Bone turnover in short-term and long-term home parenteral nutrition for benign disease

6 mo afterward. Long-HPN was assessed only at baseline. In short-HPN, there was a hyperkinetic turnover at baseline. At follow-up, OC was similar to baseline, whereas cross-links, urinary calcium and magnesium decreased (P < 0.03), and parathyroid hormone increased (P < 0.001). The variation of urinary calcium correlated with that of cross-links (r = 0.73, P < 0.04). In long-HPN, OC was low or low-normal in almost all the patients, and cross-links were normal. Mean OC was lower than that of short-HPN both at baseline (P < 0. 003) and at follow-up (P < 0.002). The results suggest that in the early period of HPN bone metabolism improved from a hyperkinetic turnover to a positive balance. A low bone-formation rate appeared to be a characteristic feature of long-term HPN.     

Shoshin beriberi: an unusual complication of prolonged parenteral nutrition

After 4 weeks of total parenteral nutrition (TPN), a 12-yr-old girl exhibited an acute heart failure with high cardiac output, anuria, and severe lactic acidosis. The clinical, hemodynamic, and biological data suggested the diagnosis of shoshin beriberi which was proved by the low erythrocyte transketolase activity with elevated "TPP effect" and by the dramatic improvement of the patient after thiamin administration. Thiamin deficiency and severe neurological disorders have been described during long-term parenteral nutrition. To our knowledge, this is the first report of the cardiovascular complication of this vitamin deprivation in long-term TPN.     

Dry beriberi: unusual complication of prolonged parenteral nutrition.

An adult with Crohn's disease on home total parenteral nutrition (TPN) for 8 months presented with peripheral neuropathy and ataxia. The patient was found to be deficient of thiamine. A prompt symptomatic response to intravenous thiamine suggests that the patient had the chronic form of dry beriberi. To our knowledge, this variety of beriberi in a patient on TPN has not previously been reported.     

Intracardiac thrombus complicating prolonged parenteral nutrition in an adult

Calcified intracardiac thrombi were identified in a 23-year-old man who had received home parenteral nutrition (HPN) for 4 years on account of Crohn's disease.     

Copper deficiency in an infant on prolonged total parenteral nutrition

An infant with the megacystis microcolon intestinal hypoperistalsis syndrome, required total parenteral nutrition due to ineffective gastrointestinal function, and developed copper deficiency after a prolonged course in total parenteral nutrition in conjunction with a chronic draining jejunostomy. She responded promptly to copper supplementation.     

Plasma vitamin levels in patients on prolonged total parenteral nutrition

Vitamins are essential in total parenteral nutrition (TPN), their importance being highlighted by repeated past documentation of various vitamin deficiencies particularly in patients on long-term parenteral nutrition therapy. This study evaluated the efficacy of water- (Soluvit) and fat-soluble (Vitalipid) vitamin supplementation in patients receiving total parenteral nutrition using the three in one 3-liter bag system. All patients received water-soluble vitamin supplements daily. Fat-soluble vitamin supplements were administered on a daily or twice weekly basis. Twenty-two patients were studied. In seven of the 22 patients vitamin status was assessed on more than one occasion during TPN support, thus bringing the total number of observations to 30. The mean duration of TPN support was 35 days. Eight of the observations were made during less than 10 days, three between 11-19 days, 15 between 20-60 days, and four during more than 60 days of TPN support. Biochemical deficiency as judged by subnormal enzyme activity or vitamin levels were present in 10% of the patients for thiamin, 3% for riboflavin, and 6% for nicotinic acid. By contrast 83% of the patients had low plasma vitamin C and B6 levels. Low plasma vitamin A and E levels were also present in 43 and 40% of the patients, respectively. According to the plasma concentrations of the vitamins studied it would appear that the commercial vitamin preparations used in this study are inadequate in maintaining optimal vitamin status.(ABSTRACT TRUNCATED AT 250 WORDS)     

Bone mineral content in patients on home parenteral nutrition

Bone mineral content (BMC) was monitored in 15 patients with short bowel syndrome receiving home parenteral nutrition (HPN). Thirteen patients had Crohn's disease and 2 ulcerative colitis (mean age 36 years, range 23-69 years). During the study the patients received HPN for a mean period of 62 months, range 20-106 months. At the time of inclusion the patients had a significantly reduced BMC of lumbar spine and femoral neck compared to normals (Z-scores = -3.35 +/- 3.49, p < 0.05 and Z-score = -2.23 +/- 2.11, p < 0.05). During HPN the Z-score of lumbar spine BMC decreased in 8 patients and increased slightly or was unchanged in 7 patients. The mean Z-score of BMC of lumbar spine declined by 1.46 +/- 2.48 (p < 0.05) and the Z-score of femoral neck BMC declined by 0.831 +/- 1.14 (p < 0.05). This corresponds to a yearly decrease of lumbar spine BMC of 4%. There was no correlation between the decline in BMC during the study and the period of length the patients were on HPN. We conclude that patients on HPN have a low bone mineral density and that the bone loss continues during prolonged HPN.     

Bone mineral density in patients on home parenteral nutrition: a follow-up study

BACKGROUND AND AIMS: The variations of bone mineral density (BMD) during home parenteral nutrition (HPN) and their relationship with general, life style, primary disease and HPN risk factors were investigated by a follow-up study. DESIGN: Patients who had BMD assessment in a previous cross-sectional survey underwent a 2nd BMD at femoral neck (FN) and lumbar spine (LS). Data about risk factors were collected by a structured questionnaire. BMD Z-score (number of standard deviations from normal values corrected for sex and age) and the annualized percent BMD change were analysed. RESULTS: Sixty-five adult patients were enrolled (follow-up: 18.1+/-5.5 months). The mean BMD Z-score significantly increased at the LS (P = 0.040) and remained unchanged at FN. In multiple regression analysis, the variations of the LS Z-score during HPN negatively correlated with the female sex (P = 0.021) and the age at starting HPN (P = 0.022). The analysis of the annualized percent BMD change confirmed the results obtained by the analysis of the Z-score. No factor was associated with BMD variation at FN. CONCLUSIONS: HPN was not associated with a decrease of BMD in most of the patients; LS BMD Z-score variations were related to general risk factors rather than to HPN factors, showing a negative association with age and female sex.     

Selenium responsive myositis during prolonged home total parenteral nutrition in cystic fibrosis

The need for routine supplementation of total parenteral nutrition solutions with selenium (Se) has not been clearly defined. Although clinical selenium deficiency in patients on prolonged total parenteral nutrition has been reported, it is rarely observed in the United States. We report a 19-year-old woman with cystic fibrosis who developed muscle pain and weakness after 3 months on total parenteral nutrition which was not supplemented with Se. Coincident with her onset of symptoms, markedly elevated serum creatine kinase values were observed compared to baseline levels. Subsequent evaluations revealed undetectable (less than 0.02 microgram/ml) serum and urine Se levels in this patient. In addition, electromyographic evidence of myositis and nonspecific membrane irritability was documented. Therapy with oral Se rapidly reversed her symptoms and normalized with serum creatine kinase values over a 10-day period. Prolonged treatment with Se was required to achieve normal values of Se in the serum. Patients with severe pancreatic insufficiency, such as cystic fibrosis, may be at risk for clinical Se deficiency if on prolonged total parenteral nutrition without supplementation. Elevated creatine kinase levels should alert physicians to the possibility of Se deficiency in such patients.     

A severe case of hyperemesis gravidarum necessitating prolonged total parenteral nutrition

A patient with severe hyperemesis gravidarum persisting throughout pregnancy is described. She had marked abnormalities of liver function and failed to respond to conservative management. Total parenteral nutrition was used to maintain her nutritional status as well as that of the foetus. Her vomiting continued and was complicated by severe oesophagitis. On delivery her symptoms settled, but she later developed an oesophageal stricture. Changes in liver function tests are described.     

Metabolic bone disease in home total parenteral nutrition

Home total parenteral nutrition (HTPN) is in its infancy but has proved to be lifesaving for patients unable to manage on enteral nutrition alone. However, this mode of nutrition therapy is not without problems. Aside from mechanical and other metabolic complications, a peculiar metabolic bone disease has been reported to occur in some HTPN recipients. The disease, characterized by abnormalities in calcium and phosphorus homeostasis, often results in osteomalacia, bone pain, and fractures. Reports of approximately 50 cases of metabolic bone disease have been published by centers in the United States and Canada. Factors that have been implicated as possible causes include infusion of excess vitamin D, aluminum, calcium, protein, or glucose; cyclic vs. continuous TPN administration; and the patient's previous nutritional state. Although removal of vitamin D or aluminum from the TPN solution and discontinuation of TPN altogether have been associated with improvement in symptoms, histology, and laboratory values, no single factor has been identified as the cause of this troubling phenomenon.     

Bone and mineral status of patients beginning total parenteral nutrition

Total parenteral nutrition (TPN) is commonly used to provide nutrition in the seriously ill. Osteomalacia has been described with long-term TPN and the administered solutions and/or vitamin D metabolites have been blamed for the occurrence of osteomalacia. These studies however were performed on patients on long-term TPN programs. We in contrast measured the serum calcium (Ca), ionized calcium (Ca2+), phosphate (Pi), bone GLA protein (BGP), alkaline phosphatase (ALK-P), 25(OH)D, 1,25(OH)2D, the iPTH (carboxyl terminal) in 25 malnourished patients just beginning TPN therapy. The patients ranged from 25 to 80 yr of age and suffered from a variety of diseases. No patient had symptoms, recent fractures, or radiographic evidence of osteomalacia. The results of our study revealed significantly lower 25(OH)D (p less than 0.001), Pi (p less than 0.01), and Ca (p less than 0.01), but higher iPTH (p less than 0.002) values when compared to normals. BGP, 1,25(OH)2D and Ca2+ and ALK-P were not significantly different. We conclude that patients requiring TPN have low serum 25(OH)D values reflecting their nutritional status with a compensatory increase in PTH secretion to maintain their serum Ca2+ levels. The normal BGP levels may indicate depressed bone formation and skeletal resistance to PTH in the very ill patient. The cause of osteomalacia in these patients may therefore be multifactorial and not only related to the TPN infusions.     

Endoscopic, radiographic, and clinical response to prolonged bowel rest and home parenteral nutrition in Crohn's disease

Total parenteral nutrition is widely used as a therapeutic measure in patients with severe, active Crohn's disease unresponsive to conventional medical management. We have reviewed our experience with 10 patients with nonfistulous Crohn's disease treated by home parenteral nutrition (HPN) and bowel rest (nothing-by-mouth) assessing the nutritional, radiologic, endoscopic, and clinical responses. After a mean of 4.1 months of treatment, all patients had a marked improvement in nutritional status and resolution of gastrointestinal symptoms; 90% reduced their corticosteroid dose. Eight of nine patients had endoscopic and/or radiographic evidence of mucosal healing. Although 60% of patients were able to avoid surgery and tolerate refeeding, all six patients continue to require steroids to control symptoms. Our study suggests that HPN and bowel rest is a useful therapeutic approach to selected patients with active Crohn's disease, which permits a reduction in corticosteroid dosage and partial healing of mucosal lesions in most. Further studies are required to determine which patients should receive HPN and its optimal duration.     

Indicators of biotin status: a study of patients on prolonged total parenteral nutrition

Plasma biotin concentration and lymphocyte propionyl CoA carboxylase (PCC) and its activation index (the ratio of enzyme activity in cells incubated with biotin to activity in cells incubated without the vitamin) were compared as markers of biotin status in patients on biotin-free prolonged total parenteral nutrition (TPN). Five patients had been on biotin-free TPN for over 1 month when the study was started, and had already developed clinical signs that may be caused by this vitamin deficiency. They had markedly reduced levels of both biotin and carboxylase levels, which increased to near normal levels 4 months after biotin was added to the TPN. They initially presented subnormal plasma zinc concentrations which were normal at the end of the study. Another 4 patients also received biotin-free TPN. After 1 month propionyl CoA carboxylase, which was already below normal in 3 of them at admission, further decreased and became undetectable in 1 of them, who presented clinical deficiency manifestations. The enzyme activation index increased more than twice, although plasma biotin remained normal. When the vitamin was added to the TPN solution, PCC activity increased and its activation index decreased to normal. Plasma zinc concentrations remained normal throughout in this other group of patients indicating that changes of biotin markers are not likely to be related to zinc status variation. Plasma biotinidase, a key enzyme in biotin endogenous recycling, was already subnormal at admission and had become normal at the end of the study, but was not associated with the biotin status.(ABSTRACT TRUNCATED AT 250 WORDS)     

Chronic cholestasis and macronutrient excess in patients treated with prolonged parenteral nutrition.

The mechanism of chronic cholestasis observed during prolonged parenteral nutrition remains unclear. We studied liver function tests in 18 consecutive gastroenterological adult patients submitted to 18 mo (median; range 6-66 mo) of parenteral nutrition. Seven patients (group B) developed a cholestatic nonobstructive jaundice, culminating after 3 mo, (1-4 mo) whereas 11 patients (group A) did not develop chronic abnormalities on liver function tests during parenteral nutrition. Liver biopsies obtained at 4 mo (3-6 mo) in 6 group B patients demonstrated bile duct proliferation in the portal area, with extensive fibrosis, cholestasis, and focal necrosis of hepatocytes. Initial intakes of calories, fat, and protein were higher (p less than 0.01) in group B than in group A patients and, when reduced, were associated with reversal of jaundice and improvement in liver function tests and histological findings. These data suggest that the development and course of cholestasis are influenced by excess parenteral intake of protein and mixed-energy sources.     

Total parenteral nutrition in pregnancy complicated by Crohn's disease

Despite the widespread use of total parenteral nutrition (TPN) in nonpregnant patients, the literature reflects limited experience with TPN in pregnancy. However, since maternal nutritional deprivation may cause adverse fetal effects, its use appears warranted. We present a case of a pregnant patient with a poor reproductive history and recently diagnosed Crohn's disease who required TPN. We discuss the use of TPN with fat emulsions in this patient who was unable to tolerate enteral feedings during pregnancy. The patient underwent a 4-week period without oral intake. Total parenteral nutrition enabled continued fetal growth, resulting in a good outcome for the patient and her infant. This report supports the early use of TPN in pregnancy complicated by inadequate caloric intake.     

Low plasma carnitine in patients on prolonged total parenteral nutrition: association with low plasma lysine

Plasma carnitine levels were determined in 17 patients maintained on long-term total parenteral nutrition (TPN) for a mean (+/- SEM) period of 69 +/- 11 months (range 12-196). All had severe malabsorption and were dependent on intravenous feeding. Plasma carnitine was determined by a modified Cederblad enzymatic method. Mean plasma carnitine was significantly below the mean normal for females (p less than 0.02) and borderline low for males (p = 0.07). In six patients the levels were below the low normal range, and in five others they were at the lowest levels of normal. Of the six patients with normal levels, three had elevated serum creatinine, indicating renal dysfunction which may by itself elevate plasma carnitine. In 10 patients the plasma levels of lysine (a carnitine precursor) were determined and found to be lower than normal (p less than 0.05). Plasma carnitine levels correlated positively with serum albumin (r = 0.62, p less than 0.05), and negatively with serum alkaline phosphatase (r = -0.64, p less than 0.05). Thus, patients maintained on long-term TPN may have low plasma carnitine, which could represent carnitine deficiency. The low plasma carnitine may be related to a deficiency of the carnitine precursor lysine. Further studies are required to determine the significance of the low plasma carnitine and whether carnitine supplementation should be required in long-term TPN.