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January 8, 2001

Daily Care, Diagnosis and Symptoms

Question from a paediatritian in India:

I have an eight day old baby girl with the following findings: White blood count 23,800 cells/cmm C-reactive protein 6 mg/dl E.S.R. 13 after end of first hour Blood Glucose 447 mg/dl [24.8 mmol/L] (random by god-pod enzyme method) Serum Sodium 129 /mEq/L Serum Chloride 91 mEq/L Serum Bilirubin: total - 6.5; direct - 3.4, indirect 3.1 Please tell me the diagnostic criteria, follow-up protocol and treatment, and guide me for further investigation and management.

Answer:

The important laboratory observation is the elevated blood sugar level. If this was taken fasting and has been confirmed then the diagnosis of neonatal diabetes is assured. Something like half of these cases will resolve in the next few weeks, but exact figures vary because this is a very rare condition, and there are few series. The only way to distinguish the two conditions, apart from letting time tell, is to look for paternal disomy of chromosome 6. This requires some rather sophisticated cytogenetics which may well not be available to you. It is important to remember too that in the transient cases insulin dependent diabetes may recur in later years. The permanent condition is not caused by autoimmunity and may be associated with dysplasia not only of the beta cells, but of the exocrine pancreas which will mean enzyme supplementation.

The fundamentals of treatment are no different from diabetes at a later age, namely to keep the blood sugars in the 100 to 200mg/dl [5.6 to 11.1 mmol/L] range until it is known whether the condition is transient or permanent. This requires insulin, but it is particularly important to avoid serious hypoglycemia with its potential for central nervous system damage.

DOB