[Multiple myeloma with significant multifocal osteolysis in a dog without a detectible gammopathy]. / Multiples Myelom mit ausgeprägter multifokaler Osteolyse ohne nachweisbare Gammopathie bei einem Hund.

Description of a variant of multiple myeloma in a dog lacking the gammopathy normally associated with this type of neoplasm. A Border Collie mongrel was presented with symptoms of progressive hind-leg weakness, lethargy and tiredness, which had started to appear 6 weeks previously. Radiographic examination showed small osteolytic areas in the spinal column, but also diffuse small areas of increased opacity as well as evidence of decreased bone density in the pelvis and of both femoral necks. Moderate regenerative anaemia, hypogammopathy and hypercalcaemia were diagnosed. Computed tomography scans displayed multifocal osteolysis and bone destruction in the skull, spinal column, scapulae, proximal humeri, pelvis and femoral necks. H&E staining of the biopsies showed bone destruction and monomorphic plasmacyotid cell populations, causing infiltrative bone marrow lesions and osteolysis. In many areas neoplastic plasma cell infiltration of the bone marrow was 70% and in some areas reached 100%. The diagnosis was non-secretory multiple myeloma without apparent secretion of paraproteins into the blood.

Association of paraoxonase gene cluster polymorphisms with ALS in France, Quebec, and Sweden.

BACKGROUND: The paraoxonase gene cluster on chromosome 7 comprising the PON1-3 genes is an attractive candidate for association in amyotrophic lateral sclerosis (ALS) given the role of paraoxonase genes during the response to oxidative stress and their contribution to the enzymatic break down of nerve toxins. Oxidative stress is considered one of the mechanisms involved in ALS pathogenesis. Evidence for this includes the fact that mutations of SOD1, which normally reduce the production of toxic superoxide anion, account for 12% to 23% of familial cases in ALS. In addition, PON variants were shown to be associated with susceptibility to ALS in several North American and European populations. METHODS: We extended this analysis to examine 20 single nucleotide polymorphisms (SNPs) across the PON gene cluster in a set of patients from France (480 cases, 475 controls), Quebec (159 cases, 95 controls), and Sweden (558 cases, 506 controls). RESULTS: Although individual SNPs were not considered associated on their own, a haplotype of SNPs at the C-terminal portion of PON2 that includes the PON2 C311S amino acid change was significant in the French (p value 0.0075) and Quebec (p value 0.026) populations as well as all three populations combined (p value 1.69 x 10(-6)). Stratification of the samples showed that this variation was pertinent to ALS susceptibility as a whole, and not to a particular subset of patients. CONCLUSIONS: These findings contribute to the increasing weight of evidence that genetic variants in the paraoxonase gene cluster are associated with amyotrophic lateral sclerosis.

[Multi-sequential arterial chemoembolization of advanced hepatocellular carcinomas: computerized tomography follow-up parameters for evaluating effectiveness of therapy]. / Multisequentielle arterielle Chemoembolisation fortgeschrittener hepatozellulärer Karzinome: Computertomographische Verlaufsparameter zur Beurteilung des Ansprechens auf die Therapie.

PURPOSE: An evaluation of clinical and computed tomography parameters for the transarterial chemoembolization (TACE) of hepatocellular carcinoma (HCC). MATERIAL AND METHODS: Retro- and prospective analysis of 123 TACE procedures in 37 patients (mean age: 63.3 years). RESULTS: Overall mean survival rate for all treated patients was 785 days with a mean value of 387 days. The 1 year survival rate was 62%. Quantitative tumor volumetry revealed a significant difference in survival rate with a mean value of 678 days for patients with a reduction of tumor volume between 0 and 50%, 976 days for a volume reduction of 51-100% and 277 days for an increase in tumor volume during therapy. Patients with a high lipiodol retention presented improved survival data (902 days) versus patients with a low lipiodol retention (513 days). Homogeneous retention of lipiodol was a positive factor on survival as compared to an inhomogenous form of retention. CONCLUSION: In patients with a positive therapy response after TACE the prognosis can be improved by repeated embolization.

Clinical and genetic study of chronic (types II and III) childhood onset spinal muscular atrophy.

We have conducted a retrospective study of 63 patients affected by chronic forms of spinal muscular atrophy (SMA) to better document the natural history of this disease. Thirty-nine patients had type II and 24 type III SMA. These patients had manual muscle testing (MMT) and forced vital capacity (FVC) studies done every six to 12 months over follow up period ranging from six to 140 months. A decline in FVC was seen in both types of SMA but there was no significant change in MMT in either group. Genetic studies were also done in a subset of 17 families (23 patients) included in this study. Homozygous deletions in the telomeric survival motor neuron (SMN) and the neuronal apoptosis inhibitory protein (NAIP) genes were observed in 100% and 11.8% of the patients tested respectively.

[Prevention of motion sickness with a transdermal therapeutic system containing scopolamine. A randomized, comparative double-blind study in the German Federal Navy]. / Kinetoseprophylaxe mit TTS-Scopolamin. Eine randomisierte, vergleichende Doppelblindstudie bei der Bundesmarine.

To test the prophylactic value of anti-motion sickness drugs, a randomized double-blind trial was undertaken on 46 young, healthy, male volunteer marines. Comparison was made between a transdermal therapeutic system (TTS) containing as the effective agent scopolamine (TTS-scopolamine) and proprietary meclozine tablets. An artificial "sea voyage" served to produce motion sickness, each subject sitting by himself on an artificially tilting "island", on two days for 30 minutes. Without treatment, 19 of the 46 subjects developed symptoms of motion sickness requiring treatment. After administration of TTS-scopolamine or meclozine tablets (double-dummy technique) the motion sickness score was reduced by 89% and 59%, respectively. There was a reduction on the visual analog scale of 98% and 59%, respectively. Probability of error (Fisher's exact probability test) for assuming therapeutic advantage of TTS-scopolamine over meclozine tablets was 13.5%. A pre-set significance level of 5% was thus not reached. This trial shows that TTS-scopolamine, even in a brief exposure, has at least the same effectiveness as meclozine, in addition to avoiding the gastrointestinal tract and maintaining with certainty a constant blood level over three days.