Osler氏病樣出血性素因の1例

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Heretofore, in Japan only 4 lineages with authentic Oslers disease have been reported. The following case also is thought to be Oslers disease with supervention of vascular purpura.The patient is a 28 years old divorcee. A younger brother of a different mother died at 15 from hemoptysis of unknown cause and his younger sister consulted us at 19 on account of continual epistaxes.The patient often noticed gingival hemorrhages since the grade school period. Menarche was at 13. Epistaxes appeared repeatedly for one year since the age of 17, and from the age of 19 the sputum began to be streaked with blood but there was no evidence of pulmonary tuberculosis. At the age of 20 sugillations were recognized at times. From about the age of 23 a dull pain was felt in the renal region and hematuria was recognized at 25. At 26 she was discovered to have gastroptosis and wandering kidney.As hematemeses and hemoptyses appeared repeatedly from about May, 1952, she was admitted to our hospital on June 12. Linear form telangiectases were observed in the alae of the nose, both shoulders, interscapular spaces and femoral regions, and Kiesselbachs area. In the right lateral chest was recognized a single spot consisting of grouped telangiectases. An occult blood test of the feces was positive. Bleeding time was 9 minutes. Tourniquet test was positive. Coagulation time, prothrombin time, and clot retraction were normal. Liver function tests positive. The hemorrhages after admission are illustrated in figure 1 in the mani text. VitaminC, rutin, and ethinylestradiol had little effect but vitamin E had some good effect.In this case the fact that vesicles appeared in the skin and lips by rubbing suggests that there was an abnormality of the general connective tissue in addition to the vascular system. Together with the instability of the autonomic nervous system this is an interesting fact.

Osler氏病樣出血性素因の1例

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