{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T02:37:44Z","timestamp":1761100664117},"reference-count":23,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2003,11,5]],"date-time":"2003-11-05T00:00:00Z","timestamp":1067990400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2004,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Malignant infantile osteopetrosis (MIOP), a rare genetic disorder of the osteoclast, is fatal without hematopoietic stem cell transplantation. Primary pulmonary hypertension (PPH), a rare progressive disorder of the pulmonary circulation, is predominately fatal in the absence of successful therapy. A clinical association between these two disorders has not been recognized and a pathophysiologic link between osteoclast function and pulmonary vascular pressure as a rationale for such an association is not readily apparent. Here, we report five infants with MIOP, without cardiac abnormalities, who were found to have PPH after undergoing stem cell transplantation. We suggest that PPH may be linked to a specific variant of MIOP and recognizing the potential for pulmonary hypertension in children with MIOP may lead to a more rapid diagnosis and life\u2010saving intervention. \u00a9 2003 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/pbc.10455","type":"journal-article","created":{"date-parts":[[2004,1,9]],"date-time":"2004-01-09T09:01:56Z","timestamp":1073638916000},"page":"190-194","source":"Crossref","is-referenced-by-count":15,"title":["Malignant infantile osteopetrosis &amp; primary pulmonary hypertension: A new combination?"],"prefix":"10.1002","volume":"42","author":[{"given":"Kimberly A.","family":"Kasow","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carmem","family":"Bonfim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julie","family":"Asch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David A.","family":"Margolis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jesse","family":"Jenkins","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert F.","family":"Tamburro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rupert","family":"Handgretinger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edwin M.","family":"Horwitz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2003,11,5]]},"reference":[{"issue":"2","key":"e_1_2_1_2_2","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1542\/peds.93.2.247","article-title":"Autosomal recessive osteopetrosis: Variability of findings at diagnosis and during the natural course","volume":"93","author":"Gerritsen EJ","year":"1994","journal-title":"Pediatrics"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.3949\/ccjm.67.3.175"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.162.17.1925"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.113.3.377"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.83.5.449"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199506153322402"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198402163100701"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.61.7.666"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-3046.1999.00063.x"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(91)90176-F"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/77131"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.17.1767"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.25.2861"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1038\/nm842"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1038\/79226"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.121.3_suppl.50S"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa021650"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1097\/00008480-199906000-00008"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1177\/000992288802700209"},{"issue":"1","key":"e_1_2_1_21_2","first-page":"103","article-title":"Circulating macrophage colony\u2010stimulating factor is not reduced in malignant osteopetrosis","volume":"20","author":"Orchard PJ","year":"1992","journal-title":"Exp Hematol"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.2001.280.6.L1148"},{"issue":"4","key":"e_1_2_1_23_2","first-page":"L715","article-title":"GM\u2010CSF enhances lung growth and causes alveolar type II epithelial cell hyperplasia in transgenic mice","volume":"273","author":"Huffman Reed JA","year":"1997","journal-title":"Am J Physiol"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.0000012303.37971.DA"}],"container-title":["Pediatric Blood &amp; 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