Checklist – Counseling “History of Acute Intermittent Porphyria”

 IntroductionPoints
Introduce and check the details1
Consent1
Presenting Compliant 
Elicit intermittent nature of abdominal pain1
Details on length of suffering and regularity of attacks1
Dermal lesions: photosensitive1
Autonomic activation/ GI: Vomiting, Hypertension, Constipation, neurological signs1
Neurological: respiratory failure, cerebellar signs (DANISH),1
Colour of urine1
Elicit relapsing course1
Family History: Autosomal dominant1
Elicits trigger: Alcohol, infections, starvation, medication, menstrual period2
Other 
Past medical history: previous admissions and what happened1
Medications1
Social history: alcohol and smoking1
Closure 
Summarise findings1
Question Investigations: urinary urobilinogen, FBC, U&E, LFTs, haematinics (increased iron), abnormal GTT, porphobilinogen deaminase in erythrocytes1
Question Management: prevent triggers and supportive1

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