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PITUITARY FAILURE H Y POPITUITARIS M

PITUITARY FAILURE H Y POPITUITARIS M. DEFINITION. Hipopituitarism is a syndrome produced by complete or partial deficiency of one or more pituitary hormones

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PITUITARY FAILURE H Y POPITUITARIS M

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  1. PITUITARY FAILUREHYPOPITUITARISM

  2. DEFINITION Hipopituitarism is a syndrome produced by complete or partial deficiency of one or more pituitary hormones The disease develops and insidiously exception be those cases in which hypopituitarism develops during post partum period. The clinical pictures is correlated with etiology of the disease Hipopituitarism may be primary disease due to disfunction or /and distruction of pituitary gland and secondary, due to the absence of hypothalamic stimulation of the pituitary gland

  3. ETHIOLOGY of HYPOPITUITARISM The 9 I • Invasive – is produced by diseases, usually tumorswhich destroy the pituitary. • Large pituitary tumors destroy the pituitary directly or by compression of pituitary stalk which produces secondary hypopituitarism • Chraniophariongiomul produces compressive effects on the hypothalamus and reduces secretion of releasing hormones , it may produces injuries of pituitary stalk or directly destroys the pituitary • Other CNS tumors may indice hypopituitarism by mass effects over hypothalamic-pituitary system

  4. CHRANIOPHARINGIOMA

  5. ETIOLOGY of HYPOPITUITARISM The 9 I 2. Pituitary infarct – Sheehan syndrome ( first case was published in 1937 It results from pituitary infarct of the pituitary due to ischemia during an important post partum hemorrhage followed by collapse or due to an intravascular coagulation syndrome Pituitary is more vulnerable to ischemia during post partum period due to its increase in volume due to multiplication of prolactine-secreting cells and and sometimes its vascularity does not increase un the same measure. 32 % of women with severe post partum bleeding develpt a certain degree of post partum hypopituitarism The pituitary has enough reserves and hypopituitarism develops after 80 % of the gland is destroyed. Pituitary apoplexy develops during infarct of large tumors with immediately hypopituitarism

  6. ETIOLOGY of HYPOPITUITARISM The 9 I 2. Pituitary infarction – sindromul Sheehan ( first case published:1937 Conditions which lead to pituitary infarction • Large surgery on open hearth, with prolonged hypoxia urată • Diabetes mellitus associated microangiopathy • During and after radiotherapy for pituitary tumors • Subclinical or silent which determines reduction of tumroal secretion. (ex. reduction of GH)

  7. Infarct of the pituitary followed by fibrosis

  8. ETIOLOGY of HYPOPITUITARISM The 9 I 3. Pituitary infiltrative diseases • Sarcoidosis infiltrates the hypothalamus and produces secondary pituitary failure. Sarcoidotic granuloma may destroy the entire hypothalamic-pituitary system with increase in volume of this area and visual field problems similar to those produced by pituitary tumors • Hemochromatosis:produces hypopituitarism on gonadotropic line and gonadal failure due to gonadal infiltration • Histiocytosis Langherhans: the infiltration of multiple organs with well differentiated histiocytes may affect the pituitary. This infiltration affects mostly posterior pituitary and the hypothalamus with diabetes insipidus and secondary pituitary failure.

  9. IRM of pituitary infiltrated by histiocytosis

  10. Histicytosis granuloma of the pituitary

  11. Pituitary PET in a patient with histiocytosis and hypopituitarism

  12. ETIOLOGY of HYPOPITUITARISM The 9 I 4. Injuries – trauma Cranial trauma during fractures of the base of the skull with section of the pituitary stalk by dura mater and disruption of relatioship between hypothalamus and pituitary; In childeren with cranial trauma and closed hematoma followed by growth failure.

  13. ETIOLOGY of HYPOPITUITARISM The 9 I 5. Immunological Autoimmune hypophisitis develops during post partum period and may be diagnosed as post partum pituitary failure The disease develops slowly and may be associated with other autoimmune diseases such as adrenal failure, premature ovarian failure or autoimmune testicular failure Pituitary imaging in MRI or CT shows empty sella in post partyum pituitary failure and increased volume of the pituitary in autoimmune hypophisitis due to lymphocytic infiltration of the gland

  14. MRI– autoimmune hypophysitis

  15. MRI – autommune hypophisitis

  16. Lymphocytic infiltration on the pituitary

  17. ETIOLOGY of HYPOPITUITARISM The 9 I 6. Iatrogenic • The most frequent cause of iatrogenic pituitary failure in surgery for pituitary or hypothalamic tumors • Pituitary irradiation for CNS tumors in children or for leukemia may result in hypopituitarism and growth failure • Pituitary irradiation in adult patients with 4500-5000 cGy results in pituitary failure in 50-60 % of cases • Heavy particles irradiation of the pituitary produces hypopituitarism in 2-50 % of cases

  18. ETIOLOGY of HYPOPITUITARISM The 9 I 7. Infectious: tuberculosis , mycosis, 8. Idiopatic • Isolated of multiple pituitary hormone defficiency • Genetic: • autosomal recesiveor x- linked • As part of other genetic syndromes such as Prader Willi syndrome or septo-optic dysplasia

  19. ETIOLOGY of HYPOPITUITARISM The 9 I 9. Isolated a. Isolated GHdeficiency in children may be sporadic or familail and results in growth failure which becomes obvios after 2 years, It may olso appear in emotionally-deprived children, in those with psychological trauma, or physical abused. b. Isolated ACTHmay be produce by pituitary failure to synthesize ACTH of due to absence of hypothalamic stimulation. It results in secondary adrenal failure with weakness, hypotension and pale skin

  20. ETIOLOGY of HYPOPITUITARISM The 9 I 9. Isolated c. Isolated gonadotropin deficiency: most fewquent form is produced by isolated Gn-RH (LH-RH) which may be associated with anosmia (loss of sense of smell) (Kallman’ syndrome) usually in Xp22.3 mutation or isolated. It may be associated with anabillity to recognize colors or deafness. d. isolated TSH deficiency may be determined by TRH deficiency or chronic kidney failure e. Isolated prolactine deficiency occurs in autoimmune hypophystis but in most cases prolactine deficiency is a part of multiple pituitary pituitary hormone deficiency f. Mutiplepituitary hormone deficiency may occur in severe developmental problems of the pituitary due to abnormalities of genes which encodes transcription factors which regulates pituitary development such as PIT-1 and PROP-1

  21. Clinical signs and symptoms of pituitary failure Clinical signs and symptoms of pituitary hormones: GH deficiency:growth failure in children and less evident in adult patients: - Hypoglicaemia, • Decreased lean body mass in increased fat body mass with increased risk for atherosclerosis • Reduced muscle strength • Weakness • Decreased quality of life

  22. Clinical signs and symptoms of pituitary failure TSH deficiency • Cold intolerance • Slow thinking and memory troubles • Cold, dry skin • Discreteskin infiltration due to mucopolysacharides accumulation • Bradicardia • Slow muscle reactions

  23. Isolated TSH deficiency

  24. Clinical signs and symptoms of pituitary failure ACTH deficiency: • Produces adrenal failure with: • Physical, psychological and sexual weakness • Arterial hypotension to shock and colaps • Inability to mobilize cortisole reserves during stress • Pale skin • Loss of sexual hair in axilarry and pubic areas especially in women whom sexual hair is dependent of adrenal androgens • Hypoglicaemiaaggravated by GH deficiency • In ACTH deficiency there are no signs and symptoms of aldosteone deficiency and its metabolic consequnces

  25. Clinical signs and symptoms of pituitary failure Gonadotropin deficiency ( primary or secondary to Gn-RH deficiency In men: • Decreased sexual desire and sexual function • Testicular atrophy and infertility with azoospermia • Sexual hair loss (beard pubic and axilary hair) In women: • Secondary (or) primary amenorrhea • Genital atrophy and loss of sexual desire • Sexual hair loss

  26. Severe sexual hair loss and pale skin in a patient with pituitary failure

  27. Panhypopituitarism due to chraniofaringioma

  28. Clinical signs and symptoms of pituitary failure To the aforementioned signs and symptoms may appear signes depending of the disease which produces the pituitary failure: • Headache • Narrowing of the visual field in pituitary tumors

  29. Clinical signs and symptoms of pituitary failure Sheehan’ syndrome: It is a pure form of pituitary failure that occurs in postpartum period and is recognized in most cases after years of evolution: • Loss of lactation in post partum period • Secondary amenorrhea • Loss of sexual hair in tge following 6 month after pituitary necrosis • Weakness which is physical, psychological and sexual (loss of sexual desire) • Loss of interest in family and professional life • Anemia • Slow speech, ideas and reactions • Premature ageing

  30. Premature ageing due to Sheehan’s syndrome

  31. MRA in Sheehan’s syndrome

  32. Pituitary coma: It occurs in the following conditions: • Stress exposure • Sedatives • Cold exposure • The occurrence of a disease which needs defending machanisms dependent of the adrenal function • Forms: • Hypoglicemic • Hypertermic

  33. Assessment of hypopituitarism Usual assessment: • Determination of hormones produced by glands normally stimulated by pituitary: fT4, cortisole, testosterone, estradiol. • Pituitary glands are stimulated with specific tropic hormon and respond confirming that their hypofunction is due to loss of pituitary stimulation : • ACTH test – if cortisole incrases: pituitary failure • TSH test – if fT4 increases: pituitary failure • LH test: if estradiol or testosteroneincrease : pituitary failure

  34. Assessment of hypopituitarism A Ft4 cortisole testosterone estradiol TSH ACTH FSH şi LH Administration in the same injection TRH, CRH şi Gn-RH TSH, ACTH, FSh şi LH do not increase TSH, ACTH, FSH şi LH increase Pituitary failure Hypothalamic failure or interruption of connections betweenhypothalamus and pituitary

  35. Treatment of hypopituitarism PRINCIPLES • The usual treatment uses hormones of peripheral glands which are not stimulated by the pituitary • At the beginning of the treatment the hormones are introduced in the following order: • Cortisole • Thyroxine • Gonadal hormones Pituitary hormones are used in the following situations: - Induction of fertility in both sexes • Recently GH treatment was introduced in the adult patients, to improve quality of life, but this is not absolutely needed.

  36. Treatment of hypopituitarism Dosage • Cortisole: 25 mg/day in 3 devided doses. The morning dose dose must be the higher. In case of stressful situations cortisole dosage must be increased 4 fold until the stressis terminated. • Thyroxine – 100 μg/dayin one morning dose • Gonadal hormones: • Men: Testosterone enanthate: 250 mg. monthly or 1000 mg testosterone undecanoateevery 3 month, or 25 mg testosterone gel/day (skin application) • Women: estro-progestitivesor Estradiol 1mg /day for 21 days in a monthwith synthetic progestativein the last 10 days of 21 days of each cycle

  37. Gh DEFICIENCY IN CHILDREN IT RESULTS IN GROWTH DEFICIENCY. IN CONGENITAL FORMS GROWTH DEFICIENCY APPEARS AS OBVIOUS AFTER THE SECOND YEAR OF LIFE AND IN ACQUIRED FORMS, FROM THE MOMENT IN WHICH GH DEFICIENCY APPEARS. CAUSES OF GH DEFICIENCY: ENDOCRINE CAUSES - GH deficiency of different causes - Abnormalities of IGF1 generation in the liver (Laron dwarfs) • Primary IGF1deficiency, pigmeys, IGF1receptor abnormalities • Psychosocial dwarfism • Hypothyroidism • Glucocorticoid excess endogenous or exogenous (iatrogenic) • Type 1 pseudohypoparathyroidim (Gs alpha protein inactivating mutation • Diabetes mellitus in children • Adrenal insufficiency

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