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<title xml:lang="en">Initial Experience With Propranolol Treatment of Lymphatic Anomalies: A Case Series</title>
<author>
<name sortKey="Wu, June K" sort="Wu, June K" uniqKey="Wu J" first="June K." last="Wu">June K. Wu</name>
<affiliation>
<nlm:aff id="aff1">Departments of Surgery,</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Hooper, Ellen D" sort="Hooper, Ellen D" uniqKey="Hooper E" first="Ellen D." last="Hooper">Ellen D. Hooper</name>
<affiliation>
<nlm:aff id="aff2">Pediatrics,</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Laifer Narin, Sherelle L" sort="Laifer Narin, Sherelle L" uniqKey="Laifer Narin S" first="Sherelle L." last="Laifer-Narin">Sherelle L. Laifer-Narin</name>
<affiliation>
<nlm:aff wicri:cut=", and" id="aff3">Radiology</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Simpson, Lynn L" sort="Simpson, Lynn L" uniqKey="Simpson L" first="Lynn L." last="Simpson">Lynn L. Simpson</name>
<affiliation>
<nlm:aff wicri:cut="; and" id="aff4">Obstetrics & Gynecology, College of Physicians and Surgeons, New York, New York</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Kandel, Jessica" sort="Kandel, Jessica" uniqKey="Kandel J" first="Jessica" last="Kandel">Jessica Kandel</name>
<affiliation>
<nlm:aff id="aff5">Department of Surgery, University of Chicago Medicine, Chicago, Illinois</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Shawber, Carrie J" sort="Shawber, Carrie J" uniqKey="Shawber C" first="Carrie J." last="Shawber">Carrie J. Shawber</name>
<affiliation>
<nlm:aff id="aff1">Departments of Surgery,</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff wicri:cut="; and" id="aff4">Obstetrics & Gynecology, College of Physicians and Surgeons, New York, New York</nlm:aff>
</affiliation>
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<idno type="RBID">PMC:5005016</idno>
<idno type="doi">10.1542/peds.2015-4545</idno>
<date when="2016">2016</date>
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<title xml:lang="en" level="a" type="main">Initial Experience With Propranolol Treatment of Lymphatic Anomalies: A Case Series</title>
<author>
<name sortKey="Wu, June K" sort="Wu, June K" uniqKey="Wu J" first="June K." last="Wu">June K. Wu</name>
<affiliation>
<nlm:aff id="aff1">Departments of Surgery,</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Hooper, Ellen D" sort="Hooper, Ellen D" uniqKey="Hooper E" first="Ellen D." last="Hooper">Ellen D. Hooper</name>
<affiliation>
<nlm:aff id="aff2">Pediatrics,</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Laifer Narin, Sherelle L" sort="Laifer Narin, Sherelle L" uniqKey="Laifer Narin S" first="Sherelle L." last="Laifer-Narin">Sherelle L. Laifer-Narin</name>
<affiliation>
<nlm:aff wicri:cut=", and" id="aff3">Radiology</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Simpson, Lynn L" sort="Simpson, Lynn L" uniqKey="Simpson L" first="Lynn L." last="Simpson">Lynn L. Simpson</name>
<affiliation>
<nlm:aff wicri:cut="; and" id="aff4">Obstetrics & Gynecology, College of Physicians and Surgeons, New York, New York</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Kandel, Jessica" sort="Kandel, Jessica" uniqKey="Kandel J" first="Jessica" last="Kandel">Jessica Kandel</name>
<affiliation>
<nlm:aff id="aff5">Department of Surgery, University of Chicago Medicine, Chicago, Illinois</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Shawber, Carrie J" sort="Shawber, Carrie J" uniqKey="Shawber C" first="Carrie J." last="Shawber">Carrie J. Shawber</name>
<affiliation>
<nlm:aff id="aff1">Departments of Surgery,</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff wicri:cut="; and" id="aff4">Obstetrics & Gynecology, College of Physicians and Surgeons, New York, New York</nlm:aff>
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<series>
<title level="j">Pediatrics</title>
<idno type="ISSN">0031-4005</idno>
<idno type="eISSN">1098-4275</idno>
<imprint>
<date when="2016">2016</date>
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<div type="abstract" xml:lang="en">
<p>Lymphatic malformations (LMs) are congenital lymphatic lesions that impose significant and costly morbidities on affected patients. Treatment options are limited due to incomplete understanding of LM pathobiology. Expression of an activated β
<sub>2</sub>
-adrenergic receptor has been described in LM tissue, suggesting that this pathway may contribute to the clinical manifestations of LM. We hypothesized that propranolol, a β-adrenergic receptor antagonist, might improve symptoms of patients with LMs and lymphatic anomalies. A retrospective chart review of patients treated with propranolol as an adjunct therapy was conducted; analyses included demographic characteristics, clinical features, and response to propranolol. Three patients with cystic and noncystic LMs displayed clinical improvement at a minimum dose of 0.7 mg/kg/d, whereas symptomatic relapses were observed when propranolol doses dropped below this threshold. Two patients with Klippel-Trenaunay syndrome demonstrated partial clinical responses with reduced edema. The fetus of a mother treated with propranolol from a gestational age of 35 weeks through delivery displayed arrested growth of a cervicofacial LM. Our retrospective review suggests that propranolol improved symptoms in a subset of patients with lymphatic anomalies. Propranolol treatment may also limit the growth of congenital LMs in utero.</p>
</div>
</front>
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<pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-ta">Pediatrics</journal-id>
<journal-id journal-id-type="iso-abbrev">Pediatrics</journal-id>
<journal-id journal-id-type="hwp">pediatrics</journal-id>
<journal-id journal-id-type="pmc">pediatrics</journal-id>
<journal-id journal-id-type="publisher-id">Pediatrics</journal-id>
<journal-title-group>
<journal-title>Pediatrics</journal-title>
</journal-title-group>
<issn pub-type="ppub">0031-4005</issn>
<issn pub-type="epub">1098-4275</issn>
<publisher>
<publisher-name>American Academy of Pediatrics</publisher-name>
<publisher-loc>Elk Grove Village, IL, USA</publisher-loc>
</publisher>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">27561730</article-id>
<article-id pub-id-type="pmc">5005016</article-id>
<article-id pub-id-type="publisher-id">peds.2015-4545</article-id>
<article-id pub-id-type="doi">10.1542/peds.2015-4545</article-id>
<article-categories>
<subj-group subj-group-type="hwp-journal-coll">
<subject>23.00</subject>
<subject>23.05</subject>
<subject>28.00</subject>
<subject>28.01</subject>
</subj-group>
<subj-group subj-group-type="heading">
<subject>Case Report</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>Initial Experience With Propranolol Treatment of Lymphatic Anomalies: A Case Series</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Wu</surname>
<given-names>June K.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="aff1">a</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Hooper</surname>
<given-names>Ellen D.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="aff2">b</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Laifer-Narin</surname>
<given-names>Sherelle L.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="aff3">c</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Simpson</surname>
<given-names>Lynn L.</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="aff4">d</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Kandel</surname>
<given-names>Jessica</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="aff5">e</xref>
</contrib>
<contrib contrib-type="author" corresp="yes">
<name>
<surname>Shawber</surname>
<given-names>Carrie J.</given-names>
</name>
<degrees>PhD</degrees>
<xref ref-type="aff" rid="aff1">a</xref>
<xref ref-type="aff" rid="aff4">d</xref>
</contrib>
<contrib contrib-type="author">
<collab>on behalf of the Lymphatics Work Group at CUMC</collab>
</contrib>
<aff id="aff1">
<label>a</label>
Departments of Surgery,</aff>
<aff id="aff2">
<label>b</label>
Pediatrics,</aff>
<aff id="aff3">
<label>c</label>
Radiology, and</aff>
<aff id="aff4">
<label>d</label>
Obstetrics & Gynecology, College of Physicians and Surgeons, New York, New York; and</aff>
<aff id="aff5">
<label>e</label>
Department of Surgery, University of Chicago Medicine, Chicago, Illinois</aff>
</contrib-group>
<author-notes>
<corresp id="cor6">Address correspondence to Carrie J. Shawber, PhD, Department of Obstetrics & Gynecology, College of Physicians and Surgeons, 16-440B, 630 W 168th St, New York, NY 10032. E-mail:
<email>cjs2002@cumc.columbia.edu</email>
</corresp>
</author-notes>
<pub-date pub-type="ppub">
<month>9</month>
<year>2016</year>
</pub-date>
<volume>138</volume>
<issue>3</issue>
<elocation-id>e20154545</elocation-id>
<history>
<date date-type="accepted">
<day>21</day>
<month>6</month>
<year>2016</year>
</date>
</history>
<permissions>
<copyright-statement>Copyright © 2016 by the American Academy of Pediatrics</copyright-statement>
<copyright-year>2016</copyright-year>
</permissions>
<self-uri xlink:title="pdf" xlink:href="PEDS_20154545.pdf"></self-uri>
<abstract>
<p>Lymphatic malformations (LMs) are congenital lymphatic lesions that impose significant and costly morbidities on affected patients. Treatment options are limited due to incomplete understanding of LM pathobiology. Expression of an activated β
<sub>2</sub>
-adrenergic receptor has been described in LM tissue, suggesting that this pathway may contribute to the clinical manifestations of LM. We hypothesized that propranolol, a β-adrenergic receptor antagonist, might improve symptoms of patients with LMs and lymphatic anomalies. A retrospective chart review of patients treated with propranolol as an adjunct therapy was conducted; analyses included demographic characteristics, clinical features, and response to propranolol. Three patients with cystic and noncystic LMs displayed clinical improvement at a minimum dose of 0.7 mg/kg/d, whereas symptomatic relapses were observed when propranolol doses dropped below this threshold. Two patients with Klippel-Trenaunay syndrome demonstrated partial clinical responses with reduced edema. The fetus of a mother treated with propranolol from a gestational age of 35 weeks through delivery displayed arrested growth of a cervicofacial LM. Our retrospective review suggests that propranolol improved symptoms in a subset of patients with lymphatic anomalies. Propranolol treatment may also limit the growth of congenital LMs in utero.</p>
</abstract>
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</front>
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