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.
Asthma is a disorder of the passage of air through the respiratory tract, particularly in small-calibre bronchial tubes. It causes difficulty breathing and the patient feels like they are drowning and must increase their effort in an attempt to breathe better.
Asthma is a chronic inflammatory disorder of the airways that results in variable airflow obstruction. It often changes throughout the day (it can get worse at night) and improves with treatment and then reappears later.
The illness is basically caused by an inflammatory mechanism.
Three phenomena occur in the airways of patients with asthma:
1. Decreased bronchial diameter, which restricts air flow.
2. Inflammation, with increased thickness of the bronchial wall, which also contributes to restricting air flow.
3. Increased activity in the glands that produce mucus, with increased secretions contributing further to breathing difficulty.
Cells that circulate in the blood are involved in the local inflammation observed in asthma: T lymphocytes, mast cells and eosinophils. These cells are responsible for the body’s normal defence and their activity is increased in asthma. Medication for asthma attempts to regulate this activity.
According to the degree of restriction, the person affected will experience breathing difficulty and a sensation of lack of air. Also characteristic of asthma are wheezing, which is the sharp whistle-like sound of the air as it passes through the smaller airways, and increased bronchial secretion.
Asthma can affect all age groups and sometimes overlaps with bronchitis. On many occasions it can be allergic in origin or come as a result of exposure to an environmental or chemical agent.
Spirometry, chest x-ray, allergy tests.
Some basic questions for diagnosis:
1) Have you ever had a whistling sound in your chest?
2) Have you been coughing, especially at night?
3) Have you had a cough, whistling sound, difficulty breathing at some times of the year or in contact with animals, plants, tobacco or whilst at work or after exercise?
4) Have you had colds that last more than 10 days or are "chesty"?
5) Have you used inhaled medication?
Treatment is based on using bronchodilators, in the form of an inhaler or tablets. Anti-inflammatory drugs also have an important role.
The most common diagnostic tests for asthma are based on:
1) Spirometry: Measures air flow on inhaling and exhaling and detects any restrictions in the airway, one of the characteristics of asthma.
2) Bronchodilator test: Tests if spirometry improves with drugs to dilate the airway.
3) Bronchial challenge test, the same test in the opposite direction with drugs that cause a slight airway obstruction, detected by spirometry.
To prevent asthma, it is fundamental not to smoke and avoid exposure to allergens that precipitate it, which are detected with the allergy tests that form part of the asthma exam.
A procedure that, by introducing a flexible tube (bronchoscope) into the nose or mouth, allows the bronchial tree to be viewed, for diagnostic and/or therapeutic purposes.
To examine the bronchial tree and obtain samples of secretions or tissues for analysis with the aim of gaining an aetiological diagnosis of the causative illness. It can also be a therapeutic test, allowing suction of secretions or clots, extraction of foreign bodies, permeability of the airway in lung tumours and treatment of complications resulting from lung transplant.
With the patient normally lying down and consciously sedated, the bronchoscope is introduced into the airway, administering local anaesthesia in the passageways (larynx, trachea and bronchi). After examining all the bronchi and identifying any possible lesions, samples are taken, which may include: bronchial aspiration, bronchoalveolar lavage, bronchial brushing, transbronchial puncture, bronchial biopsy or transbronchial biopsy.
Minor undesirable affects may appear, such as snoring, cough, fever, localised pain, nausea or sickness and coughing up small amounts of blood, which are usually self-limiting and present no risk to life. Less commonly, major complications may occur, such as haemorrhage, low blood pressure, high blood pressure, pneumothorax (entry of air into the thorax outside of the lung). In very rare cases, complications such as arrhythmia or arrest of the heart, respiratory depression or arrest and acute stroke, may be severe and require medical or surgical treatment, including a small risk of death.
Rigid bronchoscopy, CT-guided needle lung biopsy, mediastinoscopy, surgical lung biopsy.
Simple spirometry is a test to study lung function, which lets us know the amount of air patients can move and how well they do it.
This test is done using a device called a spirometer. The patient must be sat upright with their legs uncrossed. Then, clamps are placed on the nose and the patient is asked to insert a nozzle into their mouth. Once ready, the patient must fill their lungs by breathing in as deeply as they can. The care worker then asks the patient to blow as hard as they can, not stopping until their lungs are empty. The blowing stops when the healthcare professional in charge tells the patient to stop.
Next, the patient is asked to breathe in quickly as hard as they can in order to record their inhalation data.
This can be repeated until three correct readings are taken. Normally the test lasts 10 minutes.
The only way of effectively curing tuberculosis and preventing others from catching it is through treatment based on combining different antibiotics for a minimum of six months. The symptoms of tuberculosis often disappear quickly, but the disease may come back if medication is not taken correctly and for the time indicated.
Currently, tuberculosis (TB) drugs are safe and effective, and the majority of people take them without any problems. In some cases side effects may appear, so it is important to follow the treatment plan under supervision and see a doctor in case of doubt.
Once treatment begins, and to make sure it has the desired result, follow the instructions below:
Urine, faeces, sweat and tears may be red/orange in colour. This change is normal and will disappear when the treatment comes to an end.
CDC - Preguntes i respostes sobre la tuberculosis
Amyotrophic lateral sclerosis (ALS) causes muscular degeneration that can affect motor autonomy, oral communication, swallowing and breathing, but the senses, intellect and eyes muscles remain intact. It can therefore affect the respiratory muscles, which is why respiratory care is essential for patients’ quality of life.
In order to improve the respiratory difficulties in patients, ventilation therapy can be used through non-invasive ventilation.
Ventilation therapy refers to breathing support using a ventilator, usually at night during sleep, to achieve:
Ventilation is carried out non-invasively, by means of a patient-adjusted mask (nasal or full face) connected by a tube to the ventilator or respirator.
When patients need this therapy, the place and time it is started, whether outpatient or hospital admission, is planned in a personalised way with the consent of the patient and the person caring for them.
Education for the patient and their main carer should begin as soon as possible, both from the point of view of managing secretions and the resulting care, as well as the emotional support they need to receive. This means that during the patient’s admission or outpatient visit, the patient and their carer will be trained in:
The patient and the carer must take care to keep the airway in good condition to allow secretions to be managed. It is important to preserve the ability to cough where possible, but if coughing is no longer effective, the patient and carer will need to start learning how to use mechanical aids (cough assist or mechanically assisted cough). In certain cases secretion suction may also be used.
To improve the quality of life of patients it is important to follow the advice below:
Pneumonia is an infection of the lung tissue.
Depending on the extent of pneumonia in the respiratory tract, different types are identified:
It can be caused by many different microorganisms, although the most common causes are S. pneumoniae (pneumococcus) and Mycoplasma.
Other microorganisms that can also cause pneumonia include Haemophilus, Klebsiella, Staphylococcus aureus, Legionella pneumophila, Chlamydia pneumoniae and some viruses.
Characterised by high fever, coughing, with or without sputum, and often chest pain, which may increase with respiratory movements. Sometimes sputum has a brownish or rusty appearance, which points to pneumonia caused by pneumococcus.
The so-called atypical pneumonia, caused by Mycoplasma or Chlamydia among others, is often characterised by fever with very few respiratory symptoms.
Pneumonia is a very common disease (350,000 cases/year in Spain) and is a significant cause of mortality in the general population. It can affect all age groups.
In previously healthy people it is a disease of mild or moderate severity. It can even be treated at home or in outpatient care, but in patients with previous pathology (immunocompromised, heart failure, previous respiratory failure), it is generally serious.
The appropriate use of antibiotics, together with occasional respiratory support measures (oxygen therapy or even intubation), contributes significantly to improving the chances of cure in the most severe cases.
It is performed based on the patient's clinical history (age, previous pathology, evolution time and type of symptoms), auscultation, chest radiography and blood and sputum cultures to identify the causative organism.
Antigens can also be detected in urine for pneumococcus and Legionella.
The treatment is antibiotic, based on a clinical estimate of the possibility of it being caused by one germ or another (in many cases treatment is started immediately without knowing the causal organism). Treatment is later maintained or changed according to the cultures and the patient's evolution.
The criterion for inpatient or outpatient treatment depends on the estimation of the risks that may occur (older age, previous pathology, impairment of respiratory function).
In a previously healthy patient, treatment may be in outpatient care.
Chest x-ray, blood and sputum or respiratory secretion cultures and determination of antigens in urine.
Scleroderma is an autoimmune disorder characterised by increased collagen in various body tissues, structural alteration of microcirculation and certain immune abnormalities. The term scleroderma comes from the Greek “skleros”, which means hard, and “derma”, which means skin. This indicates that skin hardening is the most characteristic feature of the condition. As well as the skin, it can also affect the digestive tract, lungs, kidneys and heart. The prognosis varies. There is currently no cure, but the condition can be treated with general measures and treatment of symptoms, depending on the organs affected.
Raynaud syndrome: one of the most characteristic manifestations of the condition (97% of cases), it is the first clinical expression in most patients. It is caused by vasoconstriction of the capillaries. Patients report that with the cold their fingers change colour and turn pale (like wax) first, then turn blue after a while and finally turn reddish. The presence of Raynaud syndrome is not always an indication of scleroderma. In reality, only 5% of people with Raynaud syndrome later develop the condition. Almost half of sufferers may have digital ulcers, as an expression of a severe microcirculatory injury.
The most peculiar manifestation of the disease is the way it affects the skin. It is hard, tight and wrinkle-free (hard to pinch). The extent of the skin condition varies and is related to the prognosis. Two clinical forms are distinguished: limited (distal skin condition to elbows and knees) and diffuse (distal and proximal skin condition to elbows and knees, and torso). The face can be affected equally in both clinical forms. The limited subtype has a better prognosis than the diffuse one. Reduced aperture of the mouth (microstomy) may also be seen. In the skin there are hyperpigmented and coloured areas, telangiectasia (accumulation of small blood vessels) and sometimes subcutaneous calcium deposits can be felt (calcinosis).
Most patients experience joint and muscle pain, and in extreme cases contraction and retraction of the fingers are observed. When the digestive tract is affected, which often happens, the patient complains of a burning sensation and difficulty swallowing, as the oesophagus has lost its ability to move food towards the stomach. Pulmonary disease is the leading cause of death and may occur in the form of fibrosis or pulmonary hypertension; coughing, choking and heart failure are the main manifestations of lung involvement. When the heart is affected, heart rhythm disturbances and in some cases symptoms of angina pectoris are detected, due to the involvement of the small coronary vessels. In a small percentage (about 5%) scleroderma alters the kidney (scleroderma renal crisis) and manifests itself as malignant arterial hypertension and kidney failure.
It should be noted that not all patients with scleroderma present all the manifestations described above. It can also be concluded that there is great, almost individual, variability in the clinical expression of the disease.
Scleroderma is a rare disease with an incidence of 4-18.7/million/year and a prevalence of 31-286/million. It is more common in females, with a variable ratio, depending on the series, ranging from 3:1 to 14:1 (female/male). The age at which it presents is around 30-40 years.
When the above symptomatology is clear, the diagnosis does not offer too much room for doubt. Various complementary tests are helpful in confirming diagnosis and in assessing the degree of involvement of the various organs that may be affected.
“An incurable, but not untreatable condition”. There is currently no treatment for scleroderma that has satisfactory results, but this does not mean that it cannot be treated. Treatment is symptomatic, depending on the organ affected. For Raynaud syndrome: vasodilators, antiplatelets; gastro-oesophageal reflux: proton pump inhibitors; renal crisis: angiotensin converting enzyme inhibitors/dialysis; pulmonary fibrosis: immunosuppressants/lung transplant; pulmonary hypertension: vasodilators/lung transplant. In patients with the diffuse form and less than three years of evolution, immune modulators such as mycophenolate sodium (or mycophenolate mofetil) or methotrexate may be indicated as a basic treatment.
The most common tests to confirm and/or assess the degree of involvement of the various organs are: general analyses and immunological data (specific antinuclear antibodies); capillaroscopy, high-resolution computerised axial tomography scan of the chest, respiratory functional tests, oesophageal manometry and echocardiogram. In the follow-up for these patients, respiratory functional tests and an echocardiogram should be performed annually.
Chronic obstructive pulmonary disease or COPD is a respiratory disease that leads to obstruction of airways. The main symptoms are coughing, hawking and difficulty breathing, requiring particular effort. Although it can be due to other reasons, it is mainly caused by exposure to tobacco smoke. The main treatment is bronchodilators administered using an inhaler.
COPD is a respiratory disease that mainly appears in smokers or ex-smokers and causes the airway to become obstructed or blocked.
The main symptoms are coughing, hawking and difficulty breathing, requiring particular effort. Patients with COPD can also present with infection or worsening of symptoms, known as exacerbation.
The illness mainly affects smokers and ex-smokers. It is also associated with exposure to other sources of smoke, such as biomass smoke. In a small proportion of cases it may be due to genetic causes. The prevalence of COPD is up to 10% of the adult population aged over 40 in Spain.
Diagnosis of COPD is confirmed using a respiratory test: spirometry. This is a very simple test that can be conducted in a primary care centre (CAP). This test should be performed on all individuals over the age of 40 with a history of smoking who have respiratory symptoms such as breathlessness or coughing.
Typical treatment for COPD involves using bronchodilators administered via an inhaler. There are two different types of bronchodilator that may be administered together or separately, depending on each patient’s needs. The aim of this treatment is to reduce the sensation of breathlessness and the number of exacerbations and improve lung capacity. In some patients, administering corticosteroids via inhaler may also be necessary. The best treatment for COPD is stopping smoking.
As well as spirometry, other tests that may be required include chest x-ray, CAT scan, sputum culture or other more complete breathing tests. A blood test is normally performed to rule out genetic causes.
As tobacco is the main risk factor, the best prevention for COPD is not smoking. Exposure to environmental pollution and passive smoking should also be avoided.
Lung cancer is the general name for neoplastic lung disease in which there is the presence of tumour cells. There are different types of lung cancer, but all of them share tobacco use as a risk factor. It is usually detected by the symptoms it causes, but it can also be an incidental finding in an examination conducted for a different reason.
Lung cancer originates when a set of cancer cells proliferates and produces a local compromise in the space occupied. These cells have a tendency to spread (metastasis) to other organs and, as their biological behaviour is completely abnormal, they produce atypical neurological, dermatological or endocrine signs. There are different types of lung cancer from a cell classification perspective, which require different treatments and prognosis. Lung cancer is always a serious illness, with an overall low survival rate estimated at 20% of patients after 5 years.
Research into this disease in the last few years has led to new treatment strategies, which in some cases cause the disease to go into remission for long periods.
90% of people will have symptoms caused by local tumour growth, including non-specific respiratory symptoms such as coughing and difficulty breathing, or in some cases coughing up blood.
There can also be a wide variety of symptoms: pleural effusion (presence of fluid in the pleura), involvement of the nerve roots that pass through the chest, skin disorders and endocrine disorders because the tumour may produce products that are similar to normal hormones.
It affects both sexes, with a predominance in males. Incidence of lung cancer in women has shown a very worrying increase in the last few years. Although it can be seen in people who have never smoked, a history of smoking is almost always found.
A suspected diagnosis will be made in the clinic and imaging tests will then be conducted in the following order: Chest x-ray, CAT, PET-CT to confirm the suspicion. The types of cells involved will then be ascertained through pleural tap or bronchoscopy. Final diagnosis is always reached by confirming the presence of tumour cells, which is done by the Pathological Anatomy Department.
Lung cancer treatment must be personalised. Surgery can play its role, both in diagnosis and in treatment, as well as radiotherapy, chemotherapy, immunotherapy and the use of biological drugs aimed at blocking certain cell receptor, which are different in each patient.
The typical tests for diagnosis are chest radiography, CAT, PET-CT, pleural aspiration/tap and bronchoscopy.
In order to prevent lung cancer, completely abstaining from tobacco use is essential. Exposure to certain environmental toxins specific to some working environments, such as arsenic, asbestos and chrome should also be avoided.
Amyotrophic Lateral Sclerosis (ALS) is the most common degenerative motor neurone disease in adults. It is also known as Charcot disease after the famous French neurologist Jean-Martin Charcot who discovered it in 1869. In North America, it is known as Lou Gherig’s disease in honour of a famous baseball player who died at 38 years old as a result of this disease.
Amyotrophic Lateral Sclerosis manifests in the form of progressive paralysis that affects most of the muscles in the diaphragm. The life expectancy is less than five years. In rare cases, longer survival times may be observed, especially if artificial ventilation devices are provided.
ALS is a neurodegenerative disease caused by the death of motor neurons in the brain and the spinal cord.
There are two types of motor neuron: upper and lower. The first are found in the motor cortex and establish connections with the lower motor neurons located in the brain stem and spinal cord, which innervate muscles. When the upper motor neurons die, spasticity, weakness and hyperreflexia appear.
When the lower motor neurons die, twitching, weakness and muscle atrophy occur. Other neuron populations can also be affected, such as the temporal and frontal behavioural and executive circuits.
Epidemiologically speaking, ALS has an incidence of 1.5-2 new cases a year per 100,000 people (3 new cases are diagnosed per day in Spain). The total number of cases (prevalence) is 2-5 per 100,000. According to this data, the total number of patient with ALS in Spain is approximately 4,000 cases. This is why it is included in the rare or minority disease group.
90% of cases of ALS are sporadic (no family history). Around 10% of ALS cases are familial, usually inherited as dominant traits. The incorporation of new molecular genetics techniques in the field of research has allowed more than 25 genes involved in ALS to be identified.
As a consequence of the continuous decrease in motor neurons, symptoms of the disease appear. These usually depend on the location of the motor neurons undergoing the most advanced processes of degeneration. In most patients (70%) the first symptom is loss of strength with muscular atrophy in the hands or clumsiness when walking, with frequent falls. In approximately 25% of patients, the first symptom is difficulty talking or swallowing, which indicates that degeneration of the bulbar motor neuron population is the most intense. There are also other possibilities for clinical presentation of this disease, although much less frequent: respiratory failure, weight loss or unexplained lack of energy (asthenia), cramps and twitches in the absence of muscle weakness, spasticity in legs, rapid mood changes or cognitive impairment.
In advanced phases, the disease can also paralyse the eye muscles. In the final stages of the disease, paralysis of the respiratory muscles leads to respiratory failure, which is often the cause of death.
The condition particularly affects people aged between 40 and 70. The incidence is greater in men (3:2.2 per 100,000) in sporadic forms. The age of first onset of symptoms reaches its peak between 58 and 63 years old in sporadic cases and between 47 and 52 years in familial forms. Incidence decreases markedly after the age of 80. The risk of suffering ALS is 1:400 for women and 1:350 for men.
The differing ways in which ALS manifests is one of the two reasons for a delay in suspected diagnosis of the disease, which can be up to 15 months. The other is that there is no test or biomarker to objectively confirm the diagnosis in the initial stages of the condition. A diagnosis of ALS is a diagnosis of exclusion, based on clinical criteria and conducting tests (MRI, clinical analysis, genetic tests, electromyography, EMTC, neuropsychological exam, nuclear medicine techniques and others) to rule out other illnesses with similar clinical findings. In most specialised ALS units, the disease diagnostic criteria used are the revised El Escorial criteria and the Awaji-shima criteria.
There is currently no medication that can cure or stop the disease. Riluzole and Edaravone are the only medications approved for ALS treatment, although their effect on survival is moderate (months).
The European (EFNS) and American (ANA) associations of neurology recommend that patients with ALS be treated in specialised centres, where possible in multidisciplinary units, so that they might be prepared for any complications. These units should offer solutions to control the symptoms, including the use of a feeding tube, control of saliva secretions, cough assist devices, respirators for mechanical ventilation, technology to improve the patient’s ability to move around and facilitate communication in patients who have lost the ability to speak.
These multidisciplinary units are the centres preferred by those running new drug trials.
The reality is that there is currently no effective treatment, although patients and their relatives often desperately search online for miracle drugs that might cure the condition. ALSuntangled, a group made up of 80 international experts in ALS, was born with the aim of protecting these patients from the numerous products advertised. It mission is to review the veracity and safety of the alternative treatments offered online that have not gone through the proper regulatory channels. It publishes its results in the official magazine for the disease and on its website.
Diagnostic imaging techniques (MRI, CAT, PET), electrophysiology (electromyography, EMTC, PESs), laboratory analysis (haematology, biochemistry, antibodies, hormones, enzymes, serology, genetics), respiratory functional tests, gasometry, pulse oximetry, overnight pulse oximetry, capnography, BMI, calorimetry, lumbar puncture, functional scale for the disease (ALS-FRS-R). A muscular biopsy may be required in exceptional cases. It is advisable to admit the patient in order to arrange for testing and offer them a report on discharge detailing the ALS diagnostic category and degree of functional repercussion (ALS-FRS-R).
Although various environmental risk factors have been suggested (geographic, occupational, dietary habits, proximity to electrical channels, contact with pesticides or other neurotoxins), there is no agreement on preventative measures to take.
In family forms, it is possible to offer genetic counselling to people with a desire for offspring.
During the natural course of the disease, complications often appear that may be prevented and treated. Among the most significant are malnutrition, respiratory failure, hypersalivation, spasticity, pain, loss of independent movement and communication, depression, anxiety, sleep disorders, bed sores, cognitive deficits and burden on carers.
The Multidisciplinary ALS Unit in the Neurology Department at Vall d’Hebron University Hospital is accredited by the Generalitat de Catalunya, Spanish Government (CSUR) and by the European Reference Network for Rare Neuromuscular Diseases (EURO-NMD).
Professionals from the following specialisms make up this unit: Case handling, nursing, social care, neurology, pneumology, rehabilitation, nutritional support, neuropsychology, physiotherapy, speech therapy, endoscopy, interventional radiology, technicians for increasing communication (UTAC).
The coordinator is Dr. Josep Gamez.
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