Effects of mitotane on testicular adrenal rest tumors in congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a retrospective series of five patients
نویسندگان
چکیده
We conducted a retrospective study on the long-term effect of mitotane treatment testicular adrenal rest tumors (TARTs) in five adult patients with classic 21-hydroxylase deficiency. After 60 months treatment, decrease steroids was observed four patients. Testicular ultrasonography showed complete disappearance TART two patients, stabilization and halving volume remaining patient. Sperm count improved notably who had normal baseline inhibin B levels small inclusions, thus enabling cryopreservation subjects’ semen. Four years follow-up these after withdrawal no recurrence persistent function. In conclusion, could be used as last resort CAH cases azoospermia associated TARTs but levels, it can improve endocrine exocrine
منابع مشابه
A Case of Bilateral Testicular Tumors Subsequently Diagnosed as Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
21-hydroxylase deficiency (21-OHD) caused congenital adrenal hyperplasia (CAH) is a group of autosomal recessive genetic disorders resulting from mutations in genes involved with cortisol (CO) synthesis in the adrenal glands. Testicular adrenal rest tumors (TARTs) are rarely the presenting symptoms of CAH. Here, we describe a case of simple virilizing CAH with TARTs, in a 15-year-old boy. The p...
متن کاملa case of bilateral testicular tumors subsequently diagnosed as congenital adrenal hyperplasia due to 21-hydroxylase deficiency
21-hydroxylase deficiency (21-ohd) caused congenital adrenal hyperplasia (cah) is a group of autosomal recessive genetic disorders resulting from mutations in genes involved with cortisol (co) synthesis in the adrenal glands. testicular adrenal rest tumors (tarts) are rarely the presenting symptoms of cah. here, we describe a case of simple virilizing cah with tarts, in a 15-year-old boy. the p...
متن کاملPrevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
OBJECTIVE Testicular adrenal rest tumours (TART) are a well-known complication in adult male patients with congenital adrenal hyperplasia (CAH), with a reported prevalence of up to 94%. In adulthood, the tumours are associated with gonadal dysfunction most probably due to longstanding obstruction of the seminiferous tubules. The aim of our study was to determine the presence of TART and their i...
متن کاملTesticular Adrenal Rest Tumors in Patients with Congenital Adrenal Hyperplasia
Congenital adrenal hyperplasia refers to a group of autosomal recessive disorders caused by a deficiency of an enzyme involved in the synthesis of glucocorticoids. The enzyme deficiency generally leads to a deficiency of cortisol and/or aldosterone production within the adrenal cortex. The lack of glucocorticoids generally leads to elevated levels of plasma corticotropin (ACTH), which often res...
متن کامل[Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency].
Congenital adrenal hyperplasia is a general term applied to a group of several inherited enzymatic defects of cortisol biosynthesis. The most frequent cause of this disease is by far 21-hydroxylase deficiency which is considered one of the commonest metabolic disorders. The degree to which the activity of this enzyme is diminished correlates with the severity of congenital adrenal hyperplasia a...
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ژورنال
عنوان ژورنال: European journal of endocrinology
سال: 2021
ISSN: ['1479-683X', '0804-4643']
DOI: https://doi.org/10.1530/eje-20-0787