Clinical Nutrition (1995) 14 (Suppl. 1): 28-32 © Pearson Professional Ltd 1995 Long-term home parenteral nutrition in children M. CANDUSSO, L. GIGLIO and D. FARAGUNA Department of Paediatrics, Child Health Institute "Burlo Garofolo" Trieste, Italy (Reprint requests and correspondence to: D. F., Department of Pediatrics, Instituto per I'lnfanzia "Burlo Garofolo" Via dell'lstria 65/1, 34100 Trieste, Italy) Introduction The prognosis of children suffering from chronic intestinal failure has been dramatically improved by parenteral nutrition (PN) which allows normal growth and development in the absence of a sufficient enteral supply of nutrients. A small percentage of patients may become permanently dependent on PN and in these cases, home parenteral nutrition (HPN) is crucial for allowing growth within a normal family environment. 14 However, new problems (such as venous access availability, metabolic complications, and psychological adaptation), are emerging in the management of life-long PN dependent patients which also pose possible difficulties for HPN. In this paper, we report our experience with HPN paediatric patients and have focused on long-term patients. Patients and methods Since 1982, 19 children (6 females and 13 males) have undergone HPN. The most frequently diagnosed conditions were short bowel syndrome (n -- 6) and intractable diarrhoea (n = 6); other diagnoses were chronic intestinal pseudo-obstruction syndrome (n = 2), lymphangiectasia (n = 2), inflammatory bowel dis- ease (n = 1) and cystic fibrosis (n = 1). One patient, had biliary cirrhosis whilst undergoing liver trans- plantation. HPN was started in 13 patients during their first year of life, in three during childhood (4-10 years) and in three patients before puberty. Cuffed tunnelled silastic catheters (Broviac) were surgically inserted up to the vena cava through the jugular (external as choice) or safena veins (only if superior access was not available). The emergency site was always positioned in the thorax. Cyclic PN (12-16-hour nightly infusion) was commenced as soon as possible in stable metabolic conditions. Infu- sions were started and stopped gradually in order to reduce the risk of hyper- and hypoglycaemia, espe- cially in infants. The parenteral mixtures were prepared in our hospital pharmacy and contained glucose, amino- acids, minerals and lipids which accounted for 30- 40% of non-protein energy. Vitamins were added at home before infusion, (delivered since 1985 by an all-in-one system) and a monthly supplement of folic acid, vitamins K, A and B12 was provided. Nutrient intakes were defined according to standard daily requirements (LARN) but individually tailored to clinical status, metabolic tolerance, enteral intakes and age. After a training period in hospital, patients were discharged and continued their therapy at home. Parents took care of the infusion line. The solutions were prepared and sent to patients' homes once a week by our pharmacy service. All patients received minimum enteral feeding as soon as tolerable. When indicated, a pre-digested formula was chosen to reduce antigenic stimulation to damaged intestine. In stable conditions ad libitum oral intakes, avoiding strong antigens such as eggs, gluten and cows' milk, were allowed, depending on intestinal tolerance. Patients were visited periodically to assess nutri- tional status and to monitor technical or metabolic complications of PN and any underlying disease. Results Outcome The duration of HPN averaged 1326 days (range 92- 4785), giving us experience equivalent to 69 patient years (Table 1). None of the patients died of PN- related complications, but 3 patients died of their primary illness after an average of 346 days of PN (range 92-844). In 3 cases, PN was stopped and en- teral nutrition became possible after an average of 395 days (range 186-706). Seven patients made a com- plete recovery and reached intestinal autonomy after an average of 579 days (range 173-1592). HPN is still ongoing in 6 patients, 5 of whom will 28