Neuroimaging of Focal Cortical Dysplasia

Focal cortical dysplasia (FCD) is a common cause of pharmacoresistant epilepsy that is amenable to surgical resective treatment. The identification of structural FCD by magnetic resonance imaging (MRI) can contribute to the detection of the epileptogenic zone and improve the outcome of epilepsy surgery. MR epilepsy protocols that include specific T1 and T2 weighted, and fluid-attenuated inversion recovery (FLAIR) sequences give complementary information about the characteristic imaging features of FCD; focal cortical thickening, blurring of the gray-white junction, high FLAIR signal, and gyral anatomical abnormalities. Novel imaging techniques such as magnetic resonance spectroscopy (MRS), magnetization transfer imaging (MTI), and diffusion tensor imaging (DTI) can improve the sensitivity of MR to localize the anatomical lesion. Functional/metabolic techniques such as positron emission tomography (PET), ictal subtraction single photon emission computed tomography (SPECT), functional MRI (fMRI), and magnetic source imaging (MSI) have the potential to visualize the metabolic, vascular, and epileptogenic properties of the FCD lesion, respectively. Identification of eloquent areas of cortex, to assist in the surgical resection plan, can be obtained non-invasively through the use of fMRI and MSI. Although a significant number of FCD lesions remain unidentified using current neuroimaging techniques, future advances should result in the identification of an increasing number of these cortical malformations.     

骨纤维异常增殖症生长方式的影像研究

目的 探讨骨纤维异常增殖症起始部位与生长方式。分析其影像学特异性征象。方法 对13例经手术病理证实的骨纤维异常增殖症之CT/MR影像进行回顾性分析并作献复习。结果 病灶成像以纤维组织为主多局限于皮质内生长；病灶成份以编织骨为主。易穿入髓腔弥漫生长。结论 病变起始骨皮质，按病理组织学不同成份。决定其生长方式。CT/MR影像显示起始部位与发展趋势，具有特殊定性诊断价值。     

Success determining factors in percutaneous transluminal balloon valvuloplasty of pulmonary valve stenosis

Twenty-two percutaneous transluminal balloon valvuloplasty procedures were performed on 21 patients with congenital pulmonary valve stenosis. The peak systolic pressure gradient was immediately reduced from 79.1 +/- 7.4 to 22.2 +/- 1.8 mmHg, (P less than 0.0001) and follow-up cardiac catheterization at 5.3 +/- 0.4 months in 19 patients revealed no further significant change in gradient (23.5 +/- 3.2 mmHg). The best results were obtained when balloons larger than the pulmonary annulus were used, i.e. an immediate residual transvalvular gradient of 22.0 +/- 2.2 mmHg with a balloon/annulus ratio greater than 1, as opposed to 44.2 +/- 5.4 with a balloon/annulus ratio = 1 (P less than 0.001). The angiographically determined cusp thickness of the stenotic pulmonary valves was significantly greater than that of the control group of 24 patients without pulmonary valve stenosis (1.21 +/- 0.09 vs 0.59 +/- 0.02 mmHg, P less than 0.00001). The relationship between this parameter and the residual transvalvular gradient at follow-up was found to be significant (r = 0.77, P less than 0.001). It is concluded that balloon size is a determinant factor in achieving good results with percutaneous balloon valvuloplasty although cusp thickness, a factor to which scant regard has hitherto been paid, also plays a significant role in the residual transvalvular gradient measured at follow-up.     

Pathology of the lung in the fetus and neonate, with particular reference to problems of growth and maturation

The major forms of lung pathology in the perinatal period are reviewed with emphasis on disturbances of growth and maturation. Lung hypoplasia results from impairment in the physiological control of lung growth during the fetal period. It is more common than organogenetic defects which are discussed only briefly. Hyaline membrane disease is now seldom seen in a pure form due to improvements in perinatal care. However, its complications and sequelae such as interstitial emphysema, pneumothorax and bronchopulmonary dysplasia are encountered more frequently. In addition, a wide variety of pathological processes may localize to, or be expressed in, the lung of the newborn, notably asphyxial changes, persistent pulmonary hypertension, haemorrhage and infection.     

胃粘膜癌变初发点及其组织背景的病理学：癌变初发点的一般组织病理学

本文通过对228例各种类型胃粘膜上皮异型增生的病理切片的复查,发现并详细地描述了不同类型异型增生腺管和上皮细胞癌变的初发点(Start point)。其形态有的是从再生腺管的腺颈部或底部以“发芽”形式,向质内浸润并分枝生长;有的是从再生腺管基底膜向侧方的间质浸润。从再生的胃上皮型异型腺管所出现的癌变多为低分化型腺癌;从肠上皮型异型腺管所发生的癌变则为高分化型腺癌。强调了胃粘膜再生性异型增生有些具有癌前性质。     

胃癌癌前病变细胞动力学研究

应用自显像技术对内镜活检胃肠化生和异型增生上皮进行~3H-TdR 标志,观察标记率及增殖带的变化。异型增生标记率(18.50～19.21)及大肠化生标记率(18.57)较高,与胃癌(19.32)相似,并且其增殖带向粘膜表面及深部移动。这种细胞动力学的变化可能是胃癌癌前病变的基础。     

Biochemical Markers of Bone Turnover in Camurati–Engelmann Disease: A Report on Four Cases in One Family

Moderate increases in ``classical' biochemical markers of bone turnover have been described only in some patients with Camurati–Engelmann disease. However, the determination of the following ``new' markers has not been previously performed: serum osteocalcin (BGP), bone alkaline phosphatase (BAP), carboxyterminal propeptide of type I procollagen (PICP), aminoterminal propeptide of type I procollagen (PINP), tartrate-resistant acid phosphatase (TRAP), telopeptide carboxyterminal of type I collagen (ICTP), urinary pyridinoline (PYR), crosslinked N-telopeptides of type I collagen (NTX), and Crosslaps (CL). Such a determination may improve the evaluation of the disease activity. To evaluate the usefulness of biochemical markers of bone turnover reflecting Camurati–Engelmann disease activity we measured the levels of all these markers in four affected patients. The results were compared with bone scintigraphic indices of disease activity. Except for PICP and TRAP, bone formation and resorption markers were abnormal in all patients and were related to bone scan indices of disease activity. Among the markers of bone formation PINP, BAP, and BGP showed the highest values, whereas NTX and CL were the most sensitive markers of bone resorption. These results suggest that the determination of NTX or CL, and PINP or either BAP and BGP, associated with bone scan evaluation, provides the best assessment of Camurati–Engelmann disease activity. Received: 14 June 1996 / Accepted: 31 December 1996     

Histopathology of the teeth in segmental odontomaxillary dysplasia: new findings

Histological examination of the deciduous teeth in two cases of segmental odontomaxillary dysplasia (SOMD) showed fibrous enlargement of the pulps, an irregular pulp/dentine interface displaying many pseudoinclusions and pulp stones. There were tubular defects in the coronal dentine from pulp horn to cusp tip, an irregular tubular structure to the circumpulpal dentine of the apical half, a focally deficient odontoblast layer and widespread external resorption. Together with the clinical features of unilateral maxillary enlargement, upper alveolar expansion in the distal segment, increased spacing and delayed eruption of the deciduous molars and absence of premolar teeth, these histological appearances allow distinction of this condition from fibrous dysplasia (FD), segmental hemifacial hypertrophy (SHH) and regional odontodysplasia (ROD).     

Aplasia of submandibular salivary glands associated with ectodermal dysplasia.

We describe a 28-year-old white Caucasian man displaying many of the physical signs of ectodermal dysplasia (ED). An unusual finding was his presentation with xerostomia. Salivary gland imaging techniques revealed aplasia of both submandibular salivary glands and relatively small parotids. The case highlights that hypoplasia and aplasia of exocrine glands could be rare features of ED. In the management of ED, early detection of xerostomia is important to limit any potential damage to the already hypodontic dentition.     

胃恶性上皮核仁组成区相关蛋白的研究

用嗜银染色技术对34例良性胃溃疡、40例胃粘膜异型增生、46例胃腺癌组织石蜡切片中的核仁组成相关蛋白(AgNOR)进行研究,25例正常胃粘膜组织作对照。发现对照组、良性溃疡组,异型增生组和腺癌组的细胞核AgNOR平均计数差异有显著性(P<0.01)。恶性细胞中AgNOR的体积、形状及在细胞核内的位置也不相同。作者认为此法有助于区别胃的良性和恶性病变。