[Juvenile alpha-1-antitrypsin deficiency. Course, diagnosis and prognosis].
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Biomedical subjects
Publications and source records attributed to M Bauer.
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From October 1973 to October 1977 the ECOG prospectively evaluated cyclophosphamide, methotrexate, and fluorouracil (CMF) versus CMF plus fluoxymesterone (CMFH) maintenance therapies in responders to 6 months of induction therapy which consisted of either CMF, CMF plus prednisone (CMFP), or adriamycin plus vincristine (AV). Following the maintenance randomization 12% of the patients converted from a PR to a CR status. The median time from randomization to treatment failure was 9.5 months for CMFH and 6.7 months for CMF (p = 0.03). This difference was observed only for partial responders (p = 0.01) and not for complete responders. Patients receiving CMFH tended to maintain higher hemoglobin, leukocyte, and platelet levels, and receive a higher dosage of each of the cytotoxic drugs. The results are taken as evidence that the addition of fluoxymesterone to a maintenance CMF regimen provides a therapeutic advantage. It is hypothesized that this effect is due at least in part to fluoxymesterone associated maintenance of improved marrow function resulting in greater myelosuppressive drug delivery.
Loss of the spleen in children frequently results in overwhelming infection caused by pneumococci. In order to preserve splenic function the reimplantation of splenic tissue into the abdominal wall and the greater omentum was performed in a newborn child and six other children up to the age of thirteen. Postoperatively the complete absence of Jolly's bodies could be observed, while a continuous increase of IgM was noted. The otherwise mandatory antibiotic prophylaxis over 2 years could be reduced to 5 months in the newborn.
Changes in a number of morphological and biochemical parameters were observed in genetically obese Zucker rats and in lean controls between 3 and 58 weeks of age. By 3 weeks, the genetically obese rats had higher proportional (wt/100 g body wt) and absolute amounts of adipose tissue, hyperlipemia affecting all the lipid fractions, and hyperproteinemia compared to lean controls. Obesity, hepatomegaly, high concentrations of hepatic lipids and hyperinsulinemia did not appear until the fifth week. In obese animals, liver lipid concentration reached a maximum at 17 weeks of age and then declined. During this time, the triacylglycerol concentration in the serum remained stable, whereas the cholesterol and phospholipid concentrations continued to increase. The glycogen concentration in obese animals increased, both absolutely and compared to lean controls, between the 12th and 43rd weeks of age. From weaning, the Zucker rats, compared to lean controls, exhibited characteristics of obesity (accumulation of adipose tissue, hyperlipemia and hyperproteinemia), which persisted to the age of 58 weeks.
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Serum cholesterol and triglyceride levels were determined in 814 subjects and the relation to age, sex and body weight evaluated. In 568 persons the body mass index (BMI) and the relative body weight according to Broca were calculated. Normal values for each age group were established. Serum cholesterol, serum triglycerides and the body mass index showed an age dependent rise in the male and the female population. The body mass index showed a positive correlation with the serum triglycerides, but did not correlate with the serum cholesterol levels. The correlation between body mass index and serum triglycerides was not age dependent. Thus, our data stress the relation between overweight and hypertriglyceridaemia.
In 58 patients with extensive deep dermal burns the consequences of intubation, tracheotomy and central venous catheter in respect to pneumonia and bacteriaemia were studied. The sputum of tracheotomized patients showed twice as many pathogenic bacteria as the sputum of patients with prolonged intubation. From 93 subclavian catheters under bacteriological control 16 (17%) showed pathogenic bacteria on the tip. An identity between the bacille found in the blood stream and on the catheter tip was only found in 21%. From this study it seems conclusively that prolonged intubation is generally preverable to tracheostomy in severely burned patients and that the venous catheter can not be the most frequent cause of bacteriaemia.
Acute and subacute toxicity studies as well as reproduction-toxicologic investigations were carried out on the positive-inotropic substance 2[(2-methoxy-4-methylsulfinyl)phenyl]-1H-imidazo[4,5-b]pyridine (AR-L 115 BS) in various species. No undesirable side-effects or organ damage were observed in baboons after oral application of 2.5, 10 and 30 mg/kg AR-L 115 BS for 13 weeks. Beagle dogs, female miniature pigs and rats had brown-coloured urine after the substance. In miniature pigs and rats this was only observed at high dosage (mini-pig: 10 and 30 mg/kg; rat: 200 mg/kg). Changes in the ECG could be seen in the dose range of 20--40 mg/kg (dog i.v.), 30 mg/kg (dog p.o) and 200 mg/kg (rat p.o). Non-inflammatory mitral valve alterations (i.v. and p.o) as well as spot- and ring-shaped endocardial scleroses underneath the aortic valves in the region of the left cardiac outflow tract (30 mg/kg, p.o.) were found especially in female beagle dogs in the same dose range. The alterations are regarded as being of haemodynamic origin in view of the increased myocardial contractility caused by AR-L 115 BS. Phonocardiographic findings support this view. Besides, several dogs (30 mg/kg) and rats (200 mg/kg) showed myocardial scars. All male miniature pigs which had received 30 mg/kg AR-L 115 BS demonstrated an increase in heart and mitral valve weights. The results of the reproduction-toxicologic investigations gave no indication of an embryotoxic or mutagenic effect for the substance AR-L 115 BS. After comparing the results it can be concluded that the dog is the most sensitive of the species under investigation and that the results obtained in this species should not be generalised. Biochemical tests showed very similar metabolic patterns for AR-L 115 BS in baboons and man. From this it can be deduced that the results obtained in baboons are most likely to be transferable to man.
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The strain-gauge plethysmography, which has already successfully been applied to the calf and forearm the past years, has now, after extensive technical perfection, also been applied to the finger. Particular importance has to be paid to the selection of the measurement gauge and the cuff. Developed was a method to measure correct fit of the finger gauge to promote nearly a 100% correct registration of finger circumference. In order to get a proportionate distribution of the pressure inside the cuff; cuffs with a deep-drawn relief on the inside are used. The different temperatures on the hand, essential for functional diagnostic, are produced by an electronically regulated water-bath.
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The Urea production (PU) of three severe burned patients is presented as an example for calculating protein loss. Burn injuries are accompanied by an extensive catabolism more then any other disease, operation or trauma. The operative stress caused a further increase of PU. The highest values are measured in electric burns. Alimentation is considered as an important therapeutic mean to diminish catabolism. Urea production rate is presented as a simple method to control protein loss and can be done in every hospital.
A method of simulating the conventional radium packing method by the afterloading system is presented which consists in introducing three or four probes into the cavity of the uterus which can be spread in a defined manner by a balloon lying in a central position. The probe combination can be adapted to the individual forms and sizes of the uterus by varying the number, length and charge of the probes and by different manners of spreading and different location times of the individual probes. The method is applied for long term therapy as well as for short term irradiation.
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In 147 cases of mammary carcinoma, 388 serial mammographies were performed before final treatment. The average retrospective observation time was 27 months with a range of two months to 11 years. The number of serial mammographies per case ranged between two and 11. The tumor volume doubling times (TV); obtained by measuring the growth of the tumor nucleus shadow in the mammographies, ranged from 44 to 1869 days with an average of 212 days. No correlation between volume doubling time and histologic differentiation could be found. One hundred of these cancer patients were found in a screening population of 22,000 women receiving serial mammographies in a time period ranging from two to 16 years. An additional 40 cancer patients surfaced in this group without roentgenologic but with foregoing clinical or thermographic abnormalities before final diagnosis. An additional 21 cancer patients surfaced without any foregoing abnormalities. The follow-up tumor ranged between three months and two years with a mean time of one year and nine months. Not considering tumor size, pathologic-thermographic signs appeared with greater frequency the faster the tumor grew. Theoretically, an average of more than 16 years should elapse before an initial tumor cell develops into a 10-mm primary mammary carcinoma (30 doubling times). Therefore the length of time necessary for a 2-mm tumor to grow to a size of 10-mm is, on the average, four years.