Wilheim Krucke (1911-1988): On the Occasion of the 100(th) Birthday of the Great Neuropathologist on December 26, 2011

A short summary is presented on the life, professional development, research work, especially concerning peripheral nervous system, and personality of the former director of the Department of Neuropathology at the Max-Planck-Institute of Brain Research and Edinger Institute of the Johann Wolfgang Goethe-University, both in Frankfurt/Main

Chronic myelomonocytic leukemia in childhood: A retrospective analysis of 110 cases

Chronic myelomonocytic leukemia (CMML) is a rare hematopoietic malignancy of childhood. To define the clinical and hematologic characteristics of the disease, we performed a retrospective analysis of 110 children given the diagnosis CMML irrespective of karyotype. Median age at diagnosis was 1.8 years. Neurofibromatosis type 1 was known in 14% and other clinical abnormalities in 7% of the children. At presentation, the medium white blood count was 35 x 109/L, with a median monocyte count of 7 x 109/L. Karyotypic abnormalities in bone marrow cells were noted in 36% of the patients, whereas 26% of the children had monosomy 7. Children with monosomy 7 did not differ from those with normal karyotype with respect to their clinical presentation. However, they did display some characteristic hematologic features. Of 110 children, 38 received an allogeneic bone marrow transplant (BMT). The probability of survival at 10 years was 0.39 (standard error [SE] = 0.10) for the BMT group and 0.06 (SE = 0.4) for the 72 patients of the non-BMT group. Platelet count, age, and hemoglobin F at diagnosis were the main predicting factors for the length of survival in the non-BMT group. There is a strong need for a broad agreement on nomenclature in children with myelodysplastic syndromes (MDS). We propose here to use the French-American- British classification for MDS in childhood

Chronic myelomonocytic leukemia in childhood. A retrospective analysis of 110 cases.

none16Chronic myelomonocytic leukemia (CMML) is a rare hematopoietic malignancy of childhood. To define the clinical and hematologic characteristics of the disease, we performed a retrospective analysis of 110 children given the diagnosis CMML irrespective of karyotype. Median age at diagnosis was 1.8 years. Neurofibromatosis type 1 was known in 14% and other clinical abnormalities in 7% of the children. At presentation, the medium white blood count was 35 x 10(9)/L, with a median monocyte count of 7 x 10(9)/L. Karyotypic abnormalities in bone marrow cells were noted in 36% of the patients, whereas 26% of the children had monosomy 7. Children with monosomy 7 did not differ from those with normal karyotype with respect to their clinical presentation. However, they did display some characteristic hematologic features. Of 110 children, 38 received an allogeneic bone marrow transplant (BMT). The probability of survival at 10 years was 0.39 (standard error [SE] = 0.10) for the BMT group and 0.06 (SE = 0.4) for the 72 patients of the non-BMT group. Platelet count, age, and hemoglobin F at diagnosis were the main predicting factors for the length of survival in the non-BMT group. There is a strong need for a broad agreement on nomenclature in children with myelodysplastic syndromes (MDS). We propose here to use the French-American-British classification for MDS in childhoodnoneCM. NIEMEYER; M. ARICÒ; G. BASSO; A. BIONDI; A. CANTÙ-RAJNOLDI; U. CREUTZIG; O. HAAS; J. HARBOTT; H. HASLE; G. KERNDRUP; F. LOCATELLI; G. MANN; B. STOLLMAN-GIBBELS; ETH. VAN'T VEER-KORTHOF; E. VAN WERING; M. ZIMMERMANNNiemeyer, C. M.; M., Aricò; Basso, Giuseppe; A., Biondi; A., CANTÙ RAJNOLDI; U., Creutzig; O., Haas; J., Harbott; H., Hasle; G., Kerndrup; F., Locatelli; G., Mann; B., STOLLMAN GIBBELS; VAN'T VEER KORTHOF, E. T. H.; E., VAN WERING; M., Zimmerman

The Result: A New Design Paradigm

One of the key challenges faced by engineers is finding, concretizing, and optimizing solutions for a specific technical problem in the context of requirements and constraints (Pahl et al. 2007). Depending on the technical problem’s nature, specifically designed products and processes can be its solution with product and processes depending on each other. Although products are usually modeled within the context of their function, consideration of the product’s life cycle processes is also essential for design. Processes of the product’s life cycle concern realization of the product (e.g., manufacturing processes), processes that are realized with the help of the product itself (e.g., use processes) and processes at the end of the product’s life cycle (recycling or disposal). Yet, not just product requirements have to be considered during product development, as requirements regarding product life cycle processes need to be taken into account, too. Provision for manufacturing process requirements plays an important role in realizing the product’s manufacturability, quality, costs, and availability (Chap. 3). Further life cycle demands, such as reliability, durability, robustness, and safety, result in additional product and life cycle process requirements. Consequently, the engineer’s task of finding optimal product and process solutions to solve a technical problem or to fulfill a customer need is characterized by high complexity, which has to be handled appropriately (Chaps. 5 and 6)