POTENTIALLY FATAL ENDOCRINE CONDITIONS DR F KAPLAN February

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POTENTIALLY FATAL ENDOCRINE CONDITIONS DR F KAPLAN February 2014

POTENTIALLY FATAL ENDOCRINE CONDITIONS DR F KAPLAN February 2014

Patient one l l l 36, father of 4 3/04 Sweating, palps, BP 130/80

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

PHAEOCHROMOCYTOMA

PHAEOCHROMOCYTOMA

l Wide range presentatiins l Age 5 days to 92 yrs l Dangerous but

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

Clinical Sx l Headache 71% l Palps 65% l Sweating 65% l Tremor, anxiety,

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

Signs l HPT>90%, sustained 50%, parox 50% l Orthostatic hypotension up to 75% l

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

Ix Biochemistry l Urine METS l Plasma METS

Ix Biochemistry l Urine METS l Plasma METS

Ix: localization l CT/MRI (only 70% specific) l m. IBG (95% specific) l Octreotide

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

Treatment l Surgical l Prep: phenoxybenzamine 10 -20 mg qds l After 48 hr,

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

Drugs to avoid l Maxolon l TCA/phenothiazines l Cytotoxics l Histamine l Glucagon l

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

Post-op l Volume replacement l Normalised METS may take days

Post-op l Volume replacement l Normalised METS may take days

Familial disorders (23%) l MEN IIa – (med Ca thyroid, hyper. PTH, phaeo) l

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

Patient two l 35 yr father l AF Dx aged 33, on warfarin l

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

ADDISON’S DISEASE

ADDISON’S DISEASE

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

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

Causes l AI 70 -90% l Infections l Haemorrhage l Neoplasia l AIDS l

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

AI Addisons l 40% have >=1 associated – Thyroid – Type 1 DM –

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

Symptoms l LOA l N/V l LOW l Pigmentation l Weakness, tiredness l Abd

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

Ix/Rx l Basal cortisol, ACTH l Short Synacthen 250 mcg ACTH l. Hydrocortisone 10/5/5

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

Acute adrenal insufficiency l Cause: infection/trauma etc l Shock/low BP l Fever l Abd

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

Rx l Volume repletion l Electrolyte balance l HC IV 100 mg 6 hrly

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

Mx of stress l Fever: double dose l Vomiting once: 20 mg po HC

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

Surgery with Addison’s l Small op eg. Hernia – 100 mg 6 hrly 24

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

Precautions l Steroid card l Medic alert l HC injection and syringe

Precautions l Steroid card l Medic alert l HC injection and syringe

Patient three l l l 32 yr mother Wt gain, plethora Ix for Cushings

Patient three l l l 32 yr mother Wt gain, plethora Ix for Cushings – 2/04 du. Cort 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

CUSHING’S

CUSHING’S

Diagnosis l Low dose dex – (overnight, and low dose 48 hr) l du.

Diagnosis l Low dose dex – (overnight, and low dose 48 hr) l du. Cortisol l Midnight cortisol

Differential l ACTH l High dose Dex l CRH test – (ACTH up in

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

Cushing’s syndrome l ACTH dependent 79% – Cushings disease 80% – Ectopic ACTH (NB

Cushing’s syndrome l ACTH dependent 79% – Cushings disease 80% – Ectopic ACTH (NB oat cell Ca lung) l ACTH independent 21% – Adrenal adenoma 80% – Adrenal Ca – Adrenal hyperplasia