Improving the Recognition of Temple Syndrome
Metabolic
Of 15 patients over the age of 11 years, 3 developed diabetes mellitus; at the ages of 12 years, 19 years and 20 years. All three patients were described as having features of type 2 diabetes, and one case was confirmed as negative for antipancreatic antibodies. Where treatment was described, oral hypoglycaemics were effective. BMI was significantly raised in two of the three patients (BMI 40.8 and 30.8); however, the third had a normal BMI of 24.1.
In contrast, two patients in the cohort were reported to have recurrent hypoglycaemia in early childhood (3–5 years). One of these patients subsequently was shown to be growth hormone deficient and was started on treatment. In the other patient, it was associated with a rapid gain in weight and ketosis.
Five patients were reported with evidence of hyperlipidaemia, hypertriglyceridaemia in one and hypercholesterolaemia in the others, although in one case there was a strong family history. One patient with hyperlipidaemia and one with hypercholesterolaemia were also diagnosed with diabetes (see above).