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.
Ampullary epidermolysis is a group of genetic disorders that may present themselves in various ways, from milder forms to more severe ones: affecting the skin and mucous membranes, involving the formation of blisters and vesicles after the slightest trauma. They can also affect other organs, in different ways.
The best thing is if the patients, their families and their caregivers receive comprehensive health education, especially when they are first diagnosed, during the baby’s first few days, when skin lesions can already begin to occur.
The education aimed at preventing the evolution and complications of the disease will be given by professionals from the following disciplines:
Skin affected by ampullary epidermolysis is very sensitive to the slightest pressure or friction, which then causes a blister to form. To avoid damage, bear in mind the following recommendations:
The most effective way of avoiding the illness is vaccination. The diphtheria vaccination is highly effective and is administered as part of the Systematic Immunisation Programme in Catalonia (Programa de vacunacions sistemàtiques).
Diphtheria is transmitted via the respiratory tract, mainly, and also by direct contact with a sick person or a healthy carrier of the bacteria. The illness may affect the tonsils, pharynx, the larynx, the nasal mucous membrane and, much less frequently, the skin or other mucous membranes. The bacteria forms a thick grey membrane with a dark red swollen area around it, which in the case of the nose and throat may obstruct the respiratory tract.
Some people may carry the bacteria in their nose or throat. If these people are vaccinated they will not develop the illness, but they may transmit the bacterium to other people via droplets produced when they sneeze or cough. The existence of carriers in countries with no cases of the illness is very rare.
Patients with Asperger’s syndrome need a stable and predictable environment that can be easily adapted. It is key to their well-being to establish routines according to their interests, organise their time, avoid inactivity or over intense activity as well as sudden changes. Although the syndrome has no cure, appropriate treatment and involving family members can improve the quality of life of patients.
People with Asperger’s syndrome may have different requirements depending on their age, surroundings and the awareness that they have of their difficulties. For this reason, they need a tailor-made programme that responds to their specific case.
The aim of these customised programmes is to:
It is important to manage their development through different disciplines. These may include cognitive treatments, social skills programmes and occupational therapy for the patient. You also have to consider guidelines on how to resolve conflicts and how to manage pyschoeducational groups for families or caregivers.
In infants, from an emotional and attitudinal point of view, it is important to learn to identify the warning signs in their mood. In this way, we can prevent difficulties in anger management and low tolerance to frustration, since they are patients with a high degree of sensitivity to criticism. Avoid punishment as much as possible and establish more positive reinforcement strategies.
All these guidelines must be established in a space where the differences the child or adolescent presents are valued positively, including their limitations, but also their possibilities and positive aspects.
In adults, many of these characteristics continue, as Asperger’s cannot be cured. In any case, personalised treatment, involving family members and good communication with professionals can allow a better quality of life.
Juvenile idiopathic arthritis (JIA) is a chronic disease characterised by persistent inflammation of the joints that begins before the age of 16.
There are various types of JIA which can be identified by the number of joints affected and the presence of symptoms such as fever and skin manifestations, amongst others. The diagnosis is made by observing the symptoms during the first 6 months of the disease.
The main symptoms are pain, swelling and increased heat in the joints, with stiffness and difficulty moving. Sometimes the beginning is slow, insidious and progressive. The child may be tired or irritable, if they are younger. Older children may notice stiffness when moving their joints when they get up in the morning. At other times, the beginning is acute and serious, with the presence of general symptoms such as general malaise, fever, blemishes on the skin and several swollen joints.
JIA is a relatively rare condition that affects 1 or 2 children in every 1,000.
JIA diagnosis is based on the presence of persistent arthritis and carefully excluding any other condition by using the clinical history, physical examination and blood tests.
JIA is considered where the condition begins before the age of 16, the symptoms last for more than 6 weeks and other conditions that may be responsible for arthritis have been discounted.
The treatment must be put in place early and each child must be considered individually, which means that the therapy will have different levels of intensity depending on the type, time and seriousness of the condition.
Its aim is to care for the child’s all-round physical and psychological development, to try and improve all aspects of their quality of life.
To ensure that there are no after-effects, or that these are minimised, there must be ongoing effort and close collaboration between the child and their parents or family and the various specialists. It is essential that the family understands this disease. The child will begin to learn about it according to their age.
When it comes to diagnosis, certain analytical tests are valuable, along with examinations of the joints and eye tests for a better definition of the type of JIA and identification of the patients at risk of developing specific complications, like chronic iridocyclitis.
The rheumatoid factor (RF) test detects this autoantibody which, if positive and found persistently in high concentrations, indicates a subtype of JIA.
Antinuclear antibodies (ANA) usually test positive in tests on patients with early onset oligoarticular JIA. The population of patients with JIA has a greater risk of developing chronic iridocyclitis and, therefore, eye tests using a slit lamp should be scheduled (every three months).
HLA-B27 is a cellular marker which tests positive in up to 80% of patients with arthritis associated with enthesitis. In contrast, it is only positive in 5%-8% of healthy people.
Other examinations are valuable, such as the erythrocyte sedimentation rate (ESR), or C-reactive protein (CRP), as these measure the degree of general inflammation. Nevertheless, diagnostic and treatment decisions tend to be based more on the clinical manifestations that appear rather than the analytical tests.
Depending on the treatment, patients may need periodic tests (such as haemograms, liver function tests, or urine tests) to check for treatment side effects and any pharmacological toxicity that may not show any symptoms. Joint inflammation is mainly assessed by clinical examination and, sometimes, using imaging studies, such as ultrasound. Periodic X-rays or magnetic resonance (MRI) scans can be helpful in assessing bone health and growth and in personalising the treatment.
Associació Espanyola de Febre Mediterrània Familiar i Síndromes Autoinflamatoris
FEDER
Lliga Reumatològica Catalana
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:
A urinary tract infection is defined as the presence of invasive bacteria in the urinary system, together with signs of inflammation, such as high temperature and local pain.
Urinary tract infections may be located in the lower urinary tract (bladder and urethra), or the upper urinary system, affecting one or both kidneys. A kidney infection is also known as pyelonephritis.
Infections of the lower urinary tract are characterised by localised pain, which increases when urinating, and sometimes by cloudy or dark urine, usually without high temperatures.
Kidney infections (pyelonephritis) are characterised by high temperatures, acute local pain in the lower back, and pain or irritation when urinating.
Urinary tract infection is characterised by the presence of local pain (lower abdomen or lumbar region), which increases when urinating. The urine is often cloudy, or dark if it contains blood. There may be high fever, especially in the case of pyelonephritis (an infection of the upper urinary tract).
It can affect people at any age, from early childhood to old age. It is more frequent among women and there are factors that make people vulnerable to it (pregnancy for women and enlarged prostate for men) as well as urological anomalies (pre-existing malformation or presence of kidney stones).
Urinary infections are diagnosed by examining urine under the microscope (sediment) to see whether it contains white blood cells and/or bacteria, and by cultivating the bacteria in a microbiological culture to identify the strain and determine the most appropriate antibiotic for treatment (antibiotic susceptibility testing).
Urinary tract infections are usually treated with antibiotics. Treatment is oral in the case of lower-tract infection.
For upper-tract infections (pyelonephritis) it is usually intravenous, although in some cases outpatient oral treatment may be administered.
The standard tests are urine sediment and culture (urine culture with antibiotic susceptibility testing). An ultrasound scan may be indicated for examining the kidney and urinary tract and identifying obstructions or kidney stones that may have brought about the infection.
Ultrasounds are also used to assess the state of the kidneys. A general analysis may also be indicated to see how the urinary tract infection is affecting the rest of the body, and specifically the renal function.
Urinary tract infection can be prevented by frequent urination (every 2 to 3 hours) and, above all, by avoiding the habit of holding in urine, and by going to the toilet whenever the bladder feels full, without waiting too long.
There are two very different types of otitis, both of which children can suffer from: external otitis and middle ear infection.
External otitis affects the auditory canal and is above all related to exposure to swimming pool, bath and fresh water in general. It is most common in summer.
Middle ear infection is related to infections in the upper respiratory tracts, and can be self-limiting (it resolves itself in most cases) or purulent requiring antibiotic treatment It is most common in winter.
b) Middle ear infection: there is usually a history of catarrh in the respiratory tract, suddenly accompanied by pain (in small children this can manifest as intense crying), with or without fever. It does not hurt when the external ear is touched or moved. Sometimes the ear drum may be perforated due to inflammation and pressure, which may result in a purulent secretion, generally associated with the pain (and crying) disappearing.
In both cases, diagnosis is clinical: medical history, assessing symptomatology and exploration using an otoscope.
Otoscope exam. Occasionally, if there is suppuration, cultivation of the pus.
Bronchiolitis is an infection that causes the small respiratory passages in the lungs (bronchioles) to become inflamed and mucus to build up in them. This blocks the flow of air, making it difficult to breathe.
It happens more often in babies because their airways are smaller and more easily blocked than in older children.
Bronchiolitis is not the same as bronchitis, which is an infection in the larger and more central airways that typically causes problems in adults.
Human respiratory syncytial virus (HRSV) is the most common cause of lower respiratory tract infection in babies and small children and it is one of the viruses that causes fever in children.
When it infects the lungs and airways, it is often responsible for bronchiolitis and lung disease or pneumonia in children less than one year old. In fact, the highest incidence of HRSV occurs in babies from two to eight months old.
It occurs more often between the months of October and March.
Human respiratory syncytial virus (HRSV) is also the most common cause of hospital admission in babies under one.
Bronchiolitis often begins with the same signs as catarrh. Infection can stay in the nose or extend to the ears and lower respiratory tracts.
Babies and small children affected by HRSV may show signs of:
Treatment for bronchiolitis due to HRSV basically consists of alleviating the symptoms. Antibiotics, which treat bacteria, are useless because, as we mentioned above, it is caused by a virus.
It is therefore advisable to:
Severe cases are treated in the hospital to give humidified oxygen and medication to help the child breathe more easily. In total, the condition usually lasts between one week and ten days, although a residual cough may persist for weeks. Bear in mind that the virus does not give the child immunity; they can become infected twice in the same season and reinfected in subsequent years.
Human respiratory syncytial virus (HRSV) is very contagious. It is spread if you come into direct contact with the nasal and throat secretions of someone who has the disease. This can happen when another child or adult coughs or sneezes nearby and the tiny droplets are inhaled by the baby. Also through hands or objects that have come into contact with infected people and then come into contact with the baby.
The virus can live for half an hour or more on your hands. It can also live for up to five hours on clothing, tissues, toys or furniture.
Infection can be prevented using several measures:
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
The disease caused by the Zika virus is contracted by a bite from an infected mosquito, as in the case of dengue fever, chikungunya and yellow fever. It can also be spread through sexual intercourse, pregnant women may transmit it to their children, or through blood transfusions. In Europe there are no cases of infection by mosquito; all cases have been imported.
It is disease lasting a short time that can be overcome without complications or the need for admission to hospital. However, there is a relationship between this infection and some neurological disorders. In addition, pregnant women who are infected may give birth to babies with microcephaly.
The incubation period in humans is 3-12 days, up to 15 maximum. Although on many occasions there are no symptoms, when there are the disease is characterised by:
Since 2015, 71 countries have declared transmission of the Zika virus via mosquitoes. In addition, 13 more have stated that the disease has arrived by other means, generally through sexual contact.
In Europe, most cases have been imported from countries where it is endemic, mainly from Latin America but also from South East Asia. In Catalonia in December 2016, there were 150 registered infections, of which 32 were pregnant women.
Between the first seven to ten days of the disease, diagnosis is made using molecular biology techniques (RT-PCR) in blood and urine to detect the virus.
After this period, Zika disappears from the blood and is detected through antibodies in the serum.
There is no specific treatment for this disease. Symptoms generally disappear between three and seven days after infection. They are therefore lessened with analgesics and antipyretics.
There is currently no vaccine for this virus. For this reason, prevention is based on avoiding mosquito bites in countries where it is endemic, as well as using protection during sexual intercourse.
In the case of Catalonia, the risk is associated with the arrival of travellers from countries where it is endemic. Here there is a screening programme for pregnant women and their partners; they are a sensitive group as the virus may be passed to the foetus.
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