Dega-Osteotomie bei Hüftgelenkdysplasie

Zusammenfassung Operationsziel überdachung des Femurkopfes bei Hüftgelenkdysplasie. Indikationen Dysplastisches Acetabulum bei Patienten mit neuromuskul?ren und nichtneuromuskul?ren Erkrankungen. übergro?es, flaches Acetabulum. Fehlen einer lateralen und kranialen überdachung. Kontraindikationen Y-Fuge geschlossen, Wachstum abgeschlossen. Stark verformter Femurkopf. Operationstechnik Modifizierter Zugang nach Salter/Smith-Petersen. Freilegung und Teilung der Apophyse des Beckenkammes mit einem Messer. Freilegung der Darmbeinschaufel. Osteotomie der ?u?eren Wand des Iliums; sie wird vorsichtig mit Hilfe eines gebogenen mei?els nach unten gebogen und in dieser Stellung durch Einsetzen von trikortikalen Beckenkammsp?nen gehalten. Eine Osteosynthese ist nicht notwendig. Becken-Bein-Gipsverband mit Einschlu? des gegenseitigen Oberschenkels für sechs Wochen. Ergebnisse Zwischen 1987 und 1997 wurden 26 Hüften von 23 Patienten operiert. überwiegend handelte es sich um Kinder mit spastischen Paresen. Folgende zus?tzliche Eingriffe wurden vorgenommen: Offene Reposition zehnmal, Femurosteotomie 18mal und Tenotomien sowie Muskelabl?sungen 15mal. Der Durchschnittswert des azetabul?ren Index verbesserte sich von 32° auf 22°, der durchschnittliche Wert des Kopfzentrum-Pfannenrand-Winkels von −25° auf 22° und der durchschnittliche Migrationsindex von 62% auf 69%. Als Komplikationen wurden beobachtet: eine erneute Subluxation, ein frühzeitiger Verschlu? des Y-Knorpels, eine Fraktur des anderen Femur, einmal heterotope Ossifikationen und einmal eine Infektion der Harnwege.     

Optic nerve pseudomeningioma secondary to localized amyloidosis

A 55-year-old woman presented with a mass of the optic nerve sheath suggestive of a meningioma. On excision, this proved to be amyloid. No systemic involvement could be found. Although amyloid may arise as a solitary lesion of the orbit or extraocular muscle, this "pseudomeningiomatous" pattern has not been previously noted. A magnetic resonance scan was helpful because the amyloid imaged at a density similar to vitreous and cerebral white matter, unlike meningiomas, which appear more like bone. The diagnosis of localized amyloidosis, however, still remains dependent on histopathology and cannot be made on a clinical basis.     

Lymphotoxin but not tumor necrosis factor functions to maintain splenic architecture and humoral responsiveness in adult mice

To compare the function of the tumor necrosis factor (TNF) and lymphotoxin (LT)α/β systems in the mature immune system, these two pathways were blocked with soluble receptor-immunoglobulin (R-Ig) fusion proteins in normal adult mice. Inhibition of LTα/β signaling using LTβR-Ig or a blocking monoclonal antibody against murine LTβ had profound effects. The spleen lacked discrete B cell follicles and the marginal zone was altered. Less marked changes were detected in lymph nodes. LTα/β inhibition also prevented germinal center formation in the spleen and impaired Ig production in response to sheep red blood cells (SRBC) immunization. These results show that the LTα/β system is required for the maintenance of splenic architecture and normal immune responses, and not simply for the development of peripheral immune organs during ontogeny. In contrast, inhibition of the TNF/LTα pathway with TNF-R55-Ig did not affect the splenic architecture or the anti-SRBC response. Splenic defects and impaired antibody responses are seen in TNF-deficient mice, suggesting that TNF is important during development. Therefore relative to TNF, the LT system has the dominant influence on splenic organization and anti-SRBC Ig formation in the adult mouse.     

Differential expression of E-cadherin in lobular and ductal neoplasms of the breast and its biologic and diagnostic implications

We studied the pattern of E-cadherin expression in 183 invasive carcinomas (100 ductal, 42 lobular, 41 with mixed ductal and lobular features) and 198 in situ carcinomas (131 ductal, 53 lobular, 14 in situ with ductal and lobular features) by immunohistochemistry. We found a highly significant correlation of E-cadherin membrane expression with the histologic phenotype of the tumors. While moderate to strong membrane expression of E-cadherin was seen in all invasive and in situ ductal carcinomas, 41 of 42 invasive and 50 of 53 in situ lobular carcinomas showed complete loss of expression. All in situ carcinomas diagnosed histologically as showing mixed ductal and lobular features demonstrated complete loss of staining. Invasive carcinomas with ductal and lobular features showed 3 staining patterns: (1) complete or almost complete lack of membrane staining similar to that seen in lobular carcinomas, (2) uniform membrane expression throughout the tumor similar to ductal carcinomas, and (3) focal loss of E-cadherin staining, which correlated well with the histologic impression of focal lobular features. In tumors with histologically equivocal features, immunohistochemical detection of E-cadherin expression can be a useful diagnostic tool for the differentiation of ductal and lobular carcinomas of the breast.     

Predominance of null mutations in ataxia-telangiectasia

Ataxia-telangiectasia (A-T) is an autosomal recessive disorder involving cerebellar degeneration, immunodeficiency, chromosomal instability, radiosensitivity and cancer predisposition. The responsible gene, ATM, was recently identified by positional cloning and found to encode a putative 350 kDa protein with a Pl 3-kinase-like domain, presumably involved in mediating cell cycle arrest in response to radiation-induced DNA damage. The nature and location of A-T mutations should provide insight into the function of the ATM protein and the molecular basis of this pleiotropic disease. Of 44 A-T mutations identified by us to date, 39 (89%) are expected to inactivate the ATM protein by truncating it, by abolishing correct initiation or termination of translation, or by deleting large segments. Additional mutations are four smaller in-frame deletions and insertions, and one substitution of a highly conserved amino acid at the Pl 3-kinase domain. The emerging profile of mutations causing A-T is thus dominated by those expected to completely inactivate the ATM protein. ATM mutations with milder effects may result in phenotypes related, but not identical, to A-T.