Porphyry syndrome acuteE80.2

Author:Prof. Dr. med. Peter Altmeyer

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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.

  1. 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

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Last updated on: 18.12.2020