Porphyry syndrome acute - Altmeyers Encyclopedia

Definition
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Molecular regulatory disorder of heme synthesis (induction of hepatic delta-aminolevulin synthetase) with excessive formation of porphyrin precursors. Clinically, acute colicky abdominal and neuropsychiatric symptoms are impressive. Emergency!

Therapy
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See Table 1.

Tables
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Therapy of acute porphyria syndrome (according to Doss and Frank)

Therapeutic measures	Procedure
Immediate measures	Discontinuation of porphyrinogenic drugs and intensive medical monitoring
	Regulatory treatment with haematin and/or other haem compounds
	Glucose and/or fructose infusions (400-500 g/day, 2 l of a 20% or 1 l of a 40% solution)
	Haem (e.g. haemarginate, 3 mg/kg bw/day as a short infusion over 15 min.) on up to 3-6 consecutive days

Symptomatic measures	Electrolyte control
	Control and force diuresis (e.g. furosemide 40-80 mg/day)

	Leading symptom	Treatment
System therapy	Abdominal pain	Acetylsalicylic acid (e.g. aspirin) 500-1000 mg/day
		Buprenorphine (e.g. Temgesic) 0.30.9 mg/day
	Vomiting	Chlorpromazine (e.g., propaphenin) 100 mg/day
	Hypertension	Propranolol (e.g. Dociton) 50-100 mg/day
	Edema	Furosemide (e.g. Lasix) 40-80 mg/day
	Psychosis	Chlorpromazine (e.g. Propaphenin) 100 mg/day
	Seizures	Clonazepam (e.g. Rivotril) 12 mg/day

Follow-up	Metabolite analysis of prophyrin metabolism in urine and stool

Literature
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Doss M et al (1995) Diseases of the haem metabolism. In: Paumgartner G, Riecker G (Eds) Therapy of internal diseases. Springer Berlin Heidelberg New York p.711-720