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HomePediatrics: General MedicineBeckwith-Wiedemann Syndrome

Beckwith-Wiedemann Syndrome

Practice Essentials

Beckwith-Wiedemann Syndrome (BWS) was first characterized by Dr. J. Bruce Beckwith and Dr. Hans-Rudolf Wiedemann in the early 1960s. Patients were first noted to have abdominal wall defects, macrosomia, macroglossia, and enlarged adrenal glands. Since then, clinical presentation has expanded to recognize hemihypertrophy/lateralized overgrowth, hyperinsulinism, omphalocele, and organomegaly as classic features of BWS. Additionally, it is now recognized that there is a range of clinical features seen in patients with BWS. Presentation of BWS occurs on a spectrum ranging from isolated asymmetry to classic features of BWS.

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