Post on 26-Dec-2015
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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
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: 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
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
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
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
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’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