Adrenal and ovarian steroidogenic dysregulations in hyperandrogenic children

Objective & Hypothesis:To evaluate the etiological importance of steroidogenic dysregulations in adrenals and ovaries, and the risk of PCOS like (FOH/PCOS) features in hyperandrogenic children 34 children who admitted to Medical School of Ankara University Endoc-rinology Department are studied.

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Key Findings & Data:Adrenal steroidogenic pro-file is defined by basal and ACTH-stimulated levels of 170HP, cortisol, DHEA/S and androstenedione besides A Precursor/A Product ratios.

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Conclusion & Impact:Therefore, detailed studies have to be do-ne for early diagnosis and to define the risk lowering factors.

🔬Design:Research Article

📍Scope:Comprehensive Sample

⚙️Variables:Domain Parameters

Abstract

To evaluate the etiological importance of steroidogenic dysregulations in adrenals and ovaries, and the risk of PCOS like (FOH/PCOS) features in hyperandrogenic children 34 children who admitted to Medical School of Ankara University Endoc-rinology Department are studied. Adrenal steroidogenic pro-file is defined by basal and ACTH-stimulated levels of 170HP, cortisol, DHEA/S and androstenedione besides A Precursor/A Product ratios. Ovarian hyperandrogenism is defined by Ï70HP response to LHRH stimulation. Among 34 cases; 2.94 % were NK-210HD, 11.76 % were 210HD hétérozygote carrier, 32.36 % were 21-Hydroxylase minimal deficiency, 5.88 % were 3'sOHSD deficiency, 14.7 % were isolated FAH and 32.36 % were FAH+FOH.. 50% of our patients had functional ovarian hyperandrogenism and they had the risk of developing PCOS. Even mild forms of hyperandrogenism in children have to be taken serious because it might be thefirst sign of PCOS. Therefore, detailed studies have to be do-ne for early diagnosis and to define the risk lowering factors.

Özet

Hiperandrojenizmli çocuklarda adrenalerde ya da överdeki steroidojenik disregülasyonların etyolojik öneminin denetlenmesi ile bu olgularda hormonal ve morfolojik olarak PCOS benzeri değişikliklerin (FOH/PCOS) oluşma riskini saptamak amacıyla AÜTF Endokrin Polikliniğine başvuran hiperandrojenizmli 34 olgu incelendi. Surrénal steroidogenezi bazal ve ACTH uyarısına 170HP, kortizol, DHEA/S ve androstenodion yanıtları ve A Ön Madde / A Ürün molar oranlamaları ile denetlendi. Över hiperandrojenizmi LHRH uyarısına alınan 170HP yanıtı ile belirlen- di. Hiperandrojenizmli 34 olgunun % 2.94'ü NK-210H eksikliği; % 11.76'sı aynı enzim defektinin heterozigot taşıyıcısı; % 32.36'sı 21 OH minimal eksikliği; % 5.88'i 3(OHSD eksikliği; % 14.7'si izole FAH; % 32.36'sı FAH+FOH özelliği gösterdi. Olgularımızda % 50 oranında fonksiyonel över hiperandrojenizmi saptanması (FOH/PCOS) bu çocukların gelişebilecek PCOS yönünden risk altında olduklarını gösteren bir bulum olarak önemsendi. Çocuklarda hafif ölçülerde bile olsa hiperandrojenizm benign olarak kabul edilmemeli, PCOS'un ilk prezentasyonu olma olasılığı dikkate alınmalıdır. Bu amaçla erken tanı ve risk azaltıcı yöntemlerin belirlenmesi yönünde çalışmalara ağırlık verilmelidir.

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Cite This Article

ÇATİNKAYA, E., ÖCAL, G., ADIYAMAN, P., BERBEROĞLU, M. (2022). Adrenal and ovarian steroidogenic dysregulations in hyperandrogenic children. Endokrinolojide Yönelişler, Cilt: 11 - Sayı: 2, 61–67. https://ajindex.com/articles/500914

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