{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,1,14]],"date-time":"2025-01-14T05:23:25Z","timestamp":1736832205569,"version":"3.33.0"},"reference-count":13,"publisher":"Wiley","issue":"19","license":[{"start":{"date-parts":[[2022,4,19]],"date-time":"2022-04-19T00:00:00Z","timestamp":1650326400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Birth Defects Research"],"published-print":{"date-parts":[[2022,11,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Sirenomelia is a congenital malformation of the lower body characterized by a single midline lower limb and severe urogenital and gastrointestinal malformations. Sirenomelia is rare (estimated incidence of approximately 1\/100,000) and usually lethal in the perinatal period.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case<\/jats:title><jats:p>A 2,042\u2009g Japanese male infant, one of monochorionic monoamniotic twins, was born at 34\u2009weeks of gestation by elective caesarean section. Sirenomelia was prenatally diagnosed. Single midline lower limb, bilateral dysplastic kidneys, an omphalomesenteric fistula, colon atresia, imperforate anus, indiscernible genital structures, and myelomeningocele were detected at birth. The amniotic fluid volume was normal throughout the pregnancy course, which led to appropriate lung maturation of the twin with sirenomelia. Although renal replacement therapy was initiated soon after birth, stable peritoneal dialysis was difficult because of the limited intraperitoneal space, and the infant frequently developed peritonitis. He died of sudden cardiorespiratory arrest at 6\u2009months of age. Postmortem examination showed bilateral dysplastic kidneys, agenesis of the ureters and urinary bladder, abnormal branching and agenesis of the distal colon, bilateral inguinal hernias, and small testes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Infants with sirenomelia, even those with end\u2010stage kidney disease at birth, may survive if they have a stable cardiorespiratory status at birth and renal replacement therapy is appropriately initiated.<\/jats:p><\/jats:sec>","DOI":"10.1002\/bdr2.2016","type":"journal-article","created":{"date-parts":[[2022,4,19]],"date-time":"2022-04-19T07:16:37Z","timestamp":1650352597000},"page":"1286-1290","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Six\u2010month survival of a monochorionic monoamniotic twin with sirenomelia"],"prefix":"10.1002","volume":"114","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3148-9892","authenticated-orcid":false,"given":"Katsuya","family":"Hirata","sequence":"first","affiliation":[{"name":"Department of Neonatal Medicine Osaka Women's and Children's Hospital  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natsumi","family":"Yamamura\u2010Miyazaki","sequence":"additional","affiliation":[{"name":"Department of Pediatric Nephrology and Metabolism Osaka Women's and Children's Hospital  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haruna","family":"Kawaguchi","sequence":"additional","affiliation":[{"name":"Department of Maternal Fetal Medicine Osaka Women's and Children's Hospital  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Umeda","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Osaka Women's and Children's Hospital  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazue","family":"Moon","sequence":"additional","affiliation":[{"name":"Department of Intensive Care Medicine Osaka Women's and Children's Hospital  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuyoshi","family":"Chiba","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Osaka Women's and Children's Hospital  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masanori","family":"Nishikawa","sequence":"additional","affiliation":[{"name":"Department of Radiology Osaka Women's and Children's Hospital  Osaka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keiko","family":"Matsuoka","sequence":"additional","affiliation":[{"name":"Department of Pathology Osaka Women's and Children's Hospital  Osaka 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