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Biomedical subjects

H Stiegler

Publications and source records attributed to H Stiegler.

At least 37 records · Page 2Linked to original sources

[Alström syndrome--a rare disease of diabetic association].

BACKGROUND: Alström's disease is a rare hereditary multiple-system illness, whereas a second-messenger defect can be assumed. CASE REPORT: We describe a case-the first in Germany of 15 known cases in the world literature-, who suffers from all clinical features, such as non-insulin-dependent diabetes mellitus, retinitis pigmentosa, pancochlear damage of the ears, hypogonadism, obesity and chronic nephropathy, with the exception of acanthosis nigricans. CONCLUSION: Because of the multiplicity of affected organs the diagnosis of Alström's disease is difficult.

Adult↗

Improvement in the quality of life after i.v. PGE1 therapy for intermittent claudication.

BACKGROUND: Increasingly and justifiably, clinical studies are now being expected to investigate the influence of therapeutic measures also on the quality of life of the patient. PATIENTS AND METHODS: Since no data on the variability of changes in the quality of life of the patient following PGE1 treatment are so far available, the initial investigation was designed as an uncontrolled pilot study. 104 patients (median age 64.5 years) with a maximum of walking distance on the treadmill (3 km/12%) of 50-250 m were included and given a daily intravenous infusion of 60 micrograms PGE1 (Prostavasin) over a period of 4 weeks excluding weekends. This was followed by a treatment-free follow-up period of 3 months. Changes in the quality of life were recorded with both the newly developed disease-specific questionnaire PAVK-86, and the generic questionnaire SF 36; in addition, the pain-free and maximum walking distances on the treadmill were also established prior to and immediately following treatment, as also at the end of the follow-up period. RESULTS: The quality of life was significantly improved in all dimensions (functional status, complaints, pain, mood, anxiety, social life, treatment expectations) in addition to a marked increase in the median pain-free walking distance from 77 to 108 m (p < 0.001) and the maximum walking distance from 118 to 171 m (p < 0.001). At the end of the 3-month observation period, the improvement was essentially still demonstrable. CONCLUSION: The study has shown for the first time that treatment with intravenous PGE1 brings about not only the already known increase in the walking distance, but also a clinically relevant and significant improvement in the patient's quality of life.

Adult↗

Activation of the fibrinolytic system in patients with coronary artery disease and hyperfibrinogenemia.

Elevated fibrinogen levels as well as an impaired activity of the fibrinolytic system are regarded as important cardiovascular risk factors. To elucidate a potential interrelation between fibrinogen as an indicator of a hypercoagulable state and the endogenous fibrinolytic function hemostatic and rheological as well as lipid parameters were determined in 224 consecutive patients, who underwent elective coronary angiography. In the selected study population of 81 men and 19 women with fibrinogen concentration either > or = 3.5 g/l (n = 70) or < or = 2.5 g/l (n = 30) hyperfibrinogenemia was found to be significantly associated with increased concentrations of plasmin-alpha 2-antiplasmin complex [PAP [median (25.-75. percentile)], 534 (361-680) micrograms/l vs. 289 (243-440) micrograms/l; p < 0.001] and tissue plasminogen activator (t-PA) antigen [9 (6-11) micrograms/l vs 8 (5-9) micrograms/l; p < 0.05] while this association was lost in the subgroup of patients with angiographically normal coronary arteries (n = 26). In addition to these findings fibrinogen was significantly correlated with PAP (r = 0.40, p < 0.001; n = 224) and t-PA antigen (r = 0.2, p < 0.01; n = 224) after adjustment for age, diabetes mellitus, lipid parameters and leucocyte counts. It can be argued that elevated fibrinogen levels in patients with coronary artery disease are concomitant with an activation of the fibrinolytic system.

Blood Viscosity↗

Predictors of 10-year macrovascular and overall mortality in patients with NIDDM: the Munich General Practitioner Project.

The 10-year follow-up of the Munich General Practitioner Project was designed as a long-term prospective study to evaluate factors predicting macrovascular and overall mortality in a random cohort of non-insulin-dependent diabetic (NIDDM) patients. Of the original 290 patients (103 males, 187 females, median age 65 years) 92.5% could be assessed, 103 subjects had died, 58 from macrovascular causes. In an univariate analysis of baseline data, deceased patients, and especially those who died from macrovascular causes had significantly higher fasting blood glucose, HbA1c, von Willebrand-factor protein, urine albumin excretion, and serum beta 2-microglobulin, were significantly older, exhibited significantly more ischaemic heart disease (abnormal ECG Minnesota codes), carotid artery and peripheral vascular disease (both determined by ultrasound-Doppler), and had significantly inferior knowledge about diabetes and its treatment. No significant differences were seen for gender, blood pressure, smoking, total cholesterol, triglycerides, HDL-cholesterol, or the use of antidiabetic, antihypertensive or coronary drugs. In a multiple logistic regression analysis, the risk factors for macrovascular death were age, HbA1c and von Willebrand-factor protein. When baseline macrovascular disease was taken into account, carotid artery disease was also a determinant. The main variables from the metabolic syndrome (blood pressure, dyslipidaemia, body mass index) did not enter a multiple logistic regression analysis. The data suggest that age and haemoglobin A1c are major determinants, and that in addition von Willebrand-factor associated endothelial damage is a risk factor for macrovascular mortality in NIDDM patients.

Age Factors↗

[Multicystic peritoneal mesothelioma].

We report on a 31-year-old man with a 29 x 15 x 15 cm large abdominal tumor who came to surgery under suspicion of echinococcus cyst of the liver. Histologically, the neoplasm could identified as multicystic peritoneal mesothelioma (synonyma: benign cystic mesothelioma). Additionally some cysts were located in the greater omentum and mesocolon. In contrast to our case the benign cystic mesotheliomas most commonly occur in young women, localized in the pelvis and abdomen and often complicated by postoperative local recurrence. We performed a resection of the right hemicolon and the omentum. Postoperatively no further therapy was necessary because of the benign appearance of the lesion. Close follow-up is required in these patients due to the disposition for recurrences.

Adult↗

[Endothelin and big endothelin in coronary heart disease and acute coronary syndromes].

Endothelin (ET), the most potent endogenous vasoconstrictor with mitogenic potency, is generated from its precursor big-endothelin (BET) in a proteolytic process and discussed as a pathogenetic factor in coronary artery disease and in the acute coronary syndromes. Several studies documented elevated plasma endothelin concentrations in acute myocardial infarction, but conflicting results were reported in patients with stable and unstable angina. Only few studies determined big endothelin, although it half-life and plasma concentrations are higher in comparison to endothelin. ET and BET levels (Radioimmunoassay, Biomedica GmbH, Vienna) were determined in patients with stable angina (SAP, n = 20), unstable angina (IAP, n = 12), acute myocardial infarction (AMI, n = 12) and healthy subjects (NP, n = 11). The concentrations of ET and BET (median (minimum-maximum) in fmol/ml) of the patients with stable angina (SAP: ET 0.7 (0.3-1.1); BET 1.7 (0.7-2.9)), unstable angina (IAP: ET 1.0(0.5-1.7); BET 2.5 (1.3-4.1)) and acute myocardial infarction (AMI: ET 1.2 (0.6-2.3); BET 3.6 (3.2-5.3)) showed a significant difference compared to controls (NP: ET 0.5 (0.4-0.7); BET 1.4 (1.1-1.7)) (SAP vs. NP: ET p < 0.01; BET p < 0.05; IAP and AMI vs. NP: ET and BET p < 0.001). Also, the concentrations of the peptides differed significantly dependent on the clinical severity of coronary artery disease (AMI vs. SAP: ET and BET p < 0.001; AMI vs. IAP: BET p < 0.05; IAP vs. SAP: ET p < 0.05; BET p < 0.01). Twelve of 15 patients with big endothelin concentrations over 3 fmol/ml suffered acute myocardial infarction. Seven of 12 patients with AMI showed elevated ET and BET concentrations before the increase of creatinecinase. There was no correlation between number of risk factors per patient, cholesterin and subfractions, severity of CAD classified in one-two-three-vessel disease or coronary score according to modified criteria of the American Heart Association (AHA). We conclude that in patients with coronary artery disease endothelin and big endothelin levels are elevated and related to the clinical and not to the morphological severity of coronary artery disease. Big endothelin is the more sensitive parameter in comparison to endothelin and indicates a severe course of myocardial ischemia in patients with unstable angina. The development of assays with the possibility of a quick determination of the peptides may be valuable for risk stratification of acute coronary events.

Adult↗

[Polyangiitis overlap syndrome].

A 45-year-old woman developed cold paraesthesias, severe pain in the finger-tips of both hands and acral necroses, at first pinhead-sized but gradually enlarging. She had been a heavy smoker for many years, was hypertensive and had sustained a myocardial infarction 10 years previously. Angiography demonstrated bilateral occlusion of digital arteries and Doppler-ultrasound showed occlusion of both internal carotid arteries. Erythrocyte sedimentation rate was greatly increased to 101/130 mm. The symptoms improved during rheological treatment and administration of methylprednisolone, but then nonspecific abdominal pain occurred, together with intermittent claudication of the left leg, and she had numerous haemoptyses. The chest radiograph showed finely reticular shadows in both lungs. Histological examination of peribronchial tissue revealed typical signs of vasculitis. The patient became oliguric (creatinine up to 5.5 mg/dl). The titre of antineutrophil cytoplasm antibodies (cANCA) was very high. Despite treatment with cyclophosphamide and glucocorticoids the renal failure got worse, she had a stroke and severe anginal attacks developed. She died 25 weeks after the first admission from cardiocirculatory failure. As the patient's signs were those of several kinds of vasculitis, the polyangitis overlap syndrome seems the most likely diagnosis in retrospect.

Angina Pectoris↗

[Value of color duplex ultrasound in diagnosis of insufficiency of perforant veins. A prospective study of 94 patients].

Using colour coded duplex sonography in pre-operative diagnosis of insufficient perforant veins we succeeded in accomplishing a close correspondence between diagnostic methods and intraoperative findings. We examined 94 patients suffering from primary varicosis by duplex sonography and found 334 perforant veins in total, i.e. 2.4 per leg, which we marked with special ink. Nearly 50% of the patients had also been examined by phlebography, which was evaluated by the vascular surgeons only. One third of the phlebographies were not satisfactory. The vascular surgeon evaluated the accuracy of perforant veins marked by duplex sonography with 95.8%, whereas the sufficient phlebographies only reached an accuracy of 65%. The insufficient phlebographies showed only 16% of the duplex-sonographically marked perforant veins. The confluence of the insufficient minor saphenous vein with the popliteal vein corresponded in all of the 22 cases with the sonographic findings. The colour coded duplex sonography enables the vascular surgeon to localize the insufficient perforant veins exactly and also provides for minimal tissue damage, which means a relevant decrease of postoperative pain.

Blood Flow Velocity↗

Efficacy and tolerance of recombinant tissue-type plasminogen activator in patients with thrombotic or embolic occlusions of leg-arteries.

The efficacy and safety of recombinant tissue-type plasminogen activator (rt-PA) was evaluated in 46 patients with thromboembolic arterial occlusions in leg arteries. rt-PA was given over 1-4 h with a maximum dose of 18 mg. The effect of rt-PA treatment was determined as patency of the occluded arteries in 44 different patients 14 days after treatment. In 41 patients at least one artery was recanalized (93%) by rt-PA, and in almost half of these patients (48%) no residual stenosis were detected after the lytic treatment. A slight residual stenosis was detected in 29% of the patients and a severe residual stenosis in 21%. An additional treatment with percutaneous transluminal angioplasty was performed in 23 of the 44 patients and successful in 21 (91%). In 8 patients an addition catheter-embolectomy was performed. No difference in patency rate was detected between patients with thrombotic and those with embolic occlusions. The age of the occlusion influenced the patency rate; occlusions under the age of 5 weeks showed a patency rate of 96% compared to 82% in older occlusions. The length of the occlusion did not have any influence on the outcome of the rt-PA treatment. From the results of this open study we conclude that a dose of up to 18 mg of rt-PA is both safe and effective in the treatment of thromboembolic occlusions in leg arteries.

Adult↗

Microalbuminuria in a random cohort of recently diagnosed type 2 (non-insulin-dependent) diabetic patients living in the greater Munich area.

Still under debate is the prevalence of microalbuminuria in patients with recently diagnosed Type 2 (non-insulin-dependent) diabetes mellitus and its relation to existing macro-vascular disease and the major vascular risk markers. Hence, from a representative sample of 1512 patients with Type 2 diabetes of varied duration (recruited from 22 non-specialized medical practices of the Greater Munich Area) 68 (26 males, 42 females) of 71 eligible subjects with a known duration of diabetes of up to 17 weeks and not less than 4 weeks were examined in the present study. Median age was 61 (39 to 75) years, prevalence of ischaemic heart disease (case history plus ECG, Minnesota code, Whitehall criteria) 41.2%, and that of peripheral vascular and carotid artery disease (both assessed by ultrasound-Doppler) were 35.3 and 4.4%, respectively. Diabetes was well controlled (HbA1c: 6.9%, 5.6-8.3; fasting blood glucose: 7.7 mmol/l, 5.4-10.4; median +/- interquartile range IQ), the cardiovascular risk profile was most prominent in terms of triglycerides (3.1 mmol/l, 2.1-4.6, median +/- IQ range) and systolic blood pressure (164 mm Hg, 140-186, median +/- IQ range). 13.2% showed signs of urinary tract infection. Of the remainder, 19.0% exhibited microalbuminuria (RIA, > 30-200 mg/l), and 5.2% macroalbuminuria (> 200 mg/l). Significant correlations (p < 0.05) were found between urinary albumin concentration and beta 2-microglobulin in serum, systolic blood pressure, serum triglycerides, serum HDL-cholesterol (inversely), HbA1c, and peripheral vascular disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Pelvic vein thrombosis in protein C deficiency after infectious mononucleosis].

Subsequent to an infectious mononucleosis a 15 year old girl developed a thrombosis of the iliaca vein. Laboratory evaluation revealed a heterozygous form of protein C deficiency. An infusion with r-tPA (= recombinant tissue plasminogen activator) at a rate of 0.02 mg/kg BW/h over 96 hours failed to reopen the vessel. After intermittent heparin therapy longterm treatment with coumarin began. Three months later the iliaca vein was partially recanalised.

Adolescent↗

Preoperative systemic etoposide/ifosfamide/doxorubicin chemotherapy combined with regional hyperthermia in high-risk sarcoma: a pilot study.

From November 1990 to September 1991, 23 adults with high-risk, nonmetastatic sarcomas (20 soft-tissue sarcomas and 3 chondrosarcomas) were entered in a pilot protocol (RHT-91) involving regional hyperthermia combined with systemic chemotherapy followed by surgery. Of these patients, 12 had undergone previous surgery and/or radiation, 5 had received previous multidrug chemotherapy, and 6 were previously untreated. A tumor size of > 8 cm and/or an extracompartmental tumor location (11 patients) or local recurrence (12 patients) were defined as high-risk factors in addition to tumor grading (21 patients had grade 2 or 3 sarcomas). Regional hyperthermia was produced by an electromagnetic deep-regional-heating device. For systemic chemotherapy, all patients received etoposide/ifosfamide/doxorubicin (EIA) and mesna, with regional hyperthermia being given only on days 1 and 4 in repeated EIA/regional hyperthermia cycles every 3 weeks. Tumor temperatures (range, 40 degrees-44 degrees C) were measured by invasive thermometry in all patients during each regional hyperthermia treatment. A total of 181 regional hyperthermia treatments were applied within the pelvic region (11 patients) or extremities (12 patients) bearing relatively large tumors (mean volume, 848 cm3). By the cutoff date for this analysis (October 15, 1991), 13 patients had undergone surgery after receiving 2-6 (mean, 3.8) cycles of EIA chemotherapy combined with regional hyperthermia; all tumors except one were resected without disfiguration. In 22 evaluable patients (minimum, 2 EIA plus regional hyperthermia cycles), the clinical response rate was 27%, with 6 patients showing partial responses (PRs). In addition, a pathologic response to preoperative thermochemotherapy was evaluable in 13 patients, with 4 responders (31%) having > 50% histologic necrosis. In all, 3 of the responders (1 PR and 2 patients with > 50% histologic necrosis) relapsed within 3 months of surgical resection. The other 7 responding patients (5 PRs and 2 patients with > 50% histologic necrosis) showed stable disease with local tumor control. The study (RHT-91) is continuing as a multicenter phase II trial (opened on November 19, 1991) in patients with high-risk soft-tissue sarcomas to test the potential of preoperative thermochemotherapy in regard to local control and survival.

Adolescent↗