4.6 Article

Pubertal development and hypogonadism in males with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy: a retrospective study

Journal

EUROPEAN JOURNAL OF ENDOCRINOLOGY
Volume 188, Issue 4, Pages K17-K23

Publisher

OXFORD UNIV PRESS
DOI: 10.1093/ejendo/lvad034

Keywords

autoimmune polyendocrine syndrome type 1; gonadal disorders; testosterone; azoospermia

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The aim of this study was to investigate the puberty and hypogonadism in males with APECED. Forty-three males were followed up until adulthood, and the onset of spontaneous puberty was found to occur at a median age of 13.3 years. Testosterone medication was used to promote pubertal development in some patients. The study also identified hypogonadism and azoospermia in a subset of patients.
The aim of this study was to describe the course of puberty and hypogonadism in males with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) in a Finnish APECED cohort followed up between 1970 and 2020. Anthropometry, testicular volumes and FSH, LH, and testosterone concentrations were analyzed retrospectively. Forty-three males were followed up until the median age of 42.5 years (range, 16.2-74.8). All subjects fulfilled the clinical criteria for APECED. The median age at the onset of spontaneous puberty was 13.3 years (10.8-14.8). Testosterone medication was used to promote pubertal development from the median age of 14.9 years (13.5-15.7), for 0.7-3.3 years in 8 patients. The median adult height was 173.0cm and differed from the mid-parental target height on average -1.3 SDS (P < .001). Hypogonadism was treated in 6 patients (14%). Azoospermia was found in 3 patients. Further studies are required to explore the role of the autoimmune regulator in sperm production and testicular insufficiency.

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