Beckwith-Wiedemann Syndrome (EMG Syndrome) Observed in Three Cases With Antenatally Diagnosed Omphaloceles

DOI 13 References
  • Tajiri Tatsurou
    Department of Pediatric Surgery, Faculty of Medicine, Kyushu University
  • Suita Sachiyo
    Department of Pediatric Surgery, Faculty of Medicine, Kyushu University
  • Kubota Masayuki
    Department of Pediatric Surgery, Faculty of Medicine, Kyushu University
  • Taguchi Tomoaki
    Department of Pediatric Surgery, Faculty of Medicine, Kyushu University

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  • 臍帯ヘルニアの出生前診断をうけた Beckwith-Wiedemann 症候群 (EMG 症候群) の3例

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We experienced three typical cases of Beckwith-Wiedemann sundrome (EMG syndrome) . In common to three cases, omphalocele was antenatally found. At birth, the large statue for gastational age, the macroglossia and hypoglycemia were recognized. Type of omphalocele was a hernia into the umbilical cords and primary repair was performed on the first post-natal day in all three cases. Degree of hypoglycemia varied considerably among patients and a proper intravenous glucose infusion was necessary in each case. Three cases are alive without development of malignancies and now became 11-year-old, 7-year-old, 8-month-old, respectively. In antenatally diagnosed cases with omphalocele, association of Beckwith-Wiedemann syndrome should be carefully investigated to prevent irreversible hypoglycemia and to follow up the organ carcinogenesis.

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