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.
Below we will list the departments and units that form part of Vall d’Hebron Hospital and the main diseases that we treat. We will also make recommendations based on advice backed up by scientific evidence that has been shown to be effective in guaranteeing well-being and quality of life.
Vols saber com serà la teva estada a l’Hospital Universitari Vall d’Hebron? Aquí trobaràs tota la informació.
Congenital heart diseases are structural and/or functional malformations of the heart present at the time of birth and, in the vast majority of cases, the causes are unknown. This type of heart disease is multiple and varied, and the signs and symptoms are diverse: they range from asymptomatic, which do not require specific treatment, to severe ones, which need surgery during the first days of life.
What do you need to bear in mind if you care for an infant with congenital heart disease?
To improve the quality of life of infants with congenital heart disease, you are advised to follow these daily tips and routines.
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.
The heart is made up of four cavities, two atria and two ventricles. The atria are separated from each other by an interatrial wall or septum, and the ventricles by an interventricular wall or septum. Between the atrium and the ventricle there is the atrioventricular valve. The veins arrive into the atria and the major arteries leave the ventricles. Between the ventricle and its artery outlet there is the semilunar valve. The heart is divided into the right and left sides.
Non-oxygenated blood arrives at the right atrium via the venae cavae, from the head and arms (upper vena cava) and from the abdomen and legs (lower vena cava). This blood passes to the right ventricle through the tricuspid valve. The right ventricle pumps this blood, through the pulmonary valve, into the lungs through the pulmonary arteries, which is where the blood gets it oxygen.
This oxygenated blood returns to the left atrium via the pulmonary veins. From the left atrium it is directed to the left ventricle through the mitral valve. The left ventricle pumps the blood to the aorta through the aortic valve to distribute it to all the organs and tissues in the body.
The heart is irrigated by the coronary arteries, right and left. These coronary arteries divide into several branches to carry oxygenated blood throughout the heart tissue.
The heart contracts due to an electric stimulus triggered by the conduction system. The cardiac conduction system is made up of a series of cells that have the capacity to create this stimulus and determine heart rate. This stimulus begins in the sinus node, which is found where the superior vena cava enters the right atrium. This stimulus causes the atrium to contract. This stimulus then propagates the ventricle through another structure called the atrioventricular node. This conduction system is capable of increasing the heart rate when necessary, such as for example during exercise, when you have a fever, when you feel emotions, etc., or decreasing the heart rate when you are sleeping. This system is regulated by the action of different hormones or in response to nervous stimuli in the cardiac plexus.
The cardiac cycle has two phases: systole and diastole. In systole, the heart contracts to send blood to the major arteries and during diastole it relaxes to fill with blood to later be ejected.
Cardiac ablation is a procedure used for treating arrhythmia (abnormal heart rhythms). The child's treatment continues even after surgery and discharge from hospital.
Before they leave the hospital, the child and their family will be provided with the guidelines and recommendations for the recovery process by the medical and nursing team.
The following recommendations have to be borne in mind:
The child may have a shower 24 hours after the procedure. Immersion baths can be taken after 7 or 10 days, once the puncture area has completely healed.
The wound must be cleaned with soap and water every day, and a small amount of antiseptic applied, during the child’s first 3 or 4 days at home. We recommend use of 2% aqueous chlorhexidine. The area should be sealed afterwards with a fabric dressing.
The child must rest at home for 3 or 4 days. They must not do any heavy exercise such as walking upstairs nor should they stand up for long periods. Once that period has passed, the child can go back to school.
They can lead a normal life, but without doing any sport for the first two weeks after the procedure. During the summer, the child can swim at the beach or in a swimming pool from 10 days after their operation.
The child can continue with their usual diet. It must be complete and varied.
The following aspects need to be monitored for the first few days:
Monitoring the puncture point. If it turns red or oozes fluid, or if the child has a fever, their reference health centre must be consulted about this.
It is normal for a small bruise to appear at the puncture point. If the swelling is large, this is a sign of haemorrhaging. It can cause pain and discomfort for the child. In that case, the child’s reference doctor must be consulted about this.
A piercing, low-intensity pain of short duration may appear in the puncture area, back or abdomen.It usually gets better after a few days and can be controlled with conventional painkillers. It is common and not a cause for concern.
If the pain is more intense and prolonged, then the medical team should be consulted to avoid any possible complications.
If the child's temperature rises, the reference medical centre must be consulted.
The child may sense a stronger heartbeat for the first few days after ablation. This is normal and goes away after a few days.
The prescribed treatment and the recommendations from the cardiology and nursing teams must be followed.
Menudos corazones
This is the most serious group of primary immunodeficiency disorders (PID) - genetically-based minority immune system disorders - affecting the T lymphocytes, cells that are essential to eliminate microbes.
The global incidence is around 1/50,000 newborns, with regional differences and a greater incidence among populations with a high consanguinity rate.
This is a group of genetic diseases included in the PID group, in which the thymus (the training school for lymphocytes) does not work properly. It includes different types of genetic diseases, each of them with a specific genetic alteration.
All of them are genetic diseases and depending on the type of SCID they can be inherited (mutation in the parents) or not (newly appeared mutation). The most common way is linked to the X chromosome and only affects boys. The others can affect both boys and girls.
When there are clinical symptoms, with a blood test to see if there are lymphocytes and whether they are working properly.
Since 2017, in Catalonia it has been possible to detect it in newborns with the heel test, enabling early diagnosis before complications begin to appear and resulting in a better prognosis. The newborns that test positive in the screening are referred to the leading immunodeficiency hospital (in Catalonia, Vall d’Hebron Children’s Hospital)
This is a serious disease that is potentially fatal if not treated in time.
• Serious viral, bacterial and fungal infections.
• Skin, intestinal, lung alterations, etc.
They can be cured with a bone marrow transplant and genetic therapy.
Whilst waiting for a transplant, patients must take protective measures (antibiotics, immunoglobulines, isolation, etc.) to be in the best possible state of health when the transplant takes place.
This is a type of primary immunodeficiency disorder (PID) - genetically-based minority immune system disorders - which affects the granulocytes, the cells that are essential to eliminate microbes.
Neutrocytes or phagocytes are cells that usually destroy bacteria, but with this disease they are incapable of eliminating them. With this disease, the phagocytes look for reinforcement from the surrounding cells, creating a “ball” (called a granuloma) to try to contain the infection.
With a specific blood test:
It can also cause:
Autoinflammatory syndromes are a group of conditions characterised by spontaneous, recurring or persistent episodes of multi-systemic inflammation. They are caused by changes to innate immunity that cause deregulation of the immune system. Autoinflammatory conditions, due to various genetic mutations, cause a pathological hyperactivity in this structure, which unleashes abnormal, continuous inflammatory activity. The number of conditions the group includes has increased since then, due to the advances in genetics and immunology.
The main symptom of many of the conditions included in the group is repeated episodes of fever, which spontaneously disappear after a few days, only to reappear again cyclically after a variable period of time. This fever is not caused by an infection and, therefore, does not respond to treatment with antibiotics or antiviral medication. Depending on the genetic defect, these conditions may be associated with a wide diversity of other manifestations, including skin, abdominal, joints, eyes or lungs.
All the conditions within the group are infrequent and have an incidence of less than 5 cases per 10,000 inhabitants, for which reason they are considered to be rare conditions. The majority appear in infancy or adolescence.
Recent progress with research has clearly shown that some fevers where the cause is not found are provoked by a genetic defect.
Depending on whether or not they have a genetic cause, they can be classified as follows:
The diagnosis is based on the clinical features of each patient’s clinical picture. Blood tests are important in diagnosing the various autoinflammatory conditions, as they enable detection of the existence of inflammation. These analyses are repeated when the child is asymptomatic to see if they have normalised. Molecular or genetic analysis enables detection of the presence of mutations involved in the development of autoinflammatory conditions which are studied in patients suspected of suffering from them according to the features of the clinical picture. The diagnosis is confirmed when the patient shows evidence of being a mutation carrier and it is often necessary to study family members too.
Treatment fundamentally depends on the type of condition and the response to the therapy chosen. For example, for familial Mediterranean fever, the treatment of choice is colchicine. Other treatments used on the various autoinflammatory conditions are cytokine inhibitors, such as IL-1 or the tumour necrosis factor α. Close monitoring of the patient is essential to prevent complications arising in the long term.
Informació pràctica com a CSUR de malalties autoinflamatòries
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:
The coronavirus SARS-CoV-2 is a virus known as acute respiratory syndrome coronavirus 2 that was first observed in Wuhan (Hubei, China) in December 2019. This new virus is the cause of an infectious disease, known as COVID- 19, which causes respiratory infections to people. In most cases, eight out of ten, the symptoms are mild.
It is important to contact 061 in case of fever, cough, shortness of breath and if you have traveled or have been in contact with a person from the highest risk areas. The World Health Organization (WHO) has declared the SARS-CoV-2 coronavirus as an international public health crisis.
Coronavirus is a family of viruses that circulates among animals. Some types of coronaviruses can also affect people, causing respiratory infections, such as the coronavirus SARS-CoV-2.
In 80% of cases, the symptoms are mild and can be confused with those of a flu:
These symptoms may appear gradually accompanied by nasal congestion or sore throat. Moderate cases may be accompanied by a feeling of shortness of breath and, in the most severe, the infection causes more severe complications, such as pneumonia.
According to current data, there are people who have become infected but have not developed any symptoms or are ill. Although in most cases the symptoms are mild, some people, with a more severe prognosis, have died.
The SARS-CoV-2 coronavirus can infect anyone, regardless of their age. Even so, two groups with greater risk have been detected:
The risk of infection is higher in those areas where there are cases of SARS-CoV-2 coronavirus diagnosed. Therefore, everyone needs to take protective measures, such as maintaining good hand hygiene or covering their mouths with their elbows or with a tissue when coughing.
Studies conducted so far suggest that the SARS-CoV-2 coronavirus is transmitted by air, from person to person, through droplets from the nose or mouth that are spread when an infected person coughs or exhales. Contagion occurs when these droplets are exhaled by a healthy person or when they fall on an object or surface that the person subsequently touches and then, without disinfecting the hands, touches the eyes, nose or mouth.
Between infection with the virus and the appearance of the first symptoms of the ailment, it is estimated that there may be an incubation period of between one and fourteen days. On average it is estimated that this is five days.
The diagnosis is made through a specific COVID-19 detection test.
Currently, there is no specific treatment for SARS-CoV-2 coronavirus, only supportive treatment. In milder cases, the treatment is similar to the flu: pain relievers to control fever and stay properly hydrated.
In the most severe cases, if the patient requires ventilatory support, due to pneumonia or respiratory failure, the patient is admitted to the ICU.
In addition, clinical trials are being conducted to find a specific vaccine or drug treatment to predict or treat COVID-19.
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