PATHOLOGY OF ENDOCRINE SYSTEM. Peerayut Sitthichaiyakul,, M.D.

Transcription

1 PATHOLOGY OF ENDOCRINE SYSTEM Peerayut Sitthichaiyakul,, M.D.

2 Content Pituitary gland Anterior pituitary gland Hyperpituitarism Hypopituitarism Posteior pituitary gland Posterior pituitary syndrome

3 Thyroid gland Hyperthyroidism Hypothyroidism Thyroiditis Goiters Neoplasm Parathyroid gland Hyperparathyroidism Hypoparathyroidism

4 Endocrine pancreas Diabetes mellitus Adrenal glands Adrenal cortex Hyperadrenalism Hypoadrenalism Adrenal medualla Pheochromocytoma

5 Anterior pituitary gland Pituitary gland Growth hormone Prolactin Adrenocorticotropic hormone (ACTH) Thyroid-stimulating hormone (TSH) Follicle-stimulating hormone (FSH) Luteinizing hormone (LH) Hyperpituitarism : tumor, hyperplasia Hypopituitarism : Ischemia, injury, inflammation

6 Hyperpituitarism Prolactin Galactorrhea, amenorrhea, infertility Growth hormone Gigantism Acromegaly Adrenocorticotropic hormone Cushing syndrome Cushing disease

7 Hypopituitarism Traumatic brain injury and subarachnoid hemorrhage : most common cause Ischemic necrosis : Sheehan syndrome (postpartum ischemic necrosis)

8 Posterior pituitary syndrome Antidiuretic hormone (ADH) Diabetes insipidus (DI) ADH insufficiency Excessive urination Dehygration Syndrome of inappropriate ADH secrestion (SIADH) ADH excess Hyponatremia

9 Hyperthyroidism Thyrotoxicosis Thyroid gland Hypermetabolic state and overactivity of sympathetic nervous system Cardiac manifestration : most earilest manifestration tachycardia, palpiatation Sweating, heat intorance, increased appetite, weight loss, insomnia, Muscle weakness and osteoporosis Thyroid ophthalmopathy Malabsorption, diarrhea

10 Three common cause Diffuse hyperplasia (Graves disease) Hyperfunctional multinodular goiter Hyperfunctional adenoma Grave disease Hyperthyroidism due to diffuse enlarged thyroid glands Exophthalmos Pretibial myxedema Antibody to TSH receptors

11 Hypothyroidism Cause Developmental anomalies Post surgery or irradiaiton Autoimmune Iodine deficiency Drugs Pituitary failure

12 Cretinism Mental retardation Short stature Protruding tongue and umbilical hernia Myxedema Slowing of physical and mental activities Fatigue, apathy Cold intolerance and weight gain

13 Thyroiditis Hashimoto thyroiditis Most common cause of hypothyroidism Autoimmune to thyroglobulin and thyroid peroxidase Painless enlargement of thyroid gland

14 Goiter Enlargement of thyroid gland Most common manifestation of thyroid disease Clinically euthyroid Diffuse nontoxic (simple) goiter Multinodular goiter Toxic multinodular goiter (Plummer syndrome)

15 Neoplasm Solitary thyroid nodule Adenoma (follicular adenoma) Nonfunctional adenoma Toxic adenoma : small proportion Solitary and encapsulated lesion Unilateral painless mass

16 Carcinoma Follicular cell Papillary carcinoma (85%) Follicular carcinoma (5-15%) Anaplastic carcinoma (5%) Parafollicular cell (C-cell) Medullary carcinoma (5%) Pathogenesis Genetic abnormalities Environmental factors : radiation

17 Clinical manifestation Thyroid nodule Cervical lymph node metastasis Hoarseness, dysphagia, cough, dyspnea Hematogenous metastasis Enlarge bulky neck mass

18 Parathyroid glands Hyperparathyroidism Hypercalcemia Primary hyperparathyroism Parathyroid adenoma (85-95%) Primary hyperplasia (5-10%) Parathyroid carcinoma (~1%)

19 Clinical features Asymtomatic - Asymptomatic hypercalcemia Symptomatic - Bone pain : fracture from osteoporosis - Renal stones - GI disturbance : constipation, peptic ulcers - Central nervous system alterations - Muscle weakness and fatigue - Aortic and mitral valve calcifiaction

20 Secondary hyperparathyroidism Associated with hypocalcemia - Renal failure : most common cause - Inadequate intake of calcium -Steatorrhea - Vitamin D deficiency

21 Hypoparathyroidism Surgery Autoimmune conditions Genetic defect Congenital absence Clinical features Hypocalcemia : tetany Mental status change Calcification Dental abnormalities

22 Endocrine pancreas Diabetes mellitus Group of metabolic disorders sharing the common underlying feature of hyperglycemia Defects of insulin secretion or insulin action or both

23 Diagnosis Random glucose concentration greater than 200 mg/dl with classical signs and symptoms (polyuria, polydipsia and polyphagia) Fasting glucose concentration greater than 126 mg/dl Abnormal oral glucose tolerance test (OGTT), in which glucose concentration > 200 mg/dl 2 hours after standard carbohydrate load

24 Type 1 Diabetes Autoimmune diseases Pancreatic β-cell destruction Absolute deficiency of insulin 5-10% of all cases Most common subtype diagnosed in patient younger than 20 years Type 2 diabetes Insulin resistance: decreased response of peripheral tissue to insulin

25 Clinical features Hyperglycemia glycosuria polyuria Renal water loss with hyperosmolarity deplete intracellular water trigger thirst center in the brain polydipsia Deficiency of insulin catabolism of protein and fat polyphagia

26 Complications Macrovascular disease Large- and medium-sized muscular arteries Accelerated atherosclerosis Myocardial infarction Stroke Lower extremity gangrene

27 Microvascular disease Capillary dysfunction in target organs Diabetic retinopathy Diabetic nephropathy Diabetic neuropathy

28 Adrenal glands Adrenocortical hyperfunction Cushing syndrome Hypercortisolism Elevated glucocorticoid level Exogenous cause (iatrogenic cushing syndrome) Endogenous cause ACTH-dependent : Cushing disease (Pituitary adenoma) ACTH-independent : Adrenal neoplasm and adrenal hyperplasia

29 Clinical features Hypertension Weight gain Truncal obesity, moon facies, buffalo hump Decreased muscle mass and, proximal limb weakness Secondary diabetes Glucocorticoid: induce gluconeogenesis, inhibit glucose uptake by cells

30 Thin skin, fragile and easily bruised Poor wound healing Bone resorption : osteoporosis Increased risk of infections Mental disturbance Hirsutism and menstrual abnormalities

31 Primary hyperaldoteronism Chronic excess of aldosterone secretion Primary hyperaldosteronism Autonomous overproduction of aldosterone Decreased plasma renin activity Hypertension : most common manifestation

32 Clinical features Hypertension Most common of secondary hypertension Cardiovascular compromised : stroke, MI Hypokalemia

33 Adrenogenital syndrome Disorder of sexual differentiation Adrenal causes of androgen excess Congenital adrenal hyperplasia Autosomal recessive Deficiency of enzyme involved in the biosynthesis of cortical steroids Increased production of androgen

34 Clinical features Androgen excess Female: musculinization, clitoral hypertrophy, pseudohermaphrodite, oligomenorrhea, hirsutism, acne in postpubertal woman Male: enlargement of external genitalia, precocious puberty, oligospermia CAH should be suspected in any neonate with ambiguous genitalia Deficiency of glucocorticoids and mineralocorticoids

35 Adrenocortical insufficiency Primary hypoadrenalism Primary acute: adrenal crisis Crisis: chronic adrenocortical insufficiency precipitated by any form of stress Maintained external corticosteroid with rapid withdrawal of steoids Massive adrenal hemorrhage : Waterhouse- Friderichsen syndrome

36 Primary chronic adrenocortical insufficiency (Addison disease) 90% of cases Autoimmune adrenalitis (60-70% of cases) Tuberculosis and fungi AIDS Metastatic cancers

37 Clinical features Insidious onset Decreased glucocorticoids and mineralocorticoids level Progressive weakness and easy fatigability Gastrointestinal disturbances Anorexia, nausea, vomiting, weight loss and diarrhea

38 Hyperpigmentation : sun-exposed areas and pressure points Elevated level of pro-opiomelanocortin (POMC) precursor of ACTH and MSH Potassium retention, sodium loss Volume depletion with hypotension Hypoglycemia Adrenal crisis

39 Secondary adrenocortical insufficiency Disorder of hypothalamus or pituitary gland Metastatic cancer, infection, infarction, irradiation Lacking of hyperpigmentation Decreased cortisol and androgen Normal or near-normal aldosterone level

40 Adrenal medulla Pheochromocytoma Hypertension: 90% of cases Paroxysmal episodes: two third of cases Paroxysm may be precipitated emotional stress, exercise, change in posture and palpation in the region of tumor Congestive heart failure, pulmonary edema, myocardial infarction, ventricular fibrillation or cardiovascular accident