家兔日本血吸虫循环抗原及其特异性免疫复合物的动态观察

应用单抗酶联免疫吸附试验（ＥＬｌｓＡ），对日本血吸虫感染兔肠相关蛋白糖阳极循环抗原（ＧＡｓｃＡＰ）及其特异性循环免疫复合物（ＧＡｓｃＡｐ－ＩＣ），进行了动态检测。中度（２００尾蚴／兔）和轻度（５０尾蚴／兔）感染家兔可在感染后第２周和第４周检出ＧＡＳＣＡＰ；第４和第６周检出ＧＡＳＣＡＰ－ＩＣ。ＧＡＳＣＡＰ和ＧＡＳＣＰ－ＩＣ的升降趋势，呈显著的伴随关系。感染兔在接受一次性吡喹酮治疗后，ＧＡＳＣＡＰ和ＧＡＳＣＡＰ－ＩＣ很快阴转，表明日本血吸虫病ＧＡＳＣＡＰ和ＧＡＳＣＡＰ－ＩＣ的检测具有早期诊断和疗效考核意义。     

重症感染并发多系统脏器功能衰竭时凝血和纤溶系统变化的研究

为观察感染性多系统脏器功能衰竭（ＭＳＯＦ）时凝血纤溶系统变化的特点及意义，对６８例不同程度的感染病人做了凝血酶原时间（ＰＴ），活化的部分凝血活酶时间（ＡＰＴＴ）、纤维蛋白原（Ｆｇ）、纤维结合蛋白（Ｆｎ）和Ｄ－二聚体（Ｄ－Ｄｉｍｅｒ）含量、凝血因子Ⅱ、Ⅶ、Ⅹ、ⅩⅡ（ＦⅡ、ＦⅦ、ＦⅩ、ＦⅩⅡ）活性，抗凝血酶－Ⅲ（ＡＴ－Ⅲ）、纤溶酶原（ＰＬＧ）。α２－抗纤溶酶（α２－ＡＰ）、组织纤溶酶原活化物（ｔ－ＰＡ）和纤溶酶原活化物抑制物（ＰＡＩ）活性、血小板（Ｐｌｔ）数量的测定观察。结果表明：重症感染合并ＭＳＯＦ时，ＰＴ、ＡＰＴＴ异常率明显增高，ＦⅡ、ＦⅦ、ＦⅩ、ＦⅩⅡ活性明显减低，Ｐｌｔ明显减少，Ｆｎ含量显著降低，ＡＴ－Ⅲ、ＰＬＧ活性均明显减低，Ｆｇ、Ｄ－Ｄｉｍｅｒ明显增高，ＰＡＩ活性显著升高，ｔ－ＰＡ和α２－ＡＰ活性轻度减低，未合并ＤＩＣ时ＭＳＯＦ的发生率为３８．５％，合并ＤＩＣ时ＭＳＯＦ的发生率为７９．７％（Ｐ＜０．０１）。提示重症感染合并ＭＳＯＦ时凝血、抗凝和纤溶系统机能紊乱，ＤＩＣ为其主要表现之一，其在ＭＳＯＦ的发生发展过程中具有重要作用。     

登革热循环免疫复合物特异性的检测分析

采用ＳＤＳ－聚丙烯凝胶电泳（ＳＤＳ－ＰＡＧＥ）和免疫转印技术对２８例登革热患者血清循环免疫复合物（ＣＩＣ）进行组份分析。结果显示在ＳＤＳ－ＰＡＧＥ电泳图谱上出现２０Ｋｕ、４６Ｋｕ及５７Ｋｕ３条主要条带。免疫识别结果表明登革热ＣＩＣ中存在特异性病毒抗原及ＩｇＧ、ＩｇＡ、ＩｇＭ抗体和补体Ｃ３组份。     

柳氮磺胺吡啶对大鼠乙酸性溃疡性结肠炎肠组织中氧自由基的影响

目的探讨柳氮磺胺吡啶（ＳＡＳＰ）治疗大鼠乙酸性溃疡性结肠炎（ＵＣ）时清除氧自由基（ＯＦＲ）的特性．方法ＳＡＳＰ灌胃治疗大鼠乙酸性ＵＣ后，检测肠组织中的超氧化物歧化酶（ＳＯＤ）、丙二醛（ＭＤＡ）含量，评价其炎症指数，并与生理盐水（ＮＳ）治疗对照组比较．结果ＳＡＳＰ组和ＮＳ组ＳＯＤ含量（Ｕ／ｇ）分别为７９９８±３４４１和６３６４±２４５５．ＳＡＳＰ组和ＮＳ组ＭＤＡ含量（ｎｍｏｌ／ｇ）分别为２１５６±２０８、３５２４±４４８．ＮＳ组和ＳＡＳＰ组炎症指数分别为１６５±５１９、６３０±１２５．ＳＡＳＰ组ＳＯＤ含量显著高于ＮＳ组（７９９８±３４４１对６３６４±２４５５，Ｐ＜００１），ＳＡＳＰ组ＭＤＡ含量明显低于ＮＳ组（２１５６±２０８对３５２４±４４８，Ｐ＜００１）．ＮＳ组炎症指数明显高于ＳＡＳＰ组（１６５±５１９对６３０±１２５，Ｐ＜００１）．结论ＳＡＳＰ为氧自由基清除剂，是治疗溃疡性结肠炎的主要机理之一．     

老年慢性特发性便秘患者结肠动力学变化

目的：探讨老年慢性特发性便秘（ＣＩＣ）患者结肠运行功能变化及其与心理因素的关系,方法：应用不透Ｘ线标记物法及Ｚung抑郁自评量表（ＳＤＳ）及焦虎自评量表（ＳＡＳ）对５９例老年ＣＩＣ患者及３６例老年对照者进行结肠通过时间检查及心理状况分析,结果：（１）老年ＣＩＣ组全结肠及分段结肠通过时间较老年对照组明显延长（Ｐ＜０．０５）,（２）长期服用泻药组的老年ＣＩＣ患者其全结肠及分段结肠通过时间较间断服药和不服药者明显延长（Ｐ＜０．０５）,（３）老年ＣＩＣ患者ＳＤＳ,ＳＡＳ标准总分均明显高于对照组（Ｐ＜０．０５,（４）老年ＣＩＣ患者中结肠通过时间延长组ＳＤＳ标准通过时间延长组（Ｐ＜０．０５）,结论：老年ＣＩＣ患者存在结肠动力学异常,抑郁情绪和焦虑情绪与老年ＣＩＣ患者结肠运行功能异常有关。     

肺心病患者红细胞免疫功能与活性氧关系的研究

目的：探讨肺心病患者红细胞免疫功能。方法：对３０例肺心病急性加重期患者采用化学比色法测定血清超氧化物歧化酶（ＳＯＤ）及丙二醛（ＭＤＡ），用红细胞酵母菌花环法测定红细胞免疫粘附功能。结果：肺心病急性加重期血清ＳＯＤ下降，ＭＤＡ升高，红细胞膜表面的Ｃ３ｂ受体（ＲＢＣＣ３ｂＲ）下降，红细胞免疫复合物（ＲＢＣＩＣ）升高，与健康对照组比较均具有显著差异（Ｐ＜００５，Ｐ＜００１）。直线相关分析：ＳＯＤ与ＲＢＣＣ３ｂＲ呈显著正相关（Ｐ＜００１），ＭＤＡ与ＲＢＣＣ３ｂＲ呈显著负相关（Ｐ＜００１），ＳＯＤ、ＭＤＡ与ＲＢＣＩＣ无线性相关关系。结论：肺心病急性加重期活性氧的增加是引起红细胞免疫粘附功能下降的一个重要原因。     

高血压病人白细胞流变性与细胞粘附分子表达的变化

目的探讨白细胞流变性和细胞粘附分子（ＣＡＭＳ）表达与高血压发生及病情严重程度的关系。方法采用红细胞变形能力测定仪、体外血栓血小板粘附两用仪和酶联免疫吸附法（ＥＬＩＳＡ），检测１４９例高血压病人和１１０例健康人外周血白细胞变形能力（ＬＤ）、白细胞粘附功能（ＬＡＦ）、白细胞ＣＤ１８表达及血清可溶性细胞间粘附分子－１（ｓＩＣＡＭ－１）浓度的变化。结果高血压病人白细胞滤过指数（ＬＦＩ）、白细胞粘附率（ＬＡＲ）、白细胞ＣＤ１８表达和ｓＩＣＡＭ－１浓度均明显增高，与对照组比较差异有极显著性（Ｐ＜０．００１），三期病人各指标之间比较差异也具有极显著性（Ｐ＜０．００１），且以第３期病人各指标增高最明显。高血压病人ＬＡＲ与ＬＦＩ呈正相关（ｒ＝０．５７９，Ｐ＜０．００１）；ＬＡＲ和ＬＦＩ与白细胞ＣＤ１８表达和ｓＩＣＡＭ－１浓度呈正相关（ｒ＝０．６６２～０．８０４，Ｐ＜０．００１）。结论ＬＤ降低、ＬＡＦ及白细胞ＣＤ１８表达和ｓＩＣＡＭ－１浓度增高参与高血压的发生，且与病情严重程度有密切关系。     

谷氨酸脱羧酶抗体和胰岛细胞抗体联合检测对1型糖尿病早期诊断的意义

目的　对９８４ 例糖尿病患者的胰岛细胞抗体（ＩＣＡ） 、谷氨酸脱羧酶抗体（ＧＡＤＡ） 和血清Ｃ肽进行了检测与分析,了解ＩＣＡ 和ＧＡＤＡ 阳性率以及阳性患者的胰岛功能情况,以明确ＩＣＡ 和ＧＡＤＡ检测对１ 型糖尿病早期诊断的意义。方法　采用免疫组化法检测ＩＣＡ,ＥＬＩＳＡ 法定量检测ＧＡＤＡ, 放射免疫法检测血清空腹和餐后Ｃ 肽。结果　ＩＣＡ 和（或）ＧＡＤＡ 阳性（ 阳性组）１６８ 例（１７ ．０％ ） 高于ＩＣＡ 阳性率（１０．５ ％） 和ＧＡＤＡ 阳性率（１２．０ ％ ,Ｐ＜ ０ ．０５） 。成年患者ＧＡＤＡ 阳性率（１０ ．５％ ） 高于ＩＣＡ（７．７ ％ , Ｐ＜ ０ ．０５） , 青少年患者ＩＣＡ 阳性率（４６．５ ％） 高于ＧＡＤＡ（３１．０％ ,Ｐ＜０．０１）。抗体阳性患者的空腹和餐后Ｃ肽〔（３２５ ±３６８）ｐｍｏｌ／Ｌ和（７４５±７６７）ｐｍｏｌ／Ｌ〕明显低于抗体阴性患者〔（７０１ ±４３４）ｐｍｏｌ／Ｌ和（１ ６１４ ±９６７）ｐｍｏｌ／Ｌ,Ｐ＜０ ．００１〕。结论　自身抗体阳性患者的胰岛功能明显低于阴性患者,提示自身抗体阳性患者胰岛功能有明显损伤。在成年糖尿病患者中ＧＡＤＡ检测较ＩＣＡ 更为重要,而在青少年糖尿病患者中则ＩＣＡ阳性     

经鼻持续气道正压通气治疗阻塞性睡眠呼吸暂停综合征的压力水平研究

目的通过全夜多导睡眠图（ＰＳＧ）有关呼吸紊乱和低氧血症的指标，预测阻塞性睡眠呼吸暂停综合征（ＯＳＡＳ）患者所需的经鼻罩持续性气道内正压通气（ＣＰＡＰ）的最佳压力水平（Ｐｍ），并用于指导临床应用。方法采用ＲＨＫ－５５００型多导睡眠监测系统联接ＢｉＰＡＰ（ＳＴ／Ｄ）系统测定１８例ＯＳＡＳ患者经鼻罩ＣＰＡＰ的Ｐｍ，并推导其与呼吸紊乱指数（ＲＤＩ）或血氧饱和度低于９０％时间（ＴＳ９０）的回归方程。采用此回归方程预测Ｐｍ，并对６例（预测组）ＯＳＡＳ患者进行１周治疗（每夜８～１０小时），其结果与采用实测Ｐｍ进行ＣＰＡＰ治疗的１３例（实测组）ＯＳＡＳ患者（同样疗程）相对比。所有患者在治疗前和治疗后１～２天测定ＰＳＧ。结果ＲＤＩ或ＴＳ９０与Ｐｍ呈显著的直线正相关；用预测Ｐｍ治疗ＯＳＡＳ，其症状及ＰＳＧ指标均有显著改善，疗效与用实测Ｐｍ组者差异无显著性。结论提示临床上可根据患者的ＲＤＩ或ＴＳ９０预测ＣＰＡＰ治疗时所需的最佳压力     

DIPASIC对耐INH结核菌株耐药性的研究

耐多药结核病（ＭＤＲ－ＴＢ）的治疗成为目前全世界的一大难题。寻找有效的药物,研究合理的治疗方案是控制这些病例的当务之急。我院自１９９４年引进ＤＩ－ＰＡＳＩＣ（ＤＰＣ）,在ＭＤＲ－ＴＢ的治疗中取得了良好的临床疗效。ＤＰＣ为异胭肼（ＩＮＨ）与对氨基水杨酸钠（ＰＡＳ）化合形成的一种新型抗结核药物,ＤＰＣ与ＩＮＨ间及与ＰＡＳ间是否存在交叉耐药尚无定论,为此,我们自１９９６年起从临床标本中选择了耐ＩＮＨ结核菌株进行了ＤＰＣ耐药性的实验研究。现将结果报告如下：１材料和方法１９９６年１月～１９９８年１２月自本…     

Lymphocyte response to mitogens in progressive systemic sclerosis

Lymphocyte responses to the mitogens phytohemagglutinin P (PHA-P), concanavallin A (Con A), and pokeweed (PWM) were studied in 18 patients with progressive systemic sclerosis (PSS). A subgroup of these patients with multisystem involvement showed a significantly decreased lymphocyte response to both Con A and PWM when compared to normal controls. However those PSS patients with myositis, although having multisystem involvement, had normal lymphocyte response to all three mitogens. PHA-P stimulation was normal in all PSS patients. Antinuclear antibodies, elevated sedimentation rates, and positive latex fixation were noted only in the multisystem disease group with abnormal lymphocyte function.     

Lymphocyte response to mitogens in progressive systemic sclerosis.

Lymphocyte responses to the mitogens phytohemagglutinin P(PHA-P), concanavallin A (Con A), and pokeweed (PWM) were studied in 18 patients with progressive systemic sclerosis (PSS). A subgroup of these patients with multisystem involvement showed a significantly decreased lymphocyte response to both Con A and PWM when compared to normal controls. However those PSS patients with myositis, although having multisystem involvement, had normal lymphocyte response to all three mitogens. PHA-P stumulation was normal in all PSS patients. Antinuclear antibodies, elevated sedimentation rates, and positive latex fixation were noted only in the multisystem disease group with abnormal lymphocyte function.     

Surgical shunts for extrahepatic portal vein obstruction in pediatric patients: a systematic review

BackgroundExtrahepatic portal vein obstruction (EHPVO) causes portal hypertension in noncirrhotic children. Among surgical treatments, it is unclear whether the meso-Rex shunt (MRS) or portosystemic shunt (PSS) offers lower post-operative morbidity and superior patency over time. Our objective was to evaluate long-term outcomes comparing MRS and PSS for pediatric patients with EHPVO.MethodsA systematic review was conducted of articles reporting children undergoing surgical shunts for EHPVO from 1/2000–2/2020. Of 87 articles screened, 22 were eligible for inclusion. The primary outcome was shunt thrombosis and secondary outcomes included non-operative complications, stenosis, and re-operation.ResultsEighteen of 22 studies were of good quality and four had fair quality. Of 461 patients included, 340 underwent MRS and 121 underwent PSS. MRS were associated with a higher rate of post-operative thrombosis when compared to PSS (14.1% vs 5.8%, p = 0.021). There were 40/340 MRS patients (11.8%) that required at least one re-operation for either shunt thrombosis or stenosis, versus 5/121 PSS patients (4.1%), p = 0.019.ConclusionBoth MRS and PSS result in acceptable long-term patency rates, but the more technically demanding MRS is associated with higher post-shunt thrombosis, often requiring further operative intervention. This study suggests that PSS may offer advantages for pediatric patients with EHPVO.     

Longitudinal changes in lung function and respiratory symptoms in progressive systemic sclerosis. Prospective study

Most patients with progressive systemic sclerosis (PSS) exhibit lung involvement. However, the natural history of lung disease in PSS remains poorly defined. To evaluate lung function over time in PSS, a battery of lung function tests were prospectively performed serially between 1973 and 1982 in 61 patients with PSS. Functional indexes of restriction (vital capacity and total lung capacity) and diffusion impairment (diffusing capacity) showed greater-than-expected annual rates of change. Male subjects showed a trend toward faster declines in forced vital capacity, forced expired volume in one second, total lung capacity, and functional residual capacity and a more rapid increase in static recoil pressure at 90 percent of total lung capacity than did female subjects. Nonsmokers had greater rates of decline in total lung capacity and static lung compliance (but not in forced vital capacity or diffusing capacity) and a greater rate of increase in static recoil pressure than did current and former smokers. Level of lung function at initial study visit, age, race, and chlorambucil therapy had no significant effect on the annual rates of change in lung function, whereas longer duration of disease prior to study entry was associated with a slower annual decrease in lung volumes. Between the first and last visits (mean interval 3.1 years, maximum nine years), the frequency of abnormality in pulmonary function test results showed significant change only in the diffusing capacity (60 percent increasing to 82 percent) and static lung compliance (40 percent increasing to 54 percent), whereas the frequency of respiratory symptoms showed little change. These findings indicate an overall indolent progression of PSS-related lung disease, with substantial individual variability.     

藻酸双酯钠对实验性脑缺血大鼠急性期的干预

目的 探讨不同给药时间藻酸双酯钠对局灶性脑缺血再灌注大鼠脑组织神经细胞凋亡的保护机制。方法 经大鼠颈内动脉将一线栓插入右侧大脑中动脉1．5h后再灌注24h制成局灶性脑缺血再灌注大鼠模型。在再灌前30min或再灌注后5h经腹腔给予相同剂量的藻酸双酯钠。流式细胞术测定受损脑组织神经元细胞内钙离子浓度及凋亡率，同时观察大鼠的神经功能障碍评分。结果 藻酸双酯钠治疗组神经功能障碍较非治疗组明显减轻，相应的藻酸双酯钠治疗组细胞内钙离子浓度增高受抑制、细胞凋亡减少。不同给药时间点组间上述指标差异亦有显著性。结论 藻酸双酯钠可以减轻脑组织神经元再灌注损伤，抑制神经元凋亡。抑制受损神经元胞内钙离子浓度增高是其保护作用的可能机制。     

盐析分离皮肤作狼疮带试验在七种结缔组织病中的应用

用传统LBT法和盐析分离皮肤LBT法分别检测SLE106例,BSLE3例,SCLE8例,DLE10例,MCTD15例,PSS11例,DM8例。分离皮肤LBT法阳性率依次为89.6％,1OO％,100％,80％,80％,36％,12.5％;传统LBT法的阳性率依次为50％,100％,75％,80％,33％,18％和0。结果显示分离皮肤LBT法敏感性高于传统LBT法,其中6种疾病的Ig和补体沉积以真皮侧多见,而SCLE患者的Ig和补体在真皮侧与表皮侧的沉积率相等。单独见于表皮侧沉积的只罕见于SLE。Ig和补体以线状沉积比颗粒状多见,而颗粒状沉积者与肾损害关系密切。分离皮肤LBT法方便易行,敏感性高,特异性较强,能更有助于疾病的诊断及鉴别诊断。     

Higher myocardial strain rates during isovolumic relaxation phase than during ejection characterize acutely ischemic myocardium

OBJECTIVES: The aim of this study was to define an index that can differentiate normal from ischemic myocardial segments that exhibit postsystolic shortening (PSS). BACKGROUND: Identification of ischemia based on the reduction of regional systolic function is sometimes challenging because other factors such as normal nonuniformity in contraction between segments, tethering effect, pharmacologic agents, or alterations in loading conditions can also cause reduction in regional systolic deformation. The PSS (contraction after the end of systole) is a sensitive marker of ischemia; however, inconsistent patterns have also been observed in presumed normal myocardium. METHODS: Twenty-eight open-chest pigs underwent echocardiographic study before and during acute myocardial ischemia induced by coronary artery occlusion. Ultrasound-derived myocardial longitudinal strain rates were calculated during systole (S(SR)), isovolumic relaxation (IVR(SR)), and rapid filling (E(SR)) phases in both ischemic and normal myocardium. Systolic strain (epsilon(sys)) and postsystolic strain (epsilon(ps)) were calculated by integrating systolic and postsystolic strain rates, respectively. RESULTS: During ischemia, S(SR), E(SR), and epsilon(sys) in ischemic segments were significantly lower (in magnitude) than in nonischemic segments or at baseline. However, some overlap occurred between ischemic and normal values for all three parameters. At baseline, 18 of 28 animals had negative IVR(SR) (i.e., PSS) in at least one segment. During coronary artery occlusion, IVR(SR) became negative and larger in magnitude than S(SR) in all ischemic segments. The IVR(SR)/S(SR) and epsilon(ps) best differentiated ischemic from nonischemic segments. CONCLUSIONS: In the presence of reduced regional systolic deformation, a higher rate of PSS than systolic shortening identifies acutely ischemic myocardium.     

Alternative equations for whole-body protein synthesis and for fractional synthetic rates of proteins

In a constant infusion study of a mass isotope of leucine, two alternative equations are commonly available to calculate amino acid oxidation rate and, thence, whole-body protein synthesis. One, developed by Matthews et al (Am J Physiol Endocrinol Metab. 1980;238:E473-E479), is shown here to require assuming a tracee steady state (TSS), namely, that tracee (unlabeled) amino acid concentrations and fluxes (rates of oxidation and incorporation into protein) are unaltered compared with the preinfusion state. The other, developed by Garlick and coworkers (Melville et al, Metabolism 1989;38:248-255), stems from a protein steady state (PSS) assumption, namely, that protein synthesis is unaffected by the tracer infusion. We derive here a simple expression for the relative difference in whole-body protein synthesis computed from the two assumptions, and a simple test of the validity of TSS in the form of an equality that must be satisfied by plasma measurements at all times. We also propose two experiments to discriminate between the two assumptions. Theoretical reasons and experimental evidence from the literature are offered to support PSS. The two assumptions result in different expressions for fractional synthetic rates (FSRs) of individual or organ proteins-TSS requires the use of tracer-to-tracee ratios and PSS the use of enrichments. An expression is derived here for the relative difference in FSR with TSS vs PSS. For both whole-body synthesis and for FSR, the TSS assumption consistently results in an underestimate, the relative bias roughly equal to the precursor amino acid enrichment.     

Splenectomy in myelofibrosis with myeloid metaplasia: a single-institution experience with 223 patients

In a 20-year period, 223 patients (median age, 64.8 years) with myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy at our institution. Primary indications for surgery were transfusion-dependent anemia (45.3%), symptomatic splenomegaly (39. 0%), portal hypertension (10.8%), and severe thrombocytopenia (4.9%). Operative mortality and morbidity rates were 9% and 31%, respectively. The 203 survivors of surgery had a median postsplenectomy survival time (PSS) of 27 months (range, 0-155). Among preoperative variables, thrombocytopenia (platelet count less than 100 x 10(9/)L) and nonhypercellular bone marrow were identified as independent risk factors for decreased PSS. Durable remissions in constitutional symptoms, transfusion-dependent anemia, portal hypertension, and severe thrombocytopenia were achieved in 67%, 23%, 50%, and 0% of the patients, respectively. Histologic or cytogenetic features of bone marrow obtained before splenectomy did not predict a response in cytopenias. After splenectomy, substantial enlargement of the liver and marked thrombocytosis occurred in 16.1% and 22.0% of the patients, respectively. The thrombocytosis was associated with an increased risk of perioperative thrombosis and decreased PSS. The rate of blast transformation (BT) was 16.3%, and the risk of BT was higher in the presence of increased spleen mass and preoperative thrombocytopenia. However, the PSS of patients with BT was not significantly different from that of patients without BT. We conclude that presplenectomy thrombocytopenia in MMM may be a surrogate for advanced disease and is associated with an increased risk of BT and inferior PSS. However, the development of BT after splenectomy may not affect overall survival and does not undermine the palliative role of the procedure for the other indications.     

Progressive Systemic Sclerosis in Auckland: A Ten Year Review with Emphasis on Prognostic Features

Abstract: The clinical records of all patients with scleroderma or Raynaud's phenomenon who attended hospitals or specialist practice in Auckland during the ten year period of 1970–79 were examined. Forty-seven patients were found to satisfy the American Rheumatism Association criteria for progressive systemic sclerosis (PSS). Thirteen of these patients had CRST features which comprise calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasiae. All but two of the 23 surviving patients were interviewed. The incidence of PSS was 6.3/million/year and no significant difference in incidence was found between Caucasians and Polynesians. Raynaud's phenomenon was most frequently the presenting symptom. Oesophageal involvement was the commonest clinical visceral manifestation. Cumulative survivaI rates showed that the adverse prognostic features were renal, cardiac and to a lesser extent pulmonary involvement. Patients with the CRST features had a better prognosis and none had renal complications. The natural history of the CRST group was characterised by a long latency between the presenting symptom and the other features of the disease. Therefore, the recognition of the CRST variant was of retrospective rather than predictive clinical value. In this first clinical study of PSS patients in New Zealand, results of therapeutic intervention were disappointing .