Congenital quadricuspid aortic valve with tetralogy of Fallot and pulmonary atresia

We report the case of a 4-year-old girl who had quadricuspid aortic valve regurgitation with tetralogy of Fallot and pulmonary atresia. This combination is very uncommon. Aortic valve replacement was performed successfully due to aortic regurgitation which had progressed one year after the total repair. The dilated aortic annulus plus quadricuspid aortic valve may result in progressive aortic regurgitation for a short period.     

Ruptured pediatric posterior cerebral artery aneurysm 9 years after the onset of Kawasaki disease: a case report

CASE REPORT: A 12-year-old boy who had a history of Kawasaki disease 9 years ago experienced a subarachnoid hemorrhage by ruptured right posterior cerebral artery aneurysm. On day 1 operation, as the aneurysm was very fragile and bled easily, two intraoperative ruptures, including a very premature rupture, were encountered. As a result, a left hemiparesis especially severe in the left hand was caused by the right anterior thalamic infarction due to the occlusion of a thalamo-perforating artery arising near the neck of the aneurysm. DISCUSSION: The histopathological examination of the intraoperative excised aneurysmal dome disclosed the thickening of the endothelial inner due to the endothelial hypertrophy and the invasion of inflammatory cells. This finding of the aneurysm was partially mimicking the finding of the coronary artery of the patients with Kawasaki disease. The combination of cerebral aneurysm and Kawasaki disease has never been reported until now, and the etiology of the aneurysm of this patient is unclear.     

Cone dysfunction and supernormal scotopic electroretinogram with a high-intensity stimulus

An unusual form of scotopic electroretinogram with a bright white stimulus, which consisted of a rectangular a-wave of normal amplitude and a b-wave of supernormal amplitude, was recorded in three patients with cone dysfunction. In addition to poor visual acuity, abnormal color vision and reduced amplitude of the photopic electroretinogram, these patients showed a 2-log unit elevation of the dark-adaptation threshold. Funduscopic examination and fluorescein angiography revealed fine granular pigment disturbances at the mascula. The relationship between the response of the dark-adapted electroretinogram versus stimulus intensity was unique to these patients. The b-wave thresholds were elevated by 1 log unit. The b-waves were reduced in amplitude and markedly delayed in implicit time to dim stimuli, but supernormal in amplitude and normal in implicit time to bright stimuli.Abbreviations GMP guanosine monophosphate     

Left thoracotomy approach in reoperative off-pump coronary revascularization

Objective: Reoperative coronary bypass grafting is at high risk. Particularly in redo cases where the patent graft is running near the midline of the sternum, the graft may be exposed to injury by a median sternotomy and subsequent dissection. Whereas, off-pump bypass grafting from the left axillary artery or descending thoracic artery by a left thoracotomy approach is safe for preventing graft damage.Methods: From March 1998 to February 2002, we performed off-pump coronary artery bypass grafting by a left thoracotomy approach in 9 patients. The left axillary artery was used as the inflow vessel in 4 cases, and the descending thoracic, aorta in 5.Results: The radial artery was anastomosed proximally to the axillary artery in 4 cases and the descending thoracic aorta in one case. The saphenous vein graft was anastomosed, proximally to the descending thoracic aorta in 4 cases. Transdiaphragmatic minimally invasive bypass grafting for the right coronary artery was simultaneously performed in 3 cases. Postoperative cardiac events were ventricular arrhythmia in 6 cases and supraventricular arrhythmia in 3 cases. There was no damage to the patent grafts. Postoperative coronary angiography performed, in 8 cases revealed all the grafts to be patent without stenosis. Cardiac symptoms were not found after the operation in any of the cases.Conclusions: These procedures can prevent the injury to patent grafts caused by a median sternotomy, and will be one of the useful strategies for reoperative off-pump coronary artery bypass grafting.     

Effects of N-(2-ethylhexyl)-3-hydroxybutyramide hydrogen succinate on the metabolisms of monoamines in the rat brain

N-(2-Ethylhexyl)-3-hydroxybutyramide hydrogen succinate (M-2H) has been reported to increase REM sleep. The effects of M-2H on the metabolisms of monoamines which have been implicated in the regulation of the sleep-wakefulness cycle were studied in rats. M-2H (500 mg/kg p.o.) increased the levels of 5-hydroxyindoleacetic acid (5-HIAA), homovanillic acid and 3-methoxy-4-hydroxyphenylethyleneglycol sulfate in various regions of the rat brain. However, no appreciable changes occurred in levels of serotonin (5-HT), norepinephrine, dopamine, or 3,4-dihydroxyphenylacetic acid. The effect of M-2H (500 mg/kg p.o.) on 5-HT metabolism was investigated with intraventricularly-injected [14C]-5-HT. Although [14C]-5-HT level was not significantly changed, [14C]-5-HIAA level was above control level in four brain regions such as the hypothalamus, hippocampus, midbrain and pons-medulla at 3 h after the administration of M-2H. M-2H retarded the efflux of intraventricularly-injected [14C]-5-HIAA in all brain regions except the cortex. These results suggest that M-2H preferentially impaired the transport system for acid metabolites of monoamines, resulting in increase of monoamine metabolite levels in the brain.     

A model for estimating the potential costs and savings of osteoporosis prevention strategies

A model was developed which estimates the costs of osteoporosis risk evaluation and treatment, and the resulting savings in terms of reduced fracture frequency, for the adult female population of the United States. In the absence of treatment, the model predicts 1.44 million fractures will occur annually from non-violent causes. Treatment of all women beginning at age 50 with an agent that slows bone loss by 50% would reduce the number of these fractures by 0.59 million. Selective treatment of the 47% of women at the greatest fracture risk would reduce the number of fractures by 0.45 million, but would only cost 47% as much as treating all women. Additional data are required before the model can be used to evaluate specific treatment regimens. However, it appears that selective treatment of those at highest risk would yield the greatest benefit to cost ratio, if only benefits related to reduced fracture frequency are considered.     

Sex cord-stromal tumors of the ovary

The developmental relationship between granulosa and stromal cells in the fetal ovary was reviewed. The proliferation and expression of basic fibroblast growth factor (bFGF) and luteinizing hormone receptor (LHR) of both the granulosa and theca cells was examined in the adult ovary. Ovarian granulosa cell tumors were then studied under light and electron microscopy and for immunohistochemical expression of bFGF and LHR. In the follicular development of the adult ovary, bFGF expression in the granulosa cells was correlated with theca cell proliferation. In ovarian granulosa cell tumors, estrogen-producing tumors showed a stratified or microfollicular pattern being accompanied by theca cells which had ultrastructural features of steroid-production, and these tumor cells exhibited immunoreactivity for bFGF. In contrast, tumors with no apparent hormone-production showed a solid or gyriform pattern without admixture of theca cells, and were negative for bFGF. LHR was expressed in both types of tumor. Accordingly, bFGF may play an important role in association with the theca cell component in granulosa cell tumors.