{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,15]],"date-time":"2023-10-15T05:10:30Z","timestamp":1697346630845},"reference-count":15,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2006,5,2]],"date-time":"2006-05-02T00:00:00Z","timestamp":1146528000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2006,6]]},"abstract":"<jats:p><jats:bold>Abstract:\u2002<\/jats:bold> Bilateral pulmonary agenesis (PA) is a rare embryological defect incompatible with life. Unilateral PA has a wide range of clinical presentations: its prognosis depends on the presence and severity of other associated anomalies. Fetal biliary atresia has been associated with a number of congenital anomalies, but the etiology is still not understood. An unusual case of a child with right PA, right diaphragmatic hernia, and delayed diagnosed biliary atresia leading to liver failure is presented herein. At the age of 4 months the patient was referred to the Transplant Department at Children Healthcare of Atlanta at Egleston with cholestasis and failure to thrive. With a rapidly progressive liver insufficiency, this child was evaluated for liver transplantation. In the absence of any respiratory symptom, the patient received a deceased donor size\u2010matched left lateral segment liver transplant, which covered the diaphragmatic defect, with no further repair required. Twenty\u2010seven months post\u2010transplant, the patient has good graft function, a normal <jats:italic>Z<\/jats:italic>\u2010score and is thriving. In spite of the increased physiological and surgical challenges (absence of right lung tissue, hemi\u2010diaphragm, and ectopic position of the liver in the right chest), liver transplantation was performed with positive outcome in this high\u2010risk child. Whether PA, may have developmentally contributed to expression of biliary atresia will need further investigation.<\/jats:p>","DOI":"10.1111\/j.1399-3046.2006.00502.x","type":"journal-article","created":{"date-parts":[[2006,5,3]],"date-time":"2006-05-03T04:41:02Z","timestamp":1146631262000},"page":"513-516","source":"Crossref","is-referenced-by-count":1,"title":["Liver transplant in a four\u2010month\u2010old child with biliary atresia, unilateral pulmonary agenesis, and diaphragmatic hernia: First case report"],"prefix":"10.1111","volume":"10","author":[{"given":"Thomas","family":"Heffron","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Welch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Todd","family":"Pillen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark","family":"Guy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Massimo","family":"Asolati","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Phil","family":"Hagedorn","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"George","family":"Atkinson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlos","family":"Fasola","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rene","family":"Romero","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,5,19]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.37.7.557"},{"key":"e_1_2_5_3_2","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1097\/00007611-199703000-00015","article-title":"Unilateral pulmonary agenesis without mediastinal displacement","volume":"90","author":"Rogue AS","year":"1997","journal-title":"Southern Med J"},{"key":"e_1_2_5_4_2","first-page":"291","article-title":"Pulmonary agenesis associated with fourteen other congenital abnormalities","volume":"44","author":"Courtney SP","year":"1990","journal-title":"BJCP"},{"key":"e_1_2_5_5_2","volume-title":"Diagnosis and Management of the Fetal Patient. Chapters 38, 40. Diaphragmatic Hernia","author":"Bianchi DW","year":"2000"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.cpm.0000117687.17714.d9"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.TP.0000065740.69296.DA"},{"key":"e_1_2_5_8_2","first-page":"180","article-title":"Biliary atresia","volume":"147","author":"Utterson EC","year":"2005","journal-title":"clinical profiles, risk factors and outcomes of 755 patients listed for liver transplantation"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-200502000-00012"},{"key":"e_1_2_5_10_2","first-page":"1546","article-title":"Conventional treatment for biliary atresia","volume":"31","author":"Valayer J.","year":"1996","journal-title":"long term results"},{"key":"e_1_2_5_11_2","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1111\/j.1399-3046.2005.00328.x","article-title":"From whence does biliary atresia arise?","volume":"9","author":"Squires SH.","year":"2004","journal-title":"Pediatr Transplantation"},{"key":"e_1_2_5_12_2","first-page":"1297","article-title":"Extrahepatic biliary atresia","volume":"35","author":"Perlmutter DH","year":"2002","journal-title":"a disease or a phenotype?"},{"key":"e_1_2_5_13_2","first-page":"4","article-title":"Pathogenesis and outcome of biliary atresia","volume":"37","author":"Sokol RJ","year":"2003","journal-title":"current concepts"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.1053\/jpsu.2002.30242"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1097\/00007890-199707270-00011"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3468(93)90516-N"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1399-3046.2006.00502.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1399-3046.2006.00502.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,14]],"date-time":"2023-10-14T18:36:31Z","timestamp":1697308591000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1399-3046.2006.00502.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,5,19]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2006,6]]}},"alternative-id":["10.1111\/j.1399-3046.2006.00502.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1399-3046.2006.00502.x","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,5,19]]}}}