No abstract available.
Myositis* ; Orbital Myositis

No abstract available.
Myositis Ossificans* ; Myositis*

No abstract available.
Myositis Ossificans* ; Myositis*

Fibrodysplasia ossificans progressiva (FOP) is a debilitating, rare, autosomal dominant disorder of connective tissue characterized by malformed great toes and by progressive endochondral ossification of extra-skeletal sites (e.g., muscles, tendons, fascia) triggered by trauma, soft tissue injury, muscle fatigue, or viral infections. We present three children affected with FOP with this classic clinical presentation, the first reported cases in the Philippines, thus extending the range of classic FOP to new geographic and ethnic locations. Two of the affected children are siblings who have the common ACVR1 R206H mutation associated with classic FOP; this mutation was not found in their parents who are phenotypically unaffected, providing evidence of germline mosaicism in FOP. To our knowledge, this is the first family with genetic testing done showing presence of the classic mutation in affected siblings not seen in the unaffected parents.
Myositis Ossificans

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

No abstract available.
Blepharoptosis ; Myositis* ; Orbital Myositis ; Orbital Pseudotumor

No abstract available.
Muscles ; Orbit ; Orbital Myositis

No abstract available.
Orbit* ; Orbital Myositis*

No abstract available.
Diplopia* ; Orbit* ; Orbital Myositis*