We are the combination of four hospitals: the General Hospital, the Children’s Hospital, the Women’s Hospital and the Traumatology, Rehabilitation and Burns Hospital. We are part of the Vall d’Hebron Barcelona Hospital Campus: a world-leading health park where healthcare plays a crucial role.
Patients are the centre and the core of our system. We are professionals committed to quality care and our organizational structure breaks down the traditional boundaries between departments and professional groups, with an exclusive model of knowledge areas.
Would you like to know what your stay at Vall d'Hebron will be like? Here you will find all the information.
The commitment of Vall d'Hebron University Hospital to innovation allows us to be at the forefront of medicine, providing first class care adapted to the changing needs of each patient.
It is essential for patients to actively participate in the monitoring and treatment of their disease to increase their personal satisfaction and autonomy. Having reliable, verifiable information is also of great help in managing the disease.
Hereditary angioedema is such a rare disease that it is little-known even among healthcare workers. This means that in this case the patients themselves particularly need to know how to act in the event of an emergency, especially when they are not in their usual environment or are far away from their medical team.
It is advisable, as far as possible, to avoid possible triggers or aggravators of attacks:
The following symptoms indicate a suspected case:
The doctor must make a correct differential diagnosis in order to rule out other causes, such as appendicitis.
In this case it is important to remain calm and follow the doctor’s instructions. Here is some general advice:
1. Ask someone to help you explain what is happening to you.
2. Have the clinical report issued to you by your doctor at the ready.
3. If you have rescue or emergency medication (intravenous Berinert® or Cinryze®, or subcutaneous Firazyr®):
a) If you have been taught how, self-administer it in accordance with the instructions.
b) If you cannot administer it yourself, take it with you to the health centre.
4. Go to your nearest health centre for emergency treatment.
5. Make an appointment with your specialist once the immediate crisis has been dealt with.
In the case of a significant symptom burden in type I and II angioedemas, C1-INH may be administered as a prophylaxis.
TRANSFUSIONS – CAN I GIVE BLOOD?
It is not advisable for patients with hereditary angioedema of any type or acquired C1-inhibitor deficiency to donate blood.
LONG OR FOREIGN TRIPS
We recommend you take an up-to-date copy of the clinical report issued by your doctor with you. It is a good idea to have the report translated into the language of your destination or English.
Find out where the nearest healthcare centre is.
Always carry rescue or emergency medication with you and make sure it has not expired. Have your medical report to hand at security controls at airports or railway stations to avoid problems.
DIET
You do not have a follow a special diet because it is not an allergic oedema and it is not caused or triggered by a food allergy.
Diet does not have any impact on the evolution of the disease. You should, of course, follow the healthy diet recommendations issued to everyone.
Hereditary angioedema is a rare genetic disease that affects approximately one in 50,000 people. It is usually an inherited disorder and is characterised by the accumulation of fluids outside the blood vessels, causing swelling of the face, hands, feet, extremities, genitals, gastrointestinal tract or the upper respiratory tract. Because it is a low-prevalence disease with symptoms similar to those of other diseases and is therefore difficult to diagnose, it is important for there to be reference centres so that suspected and diagnosed cases can be centralised.
The inflammation that hereditary angioedema causes does not present associated itching and may last for 1 to 5 days. These symptoms are developed as a result of the malfunction of certain proteins that help maintain the normal flow of fluids through the blood vessels (arteries, veins and capillaries).
The seriousness of the disease shows a significant degree of variance. Angioedema episodes may be extremely incapacitating and have a serious effect on the patent’s quality of life. When it occurs in the region of the mouth or neck, the sufferer may die of asphyxia if they are not given preventive treatment.
In most cases symptoms start to manifest in childhood and/or puberty and continue throughout adult life.
There are different types of hereditary angioedema and they are classified according to whether or not they present a deficiency of the C1 component of the complement (C1-INH).
Swelling of the subcutaneous tissue in any part of the body, although it is most commonly found in:
Depending on the affected area, the symptoms may range from local discomfort to invalidity of the affected extremity, discomfort or pain when swallowing, voice changes, loss of voice, or dyspnoea (shortness of breath).
At one time of their life up to 50% of patients may present an episode that affects the throat, which if not immediately treated could lead to asphyxia.
Hereditary angioedema affects people who exhibit a mutation in certain genes, such as SERPING1, F12, PLG, KNG1 and ANGPT1. As it is a dominant autosomal disease, an affected patient has a 50% chance of passing it on to their children. Given that it is a genetic disorder, it is common to find that more than one member of the family is affected.
Depending on the type of mutation, it may affect men and women equally (types I and II) or women more frequently (HAE-nC1-INH). Cases of hereditary angioedema without C1-INH deficiency are usually associated with hyperoestrogenic states, such as pregnancy or the consumption of contraceptives that contain oestrogens.
The Allergology Clinic first assesses patients who present with recurring angioedema episodes and cases in which there are family members who also suffer them. Subsequently, a blood analysis is requested to determine the levels of the components of the complement, including the inhibitor of component C1 (C1-INH) and, finally, the diagnosis is completed with a genetic study.
Treatment depends on the number of attacks, the severity of the symptoms and the degree to which quality of life is affected. Treatment is always on a case-by-case basis and may be acute, which means the subcutaneous of intravenous administration of medication at the time of the angioedema attack, or preventive, to stop attacks occurring so frequently. The latter treatment is usually recommended for the patients who suffer the most episodes.
Angioedema treatments can be self-administered by the patients.
In the case of surgery, endoscopies, tooth extractions or certain dental procedures, treatment must be given in advance to prevent an attack.
Blood analysis normally forms part of the diagnostic procedure. Depending on the treatment, during monitoring it may be necessary to perform an abdominal ultrasound and draw blood for analysis.
Factors known to possibly trigger attacks should be avoided as far as possible:
Minority diseases, also called rare diseases, are those that affect between 5% and 7% of the population. They are very varied, affecting different parts of the body with a wide range of symptoms that change both between diseases and within the same disease. It is estimated that some 30 million people in the EU, 3 million in Spain, and around 350,000 in Catalonia suffer from one.
The complexity of most rare diseases requires multidisciplinary care with professionals from different medical specialities, case management for nursing, psychological support and also social work.
The Vall d'Hebron Barcelona Hospital Campus is home to more than 100 specialist professionals dedicated to the care of more than 2,000 rare diseases. Apart from treating the most rare diseases of any centre in Spain, it is one of the leading hospitals in Europe in this field. In fact, Vall d'Hebron is part of 20 European reference networks, known as ERN. This makes this hospital a highly specialised centre for rare diseases, from birth to adulthood, through a networked system that allows sharing of resources and knowledge with other world-class hospitals.
Adult and child
Pediatric
This concentration of patients with rare diseases at Vall d'Hebron improves knowledge and promotes research. Research in this field focuses above all on improving diagnostic capacity for diseases that are often difficult to diagnose and on developing new treatments for those diseases. In the case of diseases with few patients, publicly funded research is often the main avenue for the discovery of new drugs, and public health is the framework that provides the public with access to high medication complexity.
For more information, contact the Rare Disease Team at the following email address: minoritaries@vallhebron.cat
A tumour is an abnormal growth of tissue. In the case of orbital tumours, this growth is located in the tissues around the eye, which may be muscles, bones, fat, the lacrimal gland, nerves and blood vessels. They are rare tumours of several different types that may appear at any age. Orbital tumours may be benign or malignant. Benign tumours may cause pain due to compressing or displacing the different structures in the eye socket. Malignant tumours, on the other hand, as well as spreading to neighbouring tissue, may produce metastasis in other unconnected organs or lymphatic nodules.
The most common symptom is a protrusion of the eyeball out of its socket, known as “exophthalmos”. However it can also cause loss of vision due to compression of the optic nerve, double vision, pain and can limit the movement of the eyeball.
In some cases, tumours may be present in the eye socket for an entire lifetime with no symptoms.
It is hard to know the exact number of people affected by orbital tumours as it is a rare kind of tumour that includes several variants.
Benign tumours are the most common; capillary haemangiomas and dermoid cysts in children, and cavernous haemangiomas in adults.
The most common malignant tumours in children include rhabdomyosarcoma, and in adults lymphoma cancers of the lacrimal gland and metastases.
Imaging studies (CT and nuclear magnetic resonance scans) allow precise location of the tumour, its size to be measured and certain biological characteristics to be known. This information, together with the patient's age and the speed of the tumour's growth, enables an initial assessment of whether or not it is malignant.
A definitive diagnosis is made after a biopsy of part or all of the tumour.
In most cases, the main treatment is surgery to remove the tumour and therefore avoid the damage it may cause if left to grow within the eye socket by compressing or displacing the eyeball and other structures.
Modern-day orbital surgery techniques allow extraction of the tumour by making small incisions in areas that are hidden or not very visible. This enables faster postoperative recovery.
In the case of malignant tumours, different combinations of surgery, radiotherapy and chemotherapy are used. It should be noted that regular check ups are needed after treatment.
Where there are no symptoms, observation and monitoring of the speed of growth is usually sufficient.
There are currently no preventative guidelines to reduce the risk of orbital tumours.
These syndromes are a group of diseases characterised by insufficient blood cell production (anaemia, neutropenia and thrombocytopenia), constitutional malformations and the risk of cancer.
They are usually diagnosed in childhood but there some cases diagnosed in adults. Specifically, these syndromes are: Fanconi anaemia, dyskeratosis congenita or selectively severe congenital neutropenia, Diamond-Blackfan anaemia, Diamond-Shwachman syndrome, and amegakaryocytic thrombocytopenia.
Malformations caused by inherited bone marrow failure syndromes affect the skin, bones, heart and digestive system as well as the urinary system, the central nervous system, and others. They may also affect the area around the bone marrow.
Besides this, they also increase the predisposition to cancers such as acute myeloid leukaemia, myelodysplastic syndrome and squamous carcinoma of the head, neck and reproductive organs.
These syndromes have important biological pathways in common related to cell growth and division such as the activation of the p53 gene that is responsible for halting the cell cycle, cell ageing and cell death. In addition, mutations have been identified in more than 80 genes.
Although this is treated in childhood, once the patient reaches adulthood monitoring is very important. Early diagnosis of these syndromes is essential to ensure the patient receives appropriate treatment. The aim is to minimise toxicity at the same time as allowing genetic counselling, and implementing strategies for cancer prevention and monitoring. This treatment should focus on treating the bone marrow failure, but also any constitutional malformations and extra-haematological manifestations, in addition to cancer treatment.
A multidisciplinary team of specialists with extensive experience of these diseases is necessary to ensure optimum patient care.
The first symptoms are related to a lack of blood cell production:
There are also signs related to malformations such as:
Estimated incidence rates of the different syndromes are:
The usual tests to detect inherited bone marrow failure are:
The following methods are used to treat these pathologies:
To prevent these diseases it is important to avoid smoking, alcohol, sun exposure, and to have a balanced diet. In addition, a cancer prevention programme should be followed with regular visits to the Cancer Prevention Unit and the Ear, Nose and Throat, Maxillofacial and Gynaecology departments.
In addition to providing multidisciplinary care for patients of all ages who suffer this condition, the objectives of Vall d’Hebron Hospital’s Hereditary Angioedema Unit include teaching and research in this field.
The Hereditary Angioedema Unit (UAEH) of Vall d’Hebron University Hospital’s Allergology Department has been treating patients with this disorder for more than 25 years.
UAEH outpatients are treated by allergology specialists in a multidisciplinary manner in the Outpatient Clinic in the Old Nursing School and in the Children’s and Women’s Hospital, ensuring transference and continuity of care from childhood through to adulthood for this genetic, lifelong condition.
The Oral and Maxillofacial Surgery specialty is defined as "the medical and surgical specialty dealing with the prevention, study, diagnosis, treatment and rehabilitation of the oral cavity and face, as well as the cervical structures that are directly or indirectly related to them".
The care provided at the Oral and Maxillofacial Surgery Department at the Vall d'Hebron University Hospital covers the following areas: outpatient visits, surgery, hospitalisation and accident and emergency services. Our Department is the only one in Catalonia with a 24-hour on-call system. This means we are a leading player in the Catalan public network.
The Teaching Unit is led by the Oral and Maxillofacial Surgery Department, with participation from other specialisations such as General Surgery, Vascular Surgery, and the Traumatology Intensive Care Unit.
Oral and maxillofacial surgery training itinerary
Why specialise at Vall d’Hebron?
Maxillofacial and Oral Surgery, General Hospital
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