Do carnitine and extra trace elements change stability of paediatric parenteral nutrition admixtures?

INTRODUCTION: High concentrations of trace elements (TE), in particular zinc and selenium, along with carnitine, are often added to parenteral admixtures in paediatric patients on long-term Parenteral Nutrition (PN). We aim to evaluate whether lipid droplet diameters of these admixtures maintain the recommended range of 0.4-1.0 µm. MATERIALS AND METHODS: Stability studies were carried out on six parenteral admixtures with carnitine, trace elements and electrolytes added in different amounts. Each admixture was formulated with five different lipid emulsions with or without fish oil. Analyses were performed at time 0 (t = 0) and 24, 48, 72, 96 (t = 96) hours after compounding. Droplet diameters were determined by Light Scattering-Reverse Fourier Optics Technique. Samples, stored at 4 °C, were triple tested for a total of 450 analyses. Regression analyses were performed using panel-data techniques. RESULTS: During the 4 days, lipid droplet diameters were in the expected range of 0.4-1.0 µm regardless of trace element and carnitine amounts in all admixtures apart from those containing fish-oil based emulsions and calcium concentrations equal to 4.5 mmol/L. In these latter admixtures, 12% of droplet diameters were larger than 1.0 µm and 2% exceeded 5.0 µm immediately after compounding. CONCLUSION: Carnitine and high concentrations of trace elements do not affect PN admixtures stability and can be safely infused in long-term home-PN paediatric patients and prematures. Only high calcium concentrations in compresence with fish oil based lipid emulsions seem to change PN stability.

Parenteral nutrition admixtures for pediatric patients compounded with highly refined fish oil-based emulsion: assessment of physicochemical stability.

Fish oil-based emulsion is increasingly used in pediatric patients receiving Parenteral Nutrition (PN). However, its unique use in children on long-term PN is nutritionally debatable as some patients are better off with a mixture of long-chain (LCT) or long-chain + medium-chain (LCT + MCT) triglycerides along with Fish Oil (FO). Lipid emulsions are safely infused when particle diameter ranges between 0.4 and 1.0 micron (like chylomicra), according to European guidelines. No data exist on Fish Oil stability when added to other PN components typically present in pediatric formulations such as other lipids or micronutrients. Our goal is to evaluate the stability of a highly refined FO-emulsion in PN admixtures containing LCT or LCT + MCT triglycerides and different calcium content. Stability studies were carried out on six PN admixtures having two levels of calcium concentration compounded with olive oil LCT + FO, LCT + MCT + FO emulsion and pure FO alone, respectively. The analyses were performed immediately at time 0 (t = 0) and 24, 48, 72, 96 (t = 96) hours after compounding. Particle diameters were determined by Light Scattering-Reverse Fourier Optics Technique by means of a Laser Granulometer. Every sample was stored at 4 °C and triple tested. Statistical significance was verified by f-test. In all admixtures, physicochemical stability did not change between t = 0 and t = 96 and particle diameters were in the expected range of 0.4-1.0 micron provided calcium concentration remained below 4.5 mmol/L. When calcium exceeded that level, 12% of particle diameters was larger than 1.0 micron and 2% exceeded 5.0 micron immediately after compounding. In particular, admixtures compounded with olive oil LCT + FO emulsion or FO emulsion alone showed lower particle diameters compared to admixture with olive/soybean LCT alone, probably due to a different steric encumbrance of oleic acid and omega-3 fatty acid. In the PN admixtures tested, containing FO-emulsion alone or in combination with olive LCT or LCT + MCT, the fat emulsion appears to be stable and safe for infusion when calcium concentration is maintained below 4.5 mmol/L. If calcium level exceeds 4.5 mmol/L, as often required in premature patients, it is advisable to infuse FO emulsion alone through a second intravenous line.

Total parenteral nutrition in bone marrow transplant: what is the appropriate energy level?

OBJECTIVES: Total parenteral nutrition (TPN), recommended during bone-marrow transplant (BMT), is often withheld following complications. We aim to determine the effective amount of energy supplied and its short-term effects in children requiring BMT. METHODS: Twenty children (11 males, 9 females, mean age 8 years, range 1-18 years) receiving 13 allogenic and 7 autologous BMT for malignant (13) and nonmalignant (7) diseases, were retrospectively evaluated for energy/protein intakes, weight changes, time to engraftment and on TPN, occurrence of complications, and metabolic abnormalities. RESULTS: Each child received approximately 72% of the prescribed calories, an average of 0.87 +/- 0.2 x basal-metabolic rate, 1.14 +/- 0.4 g protein/kg/day, and 176 +/- 34:1 nonprotein calories:nitrogen ratio. Body weight improved during the 35 days (range 14-62) of TPN, with loss thereafter. Engraftment occurred in 20 +/- 7.5 days. Caloric intake and time to engraftment were related (p = 0.002). Ten central-venous-line and 12 gastrointestinal infections occurred. Among laboratory abnormalities, liver function tests resulted temporarily altered in 10 patients, and permanently in 1 child with cholestasis. Eight children developed graft-versus-host disease. Five died of cancer. CONCLUSIONS: The energy supplied with TPN in BMT is less than expected and approximately covers the BMR with mixed effects. Energy intake needs to be calibrated during TPN and adjusted during feeding resumption to expedite recovery.

Success rates and cost-effectiveness of antibiotic combinations for initial treatment of central-venous-line infections during total parenteral nutrition.

BACKGROUND: Central-venous-line infections can be successfully treated with appropriate antibiotics, thus avoiding the need for catheter removal. Based on our experience, vancomycin, gentamicin, piperacillin, ceftazidime, and amphotericin, alone or in combination, are usually administered, pending sensitivity results. This empirical list, however, has never been verified against actual sensitivity results nor has it been tested for cost or efficacy. METHODS: Medical records of inpatients on hyperalimentation over 1 year were reviewed. Success rate, therapy duration, and drug acquisition cost and charge were assessed for central-venous-line infections. Antibiotics then were paired and evaluated in terms of charge and efficacy against all microorganisms as determined by sensitivity results. RESULTS: In 500 inpatients receiving hyperalimentation for 9,698 patient-days, 8.4 central-venous-line infections/1,000 patient-days occurred. Staphylococcus non-aureus, Candida species, Enterococcus faecium, and Staphylococcus aureus predominantly were isolated. Of the infections, 51 (67%) were sensitive to one or more of the initial antibiotics. A 2-week course of antibiotics successfully treated 50 (66%) catheter infections without line removal. Appropriate initial therapy on average reduced treatment duration by 8 to 10 days and drug charges by $400 to $700. CONCLUSIONS: Amikacin-vancomycin appears to be the most cost-effective selection for presumed central-venous-line infections, pending sensitivity results, followed by valid alternatives. Lower failure rates are well worth the extra cost in pharmaceutical charges.

Total parenteral nutrition and home parenteral nutrition: an effective combination to sustain malnourished children with cancer.

A patient with Wilms' tumor and severe failure to thrive required total parenteral nutrition (TPN) for "catch-up" growth. This case underscores how TPN might be useful in the management of a child with cancer. Cancer cachexia, chemotherapy, radiation, and infections caused by immune suppression can lead to potentially serious macro- and micronutrient deficiencies.

Immune deficiencies in chronic intestinal pseudo-obstruction.

AIM: Chronic intestinal pseudo-obstruction has been associated with urinary disorders, myopathy, and ophthalmoplegia in adults and cholelithiasis in children. We observed a high percentage of total-parenteral-nutrition-dependent patients with pseudo-obstruction and recurrent infections requiring gammaglobulin infusions. METHODS: All records for 23 children with chronic intestinal pseudo-obstruction (10 females and 13 males, mean age 9.8 y +/- 4.9 y, range 4-24 y) referred for a nutritional evaluation from 1992 to 1995 were reviewed. Chronic intestinal pseudo-obstruction was diagnosed by clinical, radiographic findings and antroduodenal manometry. Intestinal full-thickness biopsies were performed in seven children. RESULTS: Hypogammaglobulinemia was diagnosed in 18 patients (78%): 16 patients had various immunoglobulin deficiencies and 2 had selective antibody deficiency. Intravenous gammaglobulin was administered in 14 patients. Other medical conditions affecting the children are summarized as follows: autonomic dysfunction in 10 patients (43%), recurrent hypoglycemia in 9 (39%), asthma in 9 (39%), cholecystitis in 7 (30%), low serum carnitine level in 6 (26%), urinary dysfunction in 6 (26%), pancreatitis in 5 (22%), behavioral problems in 5 (22%), myopathy in 2 (9%), idiopathic thrombocytopenia in 2 (8%), velopharyngeal insufficiency in 1 (4%), oculocutaneous albinism in 1 (4%), Pierre-Robin syndrome in 1 (4%), and protein C deficiency in 1 (4%). Munchausen syndrome was suspected in two patients. CONCLUSIONS: Chronic intestinal pseudo-obstruction appears to be associated with immune deficiencies. It is unclear if the immune deficiencies, intestinal pseudo-obstruction, and the other medical conditions have a common underlying etiology. Repeated infections may be due to impaired immune function in children with chronic intestinal pseudo-obstruction. We recommend screening for immune deficiencies in children with chronic intestinal pseudo-obstruction.

Aminosyn PF or trophamine: which provides more protection from cholestasis associated with total parenteral nutrition?

Cholestasis often occurs in infants on total parenteral nutrition (TPN) for long periods. Amino acid formulations developed specifically for infants, namely Aminosyn PF and Trophamine, may protect against cholestasis associated with total parenteral nutrition (CATPN). The development of cholestasis may also be caused by other risk factors such as prematurity, surgery, sepsis, and extracorporeal membrane oxygenation (ECMO). To evaluate the relative effectiveness of the pediatric amino acid formulations in reducing CATPN, the courses of 70 infants < 1 year of age who received TPN for at least 14 days were reviewed. Cholestasis was defined as a conjugated serum bilirubin > or = 2 mg/dl subsequent to the initiation of TPN; CATPN was considered present when other factors related to cholestasis were ruled out. Liver function tests were recorded 24 h before starting TPN and at day 7, 15, and 21 during TPN infusion. Thirty infants (42.8%) developed cholestasis. CATPN was judged to have occurred in 15 (21.4%) of 70 infants, while 15 (21.4%) developed cholestasis secondary to other factors. Of the 15 CATPN patients, 7 had received Trophamine, 6 had received Aminosyn PF, and 2 had received both solutions. Aminosyn PF and Trophamine, along with other potential risk factors for CATPN such as antecedent surgery, sepsis, ECMO, prematurity, and nitrogen/calorie intake were analyzed by regression-analysis methods. None was statistically significant except the length of TPN (p = 0.0063). In conclusion, we cannot support the view that Trophamine is more effective than Aminosyn PF in the prevention of CATPN.

Children with congenital heart disease: a nutrition challenge.

Growth in children with congenital heart disease (CHD) is often compromised. For several decades, investigators have tried to identify the factors affecting growth in children with CHD. Cardiac malformations are undoubtedly responsible for malnutrition, which may range from mild undernutrition to severe failure to thrive (FTT). Malnutrition may then significantly undermine the outcome of corrective surgical operations and postoperative recovery. Mechanisms linking CHD to malnutrition may be related either to decreased energy intake and/or to increased energy requirements. Decreased energy intake can involve deficiencies of specific nutrients, or insufficient total caloric intake. Increased respiratory rate accompanying congestive heart failure may be responsible for increased energy requirements. Different types of cardiac malformations and consequent interventions may have different effects on growth and require diverse strategies. Most treatment strategies aim to facilitate "catch-up" growth, providing extra calories and protein that exceed the Recommended Dietary Allowance for age. However, there is no generally accepted set of guidelines that define appropriate caloric intake for catch-up growth. We attempt to identify the most important causes of malnutrition and highlight the most effective nutrition strategies for children with CHD.

[Discordance in the onset of celiac disease in monozygotic twins]. / Discordanza nell'esordio di malattia celiaca in gemelli monozigoti.

Coeliac disease (CD) is a gluten intolerance caused by a combination of genetic and environmental factors such as nutrition and infections. Monozygotic twins appear to have a concordance for CD up to 71%. This paper reports a third case of late onset of CD in monozygotic twin girls. The twins were defined as monozygotic based upon paired clinical and laboratory examinations. Clinical examinations included genotypic, phenotypic and dermatoglyphic analysis, while laboratory examinations included HLA typing and blood groups. Following European Society of Pediatric Gastroenterology and Nutrition criteria, CD was diagnosed in both girls, though 4 years and 8/12 months apart. The twins achieved clinical, laboratory and histological remissions within 1 year, after the institution of a gluten-free diet. Genetic markers are undoubtedly the main precondition for CD development. Environmental factors, however, may play a more significant role in triggering the onset of disease.