Anesthetic management of a Rett syndrome patient with apnea and epilepsy: a case report.

Rett syndrome, which is a progressive, central nervous system disease that is caused by a gene mutation, is known to present with various symptoms. This case is that of a 15-year-old girl who was diagnosed with Rett syndrome at the age of 2 years. Laryngotracheal isolation under general anesthesia was planned due to recurrent aspiration pneumonia. Since the patient's nutritional status and control of convulsions were good, this was deemed an appropriate time for the surgery. Following careful preoperative evaluation of her airway, we performed oral endotracheal intubation using a video laryngoscope after rapid induction. Since postoperative pain control was important to prevent apneic attacks and convulsions, we used a multimodal analgesic regimen including carefully titrated fentanyl, acetaminophen, nonsteroidal anti-inflammatory drug, and wound infiltration with a local anesthetic. Postoperatively, the patient returned to the intensive care unit under spontaneous ventilation and followed a good course. Patients with Rett syndrome present several symptoms. Thus, several points must be considered during the preoperative evaluation, anesthetic management, and postoperative care of these patients.

Facilitation of adipocyte differentiation of 3T3-L1 cells by debrominated tetrabromobisphenol A compounds detected in Japanese breast milk.

Tetrabromobisphenol A (TeBBPA) is widely used type of brominated flame retardant. In this study, we newly synthesized materials for the debrominated congeners, 2,2',6-tribromobisphenol A (TriBBPA), 2,2'-dibromobisphenol A (2,2'-DiBBPA), 2,6-dibromobisphenol A (2,6-DiBBPA), and 2-monobromobisphenol A (MoBBPA) and evaluated the actual extent of contamination with bisphenol A (BPA), TeBBPA and debrominated congeners in Japanese breast milk samples. TriBBPA was detected at higher levels than that of TeBBPA, while DiBBPA and MoBBPA were detected at lower levels than that of TeBBPA. This observation suggested that humans are exposed to debrominated congeners, which might cause adverse effects. Contamination of the congeners in breast milk was concern about risk infant health, having vulnerable defense system. As pilot study by in vitro experiment, we assessed the toxic potency of debrominated congeners by studying their effect on adipocyte differentiation in 3T3-L1 cells. We observed 2,6-DiBBPA, TriBBPA and TeBBPA elevated the lipid accumulation and adipocyte-specific protein 2 expression in a manner dependent on the number of substituted bromines. Moreover, PPARγ transcriptional activities increased in a dose-dependent manner in the presence of 2,6-DiBBPA and TriBBPA as well as TeBBPA. Our study clarified that TeBBPA and its debrominated congeners accumulated in breast milk and the debrominated congeners promoted adipocyte differentiation, showing that a comprehensive evaluation of the influences of these compounds including the debrominated congeners of TeBBPA on health in infants is necessary.

Optical coherence tomographic assessment of dynamic macular changes in patients with Vogt-Koyanagi-Harada disease.

To determine the relationship between visual acuity and three-dimensional optical coherence tomographic (3D-OCT) findings of the macula in eyes with Vogt-Koyanagi-Harada (VKH) disease. Twelve eyes of six patients (three men and three woman, average age 53.2 years) in the acute phase of VKH disease were examined with a 3D-OCT instrument. All of the eyes had a serous macular detachment. The height of the sensory retinal detachment (SRD) and the sensory retinal thickness (SRT) were measured by OCT before treatment (acute stage) and at the convalescent stage. The correlation between the retinal morphology and visual acuity was evaluated. The height of the SRD and the SRT were 612.5 ± 371.2 and 136. 7 ± 22.0 µm, respectively. The initial visual acuity was significantly worse in eyes with a higher SRD (P = 0.014, r = 0.68) but the correlation between initial visual acuity and SRT was not significant. The recovery of visual acuity was attained in 50.7 ± 44.1 days and the complete resolution of the SRD was attained in 30.5 ± 23.2 days. The final visual acuity was attained several days after the complete resolution of the SRD in all four eyes of patients over 60 years of age, but the recovery of visual acuity often preceded the complete resolution of the SRD. The OCT images provided a noninvasive indicator of the severity of the disease and dynamic changes in the macular morphology, reflecting the effect of treatment in association with the improvement in visual acuity. Monitoring the SRD by 3D-OCT may guide the tapering of systemic corticosteroid treatment.

Optical coherence tomographic evaluation of the outer retinal architecture in Oguchi disease.

BACKGROUND: To report the changes in the optical coherence tomography (OCT) images of the outer retinal layers after prolonged dark adaptation in a patient with Oguchi disease. CASE: A 75-year-old woman showed the typical golden-yellow fundus reflex of Oguchi disease, and the coloration returned to normal after prolonged dark adaptation. Fourier domain OCT (FD-OCT) was performed on the patient's left eye before and after prolonged dark adaptation. OBSERVATIONS: Before dark adaptation, the FD-OCT images at the fovea had three identifiable reflection bands, namely, the external limiting membrane (ELM), the border between the photoreceptor inner and outer segments (IS/OS line), and the retinal pigment epithelium (RPE)/Bruch band. The paramacular area had only the ELM and RPE/Bruch bands. After 4 h of dark adaptation, the IS/OS line was also detected in the paramacular area. CONCLUSIONS: The absence of the IS/OS line in the extramacular regions in the partly dark adapted condition was most likely due to a defect in the rod photoreceptors of this area. The emergence of the IS/OS line after prolonged dark adaptation suggests that microarchitectural changes occur in the photoreceptors and that the changes may be correlated with the improvement of rod function.