Laparoscopic partial adrenalectomy for bilateral pheochromocytomas in a boy with von Hippel-Lindau disease

Objectives: In adults, increasing numbers of adrenalectomies for pheochromocytomas are performed laparoscopically. We report for the first time laparoscopic bilateral subtotal adrenalectomy for pheochromocytomas in an 8-year-old boy with von Hippel-Lindau disease. Methods, In July 1998, an 8-year-old boy with von Hippel-Lindau disease underwent laparoscopic adrenal-sparing surgery for bilateral pheochromocytomas. The boy presented with severe hypertension and two pheochromocytomas on both sides. Results: The child could be solely treated with laparoscopic adrenal-sparing surgery. The procedure was completed as planned. There were absolutely no intraoperative or postoperative complications. Postoperatively, catecholamine levels and hypertension went back to normal. At follow-up no residual tumor could be detected and no steroid replacement therapy was necessary. Conclusions: In experienced hands, laparoscopic adrenal-sparing surgery for pheochromocytomas is feasible and safe. Moreover, this minimal invasive approach represents an exceptional improvement in life quality, especially in children with von Hippel-Lindau disease since surgery will probably be necessary again and again in their future life. Copyright (C) 2000 S. Karger AG, Basel

Diverse and rich fortified cultural heritage of the Iberian Peninsula. Basis for culture tourism with the European Culture Route Fortified Monuments FORTE CULTURA®

[EN] Fortresses are architectural pearls, cultural sites, event locations, experience places and memorials, mostly situated at breath-taking places on mountains, rivers or in the under-ground. Fortresses are monuments of common European history, they mirror the past into the present, connect cultures and offer deep insights into the historical conflicts. Fortified monuments are part of what makes Europe unique and attractive. This cultural heritage has to be preserved and made accessible for the culture tourism at the same time. The Iberian fortified heritage has big potential for new culture touristic topics and travel routes away from mass tourism. Therefore, cultural routes are a useful instrument. The European Culture Route Fortified Monuments –FORTE CULTURA®– is the European umbrella brand for fortress tourism. It offers useful instruments for international marketing of fortified monuments. The implementation of the attractive architectura militaris of the Iberian Peninsula into the culture route FORTE CULTURA® makes it possible to network this culture asset touristically, make it visible and experienceable on international tourism markets and market it Europe-wide. By implementing a new touristic regional brand “FORTE CULTURA – Iberian Fortified Heritage” the qualified culture tourism will be addressed. This supports a balance between over and under presented monuments and extends the sphere of activity of local actors onto whole Europe.Neumann, H.; Röder, D.; Röder, H. (2020). Diverse and rich fortified cultural heritage of the Iberian Peninsula. Basis for culture tourism with the European Culture Route Fortified Monuments FORTE CULTURA®. Editorial Universitat Politècnica de València. 971-976. https://doi.org/10.4995/FORTMED2020.2020.11394OCS97197

Genetic stratification of inherited and sporadic phaeochromocytoma and paraganglioma: implications for precision medicine.

Over the past two decades advances in genomic technologies have transformed knowledge of the genetic basis of phaeochromocytoma and paraganglioma (PPGL). Though traditional teaching suggested that inherited cases accounted for only 10% of all phaeochromocytoma diagnosis, current estimates are at least three times this proportion. Inherited PPGL is a highly genetically heterogeneous disorder but the most frequently results from inactivating variants in genes encoding subunits of succinate dehydrogenase. Expanding knowledge of the genetics of PPGL has been translated into clinical practice by the provision of widespread testing for inherited PPGL. In this review, we explore how the molecular stratification of PPGL is being utilized to enable more personalized strategies for investigation, surveillance and management of affected individuals and their families. Translating recent genetic research advances into clinical service can not only bring benefits through more accurate diagnosis and risk prediction but also challenges when there is a suboptimal evidence base for the clinical consequences or significance of rare genotypes. In such cases, clinical, biochemical, pathological and functional imaging assessments can all contribute to more accurate interpretation and clinical management.We apologise to all the authors whose work we were unable to cite because of space constraints. We thank Dr Birke Bausch, University of Freiburg, Germany and Anna Roslyakowa, Department of Surgery, Endocrinology Research Center, Moscow, Russia for their expert input. RC acknowledges support from AMEND and GIST Support UK. ERM acknowledges support from European Research Council (Advanced Researcher Award), NIHR (Senior Investigator Award and Cambridge NIHR Biomedical Research Centre) and Cancer Research UK Cambridge Cancer Centre. The views expressed are those of the authors and not necessarily those of the NHS or NIHR. The University of Cambridge has received salary support in respect of EM from the NHS in the East of England through the Clinical Academic Reserve

Dynamic contrast enhancement of paragangliomas of the head and neck: evaluation with time-resolved 2D MR projection angiography

Abstract.: Our objective was to evaluate tumor hemodynamics of paragangliomas of the head and neck. A 2D magnetic resonance projection angiography (MRPA) protocol was developed. Six patients with histologically proven paragangliomas were included in the study. The sequence protocol and data post-processing procedure were optimized in view of a high temporal resolution and maximum contrast-to-noise ratio. Image-time series of two freely orientated slabs could be obtained. Correlation analysis was performed to generate selective arterial and venous weighted angiographic images. Glomus tumors showed a rapid and intense homogeneous enhancement following the intravenous administration of contrast material. The smallest tumors investigated measured 10mm and could be visualized clearly. Time-resolved analysis of the tumor enhancement revealed an early bolus arrival within the vascular bed of the tumor and prolonged incomplete washout indicating blood pooling. The dynamic contrast enhancement of glomus tumors can be evaluated non-invasively with MRPA providing a high temporal resolution and high image quality. The characteristic contrast enhancement of glomus tumors can be helpful in the diagnostic workup of lesions that may mimic glomus tumor

Effekte der Umstellung auf ökologischen Landbau auf die Segetalflora zweier Ackerbaubetriebe in Schleswig-Holstein

On two farms in Northern Germany the number of weed species in arable fields was about twice as large three to five years after conversion to organic agriculture compared with the last year of conventional cropping. Results indicate positive effects of organic farming on the weed flora although rare species are still lacking to date

Max Schottelius : Pioneer in Pheochromocytoma

First descriptions of diseases attract tremendous interest because they reveal scientific insight even in retrospect. Max Schottelius, the pathologist contributing the first histological description of pheochromocytoma, remains anonymous. We reviewed the description by Schottelius and weighed the report in modern context. Schottelius described the classical diagnostic elements of pheochromocytoma, including the brown appearance after exposure to chromate-containing Mueller's fixative. This color change, known as chromaffin reaction, results fromoxidation of catecholamines and is reflected in the name pheochromocytoma, meaning dusky-colored chromate-positive tumor. Thus Schottelius performed the first known histochemical contribution to diagnosis, which is today standard with immunohistochemistry for chromogranin. Copyright (c) 2017 Endocrine Society This article has been published under the terms of the Creative Commons Attribution NonCommercial, No-Derivatives License (CC BY-NC-ND).Peer reviewe

FLT3 mutations in Early T-Cell Precursor ALL characterize a stem cell like leukemia and imply the clinical use of tyrosine kinase inhibitors

Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) has been identified as high-risk subgroup of acute T-lymphoblastic leukemia (T-ALL) with a high rate of FLT3-mutations in adults. To unravel the underlying pathomechanisms and the clinical course we assessed molecular alterations and clinical characteristics in a large cohort of ETP-ALL (n = 68) in comparison to non-ETP T-ALL adult patients. Interestingly, we found a high rate of FLT3-mutations in ETP-ALL samples (n = 24, 35%). Furthermore, FLT3 mutated ETP-ALL was characterized by a specific immunophenotype (CD2+/CD5-/CD13+/CD33-), a distinct gene expression pattern (aberrant expression of IGFBP7, WT1, GATA3) and mutational status (absence of NOTCH1 mutations and a low frequency, 21%, of clonal TCR rearrangements). The observed low GATA3 expression and high WT1 expression in combination with lack of NOTCH1 mutations and a low rate of TCR rearrangements point to a leukemic transformation at the pluripotent prothymocyte stage in FLT3 mutated ETP-ALL. The clinical outcome in ETP-ALL patients was poor, but encouraging in those patients with allogeneic stem cell transplantation (3-year OS: 74%). To further explore the efficacy of targeted therapies, we demonstrate that T-ALL cell lines transfected with FLT3 expression constructs were particularly sensitive to tyrosine kinase inhibitors. In conclusion, FLT3 mutated ETP-ALL defines a molecular distinct stem cell like leukemic subtype. These data warrant clinical studies with the implementation of FLT3 inhibitors in addition to early allogeneic stem cell transplantation for this high risk subgroup

Розвиток духовності особистості в процесі фахової підготовки майбутніх учителів образотворчого мистецтва

(uk) У статті наголошується, що вчитель образотворчого мистецтва виступає носієм не лише спеціальних знань та умінь, але і носієм духовних цінностей. Духовне збагачення і вдосконалення кожної особистості відбувається протягом всього життя в тому числі і в процесі навчання. Гуманізація освіти та естетичне виховання спрямовано на формування гармонійної цілісної особистості, загальнокультурний її розвиток. Засобами естетичного виховання в мистецтві виступає художній образ. Художній образ є відображенням дійсності яке містить в собі не лише суб’єктивний досвід автора, його розуміння та відношення до об’єктів дійсності, але і відбитки культурно-історичного досвіду, естетичних цінностей, але і відбитки культурно-історичного досвіду, естетичних цінностей соціуму загалом. На їх базі і формується всебічно та гармонійно розвинута особистість із вищими моральними цінностями, естетичними канонами та ідеалами.(ru) В статье делается акцент на то, что учитель изобразительного искусства выступает носителем не только специальных знаний и умений, но и носителем духовных ценностей. Духовное обогащение и совершенствование каждой личности происходит на протяжении всей жизни в том числе и в процессе обучения. Гуманизация образования и эстетическое воспитание направлены на формирование гармоничной целостной личности и ее общекультурное развитие. Средством эстетического воспитания в искусстве выступает художественный образ. Художественный образ является отражением действительности, которое содержит в себе не только субъективный опыт автора, его понимание и отношение к объектам действительности, но и отпечатки культурно-исторического опыта, эстетических ценностей социума в целом. На их базе и формируется всесторонне и гармонично развитая личность с высокими моральными ценностями, эстетическими канонами и идеалами.(en) Importance of education in society is determined by the need to raise the national consciousness of the saved and nurturing genuine citizen. Spiritual enrichment and improvement of each individual occurs throughout life including during training. Spirituality determines the direction of all mental, emotional, sensual, strong-willed human qualities and its ability to self yourself as a person. Master of Fine Arts acting carrier not only specialized knowledge and skills, but also a bearer of spiritual values. Humanizing education and aesthetic education aims at forming a harmonious whole person, her general cultural development. Art is an integral part of spiritual culture, a reflection of the artistic representations of the human form of the world of reality. The means of aesthetic education in the art of acting artistic image. Artistic image is a reflection of reality, which contains not only the subjective experience of the author, and understanding related to the objects of reality, but the prints are of cultural and historical experience, the aesthetic values of society as a whole. At their base is formed fully and harmoniously developed personality with higher moral values, aesthetic canons and ideals

Umstellen auf Öko-Landbau: Informationen für die Praxis

Die Broschüre gibt grundlegende Hinweise zum Ablauf einer Umstellung auf die ökologische Wirtschaftsweise. Sie beschreibt die gesetzlichen Rahmenbedingungen, die Spezifika bei Pflanzenbau und Tierhaltung, die Bedeutung der Absatzwege und die finanzielle Förderung im Freistaat Sachsen. Adressen zu Kontrollstellen, Beratung und Verbänden sowie eine Zusammenstellung wichtiger Fachliteratur sind für die frühe Informations- und Planungsphase nützlich