ECE2019 Guided Posters Interdisciplinary Endocrinology 1 (11 abstracts)
Final (FH) and target height (TH) in male and female patients with congenital hypogonadotropic hypogonadism (CHH)/Kallmann syndrome (KS): a monocentric study of 216 patients
Luigi Maione 1 , Giovanna Pala 2 , Séverine Trabado 3 , Georgios Papadakis 1 , Mohamad Maghnie 2 , Claire Bouvattier 4 & Jacques Young 1
1Department of Reproductive Endocrinology, Bicêtre Hospital, Univ. Paris Saclay, Le Kremlin-Bicêtre, France; 2Department of Pediatric Endocrinology, IRCSS Gaslini Institute, Genoa, Italy; 3Department of Hormonology, Bicêtre Hospital, Univ. Paris Saclay, Le Kremlin-Bicêtre, France; 4Department of Pediatric Endocrinology, Bicêtre Hospital, Univ. Paris Saclay, Le Kremlin-Bicêtre, France.
Context: CHH/KS is a major cause of pubertal and bone maturation delay due to sex steroids deficiency. FH and TH have not been evaluated in large series of CHH/KS patients. Analysis of auxometric measures in patients’ siblings has never been performed.
Patients and methods: We selected 216 (164/52 men/women; 112/104 KS/normosmic CHH) with available auxometric measures. Sibling stature was available in a subgroup of 162 patients. Difference between the FH and the TH (Delta Height) was calculated. Relation between FH and age at diagnosis was evaluated. In a subgroup of 121 patients, therapeutically-induced growth gain was calculated.
Results: FHs in CHH/KS men and women were higher than in the sex-matched general population. FH was higher than TH (calculated from parents’ heights): respectively 179±8 vs 175±6 cm in men and 166±7 vs 162±5 cm in women (P<0.001 for both). Delta Height was positively correlated with age at diagnosis and age at beginning of hormonal treatment (P=0.01). FH and Delta Height were higher in patients than in their siblings (P<0.01), excluding a merely ‘generational’ effect. No difference in Delta Height was found between normosmic CHH and KS. The therapeutically-induced growth gain was inversely correlated with the age at initiation of hormonal treatment.
Conclusions: FH in CHH/KS is higher to either the general population, TH and even to their sex-matched siblings. Our findings show that CHH/KS does not negatively impact adulthood height, and there is no risk of short stature under therapy. Furthermore, the growth gain in CH/KS is disease-related and independent on a generational effect.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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