Eur J Pediatr. 1981 Feb;135(3):319-24.

A variant of the Wiedemann-Beckwith syndrome.

Abstract

The following combination of findings were established by histological examination of biopsy or autopsy material in an 11-day-old male baby: congenital mesoblastic nephroma, nodular renal blastema, dysgenetic pancreatic cyst, "cytomegaly" of the adrenal cortex, hyperplasia and hypertrophy of the islets of Langerhans, hypoplasia of the thymus and the lymphatic tissue. Since some of these changes constitute the characteristic histological findings in the Wiedemann-Beckwith syndrome and the remainder can be readily reconciled with it, the assignment of this case to this syndrome is discussed. Although the three cardinal symptoms are lacking, the combination of findings are interpreted as a variant of the wiedemann-Beckwith syndrome.

PMID:: 6262083; [PubMed - indexed for MEDLINE]

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[Mediastinal neuroblastoma in Wiedemann-Beckwith syndrome]. [Monatsschr Kinderheilkd. 1989]
[Mediastinal neuroblastoma in Wiedemann-Beckwith syndrome].
Huber A, Gutjahr P. Monatsschr Kinderheilkd. 1989 Apr; 137(4):243-4.
[The Beckwith-Wiedemann syndrome. Six observations with three familial forms (author's transl)]. [Ann Pediatr (Paris). 1981]
[The Beckwith-Wiedemann syndrome. Six observations with three familial forms (author's transl)].
Labbe A, Demeocq F, Gaulme J, Masson A, Malpuech G. Ann Pediatr (Paris). 1981 Nov; 28(9):665-70.
The Beckwith-Wiedemann syndrome in Nigerian infants (exomphalos, macroglossia and gigantism). [East Afr Med J. 1981]
The Beckwith-Wiedemann syndrome in Nigerian infants (exomphalos, macroglossia and gigantism).
Adeyokunnu AA, Adeniyi A. East Afr Med J. 1981 Sep; 58(9):684-90.
Review Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association. [Pediatr Pathol. 1988]
Review Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association.
Drut R, Jones MC. Pediatr Pathol. 1988; 8(3):331-9.
Review Beckwith-Wiedemann syndrome: historical, clinicopathological, and etiopathogenetic perspectives. [Pediatr Dev Pathol. 2005]
Review Beckwith-Wiedemann syndrome: historical, clinicopathological, and etiopathogenetic perspectives.
Cohen MM Jr. Pediatr Dev Pathol. 2005 May-Jun; 8(3):287-304. Epub 2005 Jul 14.

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Cited by 1 PubMed Central article

The cell type-specific IGF2 expression during early human development correlates to the pattern of overgrowth and neoplasia in the Beckwith-Wiedemann syndrome. [Am J Pathol. 1994]
The cell type-specific IGF2 expression during early human development correlates to the pattern of overgrowth and neoplasia in the Beckwith-Wiedemann syndrome.
Hedborg F, Holmgren L, Sandstedt B, Ohlsson R. Am J Pathol. 1994 Oct; 145(4):802-17.

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