Letters to the Editor
More on the Beckwith-Wiedemann syndrome
Sir,
We read with interest the article on Beckwith-Wiedemann syndrome: a quantitative, immunohistochemical study of pancreatic islet cell populations by Stefan et al. [1].
The authors suggest that the Beckwith-Wiedemann (BW) syndrome is a separate entity of neonatal hyperinsulinaemic hypoglycaemia characterized by a massive increase in insulin and glucagon cells together with a defect in glucagon and PP-cell segregation to distinct pancreatic regions.
We recently delivered a baby with the BW syndrome, already prenatally suspected by a combination of polyhydramnios, omphalocoele and pronounced oedema of the placenta.
In order to obtain information on lung maturity, an amniocentesis was performed at 34 weeks of gestation. C-peptide was 0.25 pmol/ml and insulin 51xU/ml of amniotic fluid, both values being low. The mother went into spontaneous labour at 35 weeks of gestation. Polyhydramnios of more than 31 was present. The birthweight was 3,500 g. The baby, a boy, survived and had the typical aspect of the BW syndrome. During the first day of life, blood Dextrostix values were around 1.8 retool/1 under a continuous glucose infusion of about 4mg-kg-l-min -1. Plasma insulin was 8 ~xU per ml and C-peptide 0.47 pmol per ml.
Although our patient had the typical aspect of the BW syndrome with marked macrosomia, amniotic and plasma insulin and C-peptide levels were not excessive during fetal and neonatal life respectively. There was also no hypoglycaemia under administration of glucose in usual dosages [1].