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C. C. SCHULMAN, MD; J. Mendlewicz, MD
Brussels

JAMA. 1970;213(10):1689.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

To the Editor.—

Turner's syndrome with normal XX karyotype has been reported in the literature in a few patients only.¹

We wish to report a unique case of gonadal dysgenesis with a normal XX karyotype (sex-chromatin positive; chromosome studies of leukocyte culture and skin biopsy suggested a normal XX female karyotype). The patient presented the stigmata of a Turner phenotype associated with a solitary pelvic kidney.

Our patient is a 17-year-old girl presenting, in addition to most of the usual stigmata of Turner phenotype (gonadal dysgenesis, short stature, webbed neck, shield chest, Kosowicz's sign), the following unusual associated abnormalities: complete absence of vagina and uterus, solitary pelvic kidney, hypoplasia of the entire right arm with alteration of the thumb joints, Klippel-Feil syndrome associated with webbing of the neck, and cervical ribs.

The solitary pelvic kidney visualized by an intravenous pyeogram, was confirmed by radioisotopic scanning and perineoscopy which revealed . . . [Full Text PDF of this Article]

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