Asunto(s)
Errores Innatos del Metabolismo de los Aminoácidos/complicaciones , Argininosuccinato Sintasa/deficiencia , Encefalopatías Metabólicas/terapia , Ligasas/deficiencia , Enfermedad de la Orina de Jarabe de Arce/complicaciones , Diálisis Renal , Aminoácidos de Cadena Ramificada/metabolismo , Amoníaco/metabolismo , Encefalopatías Metabólicas/etiología , Femenino , Glutamina/metabolismo , Humanos , Recién Nacido , Cetoácidos/metabolismo , MasculinoAsunto(s)
Enfermedad del Almacenamiento de Glucógeno Tipo I/complicaciones , Leucemia Mieloide Aguda/complicaciones , Hipertermia Maligna/complicaciones , Preescolar , Glucosa-6-Fosfato , Glucofosfatos/metabolismo , Enfermedad del Almacenamiento de Glucógeno Tipo I/patología , Granulocitos/patología , Humanos , Leucemia Mieloide Aguda/patología , MasculinoRESUMEN
The increased incidence of severe hypoglycemia reported in young children with diabetes is consistent with a defect in glucagon secretion or a generalized abnormality in islet hormone secretion. To assess pancreatic hormone and gastric inhibitory polypeptide secretion in children with early onset diabetes, 12 children with onset of diabetes prior to the age of 28 months were studied and the data compared to the hormone responses observed in 11 children with LOD, diagnosed after the age of 5 years. Plasma glucose, C-peptide, glucagon, pancreatic polypeptide, and gastric inhibitory peptide concentrations were measured during and following an arginine infusion (500 mg/kg over 60 minutes) and a mixed meal. During arginine infusion, plasma glucose and glucagon increased similarly in both groups and returned to basal concentrations following discontinuation of arginine infusion. In contrast, plasma C-peptide, hPP, and GIP concentrations did not change. Following the mixed meal plasma glucose, hPP, and GIP concentrations increased similarly in the two groups of children, but no change was observed in either plasma glucagon or C-peptide concentrations in either group. These data demonstrate that EOD and LOD are associated with insulin insufficiency alone and that abnormalities in secretion of other pancreatic islet hormone or GIP cannot be implicated in the high incidence of severe hypoglycemia observed in children with EOD.
Asunto(s)
Diabetes Mellitus Tipo 1/tratamiento farmacológico , Insulina/uso terapéutico , Islotes Pancreáticos/metabolismo , Arginina , Glucemia/análisis , Péptido C/sangre , Niño , Preescolar , Diabetes Mellitus/dietoterapia , Diabetes Mellitus/tratamiento farmacológico , Diabetes Mellitus Tipo 1/dietoterapia , Femenino , Polipéptido Inhibidor Gástrico/sangre , Glucagón/sangre , Glucagón/metabolismo , Humanos , Hipoglucemia/complicaciones , Lactante , Infusiones Parenterales , Masculino , Polipéptido Pancreático/sangre , Polipéptido Pancreático/metabolismo , Factores de TiempoAsunto(s)
Glucosa/metabolismo , Cardiopatías Congénitas/metabolismo , Homeostasis , Alanina , Glucemia/metabolismo , Preescolar , Ayuno , Glucagón/sangre , Cardiopatías Congénitas/fisiopatología , Humanos , Hidrocortisona/sangre , Hidroxibutiratos/sangre , Hipoglucemia/fisiopatología , Lactante , Cuerpos Cetónicos/sangre , Cinética , Lactatos/sangre , Tasa de Depuración Metabólica , Piruvatos/sangreRESUMEN
Thirty-six patients with Reye syndrome were admitted to St. Louis Children's Hospital during a ten-year period from January, 1966, through June, 1975; ten patients (28%) died. Seven (64%) of the ten deaths occurred in the 11 patients admitted between 1966 and 1971, whereas only three (12%) of 25 patients died thereafter. We attribute this reduction in case fatality rate to early diagnosis, recognition of milder cases, and intensive medical support including continuous intravenous infusion of hypertonic glucose, intermittent infusion of hypertonic mannitol, and early elective endotracheal intubation. Analyses of metabolic, hormonal, and blood gas data obtained serially during the acute illness in 16 of these patients provide a basis for a discussion of the pathophysiology of this disorder.
Asunto(s)
Encefalopatías/terapia , Síndrome de Reye/terapia , Desequilibrio Ácido-Base/diagnóstico , Adolescente , Glucemia/análisis , Calcio/sangre , Niño , Preescolar , Femenino , Solución Hipertónica de Glucosa , Glucosuria , Humanos , Hiperglucemia/diagnóstico , Lactante , Infusiones Parenterales , Insulina/sangre , Intubación Intratraqueal , Masculino , Manitol/uso terapéutico , Fósforo/análisis , Síndrome de Reye/diagnóstico , Síndrome de Reye/mortalidadRESUMEN
L-Dopa in a dose ranging from 125-500 mg and arginine monochloride in a dose of 0.5 gm/kg were given simultaneously to 56 children with short stature (height less than third percentile). Sixteen of these children were subsequently diagnosed as having growth hormone deficiency. The diagnosis of hyposomatotropism was based on clinical findings and on responses to the combination test and to arginine and L-dopa administered as separate tests. All of the remaining 40 children had a normal GH response of greater than 6 ng/ml to the combination test. However, in this group, nine children were identified who responded to the combination test but who failed to respond to arginine and L-dopa in individual tests. The data suggest that a positive response to arginine and L-dopa in combination in children, who do not respond to the usual provocative tests when administered individually, may fail to identify children with partial GH deficiency who would benefit from treatment. The integrated stimulated GH response in the 31 children in whom a normal GH response to all three tests occurred suggests that the effects of L-dopa and arginine are additive.