Question Scenario 1 • Make a scenario of an MCQ. Outline the signs and symptoms that you would expect in a patient with hyposecretion of adrenocortical hormones. Give 4 options for the MCQ question. Scenario 2 • Make a scenario of an MCQ. Outline the signs and symptoms that you would expect in a patient with hypersecretion of adrenocortical hormones. Give 4 options for the MCQ question. Scenario 1 (Mian Seher Munir) An obese 50-year old man presents with high blood glucose levels. He shows buffalo torso and moon face and is complaining of purple striae after injury. The laboratory blood tests show high blood RBC’s and relative polycythemia, hypokalemia and hyponatremia. He is most probably suffering from: a.Tumour of adrenal cortex b.Tumour of adrenal medulla c.Pituitary adenoma d.Increased oral steroid intake Scenario 2 (Adil Maqbool) A 40-year old woman presents to the physician with symptoms of: •Swelling in the face •Hypertension •Excessive facial hair growth •Abdominal fat deposition •Polydisia The lab results show: •↑ levels of ADH •↓ Na in urine •High plasma levels of ACTH & cortisol •↑ levels of blood glucose upto 200 mg/dl The most appropriate diagnosis is: a.Cushing syndrome b.Diabetes mellitus c.Tumor of Pituitary gland (corticotropes) d.Conn’s syndrome Scenario 3 & 4 Scenario 3 ( M. Halim) A patient was presented to the hospital having high blood pressure. His lab tests show: •Hypernatremia •Hypokalemia •High blood pH and ↓ urinary pH Which of the following disorder is he most probably suffering from: a.Metabolic alkalosis b.Diabetes insipidus c.Hypersecretion of Aldosterone d.Hypersecretion of Cortisol e.Tumour of Adrenal Medulla Scenario 4 (Mahnoor) A woman comes into the clinic and complains to the doctor about her lethargicness. The doctor observes blotches on her face along with hypertrichosis. She complains about being sick more than usual and irregular menstrual cycles. Her blood report showed ↑ glucose levels and hypokalemia was also observed. What do you think this patient is suffering from: a.Diabetes mellitus b.Hyposecretion of ACTH c.Hypersecretion of ACTH d.Hypothyroidism Scenario 5 & 6 Scenario 5 (Wadia Imtiaz) Scenario 6 (Khizra) A female of about 45 years presents to the hospital with polyuria, dehrdration, sluggishness, muscle wasting and skin pigmentation in blotches. Clinical examination reveals: •Hypotension • K levels of 7 mmol/L (Hyperkalemia) •Mild acidosis A person reported to the physician with high blood pressure, muscle weakness, nervousness, lightheadedness, numbness and hair on the face. His lab reports show decrease in K levels, decreased eosinophils and basophils, increased RBC’s and platelet count. What is the appropriate diagnosis: a.Addison’s disease b.Primary atrophy of adrenal cortices c.Primary aldosteronism d.Cushing’s syndrome What can be the most likely diagnosis? a.Leukopenia b.Hyposecretion of adrenocortical hormones c.Hypersecretion of adrenocortical hormones d.Metabolic acidosis e.Polycythemia Scenario 7 (Qasam) A patient reports to the doctor of lethargic condition with polyphagia. Upon his vital examination his/her pulse and blood pressure were low. Sexually, he/she has slow/ decreased libido and hyposecretion of sex hormones. Upon his urinary and blood glucose test, following are the values: •Urine: hyperkalemia hyervolemia hypernatremia •Blood sugar (fasting level): lower than 90 mg/ dl •Blood pH: less than 7.34 (acidosis) which hormone deficiency is possible: a.Glucocorticoid b.Mineralocorticoid c.Catecholamine d.Adrenocortical hormones e.None of the above Scenario 8 (Nida) A patient reports to the doctor with edematous appearance of his face apparently moon face with excessive deposition of fats in thoracic and upper abdominal region. His blood pressure is also high. On examination doctor comes to know that the patient has excessive growth of facial hair and acne problems. Lab reports show that his plasma ACTH & cortisol levels are high. He also has: •Low WBC’s •Hyperglycemia and hyperkalemia What would you suspect that patient will be suffering from: a.Hypocortisolism b.Hypoadrenalism c.Hyperadrenalism d.Hyperthyroidism DISORDERS OF THE ADRENAL GLAND Dr. Ayisha Qureshi Assistant Professor, MBBS, MPhil ADRENAL CORTEX Hyper or Hyposecretion of hormones of the 3 layers may occur Zona Glomerulosa Aldosterone ↑ed CONN’s Syndrome Aldosterone ↓ed ADDISON’s Disease Zona Fasciculata Cortisol ↑ed: CUSHING Syndrome Cortisol ↓ed ADDISON Disease Zona Reticularis Androgen ↑ed ADRENO GENITAL Syndrome Androgen HYPO secretion Zona glomerulosa: HYPO & HYPERSECRETION OF ALDOSTERONE DEFINITION: Conn’s Syndrome is the disorder caused by the hypersecretion of Aldosterone due to primary cause or defect in the zona glomerulosa itself. PRIMARY ALDOSTERONISM (CONN’S SYNDROME) SYMPTOMS: 1. HTN 2. Hypervolemia 3. Hypokalemia leading to muscle weakness & paralysis CAUSE: 1. Zona Glomerulosa Tumour 2.Hypersecretin g adrenal cortex PATHOPHYSIOLOGY: 1. ↑ Na & water reabsorption leading to ↑ ECF vol. & blood vol. 2. ↑ H & K excretion DIAGNOSIS Primary (Conn’s) Aldosteronism Secondary Aldosteronism ↑ Aldosterone levels ↑ Aldosterone levels ↓ Renin-Angiotensin levels ↑ ReninAngiotensin levels leading to hyperplasia of zona glomerulosa PRIMARY ADRENOCORTICAL FAILURE Causes of Primary Adrenocortical failure: It may occur due to: • Addison’s disease • Congenital Adrenal Hypoplasia • Secondary Adrenocortical failure • Pituitary destruction DEFINITION: It is a disorder caused by the inability of the adrenal cortex to produce the cortical hormones. This is frequently caused by the primary atrophy of the adrenal cortex. The patient presents with mineralocorticoid and glucocorticoid deficiency. HYPOADRENALISMADDISON’S DISEASE CAUSE: The causes can be: 1.Autoimmune disorder 2.Tuberculous destruction of the glands 3.Cancer 4.Iron overload Signs & Symptoms • • • • • • • • • • Mineralocorticoid deficiency Greatly decreased renal sodium reabsorption. Increased loss of Na, Cl & water in the urine. Decreased ECF leading to hypovolemia leading to dehydration. Hyponatremia leading to cramps. Hyperkalemia. Mild Acidosis. Hypotension. Decreased Cardiac output. Shock. Death within 4 days to 2 weeks after complete cessation of the aldosterone production. Glucocorticoid deficiency • • • • • • Patient cannot maintain normal blood glucose levels as no gluconeogenesis b/w meals. Patient cannot Fast. Nausea, vomiting, fever, diorrheoa and anemia. All metabolisms effected leading to fatigue and sluggishness. Patient highly susceptible to the deteriorating effects of stress. Even mild infections can lead to death. Pigmentation of the skin in the form of blotches b/c of increased secretion of ACTH as feedback. These brownishblack areas appear in axilla, genital areas, lips, buccal mucosa. TREATMENT • A person with complete destruction of the adrenal may die within a few days b/c of weakness & circulatory shock. • However, if small quantities of mineralo & glucocorticoids are administered daily, they can live for years. ADDISONIAN CRISIS • In a person with Addison's disease, the output of glucocorticoids does not increase during stress. Yet whenever different types of trauma, disease, or other stresses, such as surgical operations, supervene, a person is likely to have an acute need for excessive amounts of glucocorticoids and often must be given 10 or more times the normal quantities of glucocorticoids to prevent death. • This critical need for extra glucocorticoids and the associated severe disease state in times of stress is called an addisonian crisis. DEFINITION: Hypersecretion by the adrenal cortex causes a complex cascade of hormone effects called Cushing's syndrome. Many of the abnormalities are due to abnormal amounts of cortisol but increased amounts of aldosterone and androgens are also responsible. HYPERADRENALISMCUSHING’S SYNDROME CAUSES: • Exogenous • ACTH-Dependant - Pituitary adenoma - Ectopic ACTH syndrome • ACTH-Independant - Adrenal adenoma - Adrenal carcinoma When Cushing’s syndrome is secondary to excess secretion of ACTH by the anterior pituitary, it is called CUSHING’S DISEASE. Signs & Symptoms • Truncal Obesity • Supraclavicular & dorsal fat pad • Buffalo hump (diagnostic) • Moon Facies due to deposition in the cheeks and temporal area of the face, with a fish-like mouth. • Purple striae due to decreased proteins in the collagen fibres. This causes the collagen fibres to tear easily and leads to the formation of purple striae. • Thin skin. • Capillary fragility & easy bruising. • Hirsutism (excess facial hair growth) and acne vulgaris–due to increased androgens. • • • • • • • • • Hb percentage is raised. Depression and psychosis. Diabetes Mellitus also called Adrenal Diabetes. Severe muscular weakness due to increased protein catabolism. Thin extremities due to muscle wasting and protein catabolism. This leads to myopathy and inability of the patient to stand up from a sitting position. Infections due to suppressed immune system with impaired wound healing. Osteoporosis leading to frequent fractures. Hypertension and hypokalemia due to increased aldosterone. Amenorrhoea. Purple striae due to collagen breakdown in the subcutaneous tissue: Diagnosis • Very challenging. • Diagnose excess Cortisol. • Dexamethasone suppression test: this test helps differentiate between ACTHdependant and ACTH-independent Cushing’s syndrome. • Confirmation of cause. TREATMENT • Removal of the cause: Surgery of the adrenal tumour OR pituitary tumour. • Drugs that block steroidogenesis.e.g. Ketaconazole. • Drugs that inhibit ACTH secretion.e.g. Serotonin antagonists. • Partial or total adrenalectomy. The treatment is followed by administration of adrenal steroids to make up for any insufficiency that may arise. DEFINITIONS: It is a disorder caused by an occasional adrenocortical tumor secreting excessive quantities of androgens that cause intense masculinizing effects throughout the body. ADRENOGENITAL SYNDROME SIGNS & SYMPTOMS - IN FEMALES Virile characteristics in women & girls. Hirsutism: hair on the face, chin and upper lips. Deeper voice. Occasionally baldness. Masculine distribution of hair on the body. Deposition of proteins in a masculine manner. IN MALES - - - - Precocious development in boys. In prepubertal male, a virilizing adrenal tumour causes same characteristics as in the female plus rapid development of the male sex organs. In adult male, the virilising characteristics are often masked by the virilising effects of Testosterone. Diagnosis is made by the presence of excess 17ketosteroids in the urine.