Neonatal hyperglycaemia: case report with plasma insulin studies.

The infant was breast-fed and sucked well. However, he failed to gain weight, and on the 15th day began to vomit occasionally. On the 19th day he was transferred to the Children's Ward for further investigation. He then weighed 2065 g., was marasmic, wide-eyed, and thirsty, with a rectal temperature of 39*4 'C.( Fig.). Blood sugar, 720 mg./100 ml.; CSF sugar, 405 mg./100 ml.; CSF protein, chloride, and cell count normal; blood urea, 77 mg./100 ml.; urine contained 2% sugar but no ketones.A subcutaneous infusion of 1/5 normal saline was given. All urine voided was collected and tested for sugar and acetone. Soluble insulin was given two or three times daily in doses ranging from 2 to 6 units.Ketones were never found in the urine. Administered insulin totalled 12 units daily for most of the first fortnight, but thereafter the dose gradually fell. No attempt was made to keep the urine entirely sugarfree because of the particular dangers of hypoglycaemia in infancy. However, at 5 weeks a brief hypoglycaemic convulsion occurred which was terminated by intravenous glucose. From the start of treatment there was immediate clinical improvement and a steady gain in weight.At 6 weeks intermittent vomiting, abdominal distension, fever, and diarrhoea developed, associated on three occasions (2, 3, and 6 January 1964) with serum potassium over 7 mEq/l., and with low sodium and chloride levels. On these dates blood sugar levels of 550, 770, and 60 mg./100 ml. were recorded. The 24-hour urinary 17-oxosteroid and total oxogenic steroid excretion (5-6 January) were 0 * 15 and 0 * 64 mg., respectively. Blood urea was 30 mg./100 ml. and blood culture was negative. There was a tachycardia of 200/min., and the systolic blood pressure in the arm by the flush technique was 75 mm. Hg. Adrenal insufficiency was suspected and desoxycorticosterone acetate 1 mg. daily and subcutaneous 1/5 normal saline were administered from [7][8][9][10]

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“…The majority of the infants were born small for gestational age with a median birth weight of 2.1 kg below the 0.4th percentile (range 1.60–3.37 kg). Marked hyperglycaemia occurs in the face of minimal ketosis [2, 3, 4, 5, 6], with peak blood sugar levels remarkably high, at times greater than 70 mmol/l [2]. In our cohort 11 infants (37%) had insulin or ‘C’ peptide levels estimated at the time of initial diagnosis, and universally these have shown absent or minimal insulin responses in the face of severe hyperglycaemia, suggesting the primary condition is due to inadequate production and/or secretion of insulin.…”

Section: Clinical Characteristicsmentioning

confidence: 99%

Neonatal Diabetes: New Insights into Aetiology and Implications

Shield¹

2000

Horm Res Paediatr

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Neonatal diabetes mellitus (NDM) is defined as hyperglycaemia occurring in the first few weeks of life. It can be either transient (TNDM) or permanent (PNDM), and until recently, little was known about the condition. A cohort of 30 infants with a history of TNDM has been studied, and findings have suggested that NDM does not have the same aetiology as classical type 1 childhood diabetes. Uniparental isodisomy of chromosome 6 and an unbalanced duplication of paternal chromosome 6 have both been described as a genetic basis for TNDM in over 75% of the cases. In addition, cerebellar hypoplasia and Walcott-Rallison syndrome have been associated with PNDM, suggesting an autosomal recessive inheritance pattern; furthermore, a mutation in the gene insulin promoter factor 1 has been identified as a cause of pancreatic agenesis in PNDM. In the long term, TNDM may reduce beta cell functional capacity and present a predisposition to type 2 diabetes mellitus.

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Neonatal hyperglycaemia: case report with plasma insulin studies.

Cited by 12 publications

References 18 publications

A new type of transient diabetes mellitus of infancy?

A new type of transient diabetes mellitus of infancy?

Transient Neonatal Diabetes Mellitus in Sibs

Neonatal Diabetes: New Insights into Aetiology and Implications

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