新生儿阵发性血红蛋白尿临床分析

目的 探讨新生儿阵发性睡眠性血红蛋白尿（PNH）的临床及实验室检查特点。方法总结我院收治的3例新生儿PNH的病例资料，从临床，血象，溶血检查方面及红细胞，粒细胞CD55／CD59的表达检测等方面分析其特点。结果 新生儿PNH临床表现为新生儿期黄疸持续不退、面色苍白、进行性贫血，典型的酱油色样尿不易发现。3例患儿红细胞CD55表达均有缺陷，2例红细胞CD59表达缺陷，1例进行粒细胞CD55／CD59检测，发现亦有表达缺陷，诊断为PNH。结论 新生儿PNH极为少见，由于其临床表现不同于成人，不易发现，而通过血细胞CD55／CD59表达的检测，可明确诊断该病。因此，临床上在怀疑溶血病时，同时需考虑PNH的可能，利用目前先进的诊疗技术，从PNH的发病机制出发，积极进行血细胞CD55／CD59表达的枪测，以早期明确诊断新生儿PNH，以免漏症、误症。     

CD55、CD59、CD34对PNH早期诊断研究应用

目的探讨CD55、CD59、CD34抗原对阵发性血红蛋白尿(PNH)的早期诊断的价值,寻找PNH早期诊断敏感指标进而达到早期发现、早期诊断、早期治疗的目的。方法应用流式细胞仪测定PNH综合症患者的CD55、CD59、CD34抗原变化关系,并与PNH、AA等疾病组以及正常对照组CD55、CD59、CD34抗原相互变化关系,经过统计学处理,找出相关性。结果结果表明PNH CD55、CD59、CD34抗原较AA及其他疾病组及正常对照组明显减低,并与溶血程度呈正相关,且早于其他溶血指标。结论CD55、CD59、CD34抗原表达率可做PNH早期诊断最敏感指标,并与预后转归密切相关。     

间隔补铁大鼠的肠粘膜铁含量及其生物利用率

雄性SD断奶大鼠32只,随机分为缺铁和铁正常两组。喂纯合成饲料,铁含量分别为0与0.8mg,每日两次喂饲。12天后再各分为每日补铁组与间隔三日补铁组,铁剂量每日与每间隔三日均为8mg,同前方式两次喂饲。于第一次补铁时喂入~(59)Fe 3.7×10~4Bq。18天后杀死大鼠,对十二指肠与小肠各段粘膜铁、肠腔遗留铁及其~(59)Fe(cpm)进行检测,并计算出~(59)Fe利用率。结果表明,每日补铁组肠粘膜与肠腔遗留铁分别为铁正常对照组的8与20倍。~(59)Fe利用率为其1/2。间隔补铁组肠粘膜铁接近铁正常对照组,~(59)Fe利用率为其2倍,肠腔内遗留铁仅为每日补铁组1/5。间隔外铁组补铁剂量小,副作用明显少于每日补铁组,肠粘膜铁吸收与利用比较理想。     

Pathological study on the relationship between C4d, CD59 and C5b-9 in acute renal allograft rejection

Abstract:  In order to evaluate the activation or inhibition of the later phases of classical complement cascade in renal allograft presenting with acute rejection, particularly with C4d deposition on the peritubular capillary (PTC), we observed the expression of CD59 and C5b-9 on the PTC. Subjective cases were divided into two groups, an acute rejection group, of 4 males and 6 females, and a normal donor group, of 5 males and 5 females. Renal biopsies were performed at the onset of acute rejection and at the transplant operation, before reperfusion. C4d deposition on PTC was found in three of 10 cases (30%) with biopsy proven acute rejection, whereas CD59 on PTC was positively expressed in all of the rejection cases. Although C5b-9 was not observed on PTC in the acute rejection group, it was intensively deposited on the tubular basement membrane (TBM) in five cases, including the three with positive C4d on PTC. In the normal donor group, CD59 on PTC was intensively observed, whereas C5b-9 was weakly expressed on TBM. CD59, a complement regulatory factor, works as an inhibitory factor against the formation of C5b-9, a membrane attack complex. From our data, we noted the dissociation between the depositions of C4d and C5b-9 on PTC. The substantially expressed CD59 on PTC may affect this dissociation between C4d and C5b-9 on PTC. The intensive deposition of C5b-9 on TBM in acute rejection cases may suggest an independent immunological injury attacking tubular cells.     

Killing of rat glial cells by complement: Deficiency of the rat analogue of CD59 is the cause of oligodendrocyte susceptibility to lysis

In an effort to understand the mechanisms of complement-mediated injury of the myelin/oligodendrocyte complex in demyelinating disease, we have examined the lytic susceptibility of rat glial cells in culture. It is known that rat oligodendrocytes are extremely sensitive to the lytic action of autologous complement, whereas other cells in the same culture system, including type II astrocytes which derive from the same progenitor cell, are relatively insensitive. Here we demonstrate that the complement sensitivity of oligodendrocytes is associated with a lack of expression of a complement-regulatory protein, the rat homologue of human CD59, and that complement resistance can be restored by the incorporation of purified rat CD59 into the cell membrane. Furthermore, neutralisation of rat CD59 on complement-resistant astrocytes renders them susceptible to lysis. Immature oligodendrocytes were resistant to complement attack yet did not express CD59, suggesting that a complement-activating factor appears on the membrane during oligodendrocyte maturation.     

Further chromosome studies on Wilms' tumor cells of patients without aniridia

We studied chromosomes in Wilms' tumor cells of two patients without aniridia who had a normal constitutional karyotype. In both tumors, trisomy for 1q occurred as the result of a t(1;16), although the breakpoints in each chromosome differed in the two tumors. No 11p rearrangements could be detected, whereas in our previous patient an interstitial deletion of 11p13 was present in all tumor cells. Thus, trisomy for 1q may be another pathway leading to the development of Wilms' tumor, although the effect of the deletion of 16q cannot be assessed at present.     

Evaluation of the nissen antireflux procedure by esophageal manometry and twenty-four hour pH monitoring

Fifteen normal volunteers without symptoms of gastroesophageal reflux and sixteen patients with symptoms of gastroesophageal reflux unresponsive to medical management and having endoscopic esophagitis had esophageal manometry and twenty-four hour pH monitoring of the distal esophagus. The symptomatic patients underwent a Nissen antireflux procedure and were restudied at four months. After surgery, patients had less reflux, a higher sphincteric pressure, and an equal amount of sphincter within the abdomen as did asymptomatic control subjects.     

Polypoid sarcomas of the esophagus. A rare but potentially curable neoplasm.

Five patients with polypoid esophageal sarcoma are reported. All had dysphagia similar to that occurring in epithelial carcinoma of the esophagus. The clinical diagnosis of sarcoma was suspected when barium swallow showed a large polypoid lesion. Biopsy was often inconclusive concerning the nature of the lesion except to identify it as a neoplastic process. In spite of their large size, the tumors remained superficial within the esophageal wall. Nodal or distant organ metastasis was absent in 4 of the 5 patients. The histology of these tumors suggests that so-called carcinosarcoma is an epithelial carcinoma of the esophagus with varying amounts of spindle cell features and should be considered separately from sarcoma arising from mesenchymal tissue. Unlike carcinoma, esophageal sarcoma has a favorable prognosis following radical resection, and recent advances in esophageal surgery have decreased the mortality and morbidity of esophagectomy.