[MCTD (Sharp) syndrome in childhood (author's transl)].

Clinical course and prognosis of children with MCTD-Syndrome are described and six own cases are compared with the cases reported by other authors. The immunological and histological findings would seem to confirm the independence of the disease or, at least, reveal it as a particular clinical course of other connective tissue diseases. On the other hand, the initial conception, that the course of the disease can be viewed as favourable on account of the absence of kidney and CNS findings, must not be reevaluated according to our experiences of the last few years. This applies particularly to the cases of MCTD in childhood, since authors in the U.S.A. have, in the meantime, reported instances of death. One patient in our own hospital was diagnosed through biopsy as having a severe membrano-proliferative glomerulonephritis, another patient has had a severe myo-pericarditis in 1978.