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CLINICAL DISORDERS OF PITUITARY LEARNING OBJECTIVES • Be able to identify the parts of the pituitary gland • Be able to identify hormones secreted by each lobe and their main actions • Understand the common diseases caused by excess or deficiency of pituitary hormones • Understand the common clinical presentation of these disorders Anatomy of pituitary gland • Was first called “Hypophysis “ by Thomas in 1778. • Pea-sized gland located at middle cranial fossa within a recess of the sphenoid bone called “Sella Turcica”. • Suspended from hypothalamus by a stalk (infandibulum) • Has mainly 2 lobes: – Anterior gland: – Posterior gland: Adenohypophysis Neurohypophysis Anatomy of pituitary gland Anatomy of pituitary gland Development of Human Anterior Pituitary Anterior Pituitary Function Corticotroph Gonadotr Thyrotrop Lactotro oph h ph Hormone ACTH FSH, LH TSH Prolacti n Stimulat CRH, gp-130 GnRH, TRH Estroge ors cytokines Estrogen n, TRH Inhibitor Glucocortic Sex T3, T4, Dopami s oids steroids, Dopamine, ne inhibin Somatosta tin, GH Target Adrenals Ovary, Thyroid Breast Gland Testes and other tissues Somatotro ph GH GHRH, GHS Somatosta tin, IGF-1, Activins Liver, bone and other tissues Trophic Steroid & Sex Effects androgens Steroid, production Follicular growth, Germ Cell maturatio n T4 Milk IGF-1 synthesis Producti production and on , Growth secretion induction, Insulin antagonis m Etiology of Hyper/Hypo Pituitary-Hypothalamic Lesions • Non-Functioning Pituitary Adenomas • Endocrine active pituitary adenomas – – Prolactinoma -Somatotropinoma -Corticotropinoma Thyrotropinoma -Mixed endocrine adenomas • Malignant pituitary tumors • Metastases in the pituitary (breast, lung, stomach, kidney) • Pituitary cysts: Rathke's cleft cyst Mucocoeles, Others • Empty sella syndrome Etiology of Hyper/Hypo Pituitary-Hypothalamic Lesions…cont • Developmental abnormalities: Craniopharyngioma, Germinoma, Others • Primary CNS Tumors: Perisellar meningioma Hemangioblastoma Optic glioma Others • Malignant systemic diseases: Hodgkin'slymphoma Leukemic infiltration Eosinophilic granuloma Non-Hodgkin lymphoma Histiocystosis X Giant cell granuloma • Granulomatous diseases: Neurosarcoidosis Syphilis PITUIOTARY ADENOMA Wegner's granulomatosis, Tuberculosis, CRANIOPHARYNGIOMA PITUITARY ADENOMA LYMPHOCYTIC HYPOPHYSITIS INFILTRATIVE DISORDER: SARCOIDOSIS CLINICAL DISORDERS OF PITUITARY FUNCTION • Due to Hypo-secretion of Pituitary Hormones - Central Hypoadrenalism - Hypogonadism, - Hypothyroidism - GH deficiency - Pan-Hypopituitarism - Diabetes Insipidus • Due to Hyper-secretion of Pituitary Hormones - Hyperprolactinemia - Acromegaly - Cushing’s Disease - SIADH ANTERIOR PITUITARY CLINICAL DISORDERS EXCESS GROWTH HORMONE • GIGANTISM IN CHILDREN: • ACROMEGALY IN ADULTS • • • • ESSENTIALS OF DIAGNOSIS ACROMEGALY/GIGANTISM Clinical features plus; Elevated serum levels of IGF-1 (screening). Elevated serum GH levels, NOT suppressed following oral glucose test. MRI/CT brain. Symptoms and signs at presentation Facial change, acral enlargement, and soft-tissue swelling Excessive sweating Acroparesthesiae/ carpal tunnel syndrome Tiredness and lethargy Headaches Oligo- or amenorrhea, infertility Erectile dysfunction and/or decreased libido Arthropathy Impaired glucose tolerance/ diabetes Goiter Ear, nose throat and dental problems Congestive cardiac failure/ arrythmia Overall prevalence (%) 100 83 68 53 53 55 42 37 37 35 32 25 Hypertension 23 COMPLICATIONS OF ACROMEGALY Cardiovascular Ischemic heart disease Cardiomyopathy Heart failure Arrhythmias Hypertension Respiratory Kyphosis Obstructive sleep apnea Metabolic Diabetes mellitus/IGT Hyperlipidemia Neurologic Carpal Tunnel syndrome Stroke Neoplastic Colorectal (Breast and prostate - uncertain) Musculoskeletal Degenerative arthropathy Calcific discopathy, pyrophosphate arthropathy GROWTH HORMONE DEFICIENCY CLINICAL FEATURES: • Short stature • Delayed tooth development • Delayed puberty • Low energy • Decrease strength and exercise tolerance • Decreased muscle mass • Weight gain, especially around the waist ESSENTIALS OF DIAGNSIS GH-DEFICIENCY Low serum GH & IGF-1 level. Provocative GH-stimilation test: • Intravenous GHRH/Arginine sulfhydryl test. • Oral Clonidine/Levodopa (in pt’s pretreated with propranolol or estrogen). Note: GH deficiency is present in 96% of patients with three or more other pituitary hormone deficiencies. CUSHING’S DISEASE (ACTH EXCESS) CUSHING SYNDROME vs. CUSHING DISEASE • CUSHING’S SYNDROME: Is a syndrome due to excess cortisol from pituitary, adrenal or other sources (exogenous glucocorticoids, ectopic ACTH, etc.) • CUSHING’S DISEASE: Is hypercortisolism due to excess pituitary secretion of ACTH (about 70% of cases of endogenous Cushing’s syndrome) CLINICAL PRESENTATIONS OF CUSHING DISEASE • Moon facies • Facial plethora • Supraclavicular fat pads • Buffalo hump • Truncal obesity • Weight gain • Purple striae • Proximal muscle weakness • Easy bruising • Hirsutism • Hypertension • Osteopenia • Diabetes mellitus/IGT • Impaired immune function/poor wound healing Central Obesity in Cushing’s Disease Progressive Obesity of Cushing’s Disease AGE 6 AGE 7 AGE 8 9 AGE 11 Buffalo Hump & Striae in Cushing’s Disease AGE ESSENTIALS OF DIAGNSIS CUSHING’S DISEASE • Clinical features plus; • Plasma ACTH level is elevated. • Plasma cortisol levels are also high. • MRI/CT brain. CENTRAL HYPOADRENALISM (ACTH DEFICIENCY) • Acute ACTH deficiency is serious condition and can lead to death if not recognized and treated properly • CLINICAL FEATURES: Lethargy Malaise Anorexia Vomiting Abdominal pain Dizziness Weight loss Postural hypotension Pale skin Shock Coma HYPERPROLACTINEMIAS & PROLACTINOMAS Causes: • Prolactinoma • Pituitary stalk compression by non pitiutary tumor • Primary hypothyroidism • Drugs • • • • • • CRF Liver cirrhosis PCOS Acromegaly Pregnancy Lactation HYPERPROLACTINEMIAS & PROLACTINOMAS…cont CLINICAL FEATURES: • Women may present with oligomenorrhea, amenorrhea, galactorrhea or infertility. • Men often have less symptoms than women (sexual dysfunction, visual problems, or headache) and are diagnosed later. • In both sexes, tumor mass effects may cause visual-field defects or headache ESSENTIALS OF DIAGNSIS PROLACTINEMIA • Clinical features plus; • Elevated serum Prolactin levels. • MRI/CT brain. CENTRAL/SECONDARY HYPERTHYROIDISM CLINICAL FEATURES: Skin warm & sweaty Heat intolerance Palmar erythma Thinning of hair Tachycardia Dyspnea High output heart failure Weight loss Atrial fibrillation hyperdefecation Malabsorption Gynecomastia Decrease libido Erectile dysfunction Osteoporosis/fractures Anxiety Insomnia HYPERTHYROIDISM….cont • Palmar erythema • Clubbing (thyroid acropachy) Atrial fibrillation CENTRAL/SECONDARY HYPOTHYROIDISM CLINICAL FEATURES: Cold intolerance Somnolence Lethargy Impaired memory Cold pale dry skin Bardycardia Sluggishness HYPOTHYROIDISM…cont Weight gain Constipation Fatigue Weight gain Menstrual abnormalities Dry fine hair Hypothermia Pretibial myxedema Pretibial Myxedema HYPOTHYROIDISM….cont Hypothyroidism in chidren; Cretinism (meaning Christ like) ESSENTIALS OF DIAGNSIS TSH EXCESS OR DEFICIENCY • • • • Clinical features plus; Serum TSH level. Serum T3 & T4 levels. MRI/CT brain. GONADOTROPHIN DEFICIENCY (FSH/LH DEFICIENCY) CLINICAL FEATURES: • Male: Erectile Dysfunction Decreased libido Fatigue Loss of muscle mass Small & soft testes Infertility Loss of facial, axillary & pubic hair • Female: Amenorrhea Hot flushes Failure to lactate Breast atrophy Decreased axillary and pubic hairs Thin skin with fine wrinkling ESSENTIALS OF DIAGNSIS (FSH/LH DEFICIENCY) Clinical features plus; Low serum levels of FSH & LH. MRI/CT brain. POSTERIOR PITUITARY CLINICAL DISORDERS POSTERIOR HYPER-PITUITARY DISORDERS • Syndrome of inappropriate secretion of Anti-diuretic hormone, SIADH (TOO MUCH ADH!!) • CAUSES: Lung cancer Ca duodenum/pancreas Lung disease CNS disorders/infections/ Drugs Trauma CLINICAL FEATURES OF SIADH • Weight gain. • Decrease urine output • Decrease serum Na levels. – weakness – muscle cramps – Diarrhea – Seizueres POSTERIOR HYPO-PITUITARY DISORDERS DIABETES INSIPIDUS (too little ADH) • 50% idiopathic – a. central -- i.e. brain tumors – b. nephrogenic - inability of tubules to respond to ADH CLINICAL FEATURES OF DIABETES INSIPIDUS • Polydipsia • Polyuria (10L in 24 hours) • Severe fluid volume deficit – wt loss – tachycardia – constipation – shock ESSENTIALS OF DIAGNSIS DIABETES INSIPIDUS • • Clinical features plus; • A supervised “vasopressin challenge test” (for central DI). • Serum vasopressin measurement with modest fluid restriction (for nephrogenic DI). • Serum & urine osmolality. 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