Measurements of clitoral length and clitoral to urethral distance were made and analysed for a relationship in a group of 19 women with complete androgen insensitivity syndrome (CAIS) attending a specialist clinic for adult women with disorders of sexual development. These were compared with a control group of 50 women attending hospital for a gynaecological procedure. There was a positive correlation between clitoral length and clitoral to urethral distance for women with CAIS. In contrast, a negative correlation was seen between clitoral length and clitoral to urethral distance for women in the control group. Women with CAIS had a reduced mean clitoral length compared with controls (P = 0.001), but no difference was observed for the clitoral to urethral distance between the two groups (P = 0.116).Keywords Androgen, androgen insensitivity syndrome, clitoris, female genitalia, urethra.Please cite this paper as: Crouch N, Michala L, Creighton S, Conway G. Androgen-dependent measurements of female genitalia in women with complete androgen insensitivity syndrome. BJOG 2011;118:84-87.
People with major congenital urological or neurological malformations invariably require bladder reconstruction with enterocystoplasty in early childhood. The improvement of the surgical management of these children has reflected significantly on their life expectancy. As a result, more young people with enterocystoplasty are being transitioned to adolescent clinics where they receive the usual counselling about sexual health and pregnancy risks. However, the possibility of false-positive urinary pregnancy tests in these young women remains an overlooked but essential message. The lack of awareness about this fact can result in significant patient anxiety and the potential for unnecessary interventions as exemplified by the three cases we have encountered.
Clinical data and findings at necropsy are presented on a 2-year-old girl who had persistent complete common atrioventricular canal, hypoplastic left ventricle, and obstruction to the left ventricular outflow tract. Electro-cardiographic findings were atypical of common atrioventricular canal in that right axis deviation and a clockwise inscription of the QRS loop in the frontal plane were present. Findings in this case suggest that under-development of the left ventricle should be suspected when (1) displacement of the interventricular sulcus is noted on roentgenographic examinations and (2) predominant left-to-right shunting at ventricular level is found in the absence of electrocardiographic evidence of left ventricular overload.
The clinical and pathologic findings in a case of primary hyperoxaluria and calcium oxalate nephrocalcinosis in a 7-year-old boy are described and discussed in relation to similar reported cases. The diagnosis was suspected because of nephrocalcinosis in the absence of an abnormality of calcium metabolism and proven by the demonstration of increased urinary excretion of oxalate. Splenomegaly and yellow deposits in the Meibomian glands are described.
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