Pituitary disorders

pituitary disorders

1. PITUITARY DISORDERS MS SAHELI CHAKRABORTY 1 ST YEAR MSC NURSING RAMAIAH INSTITUTE OF NURSING BANGALORE

2. PITUITARY DISORDERS Pituitary disorders are conditions caused by too much or too little of one or more of the hormones produced by the pituitary gland and can produce a variety of symptoms depending on which hormones and target tissues are affected.

3. HYPOPITUITARISM Hypopituitarism is a rare disorder where there is a loss of function in the pituitary and the failure to secrete hormones that affect many of the body's functions. Patients diagnosed with hypopituitarism may be deficient in one or several hormones or have complete pituitary failure.

4. ETIOLOGY Congenital Tumor Inflammation Infection Head injuries Lesions Surgery Tuberculosis Radiographic therapy

5. DWARFISM It is an endocrine disorder resulting from hypo secretion of growth hormone during critical development period in children. The term ‘short stature’ or little people is often used too.

6. TYPES OF DWARFISM:-

7. Lesion of the anterior pituitary due to infection or injury results in hypo secretion of growth hormone. Genetic disorders. Hereditary. Lesion of hypothalamus resulting in hypo secretion of growth hormone releasing factor.

8. CLINICAL FEATURES: Stunted physical and skeletal growth. The average height is 4 feet or less than that. But dwarfism could apply to an adult who is less than 4 feet 10 inch height. Low blood glucose level. Developmental of gonads may be normal IQ may be normal. DIAGNOSTIC EVALUATION: History collection. Physical examination. CT scan, MRI scan of brain. Blood test – to rule out hormone level.

9. MANAGEMENT

10. HYPERPITUITARISM Hyperpituitarism is the excessive production of growth hormone. Hyperpituitarism is a chronic, progressive, disease marked by hormonal dysfunction and startling skeletal overgrowth. Hyperpituitarism appears in 2 forms- Gigantism- affects infants and children. Acromegaly- affects adults after epiphyseal closure.

11. GIGANTISM :- Pituitary gland secretes growth hormone (GH) which is responsible for overall body development during childhood. When too much growth hormone is secreted that augments the growth of muscle, bones and connective tissue in childhood or adolescence before the end of puberty, the condition is called Gigantism. The result is an increase in height and formation of additional soft tissues. Some individuals may achieve a height in excess of eight feet.

12. ETIOLOGY In most of the cases, non-cancerous pituitary gland tumour is behind gigantism. Genetic mutation. McCune-Albright syndrome is a disorder that causes unusual growth of bone tissues, gland irregularities and patches of light-brown skin. Carney complex is a hereditary condition which is cancerous or non-cancerous endocrine tumours and spots of darker skin. Multiple endocrine neoplasia type 1 is also a hereditary condition which cause tumours in the pancreas, parathyroid glands and pituitary gland. Neurofibromatosis is a hereditary disease that causes tumours in the nervous system.

13. CLINICAL FEATURES:- Child will be much taller than other children of the same age. Parts of the body may be visibly bigger than other parts. Common signs of gigantism include:- large hands and feet, Thick toes and fingers, A bulging jaw and forehead. Improper facial features. Children suffering from gigantism may also suffer from large heads, lips, or tongues. The symptoms of gigantism depend on the size of the pituitary gland tumour. Some children may experience vision problems, headaches and nausea from tumour.

14. Other symptoms include: Large scale sweating Weakness Onset of puberty in boys and girls may be delayed Irregularity in menstrual cycle Deafness.

15. DIAGNOSTIC EVALUATION History collection. CT scan , MRI scan- to rule out pituitary tumour. Oral Glucose Tolerance Test- to rule out hyperglycaemia. Blood test- to rule out growth hormone level, high prolactin level, increase insulin level, growth factor -1 levels.

16. MANAGEMENT :- Gigantism requires early detection and strong treatment to prevent excess production of growth hormone and to improve life expectancy. Surgery include- TRANSFENOIDAL ADENECTOMY, HYPOPHYSECTOMY Surgery is the first line of treatment with the objective of removing the tumour to minimize growth hormone levels and reduce the pressure on the nerves. Radiation therapy is another option if surgery has not provided a complete cure. It can take several years for radiation therapy to be effective. Half of the patients achieve controlled growth hormone in 5-10 years. Though radiation therapy is successful in controlling tumour growth, it may not reduce growth hormone and insulin-like growth factor 1 levels.

17. Drug therapy may also be used in certain circumstances. Drug therapy include:- Somatostatin analogs - reduces growth hormone release. Pegvisomant -blocks the effects of growth hormone. Drug therapy is used - Prior to surgery in order to control symptoms and cause the tumour to shrink. Post surgery when growth hormone levels are not managed. While radiation therapy is going on. Individuals not qualified for surgical process. Regular medical follow-up is required to scrutinize growth hormone and insulin-like growth factor 1 to notice any growth of the tumour.

18. ACROMEGALY Acromegaly is a chronic metabolic disorder in which there is a secretion of too much growth hormone and the body tissues gradually enlarge. INCIDENCE:- It occurs in about 6 of every 100,000 adults. Occurs in adulthood, usually during middle age.

19. ETIOLOGY :- Pituitary tumour. Benign tumour. Adenoma of the pituitary gland. Non pituitary tumour. Benign or cancerous tumour of the other part of the body such as lungs, pancreas, adrenal glands. Excess growth hormone and growth hormone releasing factor in the blood leads to changes in the physical appearance and functions of body

20. SYMPTOMS:- Hand swelling , ‘ sausage like’ fingers. Increase in shoe size. Diaphoresis . Thickening of the facial features especial nose. Increase prominence in the jaw and forehead. Thickened skin. Swelling of tongue. Thickening or swelling of the neck. Arthritis . Sleep apnoea. Headache. Partial loss of vision. May occur one eye or both the eyes.

21. Pain , numbness, tingling or weakness in hands and wrists. Increased thirst and urination. Hyperglycemia . Chest pain. Shortness of breath. Palpitation. Heart failure. DIAGNOSTIC EVALUATION :- History collection. Physical examination. CT scan , MRI scan of head, chest, abdomen, pelvis, adrenal glands, ovaries,

22. MANAGEMENT:- Goal of the treatment is to relieve and reverse the symptoms of acromegaly . Surgical treatment is the first line treatment. Surgery brings remission and in some people, but not all. TRANSSPHENOIDAL HYPOPHYSECTOMY

23. Radiation therapy- is an option to reduce the size of the tumour and hence reduce the production of growth hormone. Radiation therapy focuses on high intensity radiation at pituitary tumour to destroy the abnormal cells. Given in 2 forms. External beam and stereotactic. Drug therapy – Somatostatin analogs - reduces growth hormone release. Dopamine agonists- prevents the release of growth hormone. Growth hormone receptor agonist eg Pegvisomant -blocks the effects of growth hormone.

24. DIABETES INSIPIDUS

25. DEFINITION :- Diabetes insipidus is a disorder of the posterior lobe of the pituitary gland characterized by a deficiency of antidiuretic hormone (ADH), or vasopressin. Great thirst ( polydipsia ) and large volumes of dilute urine characterize the disorder.

26. TYPES OF DI A) Central diabetes insipidus B) Nephrogenic diabetes insipidus C) Psychogenic diabetes insipidus D) Gestational diabetes insipidus .

27. CAUSES:- A) Central diabetes insipidus :- Head trauma or surgery Pituitary or hypothalamic tumour Intracerebral occlusion or infection B) Nephrogenic diabetes insipidus Systemic diseases involving the kidney Multiple myeloma sickle cell anemia Polycystic kidney disease Pyelonephritis Medications such as lithium

28. PATHOPHYSIOLOGY :- A) Central diabetes insipidus :- Loss of vasopressin-producing cells, Causing deficiency in antidiuretic hormone (ADH) synthesis or release; Deficiency in ADH, resulting in an inability to conserve water, leading to extreme polyuria and polydipsia B) Nephrogenic diabetes insipidus Depression of aldosterone release or inability of the nephrons to respond to ADH, causing extreme polyuria and polydipsia

29. SIGNS AND SYMPTOMS Polyuria with urine output of 5 to 15 L daily Polydipsia , especially a desire for cold fluids Marked dehydration, as evidenced by dry mucous membranes, dry skin, and weight loss Anorexia and epigastric fullness Nocturia and related fatigue from interrupted sleep

30. DIAGNOSTIC TEST RESULTS High serum osmolality , usually above 300 mOsm /kg of water Low urine osmolarity , usually 50 to 200 mOsm /kg of water; low urine- specifi c gravity of less than 1.005 Increased creatinine and blood urea nitrogen (BUN) levels resulting from dehydration Positive response to water deprivation test: Urine output decreases and specific gravity increases

31. MANAGEMENT :- The objectives of therapy are (1) to replace ADH (which is usually a long-term therapeutic program), (2) to ensure adequate fluid replacement, and (3) to identify and correct the underlying cause. Treatments Replacement of vasopressin therapy with intranasal or I.V. DDAVP ( desmopressin acetate). Correction of dehydration and electrolyte imbalances. A thiazide diuretic to deplete sodium and increase renal water reabsorption . Restriction of salt and protein intake.

32. SIADH- SYNDROME OF INAPPROPRIATE ANTI DIURETIC HORMONE . SIADH is a disorder of impaired water excretion caused by the inability to suppress secretion or due to excessive secretion and action of Antidiuretic hormone . If water intake exceeds the reduced urine output (concentrated Urine), the ensuing water retention leads to the development of hyponatremia . Most common cause of HYPOOSMOLAR EUVOLEMIC Hyponatremia .

33. ETIOLOGY: Neoplasms Carcinomas of Lung Duodenum Pancreas Ovary Bladder, ureter Other neoplasms Thymoma Mesothelioma Bronchial adenoma Carcinoid Gangliocytoma Ewing's sarcoma Head trauma (closed and penetrating) Infections Pneumonia, bacterial or viral Abscess, lung or brain Cavitation ( aspergillosis ) Tuberculosis, lung or brain Meningitis, bacterial or viral Encephalitis AIDS Vascular Cerebrovascular occlusions, hemorrhage Cavernous sinus thrombosis Genetic X-linked recessive (V2 receptor gene)

34. Drugs Vasopressin or desmopressin Chlorpropamide Oxytocin , high dose Vincristine Carbamazepine Nicotine Phenothiazines Cyclophosphamide Tricyclic antidepressants Monoamine oxidase inhibitors Serotonin reuptake inhibitors Congenital malformations Agenesis corpus callosum Cleft lip/palate Other midline defects Neurologic Guillain-Barré syndrome Multiple sclerosis Delirium tremens Amyotrophic lateral sclerosis Hydrocephalus Psychosis Peripheral neuropathy

35. INVESTIGATIONS SENT FOR DIAGNOSIS OF SIADH: Serum Na+, potassium, chloride, and bicarbonate Plasma osmolality Urine Sodium Urine osmolality Serum creatinine Blood urea nitrogen Blood glucose Serum uric acid Serum cortisol Thyroid-stimulating hormone

36. MANAGEMENT :- Fluid restriction — is a mainstay of therapy in most patients with SIADH, with a suggested goal intake of lessthan 800 mL /day . The associated negative water balance initially raises the Serum sodium concentration toward normal and, with maintenance therapy in chronic SIADH, prevents a further reduction in serum sodium. Intravenous saline — Severe, symptomatic, or resistant hyponatremia in patients with SIADH often requires the administration of sodium chloride. If the serum sodium concentration is to be elevated, the electrolyte concentration of the fluid given must exceed the electrolyte concentration of the urine, not simply that of the plasma