Wegener-Granulomatose im Kindes- und Jugendalter

Wegener’s granulomatosis (WG) is a necrotising granulomatous small vessel vasculitis with a clinical predilection for the involvement of the upper airways, lungs and kidneys. It occurs at all ages. The pathogenesis of WG is determined by the pathological activation of phagocytes during transmigration through the vessel. Whereas most aspects of WG are similar at all ages, some features appear to be significantly different. WG in childhood is more frequently complicated by subglottic stenosis and nasal deformity, while treatment related morbidity is less common compared to adults. The introduction of combined treatment with cyclophosphamide and glucocorticoids has resulted in a dramatic improvement in patient outcome; however, commonly occurring disease relapses and the risk of chronic organ damage at all ages make long-term follow-up of all patients necessary.     

Suprakondyläre Humeruspseudarthrosen

The standard surgical protocol presented, with resection of the bone that has not healed. stable osteosynthesis with locking plates, and autologous bone graft augmentation, is an established and successful method of treating atrophic nonunion of the distal humerus. Bone healing was observed in all patients, and a single surgical operation was sufficient. In addition, elbow joint arthrolysis was performed to resect joint fibrosis and ossification and release tissue contractures. With this combined approach bony healing and improvement in range of motion and function of the elbow joint were achieved.     

Schlaf bei neurodegenerativen Erkrankungen

Zusammenfassung Die neurodegenerativen Erkrankungen bestehen aus einer Gruppe heterogener, progredient verlaufender Erkrankungen unterschiedlicher Ätiologie, die ein oder mehrere Systeme beeinträchtigen. Sie treten überwiegend im höheren Lebensalter auf, in dem sich zusätzlich sowohl die Art wie auch das Ausmaß des Schlafes ändern. Die neurodegenerativen Prozesse verursachen strukturelle Veränderungen der Schlaf-Wach-Generatoren im Hirnstamm, die Schlafstörungen wie Tagesschläfrigkeit, Insomnie, nächtliche bewegungs- und schlafbezogene Atmungsstörungen sowie Störungen des zirkadianen Schlaf-Wach-Rhythmus zur Folge haben können. Bei manchen neurodegenerativen Erkrankungen sind im Vorfeld der Krankheitsmanifestation auftretende Schlafstörungen bereits Krankheitsprädiktoren. Polysomnographisch finden sich Schlaffragmentierung, tonische oder phasische Beinbewegungen, Störungen der Atemmuskulatur, verminderter Tiefschlaf, Abwesenheit von REM-Schlaf oder REM-Schlaf ohne Muskelatonie, vermehrte Arousal- und Weckreaktionen, epileptiforme EEG-Aktivität oder schlafbezogene Atmungsstörungen. Sehr häufig sind REM-Schlaf-Verhaltensstörungen assoziiert mit neurodegenerativen Erkrankungen. In dieser Übersichtsarbeit werden Symptomatik, Pathophysiologie und polysomnographische Befunde von Schlafstörungen häufiger neurodegenerativer Erkrankungen vorgestellt.     

Prediction of phenotype for acetylation and for debrisoquine hydroxylation by DNA-tests in healthy human volunteers

Summary The debrisoquine/sparteine-type polymorphism of drug oxidation and the polymorphism for acetylation are two common inherited variations in human drug metabolism. The phenotypes for hydroxylation and acetylation can be predicted be newly developed methods based on mutation-specific amplification of DNA by the polymerase chain reaction (PCR), which also allow for identification of heterozygous carriers of one mutant allele.In the present study, the results of genotyping of 81 healthy European volunteers were compared with the phenotype obtained by the classical biochemical approach using debrisoquine and caffeine as probe drugs.Genotyping correctly predicted all 73 extensive metabolisers (EMs) and 6 out of 8 poor metabolisers (PMs) of debrisoquine. All 48 rapid acetylators and 33 of 35 slow acetylators were predicted.Overall, the DNA analysis result matched the in vivo phenotype in 97.5 % of individuals.     

Ein Beitrag zur aktuellen Antidementivadiskussion in Deutschland

In Germany, the role of acetylcholinesterase inhibitors in the treatment of Alzheimer’s disease (AD) has become a topic of recent discussion. The present article addresses issues which, in the opinion of the authors, have not received sufficient attention. These include the distinction between statistical and clinical significance, outcome parameters, the duration of clinical trials, variability in treatment response and the definition of treatment responders. The authors argue that these issues need to be considered in an in-depth evaluation of acetylcholinesterase inhibitors in the treatment of AD.     

Inzidentalome der Nebenniere

Adrenal incidentalomas are clinically inapparent masses detected incidentally with imaging studies conducted for other reasons. They are relatively common and require structured diagnostic workup. In many cases surveillance is warranted. The diagnostic workflow has to reveal whether the mass is hormonally functioning and/or if there is evidence of malignancy. If the tumor is functionally silent and not larger than 4 cm, surveillance is warranted. Functioning tumors and masses larger than 6 cm have to be resected. Fine-needle aspiration biopsy is indicated in very rare cases, but pheochromocytoma has to be ruled out first.     

Regulation der Laktation

At birth the human newborn is immature and has not yet acquired fully competent organ functions. During lactation especially the digestive tract and the immune system gain competence. Lactation is regulated by a complex interaction of numerous hormonal factors. Most important are probably prolactin and oxytocin—two hormones produced and secreted in the pituitary. The production and secretion of these two hormones is again regulated by several factors in the central nervous system and these mechanisms can be disturbed by a variety of exogenous factors such as drugs and any other substances interacting with dopamine metabolism in the brain. It is important to know these physiological interactions to be able to understand the regulation of lactation and to be able to assess which factors could interfere with this process.     

Total-videoendoskopische Strumaresektion via“axillobilateral breast approach”

BACKGROUND: The axillobilateral breast approach (ABBA) is a procedure allowing thyroid resection without scarring at the neck. We operated on a series of 26 patients with this technique. METHOD: Via incisions at the edge of the mamilla and axilla, trocars are placed subcutaneously under the platyma. Dissection is performed bluntly and with an ultrasonographic scalpel under videoscopic control. The procedure itself corresponds to conventional surgery. The specimen is removed through the axillary trocar. RESULTS: Twenty-six female patients underwent thyroid resection using the ABBA technique. Subtotal resection was performed in 24. Mean operation times were 111 min (unilateral) and 187 min (bilateral). In none of these cases was conversion necessary. One transient recurrent laryngeal nerve palsy and one paresis of the arm plexus were found postoperatively. CONCLUSION: In selected patients the ABBA technique is feasible and safe with the mandatory radicalness. The primary aim of this method is the cosmetic result.