Porphyry syndrome acuteE80.2
DefinitionThis section has been translated automatically.
TherapyThis section has been translated automatically.
TablesThis section has been translated automatically.
Therapy of acute porphyria syndrome (according to Doss and Frank)
Therapeutic measures |
Procedure |
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Immediate measures |
Discontinuation of porphyrinogenic drugs and intensive medical monitoring |
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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 | ||
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Symptomatic measures |
Electrolyte control |
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Control and force diuresis (e.g. furosemide 40-80 mg/day) | ||
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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 |
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Edema |
Furosemide (e.g. Lasix) 40-80 mg/day |
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Psychosis |
Chlorpromazine (e.g. Propaphenin) 100 mg/day |
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Seizures |
Clonazepam (e.g. Rivotril) 12 mg/day |
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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