Successful high-dose calcium treatment of aluminum-induced metabolic bone disease in long-term home parenteral nutrition

A patient who developed severe metabolic bone disease is presented. He had received long-term home parenteral nutrition (HPN) following extensive small bowel resection after mesenteric vein thrombosis. Bone disease caused by aluminum intoxication had components of osteomalacia and low-turnover osteoporosis. Aluminum was detected at the surface of mineralized bone and was elevated in the serum, resulting in a positive deferoxamine infusion test. One year of treatment with high doses of calcium (up to 24 mEq per day) significantly diminished the patient's bone pain, increased the serum levels of calcium, abolished aluminum deposits in the mineralized trabecula, improved bone formation, and increased trabecular bone volume as assessed by repeated histomorphometric analysis.     

Metabolic bone disease in patients receiving home parenteral nutrition: a Canadian study and review

BACKGROUND: Metabolic bone disease (MBD) is a significant complication in patients receiving long-term home parenteral nutrition (HPN). Pamidronate has been poorly studied in this population. We examine the prevalence and risk factors for MBD and examine changes in bone mineral density (BMD) after pamidronate administration. METHODS: First, a chart review of patients receiving HPN for >1 year was performed, and Pearson correlations were used to assess associations between MBD (defined as t score<-1) and risk factors. Second, the effect of IV pamidronate on BMD was studied prospectively in 11 HPN patients. Results were compared using a t-test. RESULTS: Charts were reviewed in 25 patients (15 F, 10 M): age, 56.9+/-3.1 years; body mass index (BMI), 21.2+/-0.57 kg/m2; months receiving HPN, 113.2+/-0.09; and days per week receiving HPN, 5.08+/-0.39. MBD was present in 33% of patients for the spine and hip and in 50% for the femoral neck; 24% had previous fractures. There was a significant negative correlation between the duration of HPN and BMD (r=-0.40) for all measurements. From those patients, 11 received IV pamidronate for a mean of 22.2+/-5.4 months. At baseline, their mean HPN treatment duration was 10.6+/-6.3 years. Overall, BMD results showed a trend toward improvement in the mean t score of the spine and hip postpamidronate therapy (pre, -3.1+/-0.75; post, -2.9+/-0.69; p=.07). After excluding 2 patients receiving corticosteroids, the mean t score of the spine showed significant improvement (prepamidronate -3.4+/-0.57 vs post-pamidronate -3.1+/-0.65, p=.036). CONCLUSIONS: In our HPN population, 76% had MBD and 24% had previous fractures. The results suggest that these patients may benefit from pamidronate. More studies are needed to assess the efficacy of pamidronate.     

Aluminium loading in children receiving long-term parenteral nutrition

Eight children on long-term, total parenteral nutrition (TPN) were found to have elevated aluminium (Al) levels in plasma (51 +/- 11 mug 1 1 ), urine (223 +/- 78 mug 24 h) and bone. The load of Al in TPN solution was 232 +/- 89 mug/day, and, among the different parenteral solution components high Al concentrations were found in amino-acids, calcium gluconate, potassium lactate, and trace elements, representing respectively 40%, 30%, 15%, and 10% of the total Al intakes. The authors conclude that children receiving long-term TPN have excessive Al intakes and are exposed to Al toxicity. The prevention of Al contamination requires careful control of the TPN components.     

Taurine supplementation in infants receiving long-term total parenteral nutrition

Twenty-one children and 23 adults receiving long-term total parenteral nutrition (TPN) for 27 +/- 23 (SD) months were investigated to determine if they were taurine-deficient because the TPN solutions were taurine-free. The fasting plasma taurine level was reduced in the children to 26 + 13 mumol/liter vs the control 57 +/- 16 mumol/liter (P greater than 0.001). The plasma taurine level was significantly reduced in those adults who absorbed less than 25% of their nutritional needs from their diet. Electroretinograms were abnormal in each of eight children who were examined; isolated cone and rod implicit times were both significantly delayed. Electroretinograms were not abnormal in those adults with low plasma taurine levels. Taurine was added to the TPN solutions of four children, and the plasma taurine level became normal in each of them. Electroretinograms of three of these children became normal. One year after discontinuing intervenous taurine supplementation, the plasma taurine level became abnormal in two of three children. These observations indicate that children, and possibly adults, receiving long-term TPN have a nutritional requirement for taurine.     

Hypermanganesemia in patients receiving total parenteral nutrition

BACKGROUND: Manganese is one of the trace elements that is routinely administered to total parenteral nutrition (TPN) patients. The recommended daily IV dosage ranges from 100 to 800 MICROg. We have used 500 microg daily. Recent reports have suggested neurologic symptoms seen in some patients receiving home parenteral nutrition (HPN) may be due to hypermanganesemia. Therefore, HPN patients and some short-term inpatients receiving TPN were studied to ascertain the relationship between dose and blood levels. METHODS: Red blood cell manganese levels were obtained by atomic absorptiometry. RESULTS: The levels in 36 hospitalized, short-term patients obtained within 48 hours of initiating TPN were all normal. The 30 patients receiving TPN from 3 to 30 days had levels that ranged from 4.8 to 28 microg/L (normal, 11 to 23 microg/L). Two patients had abnormal levels, at days 14 and 18. Fifteen of the 21 patients receiving inpatient TPN or HPN for 36 to 5075 days had elevated Mn levels. Only one patient with hypermanganesemia, an inpatient, had abnormal biochemical liver tests (bilirubin and alkaline phosphatase). One of the patients with a high level had some vestibular symptoms attributed to aminoglycoside use and had increased signal density in the globus pallidus on T1-weighted images on magnetic resonance imaging (MRI). A second patient with Mn levels twice normal had no neurologic symptoms, but had similar MRI findings. A third had some basal ganglia symptoms, confirmed by a neurologic evaluation, seizures, and very high Mn levels. The MRI showed no signal enhancement, but motion artifacts limited the study technically. CONCLUSIONS: Hypermanganesemia is seen in HPN patients receiving 500 microg manganese daily and may have resulted in some neurologic damage in three patients. Hypermanganesemia is sometimes seen after a short course of TPN in inpatients, as early as 14 days. Patients should be monitored for hypermanganesemia if they receive Mn in their TPN for >30 days. A 500 microg/d dose of Mn is probably excessive, and 100 microg/d should probably never be exceeded. Mn should be eliminated from the solution if the Mn level is elevated and should not be readministered unless the level returns to normal or subnormal. Mn should not be supplemented if the patient has liver disease with an elevated bilirubin.     

Hypocupremia in patients receiving total parenteral nutrition

Although hypocupremia is a well-known consequence of long-term total parenteral nutrition (TPN), its incidence as well as the duration of TPN necessary to induce it are still unsettled. The purpose of this study is to review the changes in serum copper level in 25 patients receiving TPN for a period longer than 2 wk (mean duration 6 wk) at the Istituto Nazionale Tumori of Milan and to evaluate the possible relationship of cupremia with the basic disease. Main indications for TPN included enterocutaneous fistulas (11 patients), cancer cachexia (10 patients), radiation enteropathy (two patients), and severe postoperative stricture following esophagogastric resection (two patients). Mean value of serum copper at the beginning of the study was 143 micrograms/100 ml (normal value 65-165 micrograms/100 ml), and the regression analysis showed a mean fall of 5.64 micrograms/100 ml/wk. Hypocupremia occurred in four patients (three with intestinal fistulas and one with radiation obstructive enteritis) at 5th, 6th, 9th, and 6th wk of TPN, respectively. No patient with cancer cachexia developed hypocupremia. No patient with hypocupremia had clinical evidence of a copper deficiency syndrome. We conclude that 1) hypocupremia does not occur within the first month of TPN; 2) its incidence is about 16% in patients intravenously fed for period longer than 2 wk; 3) it is more frequent in patients with enterocutaneous fistulas, whereas it never occurs in patients with cancer cachexia, and 4) it is not necessarily associated to a clinicometabolic syndrome of copper deficiency. Finally, the "nutritional" meaning of serum copper should be questioned in cancer patients since it could represent a "tumor marker."     

Possible biotin deficiency in adults receiving long-term total parenteral nutrition

Two adult patients receiving total parenteral nutrition on a long-term home basis presented with severe loss of hair. Both patients had extensive gut resection, consumed no biotin orally and received no biotin parenterally. Supplementation with Berroca-C, one ampule containing 200 micrograms biotin per day resulted in gradual regrowth of healthy hair. The patients now receive a parenteral solution containing biotin and have shown no recurrence of alopecia. It is suggested that biotin deficiency can occur in the adult when no preformed biotin is provided to the body and the contribution of this vitamin from intestinal microbial biosynthesis is compromised.     

Taurine supplementation in infants receiving long-term total parenteral nutrition.

Twenty-one children and 23 adults receiving long-term total parenteral nutrition (TPN) for 27 +/? 23 (SD) months were investigated to determine if they were taurine-deficient because the TPN solutions were taurine-free. The fasting plasma taurine level was reduced in the children to 26 + 13 mumol/liter vs the control 57 +/? 16 mumol/liter (P greater than 0.001). The plasma taurine level was significantly reduced in those adults who absorbed less than 25% of their nutritional needs from their diet. Electroretinograms were abnormal in each of eight children who were examined; isolated cone and rod implicit times were both significantly delayed. Electroretinograms were not abnormal in those adults with low plasma taurine levels. Taurine was added to the TPN solutions of four children, and the plasma taurine level became normal in each of them. Electroretinograms of three of these children became normal. One year after discontinuing intervenous taurine supplementation, the plasma taurine level became abnormal in two of three children. These observations indicate that children, and possibly adults, receiving long-term TPN have a nutritional requirement for taurine.     

Liver failure is uncommon in adults receiving long-term parenteral nutrition

BACKGROUND: Abnormal liver enzymes and endstage liver disease are reported to occur in 25%-100% and 15%-40% of adult patients receiving long-term parenteral nutrition (PN), respectively. The purpose of this historic cohort study was to investigate the incidence of and possible factors leading to the development of liver disease in our large home PN population. METHODS: All patients on home PN for at least 6 months from July 1991 through June 2002 were eligible. Patients were excluded if they had active malignancy, underlying liver disease, or exposure to a hepatotoxin. The presence of PN-associated liver disease was only considered if test results were elevated on more than 1 occasion over at least 6 or more months. The severity of liver-associated enzymes was based on the degree of elevation and was stratified as mild (<2 times normal), moderate (2-5 times normal), and severe (>5 times normal). Severe liver dysfunction was defined as having all of the following criteria: total bilirubin >3 mg/dL; albumin <3.2 g/dL; and prothrombin time >3 seconds prolonged. A cumulative logit model was used to compare age, gender, underlying disease, PN indication, and PN formulas in patients with normal vs abnormal laboratory test results. RESULTS: Two hundred eight patients received PN for more than 6 months, 36 had exclusion criteria, and 10 could not be analyzed, because of incomplete laboratory test results, leaving 162 in the study group. The average PN duration was 2.14 +/- 2.19 years (maximum, 10.28 years). Abnormal liver tests occurred in 154 patients, with most having a moderate elevation of alkaline phosphatase or aspartate aminotransferase; severe liver dysfunction occurred in 7 patients; 1 patient had completely normal liver enzymes. On average, patients received a PN formula that was high in amino acids (1.45 +/- 0.65 g/kg/d), modest in energy (24.7 +/- 13.4 kcal/kg/d), and in most cases with enough lipid emulsion to meet essential fatty acid requirements (0.28 +/- 0.25 g/kg/d). Only female gender was found to be associated with a greater likelihood of liver failure (p = .02). There was a trend toward a greater amount of total calories, dextrose calories, and duration of PN exposure leading to the development of severe liver dysfunction. CONCLUSIONS: When long-term PN is given with a modest amount of total energy and a minimal amount of lipid emulsion, abnormal liver enzymes are common, but severe liver dysfunction is unusual.     

Prevalence of bone disease in patients on home parenteral nutrition

BACKGROUND & AIMS: The epidemiology of bone disease in home parenteral nutrition (HPN) is unknown. The aim of this paper is to evaluate the prevalence and severity of reduced bone mineral density (BMD) in long-term HPN. DESIGN: Cross-sectional, multicentre study including patients who within the last 12 months had their BMD assessed by dual-energy-X-ray absorptiometry after at least 6 months of HPN. Data on bone pain and fractures, the primary gastrointestinal diseases, nutritional and rehabilitation status and HPN regimen were reviewed. Both the T-score (no. of SD below mean BMD of young subjects) and the Z-score (no. of SD from normal BMD values corrected for sex and age) were analysed. RESULTS: A T -score <-1 at any site of assessment was observed in 84% of the 165 patients enrolled (相似文献   

A review of the relationship between parenteral nutrition and metabolic bone disease.

Metabolic bone disease (MBD) refers to the conditions that produce a diffuse decrease in bone density and strength because of an imbalance between bone resorption and bone formation. MBD can be a potential complication in patients receiving chronic parenteral nutrition (PN) therapy and the management of this condition presents a challenge for many clinicians. The etiology of PN-associated MBD is poorly understood, but traditional risk factors can include malnutrition, vitamin and mineral deficiencies, toxic contaminants in the PN solution, concomitant medications, and presence of certain disease states. Although additional studies are warranted to further elucidate the development and management of this condition, the following review discusses some of the important factors that may play a role in the genesis of PN-associated MBD and evaluates some potential strategies for the diagnosis and treatment of this complication.     

Selenium status in patients receiving home parenteral nutrition

Selenium (Se) status was evaluated in patients with intestinal failure requiring home parenteral nutrition (HPN). Ninety-two percent of patients (11 of 12) studied just prior to starting HPN had low serum Se values, and the mean value was 42 ng/ml, significantly less than mean values in disease controls with Crohn's disease not on HPN (76 ng/ml) and healthy controls (88 ng/ml). Eighty-five percent of patients (22 of 26) already on HPN for 2 to 109 months when studied had low serum Se levels (mean 38.4 ng/ml). The mean 24-hr urinary Se values were 3.7 micrograms in patients on HPN who did not have Crohn's disease, 10.9 micrograms in HPN patients with Crohn's, and 17.9 micrograms in healthy controls. In patients with Crohn's disease on HPN, a significant direct correlation existed between serum Se and the activity of whole blood glutathione peroxidase, a selenoprotein ; and a significant inverse correlation was found between serum Se and months of HPN. This study confirms that Se deficiency is very common in patients before starting and during HPN. These data and recent reports of cardiomyopathies associated with Se deficiencies in patients on HPN increase the importance of proper Se replacement and maintenance.     

Treatment of hypertriglyceridemia in patients receiving parenteral nutrition

Background: This study aims to evaluate whether withdrawal of a soy oil–based lipid emulsion from the parenteral nutrition (PN) regimen in humans is associated with improved triglyceride and liver enzyme concentrations. Methods: In this retrospective study, patients with hypertriglyceridemia (>4.50 mmol/L) while receiving PN were retrieved from a prospective complication registration database. Patients received Intralipid 20% as part of an all‐in‐one system containing all necessary macro‐ and micronutrients, electrolytes, trace elements, and vitamins. Results: Forty patients with hypertriglyceridemia were included. Lipid emulsions were withdrawn from the all‐in‐one mixture for a median of 5 (range, 1‐23) days, after which triglyceride concentrations decreased significantly (mean difference ?2.5 ± 0.30 mmol/L, P < .001). Aspartate aminotransaminase and leukocyte count decreased significantly (mean difference ?35 ± 17 U/L, P = .049 and ?3.8 ± 1.7*10E9/L, P = .028, respectively), whereas albumin level increased significantly (mean difference 2.1 ± 0.9 g/L, P = .027). Alanine aminotransaminase showed a nonsignificant reduction (mean difference ?30 ± 22 U/L, P = .194). In 11 patients, the lipid emulsion was reintroduced, after which triglyceride levels showed a significant increase (mean difference 1.5 ± 0.30 mmol/L, P = .001).Conclusions: Short‐term withdrawal of the lipid fraction in the PN mixture is associated with a significant reduction of plasma triglyceride concentration. Reintroduction was related to an increase of triglyceride concentration. In addition, liver enzyme abnormalities and leukocyte count reduced, whereas albumin levels increased, suggesting that even short withdrawal of the lipid emulsion diminished hepatocellular damage and systemic inflammation.     

Liver function tests in patients receiving parenteral nutrition

A 5-year prospective study was performed to monitor liver function tests (LFTs) in patients receiving total parenteral nutrition (TPN). A gradual and progressive rise was seen in the plasma concentration of bilirubin, aspartate transaminase, and alkaline phosphatase. The rate of rise was not increased in patients with LFT abnormalities before the start of TPN. Half of the patients had an episode of sepsis during TPN, but overall abnormal LFTs did not appear more common in these patients than in those without obvious sepsis. Patients with malignant disease, those requiring long-term TPN, and those requiring a nonstandard TPN regimen were more likely to develop raised LFTs.     

Carnitine balance and effects of intravenous L-carnitine in two patients receiving long-term total parenteral nutrition

Two patients requiring total parenteral nutrition for 34 and 39 months, had plasma and urinary carnitine assays and plasma lipid assays performed before and during intravenous administration of 400 mg (2500 mumol) of L-carnitine for 7 days, followed by 40 mg (240 mumol) daily continuously. One patient had generalized lethargy and weakness which resolved within the first 5 days of carnitine administration. The plasma-free carnitine levels in this patient rose significantly. The other patient was asymptomatic and while there was no significant change in the plasma-free carnitine levels during carnitine administration, this patient remained in positive carnitine balance throughout the study. There were no significant changes in plasma lipid levels in either patient. In adult patients requiring long-term total parenteral nutrition who are otherwise normal, intravenous L-carnitine may be required to supplement the patients endogenous carnitine production.     

Thromboembolism in infants and children with gastrointestinal failure receiving long-term parenteral nutrition

Thromboembolism is an established complication of long-term parenteral nutrition (PN) administration which requires central venous lines in the pediatric population. Predisposing factors that increase the risk of thrombosis, as well as prophylaxis and treatment guidelines in this specific population, are not clearly defined. We performed a computerized search of PubMed, OVID databases, and pertinent articles from reference lists of related review papers. This review summarizes currently available data on the rates of thromboembolism in the pediatric population receiving long-term PN and concludes that control of factors such as location of catheter, duration of nutrition support, and prophylaxis with heparin or anticoagulants may reduce the rates of thrombosis in this patient population, although most data on the matter are inconclusive.     

Vitamin D deficiency in patients receiving home parenteral nutrition

Background: In addition to its role in bone metabolism, vitamin D has important immunomodulatory and antineoplastic effects. Patients on home parenteral nutrition (HPN) receive most of their vitamin D from intravenous (IV) supplementation. Vitamin D deficiency is common in the general population, and the adequacy of vitamin D supplementation in HPN patients is unclear. The purpose of this study is to determine the vitamin D status of patients on HPN. Methods: Consecutive patients seen in a regional home nutrition program had their oral and IV vitamin D intakes determined. Plasma 25‐hydroxyvitamin D levels were measured in all patients. Intake of calcium, magnesium, and phosphate were also determined. Results: The mean 25‐hydroxyvitamin D level in 22 patients receiving HPN for a mean of 33.5 months (range, 1–177) was 42 nmol/L. Vitamin D deficiency was present in 15 (68%) patients and vitamin D insufficiency in 6 (27%) patients. The mean dietary vitamin D intake was 79.5 IU per day, while the mean IV supplementation was 166 IU per day. Conclusions: In this study of a regional Canadian HPN program, there was a high prevalence of vitamin D deficiency/insufficiency affecting virtually all patients. All patients receiving HPN should be supplemented with vitamin D and have their 25‐hydroxyvitamin D levels monitored. Further studies are required to determine optimal methods and dosing of vitamin D replacement using oral supplements or ultraviolet light therapy.