Asunto(s)
Ácido Aminolevulínico/sangre , Ácidos Levulínicos/sangre , Errores Innatos del Metabolismo de los Aminoácidos/diagnóstico , Errores Innatos del Metabolismo de los Aminoácidos/genética , Ácido Aminolevulínico/líquido cefalorraquídeo , Cromatografía de Gases , Cromatografía Líquida de Alta Presión , Colorimetría , Humanos , Intoxicación por Plomo/diagnóstico , Hepatopatías/diagnóstico , Porfirias/diagnóstico , Valores de Referencia , Tirosina/sangreRESUMEN
A new method for sensitive measurement of delta-aminolaevulinic acid (ALA) in biological material is described. ALA is derivatized with dansyl chloride, separated by HPLC and estimated using a fluorescence detector. The pretreatment of biological samples includes desamination of L-alpha-aminoacids with L-aminoacid-oxidase before dansylation. The sensitivity of the method is slightly below 1 pmol/injection for standards and the lower limit of quantification is 0.1 mumol/l for plasma and 10 nmol/l for cerebrospinal fluid. Reference values in plasma are 3.53 +/- 1.75 (SD) (n = 43) mumol/l and in packed erythrocytes they ranged from 6 to 26 mumol/l (mean: 14.0 +/- 5.5 mumol/l). In cerebrospinal fluid of non-porphyric individuals less than 2 nmol/l were recovered.
Asunto(s)
Ácido Aminolevulínico/sangre , Ácidos Levulínicos/sangre , Ácido Aminolevulínico/líquido cefalorraquídeo , Cromatografía Líquida de Alta Presión/métodos , Compuestos de Dansilo , Eritrocitos/análisis , Humanos , Intoxicación por Plomo/sangre , Valores de Referencia , Espectrometría de FluorescenciaRESUMEN
A new organic acid, 2-hydroxy-2-methyllevulinic acid, was identified in the urine of four diabetic patients with ketoacidosis using gas chromatography-mass spectrometry. The compound was also detected in two serum samples of the four patients. The compound became undetectable in the urine of the patients after insulin therapy and was not detected in urine and serum of healthy subjects or diabetic patients without ketosis. 2-Hydroxy-2-methyllevulinic acid was also detectable in the urine of a child with elevated blood lactate and pyruvate, and ketosis. This finding suggests that the occurrence of 2-hydroxy-2-methyllevulinic acid is not specific to "diabetic" ketosis but is correlated to ketosis itself.
Asunto(s)
Cetoacidosis Diabética/sangre , Ácidos Levulínicos/sangre , Cetoacidosis Diabética/orina , Cromatografía de Gases y Espectrometría de Masas/métodos , Humanos , Ácidos Levulínicos/orina , Compuestos de TrimetilsililoAsunto(s)
Ácido Aminolevulínico/sangre , Esclerosis Amiotrófica Lateral/tratamiento farmacológico , Ácidos Levulínicos/sangre , Atrofia Muscular/tratamiento farmacológico , Penicilamina/uso terapéutico , Adulto , Anciano , Esclerosis Amiotrófica Lateral/sangre , Esclerosis Amiotrófica Lateral/diagnóstico , Femenino , Humanos , Masculino , Persona de Mediana Edad , Atrofia Muscular/sangre , Atrofia Muscular/diagnóstico , Médula EspinalRESUMEN
Whole blood delta-aminolaevulinic acid (ALA) concentrations and erythrocyte ALA dehydratase activity have been measured in patients with iron deficiency anaemia, megaloblastic anaemia and secondary anaemia, and in normal subjects. ALA concentration was found to be significantly increased in all types of anaemia compared with normal. Erythrocyte ALA dehydratase activity was significantly increased in iron deficiency and megaloblastic anaemia but not in secondary anaemia.
Asunto(s)
Ácido Aminolevulínico/sangre , Anemia/sangre , Eritrocitos/enzimología , Ácidos Levulínicos/sangre , Porfobilinógeno Sintasa/sangre , Anemia/enzimología , Anemia Hipocrómica/sangre , Anemia Hipocrómica/enzimología , Anemia Megaloblástica/sangre , Anemia Megaloblástica/enzimología , Femenino , Humanos , Intoxicación por Plomo/sangre , Intoxicación por Plomo/enzimología , MasculinoAsunto(s)
Intoxicación por Plomo/diagnóstico , Neuronas Motoras , Enfermedades Profesionales/diagnóstico , Penicilamina/uso terapéutico , Adulto , Esclerosis Amiotrófica Lateral/diagnóstico , Nitrógeno de la Urea Sanguínea , Diagnóstico Diferencial , Electromiografía , Hemoglobinas/análisis , Humanos , Plomo/sangre , Plomo/orina , Intoxicación por Plomo/sangre , Intoxicación por Plomo/tratamiento farmacológico , Intoxicación por Plomo/orina , Ácidos Levulínicos/sangre , Ácidos Levulínicos/orina , Masculino , Persona de Mediana Edad , Conducción Nerviosa , Porfirinas/orina , Reflejo Anormal , Factores de TiempoRESUMEN
In an effort to define the toxicology and disposition of lead compounds that presently exist in paint (i.e., organic driers), a controlled dose feeding study was initiated early this year with the use of 28 infant baboons as experimental animals. The infant baboon, established as a metabolic model for a child ingesting lead, will be used to determine the adequacy of present as well as recently recommended limitations for lead in paint to assure protection from this potential source of lead exposure. To accomplish this goal, research has been designed to determine basic dose-response relationships in animals ingesting constant daily doses of a dried paint, a lead octoate drier, and lead acetate. Doses for these compounds have been chosen to cover a broad range of concentrations including that recommended by the American Academy of Pediatrics from the maximum daily permissible lead ingestion, and associated estimates of paint intake by children with pica. PARAMETERS OF METABOLIC RESPONSE FOR EACH LEAD COMPOUND, INCLUDE: general clinical surveillance, lead concentrations in blood, urine and feces, erythrocytic delta-aminolevulinic acid dehydratase and free erythrocytic porphyrin. The response of several of these measures of lead exposure as a function of time will be discussed for each compound at the several dose levels administered.