Chronic verapamil therapy in pediatric and young adult patients with hypertrophic cardiomyopathy

Oral verapamil, 5.2 +/- 1.1 mg/kg/day (range 2.8 to 7), was administered to 13 pediatric patients with hypertrophic cardiomyopathy for 13 +/- 6 months (range 2 to 20). The patients had significant symptomatic improvement on verapamil therapy. Murmur intensity diminished in 6 patients during therapy and left ventricular (LV) electromotive forces on the electrocardiogram diminished in 4, increased in 5 and did not change in 4. Exercise endurance increased from 8.4 +/- 3.9 to 10.9 +/- 2.8 minutes (p less than 0.01). Seven patients had ST-segment depression (0.38 +/- 0.28 mV) before verapamil therapy, which improved after verapamil therapy in 5 (0.24 +/- 0.17 mV, p less than 0.02). Of 4 patients with exercise-induced ventricular ectopic activity, 3 had diminution or abolishment of ectopy following verapamil. By echocardiography, the patients had an increase in LV end-diastolic dimension from 3.4 +/- 0.7 to 3.9 +/- 0.8 cm (p less than 0.01), with no significant change in shortening fraction (46.1 +/- 8.0% vs 44.6 +/- 8.0%). When adjusted for body size and age there was a significant decrease in LV septal thickness (from 106 +/- 70 to 45 +/- 52% of predicted normal values, p less than 0.05) and LV posterior wall thickness (from 40 +/- 45 to 5 +/- 26% of predicted normal values p = 0.05) after verapamil. Isovolumic relaxation time decreased from 69 +/- 26 to 42 +/- 19 ms after verapamil (p less than 0.01). Systolic anterior motion of the anterior mitral leaflet disappeared in 5 of 8 patients and midsystolic closure of the aortic valve was no longer present in 4 of 8.(ABSTRACT TRUNCATED AT 250 WORDS)     

The effect of natural and surgically constructed aortopulmonary shunts on hemodynamic function in tetralogy of Fallot.

A hemodynamic model has been developed for the patient with tetralogy of Fallot. Equations representing the circulatory flow pattern and oxygen balance in this disease were incorporated into a computer program. With the computer model it is possible to simulate the effect of natural and surgically constructed aortopulmonary shunt flow on hemodynamic function in tetralogy of Fallot. Graphic representations of the computer output are presented which show how aortopulmonary shunt flow influences the physiology of exercise and hypoxic episodes. An explanation is advanced for the lack of correlation between resting arterial oxygen saturation and the incidence of hypoxic episodes. The model demonstrates the effect of surgical aortopulmonary shunts in eliminating hypoxic spells.     

Value of F-18 FDG hybrid camera PET and MRI in early takayasu aortitis

Takayasu aortitis (TA) is a chronic inflammatory and fibrotic vasculitis of large- and medium-sized arteries. Early stages of the disease show a panarteritis and inflammatory wall thickening of the aorta and its branches, whereas advanced (fibrotic) stages comprise stenosis, aneurismatic transformation and occlusion. Magnetic resonance imaging visualises early-stage disease with high accuracy and is considered to be the method of choice in the diagnosis of TA. The aim of this article is the detailed comparison of FDG-PET performed with a hybrid camera and MR imaging in five patients with early TA. Five patients (median age 60 years) were enrolled during an ongoing prospective study on [18F]2'-deoxy-2-fluoro-D-glucose (FDG) hybrid camera PET in patients with fever of unknown origin (FUO). These patients underwent MR imaging after establishing the diagnosis of TA. Abnormal FDG uptake in the wall of the aorta was noted in all patients. The bracheocephalic artery and the common carotid arteries were visualized in 3 cases. Increased uptake of the subclavian artery was found in 3 patients and in 4 patients pathological uptake was noted in the ilio-femoral vessels. Of 34 vascular regions studied, 26 (76%) showed elevated FDG uptake. On transversal MR images vessel wall thickening and contrast enhancement of the thoracic aorta was found in 4 patients (ascending aorta/aortic arch: n=2; descending aorta: n=3; abdominal aorta: n=1). Additionally, vessel wall pathologies of the subclavian and the common carotid arteries could be shown in 1 patient and in another patient in the ilio-femoral arteries. No abnormalities were found using contrast-enhanced MR angiography. Of 28 vascular regions studied, 9 (32%) showed vasculitis on MRI. The FDG-PET is a suitable whole-body screening method in the primary diagnosis of early TA, especially in those cases with early disease that present with uncharacteristic symptoms such as FUO. Both MRI and MRA remain indispensable in the exact determination of the pathomorphological changes and in the documentation of complications such as stenosis, aneurismatic transformation and occlusion. Electronic Publication     

Prognostic value of positron emission tomography in the evaluation of post-treatment residual mass in patients with Hodgkin's disease and non-Hodgkin's lymphoma

The prognostic value of 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) in the assessment of post-treatment residual masses in patients with Hodgkin's disease (HD) or non-Hodgkin's lymphomas (NHL) was evaluated. We prospectively studied 58 patients with HD (n = 43) or NHL (n = 15) who had post-therapeutic complete remission with residual masses (CRu) indicated by computerized tomography. Analysis of 62 residual locations by FDG-PET was performed separately for HD and NHL. Patients with a PET-positive residual mass [standardized uptake value (SUV) > 3] had a recurrence rate of 62.5% (5/8 patients), whereas patients with PET-negative residual mass (SUV < or =3.0) showed a recurrence rate of 4% (2/50 patients, P = 0.004). A positive FDG-PET study correlated with a significantly poorer progression-free survival (P < 0.00001). No recurrence occurred in any of the 39 HD patients with a negative PET scan (negative predictive value, 100%). Four out of four NHL patients with a positive PET study relapsed (positive predictive value, 100%). In conclusion, FDG-PET is a suitable non-invasive method with a high degree of accuracy in the prediction of early recurrence in lymphoma patients with CRu.     

E2F-1和survivin蛋白在非小细胞肺癌中的表达及其与HPV感染的相关性研究

背景与目的:人乳头状瘤病毒(human papillomavirus,HPV)与人类多种恶性肿瘤密切相关,而肺癌中亦有HPV的检出引起了众多学者的关注.本实验旨在研究非小细胞肺癌(non-small cell 1ung cancer,NSCLC)中高危HPV感染情况及其与E2F-1和survivin蛋白表达的关系,初步探讨HPV感染可能导致.NSCLC发生、发展的分子机制.方法:特异型PCR检测47例NSCLC和13例肺良性病变组织中是否有HPV16、18DNA存在:免疫组化SP法检测E2F-1和survivin蛋白在肺病变组织中的表达.结果:NSCLC组HPV16、18DNA检出率为42.55%(20/47),明显高于肺良性病变组的7.69%(1/13,P＜0.05).NSCLC组中HPV的感染率与患者的吸烟史、组织学类型及分化程度有关(P＜0.05),而与患者的年龄、性别及淋巴结转移无关(P＞0.05).NSCLC组中E2F-1蛋白表达阳性率为59.57%(28/47),其中64.29%(18/28)呈高表达,显著高于肺良性病变组23.08%(3/13,P＜0.025).NSCLC中survivin蛋白表达阳性率为61.70%(29/47),而肺良性病变组无表达.NSCLC组中E2F-1、survivin蛋白表达呈正相关(r=0.3151,P＜0.05),且两者在HPV阳性组的阳性表达率分别为85.00%(17/20)和80.00%(16/20),均显著高于HPV阴性组的40.74%(11/27)和48.15%(13/27),差异均有统计学意义(P＜0.05).结论:HPV16、18感染在NSCLC的发生中可能有病因学意义,其致癌机制可能与E2F-1和survivin蛋白表达上调有关.     

Benign Schwannoma Mimicking Metastatic Lesion on F-18 FDG PET/CT in Differentiated Thyroid Cancer

We report a case of benign schwannoma mimicking metastatic carcinoma. A 55-year-old female with papillary thyroid carcinoma underwent total thyroidectomy. F-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) demonstrated a focal hypermetabolic lesion with maximum standardized uptake value (SUVmax) 5.3 at the right chest wall. Conventional chest CT demonstrated a 5.4 cm ovoid mass lesion between the intercostal muscles and liver. Pathology revealed a schwannoma by tumor excision. This case demonstrates that benign schwannoma may demonstrate FDG uptake mimicking metastatic carcinoma.     

p14ARF、E2F-1与肺癌

p14ARF基因和核转录因子E2F-1在细胞周期调控,细胞凋亡和肿瘤发生中具有重要作用。近年来,有研究表明p14ARF在肺癌中的表达率低于正常组织,E2F-1的表达率高于正常组织。本文将就p14ARF、E2F-1与肺癌的关系进行综述。