engleski

Use of rocaltrol in the treatment of protein losing enteropathy after Fontan operation

Protein-losing enteropathy (PLE) is a serious complication after Fontan procedure and is frequently associated with a late mortality. The pathophysiology of PLE is still unknown. In the treatment of PLE many different options have been tested, but only a few successful approaches have been published. A recent report noted dramatic improvement in patients who developed PLE after Fontan operation within few weeks of subcutaneous administration of high molecular weight heparin. (HMWH) A 9-year old female with the basic diagnosis : dextrocardia, single inlet ventricle undetermined type, single AV valve of stradling type, pulmonary atresia and haemiazygos continuation, first underwent a palliative procedures Blalock-Taussig anastomosis and bidirectional Glenn. In June 1995 a modified Fontan operation was performed. Four months later she developed PLE. Recatheterisation showed obstructive lesions at the cavopulmonary junction and right pulmonary artery and mild subaortic stenosis. She was reoperated and all clinical symptoms and laboratory findings were reversed. The improvement lasted only for two months, when alongside respiratory infection, again appeared all symptoms of PLE. In June 1997 we started with HMWH therapy. Long term therapy with heparin proved very successful. Nevertheless, it was shown that long term therapy of high dose heparin increases one of the main complications of the PLE-hypocalciemia . Since the diagnosis of PLE had been established, we introduced in therapy calcium supplementation, because we were aware of the possibile development of osteopenia. It was shown that osteopenia cannot be prevented only with introduction of calcuims supplementation in the therapy. Hence Rocaltrol (in April 1999) was also introduced in the therapy. Our patients is now without back pain and last densitometry showed an increase in value of mineral density of bone. Calcium on its own is not sufficient to be inserted in bones and so prevent osteopenia. Calcitriol appears to promote intestinal absorption of calcium through binding to a specific receptor in the mucosal cytoplasm of the intestine. Calcium is absorbed through formation of a calcium-binding protein. Some evidence suggests that calcitriol acts at the level of the cell nucleus to increase plasma calcium and phosphorus. Once plasma saturation of these electrolytes occurs, bone mineralization takes place. We maintain that the in therapy, apart from the high molecular heparin which has shown a great improvement of clinical symptoms and normalization of laboratory values, Rocaltrol should be also included from the beginning, to ensure adequate saturation of plasma calcium and phosphorus which enables bone mineralization.

calcitiol; protein loosin enteropathy; densitometry; childhood

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