THERAPY FOR HEREDITARY ANGIOEDEMA

_____________________________________________________________________________________________________________

THERAPY FOR ACUTE ATTACKS :

SUBCUTANEOUS ANGIOEDEMA :

Tranex or Ugurol, 500 mg in capsule or vial

• Dosage: 1 gr every 3-4 hours
  
• Side effects: diarrhea
  
• Effectiveness: not constant (approx. 30%). Better if taken at first symptoms of attacks
  

GASTROINTESTINAL ANGIOEDEMA :

Tranex or Ugurol, 500 mg in capsule or in vial

• Dosage: 1 gr. Every 3-4 hours (500 mg by injection every 3-4 hours if there is vomit)
  
• To help reduce symptoms (pain, vomit, ....) it could be useful to take an antispasmodic and/or metoclopramide
  
• It's not always possible to distinguish with certainty a gastrointestinal angioedema attack from different situations that require surgery (example: appendicitis, peritonitis, ....) If this is the case it's useful to give C1 inhibitor concentrate and wait for the medicine to take effect during the next hours before taking a different course of action (such as surgery).
• In the early stages of the attack, you can use the synthetic product Firazyr ® (Icatibant acetate), an inhibitor of bradykinin administered with pre-filled syringe (30 mg) by subcutaneous injection in the abdominal.

ANGIOEDEMA OF SUPERIOR AIR PASSAGES :

I.V. infusion of C1 inhibitor concentrate:

C1 Human Inactivitor - C-1 Berinert P, 1000-2000 U (a prepared freeze-dry to be made ready). Time it takes for effectiveness : 15-20 minutes

Or, especially in the early stages of the attack, the synthetic product Firazyr ® (Icatibant / acetate), an inhibitor of bradykinin administered with pre-filled syringe (30 mg) by subcutaneous injection in the abdominal.

In the United States where the concentrate is not available for therapeutic use, they use fresh frozen plasma which though is less effective.

_____________________________________________________________________________________________________________

LONG TERM THERAPY

Is used for patients with frequent attacks (more than one a month)

MEDICINE :

This is done by taking androgens derived medicine. 2 types exist :

1. Danazol: Danatrol, capsules 50-200 mg.
   
2. Stanozolol: Winstrol, galenical product
   

DOSAGE:

• Is decided by the doctor. Usually the starting dosage is high (approx. 600 mg for Danatrol), which is slowly reduced upto finding the correct dosage to control the attacks (100-200 mg). The medicine needs to be taken daily. In some cases and only with medical advice, it's possible to take the medicine every other day or in cycles.
  
• Effectiveness: good, but effectiveness ends if the treatment has been suspended.
  
• Contraindication: weight gain, pregnancy (1 e 2 trimester)
  

SIDE EFFECTS:

• Is linked to their bland androgen activity : - weight gain - acne - irregular menstruation (infrequent menstruation, spotting) - increase of muscular cytolytic and liverwort - microhematuria - polyglobulia - risk of increase of hepatic neoplasma
  

MONITORING:

• Annual blood tests (hemachrome, GOT, GPT, GGT, ALP, CPK) as well as urine test and hepatic ultrasound
  
• In some patients it's possible to try a treatment with Tranex (1 gr., 3 times a day).
  
• Effectiveness is scarse
  

_____________________________________________________________________________________________________________

SHORT TERM THERAPY

When different kind of work needs to be done in the oral cavity (such as : dental extraction, gastroscope, ....) it's advisable that an adequate therapy be done :

• In adults: Danatrol, 600 mg starting 6 days before and 3 days after
  
• In children: Danatrol, 10 mg/kg upto 600 mg per day
  
• If the patient never had any symptons, in this case it's not necessary to use the treatment.
  
• The C1 inhibitor concentrate and Firazyr ® (Icatibant / acetate), should always be available.
  

_____________________________________________________________________________________________________________

For further information and/or advice regarding the therapy during the attacks, an active service is available 24 hours a day. The number is: 333/4004422