{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T14:38:54Z","timestamp":1787755134920,"version":"build-2784847793"},"reference-count":27,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec>\n                    <jats:title>Case:<\/jats:title>\n                    <jats:p>A 14-year-old boy with dyskeratosis congenita (DKC), status-post unrelated bone marrow transplant (BMT), sustained a femoral shaft fracture. Despite immediate fixation with the widest possible titanium elastic nails, fixation construct rigidity was insufficient and malunion occurred with refracture 5 years later. Revision fixation with rigid intramedullary nailing has maintained position for 1 year, although callus formation remains meager.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>This is the first article to detail fracture care for a DKC patient. Although BMT increases lifespan, patients seemingly remain skeletally frail. Rigid intramedullary fixation is optimally durable and appears hematopoietically safe. Long-term follow-up is recommended.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.2106\/jbjs.cc.20.00486","type":"journal-article","created":{"date-parts":[[2021,4,21]],"date-time":"2021-04-21T15:00:24Z","timestamp":1619017224000},"page":"e20.00486","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["Difficult Management of a Femur Fracture in a Patient with Dyskeratosis Congenita"],"prefix":"10.2106","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7065-0656","authenticated-orcid":false,"given":"Jason Shih","family":"Hoellwarth","sequence":"first","affiliation":[{"name":"Baylor College of Medicine, Department of Pediatric Orthopaedics, Texas Children\u2019s Hospital, Houston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5724-5645","authenticated-orcid":false,"given":"Eric W.","family":"Edmonds","sequence":"additional","affiliation":[{"name":"Rady Children\u2019s Hospital San Diego, Division of Orthopaedic Surgery, San Diego, California"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2021,4]]},"reference":[{"key":"bib1-20240805","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1532\/IJH97.05067","article-title":"Dyskeratosis congenita: a disorder of defective telomere maintenance?","volume":"82","author":"Walne","year":"2005","journal-title":"Int J Hematol."},{"key":"bib2-20240805","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.mad.2007.10.006","article-title":"Dyskeratosis congenita: a historical perspective","volume":"129","author":"Walne","year":"2008","journal-title":"Mech Ageing Dev."},{"key":"bib3-20240805","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1007\/s11832-008-0149-2","article-title":"Complications with flexible nailing of femur fractures more than double with child obesity and weight >50 kg","volume":"3","author":"Weiss","year":"2009","journal-title":"J Child Orthop."},{"key":"bib4-20240805","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/1471-2326-11-3","article-title":"Lung transplantation for pulmonary fibrosis in dyskeratosis congenita: case report and systematic literature review","volume":"11","author":"Giri","year":"2011","journal-title":"BMC Blood Disord."},{"key":"bib5-20240805","doi-asserted-by":"crossref","first-page":"6549","DOI":"10.1182\/blood-2008-12-192880","article-title":"Cancer in dyskeratosis congenita","volume":"113","author":"Alter","year":"2009","journal-title":"Blood."},{"key":"bib7-20240805","first-page":"157","article-title":"The diagnosis and treatment of dyskeratosis congenita: a review","volume":"5","author":"Garc\u00eda","year":"2014","journal-title":"J Blood Med."},{"key":"bib9-20240805","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1111\/j.1601-5037.2009.00364.x","article-title":"Dyskeratosis congenita: clinical report and review of the literature","volume":"8","author":"Baran","year":"2010","journal-title":"Int J Dent Hyg."},{"key":"bib10-20240805","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/j.oraloncology.2005.06.007","article-title":"Dyskeratosis congenita","volume":"42","author":"Handley","year":"2006","journal-title":"Oral Oncol."},{"key":"bib11-20240805","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1001\/jama.1965.03080160023005","article-title":"Dyskeratosis congenita and familial pancytopenia","volume":"192","author":"Bryan","year":"1965","journal-title":"JAMA."},{"key":"bib12-20240805","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1001\/archpedi.1973.02110190337020","article-title":"Dyskeratosis congenita with pancytopenia. 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