but its pathological mechanisms are largely unknown. Therefore, we performed a forward genetic screen in mice to identify ... ...

    Abstract Congenital diaphragmatic hernia (CDH) is a common birth defect that results in a high degree of neonatal morbidity and mortality, but its pathological mechanisms are largely unknown. Therefore, we performed a forward genetic screen in mice to identify unique genes, models, and mechanisms of abnormal diaphragm development. We identified a mutant allele of kinesin family member 7 (Kif7), the disorganized diaphragm (dd). Embryos homozygous for the dd allele possess communicating diaphragmatic hernias, central tendon patterning defects, and increased cell proliferation with diaphragmatic tissue hyperplasia. Because the patterning of the central tendon is undescribed, we analyzed the expression of genes regulating tendonogenesis in dd/dd mutant embryos, and we determined that retinoic acid (RA) signaling was misregulautted. To further investigate the role of Kif7 and RA signaling in the development of the embryonic diaphragm, we established primary mesenchymal cultures of WT embryonic day 13.5 diaphragmatic cells. We determined that RA signaling is necessary for the expression of tendon markers as well as the expression of other CDH-associated genes. Knockdown of Kif7, and retinoic acid receptors alpha (Rara), beta (Rarb), and gamma (Rarg) indicated that RA signaling is dependent on these genes to promote tendonogenesis within the embryonic diaphragm. Taken together, our results provide evidence for a model in which inhibition of RA receptor signaling promotes CDH pathogenesis through a complex gene network.
    MeSH term(s) Alleles ; Animals ; Body Patterning ; Cell Differentiation ; Cell Line ; Diaphragm/embryology ; Diaphragm/pathology ; Gene Expression Regulation, Developmental/genetics ; Hernia, Diaphragmatic/embryology ; Hernia, Diaphragmatic/genetics ; Hernia, Diaphragmatic/pathology ; Hernias, Diaphragmatic, Congenital ; Kinesins/genetics ; Kinesins/metabolism ; Mice ; Mice, Mutant Strains ; Muscle Proteins/genetics ; Muscle Proteins/metabolism ; Receptors, Retinoic Acid/genetics ; Receptors, Retinoic Acid/metabolism ; Retinoic Acid Receptor alpha ; Signal Transduction ; Tendons/embryology ; Tendons/pathology ; Tretinoin/metabolism ; Retinoic Acid Receptor gamma
    Chemical Substances Muscle Proteins ; Rara protein, mouse ; Receptors, Retinoic Acid ; Retinoic Acid Receptor alpha ; retinoic acid receptor beta ; Tretinoin (5688UTC01R) ; Kif7 protein, mouse (EC 3.6.1.-) ; Kinesins (EC 3.6.4.4)
    Language English
    Publishing date 2013-05-06
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 209104-5
    ISSN 1091-6490 ; 0027-8424
    ISSN (online) 1091-6490
    ISSN 0027-8424
    DOI 10.1073/pnas.1222797110
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Dissatisfaction with maintenance of certification in academic pediatrics.

    Ackerman, Kate G / Lee, Brendan / Kushner, Jake A

    Pediatric research

    2016  Volume 79, Issue 2, Page(s) 240–242

    MeSH term(s) Attitude of Health Personnel ; Certification/economics ; Certification/standards ; Clinical Competence/economics ; Clinical Competence/standards ; Cost-Benefit Analysis ; Health Knowledge, Attitudes, Practice ; Humans ; Job Satisfaction ; Pediatricians/economics ; Pediatricians/psychology ; Pediatricians/standards ; Pediatrics/economics ; Pediatrics/standards ; Perception
    Language English
    Publishing date 2016-02
    Publishing country United States
    Document type Editorial
    ZDB-ID 4411-8
    ISSN 1530-0447 ; 0031-3998
    ISSN (online) 1530-0447
    ISSN 0031-3998
    DOI 10.1038/pr.2015.189
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: KIF7 Controls the Proliferation of Cells of the Respiratory Airway through Distinct Microtubule Dependent Mechanisms.

    Coles, Garry L / Baglia, Laurel A / Ackerman, Kate G

    PLoS genetics

    2015  Volume 11, Issue 10, Page(s) e1005525

    Abstract: The cell cycle must be tightly coordinated for proper control of embryonic development and for the long-term maintenance of organs such as the lung. There is emerging evidence that Kinesin family member 7 (Kif7) promotes Hedgehog (Hh) signaling during ... ...

    Abstract The cell cycle must be tightly coordinated for proper control of embryonic development and for the long-term maintenance of organs such as the lung. There is emerging evidence that Kinesin family member 7 (Kif7) promotes Hedgehog (Hh) signaling during embryonic development, and its misregulation contributes to diseases such as ciliopathies and cancer. Kif7 encodes a microtubule interacting protein that controls Hh signaling through regulation of microtubule dynamics within the primary cilium. However, whether Kif7 has a function in nonciliated cells remains largely unknown. The role Kif7 plays in basic cell biological processes like cell proliferation or cell cycle progression also remains to be elucidated. Here, we show that Kif7 is required for coordination of the cell cycle, and inactivation of this gene leads to increased cell proliferation in vivo and in vitro. Immunostaining and transmission electron microscopy experiments show that Kif7dda/dda mutant lungs are hyperproliferative and exhibit reduced alveolar epithelial cell differentiation. KIF7 depleted C3H10T1/2 fibroblasts and Kif7dda/dda mutant mouse embryonic fibroblasts have increased growth rates at high cellular densities, suggesting that Kif7 may function as a general regulator of cellular proliferation. We ascertained that in G1, Kif7 and microtubule dynamics regulate the expression and activity of several components of the cell cycle machinery known to control entry into S phase. Our data suggest that Kif7 may function to regulate the maintenance of the respiratory airway architecture by controlling cellular density, cell proliferation, and cycle exit through its role as a microtubule associated protein.
    MeSH term(s) Animals ; Cell Proliferation/genetics ; Cilia/genetics ; Cilia/physiology ; Embryo, Mammalian ; Embryonic Development/genetics ; Fibroblasts/metabolism ; Hedgehog Proteins/genetics ; Kinesin/genetics ; Kinesin/metabolism ; Lung/growth & development ; Lung/metabolism ; Mice ; Microtubules/genetics ; Microtubules/metabolism ; Pulmonary Ventilation ; Signal Transduction/genetics
    Chemical Substances Hedgehog Proteins ; Kif7 protein, mouse (EC 3.6.1.-) ; Kinesin (EC 3.6.4.4)
    Language English
    Publishing date 2015-10-06
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 2186725-2
    ISSN 1553-7404 ; 1553-7390
    ISSN (online) 1553-7404
    ISSN 1553-7390
    DOI 10.1371/journal.pgen.1005525
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Wt1 and β-catenin cooperatively regulate diaphragm development in the mouse.

    Paris, Nicole D / Coles, Garry L / Ackerman, Kate G

    Developmental biology

    2015  Volume 407, Issue 1, Page(s) 40–56

    Abstract: The developing diaphragm consists of various differentiating cell types, many of which are not well characterized during organogenesis. One important but incompletely understood tissue, the diaphragmatic mesothelium, is distinctively present from early ... ...

    Abstract The developing diaphragm consists of various differentiating cell types, many of which are not well characterized during organogenesis. One important but incompletely understood tissue, the diaphragmatic mesothelium, is distinctively present from early stages of development. Congenital Diaphragmatic Hernia (CDH) occurs in humans when diaphragm tissue is lost during development, resulting in high morbidity and mortality postnatally. We utilized a Wilms Tumor 1 (Wt1) mutant mouse model to investigate the involvement of the mesothelium in normal diaphragm signaling and development. Additionally, we developed and characterized a Wt1(CreERT2)-driven β-catenin loss-of-function model of CDH after finding that canonical Wnt signaling and β-catenin are reduced in Wt1 mutant mesothelium. Mice with β-catenin loss or constitutive activation induced in the Wt1 lineage are only affected when tamoxifen injection occurs between E10.5 and E11.5, revealing a critical time-frame for Wt1/ β-catenin activity. Conditional β-catenin loss phenocopies the Wt1 mutant diaphragm defect, while constitutive activation of β-catenin on the Wt1 mutant background is sufficient to close the diaphragm defect. Proliferation and apoptosis are affected, but primarily these genetic manipulations appear to lead to a change in normal diaphragm differentiation. Our data suggest a fundamental role for mesothelial signaling in proper formation of the diaphragm.
    MeSH term(s) Animals ; Apoptosis ; Cell Differentiation ; Cell Proliferation ; Diaphragm/embryology ; Epithelial-Mesenchymal Transition ; Female ; Hernias, Diaphragmatic, Congenital/etiology ; Humans ; Mice ; Organ Specificity ; Repressor Proteins/physiology ; Signal Transduction ; beta Catenin/physiology
    Chemical Substances CTNNB1 protein, mouse ; Repressor Proteins ; WT1 protein, mouse ; beta Catenin
    Language English
    Publishing date 2015-11-01
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 1114-9
    ISSN 1095-564X ; 0012-1606
    ISSN (online) 1095-564X
    ISSN 0012-1606
    DOI 10.1016/j.ydbio.2015.08.009
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Siblings with lethal primary pulmonary hypoplasia and compound heterozygous variants in the

    Kiraly-Borri, Catherine / Jevon, Gareth / Ji, Weizhen / Jeffries, Lauren / Ricciardi, Jamie-Lee / Konstantino, Monica / Ackerman, Kate G / Lakhani, Saquib A

    Cold Spring Harbor molecular case studies

    2019  Volume 5, Issue 3

    Abstract: Variants in the mitochondrial alanyl-tRNA synthetase 2 ... ...

    Abstract Variants in the mitochondrial alanyl-tRNA synthetase 2 gene
    MeSH term(s) Abnormalities, Multiple/diagnostic imaging ; Abnormalities, Multiple/genetics ; Abnormalities, Multiple/pathology ; Alanine-tRNA Ligase/genetics ; Amino Acid Substitution ; Fatal Outcome ; Frameshift Mutation ; Genes, Recessive ; Heterozygote ; Humans ; Infant, Newborn ; Leukoencephalopathies/diagnosis ; Leukoencephalopathies/genetics ; Leukoencephalopathies/pathology ; Lung/abnormalities ; Lung/diagnostic imaging ; Lung/pathology ; Lung Diseases/diagnostic imaging ; Lung Diseases/genetics ; Lung Diseases/pathology ; Mitochondria/genetics ; Mitochondria/pathology ; Pedigree ; Phenotype ; Siblings ; Whole Exome Sequencing
    Chemical Substances AARS2 protein, human (EC 6.1.1.7) ; Alanine-tRNA Ligase (EC 6.1.1.7)
    Language English
    Publishing date 2019-06-03
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2835759-0
    ISSN 2373-2873 ; 2373-2873
    ISSN (online) 2373-2873
    ISSN 2373-2873
    DOI 10.1101/mcs.a003699
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Wt1 and β-catenin cooperatively regulate diaphragm development in the mouse

    Paris, Nicole D / Coles, Garry L / Ackerman, Kate G

    Developmental biology. 2015 Nov. 01, v. 407, no. 1

    2015  

    Abstract: The developing diaphragm consists of various differentiating cell types, many of which are not well characterized during organogenesis. One important but incompletely understood tissue, the diaphragmatic mesothelium, is distinctively present from early ... ...

    Abstract The developing diaphragm consists of various differentiating cell types, many of which are not well characterized during organogenesis. One important but incompletely understood tissue, the diaphragmatic mesothelium, is distinctively present from early stages of development. Congenital Diaphragmatic Hernia (CDH) occurs in humans when diaphragm tissue is lost during development, resulting in high morbidity and mortality postnatally. We utilized a Wilms Tumor 1 (Wt1) mutant mouse model to investigate the involvement of the mesothelium in normal diaphragm signaling and development. Additionally, we developed and characterized a Wt1CreERT2-driven β-catenin loss-of-function model of CDH after finding that canonical Wnt signaling and β-catenin are reduced in Wt1 mutant mesothelium. Mice with β-catenin loss or constitutive activation induced in the Wt1 lineage are only affected when tamoxifen injection occurs between E10.5 and E11.5, revealing a critical time-frame for Wt1/ β-catenin activity. Conditional β-catenin loss phenocopies the Wt1 mutant diaphragm defect, while constitutive activation of β-catenin on the Wt1 mutant background is sufficient to close the diaphragm defect. Proliferation and apoptosis are affected, but primarily these genetic manipulations appear to lead to a change in normal diaphragm differentiation. Our data suggest a fundamental role for mesothelial signaling in proper formation of the diaphragm.
    Keywords animal models ; apoptosis ; beta catenin ; developmental stages ; diaphragm ; genetic engineering ; hernia ; humans ; loss-of-function mutation ; mice ; morbidity ; mortality ; mutants ; organogenesis ; tamoxifen
    Language English
    Dates of publication 2015-1101
    Size p. 40-56.
    Publishing place Elsevier Inc.
    Document type Article
    ZDB-ID 1114-9
    ISSN 1095-564X ; 0012-1606
    ISSN (online) 1095-564X
    ISSN 0012-1606
    DOI 10.1016/j.ydbio.2015.08.009
    Database NAL-Catalogue (AGRICOLA)

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  8. Article: Development of the diaphragm and genetic mouse models of diaphragmatic defects.

    Ackerman, Kate G / Greer, John J

    American journal of medical genetics. Part C, Seminars in medical genetics

    2007  Volume 145C, Issue 2, Page(s) 109–116

    Abstract: Improving our understanding of diaphragmatic development is essential to making progress in defining the pathogenesis and genetic etiologies of congenital diaphragmatic defects in humans. As mouse genetic technology has given us new tools to manipulate ... ...

    Abstract Improving our understanding of diaphragmatic development is essential to making progress in defining the pathogenesis and genetic etiologies of congenital diaphragmatic defects in humans. As mouse genetic technology has given us new tools to manipulate and observe development, a number of mouse models have recently emerged that provide valuable insight to this field. In this article, we review our current understanding of diaphragmatic embryogenesis including the origin of diaphragmatic tissue. We use rodent models to review the muscularization of the diaphragm and review selected genetic models of abnormal muscularization. We also review models of posterior diaphragmatic defects and discuss evidence for the pleuroperitoneal fold (PPF) tissue contributing to the diaphragm. Finally, we discuss models of anterior and central hernias. It may be simplistic to subdivide this review based on anatomic regions of the diaphragm, as evidence is emerging that defects in different regions of the diaphragm in humans and in mice may be etiologically related. However, at this time we do not have enough knowledge to make more mechanistic or genetic classifications though with time, genetic progress in the field of diaphragm development will allow us to do this.
    MeSH term(s) Abdominal Muscles/embryology ; Animals ; COUP Transcription Factor II/genetics ; DNA-Binding Proteins/genetics ; Diaphragm/abnormalities ; Diaphragm/embryology ; Disease Models, Animal ; Hernia, Diaphragmatic/embryology ; Hernia, Diaphragmatic/genetics ; Humans ; Lung/abnormalities ; Mice ; Mutation ; Transcription Factors/genetics
    Chemical Substances COUP Transcription Factor II ; DNA-Binding Proteins ; Transcription Factors ; ZFPM2 protein, human
    Language English
    Publishing date 2007-05-15
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2108622-9
    ISSN 1552-4868 ; 0148-7299
    ISSN 1552-4868 ; 0148-7299
    DOI 10.1002/ajmg.c.30128
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article: Congenital diaphragmatic hernia and pulmonary hypoplasia: new insights from developmental biology and genetics.

    Ackerman, Kate G / Pober, Barbara R

    American journal of medical genetics. Part C, Seminars in medical genetics

    2007  Volume 145C, Issue 2, Page(s) 105–108

    MeSH term(s) Hernia, Diaphragmatic/complications ; Hernia, Diaphragmatic/genetics ; Humans ; Infant, Newborn ; Phenotype ; Respiratory System Abnormalities/complications ; Respiratory System Abnormalities/genetics ; Signal Transduction
    Language English
    Publishing date 2007-05-15
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2108622-9
    ISSN 1552-4868 ; 0148-7299
    ISSN 1552-4868 ; 0148-7299
    DOI 10.1002/ajmg.c.30133
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Perspectives from the Society for Pediatric Research: advice on sustaining science and mentoring during COVID-19.

    Forster, Catherine S / Nguyen, Suong T / Powell, Weston T / Moore, Daniel J / Ho, Jacqueline / Heyman, Melvin B / Wenger, Tara L / Gonzalez, Fernando / Hostetter, Margaret / Nowalk, Andrew / Rassbach, Caroline E / Boyer, Debra / Weiss, Pnina / Blankenburg, Rebecca L / Orange, Jordan S / Ackerman, Kate G / Burns, Audrea M

    Pediatric research

    2021  Volume 90, Issue 4, Page(s) 738–743

    Abstract: The COVID-19 pandemic will leave an indelible mark on the careers of current medical trainees. Given the disruptions to medical education, economic impact on institutions, and the uncertainties around future job prospects, trainees are facing ... ...

    Abstract The COVID-19 pandemic will leave an indelible mark on the careers of current medical trainees. Given the disruptions to medical education, economic impact on institutions, and the uncertainties around future job prospects, trainees are facing unprecedented challenges. This situation is especially concerning for futures of pediatric physician-scientist trainees, where concerns regarding maintaining the pipeline were well documented prior to the emergence of COVID-19. In this Perspectives article, we leverage the unique expertise of our workgroup to address concerns of physician-scientist trainees and to provide suggestions on how to navigate career trajectories in the post-COVID-19 era. We identified and addressed four major areas of concern: lack of in-person conferences and the associated decrease access to mentors and networking activities, decreased academic productivity, diminished job prospects, and mental health challenges. We also suggest actions for trainees, mentors and educational leaders, and institutions to help support trainees during the pandemic, with a goal of maintaining the pediatric physician-scientist pipeline.
    MeSH term(s) Biomedical Research/education ; COVID-19 ; Career Mobility ; Education, Medical, Graduate ; Efficiency ; Humans ; Interpersonal Relations ; Mental Health ; Mentors ; Pediatricians/education ; Pediatricians/psychology ; Pediatrics/education ; Societies, Medical
    Language English
    Publishing date 2021-01-19
    Publishing country United States
    Document type Journal Article
    ZDB-ID 4411-8
    ISSN 1530-0447 ; 0031-3998
    ISSN (online) 1530-0447
    ISSN 0031-3998
    DOI 10.1038/s41390-020-01321-5
    Database MEDical Literature Analysis and Retrieval System OnLINE

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