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Cutis verticis gyrata and mental deficiency in Sweden

, : Cutis verticis gyrata and mental deficiency in Sweden. Acta Med Scand 177(4): 459-464

A series of 47 patients with a combination of cutis verticis gyrata and mental deficiency, 46 men and 1 woman, yielded the following data of genetic interest. (The epidemio-logic and clinical data have been previously reported.) The sex-chromatin and chromosome patterns were normal. The same was true of the parental age and birth rank. About 8% of the patients were the offspring of first-cousin marriages. The parents did not differ from the general population in morbidity. The sibs contained an excess number of severely retarded subjects. There were significantly few females both among the patients and among their healthy sibs. The most plausible explanation for this seemed to be that the female fetuses in these families were more often aborted than were the male fetuses, the disease probably incurring an increased risk of abortion for female fetuses. The syndrome cutis verticis gyrata was concluded to be genetically conditioned. Different modes of transmission were discussed, but no definite conclusion could be drawn. It was emphasized that it is impossible to judge the part played by external factors, and pointed out that the syndrome might be of serveral different origins.


PMID: 14296606

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