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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Hereditary metabolic diseases (HMDs) are a group of rare genetic disorders. The genetic defect causes a structural alteration in a protein that is involved in one of the metabolic pathways, causing it to block the affected pathway. As a consequence, this causes a build up of substances that may be toxic for the body and a deficiency of others that it needs.
Hereditary metabolic diseases (HMD) are chronic progressive multi-system illnesses that may appear at any age and that in most cases pose diagnostic and therapeutic challenges. Our Unit has been recognised as a leader within Spain (CSUR) and Europe (ERN) for this pathology and takes part in the neonatal screening programme in Catalonia. We are the only centre in Catalonia to offer complete care from paediatrics to adults with particular expertise in lysosomal storage disorders.
HMDs are divided into:
- Intermediary metabolism HMD: usually with acute symptoms.
- HMD related to the organelles (lysosomal storage disorders, peroxisomal diseases, mitochondrial disorders and endoplasmic reticulum storage diseases): chronic presentation with no decompensations (with the exception of some mitochondrial disorders)
Multiple systems in the body are affected and different organs and systems are involved with varying symptoms depending on the disorder and the patient’s age. These disorders require a coordinated approach to care and programmes to manage the transition to adulthood.
Many symptoms become evident during childhood in the form of delayed physical growth and delayed psychomotor development. There may be associated heart problems, kidney conditions, and at times decompensations leading to liver or kidney failure and neurological impairment. In the case of organelle disorders, symptoms are chronic and affect the bones and organs of the senses in greater measure. They are more common in adults than intermediary metabolism disorders.
Diagnosis is carried out by:
They are chronic disorders that need to be treated in specialised centres with multidisciplinary teams to provide support for all related health problems.
The following may be necessary, depending on the type of disorder:
Prevention consists of thorough genetic and reproductive counselling if there is a family history of the disease. Early diagnosis of some diseases through the neonatal screening programme enables effective treatment and improved prognosis.
Alzheimer’s disease (AD) is the most common neurodegenerative disease worldwide. First described in 1906, it was known for years as senile dementia, but today we know that most cases of senile dementia are AD. WHO data states that it affects over 50 million people worldwide and this is set to triple by 2050. It is the main cause of disability in the elderly and the second specific cause of death in Spain.
In certain areas of the brain of someone with Alzheimer’s, two proteins (amyloid-beta and tau) are progressively produced over several years, forming deposits that eventually damage and destroy the neurons, leading to the progressive loss of higher-level cognitive brain functions such as: memory, language (aphasia), the ability to perform learned motor functions (apraxia), and to recognise different sensory stimuli (agnosia), reasoning and judgement, and changes in mood, behaviour and personality. Although the etiology of the disease is unknown, we do know of many factors that contribute to its appearance.
AD manifests in various ways. The signs and symptoms are specific to each individual and the characteristics of how the dementia develops will be different for each person.
Most patients (85% of cases) present the typical form (amnestic or hippocampus), which starts with the symptom of episodic progressive memory loss in relation to recent events and difficult taking in new information, and thereby losing the ability to adapt to new situations. Discrete constructional apraxia. Loss of fluidity of speech with normal comprehension. Early and persistent depression, anxiety or apathy (most common), with a substantial decline in initiative, motivation and interest, and with indifference and passivity.
In the mid stages, the disease presents loss of remote memory. Temporal and spatial disorientation. Ideomotor and ideational apraxia occur as well as constructional apraxia. Speech continues to worsen and comprehension issues are added to the loss of fluidity and anomia. Visual and body image agnosia (somatagnosia) develops. The mid stages are when sleep and psychiatric disorders are most evident, including becoming agitated at night, being restless, delirium (being unable to distinguish what is reality: delusional jealousy, confusing TV programmes with real life) and hallucinations (false sensory perceptions: hearing voices, seeing insects).
In the late stages, patients present severe agnosia, a loss of bladder and bowel control, become mute or almost mute, and present motor function alternations such as overall stiffness and a stooped posture. Approximately 10% present epileptic seizures. All patients show obvious weight loss during this final stage.
In around 15% of patients with AD, memory may be relatively preserved until the late stages. These are atypical forms or variants (without memory loss in the early stages) which may present in three forms: with behavioural or personality changes, with visuospatial alterations, or with changes to language as the earliest and predominant symptom of the disease. As it progresses, the other symptoms described as the typical form of the disease also appears. These atypical forms of AD are more common in cases where the onset occurs at a younger age.
Daily activities (DA) are progressively affected: first there is a reduction in work and social activities (advanced daily activities); followed by changes to everyday activities (handling domestic objects, money, cooking, housework), and in the late stages, basic daily activities are affected (washing, dressing, eating, bladder and bowel control). In the final stage, patients enter a vegetative state and die as the result of an intercurrent illness: the time from diagnosis to death is usually around » 5-10 years.
The prevalence and incidence of the disease increases after 65 years of age. It therefore affects 5% of the population over 60, 20% of those over 80 and 30% of those over 90. In Spain there are 800,000 people with the condition. The real figure is undoubtedly much higher, however, as the first symptoms are sometimes difficult to distinguish from those that naturally appear with age. For this reason it is an underdiagnosed disease, with around 1 in 3 people with AD believed to be undiagnosed.
Fewer than 5% of cases of AD are hereditary. This is known as familial or inherited AD and occurs through autosomal dominant inheritance. The clinical picture includes an earlier onset of the condition (before 65 years old) and a faster evolution.
The remaining 95% (sporadic AD) present the combination of risk factors for the development of the disease together with genetic alterations, together making the patient susceptible to the disease.
Apart from genetic factors, other risk factors for developing the disease are: ageing; gender (from 65 it is more common in women); vascular risk factors such as high blood pressure, diabetes or obesity; lifestyle (smoking, alcohol, lack of physical activity, lack of intellectual activity, little social interaction); previous head injuries, and chronic sleep disorders. People with Down syndrome (trisomy 21) have an extra copy of the gene that encodes the amyloid precursor protein (APP) making them more susceptible to AD at a younger age. Chronic sleep problems increase the risk of AD. Interrupted sleep increases levels of the amyloid-beta and tau protein.
Due to the fact that pathological alterations (amyloid deposit following tau) begin in the brain 15-20 years before symptoms appear, there are currently considered to be 3 stages of the disease:
Although there is currently no cure for the disease, there are treatments that can delay or slow the progression of the disease for a time, improving quality of life for these people. Drug treatments are: cholinesterase inhibitors (rivastigmine donepezil, galantamine) that act to facilitate cholinergic neurotransmission and are licensed for the symptomatic treatment of light or moderate AD, and memantine, a non-competitive glutamatergic NMDA receptor antagonist, which decreases levels of glutamate (an excitotoxin that destroys neurons when released chronically and in excess) and is licensed for the mid and late stages of the disease.
In addition to these treatments, proper management of lifestyle factors is very important, such as: correcting any hearing loss, reducing smoking and drinking, proper management of blood pressure and diabetes, a balanced diet, avoiding obesity, doing regular physical activity, preserving and encouraging social contact. Together with the above, cognitive stimulation is useful during a large part of the progression of the disease.
There are currently 400 studies assessing the efficacy and safety of different treatments in patients with AD.
Known preventions strategies work on the risk factors for the disease: healthy habits, controlling vascular risks (high blood pressure, diabetes, etc.), a higher level of education, changes to lifestyle (essentially increasing physical activity) giving up toxic habits (smoking and drinking). All of the above can reduce cases of AD by 35-40%, or at least delay its onset.
Education and mental activity stimulate the connections in the brain and increase the cerebral reserve capacity, so it is very important to remain mentally active.
The Dementia Unit in the Neurology department is in charge of diagnosing and looking after patients with Alzheimer’s. The unit includes neurologists with expertise in diagnosing and managing the different pathologies that can occur with dementia (changes to cognitive and behavioural functions that result in changes to daily life) as the main manifestation. Neuropsychologists, nurses and social services and healthcare staff also play a very important role in the Unit.
Other units and departments involved in the diagnosis and monitoring of these patients are: Primary Care, Nuclear Medicine, Neuroradiology, Psychiatry, Pathological Anatomy, Genetics.
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.
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