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.
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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.
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:
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 Infectious Pathology and Immunodeficiencies unit of Paediatrics is a hospitalisation unit dedicated to the study and treatment of these diseases in children, and also acts as a consultancy in the diagnosis and control of complex infections in immunosuppressed patients. It was created in January 1996 for the study and treatment of paediatric diseases of infectious origin, but over time, the increase in cases of children with infections, for various reasons, means the unit has taken over more functions to face new challenges.
In our Unit, we treat infants with infections and immunodeficiencies, a pathology that has become increasingly important in recent years. This increase in cases is due to several reasons, such as the emergence of AIDS, the emergence of almost forgotten pathogens, the importing of diseases from other geographical areas, the increase in opportunistic infections as a consequence of more aggressive treatments and more invasive diagnostic techniques, and the presence of new patterns of microbial resistance.
The Paediatrics Teaching Unit has extensive experience in training specialists. There are a total of 60 Paediatrics residents at Vall d’Hebron University Hospital, 15 per year. Over the last few years, Paediatrics at Vall d’Hebron has been the first choice for new residents, and in the 2017 exam session achieved the best results of any Spanish hospital. In addition to this, we receive residents from hospitals all over the world.
Pediatric training itineraries
Over the last few years, paediatrics at Vall d’Hebron has been residents’ first choice, and in the 2017 exam session achieved the best results of any Spanish hospital.
The Department has a Paediatrics Teaching Subcommittee, comprising twelve tutors and twenty residents overseeing the practical application of the training and its integration into healthcare activities. Thanks to the involvement of these professionals, we can ensure supervised completion of the training programme objectives.
This Teaching Unit comprises different healthcare departments and units, including the Paediatrics, Nephrology, Neonatology, Paediatric Oncology and Haematology, Intensive Care, Neurology, Endocrinology, Infectious Diseases, Allergies, Cardiology, Respiratory Medicine, Gastroenterology, and A&E Departments.
It is vital for residents to train in research methodology as this is necessary to take part in and develop research projects. From the second year onwards, we invite residents to carry out research work, and a minimum number of papers and publications is required in addition to their full cooperation in sessions within the Department.
Why specialise at Vall d’Hebron?
Meningitis is an inflammation of the meninges, which are the membranes covering the brain and spinal cord. This is generally caused by an infection that is bacterial, viral or fungal.
Meningitis may also have various non-infectious origins such as trauma and tumours.
There are two types of meningitis:
Bacterial meningitis is a serious disease requiring immediate medical attention. Three microorganisms are the cause of the disease. They are naturally present in the pharynx, where they are commensal, or “friendly”, bacteria. The disease can progress very quickly. This happens when these microorganisms invade the blood stream. Bacterial meningitis is spread through respiratory secretions, for example when we cough or kiss. People sharing a home are likely to be infected.
The most common symptoms are:
Meningitis may also include nausea and vomiting, sensitivity to bright light, drowsiness, falling into a coma or convulsions.
The first symptoms of bacterial and viral meningitis are similar. However, bacterial meningitis is generally severe and can cause serious complications such as brain damage or learning difficulties.
In bacterial meningitis, the bacteria in the blood can give rise to another serious disease, septicaemia, which is the body’s generalized reaction to infection and which may have various consequences such as:
In the most serious cases it can be fatal. In this case, the appearance of small spots on the skin is cause for alarm.
Bacterial meningitis can be contracted by any age group but the elderly and young children are most at risk. In the last twenty years its epidemiology has changed significantly with the introduction of new vaccines linked to child vaccination programmes.
Whilst meningococcus is the main cause of bacterial meningitis around the world, in Spain meningococcal disease is not endemic, with an annual rate of fewer than 5 cases for every 100,000 people. Meningococcal meningitis may lead to death in 3-15 % of cases.
Many of the viruses that cause the disease are present worldwide and others are specific to particular regions.
If meningitis is suspected, blood and cerebrospinal fluid (the liquid around the spinal cord and brain) samples will be analysed. Cerebrospinal fluid is obtained via lumbar puncture. Analysis will help confirm the disease and will identify the microorganism causing it.
Most cases of infectious or viral meningitis improve without treatment in 7-10 days. However, it is very important that people with symptoms of meningitis receive immediate medical care to make a correct diagnosis of the disease, to determine the type of meningitis and to receive the right treatment.
In the case of bacterial meningitis, antibiotic treatment must be administered intravenously as soon as possible. Anti-inflammatories may also be used to treat any complications that may arise.
Paediatric Infectious Diseases and Immunodeficiencies Unit, Children's Hospital and Woman's Hospital
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