Congenital hypogonadotropic hypogonadism/Kallmann syndrome is associated with statural gain in both men and women: a monocentric study. Issue 2 (February 2020)

• Record Type: Journal Article
• Authors: Maione, Luigi; Pala, Giovanna; Bouvattier, Claire; Trabado, Séverine; Papadakis, Georgios; Chanson, Philippe; Bouligand, Jérôme; Pitteloud, Nelly; Dwyer, Andrew A; Maghnie, Mohamad; Young, Jacques
• Is Part Of: European journal of endocrinology. Volume 182:Issue 2(2020)
• Page Start: 185
• Publication Date: 2020-02
• Subjects: Endocrinology -- Periodicals; 616.4005
• DOI: 10.1530/EJE-19-0537
• Languages: English
• ISSNs: 0804-4643

Abstract

Abstract : Context: Congenital hypogonadotropic hypogonadism/Kallmann syndrome (CHH/KS) is a rare condition characterized by gonadotropin deficiency and pubertal failure. Adult height (AH) in patients with CHH/KS has not been well studied. Objective: To assess AH in a large cohort of patients with CHH/KS. Patients: A total of 219 patients (165 males, 54 females). Parents and siblings were included. Methods: AH was assessed in patients and family members. AH was compared to the general French population, mid parental target height (TH) and between patients and same-sex siblings. Delta height (∆H) was considered as the difference between AH and parental TH. ∆H was compared between patients and siblings, normosmic CHH and KS (CHH with anosmia/hyposmia), and according to underlying genetic defect. We examined the correlations between ∆H and age at diagnosis and therapeutically induced individual statural gain. Results: Mean AH in men and women with CHH/KS was greater than that in the French general population. Patients of both sexes had AH > TH. Males with CHH/KS were significantly, albeit moderately, taller than their brothers. ∆H was higher in CHH/KS compared to unaffected siblings (+6.2 ± 7.2 cm vs +3.4 ± 5.2 cm, P < 0.0001). ∆H was positively correlated with age at diagnosis. Neither olfactory function (normosmic CHH vs KS) nor specific genetic cause impacted ∆H. Individual growth during replacement therapy inversely correlated with the age at initiation of hormonal treatment ( P < 0.0001). Conclusions: CHH/KS is associated with higher AH compared to the general population and mid-parental TH. Greater height in CHH/KS than siblings indicates that those differences are in part independent of an intergenerational effect.