Pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent
Abstract Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH...
Gespeichert in:
Veröffentlicht in: | Klinische Pädiatrie 1999-03, Vol.211 (2), p.92-95 |
---|---|
Hauptverfasser: | , , |
Format: | Artikel |
Sprache: | eng |
Schlagworte: | |
Online-Zugang: | Volltext |
Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
container_end_page | 95 |
---|---|
container_issue | 2 |
container_start_page | 92 |
container_title | Klinische Pädiatrie |
container_volume | 211 |
creator | Hussein, Ahmad Trowitzsch, Eckardt Brockmann, Michael |
description | Abstract
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH has not been reported previously in children.A 12.5-year-old boy presented with a one year history of fatigue. Pulmonary hypertension was diagnosed by echocardiography. Pulmonary function tests revealed a moderate restrictive pattern and elevated granulocytes were detected in bronchoalveolar lavage. An isolated high-titer APL was detected. Open lung biopsy established the diagnosis of PVOD, with no evidence of pulmonary thrombosis, but with accompanying interstitial and alveolar cellular infiltration. We speculate that APL may have played a role in the pathogenesis of PVOD. Prednison improved the symptoms of the interstitial pneumonitis and was stopped; on follow up of 30 months, the patient ist in stable condition on therapy with nifedipin, phenprocoumon and digoxin.CONCLUSIONS PVOD and APL may be present simultaneously as a rare cause of PH. Interstitial pneumonitis may accompany PVOD and produce the leading symptoms. Open lung biopsy is essential for early establishment of the diagnosis. |
doi_str_mv | 10.1055/s-2008-1043773 |
format | Article |
fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_proquest_miscellaneous_69889256</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>69889256</sourcerecordid><originalsourceid>FETCH-LOGICAL-c385t-a2d9b389d31f3f2fa80bbbf3a290804b997e7e04d70aebb96f7d40e9cf464e673</originalsourceid><addsrcrecordid>eNpFkcFu2zAMhoViw5plu_ZY-DD0NLW0pVjSsSjabUCA7tCeBcmiGwW25Fp2gLx9lSXtDgQJ8iNB8ifkooTrElarm0QrAElL4EwIdkYWJWeKMlnxT2QBDIDKmsE5-ZrSFqDkCtQXcp5xELJUC_Lyd-76GMy4L3YYIo1N083J77BwPqFJ-LMwYfLDJqZsnR-8-5ew0e1z4Irho3-zH3CcMCQfQ-FDrhbGxQ5Tg2H6Rj63pkv4_eSX5Pnh_unuN10__vpzd7umDZOriZrKKcukcqxsWVu1RoK1tmWmUiCBW6UECgTuBBi0VtWtcBxQNS2vOdaCLcnVce4wxtcZ06R7nxfoOhMwzknXSkpVreoMXp7A2fbo9DD6Pl-h31-TgR8nwKTGdO1oQuPTf07UFVQyY_SITRuPPeptnMeQL8yD9EEhnfRBIX1SiL0B3-mDFA</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>69889256</pqid></control><display><type>article</type><title>Pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent</title><source>MEDLINE</source><source>Thieme Connect Journals</source><creator>Hussein, Ahmad ; Trowitzsch, Eckardt ; Brockmann, Michael</creator><creatorcontrib>Hussein, Ahmad ; Trowitzsch, Eckardt ; Brockmann, Michael</creatorcontrib><description>Abstract
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH has not been reported previously in children.A 12.5-year-old boy presented with a one year history of fatigue. Pulmonary hypertension was diagnosed by echocardiography. Pulmonary function tests revealed a moderate restrictive pattern and elevated granulocytes were detected in bronchoalveolar lavage. An isolated high-titer APL was detected. Open lung biopsy established the diagnosis of PVOD, with no evidence of pulmonary thrombosis, but with accompanying interstitial and alveolar cellular infiltration. We speculate that APL may have played a role in the pathogenesis of PVOD. Prednison improved the symptoms of the interstitial pneumonitis and was stopped; on follow up of 30 months, the patient ist in stable condition on therapy with nifedipin, phenprocoumon and digoxin.CONCLUSIONS PVOD and APL may be present simultaneously as a rare cause of PH. Interstitial pneumonitis may accompany PVOD and produce the leading symptoms. Open lung biopsy is essential for early establishment of the diagnosis.</description><identifier>ISSN: 0300-8630</identifier><identifier>EISSN: 1439-3824</identifier><identifier>DOI: 10.1055/s-2008-1043773</identifier><identifier>PMID: 10407819</identifier><identifier>CODEN: KLPDB2</identifier><language>eng</language><publisher>Stuttgart: Thieme</publisher><subject>Antiphospholipid Syndrome - complications ; Antiphospholipid Syndrome - drug therapy ; Biological and medical sciences ; Biopsy ; Child ; Digoxin - therapeutic use ; Fatigue - etiology ; Humans ; Hypertension, Pulmonary - diagnosis ; Hypertension, Pulmonary - etiology ; Hypertension, Pulmonary - prevention & control ; Lung - pathology ; Lung Diseases, Interstitial - complications ; Lung Diseases, Interstitial - diagnosis ; Lung Diseases, Interstitial - drug therapy ; Male ; Medical sciences ; Nifedipine - therapeutic use ; Phenprocoumon - therapeutic use ; Pneumology ; Prednisone - therapeutic use ; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases ; Respiratory Function Tests ; Treatment Outcome</subject><ispartof>Klinische Pädiatrie, 1999-03, Vol.211 (2), p.92-95</ispartof><rights>Georg Thieme Verlag KG Stuttgart · New York</rights><rights>1999 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c385t-a2d9b389d31f3f2fa80bbbf3a290804b997e7e04d70aebb96f7d40e9cf464e673</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.thieme-connect.de/products/ejournals/pdf/10.1055/s-2008-1043773.pdf$$EPDF$$P50$$Gthieme$$H</linktopdf><link.rule.ids>309,310,314,776,780,785,786,3005,23909,23910,25118,27901,27902,54534</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1762028$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10407819$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hussein, Ahmad</creatorcontrib><creatorcontrib>Trowitzsch, Eckardt</creatorcontrib><creatorcontrib>Brockmann, Michael</creatorcontrib><title>Pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent</title><title>Klinische Pädiatrie</title><addtitle>Klin Padiatr</addtitle><description>Abstract
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH has not been reported previously in children.A 12.5-year-old boy presented with a one year history of fatigue. Pulmonary hypertension was diagnosed by echocardiography. Pulmonary function tests revealed a moderate restrictive pattern and elevated granulocytes were detected in bronchoalveolar lavage. An isolated high-titer APL was detected. Open lung biopsy established the diagnosis of PVOD, with no evidence of pulmonary thrombosis, but with accompanying interstitial and alveolar cellular infiltration. We speculate that APL may have played a role in the pathogenesis of PVOD. Prednison improved the symptoms of the interstitial pneumonitis and was stopped; on follow up of 30 months, the patient ist in stable condition on therapy with nifedipin, phenprocoumon and digoxin.CONCLUSIONS PVOD and APL may be present simultaneously as a rare cause of PH. Interstitial pneumonitis may accompany PVOD and produce the leading symptoms. Open lung biopsy is essential for early establishment of the diagnosis.</description><subject>Antiphospholipid Syndrome - complications</subject><subject>Antiphospholipid Syndrome - drug therapy</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Child</subject><subject>Digoxin - therapeutic use</subject><subject>Fatigue - etiology</subject><subject>Humans</subject><subject>Hypertension, Pulmonary - diagnosis</subject><subject>Hypertension, Pulmonary - etiology</subject><subject>Hypertension, Pulmonary - prevention & control</subject><subject>Lung - pathology</subject><subject>Lung Diseases, Interstitial - complications</subject><subject>Lung Diseases, Interstitial - diagnosis</subject><subject>Lung Diseases, Interstitial - drug therapy</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Nifedipine - therapeutic use</subject><subject>Phenprocoumon - therapeutic use</subject><subject>Pneumology</subject><subject>Prednisone - therapeutic use</subject><subject>Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases</subject><subject>Respiratory Function Tests</subject><subject>Treatment Outcome</subject><issn>0300-8630</issn><issn>1439-3824</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1999</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkcFu2zAMhoViw5plu_ZY-DD0NLW0pVjSsSjabUCA7tCeBcmiGwW25Fp2gLx9lSXtDgQJ8iNB8ifkooTrElarm0QrAElL4EwIdkYWJWeKMlnxT2QBDIDKmsE5-ZrSFqDkCtQXcp5xELJUC_Lyd-76GMy4L3YYIo1N083J77BwPqFJ-LMwYfLDJqZsnR-8-5ew0e1z4Irho3-zH3CcMCQfQ-FDrhbGxQ5Tg2H6Rj63pkv4_eSX5Pnh_unuN10__vpzd7umDZOriZrKKcukcqxsWVu1RoK1tmWmUiCBW6UECgTuBBi0VtWtcBxQNS2vOdaCLcnVce4wxtcZ06R7nxfoOhMwzknXSkpVreoMXp7A2fbo9DD6Pl-h31-TgR8nwKTGdO1oQuPTf07UFVQyY_SITRuPPeptnMeQL8yD9EEhnfRBIX1SiL0B3-mDFA</recordid><startdate>19990301</startdate><enddate>19990301</enddate><creator>Hussein, Ahmad</creator><creator>Trowitzsch, Eckardt</creator><creator>Brockmann, Michael</creator><general>Thieme</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>19990301</creationdate><title>Pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent</title><author>Hussein, Ahmad ; Trowitzsch, Eckardt ; Brockmann, Michael</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c385t-a2d9b389d31f3f2fa80bbbf3a290804b997e7e04d70aebb96f7d40e9cf464e673</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1999</creationdate><topic>Antiphospholipid Syndrome - complications</topic><topic>Antiphospholipid Syndrome - drug therapy</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>Child</topic><topic>Digoxin - therapeutic use</topic><topic>Fatigue - etiology</topic><topic>Humans</topic><topic>Hypertension, Pulmonary - diagnosis</topic><topic>Hypertension, Pulmonary - etiology</topic><topic>Hypertension, Pulmonary - prevention & control</topic><topic>Lung - pathology</topic><topic>Lung Diseases, Interstitial - complications</topic><topic>Lung Diseases, Interstitial - diagnosis</topic><topic>Lung Diseases, Interstitial - drug therapy</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Nifedipine - therapeutic use</topic><topic>Phenprocoumon - therapeutic use</topic><topic>Pneumology</topic><topic>Prednisone - therapeutic use</topic><topic>Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases</topic><topic>Respiratory Function Tests</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hussein, Ahmad</creatorcontrib><creatorcontrib>Trowitzsch, Eckardt</creatorcontrib><creatorcontrib>Brockmann, Michael</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>Klinische Pädiatrie</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hussein, Ahmad</au><au>Trowitzsch, Eckardt</au><au>Brockmann, Michael</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent</atitle><jtitle>Klinische Pädiatrie</jtitle><addtitle>Klin Padiatr</addtitle><date>1999-03-01</date><risdate>1999</risdate><volume>211</volume><issue>2</issue><spage>92</spage><epage>95</epage><pages>92-95</pages><issn>0300-8630</issn><eissn>1439-3824</eissn><coden>KLPDB2</coden><abstract>Abstract
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH has not been reported previously in children.A 12.5-year-old boy presented with a one year history of fatigue. Pulmonary hypertension was diagnosed by echocardiography. Pulmonary function tests revealed a moderate restrictive pattern and elevated granulocytes were detected in bronchoalveolar lavage. An isolated high-titer APL was detected. Open lung biopsy established the diagnosis of PVOD, with no evidence of pulmonary thrombosis, but with accompanying interstitial and alveolar cellular infiltration. We speculate that APL may have played a role in the pathogenesis of PVOD. Prednison improved the symptoms of the interstitial pneumonitis and was stopped; on follow up of 30 months, the patient ist in stable condition on therapy with nifedipin, phenprocoumon and digoxin.CONCLUSIONS PVOD and APL may be present simultaneously as a rare cause of PH. Interstitial pneumonitis may accompany PVOD and produce the leading symptoms. Open lung biopsy is essential for early establishment of the diagnosis.</abstract><cop>Stuttgart</cop><pub>Thieme</pub><pmid>10407819</pmid><doi>10.1055/s-2008-1043773</doi><tpages>4</tpages></addata></record> |
fulltext | fulltext |
identifier | ISSN: 0300-8630 |
ispartof | Klinische Pädiatrie, 1999-03, Vol.211 (2), p.92-95 |
issn | 0300-8630 1439-3824 |
language | eng |
recordid | cdi_proquest_miscellaneous_69889256 |
source | MEDLINE; Thieme Connect Journals |
subjects | Antiphospholipid Syndrome - complications Antiphospholipid Syndrome - drug therapy Biological and medical sciences Biopsy Child Digoxin - therapeutic use Fatigue - etiology Humans Hypertension, Pulmonary - diagnosis Hypertension, Pulmonary - etiology Hypertension, Pulmonary - prevention & control Lung - pathology Lung Diseases, Interstitial - complications Lung Diseases, Interstitial - diagnosis Lung Diseases, Interstitial - drug therapy Male Medical sciences Nifedipine - therapeutic use Phenprocoumon - therapeutic use Pneumology Prednisone - therapeutic use Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases Respiratory Function Tests Treatment Outcome |
title | Pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent |
url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-20T10%3A43%3A36IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Pulmonary%20veno-occlusive%20disease,%20antiphospholipid%20antibody%20and%20pulmonary%20hypertension%20in%20an%20adolescent&rft.jtitle=Klinische%20P%C3%A4diatrie&rft.au=Hussein,%20Ahmad&rft.date=1999-03-01&rft.volume=211&rft.issue=2&rft.spage=92&rft.epage=95&rft.pages=92-95&rft.issn=0300-8630&rft.eissn=1439-3824&rft.coden=KLPDB2&rft_id=info:doi/10.1055/s-2008-1043773&rft_dat=%3Cproquest_pubme%3E69889256%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=69889256&rft_id=info:pmid/10407819&rfr_iscdi=true |