A new variant of glucosephosphate isomerase deficiency (GPI-Utrecht).

A new case of glucosephosphate isomerase deficiency is described in a Dutch family. The activity of the enzyme was decreased to 20-25% of the normal value. Characterization of the defect enzyme showed a pronounced thermolability. Heating of the enzyme at 45 degrees C showed a loss of activity of 90% after one hour. The pH-optimum and the electrophoretic migration were normal. The Km-value for F-6-P, the Ki for the competitive inhibitors 2,3-DPG and 6-PG were in the normal range. The variant described here differs from all known variants. Therefore we propose to give to this new variant the name of GPI-Utrecht.     

Simultaneous estimation of urinary steroids by semi-automated gas chromatography. Investigation of neo-natal infants and children with abnormal steroid synthesis.

Development of a method for multicomponent analysis of urinary steroids on open-tubular columns is described. The urinary steroid conjugates were hydrolysed enzymatically, extracted on Amberlite XAD-2 columns, purified on columns of Sephadex LH-20, then analysed as methyloxime trimethylsilyl ethers on a gas chromatograph on which up to 24 samples may be automatically injected. All major urinary steroids from 17-oxosteroids to cortisol metabolites were analysed and these were quantified relative to internal standards, 5alpha-androstane-3alpha, 17alpha-diol and cholesteryl butyrate added to the samples prior to derivatisation. The precision of the full technique and reproducibility of repetitive automatic solid injection was found to be acceptable for the purpose of profile analysis. Examples of urinary steroid profiles in normal infants and infants with disorders of adrenal steroid production and excretion are given.     

H.E.L.P.治疗急性脑梗塞的护理

H.E.L.P.治疗急性脑梗塞有确切的疗效.本文强调了血管通路的护理、抗凝方法、血压动态监测、健康宣教在治疗过程中的重要作用.     

The effect of pre-incubation and direct procedure for the alanine transaminase assay in two kits compared.

The kinetic measurement of serum alanine transaminase was carried out on two enzyme kits (Roche and Calbiochem), done as a direct method and a pre-incubation method. Variable results from commercial control sera and patient sera by these four procedures were obtained. Pre-incubation enzyme values were generally lower than the direct values (mean difference 10%), but there were marked differences between the results from the two kits. This finding has important significance especially for laboratories participating in external QC Programmes. Frequent precision checks of the method and a local reference range are therefore necessary.     

Smoking and serum alpha 1-antitrypsin in 1296 healthy men (author's transl)]

Serum alpha 1-antitrypsin (alpha 1AT) was measured by radial immunodiffusion in 1296 healthy men aged 18--50 years. Other biological criteria, including leukocyte count and alpha 2-globulins were measured and the subjects were given a detailed questionnaire on their smoking habits. Results showed a very strong positive relationship between smoking and serum alpha 1AT: the heavy smokers had a serum alpha 1AT 20% higher than the non-smokers, and among subjects who stopped smoking, the level returned rapidly to normal. There were also close interrelationships between serum alpha 1AT, smoking, leukocyte count and a alpha 2-globulins. A discussion of these results is presented.     

A note on the practical aspects of the assay of N-acetyl-beta-glucosaminidase in human urine.

1. Some practical aspects of the assay of urinary N-acetyl-beta-glucosaminidase activity were studied in patients with renal disease. 2. Centrifugation and dialysis of urine were not necessary prior to assay. 3. When enzyme activity was related to urinary creatinine random urine samples could be used. 4. Since preservatives used to prevent bacterial growth may inhibit enzyme activities, samples should be assayed immediately after collection or stored at 4 degrees C or 20 degrees C. 5. Mild visible haemoglobinuria, eg. derived from lysed red blood cells did not interfere with the enzyme assay.     

The diagnostic value of urinary enzyme measurements in hypertension

N-Acetyl-beta-D-glucosaminidase (NAG), beta-D-galactosidase, alkaline phosphatase (ALP) and leucine aminopeptidase (LAP) were assayed in the urine of 100 normal and 112 hypertensive subjects. Age-related urinary activities for these enzymes in the normotensive control subjects are presented. A new procedure for the assay of urinary ALP using 2-methoxy-4-(2'-nitrovinyl)phenyl (MNP) phosphate is described. Thirty-five of the hypertensive patients were considered to have primary renal disease. The urinary activity of NAG was increased in 27 (77%) of these patients and the detection of primary renal disease was not enhanced by measurements of the other urinary enzymes. Testing the urine both for NAG activity and protein, led to the detection of 91% of these patients. The assay procedures described are simple to perform and can be carried out in outpatient clinics. The measurement of urinary NAG activity is a cheap and reliable method for detecting renal disease in hypertensive patients but maximum diagnostic yield is achieved when proteinuria is determined as well.     

Defective erythrocyte pyruvate kinase.

A defective pyruvate kinase (EC 2.7.1.40) is described. The abnormal PK is characterized by a shift in the R in equilibrium T equilibrium to the T-state. The Ko.5 for the substrate phosphoenol pyruvate is about 6 times higher than for the normal enzyme, while the KM value for the positive effector Fru-1, 6-P2 is increased. In agreement with a shift to the T-state is the increased affinity of the abnormal enzyme for the negative effectors ATP and alanine. The results are discussed in relation to other abnormal pyruvate kinases.     

In vitro assessment of the intra luminal phase of gliadin digestion in coeliac disease: γ-glutamyl transpeptidase-like activity in saliva

γ-Glutamyl transpeptidase (GGTP) is an enzyme of potential relevance to the digestion of dietary gliadin, and hence of possible importance in the development of coeliac disease (CD). We have confirmed that human saliva has GGTP-like activity. Comparison of this activity between treated CD patients in long-term remission, age- and sex-matched normal control subjects, and unaffected relatives of CD patients did not show statistically significant differences. Although our results have not demonstrated a primary abnormality of this particular enzyme activity in CD, the intraluminal phase of gliadin processing is potentially important in the aetiology and pathogenesis of CD and thus deserves further study.     

Investigation of the effects of naloxone upon acupuncture analgesia

Two patients with severe thalamic pain and one patient with causalgia from the shoulder-hand syndrome are described whose pain was markedly improved or abolished by anticholinesterase drugs. Both short-acting parenteral and oral anticholinesterase preparations were employed. Anticholinesterase drugs are a new and potentially valuable approach to the treatment of chronic pain.     

Assessing airway inflammation: from guessing to quantitative measurements

Diagnosing asthma and following the response to treatment have relied on lung function measurements. Improved knowledge of the cellular events leading to airflow limitation has led to new clinical methods to assess the inflammatory component of the disease. Induced sputum analysis and exhaled nitric oxide (NO) measurements are already tools for clinical practice. New cell-specific inflammatory markers and further innovations in the testing of exhaled air, e.g. breath condensates and technical development of simple methods and devices, will also benefit the busy practitioner in near future. Assessing airway inflammation by quantitative measurements, instead of guessing, will also strengthen the role of anti-inflammatory medication as first-line treatment of asthma, even in its mildest forms.     

Measurement of angiotensin II in human plasma: technical modifications and practical experience.

This paper presents our experience in measurement of plasma angiotensin II concentration according to the method described by Düsterdieck, G. and McElwee, G. (1971) Eur. J. Clin. Invest. 2, 32-38. Certain technical modifications of the procedure have been worked out. For each individual plasma sample, the recovery across extraction and elution steps is determined by addition of radio-iodinated angiotensin II. A time-saving recycling elution procedure is described. Evidence for the clinical applicability of the modified method is presented.     

冠心病患者血液流变特性改变及其与血脂水平的相关性观察

本文对８６例冠脉造影患者血粘滞度、血脂、血小板聚集率及红细胸压积等进行测定。结果发现，冠脉造影阳性者上述各项指标均显著高于冠脉正常者；血脂及血细胞压积与血粘滞度呈显著相关关系。作者认为血脂及血细胞压积是影响血粘度的重要因素；血粘度的改变在冠心病发生及发展过程中起着十分重要的作用．     

Use of Mental Health Services by Adolescents After Traumatic Brain Injury: A Secondary Analysis of a Randomized Controlled Trial

Background

Mental health problems are common after pediatric traumatic brain injury (TBI). Many patients in need of mental health services do not receive them, but studies have not consistently used prospective and objective methods or followed samples for more than 1 year.