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To ensure that you are clearly informed about how this procedure will be carried out, we invite you to read this information carefully. Your doctor is available to provide you with any additional information you may require. What is enteral nutrition? Malnutrition is a condition in which the body no longer functions properly because it is not receiving sufficient essential nutrients (carbohydrates, fats and proteins). In some situations, it becomes necessary to provide enteral nutrition: you then receive liquid food directly into the stomach or the first part of the small intestine (the jejunum), via a thin, flexible tube.  What type of tube will be used? The choice of tube depends on your disease and the anticipated duration of enteral nutrition.In most cases, a tube inserted through the nose—called a nasogastric or nasojejunal tube—is sufficient to improve your nutritional status. When enteral nutrition is required over the long term, the tube is inserted directly into the stomach (gastrostomy) or the small intestine (jejunostomy) endoscopically at the Digestive Endoscopy Clinic. How to prepare for gastrostomy placement? The gastrostomy is performed during a gastroscopy. To arrange for the tube to be placed, you will be seen by the clinical nutrition nurse or the gastroenterologist to prepare for your procedure. To prepare for your procedure: Inform your doctor about your medical history and all the medicines you take, particularly those that affect blood clotting: aspirin, Plavix, Sintrom, Xarelto, Eliquis, Pradaxa, Lixiana, Innohep, Fraxiparine (the list is not exhaustive).Inform your doctor of any known allergies.Fast for at least 6 hours before the examination: do not eat, drink or smoke.Depending on your situation, the examination will be performed under light sedation or general anaesthesia.  How is a gastrostomy placed? The procedure is performed by two gastroenterologists and generally takes between 15 and 30 minutes:The first doctor inserts an endoscope (a thin, flexible camera) through the mouth into the stomach.The second doctor identifies the best site on the abdominal wall from the outside, using the light from the endoscope.A small incision of a few millimetres is made in the skin after local anaesthesia.The tube is then passed from the inside outwards and secured in place, passing directly through the abdominal wall into the stomach (a technique commonly known as “pull gastrostomy”). What happens during the period after the gastrostomy is placed? After the procedure, you will remain in hospital for at least 2 days to ensure that enteral nutrition through the gastrostomy is well tolerated.The most frequent complications are pain around the tube opening, which is generally temporary. More rarely, bleeding or perforation may occur; these are detected promptly by means of an abdominal CT scan.Before you are discharged, the dietitian and theclinical nutrition nurse will explain the type and quantity of enteral nutrition suited to your situation, as well as how to use the tube at home. If necessary, home nursing care will be arranged before your discharge. How is follow-up carried out? Once the tube is in place, regular follow-up is essential to ensure your care proceeds smoothly:Regular consultations with the gastroenterologist and the clinical nutrition nurseDietary assessments to adapt your nutritional intake to changes in your conditionBlood tests to monitor your nutritional parametersFor young patients: the transition to adulthoodIf you have been followed in paediatrics until now, the transition to an adult service is prepared gradually from the age of 16:Your paediatrician will contact the adult team and provide them with all the information concerning your health and treatmentA first joint consultation — in the presence of your paediatrician and your new doctor responsible for your care — will be arranged, so that you do not feel alone during this stageIf necessary,, follow-up in both services (paediatric and adult) can be maintained for a maximum of 6 months in order to facilitate this transition in complete peace of mind. Contact You can make an appointment for a consultation (by email at ConsGastroMed [dot] erasme [at] hubruxelles [dot] be or by telephone on +32 (0)2 555.35.04, or contact the H.U.B Gastroenterology Department directly at SecMed [dot] GastroMed [dot] erasme [at] hubruxelles [dot] be (SecMed[dot]GastroMed[dot]erasme[at]hubruxelles[dot]be)You can also contact our clinical nutrition nurses, Asuncion Ballarin ( asuncion [dot] ballarin [at] hubruxelles [dot] be (asuncion[dot]ballarin[at]hubruxelles[dot]be)), Valerie Burion (val%C3%A9rie [dot] burion [at] hubruxelles [dot] be (valérie[dot]burion[at]hubruxelles[dot]be)), and, for the Jules Bordet Institute, Ingrid Amorison (ingrid [dot] amorison [at] hubruxelles [dot] be).  Discover the Pancreatology and Nutritional Support Clinic

When a patient can no longer eat by mouth, either temporarily or on a more permanent basis, another route must be found... What exactly is a feeding stoma?When a patient can no longer eat by mouth, either temporarily or on a more permanent basis, another route must be found. A gastrostomy is one of these alternatives: it consists of creating a small opening that connects the stomach to the skin through a tube. The procedure is performed endoscopically, using a simple technique that avoids major surgery and allows the tube to be placed safely.Who is concerned?People who require a gastrostomy have very different profiles, but they share the same reality: their body can no longer meet their nutritional needs on its own.“This may be related to neurological diseases that affect swallowing, established malnutrition, certain cancers that weaken the body, or lung diseases that use up all the available energy,” explains Ms Ballarin.When oral feeding is no longer possible or is insufficient, and this situation is expected to last, gastrostomy becomes an appropriate and safe option to ensure a reliable and continuous nutritional intake for the patient.Is it risky?“As with any medical procedure, complications can occur, but they are rare, often mild and, above all, largely preventable,” reassures the head nurse.Everything begins well before the procedure: the team meets with the patient and their relatives, explains each step, answers questions and prepares the return home. Anaesthesia or sedation may be offered to ensure the procedure takes place under the best possible conditions. This preparation phase is not a minor detail: it reduces stress and limits risks for the patient.In the long term: manageable inconveniences“Following good practices reduces the risk of complications, which is why therapeutic support for patients and their caregivers is so important. Education and training have three objectives: ensuring effective tube feeding, preventing complications and improving the patient’s comfort,” stresses Ms Ballarin.The most common complications are skin-related issues, such as inflammation, granulomas, fungal infections or local infections. These skin problems are generally mild and can usually be treated easily with appropriate care.Digestive intolerances may also occur, such as nausea, bloating, diarrhoea or constipation. Although these symptoms can be unsettling, they are usually easy to understand and, above all, can be corrected through adjustments to the way the feeding is administered.The most frequent late complications are skin irritations around the tube (redness, inflammation, fungal infections), which can also be treated easily. Digestive problems (a sensitive abdomen, altered bowel movements or constipation) may appear when enteral nutrition is started, meaning when liquid nutrition is delivered through the tube. These symptoms can sometimes worry patients, but they have explanations and, most importantly, can be treated.The right daily habits?Two simple steps every day: clean the area with water and dry it thoroughly. Clean and dry skin helps prevent irritation and limits most complications. The second step is less obvious but just as essential: gently move the tube every day with a small back-and-forth movement. This simple action prevents the tube from becoming embedded in the stomach wall, a rare but serious complication that can largely be prevented thanks to the advice provided to patients.“Each patient leaves with a clear information booklet and a detailed care protocol. Consultations take place twice a week, on Monday afternoons and Wednesday mornings. A full assessment is organised every six months. And if there is any doubt, a direct helpline is available at any time. Often, a simple phone call or a photo sent from a smartphone is enough to reassure the patient and solve the problem, sometimes without the need to come to the hospital,” explains Ms Ballarin. Discover the nursing team When should you contact us?“It is essential to inform us as soon as an adverse event occurs, whether or not it is related to the tube. Some signs, even if they seem minor at first, can quickly become troublesome for the patient and compromise the continuation of nutritional treatment. Our role is precisely to intervene early to prevent these small problems from becoming more serious,” she explains.Any redness, irritation, discharge or unusual pain around the tube should be reported immediately. These problems are common but can be treated easily when managed quickly.Digestive discomfort is often misunderstood. A bloated abdomen, nausea, constipation or pain does not necessarily mean that “the nutrition is not being tolerated”. In most cases, it simply means that an adjustment is needed: the feeding rate may be too fast, the timing may not be suitable, hydration may be insufficient, or there may sometimes be a cause unrelated to the tube itself (gastroenteritis, medication, stress, etc.).Difficulty flushing the tube and/or an inability to administer the nutrition should be reported without delay. An early blockage can usually be resolved easily; a later obstruction may require the tube to be replaced.What about the patient’s social life?A gastrostomy never occurs in isolation. It enters lives that have already been disrupted by illness. A stroke that changes everything overnight, motor neurone disease that progresses, cancer that gradually drains the body. In these challenging journeys, every new medical step can feel like an additional burden. This is precisely where the healthcare team plays a key role: helping transform a tube that may initially be perceived as a burden into a genuine source of support.A feeding stoma cannot replace what a meal represents, because eating is about much more than nutrition: it is about sharing a moment, sitting at the table with the people we love. For some patients who can no longer eat by mouth, this loss creates a deep sense of absence that can sometimes be difficult to express.“It is essential to acknowledge this loss, to listen to it and to provide support. Tube feeding does not claim to fill this void. However, it can provide the energy needed to continue experiencing these moments in a different way: being present, taking part, and staying connected with others. Feeding is not only about calories: it is also about meaning, connection and dignity,” emphasises Ms Ballarin.A word for someone who has just learned they will need a gastrostomy?For many people, gastrostomy can feel like one more difficult step in an already challenging journey with an underlying illness. These are diseases that disrupt everything: the body, independence, social life and identity. So yes, hearing that a feeding tube will be placed can be frightening. This is a normal reaction. The aim is to help patients find meaning where there is fear, and to restore a sense of perspective when everything seems to be becoming more complicated.A gastrostomy is not a failure. It is a treatment. Just as a broken leg requires rehabilitation, a weakened body may need support to receive adequate nutrition. It is not giving up; it is providing help.In an already difficult journey, being told that a gastrostomy is needed may feel like another sign that things are getting worse. This reaction deserves to be heard and acknowledged, not dismissed.And then, the way we see it changes.“A gastrostomy is not a defeat but a form of support. It is a practical way to take care of yourself when your body needs it,” concludes Ms Ballarin. Asuncion Ballarin Head Nurse, Stoma Care Nurse, Head of the Clinical Nutrition, Wound Care and Stoma Therapy Unit at H.U.B Contact our stomatherapy team Read also... “With a stoma, my body is different, but life is still possible.”It is often imagined as something impossible to overcome. A “hole in the abdomen,” a distressing image, a life turned upside down, intimacy forever changed. And yet…Read more

Good news!You can now use MyHUB to book appointments directly for:a knee MRIa MammotestAs soon as your doctor has prescribed one of these examinations, you can view the available appointments and book the time slot that suits you, directly via the app.MyHUB also provides you with all the information you need to prepare for your examination and easily manage your appointments.Don’t have MyHUB yet? The app is free and can be accessed securely via itsme®.Discover MyHUB and download the app.

What is it? Sinus node disease The sinoatrial node, located in the atrium, contains cells that send electrical signals to the other parts of the heart. There are different types of sinoatrial conduction disorders, ranging from a simple slowing to complete interruption. They may be acute or chronic, permanent or paroxysmal. There are various causes: Myocardial infarction, particularly when it affects the lower part of the heart. Medication overdoses. Most often, fibrotic degeneration of the sinus node, occurring mainly in older people. In this case, it may be associated with episodes of paroxysmal atrial fibrillation or flutter. This results in partial interruption of sinoatrial conduction, leading to bradycardia (a slow heart rate). Symptoms may range from presyncope to syncope. Treatment consists of inserting a pacemaker with two leads, one in the atrium and the other in the right ventricle. Atrioventricular blocks This is impaired atrioventricular conduction, ranging from slowing to complete interruption. It indicates lesions affecting the conduction tissue located in the atrioventricular node. There are several causes: Myocardial infarction. Acute inflammatory and/or idiopathic atrioventricular block. Medication overdoses. However, the most common cause is fibrotic degeneration of the atrioventricular conduction tissue. Its origin is undetermined. It occurs in older people. The clinical manifestations may be asymptomatic, meaning that the person feels nothing, but they mainly involve a slow heart rate (bradycardia), characterised by fatigue and even recurrent syncope. There are several classifications: First-degree atrioventricular block (which does not require a pacemaker). Second-degree atrioventricular block, which is divided into Mobitz I and II. Third-degree atrioventricular block. Treatment consists of inserting a pacemaker with two leads, one in the atrium and the other in the right ventricle. Fibrillation Atrial fibrillation is a heart condition characterised by random contractions of the atria (the small chambers of the heart), causing the overall heart rhythm to become irregular. It is the most common heart rhythm disorder in the population and can cause very serious complications, such as cerebral embolisms. These are small blood clots that block arteries in the brain and are one of the causes of cerebrovascular accidents. This condition, which is more common in people over 60, may have several causes: Heart disease (mitral valve disease, myocardial infarction, etc.). A non-cardiac condition (excess thyroid hormones, a lung infection, pulmonary embolism, alcohol consumption, etc.). Sometimes no cause is found; this is then referred to as idiopathic atrial fibrillation. The doctor may suspect the diagnosis during the clinical examination, mainly finding that the pulse is fast and irregular. However, atrial fibrillation is not always permanent, which makes diagnosis more difficult. Doctors then refer to it as paroxysmal atrial fibrillation. Treatment Cardiac arrhythmias are treated at the Arrhythmology Clinic.  An electrocardiogram can reveal the irregular, chaotic and rapid activity of the atrium. When the electrocardiogram is normal but the doctor strongly suspects the presence of atrial fibrillation, the cardiologist will perform a 24-hour electrocardiogram recording (an examination known as a 24-hour Holter monitor). Various treatments are available to treat this condition or prevent its complications. Overall, many medicines, known as antiarrhythmics, can treat the vast majority of patients. If the response to medication is inadequate—that is, if the patient continues to experience symptoms that are difficult to tolerate (troublesome palpitations, discomfort, fainting, etc.)—or if the patient cannot tolerate the medicines prescribed by their cardiologist, a more radical method is available. This involves “burning” the areas of the heart responsible for the arrhythmia in order to restore a regular heart rhythm. This burning is performed by applying an energy source (usually radiofrequency) directly to the inner wall of the atria. This beating-heart technique, generally used in some specialised cardiology centres, involves ablating or isolating the pulmonary veins (which are responsible for the arrhythmia) by introducing several probes, or catheters, into the heart under fluoroscopic guidance. The advantage of this procedure is that it can be performed under local anaesthesia, through a puncture (= the first step in advancing a catheter) in the groin and, if necessary, in the neck. Unfortunately, this technique can be laborious and lengthy, and it sometimes needs to be repeated a few days later. The final outcome cannot be predicted with certainty, but the current expectation in the best centres is a 50–60% cure rate for symptomatic atrial fibrillation (also called paroxysmal atrial fibrillation). It should nevertheless be noted that these results will probably improve with the emergence of new technological developments. Since June 2003, we have developed a surgical approach designed to increase the chances of success of the pulmonary vein isolation procedure while maintaining its minimally invasive nature. It involves encircling the areas potentially responsible for atrial fibrillation (the pulmonary veins) from outside the heart, using a surgical robot. This tool is particularly useful: not only does it facilitate the surgical procedure (the robot’s wrists have a much greater range of movement than the surgeon’s hands), but it also makes it possible to operate on the heart without actually opening the sternum or thorax, except for three small incisions of less than 1 cm between the ribs.

Aesthetic surgery aims to correct physical imperfections that may negatively affect the patient. Some procedures can be considered to enhance the patient’s appearance, while others are primarily intended to rejuvenate them. In any event, the ultimate aim of this surgery is to bring the patient’s body closer to the ideal image they have of themselves. Among the most common procedures are: Breast augmentation Breast reduction Correction of breast ptosis (sagging) Face and neck lift Blepharoplasty (eyelid surgery) Rhinoplasty (nose surgery) Correction of protruding ears and liposuction (saddlebags, abdomen, thighs, etc.) Abdominoplasty Arm lift Thigh lift This list is, of course, not exhaustive. A consultation with one of the surgeons in the Department allows each case to be assessed individually.

Hypereosinophilic syndromes and eosinophilic diseases Hypereosinophilia refers to a significant increase in the level of eosinophils in the blood and/or tissues.

Systemic lupus erythematosus is a chronic autoimmune disease known as systemic because it can affect several organs, including the skin, kidneys, joints, lungs and nervous system. Clinical manifestations vary greatly from one person to another, but also in the same person over time. Lupus mainly affects women (9 women for every 1 man) and young people (average age between 15 and 45 years). It is a rare disease. It is estimated to affect approximately 5,000 people in Belgium. Lupus is characterised by the presence of autoantibodies in the blood. These are produced inappropriately by the immune system, which, in addition to defending the body against microbes, targets the body’s own cells. Certain types of white blood cells (lymphocytes) become abnormally activated and cause inflammation and lesions in various organs. The onset of this immune dysregulation is probably linked to several factors (hormonal factors, smoking, viruses, medicines, etc.) in individuals with a genetic susceptibility to developing the disease. What are the complications of lupus? In its cutaneous form, lupus causes a rash on the face, often on the cheeks and nose (“malar rash” or “wolf mask”, hence the name lupus). Red patches may appear on areas exposed to the sun (photosensitivity). They may be accompanied by mouth ulcers and/or more or less significant hair loss. This form affects up to 80% of patients during the course of their disease. Joint pain, often affecting the small joints of the hands and wrists, may occur, sometimes associated with swollen joints. This condition is often symmetrical. Inflammation does not generally lead to joint destruction. Joint involvement is present in 75 to 90% of patients. Raynaud’s phenomenon affects approximately 30% of patients with lupus. It involves a change in the colour of the fingertips and toes caused by cold or stress, sometimes accompanied by severe pain. This symptom is not specific to lupus and may occur in the general population, particularly among young women, without any immune disease. Some autoantibodies may predispose patients to thrombosis (the formation of blood clots in the blood vessels), which can cause phlebitis, pulmonary embolism, myocardial infarction or a cerebrovascular accident. This is referred to as antiphospholipid syndrome. Kidney involvement can vary in severity and may sometimes progress to chronic disease. It may develop insidiously, without specific symptoms. It is then detected only through blood tests and testing for proteins in the urine. It may sometimes manifest as fluid retention or high blood pressure. In severe cases, this condition can lead to kidney failure. Lupus can cause inflammation of the membranes surrounding the heart (pericardium) and lungs (pleura). The patient may then experience oppressive, recurrent chest pain, coughing or shortness of breath. Blood abnormalities are observed in nearly 85% of patients with lupus. They fluctuate during the course of the disease and vary widely. They may include anaemia (a decrease in red blood cells), leukopenia (a decrease in white blood cells) or thrombocytopenia (a decrease in platelets). Neurological manifestations also vary in presentation. Headaches are fairly common (30 to 60% of patients with lupus). More rarely, patients may develop epileptic seizures, concentration or memory problems, confusion, mood disorders, depression or drowsiness. How is lupus diagnosed? The diagnosis of lupus is established by taking into account the various symptoms presented by the patient and the results of additional examinations. These examinations include blood tests (to identify antinuclear antibodies, and more specifically anti-Sm or anti-dsDNA antibodies, an increased total antibody level, and reduced levels of certain proteins known as complement factors), imaging examinations to look for complications (heart ultrasound, joint X-rays, etc.), and, if necessary, a skin biopsy. What treatment is available? Given the diversity of clinical manifestations and their combinations, on the one hand, and the parallel and simultaneous involvement of different organs, on the other, the management of patients with lupus must be multidisciplinary. It involves the various specialists for each affected organ, in order to determine the severity of each manifestation and define the best treatment, taking into account all the lesions present in a particular patient. As each patient with lupus has specific clinical manifestations of varying severity, the proposed treatment will be adapted to each patient and may change over the course of the disease. No treatment can cure lupus. The aim of treatment is to reduce inflammation, prevent long-term complications, improve the patient’s quality of life and, if possible, bring the disease into remission (that is, normalise a series of abnormalities in the blood tests and eliminate certain symptoms indicating inflammation). The immune origin of the disease means that medicines are used to regulate or reduce immune activity (immunomodulators and immunosuppressants), including cortisone (sometimes at high doses) and hydroxychloroquine (or Plaquenil, which has a preventive effect against relapses). Other medicines may sometimes be prescribed to help limit the use of cortisone, including azathioprine, mycophenolate mofetil, cyclophosphamide, methotrexate, etc. New medicines have recently begun to be used, such as rituximab and belimumab. They reduce the number of cells that produce antibodies. Other treatments are currently being evaluated for lupus as part of clinical research protocols. Useful links: www.orpha.net www.lupus.be 

Definition and symptomsMastocytoses are a group of diseases characterised by the abnormal proliferation of mast cells as a result of an acquired activating mutation of the CKIT gene (D816V), and their accumulation in various organs: the skin (mainly), haematopoietic organs (bone marrow, liver, spleen), the digestive tract and bones. They are also characterised by the autonomous, unregulated activation of mutated mast cells, which causes their excessive degranulation (release of mast-cell mediators contained in the mast-cell granules).Symptoms of mast-cell activation (MCAS) may affect all organs and include: flushing (episodes of redness), itching, anaphylactic shock, malaise, various digestive disorders (diarrhoea, pain, food intolerances), various types of pain, particularly skeletal pain and headaches, fatigue, sleep and mood disorders, rhinitis and shortness of breath.Mastocytosis is a rare disease, affecting approximately 1 in 150,000 patients per year.TypesMastocytosis is referred to as cutaneous when there is no excess of mast cells in organs other than the skin. This form is by far the most common in children.From birth or during the first weeks of life, a child may develop one or more skin lesions corresponding either to urticaria pigmentosa or mastocytomas. These lesions are particularly reactive to friction or sudden changes in temperature, which may cause blisters to appear and trigger symptoms of mast-cell activation (diarrhoea, malaise, etc.). Mastocytosis in children may regress completely during adolescence. However, the exact frequency of this spontaneous remission remains to be determined (50% of cases or more?). When mastocytosis is diagnosed in an adult, it is always considered systemic; the disease is chronic, and no treatment is considered capable of curing it. However, mastocytosis in adults is most often indolent. The most common form in adults (80% of cases) is Indolent Systemic Mastocytosis (ISM). Skin involvement is common, in the form of urticaria pigmentosa or telangiectasias (TMEP). Infiltration of organs other than the skin causes neither enlargement nor dysfunction of these organs. Most symptoms result from MCAS, which may sometimes cause significant functional impairment (for example, in cases of recurrent anaphylactic shock, urgent diarrhoea, disabling pain, repeated flushing with post-episode fatigue, cognitive and mood disorders). Quality of life may therefore be severely impaired in some patients, while other patients remain asymptomatic. In this form, screening for bone demineralisation (30% of patients) is essential in order to initiate bone-forming treatment if necessary and prevent vertebral fractures. These patients’ survival is not threatened. Outpatient follow-up takes place annually. The remaining 20% of patients have an aggressive form of mastocytosis (primarily aggressive systemic mastocytosis, and much more rarely mast-cell leukaemia or mast-cell sarcoma), with or without an associated non-mast-cell clonal haematological disorder (mainly myelodysplasia). They are managed in a haematology inpatient setting. Their survival is rapidly threatened by dysfunction of at least one organ massively infiltrated by mast cells: liver failure, bone-marrow failure, cachexia due to digestive malabsorption or hypersplenism.  MCAS is rare or absent. Cytoreductive treatment must be initiated urgently.How is mastocytosis treated?There is no curative treatment for mastocytosis. The therapeutic objective depends on the type of mastocytosis.In most cases, the disease is indolent. The therapeutic objective is to improve patients’ quality of life if it has deteriorated. Treatment includes strict avoidance of any factor identified as triggering mast-cell degranulation (food, medication, hymenoptera sting, excessively strenuous physical activity or extremely high or low temperatures), as well as symptomatic treatment (antihistamines). If, after at least 6 months of optimal and properly administered symptomatic treatment (maximum doses and good adherence to the prescription), the impairment remains too disabling, long-term cytoreductive treatment may be considered. In patients with a history of anaphylactic shock, an adrenaline auto-injector must be carried at all times.In the rarer aggressive form of mastocytosis, the primary objective is to reduce the mast-cell infiltration that is suffocating the organs. Cytoreductive medicines (cladribine-based chemotherapy and tyrosine kinase inhibitors) are essential for this purpose and must be started as soon as possible.Information for patients and their familiesPresentation of the French reference centre for mastocytoses – CEREMAST brochureMastocytosis patient associations

What is it? Systemic scleroderma is a disease that primarily affects the small-calibre blood vessels (arterioles and capillaries) throughout the body and is accompanied by widespread fibrosis.The involvement of the blood vessels explains an early symptom present in almost all patients: Raynaud’s phenomenon.This very painful phenomenon is characterised by whitening of the fingers when exposed to cold (or to other factors such as stress or cigarette smoking), often followed by a phase during which the fingers become very red or even purple. It may sometimes lead to necrosis (death) of the skin at the fingertips (known as digital ulcers). Fibrosis consists of excessive deposition of collagen, a protein found throughout the body that provides structure to tissues and organs.Immune dysregulation, particularly inflammation and autoimmunity, is also involved in this disease. This is why certain autoantibodies are tested for when establishing the diagnosis of systemic scleroderma.The cause of the disease remains unknown, but it probably involves both a particular genetic predisposition and certain environmental factors. A body of evidence suggests that there is a low genetic predisposition to systemic scleroderma.Analysis of familial risk in several groups of patients has shown that the risk of developing this disease among first-degree relatives (first generation) was 13 times higher than in the general population (15 times higher for the siblings of a patient with scleroderma).Although these results demonstrate a familial risk and support the search for genetic factors, the risk of transmission remains very low for any given individual, and families in which one parent has this disease should be reassured. The symptoms Which tissues and organs are affected by this disease? Systemic scleroderma affects various tissues and organs, including the skin, lungs, digestive tract, kidneys and heart. The fibrosis observed in systemic scleroderma corresponds to an exaggerated and poorly controlled tissue-repair process. The relationship between vascular abnormalities and the fibrotic process is poorly understood. The disease was called systemic scleroderma because of the frequency and appearance of the skin lesions (“-derma”), but the term systemic sclerosis (also used) better reflects the extension of the fibrotic process to other organs, which characterises this disease. Systemic sclerosis must be distinguished from localised scleroderma, in which only the skin is affected by fibrosis/sclerosis; this is also referred to as morphoea. Conversely, in some cases of systemic scleroderma, the skin may be unaffected even though organs are involved; this is referred to as scleroderma sine scleroderma.The extent of skin involvement assessed at its maximum extent (when the involvement was most severe) appears to be associated with the risk of developing deep-organ lesions. Assessing the severity of skin fibrosis is therefore important in guiding monitoring and treatment.Epidemiology of systemic sclerodermaSystemic scleroderma is a rare, so-called “orphan” disease. An orphan disease is defined as having a prevalence (the number of people living with the disease) of fewer than one person in 2,000 (proposed European legislation on orphan medicines). The prevalence of systemic scleroderma is still poorly understood and varies considerably between regions and countries. In Europe, the prevalence is approximately 100 to 200 per million inhabitants.In most cases, the first signs of systemic scleroderma appear at around 40–50 years of age, most often in women (four women for every one man). What are the clinical manifestations of systemic scleroderma?The symptoms and signs present at onset vary according to the two main subtypes of the disease: In the diffuse cutaneous form, all the signs usually appear at the same time, generally combining Raynaud’s syndrome, skin fibrosis that progressively extends towards the thighs and arms, and often joint and tendon pain.In the limited cutaneous form, Raynaud’s syndrome most often predates the disease by several years, and disease onset is often marked by its worsening, associated with thickening of the skin of the fingers (sclerodactyly) and, possibly, of the face and/or the appearance of telangiectasias (small dilations of the blood vessels). Oesophageal involvement is common in this form and is characterised by heartburn and gastro-oesophageal reflux.While the onset of the disease is easy to date in the diffuse cutaneous form, this is often more difficult in the limited cutaneous form. The first sign other than Raynaud’s syndrome is usually used to date the onset of the disease. Progression varies according to the two subtypes of skin involvement, although there are individual exceptions to the following:In the diffuse cutaneous form, the disease progresses rapidly and may lead to more or less significant organ damage, which must be sought from the outset. Studies have shown that after 3 to 5 years, the inflammatory process of the disease often stops or stabilises, with only the damage already established persisting. After several years, some abnormalities may even improve, particularly skin fibrosis, which tends to become more flexible. This is crucial, as it determines patient follow-up (which is particularly close during the first few years) and treatment. Disease-modifying treatments should primarily be evaluated during the active phase of the disease, that is, during the first 3 to 5 years, when fibrosis develops.In the limited cutaneous form, disease progression is slower. The main risk associated with the progression of this form is the development of pulmonary arterial hypertension, which generally occurs after 10–15 years of disease progression. Regular monitoring must therefore be continued to detect this serious complication, which can go unnoticed if the necessary tests are not performed, as it only causes symptoms relatively late. How is scleroderma diagnosed? The diagnosis of systemic scleroderma is based on a clinical examination, blood tests and capillaroscopy. In addition, other tests are carried out to assess the extent of the disease (that is, to determine which organs are affected besides the skin).Clinical signs: mainly Raynaud’s phenomenon and skin thickening; the clinical examination also looks for digestive manifestations (acid reflux, etc.), pulmonary manifestations (shortness of breath, cough, etc.), and others.Blood tests: these look for the presence of antinuclear factors (detectable in 98% of cases), such as anti-Scl-70 antibodies and anticentromere antibodies.Capillaroscopy: the base of the nail is simply examined under a microscope to look for signs of microcirculatory involvement: the presence of dilated capillaries (megacapillaries) and areas where the capillaries have disappeared (avascular areas).Assessment of the extent of the disease may include other tests, such as a chest CT scan, pulmonary function tests, a six-minute walk test, an echocardiogram, an electrocardiogram, right heart catheterisation, a gastroscopy and an X-ray of the hands. Treatment There is currently no treatment that cures the disease.The treatments currently available aim to relieve the symptoms (symptomatic treatment) and slow the progression of the disease (disease-modifying treatment).Symptomatic treatment is primarily aimed at reducing the discomfort caused by Raynaud’s phenomenon and gastro-oesophageal reflux. It consists of taking medicines that dilate the blood vessels and reduce acid secretion by the stomach.Disease-modifying treatment is mainly indicated when the inflammatory component is significant, particularly if it extends to the skin and/or affects organs such as the lungs. In such cases, low doses of oral corticosteroids are used, together with an immunosuppressant medicine (which reduces the activity of the immune system).Some examples of conventional immunosuppressants include azathioprine, mycophenolate mofetil, cyclophosphamide and methotrexate. Newer, more targeted treatments are also available and are still under investigation, including rituximab and tocilizumab.Systemic sclerosis is managed within the Multidisciplinary Centre for Systemic Scleroderma. Specific links www.sclérodermie.bewww.eustar.orgwww.fesca-scleroderma.eu Multidisciplinary Systemic Sclerosis Centre The Multidisciplinary Systemic Sclerosis Centre provides care for systemic sclerosis, a rare autoimmune disease affecting the tissue that supports the organs (connective tissue) and the small arteries (arterioles), whose main characteristic is hardening of the skin. Physicians from different specialties work together to diagnose and treat the disease.Coordination of the Multidisciplinary Systemic Sclerosis CentreTel: 02 555 3650Fax: 02 555 8247Email: msoyfoo [at] ulb [dot] ac [dot] be (msoyfoo[at]erasme[dot]ulb[dot]ac[dot]be)

These are sometimes serious chronic diseases that affect men and women and, much more rarely, children or adolescents. However, certain age groups are more commonly (or even exclusively) affected, depending on the type of vasculitis diagnosed (for example, giant cell arteritis, or “Horton’s disease”, which occurs only after the age of 50). Vasculitis (called vascularites in France) is defined by the presence of inflammation of the blood vessels: arteries, veins and/or capillaries. The walls of the affected vessels may either thicken or, more rarely, become thinner and dilate. If blood flow through the affected vessels is severely reduced or stops altogether (thrombosis – the formation of blood clots), the organs or tissues supplied by these vessels no longer receive sufficient oxygen and become dysfunctional or damaged, potentially progressing to necrosis (i.e. cell death) of the affected organ or tissue. The origin of these diseases is complex and still relatively poorly understood. The main factors are environmental (such as infection with certain viruses) and/or intrinsic to the patient’s immune system, which—in addition to defending against external agents (such as microbes)—attacks and damages the individual’s organs (the concept of autoimmunity). This predisposition to immune-system dysregulation is thought to have a genetic origin, although we still know very little about the genetic abnormalities involved. Fortunately, this genetic influence does not imply a significant risk of familial transmission. The term vasculitis encompasses a range of different diseases that share inflammatory involvement of the blood vessels. These diseases are classified according to the size of the vessels affected by the inflammation: small-, medium- or large-vessel vasculitis. Among small-vessel vasculitides, a further distinction is made between those associated or not associated with the presence in the blood of an autoantibody called ANCA (for “Anti-Neutrophil-Cytoplasmic-Antibody”), an antibody directed against proteins expressed in neutrophils (the most abundant white blood cells in the blood). Small-vessel vasculitides include granulomatosis with polyangiitis (formerly Wegener’s disease), eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome), microscopic polyangiitis, IgA vasculitis and cryoglobulinaemic vasculitis (the latter two are not associated with ANCA). Medium-vessel vasculitides include polyarteritis nodosa and Kawasaki disease (which affects almost exclusively children). Large-vessel vasculitides include giant cell arteritis (also called Horton’s disease) and Takayasu disease. Behçet’s disease involves vessels of several different sizes. Clinical presentation The clinical presentation of vasculitides varies considerably. Given the diversity of the type (for example, large or small blood vessels) and location of the vessels affected, depending on the disease, it is not possible to provide an exhaustive description here of the highly variable clinical presentations from one individual to another. By way of example, the following are some of the ways in which certain vasculitides may present in their most typical forms: Horton’s arteritis - headaches, visual disturbances, pain in the shoulders and pelvis Granulomatosis with polyangiitis - fever, weight loss, diffuse joint pain, cough, shortness of breath, coughing up blood, nosebleeds, inflammation of the sinuses with hearing problems, kidney dysfunction Eosinophilic granulomatosis with polyangiitis - increasingly severe asthma, shortness of breath, cough, loss of strength and/or sensation in a nerve distribution (for example, in a foot or hand), red/purple spots on the skin. Diagnosis Diagnostic methods naturally include investigations directed at the affected organ or organs, as well as the search for specific findings that can establish a precise diagnosis. A key step is often performing biopsies of organs affected by the disease, which are analysed under a microscope by a specialist physician (anatomical pathologist) to look for vascular inflammation (vasculitis). Other investigations are often required, such as vascular imaging (Doppler ultrasound, CT scan, PET-CT, etc.) in large-vessel vasculitides (such as giant cell arteritis, or “Horton’s disease”), or testing for antibodies directed against certain components of our body (for example, testing for ANCA in granulomatosis with polyangiitis). Treatment The aim of the treatment of vasculitides is to reduce immune-system activity. The cornerstone is a corticosteroid (commonly called “cortisone”), which is often rapidly effective but has a “bad reputation” because of its well-known side effects; these should be put into perspective according to the cumulative dose. Immunosuppressive medicines targeting a type of white blood cell—the lymphocytes—which are particularly involved in (auto)immunity, are also routinely added to some extent. More recently, targeted treatments (called monoclonal antibodies) have proved to be major advances in the treatment of vasculitides. They are directed more specifically against certain lymphocytes or against molecules involved in inflammation/immunity. Access to them is limited by their very high cost and the reimbursement conditions set by INAMI. Sometimes, they are available only through clinical studies conducted in a limited number of hospitals. Useful links The vasculitis A–Z guide The French patient association CANVASC (Canadian study group) There is no Belgian patient association, but there is a Belgian contact person for the French association, whose contact details can be obtained from that French association.

What is sickle cell disease? Sickle Cell Disease is a genetic disorder of haemoglobin, the main component of red blood cells, which transports oxygen throughout the body. In sickle cell disease, the haemoglobin is abnormal and is called haemoglobin S (normal haemoglobin is called haemoglobin A). This abnormal haemoglobin causes the red blood cells to become crescent-shaped (known as sickle-shaped red blood cells). This abnormal haemoglobin causes the red blood cells to become crescent-shaped (known as sickle-shaped red blood cells). These abnormal red blood cells have two main consequences:Abnormal rigidity, which causes obstruction of the blood vessels and prevents blood—and therefore oxygen—from reaching the tissues and organsSignificant fragility, which causes their accelerated destruction and leads to anaemia (red blood cell and haemoglobin levels that are too low)These mechanisms lead to acute and chronic complications affecting all of the patient’s organs, requiring specialised multidisciplinary follow-up. Watch our films on Sickle Cell Disease Sickle Cell Disease, those who are looking They know about Sickle Cell Disease—do you? Causes and risk factors  Sickle cell disease is an inherited genetic disorder, meaning that abnormal haemoglobin (haemoglobin S) is transmitted by the parents to their children and is present from birth. We all carry two copies of each gene: one copy is inherited from the mother and one copy is inherited from the father. To have sickle cell disease, a person must have two abnormal genes and therefore have received one copy of an abnormal gene from each parent. People carrying only one abnormal gene are referred to as “healthy carriers” and show no signs of sickle cell disease. Care: diagnosis and treatment Diagnosis involves a blood test to detect the presence of abnormal haemoglobin (not to be confused with the A, B and O blood groups, which are unrelated to sickle cell disease). This test is sometimes supplemented by genetic analysis to clarify the diagnosis. Our laboratory is recognised as a Belgian and European reference laboratory for the biological and genetic diagnosis of red blood cell diseases.The only current curative treatment is a bone marrow transplant (or haematopoietic stem cell transplant), mainly performed in paediatric care. At present, management is based on screening for and treating complications, as well as treatments capable of alleviating red blood cell abnormalities and their consequences (for example, hydroxyurea or Hydrea®). Thanks to our expertise, our centre also has several clinical study protocols enabling patients to benefit from therapeutic innovations in this field. Clinical studies enable patients to participate in research and help advance care for people with sickle cell disease, while offering them the possibility of benefiting from new treatments or approaches for this condition. Red blood cell transfusion also plays an important role in the therapeutic arsenal. Its use in patients with sickle cell disease is complex and requires close collaboration between clinicians, the laboratory and technical platforms (such as dialysis for carrying out exchange transfusions). Orphanet Search In addition to research into new treatments, our team coordinates several studies aimed at improving our understanding of the disease and its clinical consequences, as well as improving patient care (what is known as clinical research).Furthermore, our team collaborates with various research laboratories in Belgium and abroad to advance our understanding of the mechanisms underlying sickle cell disease and other red blood cell disorders. This research helps link cellular mechanisms to clinical manifestations and paves the way for new treatments (what is known as translational research). Discover our video on Sickle Cell Disease research at H.U.B. Team and multidisciplinary care Patient care is provided by a multidisciplinary team comprising physicians specialising in sickle cell disease, as well as coordinating and specialist nurses. When hospitalisation is required, patients are admitted to a dedicated inpatient unit providing care specific to this condition. Follow-up also relies on a network of referring physicians specialising in the various affected organs, with particular expertise in aspects related to sickle cell disease (Neurology, Ophthalmology, ENT, Stomatology, Cardiology, Pneumology, Gastroenterology, Nephrology, Urology, Digestive surgery, Orthopaedic surgery, etc.). The department works closely with the hospital’s Erythrocyte Chemistry, Genetics and Immunohaematology/Transfusion laboratories.Our physicians are also members of the red blood cell subgroup of the Belgian Society of Haematology and maintain close collaboration with the team of Professor P. Bartolucci at the reference centre for rare red blood cell diseases, CHU Mondor in Créteil, Paris, France.Our department is recognised as a national and European reference centre for rare red blood cell diseases (ERN) and by the French healthcare network for rare constitutional diseases of red blood cells and erythropoiesis (MCGRE). Expertise and multidisciplinary discussions Complex cases are discussed monthly during a multidisciplinary meeting attended by experts in the relevant areas. These meetings are also open to other hospitals within the network, enabling collaboration and the sharing of expertise for the benefit of patients. To register a patient for this multidisciplinary consultation meeting, please complete the information form and send it to secretariat [dot] hematologie [at] hubruxelles [dot] be (secretariat[dot]hematologie[at]hubruxelles[dot]be). A service recognised as a centre of expertise Brussels University Hospital – Erasme Hospital is recognised as a European reference centre for the management of red blood cell disorders, including Sickle Cell Disease. ERN EuroBloodNet Orphanet Associated services Our specialist doctors in Sickle Cell Disease Prof. Martin ColardProfessor of Haematology, H.U.B. – Erasme; national adult sickle cell disease expert, coordinator of the sickle cell disease inpatient unit Dr Aude Theunissen Clinical haematologist, Adult Sickle Cell Disease Reference Centre, Red Blood Cell Disorders Unit, Brussels University Hospital – Erasme Send a message Image Cross-functional collaboration: Queen Fabiola Children’s University Hospital At H.U.B, care for Sickle Cell Disease is also provided at the Children’s Hospital (HUDERF). For more information, click on the link below. Sickle Cell Disease Children’s Hospital

What is it? Lymphoma is the most common haematological malignancy (malignant tumours of blood cells) and the third most common cancer in children. It is a cancer of the lymphatic system and develops when an error occurs during the production of lymphocytes, leading to the production of abnormal cells. These cells can proliferate in two ways: by dividing more rapidly than normal lymphocytes or by living longer than them. Like healthy lymphocytes, cancerous lymphocytes develop in various parts of the body, including the lymph nodes, spleen, bone marrow and other organs. There are two main types of cancer of the lymphatic system: Hodgkin lymphoma (Hodgkin’s disease) and non-Hodgkin lymphoma (NHL). Symptoms Signs and symptoms are all the disorders observed and reported by the patient that may be related to the disease. For example, swollen lymph nodes, whether painful or not, are a common sign of lymphoma. Lymph nodes are generally swollen in the neck or armpits, but many patients may also have swollen lymph nodes in other parts of the body, causing various symptoms. Thus, swollen lymph nodes in the groin may cause heavy legs and swollen ankles, while swollen lymph nodes in the abdomen may cause abdominal discomfort, back pain or bloating. The signs of extranodal lymphoma may vary depending on the part of the body where the tumour develops. This is why lymphoma in the stomach may sometimes cause symptoms similar to those of an ulcer, such as pain and internal bleeding. More rarely, some patients with lymphoma may not have swollen lymph nodes. Certain unusual symptoms observed by the patient may also indicate the possible presence of the disease. The symptoms of lymphoma most often include chills, fever, profuse sweating (often at night), unexplained weight loss, reduced energy, itching or other symptoms caused by swollen lymph nodes. These symptoms are not specific, and most people who experience them do not have lymphoma. However, in the case of persistent symptoms, it is important to consult a doctor to make sure that lymphoma is not present. Serious diseases do not disappear overnight; they persist. Types Non-Hodgkin lymphomas NHL is not a single disease but rather a group of at least 30 closely related cancers that affect the lymphatic system. Although the different forms of NHL have features in common, particularly their lymphatic origin, they differ in their appearance under the microscope, their molecular characteristics, their growth pattern and their impact on the body. NHLs are divided into two main subtypes: B-cell lymphomas (which develop from abnormal B lymphocytes) T-cell lymphomas (which develop from abnormal T lymphocytes). The exact causes of NHL are unknown. Doctors are often unable to explain why one person develops NHL while another does not. What is known is that lymphomas are not caused by an accident and cannot be contracted through contact with someone who has the disease. Hodgkin lymphomas Hodgkin’s disease (sometimes called Hodgkin lymphoma) accounts for 15% of all lymphomas and 1% of all cancers. It occurs in approximately 3 or 4 people per 100,000 each year. This rate has not changed for several decades. It most commonly affects adolescents or young adults (aged 15 to 35) or people aged between 45 and 50. Hodgkin’s disease is one of the cancers with the highest cure rates: a cure can be achieved in more than 80% of cases through radiotherapy, chemotherapy or a combination of these two treatments. Although the cause of Hodgkin’s disease remains unknown, environmental and genetic factors, as well as infectious agents, may be involved in its development. In both Hodgkin’s disease and non-Hodgkin lymphoma, several aspects of the tumour need to be assessed before determining the best treatment option. The overall prognosis is good because the vast majority of patients can be cured. However, it depends on various factors: the patient’s age, the extent of the disease, the impact of the disease on the patient’s general health (general symptoms, inflammation), the size of the tumour mass and, above all, the response to initial treatment. Treatment Lymphoma is one of the diseases that can be treated effectively, and patients can be cured through modern treatments such as chemotherapy, radiotherapy and immunotherapy. The response to treatment of the different types of lymphoma depends on the type and stage of the lymphoma, as well as on a number of other factors. For example, localised diffuse large B-cell lymphoma, which accounts for one-third of cases of this type of large B-cell lymphoma, can be cured in 80% of cases. Extensive follicular lymphoma is more difficult to cure but remains compatible with prolonged survival. Many patients treated for NHL receive chemotherapy, radiotherapy or biological therapy, or a combination of all these treatments. An autologous or allogeneic stem cell transplant is sometimes necessary. Except in exceptional cases, surgery is only performed during the diagnosis of lymphoma. Doctors may also decide not to prescribe immediate treatment for a patient with non-Hodgkin lymphoma. The patient continues to live normally as long as NHL symptoms are absent. They consult their doctor regularly for follow-up visits and laboratory and medical imaging examinations. Watchful waiting may be considered for certain forms of follicular lymphoma or other indolent lymphomas (low-grade malignancy). As soon as the patient begins to show signs of disease progression, treatment is initiated. Ongoing research is making considerable efforts to assess the efficacy of new medicines and treatment combinations. At the same time, researchers are working to develop new protocols to minimise the short- and medium-term toxicity of these treatments. Treatment for non-Hodgkin lymphoma is progressing rapidly, and many promising new treatments are currently under investigation.