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  1. Article: Pulmonary Vein Stenosis Associated with Germline

    Yung, Delphine / Freeman, Kaitlyn / Mirzaa, Ghayda

    Children (Basel, Switzerland)

    2022  Volume 9, Issue 5

    Abstract: Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, ...

    Abstract Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, medications targeting cell proliferation, which are not uniformly effective. We present a patient with PVS and a
    Language English
    Publishing date 2022-05-05
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 2732685-8
    ISSN 2227-9067
    ISSN 2227-9067
    DOI 10.3390/children9050671
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Enhanced congenital cardiac surgery knowledge with multidisciplinary collaborative education series for paediatric cardiology fellows and nurse practitioners.

    Lee, Madonna E / Price, Joshua / Schauer, Jenna / Carlozzi, Lauren / Virk, Kathryn / Freeman, Kaitlyn / Lee, Joan / Johnston, Troy A / Bhat, Aarti

    Cardiology in the young

    2023  Volume 34, Issue 3, Page(s) 519–523

    Abstract: Introduction: Paediatric cardiologists and nurse practitioners lack structured education tools focused on basic cardiac surgery principles. However, non-surgical specialties caring for surgical patients require this knowledge for comprehensive clinical ... ...

    Abstract Introduction: Paediatric cardiologists and nurse practitioners lack structured education tools focused on basic cardiac surgery principles. However, non-surgical specialties caring for surgical patients require this knowledge for comprehensive clinical care. We created a cardiac surgical educational curriculum focused on improving knowledge and attitudes towards communication for non-surgical trainees and advanced practice providers.
    Methods: Over one academic year, six paediatric cardiology fellows and seven paediatric cardiac surgery nurse practitioners at Seattle Children's Hospital participated in this study. With surgical supervision, six lectures were prepared by each fellow and delivered monthly. Sessions were hybrid and recorded for later viewing. Pre- and post-intervention survey of attitudes regarding surgical topics and pre- and post- test-based knowledge assessments were administered.
    Results: Participants positively rated the usefulness of the lecture series (4.2/5) and would recommend it to a colleague (4.5/5). Self-reported confidence discussing surgical concepts with patients increased from 2.3 to 3.4 among paediatric cardiology fellows (p < 0.001) and from 2.8 to 3.9 among nurse practitioners (p < 0.001), out of 5. In both groups, knowledge assessment scores improved from 54 to 79% post-intervention (p < 0.001).
    Conclusions: After a six-part educational series taught by paediatric cardiology fellows, both paediatric cardiology fellows and paediatric cardiac surgery nurse practitioners demonstrated improved knowledge and reported increased comfort counselling families on basic cardiac surgery topics. Structured, active-learning lessons taught by fellows for non-surgical audiences can improve attitudes and build clinically relevant knowledge. Creating an effective level-appropriate multidisciplinary curriculum accessible to various types of medical providers could enhance comprehensive care of complex congenital cardiac surgery patients.
    MeSH term(s) Humans ; Child ; Educational Status ; Cardiac Surgical Procedures ; Nurse Practitioners ; Curriculum ; Cardiology
    Language English
    Publishing date 2023-07-26
    Publishing country England
    Document type Journal Article
    ZDB-ID 1078466-4
    ISSN 1467-1107 ; 1047-9511
    ISSN (online) 1467-1107
    ISSN 1047-9511
    DOI 10.1017/S1047951123002603
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4050: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

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  3. Article: Spatiotemporal expression of activin receptor-like kinase-5 and bone morphogenetic protein receptor type II in the ovary of shortfinned eel, Anguilla australis

    Falahati, Ali / Ozaki, Yuichi / Damsteegt, Erin L / Zadmajid, Vahid / Freeman, Kaitlyn J / Lokman, P. Mark

    Comparative biochemistry and physiology. 2021 Jan., v. 251

    2021  

    Abstract: In the eel ovary, the expression of growth differentiation factor-9 (Gdf9) appears to be largely confined to the germ cell in early stages of oogenesis. However, both the target tissue and the function of Gdf9 in fish remain unknown. This study aimed to ... ...

    Abstract In the eel ovary, the expression of growth differentiation factor-9 (Gdf9) appears to be largely confined to the germ cell in early stages of oogenesis. However, both the target tissue and the function of Gdf9 in fish remain unknown. This study aimed to describe the abundance and localization of activin receptor-like kinase-5 (Alk5) and bone morphogenetic protein receptor type II (Bmpr2), which together mediate the Gdf9 signal, in the ovary of a basal teleost, the shortfinned eel, Anguilla australis, during early folliculogenesis. The cDNA encoding eel alk5 and bmpr2 genes were cloned, characterized and the transcript abundances of these receptors quantified by quantitative real-time PCR. Ovarian transcript abundance for both receptors, along with that of gdf9 and of its paralogue bmp15, increased from the previtellogenic to early vitellogenic stage. Localization of receptor mRNAs by in situ hybridization revealed that these receptors are located in the somatic cells surrounding the oocyte. Furthermore, tissue distribution analysis showed that the expression of alk5 and bmpr2 were highest in ovary and thyroid, respectively. Unexpectedly, however, bmpr2 mRNA levels were lower in the ovary than in any of the other 17 tissues examined, and indeed, lower than ovarian gdf9 transcript abundance. These findings, together with the ovarian expression pattern of Gdf9, suggest that Gdf9, and conceivably, Bmp15, from the oocyte can signal through receptors that are located on the somatic cells surrounding the oocyte; this, in turn, facilitates elucidation of the function of these growth factors during oogenesis in teleost fish.
    Keywords Anguilla australis ; bone morphogenetic proteins ; complementary DNA ; eel ; follicular development ; genes ; growth factors ; in situ hybridization ; messenger RNA ; oocytes ; oogenesis ; quantitative polymerase chain reaction ; receptors ; somatic cells ; tissue distribution ; tissues
    Language English
    Dates of publication 2021-01
    Publishing place Elsevier Inc.
    Document type Article
    ZDB-ID 121247-3
    ISSN 1879-1107 ; 0305-0491 ; 1096-4959
    ISSN (online) 1879-1107
    ISSN 0305-0491 ; 1096-4959
    DOI 10.1016/j.cbpb.2020.110509
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    In stock of ZB MED Bonn / Germany

    Z 71/715: Show issues

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  4. Article ; Online: Living with Congenital Aortic Stenosis: Exercise Restriction, Patterns of Adherence, and Quality of Life.

    Mansfield, Laura K / Reichman, Jeffrey R / Crowley, David I / Flyer, Jonathan N / Freeman, Kaitlyn / Gauvreau, Kimberlee K / Mackie, Stewart A / Marino, Bradley S / Newburger, Jane W / Ziniel, Sonja I / Brown, David W

    Pediatric cardiology

    2023  

    Abstract: Modern consensus panel guidelines recommend restriction from most organized sports for patients with moderate or severe aortic stenosis (AS). However, there is little published data on how frequently physicians deviate from guidelines, how well patients ... ...

    Abstract Modern consensus panel guidelines recommend restriction from most organized sports for patients with moderate or severe aortic stenosis (AS). However, there is little published data on how frequently physicians deviate from guidelines, how well patients adhere to exercise restrictions, or the effect of restriction on patient-reported quality of life. In this study, we surveyed 93 subjects with AS and their cardiologists regarding participation in organized sports, physical activity, weightlifting, and exercise restriction. Subjects completed the pediatric quality of life inventory (PedsQL) and the pediatric cardiac quality of life inventory (PCQLI). We found that subjects with severe AS (n = 3) were commonly, but not universally, restricted from organized sports (n = 2, 66%). Subjects with moderate AS (n = 40) were rarely restricted from organized sports (n = 6, 17%). No physician-specific characteristics were associated with increased likelihood of recommending exercise restriction. Subjects were more likely to be restricted if they were older (16 years vs. 13 years, p 0.02) and had moderate versus mild AS (p 0.013). PCQLI scores for teens and young adults with AS (age 13-25) were lower than a comparison group of patients with mild congenital heart disease. For all age groups, the PedsQL social functioning score was lower for subjects with exercise restriction (p 0.052). In summary, cardiologists apply consensus guidelines leniently when restricting patients with moderate/severe AS from organized sports and weightlifting. Patients with AS routinely adhere to exercise restriction recommendations. Children and young adults with AS and exercise restriction have lower QOL scores in the social functioning domain.
    Language English
    Publishing date 2023-06-21
    Publishing country United States
    Document type Journal Article
    ZDB-ID 800857-7
    ISSN 1432-1971 ; 0172-0643
    ISSN (online) 1432-1971
    ISSN 0172-0643
    DOI 10.1007/s00246-023-03165-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1528: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  5. Article ; Online: Spatiotemporal expression of activin receptor-like kinase-5 and bone morphogenetic protein receptor type II in the ovary of shortfinned eel, Anguilla australis.

    Falahati, Ali / Ozaki, Yuichi / Damsteegt, Erin L / Zadmajid, Vahid / Freeman, Kaitlyn J / Lokman, P Mark

    Comparative biochemistry and physiology. Part B, Biochemistry & molecular biology

    2020  Volume 251, Page(s) 110509

    Abstract: In the eel ovary, the expression of growth differentiation factor-9 (Gdf9) appears to be largely confined to the germ cell in early stages of oogenesis. However, both the target tissue and the function of Gdf9 in fish remain unknown. This study aimed to ... ...

    Abstract In the eel ovary, the expression of growth differentiation factor-9 (Gdf9) appears to be largely confined to the germ cell in early stages of oogenesis. However, both the target tissue and the function of Gdf9 in fish remain unknown. This study aimed to describe the abundance and localization of activin receptor-like kinase-5 (Alk5) and bone morphogenetic protein receptor type II (Bmpr2), which together mediate the Gdf9 signal, in the ovary of a basal teleost, the shortfinned eel, Anguilla australis, during early folliculogenesis. The cDNA encoding eel alk5 and bmpr2 genes were cloned, characterized and the transcript abundances of these receptors quantified by quantitative real-time PCR. Ovarian transcript abundance for both receptors, along with that of gdf9 and of its paralogue bmp15, increased from the previtellogenic to early vitellogenic stage. Localization of receptor mRNAs by in situ hybridization revealed that these receptors are located in the somatic cells surrounding the oocyte. Furthermore, tissue distribution analysis showed that the expression of alk5 and bmpr2 were highest in ovary and thyroid, respectively. Unexpectedly, however, bmpr2 mRNA levels were lower in the ovary than in any of the other 17 tissues examined, and indeed, lower than ovarian gdf9 transcript abundance. These findings, together with the ovarian expression pattern of Gdf9, suggest that Gdf9, and conceivably, Bmp15, from the oocyte can signal through receptors that are located on the somatic cells surrounding the oocyte; this, in turn, facilitates elucidation of the function of these growth factors during oogenesis in teleost fish.
    MeSH term(s) Anguilla/genetics ; Anguilla/growth & development ; Animals ; Bone Morphogenetic Protein Receptors, Type II/genetics ; Female ; Gene Expression Regulation, Enzymologic ; Ovary/metabolism ; Receptor, Transforming Growth Factor-beta Type I/genetics ; Spatio-Temporal Analysis
    Chemical Substances Bone Morphogenetic Protein Receptors, Type II (EC 2.7.11.30) ; Receptor, Transforming Growth Factor-beta Type I (EC 2.7.11.30)
    Language English
    Publishing date 2020-09-28
    Publishing country England
    Document type Journal Article
    ZDB-ID 121247-3
    ISSN 1879-1107 ; 0305-0491 ; 1096-4959
    ISSN (online) 1879-1107
    ISSN 0305-0491 ; 1096-4959
    DOI 10.1016/j.cbpb.2020.110509
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 44/B: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  6. Article ; Online: Validation of Prenatal Aortic Arch Angle Measurements in the Diagnosis of Neonatal Coarctation of the Aorta.

    Freeman, Kaitlyn / Kronmal, Richard / Clouse, Michele / Conwell, Jeffrey / Bhat, Aarti / Young, Luciana / Lewin, Mark / Arya, Bhawna

    Pediatric cardiology

    2021  Volume 42, Issue 6, Page(s) 1365–1371

    Abstract: Prenatal prediction of coarctation of the aorta (CoA) is challenging. Methods identifying prenatal CoA have high sensitivity with significant false positives. We previously derived prenatal aortic arch angles for identifying CoA with high sensitivity and ...

    Abstract Prenatal prediction of coarctation of the aorta (CoA) is challenging. Methods identifying prenatal CoA have high sensitivity with significant false positives. We previously derived prenatal aortic arch angles for identifying CoA with high sensitivity and specificity and aim to validate these angles and compare them with a model utilizing ascending aorta (AAo) and isthmus (Aoi) measures. Retrospective case/cohort study of fetuses with prenatal suspicion for CoA. 35 fetuses were included. Measurements included: ascending-descending aortic angle (AAo.DAo), transverse-descending aortic angle (TAo.DAo); diameters and z-scores of Aoi from sagittal (Aoi-sag), three-vessel (Aoi-3VV) view and AAo. Discriminant functions for the 5 variables were compared using histograms and positive/negative predictive values (PPV/NPV). CoA was confirmed in 28/35 neonates. The PPV and NPV for angle measures were 100% and 77%. The AAo + Aoi-3VV model PPV and NPV were 92% and 80% and Aoi-sag + Aoi-3VV model were 82% and 71%. A linear discriminant model utilizing the 3 most predictive variables improved NPV to 90% and PPV to 100%. In conclusion, we validate that angle measures are superior to standard models of predicting CoA. An optimized 3 variable model maintains accuracy of identifying CoA while eliminating false positives.
    MeSH term(s) Aorta, Thoracic/diagnostic imaging ; Aortic Coarctation/diagnosis ; Cohort Studies ; Echocardiography/methods ; Female ; Fetus/diagnostic imaging ; Humans ; Infant, Newborn ; Predictive Value of Tests ; Pregnancy ; Retrospective Studies ; Ultrasonography, Prenatal/methods
    Language English
    Publishing date 2021-04-26
    Publishing country United States
    Document type Journal Article
    ZDB-ID 800857-7
    ISSN 1432-1971 ; 0172-0643
    ISSN (online) 1432-1971
    ISSN 0172-0643
    DOI 10.1007/s00246-021-02620-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1528: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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