[A case of endogenous familial hereditary malformation of both hands].
A case of congenital malformation of both hands in a 22 year old television-technician, who otherwise showed no abnormalities, is reported. In his case, a familiar incidence was observed constantly in all males for a period of nearly 200 years. The morphological picture of this malformation is as follows: left hand: postaxial oligodactyly with a cutaneous syndactyly of the third and fourth fingers accompanied by a V-shaped os metacarpale and a synostosis of the os triquetrum, os lunatum and possibly of the os pisiforme right hand: postaxial oligodactyly with an accessory hypoplastic third finger on the ulnar side with a common base of the proximal phalanx which was enlarged at the end and shorter than the others (symbrachybasophalanx). Os multangulum minus and os capitatum as well as os triquetrum and possibly also the os pisiforme showed a synostosis. Subjective symptomatology and further clinical investigations (Phonocardiogramm) revealed an associated heart disease. This kind of malformation of both hands is transmitted inclompletely only in males.
D000015:Abnormalities, Multiple / Q000235:genetics*; D000328:Adult; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D003878:Dermatoglyphics; D005385:Fingers / Q000002:abnormalities; D005787:Gene Frequency; D006228:Hand Deformities, Congenital; D006330:Heart Defects, Congenital / Q000150:complications; D006801:Humans; D008297:Male; D008663:Metacarpus / Q000002:abnormalities*; D010375:Pedigree; D012737:Sex Factors; D013576:Syndactyly / Q000235:genetics; D013580:Synostosis / Q000235:genetics
Ein Fall von endogener familiärer hereditärer Handmissbildung beiderseits