RESUMO
There is a significant rate of therapeutic failure in rheumatoid arthritis (RA) patients treated with leflunomide (LEF). This study investigates the utility values of teriflunomide levels (A77 1726) in identifying RA patients who remained with moderate or severe disease activity after the treatment with LEF. In this cross-sectional study, we compared: (a) RA patients who achieved a DAS28-ESR ≤ 3.2, and (b) RA patients who maintained a DAS28-ESR > 3.2 after treatment. ROC curves determined the cut-off of A77 1726 with the better performance to identify patients achieving a DAS28-ESR ≤ 3.2. Of the 115 patients treated with LEF, 69 (60%) remained with moderate/severe disease activity and 46 (40%) achieved low disease activity/remission. Higher A77 1726 levels showed a negative correlation with DAS28-ESR (r = - 0.42, p < 0.001) and other parameters of disease activity. We obtained the following utility values with the cut-off of A77 1726 > 10 µg/mL to identify RA patients who achieved a DAS28-ESR ≤ 3.2: sensitivity of 91.31%; specificity of 73.91%; positive predictive value of 70.00%; and negative predictive value of 92.73%. Serum A77 1726 discriminated between RA patients who remained with moderate/severe disease activity despite the treatment with LEF both as monotherapy and LEF as combo therapy.
Assuntos
Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Crotonatos/uso terapêutico , Hidroxibutiratos/uso terapêutico , Leflunomida/uso terapêutico , Nitrilas/uso terapêutico , Toluidinas/uso terapêutico , Adulto , Idoso , Antirreumáticos/efeitos adversos , Antirreumáticos/sangue , Artrite Reumatoide/sangue , Artrite Reumatoide/diagnóstico , Estudos Transversais , Crotonatos/efeitos adversos , Crotonatos/sangue , Monitoramento de Medicamentos , Quimioterapia Combinada , Feminino , Humanos , Hidroxibutiratos/efeitos adversos , Hidroxibutiratos/sangue , Leflunomida/efeitos adversos , Leflunomida/sangue , Masculino , Pessoa de Meia-Idade , Nitrilas/efeitos adversos , Nitrilas/sangue , Valor Preditivo dos Testes , Indução de Remissão , Índice de Gravidade de Doença , Fatores de Tempo , Toluidinas/efeitos adversos , Toluidinas/sangue , Resultado do TratamentoRESUMO
Metabolomics has been increasingly used to evaluate metabolic changes associated with morbidities. The objective of this study is to assess the metabolic profile before and after intervention with mixed dietary fiber in overweight and obese hypertensive women. This is an intervention study, and the sample consists of 14 women aged 28 to 58 years. An intervention with 12 g of mixed soluble and insoluble fiber is performed for a period of eight weeks. Serum metabolites are identified using a Bruker 1H NMR spectrometer at 400 MHz. Multivariate data analysis, including principal component analysis (PCA), is used to differentiate the two groups. After supplementation with dietary fiber, there is a significant increase in the peak intensity values of the metabolites HDL-C (0.0010*), choline (0.0012*) and hydroxybutyrate (0.0010*) as well as a decrease in systolic (0.0013*) and diastolic (0.0026*) blood pressure. The analysis of the metabolomic profile allows the identification of metabolites that have been associated in the literature with hypertension and excess weight (choline, hydroxybutyrate and amino acids) and with fiber intake (choline, hydroxybutyrate and amino acids) in addition to an increase in HDL-C. The increase in the detection of the described metabolites possibly occurs due to the presence of pathologies and the use of fiber in the intervention, which also contributes to elevated HDL-c and reduced blood pressure.
Assuntos
Colina/sangue , Fibras na Dieta/farmacologia , Suplementos Nutricionais , Hidroxibutiratos/sangue , Hipertensão/sangue , Lipoproteínas HDL/sangue , Sobrepeso/sangue , Adulto , Feminino , Humanos , Hipertensão/complicações , Espectroscopia de Ressonância Magnética , Pessoa de Meia-Idade , Obesidade/sangue , Sobrepeso/complicaçõesRESUMO
BACKGROUND: To evaluate the biological response of the sows and their offspring with oral administration of Lactobacillus plantarum CAM6 in breeding sows, a total of 20 Pietrain breeding sows with three farrowings and their descendants were used, randomly divided into two groups of 10 sows each. Treatments included a basal diet (T0) and basal diet +10 mL biological agent containing 109 CFU/mL L. plantarum CAM6 (T1). No antibiotics were used throughout the entire experimental process of this study. RESULTS: The L. Plantarum CAM6 supplementation in sows' feeding did not affect (P > 0.05) the reproductive performance of the sows; however, the number of deaths for their offspring before weaning (P ≤ 0.05) decreased. In addition, the oral administration of Lactobacillus plantarum CAM6 in sows increased (P ≤ 0.05) the content of lactose, nonfat solids, mineral salts, and the density of sows' milk, with a decrease in milk fat. Moreover, the probiotic feed orally to the sows improved the body weight (P ≤ 0.05) and reduced the diarrhea incidence of their offspring (P ≤ 0.05). Also, the probiotic administration of sows changed (P ≤ 0.05) the serum concentration of Na+, pCO2, and D-ß-hydroxybutyrate and increased (P ≤ 0.05) the leukocytes, lymphocytes, and platelets in their piglets. CONCLUSION: Oral administration of Lactobacillus plantarum CAM6 in breeding sows improved body weight, physiological status, and the health of their offspring. And preparing the neonatal piglets physiologically is of great importance to the pig farming industry which could decrease the operational cost and medication (especially antibiotics) consumption of the pig producers.
Assuntos
Lactobacillus plantarum/fisiologia , Animais , Feminino , Hidroxibutiratos/sangue , Lactação/fisiologia , Reprodução/fisiologia , Suínos , DesmameRESUMO
The purpose of this study was to determine the effects of stage and the number of lactation on plasma ascorbate concentration and to establish the association between plasma ascorbate concentrations and plasma glucose, insulin, nonesterified fatty acids (NEFA), beta-hydroxybutyrate (BHBA), aspartate aminotransferase (AST), milk somatic cell count (SCC), milk yield, and body condition score (BCS) in dairy cows. Holstein cows (n = 193) from three different herds were used in this study. Animals were randomly selected, and assigned to five groups according to stage of lactation (group 1, dry cows; group 2, 1 to 28 d; group 3, 29 to 56 d; group 4, 57 to 140 d; group 5, 141 to 280 d), and the number of lactation (primiparous or multiparous). Plasma ascorbate concentration, plasma glucose concentration, serum insulin concentration, plasma NEFA concentration, plasma BHBA concentration, serum AST concentration, milk SCC, milk yield, and BCS were measured. The results of this study demonstrate that plasma ascorbate concentrations do not change in response to stage of lactation, or number of lactations. Among the several variables studied, none was found that, singly or in combinations, could explain variations in ascorbic acid concentrations.
Assuntos
Ácido Ascórbico/sangue , Lactação/fisiologia , Leite/citologia , Animais , Aspartato Aminotransferases/sangue , Glicemia/análise , Bovinos , Contagem de Células , Ácidos Graxos não Esterificados/sangue , Feminino , Hidroxibutiratos/sangue , Insulina/sangueRESUMO
Alcohol ingestion decreases plasma free fatty acids (FFAs) and lipid oxidation. This study was conducted to determine palmitate turnover in alcoholics during a short abstinence period and after an ethanol load and in a group of nonalcoholic control subjects, looking for correlations between palmitate turnover, FFA, acetate, and acetoacetate/beta hydroxybutyrate ratio (AKBR). Palmitate C14 turnover was studied in five alcoholics during early abstinence and after a 0.8 g/kg ethanol load, and in five nonalcoholic normal controls. Plasma levels of FFA, acetate, acetoacetate, and beta hydroxybutyrate were measured before and during the ethanol load. A needle hepatic biopsy was performed in alcoholics. FFA levels, palmitate flux, oxidation, and nonoxidative disposal were similar in alcoholics compared with control subjects, decreasing significantly after the ethanol load in both groups. AKBR and ketone bodies were similar in both groups in the basal period. After the alcohol infusion, AKBR decreased significantly. Acetoacetate levels did not change, and beta hydroxybutyrate and total ketone bodies increased significantly in alcoholics and control subjects. A positive correlation was found between FFA levels and palmitate flux. Liver biopsies showed mild changes in the patients studied. The similar inhibition of lipid turnover, FFA release, and the drop in AKBR observed after an alcohol load in alcoholics and control subjects suggest that this effect is mediated by alcohol metabolism and not by metabolic alterations present in alcoholics.
Assuntos
Alcoolismo/sangue , Etanol/administração & dosagem , Lipídeos/sangue , Ácido 3-Hidroxibutírico , Acetatos/sangue , Acetoacetatos/sangue , Adulto , Alcoolismo/patologia , Biópsia por Agulha , Etanol/sangue , Ácidos Graxos não Esterificados/sangue , Humanos , Hidroxibutiratos/sangue , Corpos Cetônicos/sangue , Cinética , Fígado/patologia , Taxa de Depuração Metabólica , Ácido Palmítico/sangueRESUMO
1. The influence of nutritional status on the plasma and abomasal fluid disposition kinetics of albendazole (ABZ) and its metabolites, albendazole sulphoxide (ABZSO) and albendazole sulphone (ABZSO2), has been investigated in the calf. 2. Free fatty acid (FFA) and beta-hydroxybutyrate (beta-OHB) serum concentrations were significantly higher in the feed-restricted (poor nutritional status) compared with control calf (optimal nutritional status). 3. ABZ parent drug was not detected in plasma at any time post-treatment and ABZSO and ABZSO2 were the metabolites detected in plasma. Both metabolites were rapidly depleted from the bloodstream. ABZ and its metabolites were recovered in abomasal fluid from 0.25 up to either 48 (ABZ) or 120h (ABZSO and ABZSO2) post-treatment. 4. The plasma disposition kinetics of both ABZ metabolites was significantly changed in the feed-restricted compared with control calf. ABZSO and ABZSO2 plasma area under the curves (AUCs) were significantly higher in the restricted animal. These enhanced AUCs correlated with significantly longer plasma half-lives (T1/2el) and mean residence times (MRTs) for these metabolites. 5. The delayed elimination of ABZ metabolites from the bloodstream correlated with the higher concentration of these molecules recovered in the abomasal fluid of the calves subjected to a dietary restriction. 6. The changes observed on disposition kinetics may reflect an impairment on the hepatic metabolism and clearance of ABZ as a consequence of FFA mobilization from adipose tissue and overproduction of ketone bodies in the liver.
Assuntos
Albendazol/farmacocinética , Anti-Helmínticos/farmacocinética , Estado Nutricional , Ácido 3-Hidroxibutírico , Abomaso/metabolismo , Albendazol/sangue , Animais , Anti-Helmínticos/sangue , Bovinos , Ácidos Graxos não Esterificados/sangue , Hidroxibutiratos/sangueRESUMO
Thirty-two cows, averaging 112 DIM, were assigned to four dietary treatments: 1) control, 2) Ca salts of fatty acids, 3) nicotinamide, and 4) Ca salts of fatty acids blended with nicotinamide during manufacture. Preliminary studies showed that nicotinamide survives blending with Ca salts of fatty acids during manufacture and that a blended mixture of nicotinamide and Ca salts of fatty acids gave results similar to those from nicotinamide plus Ca salts of fatty acids supplemented separately. Calcium salts of fatty acids increased milk fat percentage, decreased milk protein percentage, but had no effect on production of milk, FCM, fat, or protein. Nicotinamide increased production of milk and protein, decreased fat percentage, but had no effect on either production of FCM and protein or percentage of protein. Calcium salts of fatty acids increased NEFA in blood, and dietary nicotinamide increased concentrations of nicotinamide in blood, but glucose and BHBA in blood were unaffected by either dietary ingredient. Therefore, in these midlactation cows, the decreased milk protein percentage caused by supplemental dietary fat was prevented by nicotinamide. Supplementation with only nicotinamide increased total production of milk protein.
Assuntos
Bovinos/fisiologia , Dieta , Gorduras na Dieta/administração & dosagem , Lactação/efeitos dos fármacos , Leite/metabolismo , Niacinamida/farmacologia , Ácido 3-Hidroxibutírico , Animais , Glicemia/metabolismo , Metabolismo Energético , Ácidos Graxos/administração & dosagem , Ácidos Graxos não Esterificados/sangue , Feminino , Hidroxibutiratos/sangue , Proteínas do Leite/metabolismo , Niacinamida/administração & dosagem , Niacinamida/sangue , ParidadeRESUMO
1. The concentrations of acetoacetate and 3-hydroxybutyrate have been determined in the blood of the green lizard Ameiva ameiva (Teiidae) in fed animals and in animals starved for periods from one week to about four months. 2. The concentrations of acetoacetate are low and unaltered in fed and starved animals, being in the range from 0.014 to 0.018 mM. 3. The concentrations of 3-hydroxybutyrate are high: 2.67 mM, in fed animals, falling during starvation down to 0.26 mM. 4. The 3-hydroxybutyrate/acetoacetate ratio is high, 151, in fed animals, falling down to 17. 5. The possible importance of ketone bodies in the metabolism of Ameiva ameiva is discussed.
Assuntos
Corpos Cetônicos/sangue , Lagartos/fisiologia , Ácido 3-Hidroxibutírico , Acetoacetatos/sangue , Animais , Hidroxibutiratos/sangue , Valores de Referência , Inanição , Fatores de TempoRESUMO
Fourteen infants requiring long-term total parenteral nutrition but able to tolerate small quantities of enteral feedings were randomized into carnitine treatment and placebo control groups. All infants had received nutritional support devoid of carnitine. Plasma carnitine levels and observed plasma lipid indices were not different before supplementation. Under standardized, steady-state conditions, 0.5 g/kg fat emulsion (intralipid) was administered intravenously over 2 hours both before and after infants received 7 days of continuous nasogastric or gastric tube L-carnitine (50 mumol/kg/day) or placebo. Plasma triglyceride, free fatty acid, acetoacetate, beta-hydroxybutyrate, and carnitine concentrations were observed at 0 (start of lipid infusion), 2, and 4 hours for pre- and post-treatment periods, and in addition at 6 and 8 hours after carnitine supplementation. Infants receiving carnitine had significantly greater beta-hydroxybutyrate plasma concentrations (P less than 0.05) and carnitine (P less than 0.001) at 0, 2, 4, 6, and 8 hours, and greater plasma acetoacetate concentrations (P less than 0.05) at 2, 4, 6, and 8 hours, compared with controls. Twenty-four-hour urinary carnitine excretion was very low for both groups before supplementation; after supplementation, excretion was higher (P less than 0.05) in the carnitine group. No significant differences were found between groups for plasma triglyceride or free fatty acid concentrations at any observation period. This study demonstrated enhanced fatty acid oxidation, as evidenced by increased ketogenesis, with L-carnitine supplementation in infants receiving long-term total parenteral nutrition.
Assuntos
Carnitina/administração & dosagem , Metabolismo dos Lipídeos , Nutrição Parenteral Total , Ácido 3-Hidroxibutírico , Administração Oral , Carnitina/sangue , Carnitina/urina , Ensaios Clínicos como Assunto , Ácidos Graxos não Esterificados/sangue , Feminino , Humanos , Hidroxibutiratos/sangue , Lactente , Masculino , Distribuição Aleatória , Fatores de Tempo , Triglicerídeos/sangueRESUMO
We compared neonatal fasting metabolism in 12 infants of obese women and 10 control infants born to lean mothers. Weight gain during pregnancy was less among obese women (7.2 vs 13.6 kg). Term infants born to obese women were heavier (3.9 bs 3.2 kg), had greater triceps and midscapular skinfold thicknesses, and had heavier placentae (687 vs 572 gm). Length and head circumference were not affected. After birth, fasting blood glucose concentrations declined in obese infants to hypoglycemic levels at 60 minutes of the study (26.3 vs 63.1 mg/dl). Insulin levels were not statistically different between the two groups. The postnatal rise of free fatty acids and glycerol was augmented in infants of obese mothers, whereas the decline of plasma beta-hydroxybutyrate values was augmented in infants of obese mothers, whereas the decline of plasma beta-hydroxybutyrate values was attenuated. Cord vein and artery plasma triglyceride concentrations were elevated in infants of obese mothers, and the usual rise of triglycerides with fasting after birth was further augmented in these infants. These data suggest that despite less than optimal maternal weight gain, sufficient or even surplus fuels are provided to the fetus of the obese mother and results in augmented fetal growth. Furthermore, the occurrence of fasting hypoglycemia necessitates careful monitoring of postnatal blood glucose values.
Assuntos
Jejum , Feto/metabolismo , Obesidade/metabolismo , Complicações na Gravidez/metabolismo , Ácido 3-Hidroxibutírico , Peso ao Nascer , Glicemia/análise , Peso Corporal , Ácidos Graxos não Esterificados/sangue , Feminino , Sangue Fetal/análise , Retardo do Crescimento Fetal/metabolismo , Idade Gestacional , Glicerol/sangue , Crescimento , Humanos , Hidroxibutiratos/sangue , Hipoglicemia/metabolismo , Recém-Nascido , Insulina/sangue , Masculino , Gravidez , Gravidez em Diabéticas/metabolismo , Triglicerídeos/sangueRESUMO
The effect of carnitine administration on lipid metabolism and carnitine and acylcarnitine plasma values of newborn infants, given total parenteral nutrition for the first 7 days of life, was studied during a 4-hour infusion of Intralipid. An increase in plasma concentrations of total carnitine, free carnitine, and short-chain and long-chain acylcarnitine was found, but no significant change in triglycerides, free fatty acids, glycerol, or beta-hydroxybutyrate plasma values was noted, as compared with values obtained without carnitine administration. Moreover, the low free carnitine and short-chain and long-chain acylcarnitine plasma levels found in newborn infants after 7 days of total parenteral nutrition did not seem to impair the utilization of infused lipids. The results support the concept that the relation between the carnitine pool and lipid metabolism can be influenced by intravenous glucose infusion. Low carnitine plasma concentrations do not necessarily signify a depletion of body carnitine, and sufficient tissue carnitine concentrations can probably maintain good lipid utilization for an extended period.
Assuntos
Carnitina/administração & dosagem , Emulsões Gordurosas Intravenosas/administração & dosagem , Metabolismo dos Lipídeos , Nutrição Parenteral Total , Nutrição Parenteral , Ácido 3-Hidroxibutírico , Carnitina/sangue , Ácidos Graxos não Esterificados/sangue , Humanos , Hidroxibutiratos/sangue , Recém-Nascido , Doenças do Recém-Nascido/metabolismo , Doenças do Recém-Nascido/terapia , Fatores de Tempo , Triglicerídeos/sangueRESUMO
To investigate whether L-carnitine supplementation may correct nutritional carnitine deficiency and associated metabolic disturbances in premature infants receiving total parenteral nutrition, an intravenous fat tolerance test (1 gm/kg Intralipid over four hours) was performed in 29 premature infants 6 to 10 days of age (15 receiving carnitine supplement 10 mg/kg . day L-carnitine IV, and 14 receiving no supplement). Total carnitine plasma values were normal or slightly elevated in supplemented but decreased in nonsupplemented infants. In both groups, fat infusion resulted in an increase in plasma concentrations of triglycerides, free fatty acids, D-beta-hydroxybutyrate, and short-chain and long-chain acylcarnitine, but total carnitine values did not change. After fat infusion, the free fatty acids/D-beta-hydroxybutyrate ratios were lower and the increase of acylcarnitine greater in supplemented infants of 29 to 33 weeks' gestation than in nonsupplemented infants of the same gestational age. This study provides evidence that premature infants of less than 34 weeks' gestation requiring total parenteral nutrition develop nutritional carnitine deficiency with impaired fatty acid oxidation and ketogenesis. Carnitine supplementation improves this metabolic disturbance.
Assuntos
Carnitina/administração & dosagem , Carnitina/deficiência , Doenças do Prematuro/tratamento farmacológico , Doenças Metabólicas/tratamento farmacológico , Carnitina/uso terapêutico , Gorduras na Dieta/farmacologia , Ácidos Graxos não Esterificados/sangue , Humanos , Hidroxibutiratos/sangue , Recém-Nascido , Doenças do Prematuro/sangue , Metabolismo dos Lipídeos , Nutrição Parenteral , Triglicerídeos/sangueRESUMO
To investigate the relationships among carnitine intake, carnitine blood concentrations, and the ability to utilize exogenous fat, total carnitine, free carnitine, acylcarnitine, beta-hydroxybutyrate, free fatty acid and triglyceride plasma concentrations were measured in 26 parenterally alimented appropriate-for-gestational-age premature infants before and at the end of a four-hour infusion of Intralipid, 1 gm/kg body weight. There was an increase in plasma levels of AC, BOB, FFA, and TG, but a decrease of FC, TC was unaffected by the infusion, but strongly correlated with calculated carnitine intake. At the end of the fat infusion, AC and BOB were positively correlated, and FFA negatively correlated with TC. The results demonstrate the proportion of AC to FC to be an additional indicator of fatty acid utilization and suggest that decreased carnitine intake in premature infants may impair fatty acid oxidation and ketogenesis.
Assuntos
Carnitina/sangue , Gorduras/metabolismo , Doenças do Prematuro/metabolismo , Infusões Parenterais/métodos , Ácido 3-Hidroxibutírico , Peso Corporal , Carnitina/administração & dosagem , Carnitina/metabolismo , Ensaios Clínicos como Assunto , Gorduras/administração & dosagem , Ácidos Graxos/sangue , Humanos , Hidroxibutiratos/sangue , Recém-Nascido , Doenças do Prematuro/terapia , Triglicerídeos/sangueRESUMO
The possible role of epinephrine deficiency in abnormal utilization of energy sources during fasting was investigated in three pairs of discordant identical twins with recurrent fasting hypoglycemia. The hypoglycemic twins, ages 2, 8 and 9 years, each had beens smaller at birth. Defective epinephrine responsiveness to hypoglycemia was established by administration of 2-deoxyglucose, 50 mg/kg, i.v. In the control twins, this resulted in a rapid increase of plasma glucose (+39 mg/100 ml), free fatty acids (+0.3 mM), and urinary epinephrine (+224 ng/mg creatine). These changes did not occur in the affected twins. Fasting metabolism in the epinephrine-deficient twins was compared to the unaffected twins as controls. Oxidation of carbohydrate and fat were estimated from hourly measurements of oxygen consumption and carbon dioxide production, and utilization of protein was determined from nitrogen excretion. PLasma glucose decreased more rapidly in the affected twins during the 8 hours prior to appearance of symptoms. During this period, carbohydrate was oxidized more rapidly than in the controls (average: 3.1 versus 1.7 mg/kg/min). Plasma á-hydroxybutyrate and free fatty acids was frequently less in relation to glucose. Symptoms occurred when the sum of both glucose and á-hydroxybutyrate was lower than in the controls. Urinary epinephrine excretion increased from an average baseline of 18 to a maximum of 134 ng/mg creatinine in the control twins. The average maximum urinary epinephrine reached in the deficient twins was only 51 ng/mg creatinine, in spite of lower glucose. Plasma insulin decreased in relation to glucose below 40 mg/100 ml in the control twins (r = 0.65), but this did not occur in the deficient twins (r = -0.38). Cortisol and growth hormone responses were similar in the two groups. THerefore, the consequences of inability to increase epinephrine when availability of glucose became acutely limiting were inappropriate persistent oxidation of carbohydrate, decreased circulating alternate substrates from fat, and lack of suppression of insulin. (AU)
Assuntos
Pré-Escolar , Feminino , Humanos , Masculino , Doenças em Gêmeos , Metabolismo Energético , Epinefrina/deficiência , Jejum , Gêmeos , Gêmeos Monozigóticos , Hipoglicemia/metabolismo , Peso ao Nascer , Glicemia/metabolismo , Carboidratos/metabolismo , Desoxiglucose/diagnóstico , Epinefrina/urina , Ácidos Graxos não Esterificados/sangue , Hidroxibutiratos/sangue , OxirreduçãoRESUMO
Fasting values of branched-chain amino acids (valine, leucine, and isoleucine) were measured by column chromatography in the sera of 27 normal infants and children, 15 days to 9 years of age, 14 children with documented ketotic hypoglycemia one to 7 years of age, and in 14 sera from six infants, 15 days to 2 years of age, with documented hyperinsulinism. In normal children and those with ketotic hypoglycemia, each individual branched-chain amino acid and their sum were significantly negatively correlated with blood sugar values ranging between 11 and 92 mg/dl (P < 0.001). In infants with hyperinsulinism, branched-chain amino acid concentrations were significantly lower (P < 0.001) without correlation with blood sugar values ranging between 13 and 51 mg/dl, and plasma insulin concentrations (9 to 85 microU/ML). In all the children the sum of branched-chain amino acids was positively correlated with blood beta OH butyrate concentrations measured at the same time (r = 0.75, P < 0.001). The association of low blood sugar and low branched-chain amino acid concentrations during fasting seems characteristic of hyperinsulinism, and the measurement of branched-chain amino acids in these infants offers a physiologic indicator of the diagnosis of hyperinsulinism.
Assuntos
Aminoácidos de Cadeia Ramificada/sangue , Hiperinsulinismo/sangue , Hipoglicemia/sangue , Doenças do Recém-Nascido/sangue , Glicemia/análise , Pré-Escolar , Jejum , Humanos , Hidroxibutiratos/sangue , Lactente , Recém-Nascido , Insulina/sangueAssuntos
Glucose/metabolismo , Cardiopatias Congênitas/metabolismo , Homeostase , Alanina , Glicemia/metabolismo , Pré-Escolar , Jejum , Glucagon/sangue , Cardiopatias Congênitas/fisiopatologia , Humanos , Hidrocortisona/sangue , Hidroxibutiratos/sangue , Hipoglicemia/fisiopatologia , Lactente , Corpos Cetônicos/sangue , Cinética , Lactatos/sangue , Taxa de Depuração Metabólica , Piruvatos/sangueRESUMO
A 3-yr-old boy was investigated for numerous episodes of fatigue, irritability, pallor, and sweating, which began at 11 mo of age, when he had an episode of symptomatic hypoglycemia with ketonuria. He had euphoria, mental confusion, drowsiness, nausea, and vomiting 1-5 hr after oral administration of glycerol in doses of 0.5-1.0gm/kg. Orally administered MCT (1 gm/kg) had similar effects. On one occasion, oral glycerol also provoked hypoglycemia, as had a 16 1/2 hr fast. Intravenously administered glycerol (0.09 gm/kg) induced an immediate loss of consciousness from which he recovered spontaneously after 30 min; there were no changes in blood glucose values. Intravenously administered fructose (0.25 gm/kg) was tolerated normally. Leukocytes showed normal activities for FDPase, glycerol kinase, and glycerol phosphate dehydrogenase. The restriction of dietary intake of fat has been associated with a marked improvement in physical and mental activities. These observations suggest a unique, yet undifined intolerance to glycerol, which suggest caution in the diagnostic use of glycerol in the investigation of hypoglycemia as well as in the therapy of increased intracranial or intraocular pressure.