Late enhanced computed tomography in Hypertrophic Cardiomyopathy enables accurate left-ventricular volumetry.

OBJECTIVES: Late enhancement (LE) multi-slice computed tomography (leMDCT) was introduced for the visualization of (intra-) myocardial fibrosis in Hypertrophic Cardiomyopathy (HCM). LE is associated with adverse cardiac events. This analysis focuses on leMDCT derived LV muscle mass (LV-MM) which may be related to LE resulting in LE proportion for potential risk stratification in HCM. METHODS: N=26 HCM-patients underwent leMDCT (64-slice-CT) and cardiovascular magnetic resonance (CMR). In leMDCT iodine contrast (Iopromid, 350 mg/mL; 150mL) was injected 7 minutes before imaging. Reconstructed short cardiac axis views served for planimetry. The study group was divided into three groups of varying LV-contrast. LeMDCT was correlated with CMR. RESULTS: The mean age was 64.2 ± 14 years. The groups of varying contrast differed in weight and body mass index (p < 0.05). In the group with good LV-contrast assessment of LV-MM resulted in 147.4 ± 64.8 g in leMDCT vs. 147.1 ± 65.9 in CMR (p > 0.05). In the group with sufficient contrast LV-MM appeared with 172 ± 30.8 g in leMDCT vs. 165.9 ± 37.8 in CMR (p > 0.05). Overall intra-/inter-observer variability of semiautomatic assessment of LV-MM showed an accuracy of 0.9 ± 8.6 g and 0.8 ± 9.2 g in leMDCT. All leMDCT-measures correlated well with CMR (r > 0.9). CONCLUSIONS: LeMDCT primarily performed for LE-visualization in HCM allows for accurate LV-volumetry including LV-MM in > 90% of the cases. KEY POINTS: • LeMDCT of relatively low contrast allows for LV planimetry in HCM. • The correlation of leMDCT-based LV volumetry with gold-standard CMR was excellent (r > 0.9). • LeMDCT requires approximately 2.0mL/kgBW of dye to achieve acceptable contrast.

[The problem of diagnosis of biliary calculi in acute pancreatitis by means of laboratory methods]. / Zur Frage der Gallensteindiagnostik bei der akuten Pankreatitis mit Hilfe von Labormethoden.

In order to assess the validity of a biochemical prediction of gallstones early in the course of acute pancreatitis by simple biochemical values (SGOT greater than or equal to 60 U/l, bilirubin greater than or equal to 1.6 mg/dl, alkaline phosphatase greater than or equal to 100 U/l) 297 patients with alcoholic, idiopathic and biliary pancreatitis were reinvestigated in a retrospective manner. The present study shows that a valid prediction by these values is not possible since 1) only about 40% of attacks of acute gallstone pancreatitis were associated with elevated SGOT, bilirubin and alkaline phosphatase concentrations and 2) there was a considerable overlap with the other etiological groups especially with idiopathic pancreatitis.

[Unusual clinical forms of pseudo-LE syndrome: an induced immune-complex disease? (author's transl)]. / Ungewöhnliche Erscheinungsformen des Pseudo-LE-Syndroms. Eine induzierte Immunkomplexkrankheit?

Two of nine patients with pseudo-LE syndrome had previously undescribed organ manifestations. In one there was the full-blown picture of cutaneous lupus erythematodes, with renal and CNS involvement, ending fatally. Immunohistologically there was severe vasculitis and nephritis, histologically generalised necrotizing angitis. It is possible that the dramatic clinical picture was due to alpha-methyldopa. In the second case lymphadenopathy with bihilar involvement and splenomegaly predominated. Histologically there was no evidence of Boeck's sarcoid. Further studies revealed systemic involvement with immune-complex nephritis, electron-dense deposits in the glomerular basal membrane, and the typical changes of lupus erythematodes in the marrow. In a third case there were similar changes in kidney and marrow. These findings support the view of an immune-complex disease which can be fatal. An unusual feature in seven of the nine patients was the proven intake of Venopyronum¿ coated tablets in temporal relationship to the manifestation of the disease. It is possible that the described cases of pseudo-LE syndrome were drug-induced. The immunological mechanism of the disease would, in that case, be analogous to penicillamine nephropathy.

[Quantitative determination and phenotyping of ceruloplasmin in morbus Wilson (author's transl)]. / Quantitative Bestimmung und Phänotypisierung der Cäruloplasmins bei Morbus Wilson

Sera of 17 patients with Wilson's disease and of 48 relatives were investigated as to a possible correlation between ceruloplasmin phenotypes and Wilson's disease. The control group included 727 healthy subjects. The results of the quantitative determination of ceruloplasmin confirmed the overlapping in the groups of so-called heterozygotes and controls known from the literature. The ceruloplasmin phenotypes in starch gel electrophoresis we observed were of the most common phenotype Cp BB in the patients as well as in the controls. Therefore, patients with Wilson's disease also show the normal Cp-phenotypes.

[Early course in acute pancreatitis (author's transl)]. / Die Beurteilung der Frühprognose der akuten Pankreatitis.

In this retrospective study the prognostic significance of 5 laboratory parameters in a low and high range (LDH) greater than or equal to 450 and 600 U/l, calcium less than or equal to 2.2 and 2.0 mmol/l, bloodglucose greater than or equal to 160 and 240 mg/dl, creatinine greater than or equal to 1.5 and 2.0 mg/dl, BUN greater than or equal to 25 and 40 mg/dl) and the patient's age was determined in 298 cases at the first attack of acute pancreatitis. The laboratory parameters were determined on the day of admission. The results presented in this study allow 2 major conclusions: 1. both the low and the high range laboratory parameters are potent indices in predicting a high risk group of patients very early. 2. a higher specificity is achieved by these high range parameters.

[Urinary copper excretion from rats following administration of aliphatic and alicyclic polyamines (author's transl)]. / Die Harn-Kupferausscheidung bei Ratten nach Gabe von aliphatischen und alicyclischen Polyaminen.

The cupriuretic effectivity of tetradentate chelating agents was measured in rats and compared with that of D-penicillamine. The applied chelators are aliphatic and alicyclic tetramines and tetramine-analogous substances with two nitrogen-atoms substituted by oxygen. Urine copper excretion was measured during 24 h (and 6 and the following 18 h, respectively) after single and repeated doses over a period of 5 days. The aliphatic tetramines, triethylenetetramine (TETA, TRIEN) and BE 6184, were the most active substances. Simultaneous application of TETA and D-penicillamine does not induce a summation or potentiation of the copper elimination. After s.c. application TETA seems to be three times more effective than orally. The potency of aliphatic and alicyclic tetramines is discussed on the basis of in vitro data.

Riboflavin synthases of Bacillus subtilis. Purification and properties.

A variety of Bacillus and Clostridium strains were found to contain two forms of riboflavin synthase which can be easily separated by density gradient centrifugation. The fast sedimenting species accounts for 12 to 44% of the total riboflavin synthase activity in the strains analyzed. Both riboflavin synthases were purified to apparent homogeneity from cell extracts of a genetically derepressed mutant of Bacillus subtilis. The specific activities of the pure proteins were 50,000 nmol mg-1 h-1 (light enzyme) and 2,000 nmol mg-1 h-1 (heavy enzyme). The sedimentation velocities (S20,w) were 4.1 and 26.5 S, respectively. Light riboflavin synthase showed a molecular weight of 70,000 in sedimentation equilibrium experiments. Sodium dodecyl sulfate polyacrylamide gel electrophoresis showed a single band corresponding to a molecular weight of about 23,500. Thus the enzyme appears to consist of three identical subunits (alpha type). Heavy riboflavin synthase has a molecular weight of 1,000,000 as shown by sedimentation equilibrium analysis. The protein appears to consist of 2 or 3 alpha subunits and approximately 60 beta subunits. A fragment apparently identical with light riboflavin synthase can be obtained from the heavy enzyme by mild dissociating treatment.