乙型肝炎病毒免疫逃避株表面抗原决定簇编码区的序列分析

应用聚合酶链反应（ＰＣＲ）产物直接测序，在国内首次发现１例持续高滴度乙型肝炎表面抗原（ＨＢｓＡｇ）和抗一ＨＢｓ共存者携带的乙型肝炎病毒ＤＮＡ第５３２位碱基Ａ被Ｇ取代，推导其“ａ”决定簇中第１２６位苏氨酸被丙氨酸取代，提示该株“ａ”决定簇第一个结构环疏水性增加，由此可能导致其抗原性改变，而诱发免疫逃避株形成。     

Tourette综合征的神经心理和血浆催乳素含量及其关系的初步研究

本研究采用中国修订的韦氏儿童智力量表（ＷＩＳＣ－ＣＲ）、韦氏儿童记忆量表（ＷＭＳＣ－ＣＲ），艾森克个性问卷（ＥＰＱ）和Ａｃｈｅｎｂａｃｈ儿童行为量表（ＡＣＢＣ），对３９例（未用药组２２例，用药组１７例）７～１４岁Ｔｏｕｒｅｔｔｅ综合征（ＴＳ）患儿进行测试，同时采用放射免疫方法测定其血浆催乳素（ＰＲＬ）含量，并与１８例正常对照组比较。结果表明：ＴＳ患儿智力测验和记忆测验中的背数、译码和视觉再生分测验量表分低于对照组。其个性测验中的神经质（Ｎ）和精神质（Ｐ）量表Ｔ分高于对照组，掩饰性（Ｌ）量表Ｔ分低于对照组。其ＡＣＢＣ总分高于对照组。用药组的记忆商（ＭＱ）、理解记忆和累加分测验量表分低于未用药组，ＴＳ患儿的血浆催乳素含量高于对照组。相关分析表明血浆催乳素含量与ＡＣＢＣ总分和精神抑制药物如氟哌啶醇和泰必利等因素有关。我们的初步研究提示ＴＳ患儿存在不同程度的神经心理缺陷，催乳素可能参与ＴＳ发病过程，氟哌啶醇和泰必利等精神抑制药物可能对ＴＳ患儿的记忆功能有影响以及可以导致血浆催乳素分泌增加，为ＴＳ的防治干预提供了一定的理论依据。     

Tonic pupil,areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature

Summary Two patients are described with the triad of tonic pupil, hyporeflexia and segmental anhidrosis (Ross syndrome). Only 18 cases of this syndrome have been reported in the literature so far. While tonic pupil and reduced sweating can be attributed to the affection of postganglionic cholinergic parasympathetic and sympathetic fibres projecting to the iris and sweat glands, respectively, the pathogenesis of diminished or lost tendon jerks remains obscure. To identify the characteristic clinical features, the previous cases of Ross syndrome are reviewed. Recent evidence of subclinical disturbances of sweating in most patients with Adie's syndrome, i.e. tonic pupil and areflexia, casts doubt on the nosological concept of Ross syndrome as a distinct clinical entity.     

Experience with the International League Against Epilepsy Classifications of Epileptic Seizures (1981) and Epilepsies and Epileptic Syndrome (1989) in Epileptic Children in a Developing Country

Four hundred eighty-three epileptic children attending the Pediatric Epilepsy Clinic at Bai Jerbai Wadia Hospital for Children, Bombay, India were classified according to the International League Against Epilepsy (ILAE) classification of epileptic seizures (1981) and epilepsies and epileptic syndromes (1989). The predominant seizures were partial (53.6), generalized (40.3%), and unclassifiable (6%). In epilepsies and epileptic syndromes, 55.3% were partial, 27% were generalized, 13.5% were undetermined, and 4.1% were special syndromes. Although our results were similar in many respects to those of other reported series, some differences were observed in the incidence of partial and generalized seizures, and partial and generalized epileptic syndromes and their subgroups, such as idiopathic, symptomatic, and cryptogenic partial syndromes, idiopathic generalized syndromes, and symptomatic specific syndromes. These differences are probably due to different age limits, methods of case ascertainment and inclusion criteria, different genetic and environmental factors, variable interpretation of clinical and EEG features, and lack of facilities for investigation in developing countries. Despite various limitations, we were able to classify most cases; the ILAE classification can be used in developing countries so that comparison can be made with other studies.     

Treatment of chronic hepatitis B

SUMMARY. Chronic infection with the hepatitis B virus (HBV) is a major cause of worldwide morbidity and mortality. A large number of therapeutic approaches has been tried, including interferon (IFN), nucleoside analogues and immunomodulators. To date controlled clinical trials have shown that only IFN is of long-term value but many patients fail to respond to treatment. New approaches to treating patients with IFN-resistant hepatitis B are currently undergoing clinical and experimental evaluation, and it seems likely that new therapeutic agents will be available in the near future.     

Detection of Epstein Barr virus in an hepatic leiomyomatous neoplasm in an adult human immunodeficiency virus 1-infected patient

We report the first case of a human immunodeficiency virus (HIV)-related primary hepatic leiomyoma in an adult patient. The diagnosis was made at autopsy and confirmed by immunohistochemistry. Epstein Barr virus (EBV) was identified in tumour cells by in situ hybridization. Review of the literature revealed 13 cases of visceral myogenic tumours occuring in acquired immunodeficiency syndrome children, and only 2 cases in adults. One was a spinal epidural leiomyoma, the other multiple smooth muscle tumours of the colon and adrenal gland. This is the first report of EBV in smooth muscle neoplastic cells in an HIV-infected adult patient.     

RADIOFREQUENCY CURRENT CATHETER ABLATION OF THE LEFT ATRIOVENTRICULAR ACCESSORY PATHWAYS WITH PAROXYSMAL SUPRAVENTRICULAR TAC

ＲＡＤＩＯＦＲＥＱＵＥＮＣＹＣＵＲＲＥＮＴＣＡＴＨＥＴＥＲＡＢＬＡＴＩＯＮＯＦＴＨＥＬＥＦＴＡＴＲＩＯＶＥＮＴＲＩＣＵＬＡＲＡＣＣＥＳＳＯＲＹＰＡＴＨＷＡＹＳＷＩＴＨＰＡＲＯＸＹＳＭＡＬＳＵＰＲＡＶＥＮＴＲＩＣＵＬＡＲＴＡＣＨＹＣＡＲＤＩＡ￥Ｗａｎｇ...     

Early pathological changes in progressive multifocal leukoencephalopathy: a report of two asymptomatic cases occurring prior to the AIDS epidemic

Serial sections of formalin-fixed, paraffinembedded blocks from two asymptomatic, non-AIDS cases of progressive multifocal leukoencephalopathy (PML) were stained with a double-label immunocytochemical method for detection of glial fibrillary acidic protein and JC virus (JCV) capsid proteins and with luxol fast blue/hematoxylin-eosin. In case 1 small, rounded lesions of about 1-mm diameter were seen within a restricted area in the posterior part of the superior frontal gyrus of both cerebral hemispheres, suggesting an early manifestation of the disease. Fully developed demyelinated lesions of the classical type with JCV-infected oligodendrocytes appeared in the white matter and along its border with the cortex. Lesswell-developed lesions, believed to be precursors to the fully developed ones, were seen in the gray and white matter. Of special interest were areas which contained small collections of enlarged, glial fibrillary acidic protein (GFAP)-positive astrocytes without capsid antigen and which seemed to lack destruction of myelin as judged from the appearance of matching serial sections stained for myelin. Large lesions in the brain of case 2 showed the well-known features of advanced PML. The close relation between some astrocytes and oligodendrocytes with viral antigen raises the possibility of early intercellular passage of virus. Vacuolation, seen within or near lesions in both cases, has previously been noted in the CNS infected by HIV, but not in PML. It is suggested that PML, a disease of both oligodendrocytes and astrocytes, may actually begin in astroglial cells which, under the influence of a restricted JCV infection, become reactive, express GFAP and pass on virus to the more highly susceptible oligodendrocytes with which they are in contact.Supported in part by a grant N.S.07596 from the National Institute of Neurological Disorders and Stroke. The work was carried out in the Laboratory of Experimental Neurophathology, NINDS, and in the Department of Pathology II, Karolinska Institute, Stockholm     

Hypothalamic-pituitary-gonadal function in two infants with Smith-Lemli-Opitz syndrome

We report on the hypothalamic-pituitary-gonadal function in 2 male infants with the Smith-Lemli-Opitz (SLO or RSH) syndrome. Both infants had abnormal external genitalia. Basal and LHRH stimulated plasma gonadotropins were normal for age (1 month). Plasma testosterone, androstenedione, and dehydroepiandrosterone sulfate were normal for age and sex. Some forms of congenital adrenal hyperplasia (17,20-desmolase deficiency, 17α-hydroxylase deficiency, and 3β-hydroxysteroid dehydrogenase deficiency) were ruled out by hormonal studies. The endocrinological findings indicate a normal hypothalamic-pituitary-gonadal function and a normal adrenal steroid biosynthesis in these 2 patients. A partial androgen receptor defect causing the genital malformations seems possible in one patient. Whether 5α-reductase deficiency is the cause of the male pseudohermaphroditism in SLO syndrome remains the subject of future studies. © 1992 Wiley-Liss, Inc.     

Liver transplantation for Budd-Chiari syndrome: A retrospective study

A retrospective study was performed on 11 patients who underwent orthotopic liver transplantation for Budd-Chiari syndrome (BCS), 3 of whom had fulminant type BCS and 8, chronic type BCS. Both the 3- and 5-year actuarial survival rates were 64%, after one patient with fulminant, and three with chronic disease died of sepsis or multiple organ failure following transplantation. Anticoagulation therapy in the early postoperative period was tailored to each individual patient. Most of the patients received heparin for several days and were then converted to Coumadin therapy, although some were not given heparin in the immediate postoperative period but were instead commenced on oral Coumadin after the prothrombin time had recovered to wihtin the normal range. All the long-term survivors had received Coumadin therapy and there was no recurrence of BCS and no early thrombotic or hemorrhagic event. One patient developed late thrombosis of the portal vein despite having received apparently adequate Coumadin therapy. It was thus concluded that liver transplantation is an effective therapy for both fulminant and chronic BCS, and that immediate postoperative heparinization is not mandatory for all patients.