Behçet's disease: diagnostic and prognostic aspects of neurological involvement

This study was conducted to describe clinical and prognostic aspects of neurological involvement in Behçet's disease (BD). Patients referred for neurological evaluation fulfilled the criteria of the International Study Group for Behçet's Disease. We analyzed disability and survival by the Kaplan-Meier method, using Kurtzke's Extended Disability Status Scale (modified for BD) and the prognostic effect of demographic and clinical factors by Cox regression analysis. We studied 164 patients; of the 107 diagnostic neuroimaging studies: 72.1% showed parenchymal involvement, 11.7% venous sinus thrombosis (VST) and the others were normal. CSF studies were performed in 47 patients; all with inflammatory CSF findings (n=18) had parenchymal involvement. An isolated increase in pressure was compatible with either VST or normal imaging. The final diagnoses were VST (12.2%), neuro-Behçet's syndrome (NBS) (75.6%), isolated optic neuritis (0.6%), psycho-Behçet's syndrome (0.6%), and indefinite (11%). VST and NBS were never diagnosed together. Ten years from onset of BD 45.1% (all NBS) reached a disability level of EDSS 6 or higher, and 95.7±2.1% of the patients were still alive. Having accompanying cerebellar symptoms at onset or a progressive course is unfavorable. Onset with headache or a diagnosis of VST is favorable. Two major neurological diagnoses in BD are NBS and VST. These are distinct in clinical, radiological, and prognostic aspects, hence suggesting a difference in pathogenesis.     

Detection of carbamyl phosphate synthetase 1 deficiency using duodenal biopsy samples

The activity of urea cycle enzymes was assayed in duodenal biopsy specimens obtained from a female infant who presented with neonatal hyperammonaemia. All enzyme levels were normal except N-acetyl glutamate-dependent carbamyl phosphate synthetase 1 (CPS1) which was half the mean activity in normal control specimens. A similar deficiency of CPS1 was also shown in duodenal specimens from the patient's mother who became slightly symptomatic after relatively high protein meals and during pregnancy, and had spontaneously modified her diet to one with protein restriction. The patient is growing normally on a dietary regimen similar to that spontaneously adopted by her mother. Urea cycle enzyme activity in the duodenal biopsy material from the controls was similar to that found in the normal human liver and appears to have distinct advantages as a means of assaying for urea cycle defects in patients with hyperammonaemia and their relatives.     

儿童癌症幸存者成年后的慢性疾病

由于治疗方法的进步,近80%的儿童和青少年癌症患者能够长期生存。在美国,约有270000例儿童癌症的幸存者,即每640名20至39岁成年人中就有一名幸存者。大量的幸存者有利于儿童癌症治疗后长期健康结果的研究。现在可以明确的是,化疗和放疗所致的儿童各器官系统损害在临床上可能潜伏多年。为了全面了解治疗儿童癌症而继发的健康问题,重要的是衡量三项长期结果:健康状况、死亡率和患病率。这三项中,关于前两项已有相当好的研究报道。在一项对20227例癌症5年生存者的回顾性分析中,Mertens等发现以下原因导致的超额死亡率具有统计学意义:继发癌症(…     

Effects of methanol extracts of Caesalpinia bonducella and Bauhinia racemosa on hematology and hepatorenal function in mice

The aim of the present investigation deals with the hematology and hepatorenal function of Caesalpinia bonducella Flem. and Bauhinia racemosa Lam. belonging to the Family: Caesalpiniaceae, and used in the traditional system of medicine. The tribal people of Kolli Hills, Tamil Nadu, India, use the leaves of Caesalpinia bonducella and the stem bark of Bauhinia racemosa in combination with some other herbs for the treatment of various tumors, liver disorders, inflammation and some other diseases. In ancient Ayurveda medicine these plants were mentioned to possess antitumor agents. Since there are no scientific reports regarding the toxicological aspects of these plants, the present investigation deals with the sub-chronic toxicity studies of a methanol extract of Caesalpinia bonducella (MECB) leaves and Bauhinia racemosa (MEBR) stem bark in Swiss albino mice. The MECB and MEBR were administered intraperitoneally (i.p) to Swiss albino mice twice a week for thirteen weeks. No significant alterations in hematological, biochemical and histopathological parameters were observed in the MECB- and MEBR-treated groups at the doses of 100 and 200 mg/kg body weight. Administration of MECB and MEBR at the dose of 400 mg/kg body weight elevated the levels of serum enzymes and altered the hematological parameters. Our results suggested that MECB and MEBR at doses 100 and 200 mg/kg body weight did not induce any toxic effects in the mice. Adverse effect was noted at the dose of 400 mg/kg body weight.