Prevalence of liver complications in children receiving long-term parenteral nutrition.

BACKGROUND/OBJECTIVES: The hepatic prognosis of long-term home total parenteral nutrition (TPN)-dependent children is poorly documented. The objective was to study outcome data in home TPN-dependent children and to describe precisely their liver biopsies in the attempt to analyze risk factors for biochemical and histological hepatic abnormalities. SUBJECTS/METHODS: Medical records of 42 children receiving home TPN for more than 2 years between January 1998 and December 2007 in a single approved home total parenteral center were reviewed. Hepatic biochemical abnormalities were analyzed. Hepatic biopsies were classified by two independent pathologists. RESULTS: Duration of TPN was 7.9±0.8 years (mean±s.e.m.), with an average age at onset of 1.5±0.5 years. A total of 24 patients (57%) developed biochemical liver abnormalities in an average of 2.9±0.4 years after starting TPN. Risk factors for biochemical abnormalities were younger age at TPN commencement, longer duration of TPN, higher rate of catheter-related infections and higher volume and energy content of TPN. Liver biopsies were carried out in 43% of patients (mean age 3.2±0.9 years). Almost all patients had fibrosis (94%). Risk factors were dependent on each histological abnormality: fibrosis was significantly associated with a shorter length of bowel and a longer duration of TPN; cholestasis correlated with a lower percentage of total parenteral energy intake due to lipids; and steatosis had no risk factor identified. CONCLUSION: Our study reports a high rate of histological liver abnormalities and analyzes risk factors in children who underwent very long-term home TPN.

[From starving to life-threatening nutrition--the history of an intestinal pseudoobstruction]. / Vom Verhungern zur lebensbedrohlichen Ernährung--Kranken-Geschichte einer chronischen intestinalen Pseudoobstruktion.

It is the story of a 70-year-old lady, who suffered from chronic intestinal pseudoobstruction since her adolescence. In the early 90ies progressive cachexia developed. In 1994 parenteral nutrition was begun via a port-à-cath-system with good success in the first years. Later, various complications occurred: thrombotic events, several catheter-related infections with various bacterial strains, an endocarditis of the aortic valve, which was replaced by a bioprosthesis, and finally a relapsing endocarditis of this artificial valve with a life-threatening paravalvular abscess and regurgitation. She also survived this second heart surgery and is currently under parenteral nutrition again, with a more than uncertain future.

Should patients with advanced, incurable cancers ever be sent home with total parenteral nutrition? A single institution's 20-year experience.

BACKGROUND: Home total parenteral nutrition (TPN) can be lifesaving and life sustaining for some patients. However, in patients with advanced, incurable cancer, its role is controversial. A retrospective study was conducted to explore whether home TPN was associated with long-term survival (>or=1 year) in patients with metastatic disease and to identify predictive factors to enable its judicious use. METHODS: The records of all adult patients with incurable cancer were identified between 1979 and 1999. Records were reviewed in depth for survival from TPN initiation to death and for a variety of demographic and clinical factors. RESULTS: Fifty-two patients were identified. Their median age was 56 years (range, 18-83 years), and 30 (58%) were women. Malignant diagnoses included carcinoid/islet cell tumor (n=10), ovarian carcinoma (n=6), amyloidosis/multiple myeloma (n=6), colorectal carcinoma (n=5), sarcoma (n=5), pancreatic carcinoma (n=4), gastric carcinoma (n=3), lymphoma (n=2), pseudomyxoma peritonei (n=2), and other (n=9). TPN was initiated for the following reasons (indications are not mutually exclusive): alimentary tract obstruction (n=20), short bowel syndrome/malabsorption (n=16), fistula (n=11), dysmotility (n=3), nausea/emesis (n=2), anorexia (n=2), and mucositis (n=1). The median time from initiation of TPN to death was 5 months (range, 1-154 months). Sixteen patients survived >or=1 year. TPN-related complications included 18 catheter infections (1 per 2.8 catheter-years), 4 thromboses, 3 pneumothoraces, and 2 episodes of TPN-related liver disease. Tumor grade, the interval between diagnosis of metastatic disease and initiation of TPN, the presence of prominent cancer symptoms, and the administration of cancer therapy after TPN were not associated in any way with overall survival. CONCLUSIONS: The initiation of home TPN can be associated with long-term survival in very select patients with incurable cancer, and complication rates with its use appear acceptable. However, the judicious use of home TPN in this setting requires careful clinical assessment on a patient-by-patient basis.

Incidence, prognosis, and etiology of end-stage liver disease in patients receiving home total parenteral nutrition.

BACKGROUND: Elevations in liver function tests have been reported in patients receiving total parenteral nutrition (TPN). The clinical aspects of end-stage liver disease (ESLD) associated with the prolonged use of home TPN have not been fully clarified. In previous series patients with duodenocolostomies appeared to be at higher risk than persons with some jejunum or ileum remaining in situ. METHODS: The records of 42 patients treated with home TPN for more than 1 year were examined. This constituted 283 person-years of home TPN. Patients with duodenocolostomies were examined as a separate group on the basis of the literature experience. RESULTS: Six of 42 patients who received chronic home TPN had ESLD with 100% subsequent mortality, at an average of 10.8 +/- 7.1 months after the initial bilirubin elevation. Thirteen of 42 patients had superior mesenteric artery or vein thrombosis (SMT) leading to duodenocolostomy. In 8 of these 13 patients with SMT and underlying inflammatory or malignant disorder, 2 had ESLD. The remaining 5 SMT patients who had only minimal liver enzyme elevation over 13.6 +/- 6.7 (range 3 to 19) years of home TPN were significantly younger (36 +/- 7 years vs 64 +/- 13 years) and did not have underlying inflammation either by clinical diagnosis or as reflected in the high normal serum albumin level (> or = 4.0 g/dL). Despite their extreme short bowel syndrome and long exposure to home TPN, ESLD did not develop. CONCLUSIONS: Approximately 15% of patients who receive prolonged TPN have ESLD with a high rate of morbidity and mortality. The combination of chronic inflammation and the short bowel syndrome appears to be necessary for the development of ESLD with prolonged home TPN.

Prolonged parenteral nutrition for cystic fibrosis patients.

To evaluate the clinical impact of prolonged parenteral nutritional (PN) therapy on patients with advanced cystic fibrosis, we conducted a retrospective chart review of 25 cystic fibrosis patients who underwent prolonged PN (median course 295 days) at our institution between August 1988 and May 1992. The patients' survival status, change in percentage of ideal body weight, need for ongoing nutritional intervention, pulmonary function test changes, i.v. antibiotic use, and complication rates were assessed. Patients gained significant weight while receiving PN, but they lost weight when PN was discontinued. PN did not clearly improve pulmonary status. IV antibiotic therapy nearly doubled during PN. Central venous catheter sepsis rates rose from 1.29 to 3.45 per 1000 catheter days during PN therapy. In conclusion, prolonged PN promotes weight gain in cystic fibrosis patients with severe disease; however, the effect is transient and involves a significantly increased risk of sepsis.

Home total parenteral nutrition in a "no-bowel" patient in Spain.

OBJECTIVE: To report a case of a patient with no functional bowel who was receiving home total parenteral nutrition in a country that has had a few professional experiences in this area of therapy. CASE SUMMARY: A woman with a history of scattered colonic polyposis developed a mesenteric tumor that caused intestinal obstruction. Tumor withdrawal required the excision of 1.5 m of jejunum-ileum. Postoperative complications required further surgical intervention and subtotal intestinal resection. Duodenocolic anastomosis was not possible and a high output and permanent fistula remained. DISCUSSION: The complications of home parenteral nutrition addressed in the literature are reviewed. The problems encountered in our patient have been resolved. CONCLUSIONS: After three years of postoperative survival, we believe the quality of life of our patient has increased and the parenteral nutrition team members are much better prepared to manage patients with similar problems.