RESUMEN
Although sickle cell disease (SCD) and systemic lupus erythematosus (SLE) are two distinct chronic diseases, many clinical features are common to both conditions. We describe a young patient who had a mild clinical course of SCD until SLE developed when he was 15 years old. His initial manifestations of SLE including fever, chest pain, and lung infiltration with pleural effusion were thought to be complications of SCD. However, a deteriorating clinical course, presence of facial and truncal rash, and persistent pleural effusion led to the diagnosis of SLE. We compare our case and the 10 previously reported cases and discuss the possible association of complement defects and the pathogenesis of SLE in patients with SCD. Our report illustrates the importance of considering other disease processes when clinical features are atypical of SCD.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Adolescente , Dolor en el Pecho , Proteínas del Sistema Complemento/análisis , Fiebre , Humanos , Enfermedades Pulmonares/complicaciones , Lupus Eritematoso Sistémico/etiología , Masculino , Derrame Pleural/complicaciones , Urticaria/complicacionesRESUMEN
Nineteen children with congenital, mycotic, traumatic and tumoral arterial aneurysms were studied neuroradiologically. The important role of computed tomography (CT) was shown in two traumatic aneurysm cases where bleeding was clinically inapparent. Among the unique cases described was a boy with a pituitary tumor in whom an aneurysm was discovered incidentally; coexistence of these lesions in childhood has not been documented previously. Nor has a tumoral aneurysm been mentioned (the case reported had a surrounding primary anaplastic sarcoma). Rare cases included a cavernous carotid mycotic aneurysm and infants with hemorrhage from congenital distal middle cerebral artery aneurysms. Marked cellular responses within the aneurysm walls, believed to be a reaction to hemorrhage, were noted in both infants.
Asunto(s)
Aneurisma Infectado/diagnóstico por imagen , Aneurisma Intracraneal/diagnóstico por imagen , Malformaciones Arteriovenosas Intracraneales/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adolescente , Neoplasias Encefálicas/complicaciones , Niño , Preescolar , Traumatismos Craneocerebrales/complicaciones , Femenino , Humanos , Lactante , Aneurisma Intracraneal/etiología , MasculinoRESUMEN
Increasing interest has developed in the use of the photodynamic agent, Hematoporphyrin derivative (HpD) for photoradiation therapy (PRT) as adjunctive therapy of malignant glial tumors of the brain. HpD, injected systemically, is preferentially taken up and retained by neoplastic tissue. Early studies of such uptake have largely relied on gross fluorescence as evidence of tissue uptake. In this study HpD was labelled with a tritiated radioisotope (3H) in order to quantify tissue uptake in visceral and in normal and neoplastic brain tissues in a rat brain model. 3H-HpD was injected intravenously at a 10 mg/kg dose into 30 Sprague-Dawley rats (Group A) without tumors in order to clarify method. Separately, 3H-HpD of like dosage was injected into 20 Fischer-344 rats (Group B), 5 control and 15 with a 9L gliosarcoma implanted in the left anterior cerebral cortex. Post injection sacrifice occurred at 6, 24 and 48 hours. From the Sprague-Dawley group multiple somatic and cerebral specimens were assayed. Differential areas within the brain showed no significant difference in uptake. The tumor area, peritumoral margin, and distant uninvolved areas of the Fischer-344 9L rats were likewise assayed. Definite uptake of normal visceral and cerebral tissue occurred with a markedly higher uptake differential in tumor areas. Such differential was relatively consistent from trial to trial, but multiple separate values obtained in the respective study groups were often unreliabe in their reproducibility and at variance with previously reported tissue level studies. These findings implied an instability of 3H-HpD, subsequently confirmed chromatographically as contamination probably due to time related degradation and exchange. Therefore, 3H-HpD appears to inherently carry such a risk for contamination. The compound Photofrin II (HpDII) represents a chromatographic fraction of HpD (HpDI), currently considered its most photodynamically active and purest component. Tritiated Photofrin II was used for quantification. An assay was performed with 5 Fischer-344 9L brain tumor rats (Group C), sacrificed at 24 hours. Photofrin II provided results more reliably reproducible. Contamination, degradation, and exchange of 3H-Photofrin II did not appear to occur. Neoplastic brain levels of the Photofrin II isotope were higher than in the HpD studies, and highly fluorescent. Normal brain values were consistently minimal and without fluorescence. The differential tumor/brain ratio in Photofrin II was consequently much higher. The isolated active substrate of HpDI and HpDII (Photofrin II) appears to be the compound DiHematoporphyrin Ether (DHE).(ABSTRACT TRUNCATED AT 400 WORDS)