Impact of intravenous immunoglobulin infusion on longitudinal left ventricular performance in patients with acute Kawasaki disease of usual course.

PURPOSE: To determine the acute change in cardiac performance after intravenous immunoglobulin infusion (IVIG) in patients with acute Kawasaki disease (KD). MATERIALS AND METHODS: Subjects were 33 patients with KD who were treated with IVIG 2 g/kg and recovered without coronary artery lesion and 27 controls. Subjects underwent combined two-dimensional, Doppler, and tissue Doppler echocardiographic (TDI) studies. In KD, these echocardiographic studies were performed before IVIG, 48 h after IVIG, and in convalescence. Echocardiographic variables were compared between KD and controls as well as among 3 time points in KD. RESULTS: Before IVIG, KD showed significantly higher peak aortic velocity and shorter aortic ejection time as results of tachycardia and significantly lower E' (p<0.04) but significantly higher E/E' (p<0.02). After IVIG, patients with KD became afebrile and showed significantly lower TDI indices such as S', E', and, A' and isovolumic acceleration (IVA) (163+/-56 vs. 208+/-70 cm/s(2), p<0.01) with higher TDI-derived Tei index (0.50+/-0.10 vs. 0.44+/-0.06, p<0.02) than controls. These differences tended to disappear in convalescence. In analysis of repeated measurements, except for hemodynamic changes associated with tachycardia, S' (7.9+/-1.3 vs. 7.0+/-1.1 vs. 7.4+/-0.9 cm/s, p<0.001), IVA (227+/-72 vs. 163+/-56 vs. 180+/-63, p<0.05), and A' (7.7+/-3.0 vs. 5.6+/-1.3 vs. 6.7+/-2.3 cm/s, p<0.001) were significantly different among these time points. CONCLUSIONS: In patients with acute KD with usual course, IVIG induced transient sub-clinical longitudinal left ventricular dysfunction.

[Neuroimaging and neurochemical studies of Rett syndrome].

We review here the current status of neuroimaging and neurochemical research on Rett syndrome (RTT), with reference to neurophysiological, neuropathological, and immuno-histochemical changes previously described. Abnormalities have been reported in the intermediates of the biogenic amine neurotransmitters/receptor systems, and of beta-phenylethylamine (PEA), an endogenous amine synthesized by the decarboxylation of phenylalanine in dopaminergic neurons of the nigrostratal system. We also discuss the roles of other neurotransmitters including beta-endrophin, substance P and neurotrophic factors including nerve growth factors. Recently, mutations in the gene encoding methyl-CpG binding protein 2 (MeCP2), mapped to Xq28, have been identified in patients with RTT. Multiple abnormalities in various neurotransmitter/receptor systems may accounts for the pervasive defects in RTT.