RESUMEN
Humpback whales (Megaptera novaeangliae) are a cosmopolitan species and perform long annual migrations between low-latitude breeding areas and high-latitude feeding areas. Their breeding populations appear to be spatially and genetically segregated due to long-term, maternally inherited fidelity to natal breeding areas. In the Southern Hemisphere, some humpback whale breeding populations mix in Southern Ocean waters in summer, but very little movement between Pacific and Atlantic waters has been identified to date, suggesting these waters constituted an oceanic boundary between genetically distinct populations. Here, we present new evidence of summer co-occurrence in the West Antarctic Peninsula feeding area of two recovering humpback whale breeding populations from the Atlantic (Brazil) and Pacific (Central and South America). As humpback whale populations recover, observations like this point to the need to revise our perceptions of boundaries between stocks, particularly on high latitude feeding grounds. We suggest that this "Southern Ocean Exchange" may become more frequent as populations recover from commercial whaling and climate change modifies environmental dynamics and humpback whale prey availability.
Asunto(s)
Yubarta/fisiología , Reproducción , Migración Animal , Animales , Cambio Climático , Conducta Alimentaria , Océanos y MaresRESUMEN
Eight newly developed polymorphic microsatellite markers are presented for Abies guatemalensis Rehder, an endangered tree endemic to Mexico and Central America. These microsatellite markers are useful for evaluating the population genetics of the species which is threatened by greenery poaching in natural populations. Novel conservation plans are needed to secure a more sustainable use strategy and to preserve the genetic diversity of the species.
RESUMEN
To evaluate whether milk production can be improved by increasing food intake, a randomized, double-blind, supplementation trial was completed among 102 lactating Guatemalan women. The subjects were undernourished, as indicated by their low values for calf circumference (CC) and the small size of their infants at birth. A high-energy (2.14 MJ/d, HES) and a low-energy (0.50 MJ/d, LES) supplement were distributed 6 d/wk from wk 5 to 25 of lactation. Data were evaluated using repeated-measures analysis of variance on the increments from initial values for each outcome variable with one-tailed tests of statistical significance. The maternal energy intake increased 1.18 MJ/d (P < 0.01) more among the HES than the LES women. Benefit from supplementation was more evident among the more undernourished (CC = median value, 29.5 cm) women. Among these 53 lower-CC women, infant milk and milk energy intakes were 10% higher (64 g/d and 14 MJ/d, respectively, at wk 25) in the HES than the LES group. After controlling for other determinants of infant milk and energy intakes in regression analyses, the significance of these differences increased to P < 0.04. However, there was no detectable effect on infant growth. Logistic regression analysis was used to show that HES women were significantly (P < 0.05) more likely than LES women to be exclusively breast-feeding their infants at wk 20, the time when the effect of supplementation was most evident. These findings establish that milk production and the duration of exclusive breast-feeding of undernourished women can be improved with the provision of supplemental food.
Asunto(s)
Lactancia Materna , Dieta , Suplementos Dietéticos , Ingestión de Energía , Lactancia/fisiología , Adulto , Análisis de Varianza , Método Doble Ciego , Ingestión de Alimentos , Femenino , Guatemala , Humanos , Recién Nacido , Modelos Logísticos , Masculino , Trastornos Nutricionales/dietoterapia , Trastornos Nutricionales/fisiopatología , Población RuralRESUMEN
To evaluate the effect of a nutritional supplement on change in women's weight during a reproductive cycle and on the difference in birth weight between one infant and the previous one, we analyzed data on 176 complete reproductive cycles from an experiment that was conducted in rural Guatemala. Women with an initial weight <50 kg were classified as marginally nourished or malnourished. Women whose intake of the supplement was in the top 2 tertiles were distinguished from those whose intake was in the lowest tertile. Linear regression modeling was used to estimate the effect of supplementation on these outcomes and to control for confounding factors. Malnourished women gained weight during the reproductive cycle, but their second (study) infant tended to weigh less at birth than their prior-born infant. Higher intakes of supplement were associated with a less negative difference in birth weight. Marginally nourished women lost weight during the reproductive cycle and their second (study) infant tended to weigh more at birth than their prior-born infant. Higher intakes of supplement were associated with a less negative weight [corrected] trend for the women themselves. Well-nourished women and their infants did not show any of these benefits from supplementation. These findings help explain past contradictory findings on maternal depletion as well as on the benefits of nutritional supplementation for mothers and their infants.
Asunto(s)
Proteínas en la Dieta/administración & dosificación , Suplementos Dietéticos , Lactancia , Desnutrición Proteico-Calórica/terapia , Reproducción/efectos de los fármacos , Adulto , Peso al Nacer/efectos de los fármacos , Proteínas en la Dieta/uso terapéutico , Femenino , Guatemala , Humanos , Recién Nacido , Modelos Lineales , Bienestar Materno , Estado Nutricional , Embarazo , Salud RuralRESUMEN
Researchers have normally considered weaning to be a non-reversible event. To determine the validity of this assumption, we interviewed 36 mothers of toddlers who were living in a poor shanty town of Lima, Peru. Data from 32 women were complete and used in this analysis. Mothers described their beliefs, practices, and decisions about breastfeeding, weaning, and relactation (the reintroduction of breastfeeding after weaning). We recorded attempted weaning events if the mother reported (1) purposefully not breastfeeding with the intention to wean, or (2) carrying out an action that was believed to cause the child to stop breastfeeding. Using a constant comparative approach, references to child-feeding decisions were coded, categorized, and analyzed. All mothers breastfed for at least 12 months; the median duration of breastfeeding was 25 months. There were several different patterns of child-feeding. Thirteen women never attempted to wean their children or had weaned on the first attempt. The majority (n = 19) of women, however, attempted to wean their children - some as early as 3 months of age but relactated between less than 1 day and 3 months later. Factors that influenced feeding decisions were primarily related to maternal and child health, and maternal time commitments. Children were weaned when there was a perceived problem of maternal health or time commitments and child health was not at risk of deterioration. Mothers postponed weaning because of poor child health. The primary reason for relactation was a child's negative reaction to weaning (e.g., incessant crying or refusal to eat). Personalities of the mother and child were important determinants of feeding decisions. These results demonstrate that maternal and child factors jointly influence child-feeding decisions and that these decisions are easily reversed. As relactation is culturally acceptable, health practitioners should consider recommending relactation when children have been prematurely weaned and human milk would improve their nutritional and health status.
Asunto(s)
Lactancia Materna , Toma de Decisiones , Madres/psicología , Destete , Adulto , Protección a la Infancia , Preescolar , Recolección de Datos , Femenino , Humanos , Lactante , Bienestar Materno , Perú , Áreas de PobrezaRESUMEN
Although breast-feeding is widely accepted as important for infant health, its benefits during the second year of life have been questioned. We analyzed data from 107 breast-fed and weaned Peruvian children living in a periurban community to determine whether breast milk contributed to improved linear growth between 12 and 15 mo of age. Breast-feeding frequency was self-reported; intakes of complementary foods and animal products were estimated from a food-frequency survey. Multivariate-linear-regression analysis was used to predict the length of the children at 15 mo of age. Determinants of length included length and weight-for-length at 12 mo of age (US National Center for Health Statistics standards), interval between 12- and 15-mo measurements, breast-feeding frequency, incidence of diarrhea, and intakes of complementary and animal-product foods. Complementary foods, animal-product foods, and breast milk all promoted toddlers' linear growth. In subjects with low intakes of animal-product foods, breast-feeding was positively associated (P < 0.05) with linear growth. There was a 0.5-cm/3 mo difference in linear growth between weaned toddlers and children who consumed the average number of feedings of breast milk. Linear growth was also positively associated with intake of animal-product foods in children with low intakes of complementary foods. The negative association between diarrhea and linear growth did not occur in subjects with high complementary-food intakes. When the family's diet is low in quality, breast milk is an especially important source of energy, protein, and accompanying micronutrients in young children. Thus, continued breast-feeding after 1 y of age, in conjunction with feeding of complementary foods, should be encouraged in toddlers living in poor circumstances.
PIP: The contribution of prolonged breast feeding to linear growth at 12-15 months of age was investigated in 107 breast-fed and weaned toddlers from a low-income neighborhood in Lima, Peru. The median duration of breast feeding in this sample was 17.1 months; by 15 months, 46 children had been weaned. The prevalence of stunting (length-for-age score -2 SD below the reference standard) was 17.8% at 12 months and 24.3% at 15 months; no child was wasted. Complementary foods, animal product foods, and breast milk all promoted toddlers' linear growth. In children with low intakes of animal product foods, breast feeding was positively associated with linear growth at 15 months (p 0.05). There was a 0.5 cm/3 months difference in linear growth between weaned toddlers and those who consumed the average number (6.3/day) of breast feeds. Linear growth was further positively associated with intake of animal product foods in children with low intakes of complementary foods. The negative association between diarrhea and linear growth did not occur in children with high intakes of complementary foods. When the household diet is of poor quality, breast feeding is an especially important source of energy, protein, and micronutrients in toddlers and should be continued beyond 12 months of age in conjunction with the provision of complementary foods. Breast milk not only added to the total diet of these young children, but also potentiated the beneficial effects of complementary foods consumed by increasing their growth promotion capacity.
Asunto(s)
Lactancia Materna , Crecimiento , Alimentos Infantiles , Fenómenos Fisiológicos Nutricionales del Lactante , Carne , Animales , Bovinos , Diarrea Infantil/epidemiología , Encuestas sobre Dietas , Humanos , Incidencia , Lactante , Perú , Vigilancia de la Población/métodos , Pobreza , Análisis de Regresión , Población UrbanaRESUMEN
BACKGROUND: Child feeding recommendations include breastfeeding beyond 12 months, however, some researchers have reported increased rates of malnutrition in breastfed toddlers. A negative association between growth and breast-feeding may reflect reverse causality; that is, the outcome (growth) is a determinant of the predictor (breastfeeding), and not vice versa. We examined this question with data from 134 Peruvian toddlers. METHODS: A linear regression analysis predicted length at the age of 15 months by length at 12 months, study interval, and 12-14.9-month breastfeeding, complementary food intake, and diarrhoeal incidence. This analysis defined the association between breastfeeding and linear growth. To elucidate the direction of the effect between breastfeeding and linear growth, logistic regression was used to predict the probability of weaning by the end of 14 months. Determinants included weight-for-age (W/A) at 12 months, complementary food intake at 9-11.9 months, and change in diarrhoeal incidence between 9 and 14.9 months. RESULTS: There was a significant (P < 0.01) interaction of breastfeeding, diarrhoeal incidence, and complementary food intake on length at 15 months. Increased breastfeeding was associated with a 1.0 cm decrease in length gain when dietary intake was low and diarrhoeal morbidity was high, implying that breastfeeding is harmful. The logistic analysis, however, demonstrated that the risk of weaning decreased only when W/A and dietary intake were low and diarrhoeal morbidity was high. CONCLUSIONS: The negative association between breastfeeding and linear growth reflected reverse causality. Increased breastfeeding did not lead to poor growth; children's poor growth and health led to increased breastfeeding. Children's health must be considered when evaluating the association of breastfeeding with anthropometric outcomes.
PIP: There has been a perplexing finding, in many developing countries, of increased rates of stunting and growth faltering in breast-fed toddlers receiving complementary foods relative to their non-breast-fed counterparts. Longitudinal data on 134 children 12-15 months of age from Lima, Peru, were used to investigate the hypothesis that the negative association between growth and breast feeding reflects reverse causality. The toddlers were participants in a broader persistent diarrhea surveillance survey conducted during 1985-87. 72.9% of children were breast-fed beyond 12 months (median duration, 16.8 months). Anthropometric measurements revealed stunting in 19.4% of toddlers at 12 months and in 29.1% at 15 months. Linear growth between 12 and 15 months had a complex relationship with breast feeding, diarrhea, and dietary factors. Increased breast feeding was associated with a 1.0 cm decrease in length gain between 12 and 15 months when dietary intake was low and diarrheal morbidity was high. However, logistic analysis demonstrated that mothers whose children had low dietary intakes, low weight-for-age, and increased incidence of diarrhea were less likely to wean their infants at 12 and 14 months. This finding that mothers modified their children's feeding practices according to the child's health and growth status supports a reverse causality process in which poor growth is a determinant rather than a result of breast feeding.
Asunto(s)
Estatura , Lactancia Materna/efectos adversos , Países en Desarrollo , Trastornos del Crecimiento/epidemiología , Alimentos Infantiles , Destete , Antropometría , Peso Corporal , Diarrea/epidemiología , Diarrea/etiología , Femenino , Trastornos del Crecimiento/etiología , Humanos , Incidencia , Lactante , Modelos Lineales , Masculino , Perú/epidemiología , Factores de TiempoRESUMEN
This study examined the relationship of several maternal variables to the duration of exclusive breast-feeding and the total duration of breast-feeding, along with attitudes, perceptions, and beliefs about breast-feeding among women living in poor neighborhoods of Managua, Nicaragua. The field work was carried out in December 1992 and January 1993 using qualitative and quantitative methods. A structured questionnaire was administered in interviews with 556 mothers of children under 12 months of age, and meetings of four directed discussion groups were held, in which a total of 20 women participated. At one week of age, almost all the children of the mothers who were surveyed had been breast-fed, but only 45% had been exclusively breast-fed. At 12 weeks old, 30% were already completely weaned. The discussion groups revealed the coexistence of positive opinions about both breast-feeding and bottle-feeding. However, exclusive breast-feeding was considered harmful for the mother, and breast milk was not thought to be sufficient nourishment for the child. Previous experience was strongly related to the duration of exclusive breast-feeding and to total breast-feeding duration. Attitudes, social support, and work situation were important factors influencing the total length of time women breast-fed a child. In general, the results obtained through the interviews and in the discussion groups were in agreement and showed that the elements needed to promote exclusive breast-feeding were social support, a favorable community environment, and policies that dealt with problems faced by working mothers. The complementary research methods were useful for obtaining information about the relative importance of different factors that determine the duration of breast-feeding and for understanding that practice in greater depth from the mother's point of view.
Asunto(s)
Lactancia Materna , Pobreza , Población Urbana , Adolescente , Adulto , Lactancia Materna/psicología , Lactancia Materna/estadística & datos numéricos , Femenino , Conocimientos, Actitudes y Práctica en Salud , Humanos , Lactante , Recién Nacido , Entrevistas como Asunto/métodos , Persona de Mediana Edad , Nicaragua , Pobreza/psicología , Pobreza/estadística & datos numéricos , Factores de Riesgo , Muestreo , Encuestas y Cuestionarios , Factores de Tiempo , Población Urbana/estadística & datos numéricosRESUMEN
The risk approach has been promoted to improve screening for nutrition interventions on the premise that indicators of risk also predict greater response to interventions. This study tested whether the determinants of the risk of poor growth (eg, low length-for-age) at 36 mo of age were the same as the determinants of differential benefit from food supplementation. The sample included 460 Guatemalan children who were exposed to either a high-energy, high-protein drink (atole) or a low-energy, no-protein drink (fresco) during their first 36 mo of life [INCAP (Institute of Nutrition of Central America and Panama) supplementation trial]. Low maternal stature, poor socioeconomic status, inadequate home diet, high diarrhea rates, and low anthropometry scores at 3 or 6 mo were all determinants of the risk of poor growth. Only indicators of child's thinness at 3 or 6 mo of age (low weight-for-age, weight-for-length, or midupper arm circumference) were determinants of differential benefit from supplementation. Thus, the development of screening indicators should be based on analyses of the predictors of differential benefit, not on conventional risk-factor analysis.
Asunto(s)
Fenómenos Fisiológicos Nutricionales Infantiles , Dieta/normas , Proteínas en la Dieta/administración & dosificación , Tamizaje Masivo/economía , Antropometría , Peso Corporal/fisiología , Preescolar , Análisis Costo-Beneficio , Diarrea/epidemiología , Diarrea/fisiopatología , Diarrea/prevención & control , Femenino , Alimentos Fortificados/economía , Humanos , Lactante , Recién Nacido , Estudios Longitudinales , Masculino , Bienestar Materno , Factores de Riesgo , Factores SocioeconómicosRESUMEN
To investigate the extent to which better maternal nutrition leads to reduction in length of postpartum amenorrhea, multivariate-logistic and linear-regression analyses were applied to data on 339 mother-infant pairs from the longitudinal Guatemalan Four Village Study, 1969-1977. Maternal triceps skinfold thickness was negatively associated with length of amenorrhea when infant supplementation (a proxy for reduced suckling) was accounted for. However, its effect was small: amenorrhea was only 0.5 mo shorter among women at the 75th percentile than among those at the 25th, equivalent to less than even one additional child during the women's reproductive years. Maternal supplementation was not associated with length of amenorrhea when infant supplementation was controlled. This is in contrast to previous studies in which breast-feeding or infant supplementation was not controlled. These results suggest that infant, not maternal, supplementation influences length of postpartum amenorrhea, and that maternal nutritional status has minimal influence.
Asunto(s)
Amenorrea , Estado Nutricional/fisiología , Periodo Posparto/fisiología , Amenorrea/dietoterapia , Lactancia Materna , Ingestión de Energía , Femenino , Guatemala , Humanos , Lactante , Recién Nacido , Embarazo , Análisis de RegresiónRESUMEN
Information on a cohort of 5539 singleton births delivered at term in two hospitals in Rosario, Argentina, was used to examine differences in early postnatal morbidity between small for gestational age (SGA) infants classified by their ponderal index (PI). SGA infants with low PI (SGA-LPI) were 4.35 (90% CI: 1.50, 12.61) times more likely to have asphyxia, 13.75 (2.48, 76.31) times more likely to have hypoglycaemia and 2.32 (1.03, 5.26) times more likely to have respiratory distress (RD) than SGA infants with adequate PI (SGA-API). The increased risks of asphyxia and hypoglycaemia observed for SGA-LPI infants diminished, but remained statistically significant after controlling for the infant's gender, birthweight, gestational age and hospital of birth. There was no difference in risk of hyperbilirubinaemia between SGA-API and SGA-LPI infants. With the exception of risk of hyperbilirubinaemia, SGA-API infants carried the same risks of morbidity as non-SGA infants. The results of the study show that SGA infants do not constitute a homogeneous group with respect to their prospects for early postnatal health and survival. Furthermore, the pattern of differences in morbidity risk between SGA-API and SGA-LPI infants observed in this study is consistent with the timing hypothesis for the aetiology of variation in PI among SGA infants.
Asunto(s)
Desarrollo Embrionario y Fetal/fisiología , Enfermedades del Recién Nacido/etiología , Recién Nacido Pequeño para la Edad Gestacional/fisiología , Argentina , Asfixia Neonatal/etiología , Estatura/fisiología , Peso Corporal/fisiología , Estudios de Cohortes , Femenino , Humanos , Hiperbilirrubinemia/etiología , Hipoglucemia/etiología , Recién Nacido , Enfermedades del Recién Nacido/fisiopatología , Masculino , Síndrome de Dificultad Respiratoria del Recién Nacido/etiología , Factores de RiesgoRESUMEN
Research has shown that the positive effect of nutritional supplementation on child growth in malnourished populations is small relative to the large negative effect of diarrheal disease. To test the hypothesis that the effects of supplementation and diarrhea are synergistic in that supplementation modifies the negative effect of diarrhea on linear growth, length and diarrheal morbidity were compared at 36 mo of age for two cohorts of Colombian children: supplemented from birth and unsupplemented. Among unsupplemented children diarrhea was negatively associated with length. Among supplemented children diarrhea had no effect on length and differed from that of unsupplemented children. Thus, supplementation completely offset the negative effect of diarrheal disease on length. Targeting supplementation programs to the critical period of high diarrheal prevalence among infants and young children should increase the effectiveness of such programs in preventing growth retardation associated with diarrhea.
PIP: To test the hypothesis that supplementation modifies the negative effect of diarrhea on linear growth, body length and diarrheal morbidity were compared at 36 months of age for 2 cohorts of Columbian children: those receiving supplements from birth and those not receiving supplements. The sample was a subset from a longitudinal study that took place in Bogota, Columbia, between 1973 and 1980 and consisted of 456 families randomly assigned to 6 experimental groups. There were 148 children in the unsupplemented group. The 140 children from the supplemented group received supplements from the 6th month of pregnancy until they were 36 months old. The supplementary feeding included 30 g of protein daily, and 7.5 mg or 15 mg of ferrous sulphate daily as well as vitamin A every 6 months. Supplemented children had a mean 16 episodes of diarrhea, compared with a mean of 18 episodes of the unsupplemented cohort, and they spent a total of 73 days ill, compared with 83 days ill for unsupplemented children. Linear regression analysis showed that the slopes for unsupplemented children were significantly different from 0 (p 0.001). Each day with diarrhea was associated with a reduction of about 0.03 cm in attained length at age 36 months. In contrast, for supplemented children diarrhea had no effect on attained length at age 36 months. 2-way analysis of variance showed that the difference between supplemented and unsupplemented children in attained length in the lowest quartile of diarrhea was small, but the difference in the highest quartile was almost 5 cm. Cumulative growth patterns of children in the high quartile of diarrheal disease revealed that the difference between unsupplemented children was a median of 13 cm, thus supplementation made up nearly 40% of the deficit, compared with the reference standard (5 cm/13 cm). Targeting supplementation programs to the critical period of high diarrheal prevalence among infants and young children should help prevent growth retardation associated with diarrhea.
Asunto(s)
Fenómenos Fisiológicos Nutricionales Infantiles , Diarrea Infantil/complicaciones , Alimentos Fortificados , Trastornos del Crecimiento/etiología , Trastornos Nutricionales/complicaciones , Preescolar , Estudios de Cohortes , Colombia , Femenino , Trastornos del Crecimiento/prevención & control , Humanos , Lactante , Recién Nacido , Masculino , Programas Nacionales de Salud , Necesidades Nutricionales , Estadística como AsuntoRESUMEN
Patterns of stress-responsiveness were studied in 19 adolescent male rhesus monkeys (Macaca mulatta) who are part of the free-ranging colony on Cayo Santiago, PR. Morphometric and blood samples were obtained as each male was captured and after holding overnight. Subject heart rate was recorded on Day 2, using surface EKG telemetry. Males showed marked individual differences in cardiac and endocrine profiles which were generally unrelated to their age, size or maternal rank. Heart rate patterns were correlated with several endocrine measures: males with low and variable heart rates showed lower cortisol, higher-prolactin and higher growth hormone levels on Day 2 relative to males with higher and less variable heart rates, and their testosterone levels increased rather than decreased. Males with low and variable heart rates appear to have an endocrine response profile that is less adversely affected by acute stress, and which may potentially give them a competitive advantage in social interactions.
Asunto(s)
Nivel de Alerta/fisiología , Frecuencia Cardíaca , Hormonas/sangre , Macaca mulatta/fisiología , Macaca/fisiología , Academias e Institutos , Animales , Electrocardiografía/instrumentación , Hormona del Crecimiento/sangre , Hidrocortisona/sangre , Masculino , Prolactina/sangre , Puerto Rico , Maduración Sexual , Telemetría/instrumentación , Testosterona/sangreRESUMEN
113 rhesus monkeys, representing 4 age classes, 3 matrilines, and immigrant adult males in a 161-member Cayo Santiago-derived troop living in a 2-acre enclosure, were sampled for levels of plasma ACTH and cortisol during a period of capture and brief cage confinement for routine veterinary examination. ACTH levels showed significant decreases over initially high values following capture in all subjects except infants, whereas cortisol levels remained elevated throughout the sampling period. Members of the lowest-ranking matriline had significantly higher ACTH levels than members of the other matrilines and immigrant males. Infants and juveniles exhibited higher cortisol levels than adolescent and adult monkeys. The overall pattern of results was generally consistent with previous findings from laboratory studies, providing not only evidence of generality across conditions and subject populations but also the basis for more detailed subsequent analyses of the relationship between pituitary-adrenocortical responsiveness, behavioral response to challenge, and age-sex-dominance status in wild-born rhesus monkeys.
Asunto(s)
Nivel de Alerta/fisiología , Macaca mulatta/fisiología , Macaca/fisiología , Sistema Hipófiso-Suprarrenal/fisiología , Medio Social , Academias e Institutos , Hormona Adrenocorticotrópica/sangre , Factores de Edad , Animales , Femenino , Hidrocortisona/sangre , Masculino , Puerto RicoRESUMEN
The autosomal recessive inherited disorder glutaryl-CoA dehydrogenase deficiency (glutaric aciduria) runs a progressive course with severe choreoathetosis and dystonia, eventually leading to total helplessness and early death. Theree patients were observed during therapeutic trials with a protein-low diet, riboflavin and GABA analogue. Diet and riboflavin had a slight-to-moderate effect on the clinical symptoms; the excretion of glutaric acid and 2-amino-adipic acid decreased considerably during treatment. Regression of neurologic symptoms was observed during treatment with GABA analogue. It is concluded that the patients should be treated as early as possible with protein-low diet, riboflavin, and GABA analogue.
Asunto(s)
Errores Innatos del Metabolismo de los Aminoácidos/terapia , Aminobutiratos/uso terapéutico , Baclofeno/uso terapéutico , Glutaratos/orina , Oxidorreductasas/metabolismo , Riboflavina/uso terapéutico , Ácido 2-Aminoadípico/orina , Errores Innatos del Metabolismo de los Aminoácidos/dietoterapia , Errores Innatos del Metabolismo de los Aminoácidos/tratamiento farmacológico , Niño , Dietoterapia , Proteínas en la Dieta/uso terapéutico , Femenino , Humanos , Lisina/metabolismo , Masculino , Triptófano/metabolismoRESUMEN
In two siblings with dystonic cerebral palsy the urinary metabolic profiles of organic acids were dominated by glutaric acid, a metabolite not normally present in urine. The exretion of glutaric acid amounted to several grams per day. The urinary excretion of beta-OH-glutaric acid and glutaconic acid was also enhanced. Imparied metabolism of glutaryl-CoA by leukocytes indicates that the patients suffer from an inborn error of lysine, tryptophan, and hydroxylysine metabolism. A defective oxidation of glutaryl-CoA to crotonyl-CoA, probably due to a deficiency of glutaryl-CoA dehydrogenase, is consistent with these findings.