RESUMO
BACKGROUND AND PURPOSE: Outcomes following hematopoietic stem cell transplantation for higher risk childhood-onset cerebral adrenoleukodystrophy are variable. We explored whether a brain MR imaging gadolinium intensity scoring system improves prediction of neurologic outcome. MATERIALS AND METHODS: We developed a 4-point scale of gadolinium intensity relative to the choroid plexus: 0 = no enhancement; 1 = hypointense; 2 = isointense; 3 = hyperintense. The interobserver concordance of the scale was assessed on 30 randomly chosen studies. Scores were generated for 64 evaluable patients and compared with CSF chitotriosidase levels, a known inflammatory marker correlating with outcomes following transplantation. For 25 evaluable higher risk patients (Loes ≥10), the gadolinium intensity score was compared with longer term posttransplantation clinical change. RESULTS: The gadolinium intensity scoring system showed good interobserver reproducibility (κ = 0.72). Of 64 evaluable boys, the score positively correlated with average concomitant CSF chitotriosidase activity in nanograms/milliliter/hour: 0: 2717, n = 5; 1: 3218, n = 13; 2: 6497, n = 23; and 3: 12,030, n = 23 (P < .01). For 25 evaluable higher risk patients, more intense pretransplantation brain MR imaging gadolinium enhancement predicted greater average loss on the adrenoleukodystrophy neurologic function scale following transplantation: 0/1: adrenoleukodystrophy neurologic function scale score difference = 4.3, n = 7; 2/3: adrenoleukodystrophy neurologic function scale score difference = 10.4, n = 18 (P = .05). CONCLUSIONS: Gadolinium enhancement intensity on brain MR imaging can be scored simply and reproducibly for cerebral adrenoleukodystrophy. The enhancement score significantly correlates with chitotriosidase. In boys with higher risk cerebral disease (Loes ≥10), the enhancement score itself predicts neurologic outcome following treatment. Such data may help guide treatment decisions for clinicians and families.
Assuntos
Adrenoleucodistrofia/patologia , Adrenoleucodistrofia/terapia , Transplante de Células-Tronco Hematopoéticas , Imageamento por Ressonância Magnética/métodos , Encéfalo/fisiopatologia , Criança , Meios de Contraste , Gadolínio , Humanos , Inflamação/patologia , Masculino , Reprodutibilidade dos Testes , Fatores de Risco , Resultado do TratamentoRESUMO
Based on this survey, the prevalence of chronic illness and disability in older adult patients with respect to current ambulatory surgery practices is worrisome, especially the fragmentation of patients' care as they progress from the decision to undergo procedures, to preoperative assessment, to postoperative discharge. Ambulatory surgery unit nurses, case managers, surgeons, and office staff members must work together to better optimize time and resources. As baby boomers age, the number of older adults undergoing ambulatory surgery will increase. It is essential for ASU staff members to be cognizant of the special needs of older adult patients. Timeliness, patient education, and appropriate coordination and follow-up of care are important ingredients to successfully plan care for older adult patients undergoing ambulatory surgery. Nurses can play a pivotal role in this success.