RESUMO
A 45-month-old girl with 5-oxoprolinuria (pyroglutamic aciduria), hemolysis, and marked glutathione depletion caused by deficiency of glutathione synthetase was followed before and during treatment with ascorbate or N-acetylcysteine. High doses of ascorbate (0.7 mmol/kg per day) or N-acetylcysteine (6 mmol/kg per day) were given for 1 to 2 weeks without any obvious deleterious side effects. Ascorbate markedly increased lymphocyte (4-fold) and plasma (8-fold) levels of glutathione. N-Acetylcysteine also increased lymphocyte (3.5-fold) and plasma (6-fold) levels of glutathione. After these treatments were discontinued, lymphocyte and plasma glutathione levels decreased rapidly to pretreatment levels. Ascorbate treatment was extended for 1 year, and lymphocyte (4-fold) and plasma (2- to 5-fold) glutathione levels remained elevated above baseline. In parallel, the hematocrit increased from 25.4% to 32.6%, and the reticulocyte count decreased from 11% to 4%. The results demonstrate that ascorbate and N-acetylcysteine can decrease erythrocyte turnover in patients with hereditary glutathione deficiency by increasing glutathione levels.
Assuntos
Acetilcisteína/uso terapêutico , Ácido Ascórbico/uso terapêutico , Glutationa Sintase/deficiência , Pré-Escolar , Feminino , Glutationa Sintase/análise , Glutationa Sintase/sangue , Humanos , Linfócitos/enzimologia , Erros Inatos do Metabolismo/sangue , Erros Inatos do Metabolismo/tratamento farmacológico , Vitamina E/uso terapêuticoAssuntos
Desenvolvimento Infantil , Recém-Nascido de Baixo Peso/crescimento & desenvolvimento , Recém-Nascido Prematuro/crescimento & desenvolvimento , Estatura , Peso Corporal , Criança , Feminino , Humanos , Recém-Nascido , Testes de Inteligência , Masculino , Exame Neurológico , Análise de RegressãoAssuntos
Desenvolvimento Infantil , Recém-Nascido de Baixo Peso , Recém-Nascido Prematuro , Estatura , Peso Corporal , Pré-Escolar , Feminino , Humanos , Recém-Nascido , MasculinoRESUMO
Six full-term severely asphyxiated newborn infants underwent evaluation with digital intravenous angiography. All infants were comatose and flaccid and had seizures, depressed brainstem function, and signs of intracranial hypertension. An initial brain CT scan revealed diffuse hypodensities and compressed ventricles. Follow-up noncontrast CT scans showed areas of increased attenuation. Digital intravenous angiography demonstrated venous sinus thrombosis in five infants, two of whom also had arterial thrombosis, and hypervascularity in the sixth infant. Our data document that occlusive vascular disease is a prominent feature of severe perinatal asphyxia.
Assuntos
Arteriopatias Oclusivas/etiologia , Asfixia Neonatal/complicações , Artérias Cerebrais/fisiopatologia , Arteriopatias Oclusivas/diagnóstico por imagem , Arteriopatias Oclusivas/fisiopatologia , Asfixia Neonatal/fisiopatologia , Angiografia Cerebral/métodos , Circulação Cerebrovascular , Humanos , Recém-Nascido , Tomografia Computadorizada por Raios XRESUMO
Previously published studies present conflicting conclusions about the growth of small, premature infants. To provide further data on these infants, a well-defined group of 86 premature infants appropriate for gestational age, weighing less than 1501 gm at birth, was observed until 12 months after their expected dates of delivery. Length, weight, and head circumference were measured at one, three, six, nine, and 12 months postterm. At 12 months postterm, infants were evaluated by neurologic examination and the Bayley Scales of Infant Development. Premature infants with mean Bayley scores greater than or equal to 85 were found to be significantly larger than infants with mean Bayley scores less than 85 by three months postterm. Birthweight, gestational age, duration of hospitalization in the neonatal intensive care unit, pneumonia, hypoglycemia, perinatal neurologic impairment, acidosis, and hypoxemia were found to be associated with reduced growth at one year. Conflicting results in previous studies may be a result of failure to separate infants with good neurobehavioral outcome from those with poor outcome.
Assuntos
Comportamento Infantil , Crescimento , Recém-Nascido de Baixo Peso/psicologia , Recém-Nascido Prematuro/psicologia , Inteligência , Estatura , Peso Corporal , Cefalometria , Feminino , Humanos , Lactente , Recém-Nascido , Testes de Inteligência , Masculino , Exame NeurológicoRESUMO
The effects of theophylline at a dose of 2 mg/kg/day (with resultant serum concentrations of 2.8 to 3.9 micrograms/ml) were observed in seven premature infants with severe idiopathic apnea. The frequency of apneic episodes decreased 62 +/- 9% (SEM) and the frequency of episodes of bradycardia decreased 73 +/- 9% following 24-48 hours of this therapy. No changes in ventilatory response to CO2 resting respiratory minute volume, arterial blood gas values, resting heart rate, or serum electrolyte concentrations were observed. Theophylline at this dose appears to be a safe and effective treatment for idiopathic apnea in premature infants. It works by a mechanism other than increasing CO2 sensitivity.
Assuntos
Apneia/tratamento farmacológico , Doenças do Recém-Nascido/tratamento farmacológico , Teofilina/uso terapêutico , Bradicardia/tratamento farmacológico , Dióxido de Carbono , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Respiração/efeitos dos fármacos , Teofilina/sangue , Teofilina/farmacologiaRESUMO
Two noninvasive methods of estimating arterial oxygenation were compared in a group of 48 infants ranging in birth weight from 870 to 4,000 gm, with diagnoses including apnea of prematurity, hyaline membrane disease, meconium aspiration, and congenital heart disease. Both transcutaneous oxygen measurements and ear oximetry gave reasonably accurate estimations of arterial oxygen levels within commonly used clinical ranges (PO2 50 to 70 mm Hg, arterial saturation 90 to 98%). Infants with shock demonstrated a wide range of values for transcutaneous oxygen levels, suggesting that this method has limited usefulness in this situation. Ear oximetry had limited ability to distinguish high, but safe, levels of arterial oxygen from excessively elevated levels. While neither method can be recommended for replacement of arterial oxygen sampling, both methods may be useful in a clinical setting if care is exercised in interpretation of the results and if the values obtained are checked against those from arterial blood.