[Alström syndrome--a rare disease of diabetic association]. / Alström-Syndrom--eine seltene Erkrankung aus dem diabetischen Formenkreis.

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.

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.

[Polyangiitis overlap syndrome]. / Polyangiitis-overlap-Syndrom.

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.

[Value of color duplex ultrasound in diagnosis of insufficiency of perforant veins. A prospective study of 94 patients]. / Wertigkeit der Farb-Duplex-Sonographie in der Diagnose insuffizienter Vv. perforantes. Eine prospektive Untersuchung an 94 Patienten.

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.

Hypertension and insulin resistance. Glycaemia and insulinaemia in overweight hypertensive patients.

The relationship between insulin resistance and hyperinsulinaemia on one hand and hypertension on the other hand has become apparent during the last few years. Insulin resistance, which may be genetically determined, is, according to our present understanding, the 'key player' in the metabolic syndrome. However, the pathophysiology of the combination of factors has not yet been fully elucidated. Early therapeutic intervention for insulin resistance, hyperinsulinaemia and hypertension may prevent the clinical manifestation of non-insulin-dependent (type 2) diabetes. Preliminary results of an ongoing study investigating the effects of trandolapril or the diuretic combination of hydrochlorothiazide and triamterene on serum glucose and insulin levels are presented.

[Incidence and symptomatology of lung embolism in relation to the site of deep venous thrombosis]. / Untersuchungen zur Häufigkeit und Symptomatologie der Lungenembolie in Abhängigkeit von der Lokalisation der tiefen Beinvenenthrombose.

The uncertainty about the true incidence of pulmonary embolism (PE) in connection with deep venous thrombosis (DVT) becomes evident when comparing the results of autopsy--vs. clinical studies, with the former showing a three-fold elevated rate of embolisation. In order to evaluate the percentage of clinically inapparent PE, all patients (65 females, 54 males, mean age 61.3 years) hospitalized between April 1989 and March 1990 with suspected DVT and/or PE underwent duplex-sonography and pulmonary scintigraphy. In 108 cases, DVT could be ascertained, whereas 11 patients only suffered from PE. In 57.4% of all DVT, PE was diagnosed. Of the 73 cases with PE, only 53.4% of the patient stated typical symptoms primarily. In more than 1/4 of the patients with primary symptoms of PE, no DVT could be diagnosed. There was no significant difference between the occurrence of PE in relation to the localisation of DVT, with 1/3 to 1/2 being asymptomatic. 1/4 of the patients with leg-thrombosis and 1/7 with thrombosis of the iliac vein did not complain of typical symptoms of thrombosis. Furthermore, an increased PE-rate seems to occur with an elevated ultrasound echogenity of the thrombus. As a result of the study pulmonary szintigrams seem to be indicated in all cases of DVT in order to evaluate the total PE risk.

[Results of local thrombolysis with special reference to diabetic metabolism]. / Ergebnisse der lokalen Thrombolyse unter besonderer Berücksichtigung einer diabetischen Stoffwechsellage.

The influence of diabetes on the primary and long-term success rate after 145 local thrombolyses in peripheral arterial disease stages III and IV was evaluated. 75 patients suffered from thrombotic, 62 patients from embolic occlusions, with eight patients suffering from thrombangitis obliterans. Regarding the localisation of vascular occlusion 0.6% suffered under an occlusion of the iliacal artery, 21% of the femoral artery, 16% of the popliteal artery, 7% of the vessel of the lower limb and 55.4% showed a combined occlusion of the femoral- and popliteal artery and the artery of the lower limb. In cases of embolic occlusions only marginal differences could be observed, while the primary success-rate of thrombotic occlusions showed greater differences between both groups (75% vs. 91%). During the follow-up, no differences between both groups could be established (patency-rate of 79% for both groups). The same applies to the prognostic factors: peripheral run off, length, duration of occlusion and the clinical stage (Fontaine IIb to IV). The remarkable differences between diabetic and non-diabetic patients in cases of occlusions of more than 16 cm (66% vs. 88% in primary and 55% vs. 77% in long-term success) can be explained by the high percentage of diabetic patients with poor run-off and microangiopathy. Regarding the above parameters, primary and long-term results seemed to be less in diabetic patients, even though a long-term patency could be observed in 2/3 of diabetic patients in stages IIb and IV with primary success.