Adrenal Disorders and Pubertal Development in Children: Hormonal Pathways, Timing Disruptions and Clinical Outcome
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Notice bibliographique
Résumé
Background: Pubertal development is tightly regulated by the hypothalamic–pituitary–gonadal axis and modulated by adrenal glucocorticoids and androgens. Disruption of adrenal function—through hyposecretion or hypersecretion—can alter the timing, tempo, and outcome of puberty, but data are scattered across heterogeneous pediatric conditions. This review integrates current evidence on how adrenal disorders shape pubertal development and growth outcomes, and to what extent these abnormalities are reversible with treatment. Methods: A structured narrative review was conducted using PubMed/MEDLINE, Scopus, and Google Scholar (January 2000–June 2025). Search terms combined adrenal disorders (e.g., adrenal insufficiency, congenital adrenal hyperplasia, Cushing syndrome, premature adrenarche, adrenal hypoplasia congenita, Triple A syndrome, McCune–Albright syndrome) with pubertal terms (delayed puberty, precocious puberty, pubertal progression, adrenarche, pubarche). Human studies in patients <20 years reporting adrenal diagnosis plus at least one pubertal or growth outcome were included. Case reports (<5 patients), adult-only series, purely biochemical reports, and non–full-text abstracts were excluded. Study quality was assessed using a modified Newcastle–Ottawa Scale and synthesized qualitatively in four domains: adrenal hyposecretion, hypersecretion, syndromic disorders, and reversibility. Results: Adrenal hyposecretion (primary and central adrenal insufficiency, adrenal hypoplasia, familial glucocorticoid deficiency, autoimmune polyglandular syndromes) was consistently associated with delayed adrenarche, delayed or sparse pubarche, slow pubertal progression, and reduced growth velocity. Syndromic forms (e.g., DAX-1–related adrenal hypoplasia, Triple A, IMAGe, APS-1) frequently combined adrenal failure with hypogonadotropic hypogonadism or primary gonadal failure, leading to profound pubertal delay or absent puberty. In contrast, adrenal hypersecretion states showed a spectrum from mild to severe precocious development. Classic 21-hydroxylase–deficient congenital adrenal hyperplasia, nonclassic CAH, premature adrenarche, and androgen-secreting adrenal tumors were linked to premature pubarche, virilization, advanced bone age, and, in many cases, central precocious puberty with compromised adult height. Cushing syndrome (ACTH-dependent and independent) uniquely caused growth failure and pubertal delay due to cortisol-mediated suppression of GnRH, gonadotropins, and growth hormone action. Across disorders, early and optimized treatment improved pubertal trajectories: strict androgen control in CAH limited progression and preserved height; surgical cure of Cushing syndrome allowed recovery of pubertal progression; metabolic management in premature adrenarche mitigated progression to early menarche and PCOS. However, in genetic syndromic adrenal insufficiency, pubertal abnormalities were rarely reversible without exogenous sex-steroid or gonadotropin replacement. Overall study quality was moderate to high, supporting the robustness of these patterns. Conclusions: Adrenal hypofunction predominantly delays puberty, whereas adrenal androgen excess accelerates or distorts pubertal onset, and cortisol excess suppresses pubertal maturation and growth. Many pubertal disturbances are partially reversible when adrenal disorders are recognized and treated early, but syndromic forms often require lifelong hormone replacement. Routine pubertal surveillance in children with adrenal disease and integrated hormonal, metabolic, and genetic evaluation are essential to optimize growth and reproductive outcomes.
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Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,001 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,001 |
| Intégrité de la recherche | 0,000 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
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Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découle