Abnormal ventricular function in patients with univentricular heart. Cineangiographic study.

In order to investigate the basis of ventricular dysfunction in patients with univentricular heart, cineangiograms of 20 patients aged between 3 weeks and 22 years were digitized frame by frame and compared with left ventriculograms of patients with other cardiac malformations. Ejection fraction was at least normal (58% to 95%) and peak rate of systolic minor axis shortening was normal in all. However in only 3 patients was the change in volume achieved by virtue of the normal change in cavity shape; in 17 the end-systolic outline was abnormally circular and shape index, 4 pi (area)/(perimeter)2, changed by less than normal (p less than 0.01). In addition, abnormalities of regional wall movement were found in 12 patients. 11 showed a consistent pattern, unlike that found in ischaemic heart disease or other congenital lesions, of delayed systolic inward movement of the antero-apical wall, associated in 9 with hypokinesis. Four of these showed secondary delay of relaxation in the same region. One patient had an isolated abnormality of isovolumic relaxation. These findings were independent of age, volume overload or degree of cyanosis, but were more common in patients with absent atrioventricular connexion. We conclude that the architectural abnormalities in univentricular hearts lead to disturbances of myocardial function which particularly affect the apex. These are detectable from an early age.     

Regional left ventricular wall movement in hypertrophic cardiomyopathy

Left ventriculograms of 20 patients with hypertrophic cardiomyopathy were digitised frame by frame and analysed using a contour display. Abnormalities of regional wall movement were present in 17, and included an abnormal sequence of inward movement during systole (13), regional delay in the onset of inward movement (10), and an abnormal dispersion of peak velocities (5). Diastolic wall movement was disturbed in 13, because of abnormal peak velocities in 7 and regional asynchrony in 6. Abnormal wall movement during the two isovolumic periods was rare in hypertrophic cardiomyopathy, unlike ischaemic heart disease. These disturbances may reflect underlying structural abnormalities.     

兔下颌垂直牵张骨形成中Smad1的表达

目的:观察细胞内信号转导分子Smad1在兔下颌骨垂直牵张后骨组织中的表达,探讨Smad1在新骨形成中的作用。方法:实验于2006-01/03在辽宁医学院动物实验中心完成。实验材料:健康日本大耳白兔18只,雌雄不限,体质量1.5～1.75kg。自制双螺旋种植型骨牵张器:外直径4.0mm,螺距0.75mm;内直径2.0mm,内螺距0.25mm。实验分组:根据随机排列表分为空白对照组3只(非手术)、牵张组15只,其中牵张组1d,1,2,4,6周每个时间点各3只。实验干预:①用摆动锯对兔下颌骨截骨,形成8mm×5mm骨升段,建立动物模型。②根据升骨段所在的位置安放种植牵张器,对兔下颌骨垂直牵张,1mm/d,2次/d,牵张4d。实验评估:分别于牵后第1天,1,2,4,6周取材,通过X射线及苏木精-伊红染色观察新骨形成情况,并用免疫组织化学法检测兔下颌骨垂直牵张过程中不同时期Smad1的表达与分布。结果:18只兔均进入结果分析。①X射线及组织学观察:牵张1周,牵张区呈大的透射区,骨皮质连续性中断,牵张间隙两端界面清晰可见,牵张间隙呈低密度影像,牵张间隙内新骨逐渐形成,6周时牵张区基本被成熟新生骨充填。②免疫组织化学观察,牵张结束后Smad1表达增强,阳性信号主要在间充质细胞和新生骨小梁边缘的成骨细胞表达。Smad1/5在牵张成骨中的定量分析结果,在牵张成骨1d,1,2,4,6周及空白对照组Smad1表达灰度值分别为:98.18±7.18,78.94±8.58,115.50±14.60,138.48±15.69,150.01±12.81,151.06±12.85。Smad1表达高峰在牵张后1周,以后逐渐下降,牵张后4周在骨细胞微弱表达,6周时恢复到正常水平(P>0.05)。结论:Smad1参与兔下颌骨垂直牵张新骨形成过程,并在牵张成骨的早期诱导间充质细胞分化为成骨细胞过程中可能起重要作用。     

Abnormalities of dynamic ventricular shape change in patients with aortic and mitral valvular regurgitation: assessment by Fourier shape analysis and global geometric indexes

The normal cardiac cycle is associated with dynamic changes in left ventricular shape, which can be disturbed in disease states. To assess the influences of diastolic volume, percent ejected volume, and abnormalities of acute or chronic systolic loading on general and detailed chamber geometry, we studied dynamic shape change recorded by x-ray contrast ventriculography in both normal patients and those with aortic (AR) or mitral (MR) valve regurgitation. While both lesions increased diastolic volume, the character of load throughout ejection differed markedly. Detailed cavity geometry was assessed by a Fourier analysis technique and general shape by eccentricity and circularity indexes. Normal hearts, showed increased systolic elongation by all indexes. AR patients displayed a similar rise in eccentricity during ejection; however, the extent of shape change when measured by Fourier and circular indexes was reduced. In contrast, MR patients displayed enhanced systolic shape change, particularly in chamber elongation. Neither simple eccentricity of circular indexes adequately differentiated these shape abnormalities, whereas detailed Fourier geometric analysis precisely characterized the abnormalities of shape change in these two diseases. Relations between the extent of shape change and ejected volume for each patient group revealed significantly more systolic deformation with a different shape versus volume relation for the MR hearts as compared with AR and controls. Thus, while dynamic left ventricular shape is certainly influenced by the extent of volume change, it also varies independently from volume related to the specific nature of loading during ejection.     

Intraocular crystals in HIV-related hypergammaglobulinemia

PURPOSE: To describe the occurrence of bilateral iris and retinal crystals in a patient with benign HIV-related gammopathy. DESIGN: Observational case report. METHODS: A 45-year-old HIV-positive male with a known, benign hypergammaglobulinemia was referred for routine ophthalmic assessment because of right ophthalmic zoster. RESULTS: Ophthalmoscopic examination revealed the presence of bilateral, multiple, refractile iris and macular crystals. No other signs of uveitis or stigmata of HIV were found. CONCLUSION: Intraocular crystals are a rare finding, usually seen in chronic uveitis with or without hypergammaglobulinemia. This finding has not been previously described in non-inflamed eyes, nor as an ocular manifestation of HIV. We hypothesize that HIV-related disruption of the blood-aqueous and blood-retinal barriers allows intraocular immunoglobulin deposition in patients with hypergammaglobulinemia.     

The treatment of digoxin overdosage.

A case of digoxin overdosage is presented in which hemodialysis and forced diuresis were employed in the treatment. Hemodialysis was found to be ineffective in removing significant amounts of the drug and additional experimental work using a dog confirmed that forced diuresis failed to alter the rate of digoxin excretion.