Anomalies of the tracheobronchial tree in patients with esophageal atresia

Bone marrow examination revealed a lipid-laden histiocytosis in seven patients undergoing long-term total parenteral nutrition necessitated by extensive short-bowel surgical resection. Clinical abnormalities occurred during this treatment which required bone marrow examination. These included hepatosplenomegaly and peripheral blood cytopenia; the median time to the detection of these abnormalities was 64 months. The most striking change within the bone marrow was the presence of many pigment-laden histiocytes which had the typical morphology of sea-blue histiocytes seen in the so-called idiopathic sea-blue histiocyte syndrome. The occurrence of sea-blue histiocytosis in the bone marrow in association with long-term parenteral nutrition for short-bowel syndrome has not, to our knowledge, been reported previously and should now be considered in the differential diagnosis of bone marrow sea-blue histiocytosis.     

Monitoring and complications of parenteral nutrition

A knowledge of the complications of parenteral nutrition is inherent in the design of any monitoring system. In the initial stages of therapy, the complications are usually of electrolyte imbalance. It must also be appreciated that the provision of nutrition to severely malnourished patients will expose underlying deficiencies, particularly of phosphates and trace elements. In long-term parenteral nutrition, the complications can be broadly divided into those associated with the line and metabolic complications. The line complications include: line blockage, sepsis, and pulmonary embolism. The most important metabolic complication is undoubtedly liver cholestasis, which may be associated with recurrent episodes of sepsis. Any department undertaking long-term parenteral nutrition should have an active nutrition team to avoid complications and audit outcome.     

Herbal medicine takes root in Germany

We report a man who developed selenium-deficient myopathy during long-term parenteral nutrition. Muscle biopsy showed marked mitochondrial depletion in the deep sarcoplasm and enlarged mitochondria at the periphery mainly in type 2 fibers. Muscle weakness improved gradually after the second course of selenium supplementation. The peculiar mitochondrial abnormalities in muscle fibers appear to play a key role in the pathogenesis of selenium-deficient myopathy.     

Familial melanoma

Short bowel syndrome is the clinical manifestation of a fundamental reduction in the functional intestinal absorptive surface area and malabsorption. The development of total parenteral nutrition has improved the natural course of this disease. Home parenteral nutrition-related complications continue to generate significant morbidity and mortality for these patients. Small-bowel transplantation is an alternative to home parenteral nutrition. There are significant risks of graft rejection and the potential complications of long-term immunosuppression. Small-bowel transplantation is an option, but it should be reserved for patients no longer considered candidates for continued home parenteral nutrition.     

Intra- and extra-cranial arterial lesions in TIA patients

BACKGROUND: Patients on total parenteral nutrition are known to be at risk of the development of essential fatty acid deficiency, presenting as a syndrome with scaly skin lesions and characterized by low plasma and erythrocyte linoleic acid concentrations. The essential fatty acid status of patients on long-term home parenteral nutrition who do have access to oral feeds has not been studied. METHODS: With the use of an isocratic high-performance liquid chromatography method, fatty acids were measured in the erythrocytes and plasma of 25 nonfasting patients on long-term home parenteral nutrition and the findings compared with those of 46 hospital outpatients not on nutrition support and five laboratory staff. RESULTS: Statistically significant differences in the two groups were limited to the erythrocytes. Linoleic acid was significantly lower (25.2 vs 40.7 mumol/10(6) red blood cells, p < .0001) and showed a significant correlation with triceps skinfold thickness (r = .52, p = .013). Palmitoleic and oleic acids were higher in patients than controls (10.8 vs 8.4 mumol/10(6) red blood cells, p = .009; 61.2 vs 51.7 mumol/10(6) red blood cells, p = .003). CONCLUSIONS: Despite IV linoleic acid administration, patients on long-term home parenteral nutrition have low erythrocyte stores of this essential fatty acid. This appears to be related to their low body fat stores. We suggest that they may be using much of the infused linoleic acid as an energy source and therefore are at risk of subclinical essential fatty acid deficiency.     

Selenium deficiency associated with cardiac dysfunction in three patients with chronic respiratory failure

We encountered three patients with chronic respiratory failure who had heart failure of cardiac arrhythmias and low levels of serum selenium. All three had tracheostomies and had received long-term parenteral nutrition that had not included selenium. All three also had refractory cardiac dysfunction, which was manifested in edema, heart failure, and various tachycardias. We suspected that selenium deficiency had caused their cardiac dysfunction. Serum selenium concentrations were found to be much lower than normal in all three, so 100 micrograms/day of selenium was administered in addition to their tube feedings. Cardiac function improved after replacement of selenium. These cases show the need for preventing selenium deficiency in patients with chronic respiratory failure during long-term administration of parenteral nutrition.     

Efficacy and safety of total parenteral nutrition in pediatric patients

Pediatric patients differ from adult patients because of active musculoskeletal growth and development of visceral organs and because they have a proportionately smaller nutritional reserve, especially premature infants. Measures of outcome of effective nutritional support in pediatric patients who have experienced trauma or medical disease or who have undergone surgical procedures include weight gain, increased height and circumference of the head, increased hepatic synthesis of plasma proteins, immunocompetence, decreased morbidity, improved survival, and fast recovery. If a pediatric patient cannot eat or be tube-fed enterally after 3 days of recovery and support with fluids, parenteral nutrition is indicated. Examples in which this treatment has dramatically decreased morbidity include gastroschisis, short-bowel syndrome, necrotizing enterocolitis, and Hirschsprung's disease. Contraindications to its use include severe congenital (usually genetic) defects and terminal cancer, conditions in which life expectancy and quality of life are severely decreased. The team approach to parenteral and enteral nutrition in pediatric patients is preferred, and stable patients receiving long-term nutritional support, including infants, should be considered for home parenteral nutrition. When administered by protocol, parenteral nutrition is safe in pediatric patients. In properly selected pediatric patients, direct and indirect costs for such therapy may be significantly less than those in adults, and the cost-to-benefit ratio is appreciably higher when life expectancy, parental pleasure, and potential work productivity are considered. Ethical and social issues in initiating and discontinuing parenteral nutrition are best decided during thorough empathic discussions between physicians and parents.     

Copper metabolism and requirements in total parenteral nutrition

Copper metabolism and requirements in patients receiving total parenteral nutrition were studied in 28 patients with gastrointestinal diseases. During each of the 3 wk of the study period, each of 24 patients received in their total parenteral nutrition solutions, a daily dose of copper amounting to 0.25 mg, 1.05 mg, or 1.85 mg, in a random order. The other 4 patients received a fixed daily dose of 1 mg throughout the 3 wk. Increased losses of copper through the gastrointestinal tract occurred in patients with diarrhea or high-output stomas or fistulas. Patients with abnormalities of liver excretory functions had decreases in gastrointestinal copper losses. Urinary copper excretion was twice that of normal subjects. Copper infused in excess of the requirements was retained and not excreted. Plasma copper did not reflect the copper balance and cannot be used as a guide for copper supplementation. Copper requirements were found to be 0.3 mg/day in patients with normal amounts of gastrointestinal excretion. In the presence of diarrhea or increased fluid loss through gastrointestinal stomas or fistulas, the copper requirements for total parenteral nutrition are 0.4--0.5 mg/day.     

Home parenteral nutrition in children: the Polish experience

BACKGROUND: Home parenteral nutrition has become routine for management of intestinal failure in patients. In Poland the main obstacle to widespread use of home parenteral nutrition is the lack of interest of commercial companies in delivering feedings and ancillaries to patients. METHODS: Twenty-five home parenteral nutrition patients aged from 4 months to more than 13 years were reviewed. The mother or both parents were trained in home parenteral nutrition techniques for 4 to 6 weeks and compounded the nutrients themselves at home. RESULTS: The mean duration of home parenteral nutrition was 10,117 patient days. Hospital stays of patients receiving parenteral feedings were significantly shorter than the duration of administration of home parenteral nutrition (p < 0.001). Eleven children are continuing the home parenteral nutrition program. Eighty-three catheters were used in these patients. The rate of catheter occlusion decreased within the observation period, and in 1997 not one case of occlusion was observed. In 1997 only three catheters were removed during 7.8 patient years, and the overall incidence of catheter-related complications was 0.38 per patient year. The overall occurrence of septicemia was one case in 516 days and of catheter infection was one in 459 days. In 1997 a catheter was infected on average of once every 1419 days. There was significant improvement in the z score for weight during therapy. The average monthly cost of nutrients and ancillary items was approximately $1200 (4200 Polish zlotys [PLN]). These costs are 1.6 to 3 times lower than those recorded in other studies. CONCLUSION: Home parenteral nutrition in children with nutrients mixed by caregivers in the home setting is a safe and appropriate method of treatment that can be used in countries where home parenteral nutrition solutions are not manufactured or where commercial home parenteral nutrition is not economically feasible.     

Perpetuation of inflammation associated with experimental arthritis: the role of macrophage activation by neutrophilic myeloperoxidase

Parenteral nutrition associated cholestasis in preterm infants and newborn children is a frequent and serious disease with an incidence of 23% depended on duration of parenteral nutrition and birthweight. The incidence of liver cirrhosis is 40% when parenteral nutrition is given 74-242 days. The pathogenesis remains unclear. Several predisposing factors are discussed like immaturity, lack of hormonal stimulation by oral feeding, bacterial infection, liver toxicity of aminoacids and their products of photooxidation, lack of taurine, lack of antioxidation substances, hypermanganesaemia and pollution of infusion solutions. Furthermore sepsis during parenteral nutrition seems to multiply the risk of cholestasis. For prevention controlled studies recommend: 1. Early enteral nutrition. 2. The reduction of parenteral amino acids to less than 3 g/kg/d. 3. Light protection for parenteral solutions. 4. Cyclic infusion of parenteral nutrition. 5. The application of antibiotics (metronidazole, gentamicin) during parenteral nutrition. The most important therapeutic intervention is the beginning of oral feeding. Most of the time this leads to a decrease of icterus within two weeks. An icterus persisting longer than 3 weeks should be treated because of the risk of liver cirrhosis. Further therapeutic interventions are: 1. Cholecystokinin, good results in case studies which still has to be verified by a controlled study. 2. Ursodeoxycholic acid, its choleretic effectiveness is verified in several liver diseases by controlled studies, but it is not proven in parenteral nutrition associated cholestasis. 3. Laparoscopic biliary irrigation, successful in several case studies.     

Intestinal adaptation after extensive resection of the small intestine and prolonged administration of parenteral nutrition

After a superior mesenteric thrombosis in a 77 year old man, all but 24 centimeters of the jejunum were resected and anastomosed to the remaining left colon. After being maintained on parenteral nutrition on a 24 hour basis for six weeks, the patient was placed on overnight parenteral nutrition in which he received 2 liters of parenteral nutrition solution containing about 2,200 calories. Over a period of nine months, hypertrophy, lengthening and dilatation took place so that the patient was almost able to support himself by mouth. We believe that this is the first patient in whom documented lengthening of the small intestine has been reported. The factors entering into the hypertrophy and dilatation are discussed. Many patients in this age group are denied therapy because of the consequences of massive enterectomy. With adaptation using prolonged parenteral nutrition, it should be possible for most patients to sustain themselves completely by mouth. Patients with massive necrosis of the small intestine should not be denied therapy even if only 1 foot of small intestine remains. This is likely to hypertrophy in length until parenteral nutrition is no longer necessary.     

The effect of minimum luminal nutrition on mucosal cellularity and immunity of the gut

Many catabolic patients can only consume small volumes of enteral nutrients. The aim of this study was to evaluate markers of cellularity and immunity in the small intestine of rats randomized to receive 6 days of parenteral nutrition, 25% enteral and 75% parenteral nutrition (i.e. minimum luminal nutrition) or enteral nutrition. The same glutamine-enriched solution was used for both parenteral and enteral nutrition. Enteral nutrition was associated with the least amount of jejunal atrophy (P<0.01), with the results from the minimum luminal nutrition group approximating those of the parenteral nutrition group. Parenteral nutrition was associated with the greatest number of CD2+ cells (P< 0.05) and the lowest CD4/CD8 cell ratio (P< 0.01) in the jejunal mucosa. In essence, we failed to demonstrate that there are any appreciable benefits associated with the enteral consumption of 25% of a nutrient load.     

Short-bowel syndrome in children and adults

Short-bowel syndrome is the malabsorptive state that follows extensive resection of the small intestine. Potential long-term survival without parenteral nutrition heavily depends on stimulation of the process of intestinal adaptation, through which the remaining small intestine gradually increases its absorptive capacity. This process is heavily nutrient dependent, and aggressive use of enteral nutrition is required to stimulate its completion. A combination of osmotic sensitivities, nutrient malabsorption, bowel dilatation and dysmotility, and changes in bacterial flora influence the symptoms and the management of this disorder. Chronic complications include parenteral nutrition-induced liver disease, nutrient deficiency states, and, frequently, small bowel bacterial overgrowth. Intestinal transplantation has been successfully developed in some centers in the United States, and preliminary experience suggest a long-term survival of 50%-75%, better in patients receiving an isolated intestinal transplant than a combined liver/bowel transplant. The ultimate role of intestinal transplantation is still undergoing evaluation.     

Nutritional management of patients with feeding-induced pain: acute pancreatitis

A 36-year-old women with severe acute pancreatitis induced by familial hyperlipidemia is presented. Ranson's score, APACHE-II score, assessment of organ function, and a computed tomography scan are used to diagnose the severity of pancreatitis. Withholding oral alimentation, parenteral analgesia, fluid resuscitation, and antibiotics all serve important roles in management of this disease. Protein-calorie malnutrition can easily develop as a result of no oral intake and hypercatabolism. Tube feeding into the jejunum using a partially hydrolyzed formula has been reported in modest to severe pancreatitis. If tube feeding is not tolerated or a feeding tube cannot be properly positioned, parenteral nutrition may be necessary to maintain bowel rest. Parenteral nutrition administered to patients with pancreatitis is associated with catheter-related infection, hyperglycemia, and hypertriglyceridemia. These complications can be managed through careful design of parenteral solutions and close monitoring.     

Chylothorax or leakage of total parenteral nutrition?

The diagnosis chylothorax is based on a chemical analysis of the pleural effusion. According to the literature, this analysis can be rather straightforward, comprising measurements of triglycerides, chylomicrons, and cholesterol. In this report we present an autopsy case that alerted us to interpret these results critically. Although the laboratory tests of the pleural effusion in this patient with parenteral nutrition suggested chylothorax, additional tests (potassium (11.3 mmol.L(-1)) and glucose (128 mmol.L(-1)) proved otherwise. Comparison of the pleural effusion analysis and the content of the parenteral nutrition led to the final conclusion that the effusion was due to a leakage of parenteral nutrition instead of chylothorax. We therefore suggest adding glucose and potassium measurements to the biochemical work-up of a patient under suspicion of chylothorax.     

High signal in the adenohypophysis on T1-weighted images presumably due to manganese deposits in patients on long-term parenteral nutrition

Hypermanganesaemia is reported in patients on long-term parenteral nutrition. Deposition of manganese, giving high signal on T1-weighted images, may involve the basal ganglia. MRI in nine patients (mean age 51 years, range 31-75 years) on long-term parenteral nutrition (mean duration 30 months, range 6-126 months), demonstrated high signal in the anterior pituitary gland on T1-weighted sagittal and coronal images. The gland appeared normal on T2-weighted images. Signal intensity in the basal ganglia on T1-weighted images was increased in all patients. Endocrine assessment showed no significant abnormality. Neurological examination showed a mild parkinsonian movement disorder in one patient. Hypermanganaesemia was present in all nine (1.3-2.8 micromol/l, mean 1.87 micromol/l). The high signal in the anterior pituitary gland was probably related to deposition of paramagnetic substances, especially manganese.     

Nutritional considerations in children undergoing bone marrow transplantation

Bone marrow transplantation is often associated with multiple organ failure which is usually reversible. Oral mucositis and dysphagia, vomiting, diarrhoea, protein losing enteropathy, transient exocrine pancreatic impairment, hypoalbuminaemia, biochemical trace element and mineral deficiencies are all common following transplantation and have profound nutritional consequences. Malnutrition affects negatively the clinical outcome. Nutritional support is provided to malnourished patients and those who suffer deterioration in nutritional status despite the provision of dietetic counselling. Only a few randomised studies comparing enteral with parenteral nutrition after transplant exist. Both enteral tube feeding (in the absence of mucositis) and parenteral nutrition are effective in maintaining nutritional status. However, enteral nutrition is associated with a better nutritional response and fewer complications than parenteral. With existing enteral and parenteral nutrition regimens close monitoring of trace element and mineral status is required.     

Hepatobiliary toxicity of total parenteral nutrition in adults

The enzymology and clinical manifestations of total parenteral nutrition (TPN)-induced liver abnormalities have been investigated extensively. The cause, pathogenesis, and treatment of TPN-related hepatic and biliary dysfunction in adults still are not well understood, however. The findings of experimental studies in animals has not necessarily correlated with the human data, and there have been few prospective, randomized controlled trials examining the mechanism, cause, or treatment of TPN-induced hepatobiliary toxicity in adults. This article examines the animal models of pathogenesis and treatment of TPN-induced intrahepatic and extrahepatic abnormalities, and provides a discussion of abnormalities seen in humans.     

Home parenteral nutrition in the management of patients with chronic idiopathic intestinal pseudo-obstruction

Home parenteral nutrition is indicated in all those patients who are unable to cover all their needs orally or enterally during prolonged periods of time, and who do not require any other general care other than the parenteral nutrition. Our objective is to prove the use of home parenteral nutrition as a nutritional support in patients with severe forms of chronic idiopathic intestinal pseudo-obstruction. In our unit, three patients with this disease, have received home parenteral nutrition between 1993 and the present date. One patient received it during four months, with the catheter being removed due to a fungemia. At present she is being maintained with oral and enteral nutrition. The other two patients continue in the program: one since October 93 and the other since July 94. The hydroelectric alterations caused during the episodes of sub-occlusion make more frequent changes in the composition of the parenteral nutrition necessary, compared to other types of patients. The low incidence of complications and the degree of acceptance by the patient makes this technique an ideal method for the long term nutritional support.