leuprolide and Hypertension

Deficiency in 11β-hydroxylase as a cause of congenital adrenal hyperplasia is uncommon. It should be considered in the differential diagnosis of hypertension with virilization in any prepubescent child.. A 12-year-old Asian boy from eastern Nepal presented with pain in his abdomen and hypertension. He was raised as a male but had absent testicles since birth and had precocious puberty. Plasma testosterone, follicle-stimulating hormone, and luteinizing hormone were below baseline level. Basal 17-hydroxyprogesterone was elevated. Magnetic resonance imaging of his pelvis showed presence of Müllerian structures and karyotyping revealed 46,XX genotype. A clinical diagnosis of 11β-hydroxylase deficiency was made in view of hypertension with severe virilization in a 46,XX individual. Our patient's legal guardian was unwilling for our patient to change gender and because our patient is underage, the condition was well explained to his parents. He was managed with steroids and antihypertensive drugs. He was on regular follow-up; after 2 years there was no hypertension but he developed true puberty with functional ovaries. He was prescribed leuprolide (gonadotropin-releasing hormone analogue), letrozole (aromatase inhibitor), and a continuation of antihypertensive drugs.. This case highlights the importance of a thorough physical examination of the external genitalia at birth and appropriate referral, and addresses issues in the management of such a disorder. Ethical issues pertaining to consent and who is entitled to give it should be clear so that the affected individual will have optimal psychological development and quality of life.

Topics: 46, XX Disorders of Sex Development; Abdomen; Adrenal Hyperplasia, Congenital; Child; Female; Gonadotropin-Releasing Hormone; Humans; Hypertension; Leuprolide; Magnetic Resonance Imaging; Male; Nepal; Puberty, Precocious

A case of a 32-year-old nulliparous white woman referred for a 5-year history of severe hypertension, hypokalemia, and resultant systolic dysfunction is presented. She additionally had a left ventricular ejection fraction of 30% including left ventricular dilation and normal left ventricular mass index, as measured by cardiac magnetic resonance imaging when she initially presented to us. Her history revealed that her severe hypertension episodes were monthly and would occur around the catamenial (menses-associated) time. Two weeks following her menses, blood pressure decreased significantly but remained elevated above 140/90 mm Hg. This cycle repeated monthly and required multiple hospitalizations for hypertensive emergency in the form of acute decompensated heart failure and severe headaches. She required potassium supplementation. This prompted a complete evaluation for secondary causes of hypertension, which was negative. Female and male sex hormone levels, including testosterone, were also within normal limits. She received an injection of leuprolide acetate depot (11.25 mg every 3 months), a gonadotropin-releasing hormone agonist. This significantly reduced the magnitude of these episodes.

Topics: Adult; Female; Fertility Agents, Female; Gonadotropin-Releasing Hormone; Humans; Hypertension; Leuprolide; Magnetic Resonance Imaging, Cine; Menstrual Cycle; Severity of Illness Index; Stroke Volume; Treatment Outcome; Ventricular Dysfunction, Left

The most frequent cause of virilization in postmenopausal women is excessive androgen production of ovarian origin. Bilateral oophorectomy is usually performed, even in cases of benign tumors or hyperthecosis. This is the first report of a case series of long-term GnRH-agonist treatment of hyperandrogenism in postmenopausal women.. We present three women with postmenopausal hyperandrogenism of ovarian origin who were treated with GnRH agonists.. We describe three cases of postmenopausal women with virilization and hyperandrogenism of presumed ovarian origin, all with slight enlargement of the ovaries but without visualization of a tumor, who had long-term treatment with GnRH agonists. No histological diagnosis was available, and therefore all patients received careful follow-up, including periodic testing of androgen levels and ovarian imaging by computed tomography scans. The three patients responded in different ways to treatment with GnRH agonists.. Long-term GnRH agonist treatment is an acceptable choice for treatment of postmenopausal hyperandrogenism in patients where ovarian origin of androgen excess is ascertained, and especially in those patients who have an increased risk for surgery due to comorbidities or who are unwilling to undergo bilateral oophorectomy.

Topics: Aged; Alopecia; Cardiovascular Diseases; Diabetes Mellitus, Type 2; Dyslipidemias; Female; Gonadal Steroid Hormones; Gonadotropin-Releasing Hormone; Hirsutism; Humans; Hyperandrogenism; Hypertension; Leuprolide; Liver Cirrhosis, Alcoholic; Magnetic Resonance Imaging; Middle Aged; Obesity; Ovary; Postmenopause; Tomography, X-Ray Computed

A 4-10/12 year-old boy presented with tall stature and advanced secondary sexual characteristics. His bone age was 13 years giving him a height prediction of 147 cm. An initial 11-deoxycortisol level of 13,770 ng/dl and associated hypertension suggested the diagnosis of 11-hydroxylase deficiency, which was confirmed by dexamethasone suppression and genotyping. Treatment strategy was based on the premise that known hypothalamic priming resulting in early pubertal development could be averted by delaying puberty with leuprolide; also that effects of hydrocortisone and leuprolide on attenuating growth could be counteracted by growth hormone. The combined treatment resulted in a final height at age 12 years which was 25.4 cm greater than predicted, and bone density above average. We conclude that delaying puberty until an appropriate age, offsetting growth suppression, and improving bone mineralization can be effectively achieved using glucocorticoids, leuprolide and growth hormone in patients with 11-hydroxylase deficiency.

Topics: Adrenal Hyperplasia, Congenital; Body Height; Bone Density; Child; Delayed-Action Preparations; Genotype; Human Growth Hormone; Humans; Hydrocortisone; Hypertension; Leuprolide; Longitudinal Studies; Male; Puberty, Precocious; Steroid 11-beta-Hydroxylase

In the syndrome of familial virilization, insulin resistance, and acanthosis nigricans, the interrelationships are not understood. Twin sisters were studied, along with a lesser affected sister and mother. They manifested amenorrhea, hirsutism, masculinization, hypertension, hyperinsulinemia, hypertriglyceridemia, and hyperprolactinemia. Medical therapy with a gonadotropin-releasing hormone agonist plus an antiandrogen resulted in reversal of the hirsutism, yet with preservation of potential fertility. In response to luteinizing hormone (LH) and follicle-stimulating hormone suppression, there was normalization of the serum androgens, but not of the hyperinsulinemia, hypertriglyceridemia, hyperprolactinemia, hypertension, or acanthosis nigricans.. (1) This syndrome may be familial. (2) Medical therapy for the virilization is successful. (3) The hyperandrogenemia is primarily LH dependent and not primarily insulin dependent, although insulin may have an amplification effect. (4) Hyperinsulinemia, hypertriglyceridemia, hyperprolactinemia, and the hypertension are not androgen dependent.

Topics: Acanthosis Nigricans; Adult; Androgen Antagonists; Antineoplastic Agents; Cyproterone; Cyproterone Acetate; Dexamethasone; Diseases in Twins; Family Health; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Hirsutism; Humans; Hyperinsulinism; Hyperlipidemias; Hyperprolactinemia; Hypertension; Insulin Resistance; Leuprolide; Luteinizing Hormone; Male; Pituitary Hormone-Releasing Hormones; Syndrome; Virilism