{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,24]],"date-time":"2026-01-24T16:25:11Z","timestamp":1769271911358,"version":"3.49.0"},"reference-count":15,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,7,19]],"date-time":"2023-07-19T00:00:00Z","timestamp":1689724800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100007786","name":"National Center for Child Health and Development","doi-asserted-by":"publisher","award":["2019A\u20104"],"award-info":[{"award-number":["2019A\u20104"]}],"id":[{"id":"10.13039\/100007786","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["obgyn.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Obstet and Gynaecol"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mucopolysaccharidosis (MPS)\u2010VII, called Sly disease, is a lysosomal storage disorder that can cause fetal hydrops, including fetal hydrothorax (FHT). We describe two fetal cases that received thoracoamniotic shunting for FHT, which was later found to be associated with MPS\u2010VII by exome sequencing. Bilateral FHT accompanied by skin edema and ascites was found before 20\u2009weeks of gestation in both cases. One fetus died in utero at 35\u2009weeks of gestation, and the other survived with preterm delivery at 30\u2009weeks of gestation. Both cases inherited compound pathogenic variants of <jats:italic>GUSB<\/jats:italic> from parents. Comparison with previously reported primary FHT cases revealed distinct clinical features in MPS\u2010VII\u2010associated FHT: early gestational age at diagnosis (&lt;26\u2009weeks), bilateral effusion, skin edema with ascites, and poor survival. A genetic analysis would be considered for FHT cases, with consideration of shunting when they show early\u2010onset bilateral effusions with skin edema and ascites.<\/jats:p>","DOI":"10.1111\/jog.15744","type":"journal-article","created":{"date-parts":[[2023,7,20]],"date-time":"2023-07-20T04:05:29Z","timestamp":1689825929000},"page":"2538-2543","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Clinical features of fetal hydrothorax associated with mucopolysaccharidosis\u2010<scp>VII<\/scp>"],"prefix":"10.1111","volume":"49","author":[{"given":"Yuki","family":"Kamihara","sequence":"first","affiliation":[{"name":"Division of Fetal Medicine, Center for Maternal\u2010Fetal, Neonatal and Reproductive Medicine National Center for Child Health and Development  Tokyo 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