{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T13:39:41Z","timestamp":1790429981836,"version":"4.1.0"},"reference-count":31,"publisher":"European Respiratory Society (ERS)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2002,1,7]]},"published-print":{"date-parts":[[2002,7]]},"abstract":"<jats:p>A case of pulmonary arterial hypertension in a patient with type-Ia glycogen-storage disease, a rare autosomal recessive disorder caused by a deficiency of glucose-6-phosphatase is reported in this study. It has been suggested that the occurrence of pulmonary arterial hypertension in type-Ia glycogen-storage disease could be due to an abnormal production of vasoconstrictive amines such as serotonin.<\/jats:p>\n                  <jats:p>To test this hypothesis, plasma serotonin concentrations were prospectively measured in 13 patients with type-Ia glycogen-storage disease, one patient with severe pulmonary hypertension and type-Ia glycogen-storage disease, 16 patients displaying severe pulmonary arterial hypertension, and 26 normal healthy controls.<\/jats:p>\n                  <jats:p>\n                    Elevated plasma serotonin concentrations were found in patients with either severe pulmonary arterial hypertension (38.8\u00b17.3\u2005nmol\u00b7L\n                    <jats:sup>\u22121<\/jats:sup>\n                    ) or type-Ia glycogen-storage disease (36.8\u00b111.5\u2005nmol\u00b7L\n                    <jats:sup>\u22121<\/jats:sup>\n                    ), as compared with controls (8.8\u00b10.6\u2005nmol\u00b7L\n                    <jats:sup>\u22121<\/jats:sup>\n                    , p&lt;0.001). Plasma serotonin was dramatically elevated in the patient with type-Ia glycogen-storage disease and pulmonary arterial hypertension (113.4\u2005nmol\u00b7L\n                    <jats:sup>\u22121<\/jats:sup>\n                    ).\n                  <\/jats:p>\n                  <jats:p>It is concluded that type-Ia glycogen-storage disease may be another condition in which abnormal handling of serotonin is one event in a multistep process leading to severe pulmonary arterial hypertension.<\/jats:p>","DOI":"10.1183\/09031936.02.00258702","type":"journal-article","created":{"date-parts":[[2002,10,1]],"date-time":"2002-10-01T19:21:54Z","timestamp":1033500114000},"page":"59-65","source":"Crossref","is-referenced-by-count":42,"title":["Pulmonary arterial hypertension and type-I glycogen-storage disease: the serotonin hypothesis"],"prefix":"10.1183","volume":"20","author":[{"given":"M.","family":"Humbert","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Labrune","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O.","family":"Sitbon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Le Gall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Callebert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Herv\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Samuel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Machado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Trembath","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Drouet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J-M.","family":"Launay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Simonneau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2002,7,1]]},"reference":[{"key":"2024101809373074000_20.1.59.1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.104.1.236"},{"key":"2024101809373074000_20.1.59.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-5231(05)70284-7"},{"key":"2024101809373074000_20.1.59.3","unstructured":"Chen YT, Burchell A. 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