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Mixed Connective Tissue Disease in Children (Penyakit Jaringan Ikat Campur pada Anak)

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A case of Mixed Connective Tissue Disease (MCTD) in an 11 years-old girl is reported. This is the first reported in the Department of Child Health, Faculty of Medicine University of Indonesia/Dr. Cipto Mangunkusumo Hospital, Jakarta. In the beginning, the diagnosis was established as scleroderma based on the clinical picture of the skin lesion and the result of the skin biopsy. Laboratory examination later on revealed anemia, thrombocytopenia, increased ESR, positive ANA, negative anti-dsDNA, increased SGOT and SGPT, and elevation of skeletal mucle enzymes (creatinine kinase, MB iso-enzyme of creatinne kinase, and lactase dehydrogenase). Electromyogram showed evidence of dermatomyositis. Based on these findings a diagnosis of dermatomyositis was established. During hospitalisation there were signs of vasculitis and thrombosis, with the elevation of anti-cardiolipin antibody titer. Hypertension and proteinuria were present and these were considered as renal involvement. Cardiology examination confirm the presence of cardiomyopathy. MRI examination of the skull was performed because he patient suffered from seizure, and showed vast cerebral ishemic area and brain infarction. Based on those clinical pictures and laboratory findings mentioned above, could be the symptoms of systemic lupus erythematosius, scleroderma, and dermatomyositis, the diagnosis of MCTD then was made, despite the negative result of antibody to ribonucleid protein. Prednisone therapy 2 mg/kg BW/day was given intially, but due to hypertension this was changed to cyclophosphamide 750 mg/m2 every 4 weeks added by furosemide and captopril. Prednison was still continued with low dose. Heparinisation to treat vasculitis and thrombosis was also given. The patient was discharged on the 7th week of hospitalisation in good clinical condition. Skin lesion were improved with neither sign of new vasculitis nor thrombosis. She was then followed up as outpatient clinical.

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Majalah Kedokteran Indonesia,46(12)1996:691-696

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: ., 1996

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child
mixed connective tissue disease

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