Adrenal hypertension BSMS 2022(1).ppt

This presentation is for private study only. It should not be given as a public presentation or otherwise disseminated. To do so may place the presenter in breech of copyright Adrenal causes of Hypertension Brighton & Sussex Medical Students 2023 Dr Trevor Wheatley Consultant Endocrinologist & Hon...

This presentation is for private study only. It should not be given as a public presentation or otherwise disseminated. To do so may place the presenter in breech of copyright Adrenal causes of Hypertension Brighton & Sussex Medical Students 2023 Dr Trevor Wheatley Consultant Endocrinologist & Honorary Clinical Senior Lecturer Princess Royal Hospital University Hospitals Sussex Sussex, UK = Section through Adrenal galand Note the Shape Note Shape Layers of Adrenal Gland (GFR! ) F = Fasciculata R = Reticularis G = Glomerulosa Medulla Cause of Hypertension? • Essential • Renal – Primary renal Disease – Renal Artery Stenosis • Endocrine Adrenal causes of Hypertension • Primary Hyperaldosteronism – Zona Glomerulosa • Adenoma • Hyperplasia • Rare genetic causes • Phaeochromocytoma ( Phaeo ) – Tumour of the adrenal medulla • Some forms of Congenital Adrenal Hyperplasia – Enzyme defect - Uncommon Liver Kidney ADRENAL CORTEX Aldosterone Secretion Primary Hyperaldosteronism (PA) Why screen? - Learning Point • Individuals with PA have more vascular and renal pathology than people with Essential Hypertension & similar Blood pressure Hypertension Is it Primary Hyperaldosteronism? • Initial Screening Tests – Supressed Renin – Normal / High Aldosterone • Confirmatory Tests – Oral or IV Na+ supression test Hypertension Is it Primary Hyperaldosteronism? (Conn’s syndrome) • Whom to screen? – Hypokalaemia – Resistant hypertension • Inadequate control on 3 (2) drugs • Control on 4 (3) drugs or more – Younger people Primary Hyperaldosteronism Tests for specific Aetiology i.e secreting Adenoma or bilateral Hyperplasia • Adrenal CT scan • Adrenal venous sampling – Is the Aldo secretion unilateral? • Metomidate PET CT Adrenal venous sampling L. Aldosterone secreting adenoma 11C Metomidate PET scan Metomidate PET CT Primary Hyperaldosteronism Treatment • Unilateral Adenoma – Laparoscopic Adrenalectomy – Medical Treatment ( sometimes ) • Bilateral Hyperplasia – Medical Treatment ( Aldosterone Antagonists) • Spironolactone • Eplerinone Phaeochromocytoma Tumour of the Adrenal Medulla The Adrenal Medulla Modified Post-Ganglionic Nerve cells inervated by preganglionic nerves Sympathetic Neurones In spinal cord Ach Adrenal Medulla Tyrosine L-DOPA Adrenalin Dopamine Noradrenalin Products of The Adrenal Medulla • Catecholamines – Dopamine – Norepinephrine ( Noradrenalin ) Cortisol – Epinephrine ( Adrenalin ) The Adrenal Medulla Neuroendocrine Tissue Not essential for life “Stress / Fight / Flight!” Catecholamines Biological effects • Noradrenalin (Alpha 1 & 2 ) – Vasoconstriction • Increased BP • Pallor – Glycogenolysis • Adrenalin ( Alpha 1, Beta 1 & 2 ) – Vasoconstriction – Vasodilatation in Muscle – Increased heart rate – Sweating Adrenal Medulla - Pathology Phaeochromocytoma 20% of Phaeos are diagnosed in the Mortuary!! e.g. Stroke , Severe cardiac failure Phaeochromocytoma Presentation • “Spells” – Headache, Sweating – Pallor, Palpitation – Anxiety • Hypertension – Permanent – Intermittent • Family history • Incidental Finding ( Incidentaloma ) Several A-D Genetic conditions are associated with Phaeochromocytoma • Neurofibromatosis Type 1 ( NF1 ) • Multiple Endocrine Neoplasia type 2 ( MEN 2 ) • Von Hippel – Lindau Syndrome Axillary Freckling in NF1 Medullary Carcinoma of the thyroid in a MEN2a family member Retinal Hemangioglioblastoma in Von Hippel - Lindau Cerebellar Haemangioglioblastoma In Von Hippel - Lindau Phaeochromocytoma Biochemical Diagnosis • 24 hour urine – Normetanephrines & Metanephrines – 3 Methoxytyromine • Plasma – Noradrenalin & Adrenalin – Metanephrines Other things elevate the measured Catecholamines • • • • • Obstructive Sleep Apnoea Amphetamine like drugs Antidepressants Labetalol L-DOPA etc. Note Urine Dopamine comes from Kidney & Nervous system NOT the Adrenal Medulla So measure Urine Methoxytyramine Adrenal Medulla - Pathology Phaeochromocytoma Meta-iodobenzylguanidine Scintiscan MIBG Scan CT Scan - Retroperitoneal Para-aortic Paraganglioma Gallium 68 Dotatate PET CT showing Paraganglioma 18 F-FDG PET CT to show Extra-adrenal “Phaeos” (i.e. Paragangliomas ) or Metastases Management • Alpha Blockers – Phenoxybenzamine – Doxazocin • Beta Blockers ( if needed ) – e.g. Propranolol • Laparoscopic adrenalectomy Post Adrenalectomy care • Consider Genetic testing – 30% are genetic ( 13 mutations so far) • Annual Metanephrines – 24 hour urine – Plasma • Additional treatment if Malignant – 10% The End