Epidemiologi ved blandet bindevevsykdom, mixed connective tissue disease (MCTD) hos barn
DOI:
https://doi.org/10.5324/nje.v18i1.79Sammendrag
There are three different classification criteria for this disease, all from 1987: The Alarcon-Segovia, Sharp and Kasukawa criteria. There is still no international concensus as to which of them should be used. This makes comparison between different reports using different classification criteria difficult. Mixed connective tissue disease seems next to systemic sclerosis to be the most infrequent systemic connective tissue disease in children. The incidence is reported to amount to 0.1-0.3/100,000/year. Female/male ratio is reported to be 2-3/1, median age for disease debut about 11 years, range 4-16 years. The most frequent cause of death is probably pulmonary hypertension, but death because of sepsis, hearth failure, renal failure, CNS disease and gastrointestinal bleeding is also reported. Mortality rate is probably lower in mixed connective tissue disease with debut in childhood than in adult life.Downloads
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