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.
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Psoriasis is a non-infectious inflammatory skin condition. It is chronic and shows up as pinky-red skin lesions covered with scaly silvery-white dead skin cells. It normally appears between 15-35 years old but can do so at any age.
The cause of this disease is an alteration of the immune system that causes an inflammatory chain reaction in the body’s defence mechanisms that results in excessive production of skin cells.
It appears as pinky-red patches covered with silvery-white scaly skin. It mostly appears on elbows, knees, the lower back and the scalp, but can also occur on other parts of the body.
Up to a third of people with psoriasis may go on to develop joint problems, or psoriatic arthritis, characterized by inflammation of the joints. This is generally intermittent and asymmetric and mainly occurs in the fingers, toes and lower spine.
It most commonly appears as skin lesions, which can sometimes itch or become sore, especially if the skin cracks or is broken. There are different types of psoriasis:
Aside from skin lesions, people with psoriasis may develop psoriatic arthritis, which occurs as pain, heat and reddening around the joint and being unable to move the joint. In its advanced stages, there may be deformities, pain in the heels and back pain.
It affects 2-3% of the world population. 10-30% of sufferers develop arthritis, which can occur at any time, although it is more likely between 30-50 years old.
Diagnosis is by observation of the lesions and the areas around them. Specialists may sometimes perform a skin biopsy to confirm diagnosis and to rule out other conditions that may appear similar or have the same symptoms.
There are currently different treatments to alleviate symptoms and signs, and which also cure the skin lesions in most cases. The dermatology specialist will decide the most suitable treatment for each patient, depending on the type of psoriasis, where it is on the body, the severity, and type of patient.
There are three types of treatment:
Oral herpes is an infection in the lips, mouth and gums caused by the herpes simplex virus (HSV-1) and which shows up as small painful blisters called herpes labialis, commonly known as cold sores. The herpes simplex infection is very contagious, common and endemic throughout the world. It is normally acquired in childhood and lasts a lifetime.
Herpes caused by the HVS-1 virus is spread by mouth-to-mouth or skin contact with ulcers or saliva and the area around the mouth and lips. It can also be spread to the genitals, resulting in genital herpes.
Although uncommon, it can be transmitted from an infected mother to her baby during birth.
Usually, herpes labialis (or the cold sore virus) is asymptomatic and most people infected do not realise. When it appears, it does so as painful blisters or ulcers on or around the mouth. People with this condition notice a feeling of stinging, tingling or burning in the affected area.
After the first infection, the blisters may periodically reappear, varying from person to person.
According to the WHO, 67 % of the population is infected with HSV-1.
Diagnosis is done in a medical centre, in other words, through examination of the patient. If there is any doubt, the specialist may request virological culture tests on the blisters during the initial stages of the disease to confirm it.
Antiviral medications such as aciclovir, famciclovir and valaciclovir are the most effective to treat those infected with HSV-1. However, despite reducing the intensity and frequency of symptoms, they do not cure the infection.
Eczema is a skin condition characterised by the appearance of small vesicles and which exudes fluid. Flaking skin produced by the disease can cause itching, inflammation and pain. It is the skin’s inflammatory response to external or internal stimuli and there are two types: endogenous (or atopic), and exogenous (or contact). Often called dermatitis, eczema has different clinical presentations and may have several different causes.
The existence of other diseases, allergies, contact with irritants and genetic inheritance are some of the causes of eczema.
Endogenous or atopic eczema is an atopic disease typically found in patients with rhinitis, conjunctivitis, asthma and dermatitis, and usually presenting as hypersensitive dry skin. It is often related to allergic reactions or external stimuli such as exposure to pollen, dust, fur, and urticaria, viral infections and bacterial skin infections.
Exogenous or contact eczema is the allergic or irritative reaction to chemical substances that have come into contact with the skin and that the body interprets as toxic.
Eczema shows up as skin lesions made up of itchy scaly red patches on different parts of the body. Sometimes there may be an inflammatory reaction in the area of the outbreak that may give rise to serum-filled blisters.
Figures show that around 30% of patients with eczema have a family history of atopic disease in the family. Atopic eczema can appear when a child is just a few months old and in these cases it does so on the scalp, face and nappy area. It usually disappears during puberty, leaving the skin dry, and in some cases, signs of atopy such as urticaria or asthma.
Contact eczema appears in patients sensitive to a particular substance, called an allergen. The affected person will have a skin reaction each time they are exposed to this substance.
Suspected diagnosis of each type of eczema must be carried out through studying the patient's medical history. In most cases, diagnosis is clinical, in other words, the dermatologist will diagnose the condition after examining the patches on the skin. If there is any doubt, a skin biopsy can confirm diagnosis.
For contact eczema, a patch test will be needed to determine the allergen responsible for the patient’s patches of eczema.
Haemophilia is a very rare congenital disorder resulting from a lack of clotting factor VIII (haemophilia A) or IX (haemophilia B). Type A affects 1 in 5,000 children and type B affects 1 in 30,000. The gene whose code carries instructions for building the proteins for these factors is located on the X chromosome. For this reason, women carry this genetic mutation but it affects men. Despite this, in most cases there is no family history of haemophilia.
Plasma levels of clotting factor VIII or IX determine the severity of this disorder and classify it as severe, moderate or mild depending on whether levels are below 1%, between 1 and 5%, or over 5%, respectively. Spontaneous haematoma during the first few months of life or caused by minor trauma are the key sign of the severe condition.
Although bleeding can be found in any part of the body, it typically occurs within a joint (haemarthrosis), mainly in the ankles, knees and elbows. It generally appears before two years of age and represents 70-80% of all haemorrhages. Repeated bleeding in the same joint ends up causing irreversible damage that affects function (haemophiliac arthropathy). Intramuscular haemorrhaging is the second most common after haemarthrosis, and intracranial bleeding the most serious.
In moderate cases, clinical signs are similar to those of the severe disorder, apart from spontaneous haemorrhaging; and in mild cases diagnosis is reached by spotting an anomaly in blood clotting tests, by bleeding after a tooth extraction or surgery, or following a familial study.
A suspected diagnosis begins by asking about a patient's personal and family history of bleeding. Basic blood clotting tests show increased time for the blood to clot (activated partial thromboplastin time) and it is confirmed by verifying low levels of clotting factors VIII or IX. Genetic testing refines the diagnosis by identifying the mutation causing the disorder. This procedure can also identify potential carriers and may be used for prenatal diagnosis.
Treatment is replacement therapy, which is the intravenous administration of the deficient factor in the case of acute bleeding, before any aggressive exploratory or surgical procedures take place. Factor VIII and IX concentrates may be human plasma or recombinant engineered using biotechnology. In mild cases, other drugs may be used such as desmopressin, a synthetic derivative of vasopressin.
For cases of severe haemophilia, preventative treatment should be started before two years of age or after the first haemarthrosis in order to avoid serious complications in the joints producing repeated haemorrhaging, and also to act as a preventative treatment against brain haemorrhaging. For haemophilia A, factor VIII must be administered three times a week, and twice in the case of haemophilia B. New treatments being developed will allow infusion therapy to be more spread out in the future.
Plasma and recombinant factors currently effectively and safely control and prevent bleeding. The most serious complication of treatment is the possible appearance of an inhibitor. This appears in 30% of severe haemophilia A and in 2-4% of haemophilia B cases.
Because this is a complex and chronic condition, it is advisable to have a multidisciplinary team that includes specialists in haematology, hepatology, infectious diseases, orthopaedic surgery, physiotherapy and rehabilitation, odontology, obstetrics, genetics, psychology and nursing. Educational programmes to show family members how to administer intravenous treatment at home, prenatal diagnosis and genetic counselling are essential.
Parkinson's disease is a dysfunction of the basal ganglia caused by degeneration of the cells that produce dopamine in the substantia nigra.
It is a progressive neurodegenerative disease of the central nervous system that affects the parts of the brain involved in controlling and coordinating movement, muscle tone and posture.
The prevalence of Parkinson’s in Catalonia is 229 in every 100,000 people.
This is focused on empowering patients and their carers to achieve behavioural changes within their own control and to motivate them to continue treatment long term. It centres on reducing medication and gaining quality of movement. The main goal is functional independence for the individual and general physical condition from the onset of the disease. It is all geared towards minimising secondary complications and the risk of falls.
There are a growing number of studies emphasising that aerobic activity may have a neuro-protective effect. Likewise, during treatment, preventing inactivity, falling and fear of getting around or falling is stressed.
Neuromuscular disease is a chronic illness that results in serious disability, loss of independence, and with significant psychosocial consequences. Respiratory alterations are the main cause of morbidity and mortality in patients with neuromuscular diseases. They are significantly affected by the evolution of the disease and are the reason for multiple hospital admissions where the patient’s life is seriously endangered.
The main causes of respiratory impairment are hypoventilation due to weak inspiratory muscles and a lack of ability to cough due to weak expiratory muscles. Ventilatory support via non-invasive mechanical ventilation or tracheotomy can prevent or reverse ventilatory failure in these patients.
The loss of expiratory strength means that patients are unable to expel bronchial secretions. If the bulbar muscles are also affected and patients run the risk of inhaling saliva, the contents of the mouth or food, this can induce multiple respiratory infections, pneumonia and atelectasis which results in obstruction of the airway and seriously endangers the patient's life.
The combination of non-invasive mechanical ventilation to assist coughing decreases morbidity and hospital admissions for these patients.
There are currently around 60,000 people with the condition in Spain.
In the Cardiorespiratory Rehabilitation Unit, we monitor maximal inspiratory and expiratory pressure (MIP and MEP) and peak expiratory flow (PEF), also known as peak cough flow (PCF) and carry out spirometry.
Treatment goals are focused on controlling the evolution of the ventilatory failure and avoiding or improving episodes of respiratory failure. To achieve these objectives, manual techniques or equipment have to be used. These are techniques to encourage pulmonary expansion, manually assist coughing, and others.
One very important objective is to train the main carer in physiotherapy techniques in order to avoid possible complications in the respiratory system.
Patients have very serious damage to the ocular surface (the cornea, conjunctiva and eyelids) generally caused by chemical burns or an inflammatory disease of the ocular surface such as cicatricial pemphigoid or Lyell’s syndrome.
These disorders of the ocular surface may result in very low visual acuity, irritation and pain. They are conditions significantly affecting the cornea, the conjunctiva and the eyelids; organs essential to maintaining a healthy ocular surface and therefore good vision.
Loss of vision, pain, severe dry eye syndrome, corneal damage and ulcers, infections, and even loss of the eyeball.
Diagnosis is essentially clinical. Changes in the ocular surface may present as corneal ulcers, corneal vascularization or conjunctivalization (normally the cornea tissue has no blood vessels) or due to symblepharon, which are scars that form between the conjunctiva and the eyelids.
Treatments are multiple and spread out, depending on how severely the ocular surface is affected. Natural tear substitutes are used, such as autologous serum or plasma rich in platelets. Surgical options that may be carried out are amniotic membrane grafts, corneal transplants, limbal stem cell transplant (autologous or heterogeneous), eyelid transplant, and in very severe cases, keratoprosthesis.
There is no way to prevent this serious condition of the ocular surface, but early diagnosis and treatment can limit the damage it causes.
Lupus is a chronic immunological disease characterised by the production of antibodies. It mainly affects women of child bearing age, evolves into flare-ups and can affect any organ.
It is characterised by the production of immune complexes found on any organ and that cause inflammation and, in some cases, even organ damage. The cause is unknown but is understood to be down to multiple factors. Genetic, environmental and hormonal factors play a role.
These cause a change to apopstosis (cell death) that means new antigens appear and the innate and the adaptive systems are activated, which are responsible for producing antibodies.
The most common symptoms are: joint pain or arthritis (85-90 %) and skin lesions (70 %), but it may also affect any organ.
Lupus can affect different parts of the body:
Lupus mainly affects women, with a ratio of 9:1 of those affected being of childbearing age, although it may appear during childhood or later in life.
Its is prevalent in 10/10,000 people of Caucasian origin, but is more prevalent and severe among African American and Hispanic patients.
With improved treatment in developed countries, the survival rate is over 90% after 20 years, although the disease’s effects on the renal and central nervous system increase morbidity and mortality.
Diagnosis is founded on clinical suspicion based on the symptoms described and laboratory data showing the presence of antinuclear antibodies and anti-dsDNA antibodies, which are specific to the disease. The criteria provided by the American College of Rheumatology is used for diagnosis. If the patient fulfils 4 of the 11 criteria, they are classified as having systemic lupus erythematosus.
Treatment is specific to each case. In general, anti-inflammatories, cortisone and antimalarial drugs are used. In severe cases, immunosuppressants may be used (Imurel®, methotrexate and mycophenolate, for example), or to avoid flare ups, lasting effects and also reduce the need for corticosteroids. In the last 50 years, only belimumab, or anti-BLys, has been approved for the treatment of lupus.
Unfortunately there is no preventative treatment for lupus. The most important factor to avoid lasting damage is early diagnosis and treatment by experts in the field.
Providing patients with the right information at the time of diagnosis is essential to prevent future complications, as is treatment monitoring.
It is a congenital heart defect characterised by the tricuspid valve sitting lower than normal over the ventricular myocardium, caused by the corresponding atrioventricular ring. In this case, the right atrium greatly increases its volume and the right ventricle greatly reduces in size and lung flow is not sufficient.
Ebstein’s anomaly encompasses a wide range of defects characterised by different grades of displacement and adherence of the septal valve of the tricuspid valve to the right ventricle chamber. It is a very variable disease that may be severe or mild.
The most serious cases are accompanied by severe cyanosis and congestive heart failure.
In less serious cases, the disease results in a transient period of cyanosis.
This is considered a rare disease and only represents 3% of congenital heart defects. It affects one in every 20,000 live births.
An echocardiogram is used to detect the disease, and to find out if it is associated with anatomical or functional atresia of the pulmonary valve.
The defect is treated with surgery which varies depending on the clinic, the anatomical form of the defect and the age of the patient.
In the case of new-born babies, for example, it is necessary to operate as soon possible. In adolescents or adults with symptoms, it is advisable to repair or replace the tricuspid valve with a prosthetic. If patients have no symptoms the best option is to wait.
Following surgery, 80% of patients diagnosed as teenagers or adults have a very good short to mid term prognosis as their ability to function has improved.
Pulmonary stenosis is a disorder of the heart valve that affects the pulmonary valve, the valve which separates the right ventricle from the pulmonary artery, which is the artery that transports blood to the lungs. Pulmonary stenosis occurs when the valve is unable to open sufficiently and as a result there is less blood flow to the lungs.
Mild stenosis produces no symptoms.
If it is more severe, symptoms may be:
An echocardiogram is the usual way to diagnose the condition. This test assesses the right ventricle, the pulmonary valve, post-stenotic dilatation of the pulmonary artery and pressure gradients through the valve.
Percutaneous pulmonary valvuloplasty is used to treat pulmonary stenosis, and is suitable for patients with a moderate condition over two years old.
In severe cases, percutaneous pulmonary valvuloplasty can be carried out at any age. This procedure is associated with a lower short term morbidity and mortality rate than surgical valvotomy. The procedures have similar long term outcomes.
Valvuloplasty generally attains excellent results. At a 15-year check up, only 4 % of cases need a second procedure. Mild pulmonary valvular insufficiency is well tolerated.
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