UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 骨形成不全症：概要osteogenesis imperfecta an overview [show details]
…anterior chamber; iris atrophy; intraocular calcification; and hypotonia . Panostotic fibrous dysplasia – Panostotic fibrous dysplasia, the extreme form of polyostotic fibrous dysplasia (McCune-Albright…
2. 不整脈原性右室心筋症：解剖学、組織学、臨床症状arrhythmogenic right ventricular cardiomyopathy anatomy histology and clinical manifestations [show details]
…fibrolipomatous (fibrofatty) pattern is characterized by myocardial atrophy and thinning with replacement of myocardium by both fibrous and fatty tissue, as well as patchy inflammatory infiltrates. RV aneurysms …
3. 末梢動静脈奇形peripheral arteriovenous malformations [show details]
…imaging that demonstrates extra fascial features consisting of dense fibrous tissue, fat, and lymphoplasmacytic aggregates with atrophy of the muscle. Adipose tissue tends to infiltrate the muscle at the…
4. 脊髄性筋萎縮症spinal muscular atrophy [show details]
…Spinobulbar muscular atrophy – Spinobulbar muscular atrophy (Kennedy disease) is an X-linked disorder characterized with onset from ages 20 to 60 years of slowly progressive weakness and atrophy affecting facial …
5. 小児や青年の良性骨腫瘍nonmalignant bone lesions in children and adolescents [show details]
… Fibrous dysplasia accounts for approximately 5 to 7 percent of benign bone tumors . Fibrous dysplasia may occur in single or multiple bones (monostotic and polyostotic fibrous dysplasia,… other diagnoses . Children with lower-extremity lesions may present with limp, swelling, muscular atrophy, leg-length discrepancy, bone deformities, muscle contractures, and local point tenderness at the …

Japanese Journal

血清 aldolase 値の上昇がみられた perifascicular atrophy をともなう非好酸球性筋膜滑膜炎の1例

小野紘彦,鈴木直輝,水野秀紀 [他],竪山真規,青木正志,糸山泰人
臨床神経学 49(2), 119-122, 2009-03-01
NAID 10024899164

血清ａｌｄｏｌａｓｅ値の上昇がみられたｐｅｒｉｆａｓｃｉｃｕｌａｒ　ａｔｒｏｐｈｙをともなう非好酸球性筋膜滑膜炎の１例

小野紘彦,鈴木直輝,水野秀紀,竪山真規,青木正志,糸山泰人
臨床神経学 49(2/3), 119-122, 2009
症例は35歳の男性である．両手の張りとむくみ感で発症し，2カ月の経過で四肢の張りや歩行時の筋痛と関節痛と四肢の筋力低下が進行した．前腕と手は腫脹・発赤し，Raynaud現象と皮膚硬化所見をみとめた．四肢の筋力は軽度低下していた．末梢血中の好酸球増多はなくCKは正常でmyoglobinは軽度上昇していたのに対し，aldolaseは正常上限の約10倍に上昇していた．MRIでは筋膜および滑膜に炎症性変化 …
NAID 130004504539