Porphyry syndrome acuteE80.2

Author:Prof. Dr. med. Peter Altmeyer

Last updated on: 18.12.2020

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DefinitionThis section has been translated automatically.

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!

TherapyThis section has been translated automatically.

See Table 1.

TablesThis section has been translated automatically.

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

LiteratureThis section has been translated automatically.

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

Authors

Last updated on: 18.12.2020

Author: Prof. Dr. med. Peter Altmeyer