Reproductive hormones, bone mineral content, body composition, and testosterone therapy in boys and adolescents with Klinefelter syndrome

López Krabbe, Hans Valdemar; Holm Petersen, Jørgen; Asserhøj, Louise Laub; Johannsen, Trine Holm; Christiansen, Peter; Jensen, Rikke Beck; Cleemann, Line Hartvig; Hagen, Casper P.; Priskorn, Lærke; Jørgensen, Niels; Main, Katharina M; Juul, Anders; Aksglaede, Lise

doi:10.1530/EC-23-0031

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Reproductive hormones, bone mineral content, body composition, and testosterone therapy in boys and adolescents with Klinefelter syndrome

research-article

Author(s): Hans Valdemar López Krabbe ¹ ^, ² , Jørgen Holm Petersen ¹ ^, ² ^, ³ , Louise Laub Asserhøj ¹ ^, ² ^, ⁴ , Trine Holm Johannsen ¹ ^, ² , Peter Christiansen ¹ ^, ² , Rikke Beck Jensen ¹ ^, ² ^, ⁵ , Line Hartvig Cleemann ¹ ^, ² , Casper P Hagen ¹ ^, ² , Lærke Priskorn ¹ ^, ² , Niels Jørgensen ¹ ^, ² , Katharina M Main ¹ ^, ² ^, ⁵ , Anders Juul ¹ ^, ² ^, ⁵ , Lise Aksglaede ¹ ^, ² ^,

Publication date (Electronic): 03 April 2023

Journal: Endocrine Connections

Publisher: Bioscientifica Ltd

Keywords: Klinefelter syndrome, body composition, truncal obesity, bone mineral content

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Abstract

Adult patients with Klinefelter syndrome (KS) are characterized by a highly variable phenotype, including tall stature, obesity, and hypergonadotropic hypogonadism, as well as an increased risk of developing insulin resistance, metabolic syndrome, and osteoporosis. Most adults need testosterone replacement therapy (TRT), whereas the use of TRT during puberty has been debated. In this retrospective, observational study, reproductive hormones and whole-body dual-energy x-ray absorptiometry-derived body composition and bone mineral content were standardized to age-related standard deviation scores in 62 patients with KS aged 5.9–20.6 years. Serum concentrations of total testosterone and inhibin B were low, whereas luteinizing hormone and follicle-stimulating hormone were high in patients before TRT. Despite normal body mass index, body fat percentage and the ratio between android fat percentage and gynoid fat percentage were significantly higher in the entire group irrespective of treatment status. In patients evaluated before and during TRT, a tendency toward a more beneficial body composition with a significant reduction in the ratio between android fat percentage and gynoid fat percentage during TRT was found. Bone mineral content (BMC) did not differ from the reference, but BMC corrected for bone area was significantly lower when compared to the reference. This study confirms that patients with KS have an unfavorable body composition and an impaired bone mineral status already during childhood and adolescence. Systematic studies are needed to evaluate whether TRT during puberty will improve these parameters.

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Most cited references 57

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A. Vermeulen, L. VERDONCK, J Kaufman (1999)

The free and nonspecifically bound plasma hormone levels generally reflect the clinical situation more accurately than total plasma hormone levels. Hence, it is important to have reliable indexes of these fractions. The apparent free testosterone (T) concentration obtained by equilibrium dialysis (AFTC) as well as the fraction of serum T not precipitated by 50% ammonium sulfate concentration (non-SHBG-T; SHBG, sex hormone-binding globulin), often referred to as bioavailable T, appear to represent reliable indexes of biologically readily available T, but are not well suited for clinical routine, being too time consuming. Several other parameters have been used without complete validation, however: direct immunoassay of free T with a labeled T analog (aFT), calculation of free T (FT) from total T and immunoassayed SHBG concentrations (iSHBG), and the free androgen index (FAI = the ratio 100T/iSHBG). In the view of substantial discrepancies in the literature concerning the free or bioavailable T levels, we compared AFTC, FT, aFT, FAI, and non-SHBG-T levels in a large number of sera with SHBG capacities varying from low, as in hirsute women, to extremely high as in hyperthyroidism. All these indexes of bioavailable T correlated significantly with the AFTC concentration; AFTC and FT values were almost identical under all conditions studied, except during pregnancy. Values for aFT, however, were only a fraction of either AFTC or FT, the fraction varying as a function of SHBG levels. Also, the FAI/AFTC ratio varied as a function of the SHBG levels, and hence, neither aFT nor FAI is a reliable index of bioavailable T. The FT value, obtained by calculation from T and SHBG as determined by immunoassay, appears to be a rapid, simple, and reliable index ofbioavailable T, comparable to AFTC and suitable for clinical routine, except in pregnancy. During pregnancy, estradiol occupies a substantial part of SHBG-binding sites, so that SHBG as determined by immunoassay overestimates the actual binding capacity, which in pregnancy sera results in calculated FT values that are lower than AFTC. The nonspecifically bound T, calculated from FT, correlated highly significantly with and was almost identical to the values of non-SHBG-T obtained by ammonium sulfate precipitation, testifying to the clinical value of FT calculated from iSHBG.

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The National Osteoporosis Foundation’s position statement on peak bone mass development and lifestyle factors: a systematic review and implementation recommendations

C Weaver, C. Gordon, K. Janz … (2016)

Lifestyle choices influence 20–40 % of adult peak bone mass. Therefore, optimization of lifestyle factors known to influence peak bone mass and strength is an important strategy aimed at reducing risk of osteoporosis or low bone mass later in life. The National Osteoporosis Foundation has issued this scientific statement to provide evidence-based guidance and a national implementation strategy for the purpose of helping individuals achieve maximal peak bone mass early in life. In this scientific statement, we (1) report the results of an evidence-based review of the literature since 2000 on factors that influence achieving the full genetic potential for skeletal mass; (2) recommend lifestyle choices that promote maximal bone health throughout the lifespan; (3) outline a research agenda to address current gaps; and (4) identify implementation strategies. We conducted a systematic review of the role of individual nutrients, food patterns, special issues, contraceptives, and physical activity on bone mass and strength development in youth. An evidence grading system was applied to describe the strength of available evidence on these individual modifiable lifestyle factors that may (or may not) influence the development of peak bone mass (Table 1). A summary of the grades for each of these factors is given below. We describe the underpinning biology of these relationships as well as other factors for which a systematic review approach was not possible. Articles published since 2000, all of which followed the report by Heaney et al. [1] published in that year, were considered for this scientific statement. This current review is a systematic update of the previous review conducted by the National Osteoporosis Foundation [1]. Lifestyle Factor Grade Macronutrients Fat D Protein C Micronutrients Calcium A Vitamin D B Micronutrients other than calcium and vitamin D D Food Patterns Dairy B Fiber C Fruits and vegetables C Detriment of cola and caffeinated beverages C Infant Nutrition Duration of breastfeeding D Breastfeeding versus formula feeding D Enriched formula feeding D Adolescent Special Issues Detriment of oral contraceptives D Detriment of DMPA injections B Detriment of alcohol D Detriment of smoking C Physical Activity and Exercise Effect on bone mass and density A Effect on bone structural outcomes B Considering the evidence-based literature review, we recommend lifestyle choices that promote maximal bone health from childhood through young to late adolescence and outline a research agenda to address current gaps in knowledge. The best evidence (grade A) is available for positive effects of calcium intake and physical activity, especially during the late childhood and peripubertal years—a critical period for bone accretion. Good evidence is also available for a role of vitamin D and dairy consumption and a detriment of DMPA injections. However, more rigorous trial data on many other lifestyle choices are needed and this need is outlined in our research agenda. Implementation strategies for lifestyle modifications to promote development of peak bone mass and strength within one’s genetic potential require a multisectored (i.e., family, schools, healthcare systems) approach.

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Author and article information

Journal

Journal ID (nlm-ta): Endocr Connect

Journal ID (iso-abbrev): Endocr Connect

Journal ID (hwp): EC

Title: Endocrine Connections

Publisher: Bioscientifica Ltd (Bristol )

ISSN (Electronic): 2049-3614

Publication date (Electronic): 03 April 2023

Publication date (Electronic preprint): 03 April 2023

Publication date Collection: 01 July 2023

Volume: 12

Issue: 7

Electronic Location Identifier: e230031

Affiliations

[1 ]Department of Growth and Reproduction , Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark

[2 ]International Centre for Research and Research Training in Endocrine Disruption of Male Reproduction and Child Health (EDMaRC) , Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark

[3 ]Section of Biostatistics , University of Copenhagen, Copenhagen, Denmark

[4 ]Department of Fertility , Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark

[5 ]Department of Clinical Medicine , University of Copenhagen, Copenhagen, Denmark