Disorders of the adrenal cortex

Hyperfunction:

▪ Hyperaldosteronism: excess aldosterone

– Primary = Conn’s disease

– Secondary = RAS hyperactivity

▪ Cushing syndrome: Excess cortisol

▪ Adrenal hyperplasia: excess sex hormones

Hypofunction:

▪ Addison’s disease

Disorders of the Adrenal medulla

▪ Hyperfunction

– Tumors of chromaffin cells (pheochromocytoma)

– Secrete catecholamines (continuous or episodic)

Conn’s Disease

▪ Increased aldosterone secretion in the absence of activation of the renin-angiotensin-aldosterone-system (RAS)

Causes:

▪ Adrenal hyperplasia

▪ Tumors: adenoma, carcinoma

Pathophysiology

▪ Salt and water retention

▪ Hypertension

▪ Hypokalemia

▪ Alkalosis

– Hydrogen is excreted with potassium

Cushing’s syndrome

▪ Increased cortisol secretion

Causes:

▪ Iatrogenic (most common)

– Long-term steroid and/or ACTH therapy

▪ Adrenal adenoma or carcinoma

▪ Cushing’s disease

– Increased pituitary ACTH

▪ Ectopic ACTH secretion (parneoplastic syndrome)

Manifestations

▪ Obesity

– Notably in the face (‘moon face’) and upper back (buffalo hump)

– Truncal obesity (most commonly from steroid therapy)

▪ Infection:

– Excess steroids impair the function of WBCs and therefore have anti-inflammatory/ anti-immune effects

– On the long-term the depressed immune system lead to increased susceptibility to infection

– Important to educate on steroid therapy; caution and monitoring if long term

▪ Hypertension

▪ Peripheral edema (from hypervolemia)

▪ Hyperglycemia; secondary DM on the long term

▪ Osteoporosis

▪ Recurrent infections

▪ Psychosis

▪ Thin and atrophic skin with bruises

Addison’s Disease

▪ Destruction of all zones of adrenal cortex

– Includes glucocorticoids, mineralocorticoids, androgens

Causes

▪ Primary (adrenal gland disorder)

– Usually autoimmune-mediated destruction of the adrenal glands (represents 80% of cases)

– Chronic infection, such as Tuberculosis (TB adrenalitis) (approximately 20% of cases)

▪ Secondary (pituitary gland disorder)

– Low ACTH secretion

– Sudden withdrawal of prolonged steroid therapy

– Destruction of the pituitary gland (e.g. from trauma or a tumor)

Manifestations

– Fatigue and muscle weakness

– Hypotension

– Anorexia

– Nausea and vomiting

– Abdominal pain

– Weight loss

– Hyperpigmentation

o High ACTH can bind to melanocyte in dermal areas which darkens skin

– Mental depression

– Acute severe conditions (Addisonian Crisis)

o Sudden drop in BP from hypovolemia (shock). Can occur from sudden withdrawal in steroid therapy

o Can be fatal

Pheochromocytoma

▪ Chromaffin cell tumors

▪ Neuroendocrine tumor of adrenal medulla secretes catecholamines leading to overstimulation of the SNS.

Clinical manifestations

▪ Hypertension

– Severe + paroxysmal

– Resistant to treatment

▪ Headache

▪ Palpitations and tachycardia

▪ Sweating

▪ Anxiety and tremors

▪ Hyperglycemia

Complications:

– Pheo crisis: in response to anesthesia, stress, drugs

– Cardiomyopathy

Lab features

▪ 24-hour urinary catecholamines and metanephrines (most specific)

▪ Urine vanillylmandelic acid (VMA)

– Tumor marker for pheochromocytoma

▪ Plasma metanephrine

4: Alterations of the Reproductive system

Selected Disorders of the Female Reproductive System

▪ Hormonal and menstrual alterations

– Amenorrhea (cessation of menstruation)

– Anovulation (failure of mid cycle ovulation)

– Dysmenorrhea and Premenstrual tension syndrome (painful menstruation)

– Dysfunctional uterine bleeding (uterine bleeding of undetermined cause)

▪ Polycystic ovarian syndrome

▪ Alterations of sexual maturation

– Delayed puberty

– Precocious puberty

▪ Sexual dysfunction

– Disorders of sexual desire (decreased or increased)

– Anorgasmia (orgasmic dysfunction)

– Dyspareunia (painful intercourse)

▪ Disorders of fertility

▪ Pelvic relaxation disorders

– Cystocele (bladder hernia), rectocele (rectum protrusion/drop), urethrocele (prolapse of the female urethra)

– Vaginal prolapse

– Uterine prolapse

▪ Growths and proliferative conditions

– Endometriosis

– Benign ovarian cysts

– Endometrial polyps

– Leiomyomas

– Cancers: cervical cancer, vaginal cancer, endometrial, vulvar cancer, ovarian

Selected Disorders of the Male Reproductive System

▪ Disorders of the urethra

– Urethritis

– Urethral strictures

scrotum and testes

– Varicocele (varicose veins in the scrotum; rarely painful but can → infertility)

– Hydrocele (fluid in the scrotum; a painless swelling of one or both testicles)

– Spermatocele (painless fluid filled cyst in the epididymis)

– Testicular torsion (Twisting of the spermatic cord → severe testicular pain)

– Epididymitis (inflammation of epididymis → severe testicular pain)

▪ Disorders of fertility

▪ Disorders of the prostate

– Benign prostatic hyperplasia

– Prostate cancer

– Prostatitis

▪ Erectile dysfunction

▪ Disorders of the breast

– Gynecomastia

– Breast cancer

Next Page