Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2019-12

AUTHORS

Karen Ka Yan Leung, Jaime Sou Rosa Duque, Kwong-Man Yu, Kai-Ning Cheong, Patrick Chun-Yin Chong, Marco Hok-Kung Ho, Pak-Cheong Chow

ABSTRACT

BACKGROUND: Idiopathic systemic capillary leak syndrome (ISCLS) is rare, and there has been about 32 cases reported in children worldwide since this disorder was first described in 1960. Clinical guidelines on the management approach stemming from robust scientific evidence are lacking. This case report presents the first reported paediatric case of severe ISCLS with significant myocardial oedema and emphasizes this disease's impact on a child's cardiac function. CASE PRESENTATION: A Chinese boy had his first attack of severe hypovolaemic shock that responded to fluid resuscitation when he was 6 years of age. His second attack developed at 8 years of age. He was then transferred to our cardiac unit for refractory hypotensive shock. The patient's echocardiogram revealed ventricular wall thickening with significant cardiac dysfunction requiring extracorporeal membrane oxygenation support. Subsequently, he made a full recovery, including his myocardial wall thickness and function. The echocardiographic findings suggested myocardial oedema that was transient in nature. Clinical and laboratory investigation from both episodes were compatible with ISCLS. CONCLUSION: ISCLS is rare, and therefore there is only a limited understanding on the pathophysiology of this disorder. The current treatment approach is based on a few case reports and series. During the acute phase, optimal supportive management is paramount. Our case highlights the importance of early recognition and consideration for extracorporeal membrane oxygenation support in patients with a life-threatening presentation, as it was lifesaving for this child who suffered myocardial oedema and ventricular dysfunction. More... »

PAGES

28

Journal

TITLE

BMC Pediatrics

ISSUE

1

VOLUME

19

Author Affiliations

Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/s12887-019-1401-2

DOI

http://dx.doi.org/10.1186/s12887-019-1401-2

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1111576817

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30665393


Indexing Status Check whether this publication has been indexed by Scopus and Web Of Science using the SN Indexing Status Tool
Incoming Citations Browse incoming citations for this publication using opencitations.net

JSON-LD is the canonical representation for SciGraph data.

TIP: You can open this SciGraph record using an external JSON-LD service: JSON-LD Playground Google SDTT

[
  {
    "@context": "https://springernature.github.io/scigraph/jsonld/sgcontext.json", 
    "about": [
      {
        "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/1103", 
        "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
        "name": "Clinical Sciences", 
        "type": "DefinedTerm"
      }, 
      {
        "id": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/11", 
        "inDefinedTermSet": "http://purl.org/au-research/vocabulary/anzsrc-for/2008/", 
        "name": "Medical and Health Sciences", 
        "type": "DefinedTerm"
      }
    ], 
    "author": [
      {
        "affiliation": {
          "alternateName": "Queen Mary Hospital", 
          "id": "https://www.grid.ac/institutes/grid.415550.0", 
          "name": [
            "Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Room 115, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Leung", 
        "givenName": "Karen Ka Yan", 
        "id": "sg:person.010261257453.10", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.010261257453.10"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Queen Mary Hospital", 
          "id": "https://www.grid.ac/institutes/grid.415550.0", 
          "name": [
            "Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Room 115, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Rosa Duque", 
        "givenName": "Jaime Sou", 
        "id": "sg:person.010213703620.99", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.010213703620.99"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Queen Mary Hospital", 
          "id": "https://www.grid.ac/institutes/grid.415550.0", 
          "name": [
            "Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Room 115, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Yu", 
        "givenName": "Kwong-Man", 
        "id": "sg:person.012052621362.34", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.012052621362.34"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Queen Mary Hospital", 
          "id": "https://www.grid.ac/institutes/grid.415550.0", 
          "name": [
            "Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Room 115, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Cheong", 
        "givenName": "Kai-Ning", 
        "id": "sg:person.014243142762.13", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.014243142762.13"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Queen Mary Hospital", 
          "id": "https://www.grid.ac/institutes/grid.415550.0", 
          "name": [
            "Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Room 115, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Chong", 
        "givenName": "Patrick Chun-Yin", 
        "id": "sg:person.015613404057.67", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.015613404057.67"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Queen Mary Hospital", 
          "id": "https://www.grid.ac/institutes/grid.415550.0", 
          "name": [
            "Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Room 115, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Ho", 
        "givenName": "Marco Hok-Kung", 
        "id": "sg:person.011042326102.90", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.011042326102.90"
        ], 
        "type": "Person"
      }, 
      {
        "affiliation": {
          "alternateName": "Queen Mary Hospital", 
          "id": "https://www.grid.ac/institutes/grid.415550.0", 
          "name": [
            "Department of Paediatric Cardiology, Queen Mary Hospital, Room 322, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong"
          ], 
          "type": "Organization"
        }, 
        "familyName": "Chow", 
        "givenName": "Pak-Cheong", 
        "id": "sg:person.0760773613.71", 
        "sameAs": [
          "https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0760773613.71"
        ], 
        "type": "Person"
      }
    ], 
    "citation": [
      {
        "id": "sg:pub.10.1186/1471-2431-14-137", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1000089832", 
          "https://doi.org/10.1186/1471-2431-14-137"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.amjmed.2014.08.015", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1000213490"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.ejpn.2016.08.003", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1001208745"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/01.pcc.0000227120.61949.fb", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1001655915"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/01.pcc.0000227120.61949.fb", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1001655915"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.amjmed.2016.02.046", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1001912995"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.jaci.2009.06.043", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1002515461"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/s0140-6736(11)61819-9", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1003144630"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/md.0000000000000591", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1004156555"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/md.0000000000000591", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1004156555"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/inf.0000000000000029", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1004347593"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/inf.0000000000000029", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1004347593"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1179/146532807x170556", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1013002137"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s001340050849", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1015077703", 
          "https://doi.org/10.1007/s001340050849"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1111/j.1365-2362.1987.tb01225.x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1015908556"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1111/j.1600-065x.1989.tb00031.x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1016350247"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4161/rdis.27445", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1019736323"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.4065/mcp.2010.0159", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1024368062"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.revmed.2010.06.002", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1024808663"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1378/chest.104.1.123", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1029570076"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1182/blood-2011-08-375816", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1034442871"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1179/2046905514y.0000000161", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1035887192"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s12098-016-2134-y", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1036626018", 
          "https://doi.org/10.1007/s12098-016-2134-y"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1177/2048872612455142", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1037018167"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1177/2048872612455142", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1037018167"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1186/s12968-015-0181-6", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1037284534", 
          "https://doi.org/10.1186/s12968-015-0181-6"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/s0022-3476(95)70163-x", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1038878552"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.jaci.2016.10.042", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1039169495"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/0002-9343(60)90018-8", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1039386024"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/ccm.0b013e31817d7c71", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1040332771"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1097/ccm.0b013e31817d7c71", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1040332771"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1542/peds.2014-2268", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1041419392"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1542/peds.2009-1691", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1067827952"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.7326/0003-4819-113-6-475", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1073694967"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.7326/0003-4819-153-2-201007200-00005", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1073711656"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.7326/0003-4819-154-7-201104050-00004", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1073712151"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://app.dimensions.ai/details/publication/pub.1076951189", 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://app.dimensions.ai/details/publication/pub.1077647683", 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://app.dimensions.ai/details/publication/pub.1078732981", 
        "type": "CreativeWork"
      }, 
      {
        "id": "https://doi.org/10.1016/j.kint.2016.11.029", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1084093842"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s00431-018-3189-8", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1105086545", 
          "https://doi.org/10.1007/s00431-018-3189-8"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s00431-018-3189-8", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1105086545", 
          "https://doi.org/10.1007/s00431-018-3189-8"
        ], 
        "type": "CreativeWork"
      }, 
      {
        "id": "sg:pub.10.1007/s00431-018-3189-8", 
        "sameAs": [
          "https://app.dimensions.ai/details/publication/pub.1105086545", 
          "https://doi.org/10.1007/s00431-018-3189-8"
        ], 
        "type": "CreativeWork"
      }
    ], 
    "datePublished": "2019-12", 
    "datePublishedReg": "2019-12-01", 
    "description": "BACKGROUND: Idiopathic systemic capillary leak syndrome (ISCLS) is rare, and there has been about 32 cases reported in children worldwide since this disorder was first described in 1960. Clinical guidelines on the management approach stemming from robust scientific evidence are lacking. This case report presents the first reported paediatric case of severe ISCLS with significant myocardial oedema and emphasizes this disease's impact on a child's cardiac function.\nCASE PRESENTATION: A Chinese boy had his first attack of severe hypovolaemic shock that responded to fluid resuscitation when he was 6\u2009years of age. His second attack developed at 8\u2009years of age. He was then transferred to our cardiac unit for refractory hypotensive shock. The patient's echocardiogram revealed ventricular wall thickening with significant cardiac dysfunction requiring extracorporeal membrane oxygenation support. Subsequently, he made a full recovery, including his myocardial wall thickness and function. The echocardiographic findings suggested myocardial oedema that was transient in nature. Clinical and laboratory investigation from both episodes were compatible with ISCLS.\nCONCLUSION: ISCLS is rare, and therefore there is only a limited understanding on the pathophysiology of this disorder. The current treatment approach is based on a few case reports and series. During the acute phase, optimal supportive management is paramount. Our case highlights the importance of early recognition and consideration for extracorporeal membrane oxygenation support in patients with a life-threatening presentation, as it was lifesaving for this child who suffered myocardial oedema and ventricular dysfunction.", 
    "genre": "research_article", 
    "id": "sg:pub.10.1186/s12887-019-1401-2", 
    "inLanguage": [
      "en"
    ], 
    "isAccessibleForFree": true, 
    "isPartOf": [
      {
        "id": "sg:journal.1024635", 
        "issn": [
          "1471-2431"
        ], 
        "name": "BMC Pediatrics", 
        "type": "Periodical"
      }, 
      {
        "issueNumber": "1", 
        "type": "PublicationIssue"
      }, 
      {
        "type": "PublicationVolume", 
        "volumeNumber": "19"
      }
    ], 
    "name": "Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome", 
    "pagination": "28", 
    "productId": [
      {
        "name": "readcube_id", 
        "type": "PropertyValue", 
        "value": [
          "22d720c993db8261c3671bd83f0b273613d5e962285b3d1982d894c62a469690"
        ]
      }, 
      {
        "name": "pubmed_id", 
        "type": "PropertyValue", 
        "value": [
          "30665393"
        ]
      }, 
      {
        "name": "nlm_unique_id", 
        "type": "PropertyValue", 
        "value": [
          "100967804"
        ]
      }, 
      {
        "name": "doi", 
        "type": "PropertyValue", 
        "value": [
          "10.1186/s12887-019-1401-2"
        ]
      }, 
      {
        "name": "dimensions_id", 
        "type": "PropertyValue", 
        "value": [
          "pub.1111576817"
        ]
      }
    ], 
    "sameAs": [
      "https://doi.org/10.1186/s12887-019-1401-2", 
      "https://app.dimensions.ai/details/publication/pub.1111576817"
    ], 
    "sdDataset": "articles", 
    "sdDatePublished": "2019-04-11T08:56", 
    "sdLicense": "https://scigraph.springernature.com/explorer/license/", 
    "sdPublisher": {
      "name": "Springer Nature - SN SciGraph project", 
      "type": "Organization"
    }, 
    "sdSource": "s3://com-uberresearch-data-dimensions-target-20181106-alternative/cleanup/v134/2549eaecd7973599484d7c17b260dba0a4ecb94b/merge/v9/a6c9fde33151104705d4d7ff012ea9563521a3ce/jats-lookup/v90/0000000325_0000000325/records_100794_00000000.jsonl", 
    "type": "ScholarlyArticle", 
    "url": "https://link.springer.com/10.1186%2Fs12887-019-1401-2"
  }
]
 

Download the RDF metadata as:  json-ld nt turtle xml License info

HOW TO GET THIS DATA PROGRAMMATICALLY:

JSON-LD is a popular format for linked data which is fully compatible with JSON.

curl -H 'Accept: application/ld+json' 'https://scigraph.springernature.com/pub.10.1186/s12887-019-1401-2'

N-Triples is a line-based linked data format ideal for batch operations.

curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1186/s12887-019-1401-2'

Turtle is a human-readable linked data format.

curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1186/s12887-019-1401-2'

RDF/XML is a standard XML format for linked data.

curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1186/s12887-019-1401-2'


 

This table displays all metadata directly associated to this object as RDF triples.

221 TRIPLES      21 PREDICATES      65 URIs      21 LITERALS      9 BLANK NODES

Subject Predicate Object
1 sg:pub.10.1186/s12887-019-1401-2 schema:about anzsrc-for:11
2 anzsrc-for:1103
3 schema:author N7d3e58a2cc084555b4cc16b0caf006b3
4 schema:citation sg:pub.10.1007/s001340050849
5 sg:pub.10.1007/s00431-018-3189-8
6 sg:pub.10.1007/s12098-016-2134-y
7 sg:pub.10.1186/1471-2431-14-137
8 sg:pub.10.1186/s12968-015-0181-6
9 https://app.dimensions.ai/details/publication/pub.1076951189
10 https://app.dimensions.ai/details/publication/pub.1077647683
11 https://app.dimensions.ai/details/publication/pub.1078732981
12 https://doi.org/10.1016/0002-9343(60)90018-8
13 https://doi.org/10.1016/j.amjmed.2014.08.015
14 https://doi.org/10.1016/j.amjmed.2016.02.046
15 https://doi.org/10.1016/j.ejpn.2016.08.003
16 https://doi.org/10.1016/j.jaci.2009.06.043
17 https://doi.org/10.1016/j.jaci.2016.10.042
18 https://doi.org/10.1016/j.kint.2016.11.029
19 https://doi.org/10.1016/j.revmed.2010.06.002
20 https://doi.org/10.1016/s0022-3476(95)70163-x
21 https://doi.org/10.1016/s0140-6736(11)61819-9
22 https://doi.org/10.1097/01.pcc.0000227120.61949.fb
23 https://doi.org/10.1097/ccm.0b013e31817d7c71
24 https://doi.org/10.1097/inf.0000000000000029
25 https://doi.org/10.1097/md.0000000000000591
26 https://doi.org/10.1111/j.1365-2362.1987.tb01225.x
27 https://doi.org/10.1111/j.1600-065x.1989.tb00031.x
28 https://doi.org/10.1177/2048872612455142
29 https://doi.org/10.1179/146532807x170556
30 https://doi.org/10.1179/2046905514y.0000000161
31 https://doi.org/10.1182/blood-2011-08-375816
32 https://doi.org/10.1378/chest.104.1.123
33 https://doi.org/10.1542/peds.2009-1691
34 https://doi.org/10.1542/peds.2014-2268
35 https://doi.org/10.4065/mcp.2010.0159
36 https://doi.org/10.4161/rdis.27445
37 https://doi.org/10.7326/0003-4819-113-6-475
38 https://doi.org/10.7326/0003-4819-153-2-201007200-00005
39 https://doi.org/10.7326/0003-4819-154-7-201104050-00004
40 schema:datePublished 2019-12
41 schema:datePublishedReg 2019-12-01
42 schema:description BACKGROUND: Idiopathic systemic capillary leak syndrome (ISCLS) is rare, and there has been about 32 cases reported in children worldwide since this disorder was first described in 1960. Clinical guidelines on the management approach stemming from robust scientific evidence are lacking. This case report presents the first reported paediatric case of severe ISCLS with significant myocardial oedema and emphasizes this disease's impact on a child's cardiac function. CASE PRESENTATION: A Chinese boy had his first attack of severe hypovolaemic shock that responded to fluid resuscitation when he was 6 years of age. His second attack developed at 8 years of age. He was then transferred to our cardiac unit for refractory hypotensive shock. The patient's echocardiogram revealed ventricular wall thickening with significant cardiac dysfunction requiring extracorporeal membrane oxygenation support. Subsequently, he made a full recovery, including his myocardial wall thickness and function. The echocardiographic findings suggested myocardial oedema that was transient in nature. Clinical and laboratory investigation from both episodes were compatible with ISCLS. CONCLUSION: ISCLS is rare, and therefore there is only a limited understanding on the pathophysiology of this disorder. The current treatment approach is based on a few case reports and series. During the acute phase, optimal supportive management is paramount. Our case highlights the importance of early recognition and consideration for extracorporeal membrane oxygenation support in patients with a life-threatening presentation, as it was lifesaving for this child who suffered myocardial oedema and ventricular dysfunction.
43 schema:genre research_article
44 schema:inLanguage en
45 schema:isAccessibleForFree true
46 schema:isPartOf N1be5dfcfc26b4320b31e0c09b4dbdc70
47 N94ce52fbd4a440feabccfa24659410ad
48 sg:journal.1024635
49 schema:name Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome
50 schema:pagination 28
51 schema:productId N442e2001fec546cb81f0570907273fff
52 N8a3aafd97da34381b485048c52cea231
53 N8a9e3366d4654a0f94c003db1b469672
54 Neb95d2a2ce5e4f2c8e7423fae33735a3
55 Nf36a7c6a45ad49e2b46390740887a751
56 schema:sameAs https://app.dimensions.ai/details/publication/pub.1111576817
57 https://doi.org/10.1186/s12887-019-1401-2
58 schema:sdDatePublished 2019-04-11T08:56
59 schema:sdLicense https://scigraph.springernature.com/explorer/license/
60 schema:sdPublisher N8411ffee2c1d492ebc19f0bcae085fa6
61 schema:url https://link.springer.com/10.1186%2Fs12887-019-1401-2
62 sgo:license sg:explorer/license/
63 sgo:sdDataset articles
64 rdf:type schema:ScholarlyArticle
65 N19ad3ffd6fad4ebaa27e4dd94be9a622 rdf:first sg:person.0760773613.71
66 rdf:rest rdf:nil
67 N1be5dfcfc26b4320b31e0c09b4dbdc70 schema:issueNumber 1
68 rdf:type schema:PublicationIssue
69 N442e2001fec546cb81f0570907273fff schema:name dimensions_id
70 schema:value pub.1111576817
71 rdf:type schema:PropertyValue
72 N70d1064f668f488ca5d9d3b6c04cb36b rdf:first sg:person.010213703620.99
73 rdf:rest Neb98fa8568394d88bca518552f5c1f97
74 N7d3e58a2cc084555b4cc16b0caf006b3 rdf:first sg:person.010261257453.10
75 rdf:rest N70d1064f668f488ca5d9d3b6c04cb36b
76 N7e1b323af71444798c229505f1a81339 rdf:first sg:person.015613404057.67
77 rdf:rest Ncc936dedcdc344b4b69e11787ec135c4
78 N8411ffee2c1d492ebc19f0bcae085fa6 schema:name Springer Nature - SN SciGraph project
79 rdf:type schema:Organization
80 N8a3aafd97da34381b485048c52cea231 schema:name readcube_id
81 schema:value 22d720c993db8261c3671bd83f0b273613d5e962285b3d1982d894c62a469690
82 rdf:type schema:PropertyValue
83 N8a9e3366d4654a0f94c003db1b469672 schema:name pubmed_id
84 schema:value 30665393
85 rdf:type schema:PropertyValue
86 N94ce52fbd4a440feabccfa24659410ad schema:volumeNumber 19
87 rdf:type schema:PublicationVolume
88 Ncc936dedcdc344b4b69e11787ec135c4 rdf:first sg:person.011042326102.90
89 rdf:rest N19ad3ffd6fad4ebaa27e4dd94be9a622
90 Ne9c7a07697f84e0989d767d6c44a2f0d rdf:first sg:person.014243142762.13
91 rdf:rest N7e1b323af71444798c229505f1a81339
92 Neb95d2a2ce5e4f2c8e7423fae33735a3 schema:name doi
93 schema:value 10.1186/s12887-019-1401-2
94 rdf:type schema:PropertyValue
95 Neb98fa8568394d88bca518552f5c1f97 rdf:first sg:person.012052621362.34
96 rdf:rest Ne9c7a07697f84e0989d767d6c44a2f0d
97 Nf36a7c6a45ad49e2b46390740887a751 schema:name nlm_unique_id
98 schema:value 100967804
99 rdf:type schema:PropertyValue
100 anzsrc-for:11 schema:inDefinedTermSet anzsrc-for:
101 schema:name Medical and Health Sciences
102 rdf:type schema:DefinedTerm
103 anzsrc-for:1103 schema:inDefinedTermSet anzsrc-for:
104 schema:name Clinical Sciences
105 rdf:type schema:DefinedTerm
106 sg:journal.1024635 schema:issn 1471-2431
107 schema:name BMC Pediatrics
108 rdf:type schema:Periodical
109 sg:person.010213703620.99 schema:affiliation https://www.grid.ac/institutes/grid.415550.0
110 schema:familyName Rosa Duque
111 schema:givenName Jaime Sou
112 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.010213703620.99
113 rdf:type schema:Person
114 sg:person.010261257453.10 schema:affiliation https://www.grid.ac/institutes/grid.415550.0
115 schema:familyName Leung
116 schema:givenName Karen Ka Yan
117 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.010261257453.10
118 rdf:type schema:Person
119 sg:person.011042326102.90 schema:affiliation https://www.grid.ac/institutes/grid.415550.0
120 schema:familyName Ho
121 schema:givenName Marco Hok-Kung
122 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.011042326102.90
123 rdf:type schema:Person
124 sg:person.012052621362.34 schema:affiliation https://www.grid.ac/institutes/grid.415550.0
125 schema:familyName Yu
126 schema:givenName Kwong-Man
127 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.012052621362.34
128 rdf:type schema:Person
129 sg:person.014243142762.13 schema:affiliation https://www.grid.ac/institutes/grid.415550.0
130 schema:familyName Cheong
131 schema:givenName Kai-Ning
132 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.014243142762.13
133 rdf:type schema:Person
134 sg:person.015613404057.67 schema:affiliation https://www.grid.ac/institutes/grid.415550.0
135 schema:familyName Chong
136 schema:givenName Patrick Chun-Yin
137 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.015613404057.67
138 rdf:type schema:Person
139 sg:person.0760773613.71 schema:affiliation https://www.grid.ac/institutes/grid.415550.0
140 schema:familyName Chow
141 schema:givenName Pak-Cheong
142 schema:sameAs https://app.dimensions.ai/discover/publication?and_facet_researcher=ur.0760773613.71
143 rdf:type schema:Person
144 sg:pub.10.1007/s001340050849 schema:sameAs https://app.dimensions.ai/details/publication/pub.1015077703
145 https://doi.org/10.1007/s001340050849
146 rdf:type schema:CreativeWork
147 sg:pub.10.1007/s00431-018-3189-8 schema:sameAs https://app.dimensions.ai/details/publication/pub.1105086545
148 https://doi.org/10.1007/s00431-018-3189-8
149 rdf:type schema:CreativeWork
150 sg:pub.10.1007/s12098-016-2134-y schema:sameAs https://app.dimensions.ai/details/publication/pub.1036626018
151 https://doi.org/10.1007/s12098-016-2134-y
152 rdf:type schema:CreativeWork
153 sg:pub.10.1186/1471-2431-14-137 schema:sameAs https://app.dimensions.ai/details/publication/pub.1000089832
154 https://doi.org/10.1186/1471-2431-14-137
155 rdf:type schema:CreativeWork
156 sg:pub.10.1186/s12968-015-0181-6 schema:sameAs https://app.dimensions.ai/details/publication/pub.1037284534
157 https://doi.org/10.1186/s12968-015-0181-6
158 rdf:type schema:CreativeWork
159 https://app.dimensions.ai/details/publication/pub.1076951189 schema:CreativeWork
160 https://app.dimensions.ai/details/publication/pub.1077647683 schema:CreativeWork
161 https://app.dimensions.ai/details/publication/pub.1078732981 schema:CreativeWork
162 https://doi.org/10.1016/0002-9343(60)90018-8 schema:sameAs https://app.dimensions.ai/details/publication/pub.1039386024
163 rdf:type schema:CreativeWork
164 https://doi.org/10.1016/j.amjmed.2014.08.015 schema:sameAs https://app.dimensions.ai/details/publication/pub.1000213490
165 rdf:type schema:CreativeWork
166 https://doi.org/10.1016/j.amjmed.2016.02.046 schema:sameAs https://app.dimensions.ai/details/publication/pub.1001912995
167 rdf:type schema:CreativeWork
168 https://doi.org/10.1016/j.ejpn.2016.08.003 schema:sameAs https://app.dimensions.ai/details/publication/pub.1001208745
169 rdf:type schema:CreativeWork
170 https://doi.org/10.1016/j.jaci.2009.06.043 schema:sameAs https://app.dimensions.ai/details/publication/pub.1002515461
171 rdf:type schema:CreativeWork
172 https://doi.org/10.1016/j.jaci.2016.10.042 schema:sameAs https://app.dimensions.ai/details/publication/pub.1039169495
173 rdf:type schema:CreativeWork
174 https://doi.org/10.1016/j.kint.2016.11.029 schema:sameAs https://app.dimensions.ai/details/publication/pub.1084093842
175 rdf:type schema:CreativeWork
176 https://doi.org/10.1016/j.revmed.2010.06.002 schema:sameAs https://app.dimensions.ai/details/publication/pub.1024808663
177 rdf:type schema:CreativeWork
178 https://doi.org/10.1016/s0022-3476(95)70163-x schema:sameAs https://app.dimensions.ai/details/publication/pub.1038878552
179 rdf:type schema:CreativeWork
180 https://doi.org/10.1016/s0140-6736(11)61819-9 schema:sameAs https://app.dimensions.ai/details/publication/pub.1003144630
181 rdf:type schema:CreativeWork
182 https://doi.org/10.1097/01.pcc.0000227120.61949.fb schema:sameAs https://app.dimensions.ai/details/publication/pub.1001655915
183 rdf:type schema:CreativeWork
184 https://doi.org/10.1097/ccm.0b013e31817d7c71 schema:sameAs https://app.dimensions.ai/details/publication/pub.1040332771
185 rdf:type schema:CreativeWork
186 https://doi.org/10.1097/inf.0000000000000029 schema:sameAs https://app.dimensions.ai/details/publication/pub.1004347593
187 rdf:type schema:CreativeWork
188 https://doi.org/10.1097/md.0000000000000591 schema:sameAs https://app.dimensions.ai/details/publication/pub.1004156555
189 rdf:type schema:CreativeWork
190 https://doi.org/10.1111/j.1365-2362.1987.tb01225.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1015908556
191 rdf:type schema:CreativeWork
192 https://doi.org/10.1111/j.1600-065x.1989.tb00031.x schema:sameAs https://app.dimensions.ai/details/publication/pub.1016350247
193 rdf:type schema:CreativeWork
194 https://doi.org/10.1177/2048872612455142 schema:sameAs https://app.dimensions.ai/details/publication/pub.1037018167
195 rdf:type schema:CreativeWork
196 https://doi.org/10.1179/146532807x170556 schema:sameAs https://app.dimensions.ai/details/publication/pub.1013002137
197 rdf:type schema:CreativeWork
198 https://doi.org/10.1179/2046905514y.0000000161 schema:sameAs https://app.dimensions.ai/details/publication/pub.1035887192
199 rdf:type schema:CreativeWork
200 https://doi.org/10.1182/blood-2011-08-375816 schema:sameAs https://app.dimensions.ai/details/publication/pub.1034442871
201 rdf:type schema:CreativeWork
202 https://doi.org/10.1378/chest.104.1.123 schema:sameAs https://app.dimensions.ai/details/publication/pub.1029570076
203 rdf:type schema:CreativeWork
204 https://doi.org/10.1542/peds.2009-1691 schema:sameAs https://app.dimensions.ai/details/publication/pub.1067827952
205 rdf:type schema:CreativeWork
206 https://doi.org/10.1542/peds.2014-2268 schema:sameAs https://app.dimensions.ai/details/publication/pub.1041419392
207 rdf:type schema:CreativeWork
208 https://doi.org/10.4065/mcp.2010.0159 schema:sameAs https://app.dimensions.ai/details/publication/pub.1024368062
209 rdf:type schema:CreativeWork
210 https://doi.org/10.4161/rdis.27445 schema:sameAs https://app.dimensions.ai/details/publication/pub.1019736323
211 rdf:type schema:CreativeWork
212 https://doi.org/10.7326/0003-4819-113-6-475 schema:sameAs https://app.dimensions.ai/details/publication/pub.1073694967
213 rdf:type schema:CreativeWork
214 https://doi.org/10.7326/0003-4819-153-2-201007200-00005 schema:sameAs https://app.dimensions.ai/details/publication/pub.1073711656
215 rdf:type schema:CreativeWork
216 https://doi.org/10.7326/0003-4819-154-7-201104050-00004 schema:sameAs https://app.dimensions.ai/details/publication/pub.1073712151
217 rdf:type schema:CreativeWork
218 https://www.grid.ac/institutes/grid.415550.0 schema:alternateName Queen Mary Hospital
219 schema:name Department of Paediatric Cardiology, Queen Mary Hospital, Room 322, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong
220 Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Room 115, New Clinical Building, 102 Pokfulam Road, Pokfulam, Hong Kong
221 rdf:type schema:Organization
 




Preview window. Press ESC to close (or click here)


...