Is High-Dose Aspirin Necessary in the Acute Phase of Kawasaki Disease?

BACKGROUND AND OBJECTIVES: We sought to determine whether high-dose aspirin is necessary for the acute therapy of Kawasaki disease (KD) in the intravenous immunoglobulin (IVIG) era. SUBJECTS AND METHODS: Two groups of KD patients treated during the different periods were included. Study group (n=51, treated with IVIG without concomitant use of aspirin in the acute phase) was compared with control group (n=129, treated with IVIG plus high-dose aspirin) with regard to the response to IVIG, duration of fever after IVIG completion, time to C-reactive protein (CRP) <3 mg/dL, and the incidence of coronary artery lesions (CALs). RESULTS: There was no difference between the groups in age, sex, and duration of fever before treatment. Pre-IVIG laboratory measures also did not differ from each other. IVIG-resistant cases were 8 (15.7%) in study group and 22 (17.1%) in control group (p=1.000). Mean duration of fever after IVIG completion in IVIG-responsive patients was 13.3+/-13.5 hours in study group compared to 6.2+/-8.3 hours in control group (p=0.000). The mean time to decrease in CRP was 4.0+/-1.7 days in study group and 4.1+/-2.2 days in control group (p=0.828). There were 2 (3.9%) patients with CALs in study group and 10 (7.8%) in control group (p=0.514). CONCLUSION: Although high-dose aspirin shortens the duration of fever, treatment without aspirin in the acute phase has no influence on the response to IVIG, resolution of inflammation, or the development of CALs. In the IVIG era, high-dose aspirin may provide little benefit to the treatment in the acute phase of KD.

Posterior reversible encephalopathy syndrome in pediatric patients undergoing treatment for hemophagocytic lymphohistiocytosis: clinical outcomes and putative risk factors

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare multiorgan disease of toxic immune activation caused by the interaction of cytotoxic T cells and innate immune cells and frequently involves the central nervous system (CNS). Posterior reversible encephalopathy syndrome (PRES) might develop during treatment with the HLH-2004 protocol from the Histiocyte Society. The aims of this study were to evaluate clinical outcomes and putative risk factors for prediction of PRES related to HLH. METHODS: We reviewed the medical records of 28 patients with HLH who were treated between April 2005 and April 2012. We compared various clinical and laboratory parameters in patients without or with PRES to evaluate putative risk factors related to development of PRES. RESULTS: Six (21.4%) of the patients experienced PRES during treatment with the HLH-2004 protocol. Clinical and laboratory manifestations were not different compared with other conditions causing PRES. The main mechanism of PRES may be related to the HLH-2004 protocol and a high pro-inflammatory state. Most patients recovered quickly from neurologic manifestations without significant long-term sequelae. Preceding hypertension, an increase in ferritin level >50% compared with 1 week before development of PRES and hyponatremia were statistically significant factors. CONCLUSION: PRES is clinically reversible and has a favorable outcome in patients with HLH. Awareness of PRES and a differential diagnosis of other causes of neurologic complications, including CNS involvement of HLH, can help avoid unnecessary treatment or delayed management. Patients with preceding hypertension, hyponatremia, and rising ferritin levels during HLH treatment should be closely monitored for PRES.