We analyzed data from 20 patients with late-onset 21-hydroxylase deficiency (LOHD). Three clinical phenotypes could be distinguished among the 18 women. Seven (39%) presented with clinical features suggesting polycystic ovarian disease (PCOD). However, despite androgen levels similar to those of patients with typical PCOD, high serum LH to FSH ratios were not consistently found. Seven other women (39%) presented with isolated hirsutism, suggesting idiopathic hirsutism. The remaining 4 women (22%) had no manifestations of androgen excess and were considered to have the cryptic form of LOHD. Serum 17-hydroxyprogesterone (17-OHP) and androgen levels were similar in the 3 phenotypes, suggesting that the clinical expression of LOHD in women is modulated by individual factors, such as androgen sensitivity. The 2 men were detected by family study and were clinically normal. Since clinical diagnosis of LOHD is impossible, we concentrated on hormonal data with the aim of providing guidelines for the biological diagnosis of LOHD. Assay of basal serum 17-OHD at 0800 h in both sexes and in the early follicular phase in women was sufficient to establish the diagnosis of LOHD in most patients. If doubtful results are obtained, i.e. serum 17-OHP levels between 2 and 5 ng/ml, an ACTH test must be performed. Post-ACTH serum 17-OHP levels exceeding 10 ng/ml confirm the diagnosis of LOHD. Such results should avoid confusion with heterozygotes for 21-hydroxylase deficiency, whose frequency is high within the general population and may be even higher in patients with idiopathic hirsutism or PCOD.

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We studied eight cases of male anorexia nervosa selected according to Feighner's criteria. At the time of their maximum weight loss, all had plasmatic testosterone assessment (J). Five of them also had assessment of plasmatic estradiol (E2) and baseline levels of serum gonadotropins as well as of their response to LH‐RH. The response of the testicular hormone to HCG was studied in three cases. During weight recovery, six patients again had the same assessments, which were repeated at variable intervals. At the time of their maximum weight loss, T was decreased in every case; E2 twice in five cases; FSH five times in five cases; and LH four times in five cases. In four cases out of five, gonadotropins did not respond to LH‐RH. Mean levels of the four hormones were significantly lower than those of an age‐matched control group of 12 normal‐weight subjects. During weight recovery, the response of four hormones and the gonadotropins to LH‐RH increased. We found a highly significant correlation between testosterone and weight. Male anorexia nervosa, therefore, is associated with an intense hypogonadotropic hypogonadism, which mainly results from weight loss. However testosterone and weight do not always move in the same way. Other mechanisms, particularly psychosomatic ones, seem to act in the determinism of hypogonadism.

Objective: Assess the feasibility of performing a robotically-assisted laparoscopic cervical cerclage in the setting of a known uterus didelphys during the second trimester of pregnancy utilizing the DaVinci Xi surgical system. Design: Case report.