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2013•Nepalese Journal of RadiologyRequires access

The Role of Radiopathological Evaluation in Diagnosis of Beckwith-Wiedemann Syndrome

Kiran Gangadhar, Deepa Santhosh, Ishwar Chandra Maurya

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Abstract

Beckwith-Wiedemann Syndrome (BWS), an overgrowth syndrome is associated with early development of embryonal tumours usually in the first four years of life. Hepatoblastoma is observed in 1% to 3% of persons with Beckwith-Wiedemann Syndrome or isolated hemihypertrophy(HH). We report a case of hepatoblastoma associated with both BWS and HH with raised serum alpha-feto protein levels and hypoglycaemia. Nepalese Journal of Radiology; Vol. 2; Issue 2; July-Dec. 2012; 86-91 DOI: http://dx.doi.org/10.3126/njr.v2i2.7693

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What this paper is about

Beckwith-Wiedemann Syndrome (BWS), an overgrowth syndrome is associated with early development of embryonal tumours usually in the first four years of life. Hepatoblastoma is observed in 1% to 3% of persons with Beckwith-Wiedemann Syndrome or isolated hemihypertrophy(HH). We report a case of hepatoblastoma associated with both BWS and HH with raised serum alpha-feto protein levels and hypoglycaemia. Nepalese Journal of Radiology; Vol. 2; Issue 2; July-Dec. 2012; 86-91 DOI: http://dx.doi.org/10.3126/njr.v2i2.7693

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Available abstract

Beckwith-Wiedemann Syndrome (BWS), an overgrowth syndrome is associated with early development of embryonal tumours usually in the first four years of life. Hepatoblastoma is observed in 1% to 3% of persons with Beckwith-Wiedemann Syndrome or isolated hemihypertrophy(HH). We report a case of hepatoblastoma associated with both BWS and HH with raised serum alpha-feto protein levels and hypoglycaemia. Nepalese Journal of Radiology; Vol. 2; Issue 2; July-Dec. 2012; 86-91 DOI: http://dx.doi.org/10.3126/njr.v2i2.7693

Key concepts: Beckwith–Wiedemann syndrome, Hepatoblastoma, Hemihypertrophy, Medicine, Pediatrics, Internal medicine, Endocrinology, Pathology

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