Chronic renal failure and its treatment in tuberous sclerosis

BACKGROUND.: Chronic renal failure is rare in tuberous sclerosis, but itsprecise frequency is not known and treatment modalities havenot been evaluated. METHODS.: A questionnaire was addressed to the 260 French dialysis centresand the characteristics of 65 patients with tuberous sclerosisand chronic renal failure were analysed. RESULTS.: In France the approximate prevalence of tuberous sclerosis withend-stage renal failure is 0.7 cases per million and that ofend-stage renal failure in tuberous sclerosis 1 per 100. Tuberoussclerosis with chronic renal failure was more frequent in females(63.1%) and was diagnosed at a mean age of 29 years. Renal impairmentwas the first manifestation of tuberous sclerosis in about halfthe cases. Renal tumours were frequent, with angiomyolipomasin 15 cases (23.1%), cysts in 12 cases (18.5%), and both in35 cases (53.8%). Malignancies were associated in nine cases(13.8%). Nephrectomy was done before dialysis in 21 cases (32.3%),and after the start of dialysis in six cases (9.2%). All butone of the 48 patients with end-stage renal failure were treatedby dialysis; 20 were transplanted, with good results. CONCLUSIONS.: Tuberous sclerosis with end-stage renal failure is rare. Thesepatients require dialysis and renal transplantation, but werecommend binephrectomy after starting dialysis and before transplantation,given the risk of cancer and bleeding related to angiomyolipomas.     

Human T cell responses to recombinant mite antigens of Dermatophagoides farinae

We studied T cell responses to four glutathione S transferase (GST)-fused mite antigens prepared in our laboratory using peripheral blood lymphocytes from mite-sensitive patients with bronchial asthma. Of the four recombinant antigens, purified GST-Mag3 had the strongest ability to cause patients' lymphocytes to proliferate, and its potency was almost comparable to that of crude mite bodies (Dfb) and faeces (Dff) antigens and a purified major antigen, Der f 2. The responder lymphocytes were mainly T cells, because the proliferative response was depleted by the treatment of lymphocytes with anti-CD3 antibody and complement, but not with anti-CD20 antibody and complement. The responsiveness of lymphocytes to GST-Mag3 correlated with that to Der f 2, but GST-Mag3 displayed slightly higher activity to stimulate lymphocytes than Der f 2. Simultaneously, the levels of Dff- and GST-Mag3-specific IgE antibodies correlated with the responsiveness of lymphocytes to GST-Mag3. These results suggest that Mag3 is a new valuable antigen for the response of T cell proliferation in mite-sensitive patients.     

Epitope Context and Reshaping of Activated T Helper Cell Repertoire

In recent years, a growing interest in the study of peptide antigenicity in relation to the role of flanking sequences and protein topology in processing, presentation, and recognition has been observed. However, the information available on the antigenicity of recombinant fusion proteins and their effect on the selection of antigen receptor repertoires is limited. To analyze the role of molecular topology of T epitopes in a system relevant to human pathology, we have used the bacterially expressed Schistosoma japonicum glutathione S transferase (GST) to construct recombinant antigens containing HIV-1 derived T cell determinants, and human T cell clones specific for these determinants. We found that antigenicity of a given GST—peptide combination was not the same when T cells and antigen presenting cells from different individuals were tested. Our results show that differences in processing and presentation of chimeric proteins are not dictated by the use of diverse restriction elements. We also found that the context in which an antigenic peptide is delivered affects the recruited repertoire as defined according to T cell receptor Vβ usage and fine specificities of selected T cells.     

Testosterone and androstanediol production by regenerating Leydig cells in the ethylene dimethane sulphonate-treated mature rat

Mature (60-65 day old) male Sprague-Dawley rats received a single intraperitoneal injection of ethylene dimethane sulphonate (EDS; 100 mg/kg) and were subsequently killed at various times from day 2 to day 40 post-treatment. Testes were removed from these animals and age-matched controls and utilized either for light and electron microscopical analyses or for in-vitro assessment of Leydig cell function. Interstitial cells were prepared by collagenase digestion and used to measure 125I-labelled human chorionic gonadotrophin (hCG) binding capacity and androgen production in the presence or absence of hCG or dibutyryl cyclic AMP (dbcAMP). At day 2 after EDS treatment, 125I-labelled hCG binding capacity was reduced to 10% of control values, while the production of testosterone and 5 alpha-androstane-3 alpha, 17 beta-diol (adiol) were non-detectable. Histological observations confirmed the lack of identifiable Leydig cells at day 2-16 after EDS treatment. Between days 24 and 40 post-treatment, Leydig cell regeneration occurred, as indicated by a rise in 125I-labelled hCG binding capacity, increased androgen production and the presence of histologically identifiable Leydig cells. A pattern of adiol production similar to that seen in the immature rat during Leydig cell development was observed with peak synthesis occurring at day 30 post-treatment. Adiol production fell to barely detectable levels by day 36 and remained low at day 40. It is concluded that the steroidogenic pattern of regenerating Leydig cells in the EDS-treated animal is similar to that of developing Leydig cells in the immature animal.     

雷公藤挥发油对小鼠免疫功能的影响

观察雷公藤挥发油对小鼠免疫功能的影响,发现给药1周后,其可减少脾脏有核细胞数,减少 PFC 和 RFC 数目,抑制脾细胞对 ConA 诱导 T 细胞增殖的反应性,减轻DTH。这说明雷公藤挥发油对小鼠体液免疫和细胞免疫均有明显的抑制作用。     

溴脱氧尿苷免疫细胞化学方法显示鸡内耳毛细胞增殖活动

建立溴脱氧尿苷(bromodeoxyuridine,BrdU)免疫细胞化学方法显示增殖细咆.按100mg/kg剂量给鸡腹腔注射DNA合成的前体物质BrdU,6h后处死动物,取小肠组织制备石蜡切片,通过免疫细胞化学方法,成功地显示了小肠上皮细胞中的S期增殖细胞,将此法用于观察鸡内耳感觉上皮细胞的生理性再生状态,结果发现鸡前庭感觉上皮中存在着低水平的增殖活动。BrdU免疫细胞化学方法省时,无需特别防护,且定位准确,是显示增殖细胞的理想方法。     

急速进入高原人员红细胞免疫粘附功能的动态变化研究

本文观察了40名快速进入海拔3600米地区人员的RCR、RICR水平，及同海拔移居适应者36名和世居者30名的上述指标。结果表明：观察组进入高原第2天RCR急剧下降，RICR急剧升高，以后逐渐降低或升高，至第30天时与移、世居两组接近（P＞0．05）；高原反应越重RCR及RICR降低或升高越明显（P＜0．05或P＜0．01）。     

Observations on two members of the Swedish family with congenital dyserythropoietic anaemia,type III

Abstract: Two affected individuals of the Swedish family with CDA, type III, in which the disease is transmitted as an autosomal dominant character, were studied. Both cases displayed features hitherto undescribed in this family but described in patients with CDA, type III, in whom the inheritance may have been as an autosomal recessive character. Such features were: (a) haemosiderinuria, (b) grossly disorganised erythroblast nuclei, (c) differences in the ultrastructural appearances of individual nuclei within the same multinucleate erythroblast and (d) intraerythroblastic inclusions resembling precipitated globin chains. In both cases the giant mononucleate erythroblasts and the multinucleate erythroblasts had total DNA contents up to 28c (1c = haploid DNA content) and 48c respectively, and some DNA synthesising bi- and multinucleate erythroblasts contained one or more nuclei which were unlabelled with ³H-thymidine. These findings are similar to those in patients with the autosomal recessive type of disease. Thus no major phenotypic differences are yet apparent between cases of CDA, type III, with different patterns of inheritance. Analysis of the surface erythrocyte proteins of the 2 Swedish CDA, type III, patients with monoclonal antibodies recognising Band 3, glycophorins A, B, C and D, Rh, CD44, CD47, CD55, CD58, CD59, Lutheran, Kell, LW and acetylcholinesterase did not reveal any gross abnormality of expression of these proteins. A slightly altered expression of blood group antigens A and H was revealed by the lectins Dolichos biflorus and Ulex europaeus and the Mr of Band 3 as judged by SDS polyacrylamide gel electrophoresis was also slightly reduced, suggesting that there may be minor alterations in the degree of N-glycosylation of some red cell membrane constituents.