狼疮肾炎小鼠肾脏的比较蛋白质组学研究

目的探讨狼疮肾炎(LN)小鼠肾脏和正常小鼠肾脏蛋白质组的差异。方法应用双向凝胶电泳技术(two-dimensional gel electrophoresis,2-DE)分离肾组织总蛋白质,凝胶用银染显色,扫描仪获取凝胶图像并对其进行软件分析。结果同组实验重复3次,其中正常对照组3张图找到蛋白质点平均573±52个,匹配率为83%;LN组找到蛋白质点平均658±43个,匹配率为87%。经软件分析,LN组和对照组比较表达增加大于2倍的点有119个;降低大于0.5倍的点有140个。结论LN小鼠肾脏蛋白质表达发生了显著变化,这可能和LN的发生发展有着密切关系。     

Renal involvement in visceral leishmaniasis

In a prospective study of 50 patients with visceral leishmaniasis, laboratory abnormalities suggestive of renal involvement were not infrequent. Proteinuria and/or microscopic hematuria or pyuria were observed in 51% of such cases. Twenty-four hour urinary protein excretion was elevated in 57% of patients in all cases below 1g/24 hours. An abnormal acid-load test was demonstrated in 12 of 18 patients studied before therapy of the parasitic infection with N-methyl-glucamine. Of interest was the demonstration of tubulo-interstitial involvement in the renal histology of all seven patients studied; also, in five of seven patients there was a proliferative glomerulonephritis, usually mild, on histologic examination. In general, there was a tendency to subsidence of abnormal laboratory findings within one month after specific therapy. Renal involvement in visceral leishmaniasis was mild and seemed to revert with the cure of the leishmanial infection.     

Immunogenetic studies of the platelet-specific antigen P1A1 (Zw(a))

The phenotype frequency of the platelet-specific antigens PI^A1/^A2 (= Zw(a,b)) in the German population (N = 711) is 97.75 % and 2.25 % respectively. The corresponding gene frequencies are PI^A2 0.85; PI^A2 0.15. The PI^A1 determinant is codominantly inherited (19 families; 151 family members). There is a strong gene dosage effect between PI^A1-homoand heterozygous individuals. No definite linkage of the locus PIAl versus the loci HLA-ABC, ABO, or Rh was found.     

Alport's syndrome and achalasia

A 7-year-old boy presented with a history of postprandial vomiting, failure to thrive, hematuria, proteinuria and decreased renal function. Electron microscopy of a renal biopsy specimen demonstrated the typical glomerular basement membrane changes associated with Alport's syndrome. Audiometry revealed a moderate bilateral high-tone sensorineural hearing loss. Bilateral anterior lenticonus and a unilateral cataract were also diagnosed. Achalasia diagnosed radiologically and confirmed by biopsy was corrected by surgery. Evaluations of the parents and three siblings were negative. The patient subsequently developed end-stage renal failure. This case report and a review of the literature suggest that achalasia may be part of Alport's syndrome in some patients.     

Posttransfusion purpura: therapeutic failure of PlAl-negative platelet transfusion

The effect of platelet type-specific transfusion in posttranfusion purpura is reported. Seven days after receiving 4 units of whole blood during total hip replacement a 69-year-old woman developed fulminant thrombocytopenic purpura. Her undiluted serum inhibited the clot retraction of PlAl-positive but not PlAl-negative blood. Anti-PlAl titer of her serum, determined by 51Cr platelet lysis technique, was 1:64. The serum had no lytic activity against platelet-rich plasma from two PlAl-negative donors. No anti-HLA antibody was detectable in the serum by lymphocytotoxicity technique, and serum obtained prior to transfusions had no platelet lytic activity. Four units of PlAl-negative platelet concentrate were administered, the first instance in which this treatment has been used. No rise in platelet count ensued, and the patient succumbed to purpura. Exchange transfusion or plasmapheresis remain the treatments of choice.     

霉酚酸酯联合强的松治疗难治性狼疮性肾炎20例

目的　观察新型免疫抑制剂霉酚酸酯 (MMF)治疗难治性狼疮性肾炎 (RLN)的疗效。方法　采用MMF(10 0 0～ 2 0 0 0mg/d)联合强的松 (10～ 3 0mg/d)口服治疗难治性狼疮性肾炎 2 0例 ,疗程为 3～ 12个月。 结果　MMF +强的松治疗 3个月 ,完全缓解 8例 ,显效 5例 ,有效 3例 ,无效 4例 ,总有效率为 80 %。血清蛋白、2 4h尿蛋白显著减少 (P <0 .0 1)。血尿亦较治疗前明显减少(P <0 .0 1)。结论　MMF联合强的松治疗难治性狼疮性肾炎有较好的近期疗效 ,并有较好的耐受性。     

Autoimmune pancreatitis associated with biliary stricture,immune thrombocytopenic purpura and lung fibrosis

We report an autoimmune pancreatitis case with diverse manifestation of biliary stricture, lung ?brosis and immune thrombocytopenic purpura. The patient was a 70‐year‐old man who presented with jaundice. Abdominal ultrasonography and computed tomography revealed enlargement of the entire pancreas and endoscopic retrograde pancreatography showed diffuse irregular narrowing of the main pancreatic duct. Endoscopic retrograde cholangiography revealed a band‐like stricture of the bile duct in the hilar hepatic region with intrahepatic duct dilation. Chest X‐rays and computed tomography revealed interstitial ?brosis of the bilateral lower lungs. Thrombocytopenia and purpura appeared. Antinuclear antibody, anti‐Ro (SS‐A) and anti‐La (SS‐B) antibodies and rheumatoid factor were positive. All manifestations responded well to steroid therapy. From the present case it would appear that a multitude of manifestations are linked within one syndrome. If we encounter patients with autoimmune pancreatitis, we should not focus on a disease but rather on the possibility of a syndrome complex.     

过敏性紫癜肾炎180例临床分析与研究

本文对儿科医院1956年9月至1983年8月住院的过敏性紫癜肾炎(APN)180例作了临床分析,其临床表现为血尿和/或蛋白尿者占51.1%、肾炎综合征者占43.3%、肾病综合征伴血尿者占5.6%。肾损害与肾外症状无明显关系,用激素治疗与否对近期结局无明显影响,近期临床痊愈40.6%、好转42.2%、无变化16.1%、死亡1.1%。 76例随访11月至26年(平均9年),体检无异常发现。在资料完整的50例中痊愈30%、尿异常24%、尿异常伴轻度氮质血症46%。作肾活检7例,其血清C_3均正常,经光镜、电镜、免疫荧光检查2例呈膜增殖肾炎、5例呈系膜增殖肾炎,未见新月体形成,有IgA沉积者5/6例。本组APN的预后较其它文献报告佳。     

Thrombotic thrombocytopenic purpura: Recovery after plasmapheresis,corticosteroids, splenectomy,and antiplatelet agents

A 68-year-old woman severely ill with thrombotic thrombocytopenic purpura (TTP) made a complete recovery after splenectomy was added to a program of corticosteroids, antiplatelet agents, and plasmapheresis. The authors review the subject of therapy of TTP. They advise high-dose corticosteroids, antiplatelet agents, and plasmapheresis, with splenectomy to be considered as an effective alternative.