Voice and emotion processing in the human neonatal brain.

Although the voice-sensitive neural system emerges very early in development, it has yet to be demonstrated whether the neonatal brain is sensitive to voice perception. We measured the EEG mismatch response (MMR) elicited by emotionally spoken syllables "dada" along with correspondingly synthesized nonvocal sounds, whose fundamental frequency contours were matched, in 98 full-term newborns aged 1-5 days. In Experiment 1, happy syllables relative to nonvocal sounds elicited an MMR lateralized to the right hemisphere. In Experiment 2, fearful syllables elicited stronger amplitudes than happy or neutral syllables, and this response had no sex differences. In Experiment 3, angry versus happy syllables elicited an MMR, although their corresponding nonvocal sounds did not. Here, we show that affective discrimination is selectively driven by voice processing per se rather than low-level acoustical features and that the cerebral specialization for human voice and emotion processing emerges over the right hemisphere during the first days of life.

Harlequin ichthyosis: report of one case.

Harlequin ichthyosis (HI) is the most devastating form of skin disorder, which is inherited as autosomal recessive trait related to consanguineous marriage. Although prenatal examination has become scheduled and convenient throughout Taiwan, an unexpected case of HI in a male premature infant born at 32 weeks of gestation was presented. The parents were healthy, neither relatives nor having history of congenital abnormality. We report our management and the massive impact left on both parents. We believe this is an extremely rare case in Taiwan.

SUMO regulates the cytoplasmonuclear transport of its target protein Daxx.

It is known that Fas death domain-associated protein (Daxx) possesses both putative nuclear and cytoplasmic functions. However, the nuclear transport mechanism is largely unknown. This study examined the nuclear location signal (NLS) of Daxx and whether the nuclear transport of Daxx was mediated by small ubiquitin-related modifier (SUMO). Two NLS motifs of Daxx, leucine (L)-rich nuclear export signal (NES)-like motif (188IXXLXXLLXL197) and C-terminal lysine (K) rich NLS2 (amino acids 627-634) motif, were identified and the K630 and K631 on the NLS2 motif were characterized as the major sumoylation sites of Daxx by in vitro sumoylation analysis. Proteins of inactive SUMO (SUMO-delta), a sumoylation-incompetent mutant, and Daxx NLS mutants (Daxx-NES(mut) and Daxx NLS2(mut)) were dispersed in cytoplasm. The cytoplasmic dispersed Daxx mutants could be relocalized to nucleus by cotransfection with active SUMO, but not with inactive SUMO-delta, demonstrating the role of SUMO on regulating the cytoplasmonuclear transport of Daxx. However, inactive SUMO-delta could also be relocalized to nucleus during cotransfection with wild-type Daxx, suggesting that SUMO regulation of the cytoplasmonuclear transport of its target protein Daxx does not need covalent modification. This study shows that cytoplasmic SUMO has a biological role in enhancing the cytoplasmonuclear transport of its target protein Daxx and it may be done through the non-sumoylation interactions.

Significance of pulmonary venous obstruction in total anomalous pulmonary venous return.

BACKGROUND: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital cardiovascular anomaly with poor natural prognosis. It has been detected more frequently in recent year due to the advent of echocardiography and cardiovascular magnetic resonance imaging (MRI). The aim of this study was to evaluate the clinical manifestations and outcomes in TAPVR patients with or without pulmonary venous obstruction (PVO). METHODS: From January 1985 to December 2002, a total of 27 cases with TAPVR at our institution were reviewed. Accurding to the preseace or assence of PVO, patients were divided into PVO group and non-PVO group. Patients' sex, age at diagnosis, types of TAPVR, clinical manifestations, surgical treatment and outcomes were evaluated. RESULTS: All of them had received 2-dimensional (2-D) and color Doppler echocardiography examination. Cardiac catheterization was performed in all but 1 patient who died at the first day of birth. In addition, 10 of 27 cases had cardiovascular MRI for further study. The number of cases in PVO group and non-PVO group were 15 (56%) and 12 (44%), respectively. There was no significant difference in sex or pulmonary venous drainage sites between both groups. Cyanosis was more prevalent in the PVO group (80% vs. 30%, p = 0.038). Four (27%) cases PVO group and 3 (25%) cases of the non-PVO group had of the non-isolated cardiac lesions. Pulmonary hypertension was present in 18 (69%) of 26 cases who had received cardiac catheterization. Among them, 10 had PVO and 5 had systemic level of pulmonary arterial pressure. Seven (30%) of 23 patients who had received operation died; in contrast, 3 of 4 patients without operation expired. The remaining 1 did not had surgery because of complex heart disease. There was no significant difference in surgical mortality between PVO and non-PVO groups (33% vs. 27%). CONCLUSIONS: Cyanosis is an obvious clinical symptom of obstructed TAPVR. Surgical mortality made no significant difference between obstructed and non-obstructed groups. Early detection and surgical treatment for TAPVR are important. Although cardiac catheterization and angiocardiography is the golden standard for the diagnosis, 2-D and color Doppler echocardiography can also provide quick and accurate diagnostic images of TAPVR.

Transcatheter implantation of intravascular stents for postoperative residual stenosis of peripheral pulmonary artery stenosis.

This is a prospective study of transcatheter implantation of 11 intravascular stents in 7 patients with status/post (S/P) surgical correction of major cardiovascular lesions. The safety and efficacy of balloon-expandable stents for treatment of peripheral pulmonary artery stenosis (PPAS) is evaluated and analyzed. Although the transcatheter implantation of intravascular stents has been reported as a possible treatment for stenotic peripheral pulmonary arteries, the results of intermediate follow-up studies on patients with S/P surgical correction for residual PPAS need to be evaluated. From June 1998 to December 2001, a total of 15 patients with PPAS having S/P surgery for major cardiovascular lesions were enrolled in this study. Eight of them had redo surgery after complete evaluation and the other 7 patients who might be at higher risk of mortality or morbidity from redo surgery, underwent transcatheter implantation of stents to dilate significant PPAS. Tetralogy of Fallot, S/P total correction, was done in 6 and transposition of great vessels, S/P Jatene operation, was done in 1. There were 10 stents (P 308 Palmaz stent x8 and Intrastent x2) implantation for 10 sites of the stenotic PPAS in these 7 patients, who were aged from 3.6 to 17.3 (10.1 +/- 5.6) years and had body weights ranging from 17 to 72.5 (37.1 +/- 23.0) kg. The narrowest diameter of the stenotic peripheral pulmonary arteries and pressure gradients across the stenosis were measured before and after implantation of stents. A follow-up catheterization and pulmonary angiography was performed 1 year later to evaluate the intermediate efficacy of stents implantation. All the stenotic peripheral pulmonary arteries of these 7 patients had a significant reduction of pressure gradients immediately after the procedure. The narrowest mean diameter of pulmonary arteries increased from 6.7 +/- 3.4 to 11.3 +/- 3.0 mm (p < 0.001), and the mean pressure gradient dropped from 31 +/- 9.9 to 11.4 +/- 4.6 mm Hg (p < 0.001). The follow-up catheterization 1 year later revealed a persistent effect in all but 1 patient. Only a young male presented with a recurrent stenosis with a pressure gradient of > or = 20 mm Hg, which was relieved by redilation with implantation of another stent. There was no immediate or intermediate complication. Transcatheter stent implantation for treatment of a significant residual PPAS after surgical correction of complicated congenital heart disease is a safe and effective procedure. Since children are growing with age, a long-term follow-up study to evaluate the effects and possible problems of stent implantation is mandatory.