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.     

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

ＲＡＤＩＯＦＲＥＱＵＥＮＣＹＣＵＲＲＥＮＴＣＡＴＨＥＴＥＲＡＢＬＡＴＩＯＮＯＦＴＨＥＬＥＦＴＡＴＲＩＯＶＥＮＴＲＩＣＵＬＡＲＡＣＣＥＳＳＯＲＹＰＡＴＨＷＡＹＳＷＩＴＨＰＡＲＯＸＹＳＭＡＬＳＵＰＲＡＶＥＮＴＲＩＣＵＬＡＲＴＡＣＨＹＣＡＲＤＩＡ￥Ｗａｎｇ...     

马来西亚寒邪致病及其证治初探

根据第一作者在马来西亚从事中医临床工作50余年的经验,以中医理论为指导,探讨了在炎热的马来西亚,寒邪致病的可能性、客观性以及寒邪致病的病因、病机、临床表现特征及其治疗方法。     

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.     

DOWN－REGULATION OF C－MYC ONCOGENE DURING NGF－INDUCED DIFFERENTIATION OF NEUROBLASTOMA CELL LINES

ＤＯＷＮ－ＲＥＧＵＬＡＴＩＯＮＯＦＣ－ＭＹＣＯＮＣＯＧＥＮＥＤＵＲＩＮＧＮＧＦ－ＩＮＤＵＣＥＤＤＩＦＦＥＲＥＮＴＩＡＴＩＯＮＯＦＮＥＵＲＯＢＬＡＳＴＯＭＡＣＥＬＬＬＩＮＥＳＣｈｅｎＪｉｅ（陈杰），ＬｉｕＴｏｎｇｈｕａ（刘彤华）ａｎｄＡｌｏｎｚｏＨＲｏ...     

颌面部髓外浆细胞瘤

髓外浆细胞瘤为少见疾病,发生在颌面部软组织者更少见。本文结合一典型病例的临床诊治过程,分析研究其发病原因,临床及病理分型和诊断方面存在的问题。提出颌面部髓外浆细胞瘤良性及恶性其主要临床特征和有关的诊断依据。治疗仍多采用综合方法。恶性者放疗总剂量多在5000Gy以上,化疗以COP,COAP、COAAP方案为主,散在病损可行激光治疗效果良好。     

Irritable bowel syndrome: The role of psychosocial factors

The irritable bowel syndrome (IBS) is a very common disorder of intestinal function characterized by variable symptoms of abdominal pain, diarrhea, constipation and bloating. The spectrum of severity ranges from mild symptoms, not worthy of clinical attention, to intense and continuous symptoms associated with impaired health-related quality of life and high health care use. Psychosocial disturbance is commonly found among patients with irritable bowel, but until recently, its precise role in the disorder was uncertain. The data now suggest that irritable bowel is not a psychiatric disorder per se, but psychosocial factors including life stress, psychiatric comorbidity and abnormal illness behavior, among others, influence how the illness is experienced and acted upon. Patients with mild symptoms usually respond to education, reassurance, dietary modification and, when needed, antimotility agents. However, patients with more severe symptoms, who usually have greater psychosocial disturbance, will also require behavioral interventions, and possibly psychopharmacological agents. In sum, a graduated, multicomponent plan of care that includes dietary, behavioral and pharmacological treatments is recommended.     

跟骨撕脱性骨折的分类和治疗

作者将5个足标本人为造成跟骨撕脱性骨折,对其受伤机制和X线检查特点进行观察,通过22例跟骨撕脱骨折病人的临床研究,对跟骨撕脱骨折根据受伤机制和撕脱部位进行了分类。提出了各类骨折的X线投照方法和意义,也提出了各类骨折的最佳治疗方法。