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The corrected QT interval is often pathologically prolonged in patients with Gitelman disease, suggesting that there is an increased risk for development of dangerous arrhythmias. Further investigations are required in patients with a prolonged QT interval to assess the true hazard of dangerous arrhythmias.

The QT interval is often prolonged in primary renal hypokalaemia-hypomagnesaemia, confirming that potassium and magnesium depletion tends to prolong the duration of the action potential of the cardiomyocyte. The results of continuous ambulatory electrocardiography, exercise testing and echocardiography are reassuring. Nonetheless, we assume that dangerous cardiac arrhythmias may occur in patients with very severe hypokalaemia, during medication with drugs that prolong the QT interval or in the context of short-term non-adherence to the recommended regimen of care.

The results of a medium-chain triglyceride loading test in a patient with severe carnitine-acylcarnitine translocase deficiency clearly demonstrated impaired in vivo utilization of medium-chain triglycerides. The loading test was performed at the ages of 7 and 36 months. The diet was adjusted accordingly. The clinical course has been favourable and the child is now in very good condition at age 4 years. We conclude that the utilization of medium-chain triglycerides is only partial in carnitine-acylcarnitine translocase deficiency and cannot reasonably be considered an optimal source of energy for these patients. Careful adjustment of dietetic treatment may help to improve prognosis.

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