RT Journal Article
SR Electronic
T1 Congenital absence of islets of Langerhans.
JF Archives of Disease in Childhood
JO Arch Dis Child
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 411
OP 413
DO 10.1136/adc.52.5.411
VO 52
IS 5
A1 J A Dodge
A1 K M Laurence
YR 1977
UL http://adc.bmj.com/content/52/5/411.abstract
AB A small-for-dates male infant who developed acute metabolic acidosis shortly after birth had diabetes and died aged 40 hours. At necropsy there was an absence of any recognizable islets of Langerhans though lymphorecticular aggregates were found. This was the fourth child of healthy unrelated parents whose first child, also a male, died at 48 hours under similar circumstances. It is suggested that both boys had the same underlying pathology and this might be a previously undescribed recessive or x-linked inherited condition.