Læknablaðið : fylgirit - 01.05.1978, Qupperneq 16

Læknablaðið : fylgirit - 01.05.1978, Qupperneq 16
the impression that this is anything but a difficult and capricious disease requiring vigorous and continuous care of the many articular, systemic, ocular, and psychologic problems as they arise. It has been shown that early comprehensive management is apparently related to a favorable prognosis - a concept first proposed by Dr. Barbara Ansell of Liondon. In our series, children treated during the first year of disease also did comparatively better (only 5 per cent in classes III and IV) than those seen 2 to 4 or more years after onset (16 to 20 per cent). Incidentally, this impression appears to be con- firmed in our second group of 100 patients who have now been followed for up to 13 years. Other long-term studies report a mortality of 2 to 5%. Most deaths occur in patients with prolonged active polyarthritis and is due primar- ily to secondary amyloidosis. In the most recent reporting of patients followed for 10 years or more, remission has been observed in at least 5CF/o while normal function has been noted in at least 70/o. Occasionally, however, a patient in remission for as long as 20 years will have a severe exacerbation as an adult, resulting in joint destruction and severe disabílity for the first time. In our series, a poor prognosis has been associated with - an unremitting course of poly- arthritis, progressive hip involvement, subcu- taneous nodules, and undue delay in comprehen- sive care. The long-term prognosis of this disease is mitigated only by early diagnosis and the avail- ability of a multidisciplinary approach to manage ment. Couple this with regular followup care and consistent home treatment from steadily supportive parents and the outlook is optimal. Clearly, we have come a long way in our understanding of Still's disease since the earliest writings by George Frederick Still. We can now look to the future for answers to unresolved issues, such as etiology, by young investigators like this young chap, my son John who as an infant was already reading my article on rash in the Journal of Pediatrics. CONFERENCE IN PROGRESS 14
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