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    SEMOGA BERMANFAAT

    Copyright:

    © All Rights Reserved
    Download as PDF or read online from Scribd
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    8 views8 pages

    Adrenal Cortex Disease & Pheochromocytoma

    Uploaded by

    Puji Astuti
    SEMOGA BERMANFAAT

    Copyright:

    © All Rights Reserved
    Download as PDF or read online from Scribd
    Flag for inappropriate content
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    Competencies © Ackenol Corlexfaiure ga CORTEX DISEASE AND PHAEOCHROMOCYTOMAS 8 | Phaeochtomocytomas 1 3 Addison's disease 1 Lothfan Bedi Purnono Sub Division of Endocrinology Internal Medicine Department Discussion ‘Adtenol hormones: glucocotticald, minert ; ‘nd odrenal ondrogen o Mneroeoricea ‘Ackenot cortex diseases: Cushing's synerome ‘and Addison's disease, mineralocoricod excess Phaeochromocytoma Adrenal Gland: Anatomy Hormones of Adrenal Cortex © Minerolocorticcic: (aldosterone 100-150ug/d}, 2900 ‘Glomeruioso, predominantly unde the contro o he renie- ‘angiotensin systern, only 5-10% uncer ACTH requicticn. imaintenances normal Na* and k° concentrations anc extracel tuid volume: 8 Glucocorticoid: (corso! 10-20 mg/d). zone fosccuiaicane reliculars, under ACTH contrat 1& Adrenal androgen: 2one fasciculata and reticulors. unter ACTH conttot 12 The mojortyof cases are due to aldosterone excess production (primary oF secondary], and are tipically ‘ossociated with hypertension and hypokalaemia | Primary hyperakiosteronism is a disorder of ‘outonomous hypersecretion with suppressed renin level © Secondary hyperaidosteronism when hypersecretion occurs secondary to elevated circulating renin tevel Couses of mineraloorticoid excess Causes of primary hyperaidosteronisn Faery Wipers = Cone aK (ISA ABI ALOIS Coens | eae | ee = | Pamoooy When ocr YAEL. ACO saree aia a = hrypwecscenbet 20 (GN). BAC ROBE CEA precrgreac eia ae ‘Sena tenon cane © Seconctany hypmcksonencrien = a ered criny sentra, red ycrrpethion, chen, congpetve sean, comes” | teonee | mont - = = eal Farce |Fere> 4 amin dt ea bets acs cocoa | Rae * ton oe 2 Othe syrccrree, forge elernn peewee ree tCcNC56 Baht, HAC erate. eomctesnetChin 0 Chernin, eta KTM (Gcercad [ave | Canon |Nao —| tana pas RIOBOR. CRABS SLEOE TY PTAIA, HAAICD commie ‘stormen | tama amends ince hott emcees bo ead a Pemstoricentenorian cae to ceca tench bios Wao Steen opcSioenie, Rasen 8 CEA ABS HATO Adrenal Cushing's syndrome frevalenee of Symploms ar ins of Cushing's Syeome Okra indox Compared with Nevalonce aes ont BP liars bev cx cen dca eae neg ond AC capendnt mete doves (Cnn once cr ‘ectopic secretion Srcreye ae Co ome: clcoholim (<1 [ccpenie @ ACTH dependent: pullory adenoma/Cushing’s disease (68%). Folopke ACTH syndrome (12%). ectopic CRH secretion (<1) « ACTHHindependen!: adrenal adenoma (10%). adrenal co (8%), ‘nodular hyperplasia (1%) Patients with Simple Obesity eae Findings (Symptoms) | 3, | O*aiinant Weight oan [ieee Monsinaimoguenty |e | 14 res | as Psychic Dytonction | 42 Headache © Musclewecknes | 9 | 80 fractures 9 Lois of calp hoi 3 prevalence of Symptoms and Signs of Cushing's Syndrome and Discriminant index Compared with Prevalence ol Features in patients wih Simple Obesity ‘Dscriminant Index Focings (Sons) % Obesly ‘a 7 ‘runcal 4“ 6 Goneratzes 55 08 Pethora ” 30 Moon face 8 Hypertension ™ 4a een #2 103 Rec purple sae 56 25 Prevalence of Symptoms and Signs of Cushing's Syndrome anc disciminan Index Compared with Prevalence of Features in Patients with Semple Obesity Discriminant Index Findings (Signs) oscle weakness Anko edema Pigmentation Hypectension geseeagsle ADRENAL INSUFFICIENCY 1 Inadequate adrenocortical function 1 Destruction of adrenal cortex (primary or Addison's disease}, or due to disordered pituitary and hypothalamic function (secondary) 10 The incidence of Addison's disease 40-60/million adults @ Secondary adrenal insulficiency is most commonly due to suppression of pitvitary-hypothalamic function by exogenous glucocorticoid administration Treatment of primary hyperaldosteronism 1p Surgery 12 Medical treatment: spironolactone {200-400 ma/d) ‘or combination with ACE! and calcium channel blockers. Side effect: gynaecomastia, impotence, menstrual iigulorities, gastrointestinal effects 1@ GSH: dexamethasone 0.5 mg on going to bed and (0:25 mg on waking, or spironolactone Glucocorticoids bars 1 In the liver: ingreased glycogen deposition: increased gluconeogenesis 5 Muscle & Fat:linhibits glucose uptake & utilization; incteased lipolysis, > FFA > increased cholesterol & triglycerides: decreased HDL-cholesterol 2 Permissive etHect of ohhes bom increase blood glucose; rotein & lipid catabolism) ACTION OF GLUCOCORTICOIDS ‘2 Endoctine system: ILH, FSH release, 1TSH release, AGH secretion 12 Gi fract: peptic uiceration | Bg otc relate tarot rcoaen | deposition, tpetpheral insulin resstance. igluconeogeness, Tiree falty acid production Overall diabetogenic effect 5 1D Adipose issue distribution: promotes visceral obesity ACTION OF GLUCOCORTICOID cont. 19 Cordiovascular/renat: salt and water retention, hypertension 1 Skin/muscle/connective tissue: protein catabolsm/ collagen breakdown, skin thinning. muscular atrophy @ Bone and calcium metabolism: ibone formation bone mass and osteoporosis, linear growth 1g Immune system: onti-inflammatory action. immuno- suppression Clinicol Features of Primary Adrenal Addison's Disease ‘Sign 2 Who rr 8 Mpotonson rma (eorex symoo 9 8 Aer calcteston tad 2 acroyte ton 2) ‘tipononema aay Siopenaomes fers} ihtpmcacema 2 some so, ( 3 fora foo Gnnctecrfont pons (6-19R) 5 fennoprata a © Described by Thomas Addison in 1855 1 The incidence in the developed world Cases per 100,000 and rreveleriee of ae Cases per 100,000 population 9 Associated with significant morbid a ‘ond mortality; can be easily treated S Essential of D/: Addison's Disease Therapy of Addison's disease General measures 18 Weakness, easy fatigabilty, anorexia, weight loss; nausea & vomiting. diarhea: abdominal pain, muscle & joint pains; amenorrhea 8 Sparse axillary hair: increased pigmentation of skin, esp. of creases, pressure area. and ripples 1@ Hypotension, small heart 1 Low sodium (Na), elevated K. Ca, and BUN: neutfopenia, mild anemia, relative lymphocytosis 1 Low plasma cortisol, elevated ACTH 2 ee | 1 Treat all infections if occurred a Increases the dose of hydrocortisone appropriately © Maximal dose of hydrocortisone for severe stress is 50 mg IV or IM every 6 hours a Advice patients to wear a medical alert bracelet or medal reading “Adrenal insufficlency—takes hydrocortisone” Therapy of Addison's disease Specific @ Hydrocortisone — 15-25 mg/d 1 Prednisone 2-3 mg/moming; 1-2 mg/early ‘evening Fludrocortisone acetate, has a potent Na- fetaining effect; 0.05-0.3ma/d 5 Dehydroepiandrosterone (DHEA) 50 mg/d for some women > improvement of the sense of well being. r ‘and sexuality 5 Adrenal Crisis & Dehydration, hypotension, or shock out of proportion to current illness severity a Nausea and vomiting with a history of weig} loss and anorexia @ Abdominal pain > so-called acute abdom 19 Unexplained hypoglycemia 1 Unexplained fever oe AGENT Crisis ~ alyponatremia, hyperkalemia, azotemia, ~ hypercalcemia, of eosinophilia Hyperpigmentation or vitiligo a Other autoimmune endocrine deficiencies, such as gonadal failure or hypothyroidism alfsuspected: give hydrocortisone 100-300 mg L.V.; refer to hospital 1 The adrenal medulla secretes epinephrine and other inbstonces ite the eran eeesation for widespread distribution and effect 1 Tt’s importance is to maintain the body's homenstasts during stress HISTORY 12 Early 19° century adrenal medulla was distinguished from adrenal cortex 12 1886 pheochromocytomas were described by Frankel 1p 1896 Manasse: chromaffin tumors 9 1901 J. Takamine: adrenalin T.8. Aldrich: epinephrine 1 1926 successful surgery of pheochromocytoma by CH, Mayo and Roux CATECHOLAMINES 13 Include: dopamine, nor-epinephrine, epinephrine 10 Epinephrine is synthesized mainly in adrenal medulla 13 Nor-epinephrine is found in adrenal medulla, centrol nervous system, peripheral sympathetic nerves 15 Dopamine: precursor of nor-epinephrine 1 Catestatin inhibits catecholamine release SYNTHESIS OF CATECHOLAMINES “Trosine nydroxvlose JYROSINE — —* _L-dinydroxyphenylalanine (t-dopa) opa decarboxylase Dopamine: -nyaronyiase Dopamine. norepinephrine Neer Epinephrine [PNMT 4-phenylethanolamine-N-methyi transferase {In adrenal mediulo: 80% epinephrine, 20% norepinephrine 2 Catecholamines are stored in granules 1p Secretion is increased by exercise, angina pectoris myocardial infarction, hemorchage. ether anesthes ‘surgery, hypoglycemia, anoxia, and asphyxia 9 When released into circulation, catecholamines are bound te albumin z sickly metabolized into inactive compo T inlets Ysetiyimandelic acid (VMA), and conjugated catecholamines i aD I alent ADRENAL MEDULLARY HYPERFUNCTION © Catecholamines can increase blood pressure by increasing cardiac output, by increasing peripheral resistance, and by increasing renin release. 18 The adrenal medulla is not known to play a significant role in essential hypertension 2d EBEOCEROM ES aaa 1 Fore tumors, 2 patients per milion people yearly of 0.1% of hypertensive population 18 Pheochromocytomas ore usually derived frorn the ‘odrenal medulla but may develop frorn Cchiomattin cells in or about sympathetic ganglia (exhoadrenal or poraganglionas) 0 >1/3 of pheochromocytomas couse death pri to diagnosis. Cause of death: myocardial infarction. cerebrovascular accident, antiyinrrios, kreversioke shock. renal failure, dissecting aortic oneurysrn © 15% of pheochromocylome are malignant PATIENTS TO BE SCREENED FOR PHEOCHROMOCYTOMA, 18 Young with hypertension 1 Hypertensive patients with: symptoms of pheochromocytoma, weight los. eines. orthostatic hypotension. unexplained shock. tomy hislory of ‘or medulla Carcinoma of thy, eurotiromatoss and other neuaculaneus synckomes, mucoral neuiomas, hyperglyceenio, carchomyopathy 8 Matted labo boos resin Shock cr severe pranier responses with: induction of ‘anesthesia, parnon, sntry, Cranes, Patation. natty. wove peocedhwes. 1 Radologic evidence of actendl moss COMMON SYMPTOMS IN PATIENTS WITH HYPERTENSION DUE TO PHEOCHROMOCYTOMA 18 Symptoms during oF following paroxyerns: headache (80%), sweating (70%), forcetut heartbeat with or without lactycordia (40%) oruiety (50% oF fear of impending death. remce (408), fatigue or exhaustion, nausea and vorniting. abdominal or chest pain, visual disturoanc 8 Symptoms between paroxysms: Increaved sweating. cold hands and leet, weight loss constipation 18 Fatal paronysms may be induced by opictes, histonine, glucagon HYPERTENSION IN PHEOCHROMOCYTOMA, 18 Hypertension (90%), paroxysms of severe hypertension (50%), orthostatic changes in blood pressure, it may drop even to hypotension levels after arising from supine position standing for 3 minutes (especially when accompanied by a se in heart rate) 8 Epinephrine secretion may cause episodic hypotension and even syncope DIAGNOSIS 1 Uinary est of metanephine or catecholamine 18 Anois ofc ful +h une somple's preferable, {where possbie] the collection shoud be made bn est no medication no recent expome 0 fadogophic contrast media, wine should be ciated 18 Normal upper fimit of cotechotomine 590-885 tro (100-150 v/24 8 ‘a Nownal upper limit of metoneptvine 7 umm 13 mgl/ah TREATMENT gs a coe sat; Blockade of alpha a monogere ne 8 rreceeer. joxypenzamine Initial dose rrogomtav roretgerersre, olde Oy eyes 10200 mma/t2h od8e4 PY Notog one PONTE, Blood preg snouts be meee ret upright blood pressures PROGNOSIS fo Nter surgery: 5-y@0r survivalrate 295% with recurence <10% ce 2 matignant pheochromocytoma: year sro!

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