SELECT ISSUE

Revista Romana de PEDIATRIE | Volumul LXI, Nr. 1, An 2012
ISSN 1454-0398  |  e-ISSN 2069-6175
ISSN-L 1454-0398

Indexed in / abstracted by

Thomson Reuters Embase
Scopus Cross-ref
Index Copernicus
Ulrichs Ebsco Host
Medline - Ebsco PubMed

HIGHLIGHTS

Publicarea de articole stiintifice

Stimati cititori, va reamintim ca autorii primi ai articolelor stiintifice pot acumula 80 de credite EMC in urma publicarii. Daca un articol are mai multi autori, cele 80 de credite [...]

Premiul Societatii pentru autori

Incepand cu 2016, Societatea Romana de Pediatrie ofera Premiul Societatii - pentru autorii celor mai bune articole stiintifice publicate [...]

Plagiatul – in actualitate

Tema plagiatului este tot mai mult discutata in ultima vreme. Aparitia unor programe performante de cautare si identificare a similitudinilor intre texte [...]

Adrenal rest tumours in an adolescent with congenital adrenal hyperplasia – one year follow-up

, , , , , and

ABSTRACT

Congenital adrenal hyperplasia (CAH) is an inherited disorder resulting in impaired production of cortisol and aldosterone. Male patients with CAH may present testicular adrenal rest tumours (TART).

Aim. To discuss the embryological, clinical features and treatment options of TART in a patient with CAH.

Case report. We present a case of TART in a 16 years and 6 months adolescent with salt-wasting type of CAH. The patient had periodic clinical evaluations, complete metabolic and endocrinological profile which guided the treatment. He was diagnosed at 6 weeks, when he begun substitution treatment with hydrocortisone. He was a compliant patient until age 14 years and 8 months, when he started high school and moved from home. At the age of 15 years and 6 months the clinical exam showed bilateral testis enlargement. The scrotal ultrasound sowed bilateral non-homogeneous, extensive masses. Hormone profile revealed increased ACTH and 17-OHprogesterone levels. Treatment was made by changing the night dose of hydrocortisone into dexamethasone to prolong ACTH suppression. After one year of treatment the testicular masses underwent important volume decrease.

Conclusions. Puberty has a negative impact on treatment compliance, so increasing the risk for TART. They are not malignant, but can result in gonadal dysfunction and infertility. It is important to screen for these tumours in CAH patients, to inform, discuss and offer cryopreservation of semen as soon as possible.

Key words: congenital adrenal hyperplasia, testicular adrenal rest tumours, adolescent

Full tet | PDF

Leave a Reply