An endobronchial inflammatory myofibroblastic tumor in a 10-yr-old child after allogeneic hematopoietic cell transplantation.

IMTs belong to the group of soft tissue tumor and could occur at any anatomical site; however, the causes and growth feature remain unclear. This case report documents a 10-yr-old male suffering from slowly developing dyspnea on exertion and cough around seven months post-HCT. He was diagnosed with an endobronchial tumor based on imaging, and histology confirmed ALK-positive submucosal spindle-shaped cells with infiltrative cells, compatible with IMT.

Congenital dyserythropoietic anemia type 1 with a novel mutation in the CDAN1 gene previously diagnosed as congenital hemolytic anemia.

The congenital dyserythropoietic anemias (CDAs) are a heterogeneous group of genetic disorders of red cell production. They are characterized by ineffective erythropoiesis and dyserythropoiesis. Here, we present the clinical description and mutation analysis of a Japanese female with CDA type 1.

Development of two-grating spectrometer for the charge exchange spectroscopy system on KSTAR.

The charge exchange spectroscopy (CES) system on Korea Superconducting Tokamak Advanced Research (KSTAR) was installed last year and had been applied to measure the C VI ion temperature and rotation velocity profiles. The ion temperature and rotation velocity profiles had been estimated from the C VI 5290.5 Å (n = 8-7) charge-exchange spectrum signal measured by a Czerny-Turner type spectrometer and a thinned back-illuminated charge coupled device (CCD) camera.

Solvent-assisted conformational isomerization and the conformationally-pure REMPI spectrum of 3-aminophenol.

3-Aminophenol (3AP) has two conformers, cis and trans, depending on the orientation of the OH group relative to the NH(2) group. While both conformers are found in the jet-cooled spectra of 3AP, only the trans isomer was found in the REMPI spectrum of the 3AP(NH(3))(1) cluster. It was suggested that the cis conformer of the cluster isomerizes to the more stable trans conformer in the ground state during supersonic expansion.

[End of life care in children with cancer and problems--pediatric palliative care at hospital and home].

We cared 28 life limiting children with cancer in the past five years. Two patients died at home and the rest of them died at hospitals. Most children older than 11 years understood their disease.

Comparative study of charge division in substituted benzene cations.

A recently proposed phenomenon of charge division in a molecular cation [K. T. Lee et al.

Female hyper IgM syndrome type 1 with a chromosomal translocation disrupting CD40LG.

Hyper-IgM syndrome type 1 (HIGM1) is a primary immunodeficiency characterized by recurrent bacterial and opportunistic infections, associated with normal or high serum level of IgM and decreased serum levels of IgG, IgA and IgE due to the defect of class switch recombination. CD40LG, located in Xq26, has been reported to be mutated in male HIGM1 patients. Here, we report the second case of a female HIGM1 with the defect of CD40 ligand (CD40L) expression and of soluble serum CD40L.

A case of idiopathic sclerosing mediastinitis in a 7-year-old Japanese boy.

Unlabelled: Sclerosing mediastinitis is a very rare benign disorder characterised by the development of dense fibrous tissue within the mediastinum. Affected patients are typically young adults with infant cases being uncommon especially in areas without endemic histoplasmosis. We report a Japanese boy with markedly elevated serum inflammatory markers for more than 1 year in the absence of any clinical manifestations.

[Membranous nephropathy following allogeneic peripheral blood stem cell transplantation in a boy with anaplastic large cell lymphoma].

We report the case of 13-year-old boy who had been diagnosed as having anaplastic large cell lymphoma (ALCL) when he was 11 years old. He suffered a relapse despite the chemotherapy regimens he had been subjected to. Since anaplastic lymphoma kinase (ALK), one of the important prognostic factors of ALCL, was not expressed in the tumor cells, allogeneic peripheral blood stem cell transplantation (PBSCT) from his HLA-matched elder brother was performed.

Characteristics of the cell membrane fluidity, actin fibers, and mitochondrial dysfunctions of frozen-thawed two-cell mouse embryos.

Physical and chemical alterations caused by the freezing and thawing and their effects on survivals/developments in vitro were investigated. Of a total of 452 two-cell mouse embryos, the overall survival rate of the frozen-thawed embryos was 76.1% (344/452).