A variant of the Wiedemann-Beckwith syndrome.
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.
D000311:Adrenal Glands / Q000473:pathology; D005877:Gigantism / Q000175:diagnosis*; D006554:Hernia, Umbilical / Q000175:diagnosis*; D006801:Humans; D007231:Infant, Newborn; D007515:Islets of Langerhans / Q000473:pathology; D007680:Kidney Neoplasms / Q000151:congenital; D008208:Lymphatic System / Q000473:pathology; D008260:Macroglossia / Q000175:diagnosis*; D008297:Male; D010181:Pancreatic Cyst / Q000151:congenital; D013577:Syndrome; D013950:Thymus Gland / Q000473:pathology; D009396:Wilms Tumor / Q000151:congenital