panhypopituitarism Pattawan Wongwijitsook Maharat Nakhon Ratchasima hospital 17 Nov 2013

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1 panhypopituitarism Pattawan Wongwijitsook Maharat Nakhon Ratchasima hospital 17 Nov 2013

2 PITUITARY GLAND (HYPOPHYSIS CEREBRI) The master of endocrine glands master of endocrine glands It is a small oval structure 1 cm in diameter.

3 PITUITARY GLAND X-RAY SKULL: LATERAL VIEW SAGITTAL SECTION OF HEAD & NECK Hypophyseal fossa Pituitary gland Sphenoidal air sinus

4 A fold of dura mater (Diaphragma sellae) covers the pituitary gland & has an opening for passage of infundibulum (pituitary stalk) connecting the gland to hypothalamus.

5 BLOOD SUPPLY OF PITUITARY GLAND ARTERIES: Superior & inferior hypophyseal arteries (branches of internal carotid artery) VEINS: Hypophyseal veins drain into Cavernous Sinuses.

6

7 PITUITARY GLAND Anterior pituitary Posterior pituitary

8 ANTERIOR PITUITARY CELLS Anterior pituitary cell hormone effect somatotrophs GH -Stimulate general body growth -Regulate metabolism thyrothrophs TSH -control secretion and others activity of thyroid gland gonadotrophs FSH LH -Stimulate secretion of estrogen and progesterone - secretion of testosterone -maturation of oocyte in ovary and sperm in testes Pituitary society

9 Anterior pituitary cells hormone effect Lactothrophs prolactin -Initiated milk production in mammary gland corticothrophs Posterior pituitary (storage from hypothalamus) ACTH MSH Oxytocin ADH -Stimulate adrenal cortex secrete glucocorticoid -Affect skin pigmentation -Contract uterine (delivery) -milk inject (breast feeding) -control body water balance Pituitary society

10

11 Harrison s 18 th edition

12 hypopituitary Definition Partial or complete deficiency of one or more pituitary hormone. It may arises as a congenital defect during the development of the pituitary gland or as a result of acquired disease of the pituitary gland. The parasellar structures, or the hypothalamus. Williams Textbook of endocrinology 10 edition

13 Epidermiology Prevalence 45 cases per 100,000 Incidence of about 4 cases per 100,000 per year in the normal population. Pituitary adenomas or incidentalomas found 27% of postmortem studies 10% of MRI studies Williams Textbook of endocrinology 10 edition

14 Epidermiology No specific gender, ethnic, geographic or age group Associated 1.8 fold higher mortality compared with an age- and sex- match population. Cardiovascular and cerebrovascular death rate are higher Williams Textbook of endocrinology 10 edition, pituitary society

15 hypopituitary The clinical presentation of anterior pituitary Acquired pituitary insufficiency - degree of hormone deficiency - the number of hormone impaired -the rapidity of onset Williams Textbook of endocrinology 10 edition

16 hypopituitary The clinical presentation of anterior pituitary Congenital pituitary insufficiency -the eariler the age of onset -the greater thr severity of thyroid, gonadal, adrenal, growth, or water disturbance

17 hypopituitary The resilience of the individual pituitary cell lineages in the presence of each causes also differs. Prolactin deficiency is rare except for complete pituitary destruction or genetic syndrome Williams Textbook of endocrinology 10 edition

18 hypopituitary The lactotrophs cell is often hyperfunctional as a result of decrased tonic inhibitory signals. GH was diminished function prior than others trophic hormones. The corticotrophs and thyrotrophs cell are usaully the last to lost function. Williams Textbook of endocrinology 10 edition

19 Hypopituitary causes

20

21

22

23 Symptom of hypopituitarism

24 Symptom of hypopituitarism

25 Gonadotropin Deficiency Women Oligomenorrhea or amenorrhea Loss of libido Vaginal dryness or dyspareunia Loss of secondary sex characteristics (estrogen deficiency) Men Loss of libido Erectile dysfunction Infertility Loss of secondary sex characteristics (testosterone deficiency) Atrophy of the testes Gynecomastia (testosterone deficiency)

26 ACTH Deficiency Results in hypocortisolism Malaise Anorexia Weight-loss Gastrointestinal disturbances Hyponatremia Pale complexion Unable to tan or maintain a tan No features of mineralocorticoid deficiency Aldosterone secretion unaffected

27 TSH Deficiency Hypothyroidism Atrophic thyroid gland

28 Prolactin Deficiency Inability to lactate postpartum Often 1 st manifestation of Sheehan syndrome

29 Growth Hormone Deficiency Adults Often asymptomatic May complain of Fatigue Degrees exercise tolerance Abdominal obesity Loss of muscle mass Children GH Deficiency Constitutional growth delay

30 hypopituitary Two thirds of pituitary macroadenoma, craniopharyngioma, and others parasellar lesion pituitary reserve function. Intrasellar aneurysm, pituitary metastasis, parasellar meningioma, optic glioma, hypothalamic astrocytoma pituitary failure Williams Textbook of endocrinology 10 edition

31 hypopituitary Prolactin should be measure because many pt. with hypopituitarism also present with secondary hyperprolactinemia. 25% of pt. loss of pituitary function after surgery Williams Textbook of endocrinology 10 edition

32 Screening for pituitary failure Pt with hypothalamic or pituitary mass lesion. Developmental craniofacial abnormality. Inflammatory disorder Brain granulomatous disease Prior head or neck radiation Williams Textbook of endocrinology 10 edition

33 Screening for pituitary failure Prior skull base surgery Head trauma Newly discover empty sella Experienced pregnancy-associated hemorrhage or blood pressure change To prevent long term morbidity Williams Textbook of endocrinology 10 edition

34 investigaton

35 investigation

36 Harrison s 18 th edition Pituitary test ล

37 Pituitary test Harrison s 18 th edition

38 Pituitary test Harrison s 18 th edition

39 treatment Harrison s 18 th edition

40 treatment Harrison s 18 th edition

41 Acquire hypopituitary Pituitary apoplexy Cranial irridation Lymphocytic hypophysitis Empty sellar Sheehan syndrome (pituitary necrosis) ETC.

42 Harrison s 18 th edition Pituitary apoplexy Acute intrapituitary hemorrhage Occur spontaneous in preexitting adenoma, postpartum (sheehan syndrome) Associated DM, HT, sickle cell anemia, shock, warfarin use Endocrine emergency hypopituitary is very common after apoplexy

43 Harrison s 18 th edition Pituitary apoplexy pituitary apoplexy occurs within pituitary adenomas it may also occur in non-adenomatous or even the normal pituitary gland especially during pregnancy sheehan s syndrome pituitary necrosis

44 Harrison s 18 th edition Pituitary apoplexy Acute symptom : severe headache, nausea vomitting, bilateral visual change (bitemporal hemianopsia), opthalmoplegia Others symptoms : severe hypoglycemia hypotension, loss of consciousness, death Diagnosis : Pituitary CT, MRI intratumural or sellar hemorrhage, deviated pituitary stalk and compress pituitary tissue

45 Pituitary apoplexy Acute phase MRI PITUITARY

46 Harrison s 18 th edition Pituitary apoplexy Management no evidence impair consciousness or visual loss : conservative corticosteroid if adrenal insufficiency significant visual loss, severe opthalmoplegia or loss of consciousness : urgent surgical compression Visual recovery the length of time after acute event

47 Harrison s 18 th edition Cranial irridation Whole brain or head and neck therapeutic irridation Two third of pt develop hormone deficiency after a median dose 50 Gy (5000 rad) direct base skull GH is most common. Follow by gonadotropin and ACTH

48 Harrison s 18 th edition Lymphocytic hypophysitis Most common in postpartum woman. Suggests a selective autoimmune process target to specific cell types. Hyperprolactinemia with MRI :prominence pituitary mass like adenoma. Most manifestration of progressive mass effects with headache and visual disturbance. Resolve after several month of glucocorticoid treatment.

49 Harrison s 18 th edition Empty sella Develop insidiously. Pituitary mass undergo clinically silent infarct. CSF filling the dural herniation Usually normal pituitary function. Functional pituitary adenoma may arise within the rim of pituitary tissue. Not always visible in MRI

50 Sheehan syndrome Ischemic pituitary necrosis due to severe postpartum hemorrhage, Vasospasm, thrombosis and vascular compression of the hypophyseal arteries. about 3% for women above 20 years of age, almost two-thirds of whom had delivered babies at home. Indian J Endocrinol Metab September; 15(Suppl3): S203 S207.

51 Pathophysiology of sheehan s Indian J Endocrinol Metab September; 15(Suppl3): S203 S207.

52 Sheehan syndrome the average time between the previous obstetric event and diagnosis of SS was 13 years. Lactation failure is a very common clinical feature The absence of amenorrhea or the presence of postpartum lactation, however, does not rule out the diagnosis. Indian J Endocrinol Metab September; 15(Suppl3): S203 S207.

53 Sheehan syndrome The main involvement was the secretion of growth hormone (GH) and prolactin (90 100%). deficiencies in cortisol secretion, gonadotropin and thyroid stimulating hormone (TSH) ranged from 50 to 100% Treatment : hormone therapy glucocorticoid, GH etc. Indian J Endocrinol Metab September; 15(Suppl3): S203 S207.

54 Thank you

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