Background. Neonatal diabetes mellitus is rare (60 cases reported to d
ate) and usually transient. Acute dehydration or gradual onset of poly
uria and failure to thrive are the most common presentations. Brittlen
ess is the rule, and special care should be taken to avoid insulin-ind
uced hypoglycemia, which is particularly dangerous in neonates. Case-r
eports: Two small-for-dates neonates developed diabetes mellitus. Seve
re acute dehydration, metabolic acidosis, polyuria, and massive glycos
uria revealed the disease at the 17th day of life in one patient, in w
hom an infection was apparently a precipitating factor, and insulin th
erapy was required until the age of eight months. The second patient w
as admitted at birth for growth retardation and hypoglycemia, and subs
equently developed failure to thrive, hyperglycemia, and glycosuria. T
hese manifestations resolved under Insulin therapy, which was given fo
r 53 days. Neither patient had a family history for diabetes mellitus
or positive tests for anti-insulin antibodies. The second patient carr
ied the DR3 haplotype. Conclusion: Whereas the diagnosis of neonatal d
iabetes mellitus is readily established, the prognosis is difficult to
determine since no factors capable of predicting whether the disease
will be transient or permanent have been identified to date. Heated de
bate surrounds the pathophysiology of this disease, which may involve
immaturity of pancreatic beta cell function.