Internal medicine
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A 49-year-old woman with a malignant solitary fibrous tumor of the pleura presented with hypoglycemia. Most of the serum insulin-like growth factor II (IGF-II) existed as high molecular weight IGF-II. ⋯ Immunohistochemically, IGF-II was localized in the so-called Golgi area of the tumor cell. These findings suggest that hypoglycemia in this patient was caused by the high molecular weight IGF-II produced by the tumor.
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Case Reports
Compound heterozygosity for alpha-1-antitrypsin (S(iiyama) and QO(clayton)) in an Oriental patient.
Alpha-1-antitrypsin (alpha1AT) deficiency is extremely rare among Orientals. We treated a 37-year-old man with severe pulmonary emphysema associated with a low serum concentration of alpha1AT. ⋯ The patient proved to be a compound heterozygote carrying a S(iiyama) deficient allele and a QO(clayton) null allele. This is the first Japanese case of alpha1AT deficiency to arise from such compound heterozygosity in a family with no apparent consanguineous marriage, suggesting that the gene frequency for deficient alleles might be somewhat higher than previously estimated.
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Lung transplantation has been performed successfully outside Japan since 1983 in patients with end-stage lung disease. More than 9,000 lung transplants have been reported in The Registry of the International Society for Heart and Lung Transplantation. In contrast, a transplant law became effective in Japan only recently, and four universities were designated as official lung transplant centers (Okayama, Osaka, Kyoto and Tohoku Universities). ⋯ Since then, seven lung transplants (four from living donors and three from cadaveric donors) have been successfully performed in Japan. Although lung transplantation offers acceptable prospects for 5-year survival, chronic rejection and donor shortage remain to be major problems. In an effort to address the donor shortage issue, living-donor lobar lung transplantations have been performed with satisfactory intermediate survival and functional results.