The aim of the present study was to obtain a comprehensive picture of the rate of insulin secretion and of tissue sensitivity to the endogenous hormone in myotonic dystrophy patients (MyD). The minimal model approach was utilized for the analysis of frequently sampled intravenous glucose tolerance test data (FSIGT). This method provided the characteristic parameters: Si, insulin sensitivity index; Sq fractional glucose disappearance independent of dynamic insulin; n, fractional insulin clearance; φ1 and φ 2 first and second phase insulin delivery sensitivities to glucose stimulation. In MyD patients S(1) was reduced (p < 0.01) by 71% to 1.4 ± 0.3 X 10^-4 min^-1/(μU/ml), whereas in controls it was 4.85 ± 0.77; Sg was within the normal range: 0.044 ± 0.012 min^-1 in MyD patients and 0.036 ± 0.017 min^-1 in controls; φ 1 increased in MyD patients (7.4 ± 1.3 min (μU/ml)/(mg/dl) versus 4.1 ± 1.2 in controls); φ 2 increased in MyD patients (126 ± 47 X 104 min-2/(μU/ml)/(mg/dl) versus 17 ± 6 in controls; p < 0.05). MyD patients showed a normal tolerance with the glucose disappearance constant, Kq within the normal range: 2.75 versus 2.62% min'1 in controls. In MyD patients insulin resistance was associated with a higher than normal insulin delivery for both secretory phases, although the second phase was responsable for releasing a greater amount of hormone. In conclusion MyD patients try to compensate for overall insulin resistance by a more marked pancreatic response.
Mellituria was studied in 83 subjects: 25 normal adults and children and 58 patients with several metabolic diseases. In comparison to the controls, no significant differences were found in 9 patients with cystinuria and in 2 patients with Apert’s syndrome. The large excretion of glucose was the only important pattern of the 11 patients with insulindependent diabetes. In 23 patients with porphyria cutanea tarda a statistically significant increase in the excretion of pentose was observed. In 16 children with the classical form of phenylketonuria, a significant hypoexcretion of glucose was found. This latter observation could be explained by the carbohydrate metabolic alterations described in experimental hyperphenylalaninemia.
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