Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature

<p>Abstract</p> <p>Background</p> <p>Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings.</p> <p>Case Presentation</p> <p>We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy.</p> <p>Conclusions</p> <p>Our results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH.</p

Dlk/ZIP kinase-induced apoptosis in human medulloblastoma cells: requirement of the mitochondrial apoptosis pathway

Dlk/ZIP kinase is a member of the Death Associated Protein (DAP) kinase family of pro-apoptotic serine/threonine kinases that have been implicated in regulation of apoptosis and tumour suppression. Expression of both Dlk/ZIP kinase and its interaction partner Par-4 is maintained in four medulloblastoma cell lines investigated, whereas three of seven neuroblastoma cell lines have lost expression of Par-4. Overexpression of a constitutively pro-apoptotic deletion mutant of Dlk/ZIP kinase induced significant apoptosis in D283 medulloblastoma cells. Cell death was characterized by apoptotic membrane blebbing, and a late stage during which the cells had ceased blebbing and were drastically shrunken or disrupted into apoptotic bodies. Over-expression of the anti-apoptotic Bcl-xL protein had no effect on Dlk/ZIP kinase-induced membrane blebbing, but potently inhibited Dlk/ZIP kinase-induced cytochrome c release and transition of cells to late stage apoptosis. Treatment with caspase inhibitors delayed, but did not prevent entry into late stage apoptosis. These results demonstrate that Dlk/ZIP kinase-triggered apoptosis involves the mitochondrial apoptosis pathway. However, cell death proceeded in the presence of caspase inhibitors, suggesting that Dlk/ZIP kinase is able to activate alternative cell death pathways. Alterations of signal transduction pathways leading to Dlk/ZIP kinase induced apoptosis or loss of expression of upstream activators could play important roles in tumour progression and metastasis of neural tumours. © 2001 Cancer Research Campaign http://www.bjcancer.co

DNA methylation-based classification of central nervous system tumours.

Accurate pathological diagnosis is crucial for optimal management of patients with cancer. For the approximately 100 known tumour types of the central nervous system, standardization of the diagnostic process has been shown to be particularly challenging-with substantial inter-observer variability in the histopathological diagnosis of many tumour types. Here we present a comprehensive approach for the DNA methylation-based classification of central nervous system tumours across all entities and age groups, and demonstrate its application in a routine diagnostic setting. We show that the availability of this method may have a substantial impact on diagnostic precision compared to standard methods, resulting in a change of diagnosis in up to 12% of prospective cases. For broader accessibility, we have designed a free online classifier tool, the use of which does not require any additional onsite data processing. Our results provide a blueprint for the generation of machine-learning-based tumour classifiers across other cancer entities, with the potential to fundamentally transform tumour pathology

Einsatz des Brainlab Kolibri® Navigationssystems im Rahmen der endoskopischen Nasennebenhöhlenchirurgie in Lokalanästhesie

Einleitung: In einer randomisierten Studie wurde die Zuverlässigkeit und Benutzerfreundlichkeit des Kolibri®-Navigationssystem der Firma Brainlab bei endoskopischen NNH-Operationen in Lokalanästhesie untersucht.Material/Methoden: Das System wurde bei 35 Patienten, die wegen chronischer Sinusitis oder Polyposis nasi in LA operiert wurden, eingesetzt. Zum Vergleich wurden 35 Patienten ohne Navigationssystem operiert. Die intraoperative Genauigkeit wurde durch eine klinischen Plausibilitätsprüfung an definierten anatomischen Messpunkten überprüft. Mit einem standardisierten Fragebogen wurden Rüstzeit, Registrierungszeit und Referenzierungszeit dokumentiert.Ergebnisse: Die Rüstzeit für den Navigationscomputer lag bei ca. 5,8 Minuten. Die Zeit für Aufbau und Lagerung und Vorbereitung des Patienten betrug im Mittel 4,2 Minuten. Für die Referenzierung mit dem Z-Touch®-System wurden im Durchschnitt 4,2 Minuten benötigt. Die Referenzierung mit dem Soft-Touch®-System lag mit ca. 8,1 Minuten im Mittel ca. doppelt so hoch. Durch die Verwendung des Navigationssystems verlängerte sich die gesamte OP- und Vorbereitungszeit um ca. 50 %. Für die intraoperative Genauigkeit ergaben sich in allen Raumebenen durchschnittliche Werte von 1,5 bis 2,3 mm.Schlussfolgerung: Das Brainlab-Kolibri®-Navigationssystems zeichnet sich durch eine gute Genauigkeit, eine kurze Rüstzeit und einfache Registrierungsmodi aus. Diese Eigenschaften sowie die intuitive Bedienerführung prädestinieren das System für den routinemäßigen Einsatz bei NNH - Operationen.Die kompakten Abmessungen des Kolibri®-Navigationssystems begünstigen den Transfer in räumlich getrennte OP-Bereiche und erlauben den Einsatz in kleinen Eingriffsräumen (z.B. OP - Container in Feldlazaretten )

Einsatz des Brainlab Kolibri® Navigationssystems im Rahmen der endoskopischen Nasennebenhöhlenchirurgie in Lokalanästhesie

General view photographed 1931. Digitisation and record funded by the Pilgrim Trust. Original at 178C78.24