腹部大動脈瘤ヲ伴ツタMarfan氏症候群:(蜘蛛状指趾症Arachnodaktylie) ノ1例
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概要
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Since the original description of arachnodactylia by Marfan 1896, about 280 cases of this disease have been reported, of which about 40 cases are found in Japanese literatures.<BR>The auther observed a patient, who showed a typical Marfrn's syndrome.<BR>The patient was an unmarried japanese man, aged 31 years. Nothing noteworthy was found in his past history except for abnormal body form. In June, 1948, illness began with severe agonizing, compressing pain over the anterior chest and the right back, accompanied with palpitation and dyspnoea.<BR>In October, 1948, the patient was transferred to our hospital.<BR>Physical examination revealed as follows- His height was 175.6 cm (taller than common japanese man), and his weight was 56.0 kg. His extremities were slender and long, and showed typical arachnodactylic forms. Disharmonious bodily development, dolichocephalic head with old-appearing features, lordoscoliosis, pes planus, and hallux valgus were marked. Vision was short sighted. No dislocatio lentis.<BR>The heart was markedly enlarged, rough systolic and diastolic murmurs were loudly heard over the entire precordium, and they were most intensive at the aortic ostium. (aortic steno. insufficiency and mitral stenoinsufficiency) Electrocardiogram showed the existence of myocardial disturbance.<BR>Roentgenologic examination revealed marked aortic aneurysm.<BR>The blood pressure was 117/45 in the right arm, 131/33 in the left arm.<BR>The blood Wassermann was negaiive. There was a marked pulsation over the middle abdomen. Palpatoric finding and roentgenologic examination revealed aneurysm of aorta abdominalis.<BR>Sella turcica was shallow and very small. Long tubural bone showed remainning epiphyseal line and pseudo-epiphyseal formation. These findings may prove the existence of endocrine dysfunction. Hematologically nothing was abnormal, except for eosinophilia (8.0%). Neurologically normal.<BR>In this case familiary relationship was evidently recognized. Bloodrelational appearance of this disease showed Menders dominat hereditary type.<BR>Except for a attack of asthma cardiale, the patient was almost asymptomatic during his hospital stay.<BR>On December 3., 1948, he was sent home. In the middle of December, 1948, symptoms of decompensation rapidly developed, and he died suddenly on December 23., 1948.
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