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Aglaé Terray, Bertrand Baussart, Marie Zins, Marcel Goldberg, Sofiane Kab, Laure Cazabat, Mathilde Brière, Thierry Brue, Sara Barraud, Yves Reznik, Sophie Christin-Maitre, Frédéric Illouz, Gérald Raverot, Jacques Young, Marie-Laure Raffin-Sanson, Mirella Hage, Gonadotropic status in adult women with pituitary stalk interruption syndrome, European Journal of Endocrinology, Volume 190, Issue 6, June 2024, Pages 501–508, https://doi.org/10.1093/ejendo/lvae064
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Abstract
Pituitary stalk interruption syndrome (PSIS) is a rare cause of congenital hypopituitarism. Limited data exist on the gonadotropic status and fertility of adult women with PSIS. Our study aims to describe pubertal development and the evolution of gonadotropic function and fertility in adult women with PSIS.
A retrospective multicentric French study.
We described gonadotropic function in 56 adult women with PSIS from puberty onward. We compared live birth rates per woman with PSIS with age-matched controls from the large French epidemiological cohort (CONSTANCES). Additionally, we assessed height, body mass index (BMI), blood pressure, other metabolic parameters, and socioeconomic status.
Among 56 women with PSIS, 36 did not experience spontaneous puberty. Of these, 13 underwent ovarian stimulation, resulting in 7 women having a total of 11 children. In the subgroup with spontaneous puberty (n = 20), 4 had a total of 8 pregnancies, while 6 developed secondary gonadotropic deficiency. Women with PSIS had fewer children than controls (0.33 vs 0.63, P = .04). Median height was also lower (160.5 vs 165.0 cm, P < .0001). Although mean blood pressure was lower in women with PSIS compared with controls (111.3/65.9 ± 11.2/8.1 vs 118.7/72.1 ± 10.1/7.7 mmHg, P < .001), there were no significant differences in other metabolic parameters, notably BMI and lipid profile. Employment/academic status was not different in the 2 groups, but fewer women with PSIS were in relationships (42% vs 57.6% in controls, P = .02). The fertility prognosis in patients with PSIS needs optimization. Patients should be informed about the likelihood of declining gonadotropic function over time.