{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,29]],"date-time":"2025-10-29T06:18:37Z","timestamp":1761718717970},"reference-count":10,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:p>Incontinentia pigmenti (IP) is a multisystemic disorder in which pulmonary arterial hypertension (PAH) is a severe and rarely reported association. The prognosis has been poor in reported cases. In our patient, IP was diagnosed during the neonatal period with a combination of cutaneous, ophthalmic, and neurological symptoms. The infant experienced severe collapse with bradycardia during general anesthesia to treat retinal telangiectasia. Echocardiography after resuscitation revealed suprasystemic pulmonary hypertension (PH). Right heart catheterization (RHC) confirmed precapillary PAH not responding to acute vasodilatation test. Lung biopsy was performed to exclude alveolo\u2010capillary dysplasia. Upfront triple therapy with endothelin receptor antagonist, PDE5\u00a0inhibitors, and prostacyclin was started. Due to a potential inflammatory mechanism of this acute PAH in the setting of IP, TNF\u2010alpha blockers and steroids were associated. The evolution was favorable with progressive normalization of the pulmonary artery pressure confirmed by RHC after six months. Doses of PAH drugs were tapered down, and finally all PAH treatments could be stopped after 18\u00a0months. Subsequent controls including physical exams and echocardiograms did not show signs of PH. This unusual reversible case of pediatric PAH without associated congenital heart disease or portal hypertension highlights the potential reversibility of pediatric PH when an inflammatory mechanism can be suspected. This is the first reported case of non\u2010fatal isolated PAH associated with IP.<\/jats:p>","DOI":"10.1177\/2045894018793983","type":"journal-article","created":{"date-parts":[[2018,7,23]],"date-time":"2018-07-23T16:17:36Z","timestamp":1532362656000},"page":"1-3","update-policy":"http:\/\/dx.doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":6,"title":["A case of reversible pulmonary arterial hypertension associated with incontinentia pigmenti"],"prefix":"10.1002","volume":"8","author":[{"given":"Veronique","family":"Atallah","sequence":"first","affiliation":[{"name":"Department of Pediatric Cardiology Necker Enfants Malades Hospital Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mathilde","family":"Meot","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology Necker Enfants Malades Hospital Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manoelle","family":"Kossorotoff","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology Necker Enfants Malades Hospital Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Louise","family":"Galmiche\u2010Rolland","sequence":"additional","affiliation":[{"name":"Department of Pathology Necker Enfants Malades Hospital Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claude","family":"Lardeux","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology Necker Enfants Malades Hospital Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benedicte","family":"Neven","sequence":"additional","affiliation":[{"name":"Department of Immunology Necker Enfants Malades Hospital Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"Bodemer","sequence":"additional","affiliation":[{"name":"Department of Dermatology National Reference Centre for Genodermatoses (MAGEC) Necker Enfants malades Hospital Paris France"},{"name":"UMR 1163, Imagine Institute University Ren\u00e9 Descartes PRES Sorbonne Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Damien","family":"Bonnet","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology Necker Enfants Malades Hospital Paris France"},{"name":"UMR 1163, Imagine Institute University Ren\u00e9 Descartes PRES Sorbonne Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,9]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/ped.12831"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jp.7211250"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30698"},{"key":"e_1_2_7_5_1","first-page":"773","article-title":"[Cerebral complications of incontinentia pigmenti. A clinicopathological study of a case]","volume":"148","author":"Triki C","year":"1992","journal-title":"Rev Neurol (Paris)"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1525-1470.2001.018001054.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.2147\/TCRM.S134705"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1161\/STROKEAHA.116.012888"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.06-2243"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddi470"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201408-1509OC"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894018793983","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1177\/2045894018793983","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894018793983","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,28]],"date-time":"2023-08-28T08:43:47Z","timestamp":1693212227000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1177\/2045894018793983"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9]]},"references-count":10,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2018,10]]}},"alternative-id":["10.1177\/2045894018793983"],"URL":"https:\/\/doi.org\/10.1177\/2045894018793983","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9]]}}}