Corticomotoneuronal dysfunction in ALS patients with different SOD1 mutations.

OBJECTIVE: To examine corticomotoneuronal function in amyotrophic lateral sclerosis (ALS) patients carrying superoxide dismutase 1 (SOD1) mutations using peristimulus time histograms (PSTH). METHODS: Six I113T, 3 A4V, one G41D and one G114A patient were studied along with 21 healthy control subjects. Analyses included comparison with previously reported data from 8 D90A homozygous and 12 sporadic ALS (SALS) patients examined by the authors using identical methodology. RESULTS: Cortical threshold was significantly reduced in A4V patients (41.3%) compared to I113T (58%), SALS (57%) and D90A (71%) patients, as well as healthy controls (49.7%). Estimated excitatory postsynaptic potentials (EPSPs) were significantly larger in A4V patients (4.39 mV) compared to healthy controls (2.95 mV), I113T (2.71 mV) and SALS (2.39 mV) patients. Clinical features and PSTH parameters in I113T were similar to SALS, however, PSTH primary peaks (PP) were significantly more dispersed, 9.5 ms compared to 4ms in SALS. PSTHs from single G41D and G114A patients were unremarkable, apart from large EPSP amplitudes in the G114A patient. CONCLUSIONS: ALS patients with A4V and I113T SOD1 mutations have distinctive corticomotoneuronal changes that are different from those in D90A homozygous and SALS patients. SIGNIFICANCE: PSTH studies should be considered for future in vivo studies of SOD1 pathophysiology in ALS.     

New surface modification for Ti-6Al-7Nb alloy: oxygen diffusion hardening (ODH)

PVD-TiN coating and N+ implantation of Ti-6Al-7Nb alloy resulted in surface hardening to a depth of less 3 microns. The new oxygen diffusion hardening (ODH) treatment increased the hardness gradually to 50 microns. PUD-TiN showed an improvement in the tribological properties, while N+ implantation increased the PE wear rate. The wear rate of the ultrahigh-molecular-weight as well as the friction coefficient were reduced to one-half of the values achieved with the combination of CoCrMo-alloy when paired against the ODH-treated surface. In pairing with ZrO2-containing bone cement the ODH-treated surface showed only a minimal reaction.     

Nicardipine improves myocardial perfusion in systemic sclerosis

Primary scleroderma myocardial disease may be due in part to myocardial ischemia caused by a disturbance of the coronary microcirculation. We evaluated the effect of the calcium channel blocker nicardipine on myocardial perfusion assessed by thallium-201 scanning in 16 patients with systemic sclerosis. Thallium-201 single photon emission computerized tomography was performed at baseline and 90 min after 40 mg of oral nicardipine. The mean (+/- SD) number of left ventricular segments with perfusion defects significantly decreased from 6.0 +/- 2.0 at baseline to 4.1 +/- 2.3 after nicardipine (p less than 0.01). The mean global perfusion score significantly increased from 10.2 +/- 1.9 at baseline to 11.9 +/- 2.6 after nicardipine (p less than 0.02). Our study demonstrates short-term improvement in thallium-201 myocardial perfusion with nicardipine in patients with systemic sclerosis.     

Population kinetics of gentamicin in neonates

A population kinetic analysis was carried out on sparse plasma gentamicin (GE) concentration data from 469 neonates obtained as part of a routine therapeutic drug monitoring (TDM) programme in the hospital neonatology unit.The best predictors of the kinetic parameters of the monoexponential model, volume of distribution (Vd) and clearance (CL), were the weight (WT) and gestational age (GA). Vd of the neonates was only related to WT, whereas the half-life was only related to the GA.     

Experimental studies on the recovery of anesthetic gases]

The volatile anesthetic agents halothane, enflurane, and isoflurane are chlorofluorocarbons (CFC) and contribute to ozone depletion. Although the contribution is small, its importance is rising, as technical CFCs will be phased out according to the Montreal protocol (1987) and the London conference (1990) by the year 2000. Alternative procedures and CFC-free volatile agents such as des- and sevoflurane do not contribute to depletion of the ozone layer, but will not replace standard methods using volatile anesthetic agents in the near future. METHODS. In an experimental setup, we filtered anesthetic waste gases from scavenging systems of rebreathing circles by activated carbon filters. The filtered substances were desorbed by a heat chamber and condensed in a cold trap. RESULTS. By this method, it was possible to retrieve 50%-60% of the applied gases. Gas chromatographic analysis showed halothane containing traces of pollutants and isoflurane and enflurane as pure substances. DISCUSSION. The retrieval of anesthetic waste gases is easy; no sophisticated technical equipment is necessary. Purity of substances could make recycling possible and offer a method to avoid environmental pollution by volatile anesthetics.     

Arbeitsunfähigkeit im ersten Jahr der chronischen Polyarthritis. Ein Vergleich mit Pflichtmitgliedern der gesetzlichen Krankenversicherung

Zusammenfassung Obwohl Arbeitsunf?higkeit (AU) eine direkte Folge der durch die Erkrankung reduzierten Arbeitskraft ist, wurde sie im Frühstadium der chronischen Polyarthritis (cP) kaum untersucht. Deshalb wurden die H?ufigkeit und die Dauer der AU in einer multizentrischen Querschnittsstudie von Patienten im Frühstadium der cP (≤12 Monate Krankheitsdauer) im Vergleich zu Pflichtmitgliedern der gesetzlichen Krankenversicherung ermittelt. 134 ambulante erwerbst?tige Patienten erfüllten die Kriterien des American College of Rheumatology für die cP von 1987: 85 Frauen (63% von 134 Patienten), Alter im Median 50 Jahre. AU wurde entsprechend den Patientenangaben zur „Krankschreibung“ dokumentiert. Bei der im Median 7 Monate bestehenden Erkrankung trat bisher wenigstens einmal AU wegen cP bei 102 Patienten (76%) auf. Sie bestand bei den M?nnern für ca. 11 Tage pro Monat, d.h. ca. ein Drittel der Krankheitszeit, und bei den Frauen für 8 Tage pro Monat entsprechend ca. einem Viertel der seit Beginn der cP vergangenen Zeit. AU wegen cP dauerte fast fünfmal l?nger als aufgrund der Referenzdaten zu erwarten war. über die „Krankschreibung“ wegen cP hinaus waren diese Patienten wegen anderer Erkrankungen etwa im gleichen Ausma? wie in die Referenzpopulation arbeitsunf?hig. Der gro?e Anteil der von Arbeitsunf?higkeit wegen cP betroffenen Patienten und die Dauer der AU verdeutlichen das Ausma? gravierender Beeintr?chtigungen der Erwerbst?tigkeit bereits im ersten Jahr der cP. Eingegangen: 21. M?rz 1996 Akzeptiert: 11. November 1996     

Linkage studies and mutation analysis of the PDEB gene in 23 families with Leber congenital amaurosis.

The phenotype in the rd mouse is similar to the clinical presentation of Leber congenital amaurosis (LCA) in humans. Recently a nonsense mutation in the beta subunit of the cGMP phosphodiesterase (Pdeb) gene has been defined as the cause for the rd phenotype in the mouse and has raised the question as to whether mutations in the human PDEB gene might cause LCA. We have previously cloned and characterized the human homologue of the mouse Pdeb gene and have mapped it to chromosome 4p16.3. In this study, a total of 23 LCA families of various ethnic backgrounds have been investigated. Linkage analysis using highly polymorphic (CA)n microsatellites has excluded the PDEB gene as a cause for LCA in 6 families. In the remaining 17 families, we have searched for mutations in the 22 exons of the PDEB gene using single-strand gel electrophoresis (SSGE). Multiple exonic polymorphisms have been determined. However, no DNA changes in the PDEB gene have been identified in our study population which could be causative for the LCA phenotype.