Ammonaps

What is it?

A preparation of 940mg sodium phenylbutyrate per g, available as granules. Also, AMMONAPS TABLETS, containing 500mg sodium phenylbutyrate per tablet. Tablets are grey-white coloured, oval and marked UCY 500.

Medical Uses

Additional treatment in patients with complete enzyme deficiencies/ urea cycle disorders.

Dosage

Adults; 9 to 13 g daily, depending on body size, in equal divided doses taken at meal times. Maximum daily dose is 40 tablets (20g). Children; infants and children weighing less than 20kg: 450 tp 600mg per kg of body weight. Children weighing more than 20kg: same as adult dose. All as equal divided doses taken at meals or with feeds; in infants and small children, given by nasogastric tube or gastronomy.

Special Care

All treatment is as directed by specialist in urea cycle disorders; liver or kidney disease, congestive heart failure, retention of sodium with fluid build up. Careful monitoring of blood biochemistry is needed.

Avoid Use

Pregnancy, breast feeding.

Possible Drug and Food Interactions

Haloperidol, corticosteroids, probenecid, valproate.

Known Side Effects

Absence of or disruption of menstrual cycles, metabolic disturbances (acidosis, hypophosphataemia, alkalosis, hyperchloraemia), loss of appetite, bad taste in mouth, aversion to certain tastes, body odour.

Manufacturer

Orphan Europe.