pancreatic anomalies in BWS
Synospsis
pancreatic anomalies
- pancreatic enlargement
- pancreatic cystic dysplasia
- pancreatic ductular proliferation
- virtual absence of normal exocrine tissue
- increase in endocrine pancreatic tissue (islet cell hyperplasia)
References
Muguerza R, Rodriguez A, Formigo E, Montero M, Vazquez JL, Paramo C, Campos C. Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature. J Pediatr Surg. 2005 Aug;40(8):1341-4. PMID: 16080945
Hussain K, Cosgrove KE, Shepherd RM, Luharia A, Smith VV, Kassem S, Gregory JW, Sivaprasadarao A, Christesen HT, Jacobsen BB, Brusgaard K, Glaser B, Maher EA, Lindley KJ, Hindmarsh P, Dattani M, Dunne MJ. Hyperinsulinemic hypoglycemia in Beckwith-Wiedemann syndrome due to defects in the function of pancreatic beta-cell adenosine triphosphate-sensitive potassium channels. J Clin Endocrinol Metab. 2005 Jul;90(7):4376-82. PMID: 15811927
Steigman CK, Uri AK, Chatten J, Peckham GJ. Beckwith-Wiedemann syndrome with unusual hepatic and pancreatic features: a case expanding the phenotype. Pediatr Pathol. 1990;10(4):593-600. PMID: 2371181
Drut R, Jones MC. Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association. Pediatr Pathol. 1988;8(3):331-9. PMID: 2845376
Stefan Y, Bordi C, Grasso S, Orci L. Beckwith-Wiedemann syndrome: a quantitative, immunohistochemical study of pancreatic islet cell populations. Diabetologia. 1985 Dec;28(12):914-9. PMID: 2868957