The association, a voluntary and non-profit organization, was established March 29, 1980, and recorded in Milan September 24, 1980.
The association aims to spread awareness of the disease in order to allow a correct diagnosis; act because in Italy you realize the full availability, and the highest levels of quality, of medicines and therapeutic aids needed to prevent and fight against the disease; support and facilitate the access of all patients to appropriate therapies; collaborate in the organization of care for patients with hereditary angioedema; create a health document identification, officially recognized with the description of the disease, therapeutic indications emergency and address of doctors turn to for more information; promote medical meetings, conferences and congresses at national and international level, to promote exchanges of information.
We recommend all patients, in case of acute attack, if they cannot self-infuse at home, to go promptly to a first aid or activate the emergency medical service preventing the attack from reaching its maximum swelling.
You should be accompanied by a relative as anyone who is aware of your condition as a rare disease patient so that he/she can assist you and communicate with caregivers in case you were not able to be fully self-sufficient.
Invite health care professionals to visit this site and especially this page.
We recommend all patients to take with them the concentrated plasma C1 Berinert-P or the Firazir (Icatibant / acetate) depending on the instructions received from their doctors.
If you need any help invite the health staff to contact the emergency number.
Angioedema deficiency of C1-inhibitor (C1-INH) is a disease caused by the deficiency in the serum of affected, the inhibitor of the first portion of the complement system. It can be caused by a genetic defect (hereditary angioedema) or by an increased consumption (acquired angioedema).
IT HAPPENS WITH THE DISEASE:
Angioedema for subcutaneous edema
Abdominal colic type for gastrointestinal mucosal edema (acute abdomen)
Dyspnea with signs of asphyxia due to laryngeal edema. Particularly frightening is the glottis edema that can lead to death by asphyxiation. Symptoms last 1-3 days – is not allergic disease
For laryngeal edema and severe abdominal colic administer:
The patient usually has availability of one or more packages of the life-saving medicine in the type and dosage indicated to him by the medical practitioner who follows him in his course of treatment. If you have further questions, please visit the association or the doctor available in an emergency.
the protein deficient 1000-2000 through customized units of C-1 Berinert-P, produced by CSL Behring GmbH, or the synthetic product Firazyr® (Icatibant acetate), an inhibitor of bradykinin; administered with pre-filled syringe (30 mg) by subcutaneous injection in the abdominal area; The drug is marketed in Italy by: Shire Italian spa.
The patient usually is in possession of drugs.
In the absence of these drugs, use fresh frozen plasma.
Cortisone ANTIHISTAMINES AND ARE INEFFECTIVE
In case of attack of the face, oral cavity and pharynx, neck or severe abdominal attack, it is recommended that the staff of the emergency room triage officer to be assigned to the patient:
YELLOW CODE
If there is even one sign of laryngeal involvement (dysphonia, stridor, retraction of the jugular, dyspnea, desaturation) assign:
RED CODE
(Cicardi et al. Intern Emerg Med 2014; 9: 85-92.)
The patient usually has availability of one or more packages of the life-saving medicine in the type and dosage indicated to him by the medical practitioner who follows him in his course of treatment. If you have further questions, please visit the association or the doctor available in an emergency.
Doctor available in case of emergency: +39 333 4004422
In case of lack of C1- BERINERT, call:
CSL Behring S.p.A. – P.le Stefano Turr, n. 5 – 20149 – Milan – Italy – Tel. +39 02 34964207 / 208 – Fax +39 02 34964264
In case of lack of Firazyr ® (Icatibant / acetate), call:
Shire Italia SpA – Piazza della Nunziata, 5/4 – 16124 – Genova – Italy – Tel: +39 010 2758377 – Fax: +39 010 2745341
To contact the association:
Vincenzo Penna – Fraz. Quarto Inf. 192 – 14030 Asti – Tel/Fax: +39 0141 299274 – Email: info@angioedemaereditario.org
Nilla Ciairano – Via Piedicavallo 20 – 10145 Torino – tel. +39 3334023513 – Email: nillaci@fastwebnet.it
Italian centers for hereditary angioedema: requirements
• Name of the person responsible and reference figure
• Outpatient Activities • Diagnostic tests carried out by the center or by external structures
• Management of acute attacks (in the emergency room with availability of drugs, assistance of patients during operations and / or maneuvers in the mouth, etc.) • Medication Management (treatment plans and supply of drugs)
• Training: for the physicians of the center, meeting / training for self-administration of medicines for patients
• Support Specializations (dentistry, gynecology / obstetrics)
• Official recognition (authorized certification centers of rare diseases have official recognition in Campania, Lazio, Lombardy, Puglia, Veneto, Sicily)
• Inclusion in the list of centers with such features
Please remember that maneuvers on the oral cavity, such as tooth extractions, endoscopic examinations, etc. can cause edema of the glottis. People can have treatment and dental advice in the specialized centers listed below:
Dentistry Surgery Manager: Dr. Antonella Sparaco A.O. Luigi Sacco – Via G.B Grassi, 74 20157 – Milan – e-mail: odontoiatria@hsacco.it – FAX: 02 39042397
For appointments contact Mrs. Beba: Tel. 02 39042298
For clinical information contact Drs: Antonella Sparaco and Mario Ghezzi (ghezzi.mario@hsacco.it) – Tel .: 02 39042638
The service is available on Wednesday
University of Padova Dental Clinic Studies: Via Venezia 20, 35100 Padova
Director: Prof. Edward Stellini Dental surgeon
Reference: Dott. Stefano Sivolella
Anesthesiologists: Prof. John Manani, Prof. Enrico Facco, Dr. Gastone Zanette
Patients can refer directly to Dott. Mauro Cancian Medical Clinic 1st tel. +39,049,821 4452- Fax +39,049,821 2151-
e-mail: mcancian@unipd.it
University – Policlinico Tor Vergata
Professor Barlattani
Referent for Dentistry Patients can refer directly to Prof. Perricone: Cell. 3204333759 e-mail: roberto.perricone@uniroma2.it
A.S.U.R. MARCHE – WIDE AREA N.2 – HOSPITAL FABRIANO (AN)
Unit of Oral Surgery and Stomatology Director Dott. Roberto Mazzanti
Dental Therapy service for patients A.E.E. Responsible Dott. Roberto Volpi Tel. 0732/707 294 e-mail: roberto.volpi@sanita.marche.it
AZIENDA OSPEDALIERO UNIVERSITARIA OSPEDALI RIUNITI di ANCONA
SOD Surgical Dentistry and Specialization Director Dr. V. Zavala Via Conca 71 – ANCONA Tel .: +390715963144
Regional Reference Center for prevention and treatment of oral disease in patients with disabilities and at risk Manager: Dr. Alessandra Nori Tel + 39071-5963064 e-mail: a.nori@ospedaliriuniti.marche.it
In collaboration with Prof. Caterina de Carolis, it was launched a consulting service aimed at all gynecological patients suffering from hereditary angioedema.
Clinica Villa Pia – Polispecialistica Privata Convenzionata
Via Ramazzini, 93 – Roma
Responsible Physician: Prof. Caterina De Carolis
For information and reservations: 065827875 – e-mail: caterina.decarolis@clinicavillapia.it – www.clinicavillapia.it