Nachum Vaisman

Energy expenditure of patients with cystic fibrosis

Resting energy expenditure was measured by open-circuit indirect calorimetry in 71 patients, aged 8.9 to 35.5 years, with cystic fibrosis who had no recent history of acute lung infection. Pulmonary function and nutritional status were studied simultaneously. In most patients, resting energy expenditure was above normal (range 95% to 153% of predicted values for age, sex, and weight as derived from the Harris Benedict equations), and was negatively correlated with pulmonary function (P less than 0.01) and nutritional status (P less than 0.01) when expressed as a percentage of body fat. Pulmonary status was positively correlated with nutritional status (P less than 0.01). We conclude that resting energy expenditure in patients with cystic fibrosis exceeds normal values and that the increase correlates with a deterioration in lung function and nutritional status.

Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs

BACKGROUND AND AIMS Nutritional status is a prognostic factor for survival in amyotrophic lateral sclerosis (ALS) patients. We investigated the contribution of some of the components contributing to resting energy expenditure (REE) in order to determine whether potentially higher energy needs should be considered for these patients. METHODS Thirty three ALS patients and 33 age- and gender-matched healthy controls participated. REE was measured by an open-circuit indirect calorimeter, body composition by dual energy X-ray absorptiometry, and estimated caloric intake by 7-day food records. RESULTS Patients had lower body mass indices and lower lean body mass (LBM) than healthy controls. REE values (as a percentage of predicted) was similar but increased when normalized by LBM (P<0.001). LBM and REE decreased while REE/LBM increased in ten patients who were reassessed 6 months later. A model for predicting measured REE was constructed based on the different components, with 86% prediction of its variability. CONCLUSIONS ALS is associated with increased REE. Various factors, such as poor caloric intake and mechanical ventilation, may mask this tendency. All the above parameters need to be considered during nutritional intervention to prevent additional muscle loss.

Changes in body composition during refeeding of patients with anorexia nervosa

Changes in body composition were studied in 13 girls with anorexia nervosa before and during 2 months of refeeding. Fat body mass and fat-free body mass were derived from skin-fold measurements. Total body potassium was measured by whole body counter, and intracellular water was calculated from it. Extracellular water was measured as the bromide space after oral bromide administration. A gradual increase was noted in weight, fat body mass, fat-free body mass, and total body potassium during refeeding. Extracellular water was expanded on admission and increased in all patients in the first weeks of treatment; later it fell to normal. Most of the changes in fat-free body mass over the first weeks of refeeding could be accounted for by an expansion in extracellular water. Particular care must therefore be taken with fluid balance during the first few weeks of refeeding.

Nutritional rehabilitation increases resting energy expenditure without affecting protein turnover in patients with cystic fibrosis

The effect of nutritional rehabilitation on several nutritional parameters was studied in eight malnourished patients with cystic fibrosis during the first year after gastrostomy tube insertion. Body composition was studied by fat skinfold measurements and by total body potassium count, resting energy expenditure (REE) by indirect calorimeter, and protein turnover by a single dose administration of [15N]glycine. Weight gain was accompanied by a significant increase in the various body compartments: weight 41.4 ± 7.5 to 46.1 ± 8.4 kg (p < 0.0002), fat body mass 5.6 ± 2.8 to 7.7 ± 3.4 kg (p < 0.005) and fat-free body mass (FFBM) 35.7 ± 6.3 to 38.3 ± 6.9 kg (p < 0.0003). REE increased significantly per kg of body weight as well as per kg of FFBM. No significant differences were found in protein turnover during reefed-ing nor in pulmonary function. We conclude that nutritional support restores body composition, but is accompanied by an increase in energy expenditure. This increase could not be attributed to increased protein turnover

Energy metabolism in children with newly diagnosed acute lymphoblastic leukemia.

ABSTRACT: The effect of tumor burden in acute lymphoblastic leukemia on resting energy expenditure, thermic effect of food, and substrate utilization was investigated with open-circuit indirect calorimetry after an overnight fast. Nine patients (six females, three males) ages 6.5 to 15.8 y were studied. Patients were divided into two groups according to their tumor burden at diagnosis (i.e. white cell count, presence or absence of mediastinal mass, or massive organomegaly). The patients with a greater tumor burden had increased energy expenditure. Their resting energy expenditure returned to normal in response to chemotherapy. These results must be interpreted with caution due to the small patient numbers (high tumor burden n = 3; low tumor burden n = 6). Substrate utilization was altered by chemotherapy with an increase in carbohydrate utilization and a decrease in fat oxidation (p < 0.009). The magnitude of the thermic effect of food tended to increase on treatment (p < 0.016). Inasmuch as most chemotherapy programs for children last up to 3 y, we believe it is important that the effects of chemotherapy on intermediary metabolism be studied, particularly in relationship to any possible permanent effects on growth and development.

Tube feeding with a diabetes-specific feed for 12 weeks improves glycaemic control in type 2 diabetes patients

BACKGROUND AND AIMS Assess longer-term (12 weeks) effects of a diabetes-specific feed on postprandial glucose response, glycaemic control (HbA1c), lipid profile, (pre)-albumin, clinical course and tolerance in diabetic patients. METHODS In this randomized, controlled, double-blind, parallel group study 25 type 2 diabetic patients on tube feeding were included. Patients received a soy-protein based, multi-fibre diabetes-specific feed or isocaloric, fibre-containing standard feed for 12 weeks, while continuing on their anti-diabetic medication. At the beginning, after 6 and 12 weeks, several (glycaemic) parameters were assessed. RESULTS The postprandial glucose response (iAUC) to the diabetes-specific feed was lower at the 1st assessment compared with the standard feed (p=0.008) and this difference did not change over time. HbA1c decreased over time in the diabetes-specific and not in the standard feed group (treatment*time:p=0.034): 6.9+/-0.3% (mean+/-SEM) at baseline vs. 6.2+/-0.4% at 12 weeks in the diabetes-specific group compared to 7.9+/-0.3% to 8.7+/-0.4% in the standard feed group. No significant treatment*time effect was found for fasting glucose, insulin, (pre-) albumin or lipid profile, except for increase of HDL in the diabetes-specific group. CONCLUSIONS The diabetes-specific feed studied significantly improved longer-term glycaemic control in diabetic patients. This was achieved in addition to on-going anti-diabetic medication and may affect clinical outcome.

Defective in vitro granulopoiesis in patients with anorexia nervosa

Patients with anorexia nervosa (AN) frequently suffer from a mild degree of anemia and from moderate leukopenia on top of their undernourished state and metabolic disarrangements. To evaluate in vitro granulopoiesis and its relationship to cytokine production and undernutrition, we have studied 10 adolescent girls with moderate AN (age range, 13.5-18.0). Study methods included assessment of peripheral blood (PB) granulocyte-macrophage colony-forming cells (GM-CFC) of the patients and age-matched controls, and determination of plasma and conditioned medium (CM) of mononuclear cells levels of IL-1, IL-3, IL-6, granulocyte-macrophage colony-stimulating factor(GM-CSF) and tumor necrosis factor (TNF), all of which may play a role in GM-CFC growth regulation. GM-CFC numbers were significantly lower in AN patients compared with the normal controls (13.09 ± 11.15versus 39.33 ± 26.61 colonies/5 × 105 cells,p < 0.01). No inhibitory effect was found in either plasma or CM of patients with AN. However, when CM were applied to non-recombinant human GM-CSF-stimulated normal bone marrow GM-CFC targets, the number of colonies stimulated by the CM of patients with AN was significantly lower than those stimulated by the CM of the controls (73.5 ± 20.1 versus 113.0 ± 11.6, p < 0.025). GM-CSF concentrations in CM were significantly lower in patients with AN compared with normal controls, but no such differences were found in IL-1, IL-3, IL-6, or TNF concentrations. These results indicate defective in vitro granulopoiesis in AN patients, manifested by a reduction of both GM-CFC and GM-CSF. It has to be determined whether these changes are the result of the basic disease process or are they due to malnutrition.

Pharmacokinetics of inhaled salbutamol in patients with cystic fibrosis versus healthy young adults

We studied the disposition of inhaled salbutamol in adolescents with cystic fibrosis (CF) and compared it with the pharmacokinetics of the drug given by the intravenous and inhaled routes in healthy adults. After inhalation of salbutamol, CF patients had a significantly larger area under the concentration-time curve derived from amounts of drug in the systemic circulation. The differences in serum concentration of salbutamol were not reflected in differences in change of heart rate. We conclude that the rate and extent of pulmonary absorption of inhaled salbutamol in patients with CF differ from those in healthy adults.

Outcome of percutaneous endoscopic gastrostomy insertion in patients with amyotrophic lateral sclerosis in relation to respiratory dysfunction

Abstract Our objective was to describe a group of ALS patients who underwent percutaneous endoscopic gastrostomy (PEG) insertion, with emphasis on the respiratory function, by comparing patients with forced vital capacity (FVC) > 30% versus FVC ≤ 30%, and the effect of respiratory dysfunction on the perioperative complication rate and survival. Thirty consecutive ALS patients in whom FVC status was known underwent PEG insertion at our centre. Twenty of them had FVC > 30% (50.1% ± 20) at the time of the procedure, and 10 had FVC ≤ 30% (20.1% ± 7). Demographic and clinical data were reviewed in each patient. Results showed that all patients had successful PEG insertion without any complications. There was no statistically significant difference between the two FVC groups regarding survival after the date of PEG insertion. In conclusion, in this relatively small patient sample there was no difference in complication rate and survival after PEG insertion between patients with poor respiratory function (FVC ≤ 30%) at the time of the procedure and patients with better respiratory function (FVC > 30%). Therefore, according to our data, PEG insertion may be regarded as safe even in patients with low FVC and should be offered even to patients with respiratory dysfunction.

Changes in body composition, resting energy expenditure, and thermic effect of food in short children on growth hormone therapy

The effect of growth hormone (GH) treatment on body composition, resting energy expenditure (REE), and the thermic effect of food (TEF) was studied in 10 prepubertal boys (aged 6.2 to 9.5 years, with subnormal spontaneous GH secretion during the first 6 months of treatment [0.2 IU/kg.dl]). Patients were studied before and at 2, 4, and 6 months after commencing treatment. Height and weight increased significantly during treatment (112.2 +/- 4.5 to 117.2 +/- 6.0 cm and 18.0 +/- 2.8 to 20.8 +/- 3.2 kg, respectively). Body fat percent decreased significantly (15.93% +/- 4.08% to 11.97% +/- 3.30%, P < .0002), but was not different at 4 and 6 months. Total body potassium (TBK) increased significantly (39.15 +/- 5.77 to 48.70 +/- 6.35 g, P < .001) during treatment. When correcting for the expected changes in body composition over time, height and weight were still shown to increase, fat percent decreased significantly, but TBK and mid-arm muscle circumference (MAMC) were not different from the expected values. REE increased significantly during treatment, but when it was expressed per TBK or corrected for the change in kilograms of fat-free body mass (FFBM), it increased only at 2 months and stabilized thereafter. The TEF was increased at 2 and 4 months of treatment and returned to pretreatment levels at 6 months of treatment. Substrate utilization as studied by indirect calorimetry pointed toward a significant protein-sparing effect during the first 4 months of treatment; this change tended to disappear in resting energy metabolism by 6 months of GH treatment.(ABSTRACT TRUNCATED AT 250 WORDS)