[Macroamylasemia: one pediatric case]. / A propos d'un cas pédiatrique de macroamylasémie.

We report a case of macroamylasemia in an 11-year-old boy. We compare our clinical and paraclinical data with those described in pediatric literature. Macroamylase resulted in a complex of amylase and immuglobulin. Its fortuitous detection did not reveal, up to now, any associated pathology, in particular any autoimmune disorders or celiac disease. Identification of this biochemical abnormality is essential in order to avoid invasive investigations and/or unnecessary therapies.

[Hypertransaminasemia in an adolescent]. / Macrotransaminasémie chez un adolescent.

CASE REPORT: An adolescent admitted to hospital because of an obvious convulsion seizure presented with a high level of serum macroaspartate aminotransferase. This macroaspartate aminotransferase was discovered by chance when blood tests were made. DISCUSSION: Macroaspartate aminotransferase is a persistent, benign phenomenon, probably not congenital, discovered either in healthy patients, or in adults suffering from malignancies or autoimmune diseases. Macroenzymes have been identified as a cause of benign increase in a number of serum enzymes, like macroamylase serum levels. The macroenzyme is often an immunoglobulin G-complexed enzyme. CONCLUSION: It is important for clinicians to be aware of their existence in order to avoid unnecessary procedures. It is important that the patient is informed of the macroaspartate aminotransferase and that the same is stated in his health record.

Intestinal permeability in liver cirrhosis: relationship with severe septic complications.

OBJECTIVES: Patients with liver cirrhosis are at high risk of severe septic complications such as spontaneous bacterial peritonitis (SBP) and bacteraemia. The aims of this study were to assess intestinal permeability in patients with liver cirrhosis and to search for a relationship between an impaired intestinal permeability and the occurrence of severe septic complications. METHODS: Intestinal permeability was assessed in a group of 80 cirrhotic patients (Child A, n = 13; Child B, n = 26; Child C, n = 41) and 28 healthy control subjects. A severe septic complication (bacteraemia and/or SBP) occurred in 16 patients, within 10 days before (n = 8 cases) or after (n = 8 cases) the test was performed. Lactulose (LAC) 10 g was given orally together with mannitol (MAN) 5 g, and urinary excretion rates were determined. RESULTS: Urinary mannitol excretion (MAN%) was lower while the LAC/MAN ratio was higher in patients than in control subjects (P < 0.001); these abnormalities were more marked in Child C patients (Child C patients vs control subjects: MAN%, 8.20 +/- 0.79 vs 14.59 +/- 0.58, P < 0.001; LAC/MAN, 0.066 +/- 0.026 vs 0.017 +/- 0.001, P < 0.02). When compared with non-infected patients, septic patients had a lower MAN% and an increased LAC/ MAN ratio (5.45 +/- 1.12 vs 9.83 +/- 0.87, P < 0.02; 0.130 +/- 0.063 vs 0.029 +/- 0.005, P < 0.02). CONCLUSION: Although the main mechanism involved in the decrease in MAN% is likely a reduction in area of the intestinal absorptive surface, these results argue in favour of an increased intestinal permeability in liver cirrhosis, especially in patients with severe infectious complications. The impairment of intestinal function barrier may contribute to severe septic complications in these patients.

Influence of liver failure, ascites, and energy expenditure on the response to oral nutrition in alcoholic liver cirrhosis.

The influence of liver failure, ascites, and energy expenditure on the response to oral nutrition was assessed in a group of 55 alcoholic cirrhotic patients. Caloric intake, nutritional status, resting energy expenditure (REE), and Child-Pugh score were evaluated before and after 1 mo of oral nutrition. Patients were severely malnourished, 73% had muscular midarm circumference (MMAC) below the 5th percentile of a reference population, 51% had triceps skinfold thickness below the 25th percentile. Eleven patients were in class A of Child, 19 in class B, and 25 in class C. Twenty-six patients were nonascitic, whereas ascites was resolved in 10 ascitic patients by the end of the study and 19 patients had refractory ascites. Liver damage was more pronounced and did not improve during the study in patients with refractory ascites. Caloric intake was approximately 40 kcal/kg of body weight and was in the same range in the three groups according to Child classification. Fat mass (FM) increased, respectively, from 17.4% +/- 1.7% to 19.5% +/- 1.4%, P < 0.01, in Child A patients; from 17.1% +/- 1.4% to 19.3% +/- 1.4%, P < 0.001, in Child B patients; and from 17.6% +/- 1.5% to 18.8% +/- 1.5%, P < 0.05, in Child C patients. The increase in FM was comparable in the three groups, whereas MMAC and the creatinine/height ratio did not change significantly. FM was lower and did not increase in patients with refractory ascites. Child C patients were characterized by an increase in the rate of glucose oxidation (P < 0.02) and a decrease in the rate of lipid oxidation (P < 0.05). High-density lipoprotein cholesterol and apolipoprotein (Apo) A1 were reliable indices of improvement of liver function in patients with severe liver failure, ApoA1 was also a marker of improvement of metabolic impairment. With respect to the measured REE/predicted REE ratio calculated according to Harris-Benedict equation (r), 19 patients were considered hypermetabolic (r < 1.1), 30 normometabolic (0.9 < r < 1.1), and 6 hypometabolic (r < 0.9). An increase in FM correlated with r (P < 0.01) and was more marked in hypermetabolic patients. In contrast to the other two groups, Child-Pugh score and nutritional status remained unchanged in the hypometabolic patients. These results show that severe liver failure did not preclude improvement of nutritional status provided caloric intake was high. In Child C patients, improvement of nutritional status paralleled improvement of liver function and normalization of oxidative metabolism. Refractory ascites had negative effects on changes in nutritional status and liver function. Despite adequate caloric intake to energy requirements, hypometabolism has a poor prognosis regarding both nutritional status and liver function.

Markers of oxidative stress in fit, health-conscious elderly people living in the Paris area. The Research Group on Ageing (GERBAP)

Lipid peroxidation index and antioxidant indicators were assessed by biochemical means in 193 healthy elderly volunteers (103 men and 90 women), ages 70-89 y and living freely in the Paris area. Lipid peroxidation index was in the same range as in young adults. Zinc, copper, and selenium levels were satisfactory and similar to those in young adults, though the range of copper values tended to be higher. Copper and selenium levels were higher in elderly women than in men. However, for selenium values this sex-related difference disappeared in elderly volunteers > 75 y. Copper-zinc-superoxide dismutase and glutathione peroxidase activities were similar to those in young adults, with no influence of sex or age. Vitamin E and total carotene, closely related to cholesterol levels, were satisfactory. Our findings show that markers of oxidative stress are not influenced by old age when good health and nutritional status are preserved, as in this selected population.

[Reference intervals and biological profile in a group of healthy elderly population in the Paris region]. / Intervalles de référence et profil biologique d'une population de sujets âgés "en bonne santé" habitant la région parisienne. (Group d'évaluation et de recherche des biologistes de l'APHP).

The superimposition of pathological processes on ageing-related metabolic changes makes the interpretation of laboratory data difficult in the elderly. Therefore, in order to establish reference values of anthropometric, hematological and biochemical variables, we have carefully selected fit, health-conscious elderly subjects on the basis of clinical and biological criteria. We observed a trend for a wider range of values than in young adults. For some variables, the values were shifted down (eg: albumin, vitamin D) or up (eg: glucose, urea, cholesterol, ferritin), as a result of slow metabolic changes or progressive functional decline of different organs. The high prevalence of hypovitaminosis D was worthy of note, particularly in women, suggesting a high risk of deficiency.

Short-term changes in energy metabolism after 1 month of a regular oral diet in severely malnourished cirrhotic patients.

Malnutrition in patients with liver cirrhosis is currently associated with abnormal fuel metabolism. The aim of this study was to evaluate changes in energy production and substrate oxidation rates in a group of 26 nonanorectic severely malnourished cirrhotic patients in stable clinical condition after 1 month of an oral diet. Child-Pugh score, nutritional status, energy expenditure, rates of nutrient oxidation, and plasma levels of intermediary metabolites in the postabsorptive phase were assessed before and after 1 month of oral nutrition. Upon entry onto the study, caloric and protein intakes were 40.1 +/- 2.0 kcal/kg and 1.44 +/- 0.8 g/kg, respectively. The Child-Pugh score did not change during the study, whereas nutritional status improved as shown by increased muscular midarm circumference, ([MMAC] P < .02), height-creatinine index (P < .05), triceps skinfold thickness ([TST] P < .01), and fat mass (P < .001). Inflammatory state improved during the study, as shown by the decrease of C-reactive protein ([CRP] P < .01) and orosomucoid (P < .001). The ratio of caloric intake to resting energy expenditure (REE) increased (1.53 +/- 0.06 v 1.66 +/- 0.07, P < .05), as well as the rate of glucose oxidation ([Gox] 73.6 +/- 9.9 v 128.1 +/- 10.3 mg/min, P < .001) and urine nitrogen excretion (6.69 +/- 0.47 v 7.96 +/- 0.48 g/d, P < .02). On the other hand, the rate of lipid oxidation (Lox) decreased (67.3 +/- 3.9 v 47.3 +/- 4.9 mg/min, P < .001) and was correlated with the decrease of free fatty acid (FFA) levels (P < .05).(ABSTRACT TRUNCATED AT 250 WORDS)

Extensive laboratory assessment of nutritional status in fit, health-conscious, elderly people living in the Paris area. Research Group on Aging.

OBJECTIVE: Nutritional status-related biological indexes were measured in fit, health-conscious elderly subjects in order to establish reference values for people over 70 years. SUBJECTS: 103 men and 90 women aged 70-89 years living freely in the Paris area volunteered to participate. METHODS: Nutritional status was assessed by anthropometric and biochemical methods. RESULTS: Serum protein and amino acid status was similar to that of young adults, with only 5.2% of the elderly subjects showing transthyretin concentrations < 0.20 g/L, as well as decreased essential amino acid levels. Iron status, assessed in terms of serum and erythrocyte ferritin levels, total iron binding capacity and erythrocyte protoporphyrin tended to be satisfactory, but iron depletion was detected in 8.8% of the subjects. Serum ferritin levels were elevated in 19.7% of the subjects. Folate and vitamin B12 status was satisfactory, while hypovitaminosis D was observed in 48.2% of cases. CONCLUSION: Our findings suggest that, in aging uncomplicated by disease, nutritional status is similar to that in younger adults, although the range of values tended to be wider, with a higher risk of certain nutrient deficiencies.

Nutritional and metabolic consequences of basal hyperinsulinemia in alcoholic liver cirrhosis: relationship with postprandial changes in erythrocyte insulin-receptor affinity.

The nutritional and metabolic consequences of basal hyperinsulinemia were investigated in a group of 13 alcoholic cirrhotic patients; 7 healthy subjects were studied as a control group. Two groups of patients were defined on the basis of fasting insulin level: group 1 (n = 7) displayed acute hyperinsulinemia (> mean of control group + 2SD), and group 2 (n = 6) had lower insulin levels. Nutrition status was assessed by means of anthropometric parameters; the rates of nutrient oxidation were measured after an overnight fast and 2 h after a standard meal intake. Group 1 had better nutrition status in terms of fat mass than group 2 (p < 0.05). Although the basal rates of nutrient oxidation were in the same range in the three groups, postprandially, the rate of lipid oxidation was significantly different (p < 0.01). Moreover, group 1 showed greater inhibition of postprandial lipid oxidation than the control group (p < 0.05), whereas there was no difference between group 2 and the control group. In the postprandial phase, erythrocyte insulin-receptor binding and affinity increased paradoxically in group 1, whereas they decreased in group 2 and healthy subjects (changes in binding, p < 0.025; changes in affinity, p < 0.01). In conclusion, basal hyperinsulinemia in alcoholic liver cirrhosis is related to more marked inhibition of postprandial lipid oxidation and better-preserved nutrition status and may lead to a paradoxical postprandial increase in insulin-receptor affinity.

Iron status of a healthy French population: factors determining biochemical markers.

The iron status of a representative population sample in a district of Paris area (France) was assessed using a biochemical and dietary approach. Complete data were obtained for 1,108 subjects 6 months to 97 years old. Total iron intake increased up to adolescence and then remained stable in adult life. Iron intake was higher in men than in women. Most children and menstruating women presented a dietary iron intake below the recommended allowances. While anemia was not very common, iron deficiency (defined as the existence of at least two abnormal values in the four independent indicators of iron status: serum ferritin, erythrocyte protoporphyrin, transferrin saturation and MCV) was particularly common in infants, young children, menstruating women and elderly men. Serum ferritin, erythrocyte protoporphyrin and transferrin saturation were significantly correlated with inflammatory markers. Significant correlations were found between dietary total iron and serum ferritin (r = 0.29, p < 0.001) and hemoglobin (r = 0.44, p < 0.001). After adjustment for age, sex and inflammation, using multiple linear regression models, the relationship between both heme and nonheme iron intake and serum ferritin remained significant. Serum ferritin and hemoglobin levels were negatively correlated with calcium and phosphorus intake.

Vitamin status of a healthy French population: dietary intakes and biochemical markers.

The vitamin status of a representative population sample in a French district in the Paris area was assessed using a biochemical and dietary approach. Complete data were obtained for 1039 subjects 6 to 97 years old. Dietary intakes of most vitamins increased up to adolescence and then remained stable or decreased slightly in adult life. Total vitamin intakes were higher in men than in women (except for ascorbic acid), but the vitamin density of the diet was higher in females. Most subjects presented dietary intakes below the French recommended allowances for vitamin B1, B6, C, A and E. A variation in biochemical parameters according to age and sex was observed only for serum concentration of retinol, beta-carotene, vitamin E and C. Multiple regression analysis showed that dietary vitamin intakes were related to the biological status for vitamin B2, B6, C, folates, beta-carotene and vitamin E. Tobacco smoking, alcohol consumption and oral contraceptive were significantly correlated with biological status for several vitamins. While biochemical evidence of severe vitamin deficiency was not observed, a sizeable minority of the French population is not lavishly supplied with some vitamins, and this situation deserves investigation to see if any deleterious effects are associated with it.

The thermogenic and metabolic effects of food in liver cirrhosis: consequences on the storage of nutrients and the hormonal counterregulatory response.

The thermogenic effect of food and the rates of oxidation and storage of nutrients were evaluated by indirect calorimetry in 10 cirrhotic patients and seven normal controls for a 6-hour period, after they had consumed a standard meal supplying 15 kcal/kg body weight with 15%, 30%, and 55% protein, fat, and carbohydrate calories, respectively. Although the thermogenic response to food was not significantly lower in patients than in controls (51.6 +/- 13.5 v 72.2 +/- 8.8 kcal/6 h), patients exhibited a delayed and blunted increment of energy expenditure after the meal intake (P less than .025). The greater part of the glucose load was oxidized in patients (70.2 +/- 3.9% v 50.4 +/- 3.9% in controls; P less than .01), suggesting a defective glucose storage as glycogen. This result could be related to insulin resistance, which was evidenced by a large increase in glucose and insulin levels after the meal intake in patients (P +/- .001). Conversely, lipid oxidation was sharply reduced and de novo lipogenesis occurred in patients, so that the rate of lipid storage was increased. The profiles of circulating levels of catecholamines, thyroid hormones (free thyroxine [FT4] and triiodothyronine [T3]), and glucagon were assayed during the test. Norepinephrine and glucagon levels remained higher in patients throughout the test (P less than .001), whereas thyroid hormones stayed in the same range in the two groups. After an initial increase, glucose levels decreased sharply, inducing an activation of counterregulatory hormones, glucagon, and notably, epinephrine, for which the increment was correlated with the decrease of glucose (r = -.917; P less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)

Aging, energy expenditure and nutritional status: evidence for denutrition-related hypermetabolism.

Resting energy expenditure (REE) and nutritional status have been evaluated in 39 elderly inpatients (mean age 84.0 +/- 6.9 years). The nutritional status declined with aging as shown by significant negative correlations between age and, respectively, weight (p < 0.001), fat free mass (FFM) (p < 0.01) and body mass index (BMI) (p < 0.01). The best prediction for REE when considering the whole population was given by FFM (p < 0.001). Increased REE was observed in patients after recent surgery, related to an inflammatory state, as shown by increased plasma levels of C-reactive protein and orosomucoid. Irrespectively of the inflammatory state, REE was also found to be related to the nutritional status: patients who showed a BMI < 20 had higher REE than patients with a BMI > 20 (28.4 +/- 1.3 vs. 22.1 +/- 0.7 kcal/kg of body weight; p < 0.001; 35.7 +/- 1.6 vs. 30.9 +/- 1.0 kcal/kg of FFM; p < 0.02). These results increasingly suggest that elderly patients may suffer from denutrition relevant to hypermetabolism.

[Thermic and metabolic effects of meals in liver cirrhosis assessment of oxidation and storage rates of nutrients]. / Effets thermogénique et métabolique du repas chez le cirrhotique: étude des capacités d'oxydation et de stockage des nutriments.

The thermic effect of food was evaluated in 10 cirrhotic patients and 7 normal controls by indirect calorimetry during 6 hours following intake of a mixed meal supplying 15 kcal/kg of body weight and containing 30, 15 and 55 percent as lipid, protein and carbohydrate calories respectively. The rates of storage and oxidation of nutriments, as well as variations of blood glucose, insulin, plasma lactates, free fatty acids, glycerol, and ketonic corps were also evaluated. The thermogenic response to food was lower (p less than 0.025) and delayed in cirrhotic patients. In cirrhotic patients the rate of glucose oxidation was significantly increased (70.2 +/- 3.9 vs 50.4 +/- 3.9 percent of the glucose load; p less than 0.01) suggesting a defect in glycogen storage. These results can be related to insulin resistance as attested by a larger increase of glucose and insulin levels in cirrhotics than in controls (p less than 0.001 and p less than 0.001, respectively). Compared with controls, lipolysis in cirrhotic patients was more suppressed as shown by a sharper decrease of free fatty acids and glycerol levels (p less than 0.001 and p less than 0.02, respectively). Furthermore, the rate of lipid oxidation decreased more in cirrhotic as compared with controls (p less than 0.001) before becoming completely suppressed. De novo lipogenesis appeared between the 2nd and 4th hours. Consequently, the rate of lipid oxidation was significantly reduced in cirrhotic vs controls (14.3 +/- 5.0 vs 30.5 +/- 3.7 percent of the lipid load; p less than 0.02) showing an increased rate of lipid storage.(ABSTRACT TRUNCATED AT 250 WORDS)

Hormonal and metabolic changes during exercise in cirrhotic patients.

The metabolic response to exercise was compared in 10 cirrhotic patients (P) in a stable clinical condition and in 6 sedentary, age-matched, normal subjects (C) performing 32 minutes of treadmill exercise with the same constant workload corresponding to three to four times their resting oxygen uptake. Taking indirect calorimetry as reference, respiratory exchanges indicated that cirrhotic patients consumed carbohydrates almost exclusively, unlike the normal controls, who consumed lipids and glucids in about the same proportions (RQ: 0.98 +/- 0.04 v 0.87 +/- 0.04, P less than .0001). In the patients, this carbohydrate path of exercise metabolism lowered glycemia from the resting value of 5.23 +/- 0.16 mmol/L to 4.03 +/- 0.37 mmol/L (P less than .0001) and raised the plasma lactate concentration from 2.08 +/- 0.24 mmol/L at rest to 3.48 +/- 0.32 mmol/L at the eighth minute of exercise (P less than .001), thus suggesting defective liver glyconeogenesis. Fatty free acids and glycerol remained almost constant during exercise, whereas catecholamines increased. Insulin levels were high in patients at rest (67.1 +/- 14.5 U/mL v 15.1 +/- 3.5 U/mL); they declined sharply at the onset of exercise but nevertheless remained high compared to those observed in the controls (P less than .0001). Glucagon increased in exercising patients from 88.3 +/- 21.3 pg/mL to 127.4 +/- 30.6 pg/mL (NS). Esterified plasma carnitine declined in the patients from 13.0 +/- 2.2 mumol/L to 8.6 +/- 1.5 mumol/L (P less than .05).(ABSTRACT TRUNCATED AT 250 WORDS)

[Energy expenditure and the use of nutriments in cirrhotic patients fasting and at rest. Influence of alcoholic hepatitis and the severity score of the disease]. / Dépenses énergétique et utilisation des nutriments chez le cirrhotique à jeun et au repos. Influence de l'hépatite alcoolique et du score de gravité de la maladie.

Resting energy expenditure and nature of fuels consumed after an overnight fast have been evaluated in 30 cirrhotic patients and 10 normal subjects by indirect calorimetry. Basal metabolic requirements in patients were slightly increased although most of them had as poor nutritional condition assessed by a decreased muscular mass (17.3 +/- 0.8 vs 22.5 +/- 1.2 kg, p less than 0.001). Resting energy expenditure was significantly elevated in 16.7 p. 100 of patients. This hypermetabolic state was related to alcoholic hepatitis. Surface area and the Harris-Benedict equation did not accurately predict the resting energy expenditure. Compared to normal subjects, all patients had higher levels of free fatty acids (0.408 +/- 0.05 vs 0.182 +/- 0.03 mmol/l, p less than 0.001), glycerol (0.109 +/- 0.009 vs 0.035 +/- 0.003 mmol/l, p less than 10(-9)) and ketone bodies (0.137 +/- 0.012 vs 0.099 +/- 0.012 mmol/l, p less than 0.03). The respiratory quotient was lower in cirrhotic patients (0.740 +/- 0.01 vs 0.832 +/- 0.02, p less than 10(-5)). These results show increased lipolysis and a preferential use of fat as fuel substrates in cirrhotic patients: 70 p. 100 of the total calorie expenditure was derived from fat. Fat mobilization was influenced by nutritional status: a positive correlation has been found between fat mass and free fatty acids (p less than 0.02) and between fat mass and glycerol (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)