RESUMEN

Calcifying fibrous pseudotumour is a distinct pathological entity usually occurring in the soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum. This report describes the hitherto unreported occurrence of this tumour of the adrenal gland in a 10-year-old girl whose imaging findings closely resembled a neuroblastoma. This entity is a potential pitfall in diagnosing adrenal neuroblastoma.

Asunto(s)

Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Calcinosis/diagnóstico por imagen , Granuloma de Células Plasmáticas/diagnóstico por imagen , Neuroblastoma/diagnóstico por imagen , Neoplasias de las Glándulas Suprarrenales/cirugía , Calcinosis/cirugía , Niño , Diagnóstico Diferencial , Femenino , Secciones por Congelación , Granuloma de Células Plasmáticas/cirugía , Humanos , Neuroblastoma/cirugía , Tomografía Computarizada por Rayos X

RESUMEN

The risk of malignancies among persons with neurofibromatosis 1 (NF1) is higher than in the general population, but the excess risk has not been precisely estimated. The effects of gender and inheritance pattern on cancer risk are unclear. Therefore, we conducted a historical cohort study to determine cancer risk factors by contacting 138 Caucasian NF1 patients originally seen at Baylor College of Medicine (BCM) in Houston between 1978 and 1984. A total of 304 patients of all ethnic groups were evaluated at BCM during this period. We successfully located 173 patients, 138 of who were Caucasian. We computed standardized incidence ratios (SIRs) with the age-, gender-, and time period-specific rates from the Connecticut Tumor Registry for 2,094 person-years of observation (median follow-up = 16 years). Eleven incident tumors were reported. Females were at much higher risk of cancer than males (SIR = 5.6, 95% confidence interval (CI) 2.7-10.3 and SIR = 0.6; 95% CI, 0.0-3.0, respectively). We found no elevated cancer risk in unaffected first-degree relatives, regardless of whether the proband had cancer or not (SIR = 1.1 95% CI, 0.6-1.8 and SIR = 1.0, 95% CI, 0.6-1.5, respectively). Our results suggest that malignancy in the proband is not the result of a modifying gene that has a significant impact on general cancer risk.

Asunto(s)

Neoplasias/genética , Neurofibromatosis 1/genética , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Neoplasias/epidemiología , Neurofibromatosis 1/epidemiología , Glioma del Nervio Óptico/epidemiología , Glioma del Nervio Óptico/genética , Valores de Referencia , Factores de Riesgo , Factores Sexuales , Texas/epidemiología

RESUMEN

PURPOSE: To assess the long-term neuropsychologic effects experienced by children who have tumors in the cerebellum that are diagnosed and treated during infancy. PATIENTS AND METHODS: Twenty-seven children with posterior fossa tumors diagnosed at less than 36 months of age were assessed prospectively with a comprehensive set of age-appropriate tests. Group means and SDs are reported for assessments conducted at diagnosis (analysis 1) and at the most recent follow-up appointment (analysis 2). Cognitive developmental growth curves were derived from the prospective data (analysis 3) using mixed model regression analyses and controlling for age at diagnosis and socioeconomic status. RESULTS: In the first analysis, eight of 11 infants at diagnosis scored within normal limits on all neuropsychologic domains, except for motor skills, which were impaired. In the second analysis, mean scores at the most recent follow-up of 21 of 27 patients were mostly in the normal range; however, group comparisons between those who had (n = 7) and had not (n = 14) been treated with cranial radiation therapy (CRT) showed that patients in the irradiated (CRT) group scored significantly lower than those in the nonirradiated (No-CRT) group on verbal intelligence quotient (IQ) and in the motor domain. In the third analysis (growth curves of CRT and No-CRT groups), statistically significant differences in slope were found on verbal IQ, performance IQ, perceptual-motor skills, language, and attention/executive skills. Slopes on the fine-motor domain were similar; both groups declined at approximately the same rate. CONCLUSION: Neurocognitive development and outcome of children with cerebellar tumors diagnosed in infancy is very positive among those who were treated with surgery and chemotherapy. Declines in performance across time were minimal, and scores tended to remain within normal limits. By itself, a cerebellar tumor in infancy does not seem to have a significant impact on children. However, those who received CRT as part of their treatment are likely to have neurocognitive and psychosocial deficits that require remediational interventions.

Asunto(s)

Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/radioterapia , Trastornos del Conocimiento/etiología , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Encefálicas/patología , Desarrollo Infantil , Preescolar , Terapia Combinada , Femenino , Humanos , Lactante , Estudios Longitudinales , Masculino , Pruebas Neuropsicológicas

RESUMEN

Neurofibromatosis type 1 is a common autosomal dominant genetic disorder associated with numerous physical anomalies and an increased incidence of neuropsychological impairment. Tumors of the CNS occur in approximately 15% of children with neurofibromatosis, presenting additional risk for cognitive impairment. This study examines the impact of an additional diagnosis of brain tumor on the cognitive profile of children with neurofibromatosis. A comprehensive battery of neuropsychological tests was administered to 149 children with neurofibromatosis. Thirty-six of these children had a codiagnosis of brain tumor. A subset of 36 children with neurofibromatosis alone was matched with the group of children diagnosed with neurofibromatosis and brain tumor. Although mean scores of the neurofibromatosis plus brain tumor group were, in general, lower than those of the neurofibromatosis alone group, these differences were not statistically significant. Children in the neurofibromatosis plus brain tumor group who received cranial irradiation (n = 9) demonstrated weaker academic abilities than did children with brain tumor who had not received that treatment. These results suggest that neurofibromatosis is associated with impairments in cognitive functioning, but the severity of the problems is not significantly exacerbated by the codiagnosis of a brain tumor unless treatment includes cranial irradiation.

Asunto(s)

Neoplasias Encefálicas/psicología , Trastornos del Conocimiento/etiología , Irradiación Craneana/efectos adversos , Neurofibromatosis 1/psicología , Traumatismos por Radiación/psicología , Adolescente , Antineoplásicos/uso terapéutico , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/terapia , Estudios de Casos y Controles , Niño , Terapia Combinada , Femenino , Glioma/complicaciones , Glioma/genética , Glioma/psicología , Glioma/radioterapia , Glioma/terapia , Humanos , Inteligencia , Trastornos del Lenguaje/etiología , Discapacidades para el Aprendizaje/etiología , Masculino , Trastornos de la Memoria/etiología , Neurofibromatosis 1/complicaciones , Pruebas Neuropsicológicas , Quiasma Óptico/patología , Neoplasias del Nervio Óptico/genética , Trastornos Psicomotores/etiología , Traumatismos por Radiación/etiología

RESUMEN

BACKGROUND: Mass screening of infants for neuroblastoma began in Japan after studies suggested that survival rates could be improved by early detection. This study was initiated in 1991 to test the methodology and feasibility of screening for neuroblastoma within the U. S. health care system. METHODS: Infants ages 5-10 months (mean age, 9 months, 25 days) who were born in Texas were screened for neuroblastoma. An enzyme-linked immunoadsorbent assay (ELISA) for homovanillic acid (HVA) and vanillylmandelic acid (VMA) used to quantify the HVA and VMA was performed on urine extracted from specimens dried on filter paper. Infants were recruited to participate in the study by several methods, and the effectiveness of each method was determined by calculating compliance rates. RESULTS: Between February 1991 and June 1994 a total of 14,046 infants were recruited to participate in neuroblastoma screening. Neuroblastoma was detected in 2 children for an incidence rate of 1 in 7023. A total of 291,158 screening kits were distributed to the parents of these infants, resulting in an overall compliance rate of only 4.8%. Compliance rates varied by method of distribution of the test kits: Houston Women, Infants, and Children (WIC) clinic (53%), volunteers (31%), Rio Grande Valley WIC clinics (14.5%), the patient's private physician (9.9%), and by mail (4.7%). CONCLUSIONS: Early detection of neuroblastoma in infants ages 5-10 months was achieved using ELISA. Compliance rates were poor, but clinics with a preventive health focus, such as the WIC clinics, achieved higher compliance rates than did private physicians.

Asunto(s)

Tamizaje Masivo , Neuroblastoma/diagnóstico , Ensayo de Inmunoadsorción Enzimática/métodos , Estudios de Factibilidad , Femenino , Ácido Homovanílico/orina , Humanos , Lactante , Masculino , Neuroblastoma/epidemiología , Neuroblastoma/orina , Sensibilidad y Especificidad , Texas/epidemiología , Ácido Vanilmandélico/orina

RESUMEN

Infants and young children who have brain tumors have a poor rate of survival and high treatment associated morbidity. A trial of mechlorethamine, vincristine (oncovin), procarbazine, and prednisone (MOPP) was performed to test the hypothesis that replacing radiotherapy with chemotherapy would improve survival and decrease long term morbidity of infants who have brain tumors. Between 1976 and 1988, 17 consecutive children less than 36 months old when diagnosed with medulloblastoma or ependymoma were treated with MOPP chemotherapy as primary therapy following surgical excision or biopsy of the tumor. Radiotherapy was reserved for recurrent disease. Ten of 17 children have survived without evidence of disease: medulloblastoma eight of 12 with median survival time of 10.6 years (range, 6.2 to 15.2 yrs); and ependymoma, 2 of 5 (at 13.0 and 16.0 yrs). Four of the 10 children with medulloblastoma and ependymoma who relapsed are now disease free at 7.5, 11.7, 12.2 and 13.5 yrs post relapse after receiving salvage therapy with cisplatin (n = 1) or irradiation (n = 3). All relapses occurred within 26 months of diagnosis. Data on growth demonstrated height less than the 5th percentile in all children who received cranial irradiation compared to 25 to 95th percentile for nonirradiated children. Intellectual ability for the group who did not require radiation was within normal range (mean IQ 100.1) and stable across annual assessments. Those who required radiation had lower IOs which continued to decline over time (mean IQ 85 at mean age of 5.8 years, declining to 63 at 10 years). In young children with brain tumors, primary chemotherapy with MOPP, omitting radiotherapy, provides improved neurodevelopmental outcome and survival.

Asunto(s)

Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Encefálicas/tratamiento farmacológico , Neoplasias Cerebelosas/tratamiento farmacológico , Ependimoma/tratamiento farmacológico , Meduloblastoma/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/psicología , Neoplasias Encefálicas/cirugía , Neoplasias Cerebelosas/mortalidad , Neoplasias Cerebelosas/psicología , Neoplasias Cerebelosas/cirugía , Quimioterapia Adyuvante , Ependimoma/mortalidad , Ependimoma/psicología , Ependimoma/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Pruebas de Inteligencia , Masculino , Mecloretamina/administración & dosificación , Mecloretamina/efectos adversos , Meduloblastoma/mortalidad , Meduloblastoma/psicología , Meduloblastoma/cirugía , Prednisona/administración & dosificación , Prednisona/efectos adversos , Procarbazina/administración & dosificación , Procarbazina/efectos adversos , Resultado del Tratamiento , Vincristina/administración & dosificación , Vincristina/efectos adversos

RESUMEN

Neuropsychological studies of children who have brain tumors have yielded diverse results with respect to identifying factors that contribute to poor intellectual outcome. The purpose of this study was to evaluate the relationship between pre- and perioperative events, tumor-related factors, and the neuropsychological status of children diagnosed with astrocytoma. Events that could potentially be detrimental to neuropsychological outcome were quantified utilizing a new "neurological severity score." The Neurological Severity Score was developed as a research tool to test our hypothesis that ultimate intellectual outcome is a result of cumulative, interactive insults on the central nervous system. This study constitutes a first step in examining the predictive value of the Neurological Severity Score by evaluating its correlation with baseline neuropsychological status. Fifty-nine children who had astrocytoma (36 supratentorial and 23 infratentorial) received complete neurological and neuropsychological evaluations within 3 months of diagnosis. Each child's neurological history and examination results were scored by an independent observer using the Neurological Severity Score. Neuroimages obtained at diagnosis and at the time of neuropsychological testing were evaluated as well. For the group as a whole, memory, attention, and motor abilities were significantly below age-appropriate norms, whereas intelligence, language, and academic skills were preserved. Patterns of deficits were identified and related to tumor site. There were no significant differences in mean neuropsychological domain scores between groups based on gender, pre-versus post-operative status, ethnicity, tumor grade, or abnormalities on magnetic resonance imaging (MRI). The Neurological Severity Score was significantly inversely correlated with visual-spatial skills, memory, attention, performance IQ, and global IQ. In conclusion, among all the medical and neurological factors present at diagnosis, the neurological severity score had the highest correlation with neuropsychological scores. This instrument has promise as a research tool in investigations of the psychological effects of cancer and its treatment on children.

Asunto(s)

Astrocitoma/diagnóstico , Neoplasias Encefálicas/diagnóstico , Glioblastoma/diagnóstico , Trastornos Neurocognitivos/diagnóstico , Examen Neurológico/estadística & datos numéricos , Pruebas Neuropsicológicas/estadística & datos numéricos , Complicaciones Posoperatorias/diagnóstico , Logro , Adolescente , Astrocitoma/psicología , Astrocitoma/cirugía , Daño Encefálico Crónico/diagnóstico , Daño Encefálico Crónico/psicología , Neoplasias Encefálicas/psicología , Neoplasias Encefálicas/cirugía , Niño , Preescolar , Trastornos del Conocimiento/diagnóstico , Trastornos del Conocimiento/psicología , Femenino , Glioblastoma/psicología , Glioblastoma/cirugía , Humanos , Inteligencia/fisiología , Masculino , Trastornos Neurocognitivos/psicología , Complicaciones Posoperatorias/psicología , Psicometría , Resultado del Tratamiento

Asunto(s)

Neoplasias Encefálicas , Ganglios Simpáticos , Neuroblastoma , Neoplasias de la Médula Espinal , Adolescente , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Niño , Preescolar , Atención Dental para Enfermos Crónicos , Humanos , Estadificación de Neoplasias , Neuroblastoma/diagnóstico , Neuroblastoma/terapia , Grupo de Atención al Paciente , Pronóstico , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/terapia

RESUMEN

Neurofibromatosis type 1, a common autosomal dominant genetic disorder, is associated with numerous physical and medical anomalies as well as an increased incidence of learning disability. Tumors of the central nervous system have been estimated to occur in 15%, but their contribution to neuropsychological status is unknown. This study examines the relative contribution of neurofibromatosis and brain tumor to the cognitive profile of children with neurofibromatosis. A comprehensive battery of neuropsychological and behavioral tests was administered to a group of 65 children with neurofibromatosis type 1. Fourteen were then matched on demographic variables with two other groups of children who had either a brain tumor in addition to neurofibromatosis or a brain tumor alone. The two brain tumor groups were also matched on tumor type, location, and therapy. Mean scores of the neurofibromatosis-brain tumor group were generally the lowest of the three groups; those of the brain tumor group were highest, and performance of the neurofibromatosis group was generally between the other two groups. These results suggest that neurofibromatosis is, by itself, associated with significant cognitive morbidity, but that the severity of the problems is increased somewhat if a brain tumor is also present.

Asunto(s)

Neoplasias Encefálicas/genética , Discapacidades para el Aprendizaje/genética , Neurofibromatosis/genética , Pruebas Neuropsicológicas , Adolescente , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/psicología , Niño , Preescolar , Escolaridad , Femenino , Genes Dominantes , Humanos , Pruebas de Inteligencia , Discapacidades para el Aprendizaje/diagnóstico , Discapacidades para el Aprendizaje/psicología , Masculino , Neurofibromatosis/diagnóstico , Neurofibromatosis/psicología , Examen Neurológico , Valores de Referencia

RESUMEN

BACKGROUND: Abdominal pain in children receiving chemotherapy for cancer presents the clinician with unique problems due to the altered immunity of these patients or to the oncologic setting. The major clinical decisions regarding these patients are to determine if and when operative intervention is indicated. METHODS: A retrospective study was done to examine the clinical, radiographic, and laboratory findings that indicate the need for surgical intervention. Sixty-eight of 1090 children who underwent treatment for cancer from October 1982 to December 1990 developed abdominal complaints requiring them to be hospitalized. Nineteen of these patients underwent exploratory laparotomy (operative), and the other 49 were observed (nonoperative). RESULTS: No significant differences were observed in the phase of chemotherapy, treatment with vincristine or corticosteroids, or the hematologic indices between the operative and nonoperative groups. Eighteen of nineteen patients survived their surgeries. Seventeen (89%) of these laparotomies were positive based on the surgical pathology and the operative report. Peritoneal signs on physical examination (P < 0.001) or pneumatosis intestinalis on abdominal radiographs correlated with positive laparotomies (P = 0.001). CONCLUSIONS: Peritoneal signs on physical examination or pneumatosis intestinalis on abdominal X-rays were associated with and specific for the presence of acute surgical disease of the abdomen in immunocompromised pediatric oncology patients.

Asunto(s)

Abdomen Agudo/cirugía , Antineoplásicos/uso terapéutico , Neoplasias/tratamiento farmacológico , Abdomen Agudo/complicaciones , Abdomen Agudo/diagnóstico , Adolescente , Adulto , Apendicitis/complicaciones , Apendicitis/cirugía , Niño , Preescolar , Urgencias Médicas , Femenino , Humanos , Huésped Inmunocomprometido , Laparotomía , Masculino , Neoplasias/complicaciones , Neoplasias/inmunología , Examen Físico , Neumatosis Cistoide Intestinal/diagnóstico por imagen , Radiografía , Estudios Retrospectivos

RESUMEN

Two young children who presented with lower spinal cord dysfunction manifested by bilateral leg weakness and urinary retention were diagnosed with intraspinal soft-tissue sarcoma. Neither patient had a significant extradural mass. Both tumors had histochemical features of rhabdomyosarcoma. Temporary responses were noted after combination chemotherapy either with vincristine, actinomycin D, and cyclophosphamide or with ifosfamide/mesna and etoposide. However, both patients developed uncontrollable cerebrospinal fluid (CSF) dissemination of tumor and died within 6 months of diagnosis, despite intrathecal chemotherapy and irradiation for one and very high-dose intravenous methotrexate (33 g/m2) for the other. This rare tumor can respond to parenteral antisarcoma chemotherapy, but better strategies are needed to prevent CSF spread and ultimate demise. Early institution of intrathecal cytostatic agents may retard or prevent CSF dissemination and prolong survival.

Asunto(s)

Rabdomiosarcoma , Sarcoma , Neoplasias de la Médula Espinal , Preescolar , Femenino , Humanos , Lactante , Masculino , Rabdomiosarcoma/diagnóstico , Rabdomiosarcoma/patología , Rabdomiosarcoma/terapia , Sarcoma/diagnóstico , Sarcoma/patología , Sarcoma/terapia , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/terapia , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/patología , Neoplasias de la Médula Espinal/terapia

RESUMEN

Neuropsychological outcome of 28 patients with brain tumors diagnosed before the age of 36 months (mean, 19 months) was assessed using a comprehensive battery of tests. Elapsed time between diagnosis and testing averaged 6.2 years. Half the patients had received cranial radiation therapy and surgery, with and without chemotherapy, whereas the rest had received only surgery, with or without chemotherapy. Groups were comparable with respect to tumor diagnosis and location, age at diagnosis, race, and sex. Intellectual functioning was significantly lower in children whose treatment included cranial irradiation than in those treated without cranial irradiation, and this effect was more pronounced in nonverbal than in verbal intellectual abilities. Mean scores for the radiation group were lower than for the no-radiation group in all areas assessed and were significantly below age-based normative means in five of the eight cognitive areas: intellectual, memory, attention, motor, and visual-spatial skills. Mean scores for children in the no-radiation group were generally within the average range in all cognitive areas except visual-spatial skills, which were significantly below age-based normative means. Endocrine deficiencies and growth retardation were much more prevalent in patients treated with cranial irradiation. Because the immature brain is susceptible to treatment-related pathologic changes, infants are at greater risk than older children for significant, long-term neuropsychological, endocrine, and growth sequelae. In children treated without cranial irradiation, morbidity was minimized without an increased rate of mortality.

Asunto(s)

Daño Encefálico Crónico/diagnóstico , Neoplasias Encefálicas/congénito , Encéfalo/efectos de la radiación , Irradiación Craneana , Pruebas Neuropsicológicas , Traumatismos por Radiación/diagnóstico , Daño Encefálico Crónico/psicología , Neoplasias Encefálicas/psicología , Neoplasias Encefálicas/radioterapia , Niño , Desarrollo Infantil/efectos de la radiación , Preescolar , Terapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Traumatismos por Radiación/psicología

Asunto(s)

Fiebre por Garrapatas del Colorado/microbiología , Interferones/sangre , Infecciones por Reoviridae/microbiología , Adulto , Niño , Fiebre por Garrapatas del Colorado/sangre , Fiebre por Garrapatas del Colorado/complicaciones , Fiebre/etiología , Humanos , Interferones/biosíntesis , Células L/microbiología , Recuento de Leucocitos , Leucopenia/etiología , Pruebas de Neutralización , Virus de la Estomatitis Vesicular Indiana

RESUMEN

This report describes two cousins with Wilms' tumor and mucin detected in the sera with rapidly fatal courses. A review of the literature reveals five additional reported cases of Wilms' tumor where mucin was observed in the sera. The patients in this report are similar to those previously reported in that all had metastatic disease at diagnosis, and four of five died within one year of diagnosis. Both the extent of disease at diagnosis at the poor response to therapy in these two cases, and those from the literature suggest that evidence of mucin production may indicate poor prognosis in Wilms' tumor. In addition, evidence is reviewed that mucin itself may contribute to a rapid tumor growth and metastases. The genetics of Wilms' tumor and familial nature of these cases is also discussed.

Asunto(s)

Mucinas/sangre , Tumor de Wilms/metabolismo , Preescolar , Femenino , Humanos , Lactante , Linaje , Pronóstico , Tumor de Wilms/genética

RESUMEN

Significant alterations in hemotologic function in cystic fibrosis are suggested by the observation that polycythemia is uncommon, even among cyanotic patients. To elucidate those factors that influence hematologic equilibrium, 39 stable patients with cystic fibrosis were evaluated with regard to hemoglobin, hematocrit, RBC indices, reticulocyte count, serum iron and total iron binding capacity, serum ferritin, vitamin E, and carboxyhemoglobin levels. Hemoglobin concentrations were below the 50th percentile for age in 90% of the patients, including the 23% who were cyanotic. Serum ferritin levels were below the mean for age in 85% and below 12 ng/mL in 33% of patients. Vitamin E levels were less than 5 micrograms/dL in 33%, indicating deficiency. Carboxyhemoglobin values were elevated in 64% of the patients. These data indicate that relative anemia is common in cystic fibrosis and suggest that iron and vitamin E deficiency may contribute to that anemia. Twenty-two patients with cystic fibrosis were then given 2 weeks of oral iron therapy followed by two to three additional weeks of iron and vitamin E. This therapeutic trial resulted in an increase in mean hemoglobin concentration from 13.87 to 14.50 g/dL (P less than 0.01) associated with a significant increase in levels of serum ferritin (P less than 0.001). The increase in hemoglobin occurred primarily during the second 2 weeks when patients were receiving both iron and vitamin E. However, we were unable to document evidence of increased hemolysis when patients were receiving iron therapy alone. This response to oral iron therapy is confirmation that iron deficiency contributes to the failure of some patients with cystic fibrosis to compensate hemotologically for hypoxia.

Asunto(s)

Anemia Hipocrómica/etiología , Anemia/etiología , Fibrosis Quística/complicaciones , Adolescente , Adulto , Anemia/tratamiento farmacológico , Anemia Hipocrómica/tratamiento farmacológico , Niño , Preescolar , Fibrosis Quística/tratamiento farmacológico , Femenino , Pruebas Hematológicas , Humanos , Lactante , Hierro/uso terapéutico , Masculino , Estudios Prospectivos , Vitamina E/uso terapéutico , Deficiencia de Vitamina E/tratamiento farmacológico , Deficiencia de Vitamina E/etiología

Asunto(s)

Aminoácidos/metabolismo , Encéfalo/metabolismo , Metabolismo de los Hidratos de Carbono , Metabolismo Energético , Cloruro de Sodio/farmacología , Intoxicación por Agua , Alanina/análisis , Animales , Ácido Aspártico/análisis , Glucemia , Peso Corporal , Glucosa/análisis , Glutamatos/análisis , Glutamina/análisis , Ratones , Proteínas/análisis , Privación de Agua , Equilibrio Hidroelectrolítico , Ácido gamma-Aminobutírico/análisis

RESUMEN

Alloxan-induced diabetes of 4 days duration produced metabolite changes in brain compatible with severe reduction in cerebral metabolism (phosphocreatine increased 70%), and reduced phosphofructokinase activity (fructose diphosphate levels fell 38%). There was a 56% reduction in brain lactate concentration, but pyruvate levels were unchanged. In 5 of 23 animals, brain glycogen levels increased; in the remainder blycogen levels decreased. Brain fructose concentration, 0.4 mmol/kg, was only 1/30 of the glucose concentration. The alloxan-treated animals were also severely dehydrated. Therefore, to determine the casual relation of insulin deficiency to these findings, the effects of chronic dehydration and acute insulin deficiency were investigated. Findings in the brains of severely dehydrated animals (water deprivation and mannitol injections for 4 days) were almost identical with those seen after alloxan treatment. The exceptions were that, in the dehydrated mice, reductions in lactate and pyruvate were proportional, and glycogen levels were consistently reduced. In acute diabetes (6 to 24 hours after repeated anti-insulin serum injections) P-creatine, fructose diphosphate, and lactate levels were normal. Pyruvate levels were normal at 6 hours, but increased 39% by 12 to 24 hours; glycogen was 36% higher at 6 hours and 63% at 12 to 24 hours. Insulin (and glucose) appeared to be specific in correcting the metabolic abnormalities found in the brains of animals with alloxan-induced diabetes. At 4 and one half hours after treatment with insulin and glucose, glucose 6-phosphate levels fell 25%, fructose diphosphate increased 28%, and lactate and the lactate to pyruvate ratio returned to normal; glycogen increased 50%. However, the treatment also had a dramatic clinical effect. Since animals gained 8 to 27% of body weight during therapy, at least some of the improvements in metabolite levels could be related to rehydration.

Asunto(s)

Encéfalo/metabolismo , Metabolismo de los Hidratos de Carbono , Deshidratación/metabolismo , Diabetes Mellitus Experimental/metabolismo , Diabetes Mellitus/metabolismo , Anticuerpos Insulínicos , Animales , Glucemia/metabolismo , Metabolismo Energético , Glucógeno/metabolismo , Hidroxibutiratos/sangre , Sueros Inmunes , Insulina/farmacología , Manitol , Ratones