Parenteral nutrition short-bowel syndrome

In short bowel syndrome, parenteral nutrition should be used to meet nutritional needs in the immediate postoperative period after intestinal resection. 

Acquired biotin deficiency is extremely rare but may occur in special conditions such as long-term parenteral nutrition without biotin supplementation, short bowel syndrome and after excessive intake of raw egg white, which contains the potent bio-tin-binding protein avidin. The main symptoms are alopecia and skin abnormalities which resolve after administration of biotin [2, 30]. 

When one young woman on chronic parenteral nutrition for short bowel syndrome presented with hepato-splenomegaly and pancytopenia (78), bone marrow examination showed the sea-blue histiocytes to be lipidladen macrophages. The total amount of fat in the regimen was subsequently reduced, and there was partial hematological improvement. The condition observed in this patient is analogous to that which occurs in Gaucher s disease and Niemann-Pick disease. 

Intestinal transplantation is combined with liver transplantation in 46% of cases, because of terminal liver failure (93). Of 78 patients who had received parenteral nutrition for more than 2 years n — 66) and/ or had short bowel syndrome and could not be weaned from parenteral nutrition (n = 12), 58 developed chronic cholestasis and 37 developed one or more severe liver complication (serum bilirubin concentration 60 pmol/l, factor V (proaccelerin) 50%, portal hypertension, encephalopathy, ascites, bleeding from the gastrointestinal tract, or histological findings consisting of extensive fibrosis and cirrhosis) after 6 (3-132) months and 17 (2-155) months respectively. Liver disease was responsible for deaths in 6.5% of the patients (22% of deaths). 

In adults receiving long-term parenteral nutrition, despite its anabolic effects on other tissues, there is no improvement in bone density. Infants treated with parenteral nutrition from birth also develop low bone density for age, suggesting that parenteral nutrition treatment in some way contributes to the osteopenia (5). A 17% long-term increase in spinal bone mineral content has been shown in patients who have received parenteral nutrition solntions without vitamin D. However, this rise was nearly balanced by a 15% fall in hip bone mineral content (115). In a Danish study of bone mineral content in adults receiving home parenteral nutrition for short bowel syndrome, despite the fact that all were on free oral intake as a supplement to the parenteral nutrition, 47% had mandibular osteoporosis while 33% had osteoporosis in the forearm and radiographic changes of osteoporotic fractures in the vertebral column. Dental and periodontal tissues were normal (116). 

Burstyne M, Jensen GL. Abnormal liver functions as a result of total parenteral nutrition in a patient with short-bowel syndrome. Nutrition 2000 16(ll-12) 1090-2. 

Norose N, Terai M, Norose K. Manganese deficiency in a child with very short bowel syndrome receiving long-term parenteral nutrition. J Trace Elem Exp Med 1992 5 100-1. 

Messing B, Crenn P, Beau P, et al. Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome. Gastroenterology 1999 117 1043—1050. 

As small bowel adaptation occurs, some short bowel syndrome patients receiving parenteral nutrition can be transitioned successfully to enteral nutrition. Early initiation of enteral intake affects adaptation because intraluminal nutrients are a stimulus for this process. 

Forbes A, Chadwick C. Short bowel syndrome. In Souba WW, et al, eds. The A.S.P.E.N. Nutrition Support Practice Manual. Silver Springs, American Society for Parenteral and Enteral Nutrition, 1998 1-10. 

A fish oil-based intravenous lipid emulsion in the treatment of liver disease associated with parenteral nutrition has been compared with soybean oil in an open study in 42 infants with short bowel syndrome who developed cholestasis [35 ]. There were three deaths and one liver transplantation in those who received the fish oil, compared with 12 deaths and 6 transplants in those who received soybean oil The fish oil was not associated with hypertriglyceridemia, coagulopathy, or deficiency of essential fatty acids. 

Rollins MD, Scaife ER, Jackson WD, Meyers RL, Mulroy CW, Book LS. Elimination of soybean lipid emulsion in parenteral nutrition and supplementation with enteral fish oil improve cholestasis in infants with short bowel syndrome. Nutr Clin Pract 2010 25(2) 199-204. 

Parenteral nutrition formulations provide nutritive support for patients who are not able, not allowed or not willing to eat for a critical period of time. Indications for parenteral nutrition include pre- and postoperative periods of major surgery or trauma, severe obstruction of the gut (e.g. by tumours), severe motility disorders (e.g. ileus), severely impaired absorption capacity (e.g. short bowel syndrome, mucositis in stem cell transplantation patients). Parenteral... 

The ill effects of total or near total starvation have been documented to the point where, in children, whenever the therapeutic choice is starvation versus parenteral nutrition, there is, in fact, no choice to be made (Dho-peshwarkar, 1983). This lack of choice is perhaps best illustrated by considering the short bowel syndrome. Although the potential for survival... 

Catheter-related sepsis is the most fire-quent complication in patients receiving home parenteral nutrition for short bowel syndrome. A low-grade systemic inflammatory state and an altered mucosal immune response, as well as diminished intestinal barrier function have been characterized in these patients. The possibility of systemic immunocompromise has only recently been suggested. 

In a case report, a 49-year-old female with short bowel syndrome who underwent multivisceral transplant due to total parenteral nutrition-related liver disease developed posterior reversible encephalopathy syndrome (PRES) which was thought to be due to sirolimus. PRES resolved after sirolimus was discontinued [48 ]. 

The fact that normal humans have a requirement for biotin has been clearly documented in two situations prolonged consumption of raw egg white and parenteral nutrition without biotin supplementation in patients with short bowel syndrome and other causes of malabsorption. Based on lymphocyte carboxylase activities and plasma biotin levels, some children with severe protein-energy malnutrition are biotin deficient. Investigators have speculated that the effects of biotin deficiency may be responsible for part of the clinical syndrome of protein-energy malnutrition. 

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