Neuropathy with lysosomal changes in Marinesco-Sj?gren syndrome: fine structural findings in skeletal muscle and conjunctiva.

The light- and electron-microscopical findings in the skeletal muscle and conjunctiva of 6 patients with Marinesco-Sj?gren syndrome (MSS) were presented. All patients were related and showed the cardinal clinical symptoms of the syndrome: congenital cataracts, mental retardation, delayed statomotor development, and cerebellar ataxia. The most prominent alteration found in the skeletal muscle of four patients was extensive neurogenic atrophy with conspicuous groups of atrophic muscle fibers. Additional findings were vacuolar degeneration and secondary, unspecific changes like slight mitochondrial alterations and increased variability in muscle fiber calibers. The ultrastructural examination revealed double-membrane structures near to, but not in direct contact with the nucleus. The nuclear changes described earlier (7, 20) were not found in any of the cases. Conjunctival biopsies revealed a marked increase in the number of lysosomes in fibroblasts. In summary, to the well known myopathic damage of muscles in MSS a neurogenic component should be added. Disturbed lysosomal function is to be considered as the basic abnormality, though the enzyme defect has not yet been identified. In cases of clinically suspected MSS, examination of a conjunctival biopsy is highly recommended.     

Management of Patients After Catheter Ablation of Ventricular Tachycardia

The management of patients after catheter ablation of ventricular tachycardia is not well defined. In this article we summarize recently published results and report our own experience. Factors influencing the clinical outcome of these patients and methods to identify patients with an increased risk of recurrence of ventricular tachycardia are discussed. Furthermore, a review is given on current concomitant therapeutic tools including antiarrhythmic drugs and the implantation of an automatic cardioverter defibrillator.     

Effect of titanium surface texture on the cell-biomaterial interface.

To improve the in vivo performance of engineered implants, this study examines the independent effects of surface chemistry and topography on fibroblast morphology and density in vitro. Titanium (Ti) was sputter-coated onto smooth and microtextured polyethylene terephthalate (PET). Test specimens were evaluated in 24-h, fibroblast cultures and assessed with scanning electron microscopy (SEM) for cellular morphology and density. Fibroblast density increased, as the Ti film thickness increased. The fibroblasts exhibited contact guidance on the textured test specimens. The greatest cellular density was found on the Ti-coated, textured test specimens. In conclusion, Ti and surface texture appeared to strongly influence fibroblast density and morphology as compared to PET and smooth surfaces.     

Stage C adenocarcinoma of the prostate. An analysis of 551 patients treated with external beam radiation

We retrospectively reviewed records of 551 patients with clinical Stage C prostatic adenocarcinoma treated with 60 to 70 Gy external beam radiation. Elective pelvic node irradiation was given to 247 patients (45%). Follow-up for all surviving patients ranged from 16 to 201 months (median, 6.5 years; mean, 7 years). The 5-, 10-, and 15-year uncorrected actuarial survival rates were 72%, 47%, and 27%, respectively. Disease-free survival rates were 59%, 46%, and 40% at the corresponding times. Actuarial local control rates were 88%, 81%, and 75% at 5, 10, and 15 years, respectively. Disease-free survival was adversely affected by high pathologic grade, disease fixed to the pelvic sidewall, invasion of the bladder, prior transurethral resection, hydronephrosis, and elevated serum levels of prostatic acid phosphatase and creatinine. Elective pelvic node irradiation did not improve the outcome. Complications of treatment were acceptable: minor anorectal and/or urinary symptoms, 11%; mild to moderate complications, 19%; serious problems requiring surgery, 3%. It is concluded that localized, high-energy external beam irradiation provides excellent local control of disease, low morbidity, and 5-, 10-, and 15-year survival rates that have not been rivaled by other treatment.     

Auditive Verarbeitungsgeschwindigkeit und Sprachleistungen

ZusammenfassungHintergrund Lernstörungen werden zunehmend auf Defizite bei der Sprachwahrnehmung und diese wiederum auf eine Verlangsamung der auditiven Verarbeitungsgeschwindigkeit zurückgeführt. In der Studie wird untersucht, ob sich bei Kindern mit Schulproblemen sprachliche Leistungen durch ein Zeitverarbeitungstraining verbessern lassen.Patienten und Methoden Bei 21 Kindern mit spezifischen Lernstörungen wurden über 8 Wochen Ordnungsschwelle und Richtungshören trainiert und im Vergleich zu einer Kontrollgruppe (n=21) Sprachleistungen bis 6 Monate danach überprüft.Ergebnisse Leistungssteigerungen wurden hinsichtlich der Ordnungsschwelle, nicht aber der sprachlichen Fähigkeiten beobachtet. Die Eltern schätzten die Wirkung des Trainings eher positiv ein.Schlussfolgerungen Ein Zeitverarbeitungstraining verbessert unmittelbar geübte Leistungen, nicht aber sprachliche Fähigkeiten. Von den Eltern wahrgenommene Verbesserungen sind nicht objektivierbar und als unspezifische Effekte zu bewerten. Die Ergebnisse sprechen insgesamt gegen die Wirksamkeit eines Zeitverarbeitungstrainings bei Kindern mit Schulschwierigkeiten.     

Primary osteosarcoma of the liver

Extraskeletal osteosarcomas are infrequent, and those that arise within parenchymal organs are very rare indeed. This case of a 71-year-old man with a primary osteosarcoma of the liver is the second reported in man. Pathologic findings, including ultrastructural features, at surgery and autopsy are presented. The differential diagnosis is discussed in detail.