1. POTENTIALLY FATAL ENDOCRINE CONDITIONS DR F KAPLAN February 2014

2. Patient one 36, father of 4 3/04 Sweating, palps, BP 130/80 Ix Ireland ?details PMHx PUD Admitted 12/04 Palpitations, abnormal ECG Died 8 hrs later PM: Lt adrenal phaeo

3. PHAEOCHROMOCYTOMA

4. Wide range presentatiins Age 5 days to 92 yrs Dangerous but treatable Frequently only diagnosed at PM!

5. Clinical Sx Headache 71% Palps 65% Sweating 65% Tremor, anxiety, SOB, weakness, N/V Chest/abd pain LOW, constipation

6. Signs HPT>90%, sustained 50%, parox 50% Orthostatic hypotension up to 75% Brady/tachy Pallor/flushing Tremor Pyrexia

7. Ix Biochemistry Urine METS Plasma METS

8. Ix: localization CT/MRI (only 70% specific) mIBG (95% specific) Octreotide scanning PET scanning 10% extraadrenal/bilat/malignant

10. Treatment Surgical Prep: phenoxybenzamine 10-20mg qds After 48hr, betablocker (prop 40mg tds) IV PB 3 days prior to op

12. Drugs to avoid Maxolon TCA/phenothiazines Cytotoxics Histamine Glucagon Naloxone ACTH

13. Post-op Volume replacement Normalised METS may take days

14. Familial disorders (23%) MEN IIa – (med Ca thyroid, hyperPTH, phaeo) MEN IIb – A/A+ Marfanoid, visceral neuromas Neurocutaneous syndromes

15. Patient two 35 yr father AF Dx aged 33, on warfarin Admitted after viral illness of 3/7 BP 90/60 Died after 3 hrs PM: bilat adrenal haemorrhage.Addisons

16. ADDISON’S DISEASE

17. Addison’s 93-140/million Peak in 40s Women>>men

18. Causes AI 70-90% Infections Haemorrhage Neoplasia AIDS CAH etc

19. AI Addisons 40% have >=1 associated disease – Thyroid – Type 1 DM – Gonadal failure – Coeliac – Sjogrens – PA, vitiligo – hypoparathyroidism

20. Symptoms LOA N/V LOW Pigmentation Weakness, tiredness Abd pain, dizzy, joint pain, fever, vitiligo

21. Ix/Rx Basal cortisol, ACTH Short Synacthen 250mcg ACTH Hydrocortisone 10/5/5mg Fludrocortisone 50-200mcg/d ?DHEA 25-50mg/d

22. Acute adrenal insufficiency Cause: infection/trauma etc Shock/low BP Fever Abd pain Reduced LOC

23. Rx Volume repletion Electrolyte balance HC IV 100mg 6hrly Treat cause

24. Mx of stress Fever: double dose Vomiting once: 20mg po HC Persisting vomiting – Medical help – HC IM/IV Emotional stress: no change

25. Surgery with Addison’s Small op eg. Hernia – 100mg 6hrly 24hr Major op – A/A 72hr

26. Precautions Steroid card Medic alert HC injection and syringe

27. Patient three 32yr mother Wt gain, plethora Ix for Cushings – 2/04 duCort high – 4/04 admitted for further Ix – Drug error so admitted 5/04 6/04 metyrapone started – did not tolerate 7/04 acute abdomen, died PM: perf DU

28. CUSHING’S

29. Diagnosis Low dose dex – (overnight, and low dose 48 hr) duCortisol Midnight cortisol

30. Differential ACTH High dose Dex CRH test – (ACTH up in pituitary not ectopic)

31. Cushing’s syndrome ACTH dependent 79% – Cushings disease80% – Ectopic ACTH (NB oat cell Ca lung) ACTH independent 21% – Adrenal adenoma80% – Adrenal Ca – Adrenal hyperplasia