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Adult hypophosphatasia. 成人低磷酸盐血症。
Pub Date : 2020-02-10 DOI: 10.32388/ag8ws5
E. Sorensen, H. Flodgaard
A case of adult hypophosphatasia under treatment with a high orthophosphate (P1) intake is described. The patient is a 53-year-old woman. Her symptoms have progressed for seven years, and it has been necessary to perform osteosynthesis of both crura. The diagnosis rests upon a characteristic clinical picture, low serum alkaline phosphatase activity, high urinary excretion of phosphoethanolamine, and an invariably elevated concentration of inorganic pyrophosphate (PP1) in plasma accompanied by a very high excretion of this compound in the urine. An improved technique allowed specific determinations of microquantities of PP1 in biologic materials. The concentrations of PP1 in the plasma and urine remained unchanged when the patient's intake of phosphorus was increased to 1.98 g/day. The PP1/P1 ratio in the urine was 10-20 before treatment. During treatment P1 excretion increased. PP1 excretion did not change, and the ratio decreased to around 7. The renal tubular transport of PP1 probably was saturated, and therefore PP1, which was circulating in abnormally high concentrations in the patient's fluids, could not be removed by loading with P1. Four months of treatment did not benefit the patient.
描述了一例在高正磷酸盐(P1)摄入量治疗下的成人低磷酸盐血症。病人是一位53岁的妇女。她的症状已经发展了七年,有必要对两个小腿进行骨合成。诊断基于特征性临床表现,血清碱性磷酸酶活性低,尿中磷酸乙醇胺排泄量高,血浆中无机焦磷酸盐(PP1)浓度始终升高,同时尿液中该化合物排泄量非常高。一种改进的技术允许对生物材料中的微量PP1进行特定测定。当患者的磷摄入量增加到1.98g/天时,血浆和尿液中PP1的浓度保持不变。治疗前尿液中PP1/P1的比例为10-20。治疗期间,P1排泄增加。PP1排泄量没有变化,比率降至7左右。PP1的肾小管转运可能是饱和的,因此在患者体液中以异常高浓度循环的PP1不能通过装载P1来去除。四个月的治疗对患者没有益处。
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引用次数: 1
Thiazide prophylaxis of urolithiasis. A double-blind study in general practice. 噻嗪预防尿石症。一般情况下的双盲研究。
Pub Date : 2009-04-24 DOI: 10.1007/978-1-4684-7272-1_101
E. Lærum, Stig Larsen
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引用次数: 163
Assessment of D-dimer in plasma: diagnostic value in suspected deep venous thrombosis of the leg. 血浆d -二聚体的评估:对疑似下肢深静脉血栓的诊断价值。
Pub Date : 2009-04-24 DOI: 10.1016/0268-9499(88)90432-8
P. Ott, L. Astrup, R. Jensen, B. Nyeland, B. Pedersen
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引用次数: 53
Plasma and skeletal muscle electrolytes in patients on long-term diuretic therapy for arterial hypertension and/or congestive heart failure. 长期利尿剂治疗动脉高血压和/或充血性心力衰竭患者血浆和骨骼肌电解质的变化
Pub Date : 1988-10-01 DOI: 10.1097/00132586-198810000-00008
T. Dyckner, P. Wester
Investigations regarding plasma and skeletal muscle electrolytes were carried out in 537 patients on long-term diuretic treatment (greater than 1 year) for arterial hypertension (n = 240) and/or congestive heart failure (n = 297). In both groups there were significant decreases in both plasma and skeletal muscle K and Mg, while the muscle Na values as well as the total and extracellular water content of skeletal muscle were increased.
对537例接受长期利尿剂治疗(1年以上)的动脉高血压(n = 240)和/或充血性心力衰竭(n = 297)患者的血浆和骨骼肌电解质进行了调查。两组血浆和骨骼肌K、Mg含量均显著降低,肌Na值升高,骨骼肌总含水量和细胞外含水量升高。
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引用次数: 34
Fats and diet. 脂肪和饮食。
Pub Date : 1988-01-01
K R Norum, C A Drevon
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引用次数: 0
Bartter's syndrome. A review of 28 patients followed for 10 years. 巴特氏综合症。对28名患者进行了10年的随访。
Pub Date : 1988-01-01
A Rudin

Twenty-eight patients with Bartter's syndrome diagnosed during the years 1964-86 and followed for an average of 9.9 years have been reviewed. Their mean age at the time of diagnosis was 32.9 years. As a group they were shorter than normal subjects. In 19 patients hypokalaemia was detected incidentally. Neuromuscular symptoms, usually minor, had occurred in 19 subjects. Pregnancies and deliveries were unremarkable. One patient has died from malignant lymphoma, the others are alive. Of these, one patient has developed renal failure and received a renal transplant. The other patients have preserved a normal renal function and the majority have been healthy and working full time. Treatment rarely resulted in normokalaemia. The annual incidence of the syndrome has been estimated at 1.2 per million people.

本文回顾了在1964- 1986年间诊断出的28例Bartter综合征患者,并对其进行了平均9.9年的随访。他们确诊时的平均年龄为32.9岁。作为一个群体,他们比正常受试者矮。19例患者偶然发现低钾血症。19名受试者出现神经肌肉症状,通常较轻。怀孕和分娩都很平常。一名病人死于恶性淋巴瘤,其余的都还活着。其中,一名患者发展为肾衰竭并接受了肾移植。其他患者肾功能保持正常,大多数人身体健康,全职工作。治疗很少导致正常钾血症。据估计,该综合征的年发病率为每百万人中有1.2人。
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引用次数: 0
Social isolation and mortality in ischemic heart disease. A 10-year follow-up study of 150 middle-aged men. 社会孤立与缺血性心脏病的死亡率对150名中年男性进行了为期10年的跟踪研究。
Pub Date : 1988-01-01
K Orth-Gomér, A L Undén, M E Edwards

The effects of psychosocial and clinical factors on mortality in ischemic heart disease (IHD) were examined in a 10-year follow-up of 150 middle-aged men. Three groups of men were included: men with clinically manifest IHD, men with risk factors and healthy men. Psychosocial factors were assessed by means of standardized questionnaires. They comprised educational level, social class, marital status and a comprehensive assessment of the daily rounds of life of these men. Furthermore, a subjective rating of the own general health status was obtained. The clinical investigation included a standard physical examination, fasting serum lipids, glucose and urate, a frontal and sagittal chest X-ray and a 24-hour ambulatory ECG monitoring. During follow-up 37 men died, 20 of them from IHD. Non-survivors were discriminated from survivors by the following factors: older age, lower education, lower social class, higher systolic blood pressure, increased ventricular irritability and cardiac enlargement. Furthermore, a relative social isolation as indicated by a low social activity level and a poor self-rated general health status was characteristic of non-survivors. In multivariate analyses three factors emerged as the equally strong predictors of mortality, both from all causes and from IHD: social isolation, a poor self-rated health status and ventricular irritability. The psychosocial mortality predictors were independent of and of similar strength as the clinical predictors.

对150名中年男性进行了为期10年的随访,研究了社会心理和临床因素对缺血性心脏病(IHD)死亡率的影响。男性分为三组:临床表现为IHD的男性、有危险因素的男性和健康男性。采用标准化问卷对心理社会因素进行评估。调查内容包括教育水平、社会阶层、婚姻状况以及对这些男性日常生活的综合评估。此外,还获得了对自身总体健康状况的主观评价。临床调查包括标准体格检查、空腹血脂、血糖和尿酸、胸部正位和矢状位x线检查和24小时动态心电图监测。在随访期间,37人死亡,其中20人死于IHD。非幸存者与幸存者的区别在于以下因素:年龄较大,受教育程度较低,社会阶层较低,收缩压较高,心室易怒性增加和心脏增大。此外,社会活动水平低和自我评定的一般健康状况较差所表明的相对社会孤立是非幸存者的特征。在多变量分析中,有三个因素作为所有原因和IHD的死亡率同样强有力的预测因素:社会孤立、自我评估健康状况不佳和心室烦躁。社会心理死亡率预测因子独立于临床预测因子,其强度与临床预测因子相似。
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引用次数: 0
Hyperuricaemia and risk of cardiovascular disease and overall death. A 12-year follow-up of participants in the population study of women in Gothenburg, Sweden. 高尿酸血症与心血管疾病和总体死亡的风险。对瑞典哥德堡妇女人口研究的参与者进行了为期12年的随访。
Pub Date : 1988-01-01
C Bengtsson, L Lapidus, C Stendahl, J Waldenström

Serum uric concentration was determined in a series of 1462 women, aged 38-60 when first examined in 1968-69, as the first phase of a longitudinal population study in Gothenburg, Sweden. Serum uric acid concentration was positively correlated to the 12-year overall mortality in univariate analysis. No relationship was observed between initial serum uric acid values and incidence of myocardial infarction, angina pectoris, ECG changes indicating ischaemic heart disease or stroke. The association between serum uric acid concentration and mortality was independent of age, body mass index, systolic blood pressure, adipose tissue distribution, smoking habits, serum cholesterol concentration, serum triglyceride concentration, serum creatinine concentration, serum calcium concentration, use of diuretics, and haematological disease. The increased mortality could not be explained by any increase in malignant neoplastic disease.

作为瑞典哥德堡纵向人口研究的第一阶段,在1968-69年首次检查时,测定了1462名38-60岁女性的血清尿酸浓度。单因素分析显示,血清尿酸浓度与12年总死亡率呈正相关。血清尿酸初始值与心肌梗死、心绞痛发生率、缺血性心脏病或中风的心电图变化无关系。血清尿酸浓度与死亡率之间的关系与年龄、体重指数、收缩压、脂肪组织分布、吸烟习惯、血清胆固醇浓度、血清甘油三酯浓度、血清肌酐浓度、血清钙浓度、利尿剂使用和血液学疾病无关。死亡率的增加不能用任何恶性肿瘤疾病的增加来解释。
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引用次数: 0
Reduction of blood pressure by treatment with alphacalcidol. A double-blind, placebo-controlled study in subjects with impaired glucose tolerance. 用α骨化醇治疗降低血压。糖耐量受损患者的双盲、安慰剂对照研究。
Pub Date : 1988-01-01
L Lind, H Lithell, E Skarfors, L Wide, S Ljunghall

Disturbances of calcium or vitamin D metabolism have been suggested to be of pathogenetic importance both for hypertension and impaired glucose tolerance, two disorders that are commonly associated. In the present study 65 men, aged 61-65 years, with impaired glucose tolerance were enrolled in a prospective, double-blind, placebo-controlled study over 12 weeks evaluating the effects of 0.75 microgram alphacalcidol, a synthetic analog to the active metabolite of vitamin D. In the 26 patients with blood pressure greater than or equal to 150/90 mmHg before treatment a significant reduction (p less than 0.01) of both the systolic (SBP) and diastolic (DBP) blood pressure was found after therapy (from 171/95 to 150/88 mmHg). The effect was additive to concomitant antihypertensive treatment and was correlated (p = 0.03) to a reduction of serum levels of parathyroid hormone. Also in the whole group of patients given alphacalcidol blood pressure was moderately lowered from a mean of 152/87 +/- 22/10 (SD) to 143/84 +/- 17/8 mmHg. There were no relationships between the changes in body weight, blood glucose or insulin parameters and the changes in blood pressure during the trial. The findings are compatible with the concept that calcium metabolism influences blood pressure regulation and suggest that supplementation with a physiologic dose of active vitamin D could be beneficial for patients with high blood pressure.

钙或维生素D代谢紊乱已被认为对高血压和糖耐量受损具有重要的病理意义,这两种疾病通常与高血压和糖耐量受损相关。在本研究中,65名年龄在61-65岁、糖耐量受损的男性被纳入了一项为期12周的前瞻性、双盲、安慰剂对照研究,以评估0.75微克α骨化醇的效果。在治疗前血压大于或等于150/90 mmHg的26例患者中,发现治疗后收缩压(SBP)和舒张压(DBP)均显著降低(p < 0.01)(从171/95降至150/88 mmHg)。这种效果与同时进行的抗高血压治疗是叠加性的,并与血清甲状旁腺激素水平的降低相关(p = 0.03)。此外,在整个给予α骨化醇的患者组中,血压从平均152/87 +/- 22/10 (SD)中度降低到143/84 +/- 17/8 mmHg。在试验期间,体重、血糖或胰岛素参数的变化与血压的变化之间没有关系。这些发现与钙代谢影响血压调节的概念相一致,并表明补充生理剂量的活性维生素D可能对高血压患者有益。
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引用次数: 0
Coronary thrombolysis. Principles and practice. 冠状动脉溶栓。原则和实践。
Pub Date : 1988-01-01
L Erhardt, H Emanuelsson, M Hartford, H Johnsson, L E Strandberg
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引用次数: 0
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Acta medica Scandinavica
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