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...

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Veröffentlicht in:Klinische Pädiatrie 1999-03, Vol.211 (2), p.92-95
Hauptverfasser: Hussein, Ahmad, Trowitzsch, Eckardt, Brockmann, Michael
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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
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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 &amp; 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. 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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 &amp; 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. 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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 &amp; 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>
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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
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