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Barksterar Cortex Medulla Pregn Kynsterar Adrenaline (85%)
90% 10% Saltsterar Sykursterar Pregn Kynsterar Adrenaline (85%) Noradrenaline 1
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Tvö aðskilin stjórnkerfi
SYKUR SALT 2
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Heiladinguls-nýrnahettuöxullinn
ACTH AVP CRF AVP SON PVN Amygdala BST Hippocampus - + ? +? CBG Type 1 Type 2 11b-HSD 3
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Renín - Aldó - SV öxull ACE A-I Angíótensínógen + A-II + Renín +
Aldósterón + Renín òBÞ + (ACTH) ò s-K+ + - ò þvag-Na+ ñ þvag-K+ ñ þvag-H+ + SV 4
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Orsakir nýrnahettubilunar
Prímer (Addison´s Disease) Autoimmune Uniglandular Polyglandular Sýkingar / sepsis Meinvörp Adrenomyeloneuropathy Sekúnder Sterameðferð Æxli í heiladingli eða hypothalamus Sheehan syndrome Granuloma / hypophysitis Skurðaðgerð / geislun 5
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Einkenni við nýrnahettubilun
Heiladingulsæxli Staðbundin bitemporal hemianopia höfuðverkur brottfallseinkenni Almenn Ofgnótt hormóns Vöntun hormóns Addison Þreyta, slen og slappleiki Þyngdartap Réttstöðusvimi Lystarleysi - ógleði Kviðverkir - uppköst Saltfíkn Niðurgangur 6
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Teikn sjúkdóms Addisons
Skoðun Teikn prímer sjúkdóms Horaður og slappur einstaklingur Hyperpigmentation Axilla og pubis hártap Orthostatismi hypotensio CV-collapse Rannsóknir Hyponatraemia Hyperkalaemia Acidosis Hypoglycaemia Hypercalcaemia Hækkað urea Normochrome/cytic anaemia, lymphocytosis, eosinophilia Adrenal mótefni jákvæð 7
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Hyperpigmentation af völdum ACTH
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What diagnostic test should be performed ?
A 55y old housewife is treated for hypothyroidism with thyroxine 150mg/d but remains fatigued 3/12 later Hb 112 g/l MCV 103 fl FT4 18 pmol/l TSH 2.2 mU/l Na mmol/l K mmol/l Cl- 97 mmol/l U 8.9 mmol/l BG 3.1 mmol/l HCO mmol/l Why is she fatigued ? What diagnostic test should be performed ? Give two likely causes of the raised MCV 9
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Greining Grundvallarreglur efnaskiptalækninga Synacthen próf s-ACTH
Grunur um skort è örvunarpróf Grunur um ofgnótt è bælipróf Synacthen próf Samtengt ACTH (Tetracosactrin b1-24) 250mg iv/im è 30 mín (ok > 550nM) s-ACTH Aldosterone og renín 10
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Bráðameðferð Meta vökvaástand. Inj. Hydrocortisone (kortisól) Dagur 2
Inf NaCl 0.9% hratt ± iv glúkósa 5-10%. Inj. Hydrocortisone (kortisól) 100mg iv stat svo 100mg x 3 iv (im). (Dexamethasone 4mg iv ef gert Synacthen próf) Dagur 2 Inj. Hydrocortisone 50mg x 3 iv (im). Þegar borðar Tabl. Hydrocortisone 20mg kl. 08 og 10mg kl.17. Meta þörf á fludrocortisone. Meðhöndla meðvirkandi/framkallandi sjúkdómsástand Ath að gefa kortisól á undan T4 ef einnig hypothyroidism 11
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Viðhaldsmeðferð Langtíma uppbót per os Uppbót við álag
Hydrocortisone mg mane og mg kl 17. Fludrocortisone 0,25 - 0,1 mg / d (einungis í 1°) Uppbót við álag Lítið è engin breyting Meðal è tvöfaldur po skammtur þar til betri Svæsið è iv eins og bráðameðferð Periop kir minor è HCS 100 mg x 1 iv með pre-med kir major è HCS 100 mg x 3 iv aðgerðardag svo 50 mg x 3 næsta dag, síðan viðhald 12
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Vandamál við uppbót kortisóls
Erfitt að bæla ACTH Erfitt að líkja eftir eðlilegri kortisól dægursveiflu Hefðbundnir HCS skammtar (30 mg / d) bæla osteocalcin 13
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Er þörf á androgen uppbót ?
Dehydroepiandrosterone Replacement in Women with Adrenal Insufficiency. Arlt W et al. N Engl J Med 1999;341: Dehydroepiandrosterone (D) significantly improved overall well- being as well as scores for depression and anxiety. For the global severity index, the mean (± sSD) change from base line was ± 0.29 after four months of D therapy, as compared with 0.03 ± 0.29 after four months of placebo (P = 0.02). D increased the frequency of sexual thoughts (P = 0.006), sexual interest (P = 0.002), and satisfaction with both mental and physical aspects of sexuality (P = and P = 0.02). 14
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Ofgnótt Kortisóls Cushing´s Syndrome Cushing´s Disease
Ofgnótt kortisóls án tillits til orsakar - oftast afleiðing lyfjameðferðar Cushing´s Disease Ofgnótt kortisóls vegna ACTH framleiðandi æxlis í heiladingli 15
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Hvaða teikn skipta máli
Discriminant Index Bruising 10,5 Myopathy 7,1 Hypertension 5,1 Plethora 3,6 Oedema 3,3 Red Striae 3, Lancet 1982;2: 16
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Cushing’s syndrome: Establish the diagnosis
Suspected Cushing’s Syndrome 1mg overnight dexamethasone test or 24h U free cortisol + 48h low dose dexamethasone suppression + circadian plasma cortisol Confirmed Cushing’s Syndrome ? depression ITT ? alcohol abstinence 1mg dexamethasone at 11pm and blood at 9am. s-cortisol < 60 nmol/l excludes Cushing’s (sensitivity 98% and specificity 99%) 24h U free cortisol < 150 nmol/24h excludes Cushing’s (sens 94% & spec 97%) Low dose dexamethasone test is 0.5mg dex q6h for 48h which should suppress cortisol to undetectable levels (sens 94% & spec 100%) Alcoholism and depression can give false positive on all of the above and therefore those need to be excluded specifically. On ITT, depression gives a normal cortisol response whereas a lack of response is characteristic for Cushing’s 17
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Cushing’s syndrome: Differential diagnosis
Confirmed Cushing’s Syndrome ACTH dependent ACTH DHEAS DA4 U/E’s BG Adrenal cause CT adrenal + adrenal vein sampling radio-cholesterol scanning angiography CRH test & 48h high dose dexamethasone suppression + inferior petrosal sinus sampling Ectopic source Plasma ACTH (x3) will define if the cause is adrenal or extra-adrenal. Pituitary dependent Cushing’s (Cushing’s Disease) does suppress on high dose dex (2mg q6h for 48h) but ectopic ACTH not. Pituitary Cushing’s shows a brisk response to CRH with peak cortisol >120% of baseline or peak ACTH >150% of baseline. Pituitary source CXR, CT chest & abdomen tumour markers + multiple venous sampling for ACTH MRI Pituitary 18
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Meðferð við Cushing´s Disease
Transsphenoidal skurðaðgerð Erfið tæknilega Geislameðferð 1/5 læknast á árum 2/5 þurfa adrenalectomy Adrenalectomy 25% Nelson´s Syndrome Lyfjameðferð Metyrapone Ketoconazole Aminoglutethimide op-DDD 19
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