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Beckwith-Wiedemann syndrome

Summary of Beckwith-Wiedemann syndrome
Beckwith-Wiedemann syndrome is an overgrowth disorder characterized by an increased risk of childhood cancer. Patients with Beckwith-Wiedemann often present with Wilms tumor, macroglossia, organomegaly, and hemihypertrophy. The treatment medical treatment of choice includes octreotide and blood glucose maintenance. Surgical treatment may include nephrectomy or macroglossia reduction.




Renal system

Renal and ureteral disorders
Bladder and urethral disorders
Renal system pathology review

Beckwith-Wiedemann syndrome


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High Yield Notes
4 pages

Beckwith-Wiedemann syndrome

5 flashcards

USMLE® Step 1 style questions USMLE

1 questions

A 1-day newborn male infant is admitted into neonatal intensive care unit because of severe developmental anomalies. The baby was prematurely born to a gravida 1, para 0, mother via cesarean section following a pregnancy complicated by polyhydramnios. Upon further interrogation with the mother, she denies any hereditary disorders in the family. A physical exam shows an abdominal wall defect in which the intestines and liver are exposed (enclosed in visceral peritoneum) and an unusually large tongue.  An abdominal ultrasound shows hydronephrosis and a hyperechoic left-kidney mass.  His temperature is 36.7°C (98°F), pulse is 130/min, respirations are 30/min, blood pressure is 110/70 mmHg, weight is 5 kilograms (11 lb), and pulse oximetry on room air shows an oxygen saturation of 93%. Which of the following is the most likely diagnosis?

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