POTENTIALLY FATAL POTENTIALLY FATAL ENDOCRINE CONDITIONSENDOCRINE CONDITIONS

DR F KAPLAN

February 2014

Patient onePatient one

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

Ix Ireland ?details PMHx PUD Admitted 12/04

Palpitations, abnormal ECGDied 8 hrs later

PM: Lt adrenal phaeo

PHAEOCHROMOCYTOMAPHAEOCHROMOCYTOMA

Wide range presentatiinsAge 5 days to 92 yrsDangerous but treatableFrequently only diagnosed at PM!

Clinical SxClinical Sx

Headache 71%Palps 65%Sweating 65%Tremor, anxiety, SOB, weakness, N/V Chest/abd painLOW, constipation

SignsSigns

HPT>90%, sustained 50%, parox 50%Orthostatic hypotension up to 75%Brady/tachyPallor/flushingTremorPyrexia

Ix BiochemistryIx Biochemistry

Urine METSPlasma METS

Ix: localizationIx: localization

CT/MRI (only 70% specific)mIBG (95% specific)Octreotide scanningPET scanning

10% extraadrenal/bilat/malignant

TreatmentTreatment

SurgicalPrep: phenoxybenzamine 10-20mg qdsAfter 48hr, betablocker (prop 40mg tds)IV PB 3 days prior to op

Drugs to avoidDrugs to avoid

MaxolonTCA/phenothiazinesCytotoxicsHistamineGlucagonNaloxoneACTH

Post-opPost-op

Volume replacementNormalised METS may take days

Familial disorders (23%)Familial disorders (23%)

MEN IIa – (med Ca thyroid, hyperPTH, phaeo)

MEN IIb– A/A+ Marfanoid, visceral neuromas

Neurocutaneous syndromes

Patient twoPatient two

35 yr fatherAF Dx aged 33, on warfarinAdmitted after viral illness of 3/7BP 90/60Died after 3 hrsPM: bilat adrenal haemorrhage.Addisons

ADDISON’S DISEASEADDISON’S DISEASE

Addison’sAddison’s

93-140/millionPeak in 40sWomen>>men

CausesCauses

AI 70-90%InfectionsHaemorrhageNeoplasiaAIDSCAH etc

AI AddisonsAI Addisons

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

SymptomsSymptoms

LOA N/VLOW PigmentationWeakness, tirednessAbd pain, dizzy, joint pain, fever, vitiligo

Ix/RxIx/Rx

Basal cortisol, ACTHShort Synacthen 250mcg ACTH

Hydrocortisone 10/5/5mg

Fludrocortisone 50-200mcg/d

?DHEA 25-50mg/d

Acute adrenal insufficiencyAcute adrenal insufficiency

Cause: infection/trauma etcShock/low BPFeverAbd painReduced LOC

RxRx

Volume repletionElectrolyte balanceHC IV 100mg 6hrlyTreat cause

Mx of stressMx of stress

Fever: double doseVomiting once: 20mg po HCPersisting vomiting

– Medical help– HC IM/IV

Emotional stress: no change

Surgery with Addison’sSurgery with Addison’s

Small op eg. Hernia– 100mg 6hrly 24hr

Major op– A/A 72hr

PrecautionsPrecautions

Steroid cardMedic alertHC injection and syringe

Patient threePatient 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

CUSHING’SCUSHING’S

DiagnosisDiagnosisLow dose dex

– (overnight, and low dose 48 hr)

duCortisolMidnight cortisol

DifferentialDifferentialACTHHigh dose DexCRH test

– (ACTH up in pituitary not ectopic)

Cushing’s syndromeCushing’s syndrome

ACTH dependent 79%– Cushings disease 80%– Ectopic ACTH (NB oat cell Ca lung)

ACTH independent 21%– Adrenal adenoma 80%– Adrenal Ca– Adrenal hyperplasia