Развитие эктопической надпочечниковой ткани в яичках (TART) у детей и подростков с врожденной дисфункцией коры надпочечников

Оценивали распространенность TART среди 71 пациента с ВДКН в возрасте от 1 года до 19 лет и сравнивали методы визуализации (УЗИ и МРТ) TART. TART была выявлена в 14,1% случаев ВДКН (10 из 71; 1 пациент с простой вирильной и 9 - с сольтеряющей формой). Большинство TART (9 из 10) были выявлены с помощью УЗИ, МРТ позволило уточнить размеры образований. Обнаружена взаимосвязь между наличием TART и уровнями 17-ОНП и АРП на момент обследования, количеством определений и уровнем АРП в течение последнего года. TART чаще возникает у детей с плохо контролируемым лечением: недостаточность как глюко-, так и минералокортикоидов является предрасполагающим, но не единственным фактором риска развития этого заболевания.

врожденная дисфункция коры надпочечников (ВДКН), УЗИ тестикул

1. Van der Kamp Н. Neonatal screening for congenital adrenal hyperplasia. Eur J Endocrinol. 2004;151:(Suppl_3):U71-U75. doi:10.1530/eje.0.151U071.

2. Urban MD, Lee PA, Migeon CJ. Adult height and fertility in men with congenital virilizing adrenal hyperplasia. N Engl J Med. 1978;299(25):1392-1396. doi: 10.1056/NEJM197812212992505.

3. Reisch N, Flade L, Scherr M, Rottenkolber M, Pedrosa GilF, Bidlingmaier M, et al. High prevalence of reduced fecundity in men with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2009;94(5):1665-1670. doi: 10.1210/jc.2008-1414.

4. Falhammar H, Nystrom HF, Ekstrom U, et al. Fertility, sexuality and testicular adrenal rest tumors in adult males with congenital adrenal hyperplasia. Eur J Endocrinol Eur Fed Endocr Soc. 2012;166:441-449. doi: 10.1530/EJE-11-0828.

5. Jääskeläinen J, Kiekara O, Hippeläinen M, Voutilainen R. Pituitary gonadal axis and child rate in males with classical 21-hydroxylase deficiency. J Endocrinol Invest. 2014;23(1):23-27. doi: 10.1007/bf03343671.

6. Stikkelbroeck NMML, Otten BJ, Pasic A, Jager GJ, Sweep CGJF, Noordam K, et al. High Prevalence of Testicular Adrenal Rest Tumors, Impaired Spermatogenesis, and Leydig Cell Failure in Adolescent and Adult Males with Congenital Adrenal Hyperplasia. J Clin Endocrinol Metab. 2001;86912):5721-5728. doi:10.1210/jc.86.12.5721.

7. Claahseb-van der Grinten HL, Otten BJ, Stikkelbroeck MMK, et al. Testicular adrenal rest tumours in congenital afrenal hyperplasia. Beest Pract Res Clin Endocrinol Metab. 2009;23(2):209-220. doi:10.1016/j.beem.2008/09.007.

8. Hatano O, Takakusu A, Nomura M, Morohashi K-i. Identical origin of adrenal cortex and gonad revealed by expression profiles of Ad4BP/SF-1. Genes Cells. 1996;1(7):663-671. doi: 10.1046/j.1365-2443.1996.00254.x.

9. Val P, Jeays-Ward K, Swain A. Identification of a novel population of adrenal-like cells in the mammalian testis. Dev Biol. 2006;299(1):250-256. doi: 10.1016/j.ydbio.2006.07.030.

10. Claahsen-van der Grinten HL, Otten BJ, Sweep FCGJ, et al. Testicular Tumors in Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency Show Functional Features of Adrenocortical Tissue. The Journal of Clinical Endocrinology & Metababolism. 2007;92(9):3674-3680. doi:10.1210/jc.2007-0337.

11. Bercovici JP, Fiet J, Gibault L, et al. Testicular adrenal rest tumours in salt wasting congenital adrenal hyperplasia (in vivo annd in vitro studies). J Steroid Biochem Mol Biol. 2005;93(1):67-72. doi: 10.1016/j.jsbmb.2004.10.023.

12. Hamwi GJ, Gwinup G, Mostow JH, Besch PK. Activation of Testicular Adrenal Rest Tissue by Prolonged Excessive ACTH Production. J Clin Endocrinol Metab. 1963;23:861-869. doi: 10.1210/jcem-23-9-861.

13. Jain SH, Sadow PM, Nosé V, Dluhy RG. A patient with ectopic cortisol production derived from malignant testicular masses. Nat Clin Pract Endocrinol Metab. 2008;4(12):695-700. doi: 10.1038/ncpendmet0985.

14. Weeks DC, Walther MM, Stratakis CA, et al. Bilateral testicular adrenal rests after bilateral adrenalectomies in a cushingoid patient with von Hippel-Lindau disease. Urology. 2004;63(5):981-982. doi:10.1016/j.urology.2004.01.023.

15. Martinez-Aguayo A, Rocha A, Rojas N, Garcia C, Parra R, Lagos M, et al. Testicular adrenal rest tumors and Leydig and Sertoli cell function in boys with classical congenital adrenal hyperplasia. J Clin Endocrinol Metab. 2007;92(12):4583-4589. doi:10.1210?jc.2007-0383.

16. Cole TJ, Freeman JV, Preece MA. British 1990 growth reference centiles for weight, height, body mass index and head circumference fitted by maximum penalized likelihood. Stat Med. 1998;17(4):407-429. doi: 10.1002/(SICI)1097-0258(19980228)17:4<407::AID-SIM742> 3-0.CO;2-L.

17. Marshall WA, Tanner JM. Variations in the Pattern of Pubertal Changes in Boys. Arch Dis Child. 1970;45(239):13-23. doi: 10.1136/adc.45.239.13.

18. Claahsen-van der Grinten HL, Otten BJ, Hermus ARMM, et al. Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia can cause severe testisular damage. Fertil Steril. 2008;89(3):697-601. doi: 10.1016/j.fertnstert.2007.03.051.

19. Greulich WW, Pyle SI. Radiographic Atlas of Skeletal Development of the Hand and Wrist. Stanford University Press. 1959.

20. Stikkelbroeck NMML, Suliman HM, Otten BJ, Hermus ARMM, Blickman JG, Jager GJ. Testicular adrenal rest tumours in postpubertal males with congenital adrenal hyperplasia: sonographic and MR features. Eur Radiol. 2003;13(7):1597-1603. doi:10.1007/s00330-002-1786-3.

21. Delfino M, Elia J, Imbrogno N, Argese N, Mazzilli rR, Toscano V, et al. Testicular adrenal rest tumors in patients with congenital adrenal hypeplasia: prevalence and sonographic, hormonal, and seminal characteristics. J Ultrasound Med Off J Am Inst Ultrasound Med. 2012;31(3):383-388.

22. Kang MJ, Kim JH, Lee SH, et al, The prevalence of testicular adrenal rest tumors and associated factors in postpubertal patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency. Endocr J. 2011;58(6):501-508. doi: 10.1507/endocrj.K11E-034.

23. Nermoen I, Rorvik J, Holmedal SH, et al, High requency of adrenal myelolipomas and testicular adrenal rest tumours in adult Norwegian patients with classical congenital adrenal hyperplasia because of 21-hydroxylase deficiency. Clin Endocrinol (Oxf). 2011;75(6):753-759. doi: 10.1111/j.1365-2265.2011.04151.x.

24. Poyrazoglu S, Saka N, Agayev A, Yekeler E. Prevalence of testicular microlothiasis in males with congenital adrenal hyperplasia and its association with testicular adrenal rest tumors. Horm Res Paediatrics. 2010;73(6):443-448.