Association of isolated hypogonadotropic hypogonadism, pronounced hypodontia and the Wolff-Parkinson-White syndrome.
Diagnosis of idiopathic isolated hypogonadotropic hypogonadism was made in a 22-year-old female patient referred for primary amenorrhoea. It was considered a separate entity from Kallmann's syndrome, because it was not accompanied by anosmia or other specific pleiotropic features. On the other hand, the patient showed severe hypodontia and an intermittent Wolff-Parkinson-White syndrome. To our knowledge, this association has never been reported before. This unusual phenotype points to a nonrandom association. However, no information in the literature is available to consider a new single gene defect or a contiguous gene syndrome.
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Department of Endocrinology, University of Antwerp, Belgium.
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Vercruysse
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D000328:Adult; D000848:Anodontia / Q000150:complications*; D005260:Female; D006065:Gonadotropins, Pituitary / Q000172:deficiency*; D006801:Humans; D007006:Hypogonadism / Q000150:complications* / Q000503:physiopathology; D014927:Wolff-Parkinson-White Syndrome / Q000150:complications*
D006065:Gonadotropins, Pituitary