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Paediatric age onset systemic autoimmune diseases are infrequent, complex entities that require a multi-disciplinary approach. The most frequent include juvenile onset systemic lupus erythematosus, mixed connective tissue disease, juvenile onset Sjögren’s syndrome, juvenile dermatomyositis, juvenile scleroderma, and paediatric age onset vasculitis, such as Kawasaki disease, IgA vasculitis (also known as Schönlein-Henoch purpura), polyarthritis nodosa and Takayasu disease.
The clinical manifestations of these diseases are highly varied. Juvenile systemic lupus erythematosus may affect several organs in the body, particularly the skin, joints, blood, kidneys and the central nervous system. In children, it is common for fever to appear without an infectious cause or an increase in the size of the lymph nodes.
Juvenile dermatitis is characterised by the presence of fatigue, muscle pain, weakness and the appearance of rashes that may affect the face, with inflammation around the eyes (periorbital oedema) There may also be reddening of the cheeks (malar rash) and other parts of the body (top part of the knuckles, knees and elbows), where the skin may become thicker (Gottron’s papules). Juvenile scleroderma, whose name comes from the Greek and means “hard skin”, is characterised by the presence of lesions on the skin and affects various organs. Two types can mainly be identified: localised scleroderma and systemic scleroderma.
Kawasaki disease is characterised by the presence of a high fever of unknown origin, irritability, reddening of the eyes and various skin lesions, such as a rash on the torso, flaking fingers and reddening of the tongue (normally called “strawberry tongue”). The involvement of the heart is the most serious manifestation of Kawasaki disease, due to the possibility of long-term complications.
Schönlein-Henoch purpura is characterised by a rash on the legs called “palpable purple” because the skin lesions can be touched, and painful and swollen joints, abdominal pain and kidney problems may appear.
All the conditions within the group are infrequent and have an incidence of less than 5 cases per 10,000 inhabitants, for which reason they are considered to be rare conditions. The spread is different depending on the disease. For example juvenile systemic lupus erythematosus, along with juvenile dermatomyositis and scleroderma, are more common in girls, while Schönlein-Henoch purpura is more common in boys.
Diagnosis of paediatric systemic autoimmune diseases is eminently clinical and we are guided by classification and diagnostics criteria in many of them. Blood tests are important for diagnosing the different systemic autoimmune diseases, as various autoantibodies can be identified that can help with the diagnosis and monitoring of these diseases. Supplementary tests, such as a capillaroscopy, chest X-ray, respiratory function tests, nuclear magnetic resonance and echocardiogram, amongst others, can be helpful when we come to approaching a paediatric patient with a suspected systemic disease.
Treatment fundamentally depends on the type of condition and the response to the therapy chosen. There is not currently any specific curative treatment for each one of the diseases, but the treatments available will help to control the signs and symptoms of the disease and prevent complications, including permanent damage to organs and tissue.
Paediatrics, Children's Hospital and Woman's Hospital
It is essential for patients to actively participate in the monitoring and treatment of their disease to increase their personal satisfaction and autonomy. Having reliable, verifiable information is also of great help in managing the disease.
Hereditary angioedema is such a rare disease that it is little-known even among healthcare workers. This means that in this case the patients themselves particularly need to know how to act in the event of an emergency, especially when they are not in their usual environment or are far away from their medical team.
It is advisable, as far as possible, to avoid possible triggers or aggravators of attacks:
The following symptoms indicate a suspected case:
The doctor must make a correct differential diagnosis in order to rule out other causes, such as appendicitis.
In this case it is important to remain calm and follow the doctor’s instructions. Here is some general advice:
1. Ask someone to help you explain what is happening to you.
2. Have the clinical report issued to you by your doctor at the ready.
3. If you have rescue or emergency medication (intravenous Berinert® or Cinryze®, or subcutaneous Firazyr®):
a) If you have been taught how, self-administer it in accordance with the instructions.
b) If you cannot administer it yourself, take it with you to the health centre.
4. Go to your nearest health centre for emergency treatment.
5. Make an appointment with your specialist once the immediate crisis has been dealt with.
In the case of a significant symptom burden in type I and II angioedemas, C1-INH may be administered as a prophylaxis.
TRANSFUSIONS – CAN I GIVE BLOOD?
It is not advisable for patients with hereditary angioedema of any type or acquired C1-inhibitor deficiency to donate blood.
LONG OR FOREIGN TRIPS
We recommend you take an up-to-date copy of the clinical report issued by your doctor with you. It is a good idea to have the report translated into the language of your destination or English.
Find out where the nearest healthcare centre is.
Always carry rescue or emergency medication with you and make sure it has not expired. Have your medical report to hand at security controls at airports or railway stations to avoid problems.
DIET
You do not have a follow a special diet because it is not an allergic oedema and it is not caused or triggered by a food allergy.
Diet does not have any impact on the evolution of the disease. You should, of course, follow the healthy diet recommendations issued to everyone.
Not all upset stomachs are the same. There are two types: organic, with a clear cause, and functional or recurrent. This advice is focused on the former. The causes of upset stomach vary greatly. The most frequent cause is associated with gastroenteritis, constipation or indigestion. Often children express their physical discomfort (fever, sore throat, etc.) or emotional discomfort (stress situations above all) in the form of stomach pain. Other factors that pull the wool in front of our eyes are appendicitis, urinary infections, intussusception and a long list of diseases that cannot be overlooked, which have a significant impact on children because they make them feel ill.
Upset stomach appears when viruses, parasites or bacteria damage the cells in the mucous membrane in the intestine. Normally, this membrane absorbs fluids, but when it is damaged it can no longer do it as effectively. The body therefore loses fluid through vomiting and diarrhoea.
Diarrhoea causes loss of fluids and salts. Children are more vulnerable to loss of fluids than adults; as they are smaller, they become dehydrated more quickly. Vomiting may lead to difficulties retaining the fluids consumed. Smaller children may deteriorate if they lose more fluids than they take in. This is why it is important that they drink from the onset of the stomach infection.
Vomiting and diarrhoea are the most common symptoms of upset stomach caused by a virus in children. The illness almost always cures itself and disappears in a couple of days. It is very important for children to drink fluids, especially if they have diarrhoea, which is particularly relevant in children under one year.
When children are suffering from upset stomach they may present different symptoms, including: vomiting, diarrhoea, discomfort, tummy ache, fever, tiredness and loss of appetite.
The symptoms normally appear all of a sudden, a couple of days after being infected, and disappear after a few days. Diarrhoea may last up to a week. Upset stomach is more common in winter, when children spend longer indoors. Although it may also be caused by bacteria, parasites or viruses, eating food that has gone off or travelling abroad.
You should go to your doctor or call 061 CatSalut Respon (Catalan Health Service care line) in the following cases:
You should seek urgent medical assistance if the child has diarrhoea, vomiting and any of these symptoms:
Children need fluids. They need to drink often. They can drink a bit at a time, in small quantities given to them with a spoon or bottle. Offer them water occasionally. It is important that they drink a bit at time, as if they drink too much in one go they may start vomiting again.
Avoid sweet drinks such as fizzy drinks or strong juices. They should also avoid diet drinks, as although they do not contain sugar, they may contain other products that cause diarrhoea. Infants may drink what they like, the most important thing is that they drink fluids.
If the child does not want to drink, try giving them ice lollies. You still need to offer them fluids. Even if they are eating ice lollies, they need to drink fluids to rehydrate themselves.
If the child is breast or bottle feeding, they should keep doing so, only more often, even if they vomit. If they do not want to breastfeed, you can also extract milk and feed them from a spoon.
A special syringe for medication is a good solution for oral rehydration. Make sure the liquid does not land directly on their palate, as this may cause nausea.
If vomiting is continuous, the child needs to drink two spoonfuls of liquid every five minutes, which is the equivalent of about 10 ml. An infant needs approximately 1 litre of liquid every 24 hours.
Oral solutions contain the amounts of salts and sugars required to help restore the body’s water balance. When children vomit often and have serious diarrhoea, it is a good idea for them to drink oral rehydration solutions. The infant may not want to drink the solution. If that is the case, try adding sugar.
You can find these solutions in pharmacies.
In most cases, it may be that the child stops vomiting but the diarrhoea continues for a while. Breastfeeding babies can continue feeding and babies drinking from a bottle can eat different types of preparations: rice, corn, formula milk, etc. depending on their age. You can find these preparations in pharmacies. You should start with small quantities.
If the child is older than six months, they may eat carrot soup.
When they are ready to start eating, it is better for them to eat normal foods. It is better to start with small quantities of food and avoid fruits and foods that have a fibre content.
If the child starts vomiting again and is more tired than usual, does not have enough energy to play or loses their interest in their surroundings, see a paediatrician.
Often, upset stomach is caused by a viral infection that is highly contagious. It is normal for people in the same family to be ill at the same time.
To prevent it from being passed on:
It is advisable for the child not to go back to nursery or school until two days after the symptoms disappear.
Gastroenteritis is an infection that causes diarrhoea, an increase in loose stools. It is normally accompanied by vomiting, fever and stomach ache.
Every time the child passes diarrhoea or vomits they lose fluids and they need to replace them orally (by drinking). To achieve this, electrolyte solutions can be used.
If the child is vomiting, they will need to drink the solution bit by bit (one teaspoon every 5 minutes). If they are not vomiting, increase the amount gradually.
When they are not vomiting, offer them small amounts of food. Never force them to eat and make sure they drink plenty of fluids between meals.
The child should not be fasting. Offer them food without forcing them to eat. Infants with gastroenteritis normally lose some of their appetite. If they are breastfeeding, the number of feeds should be increased. Milk bottles should continue to be given in the normal doses, they should not be diluted.
A dry diet is not necessary, soft foods can be eaten if preferred. The foods that tend to be tolerated better are cereals (rice and wheat), potatoes, bread, lean meat, vegetables, fish, yoghurt and fruit. Avoid foods that are difficult to digest, with lots of fat and sugar.
To recover lost fluids, do not use homemade solutions or commercial drinks. Solutions prepared specifically for rehydration are recommended.
Do not administer medications for vomiting or diarrhoea without consulting a paediatrician.
Fever is the body’s response against an infection and is the most common sign of illness. It is considered a fever when the body’s temperature is greater than 38°C at the rectum or 37.5°C in the armpit. Between 37 and 38 degrees is known as low-grade fever. A sensation of cold, discomfort and loss of appetite may all accompany a fever, but children normally tolerate fever well.
Fever is not an illness in itself, but the body’s defence response. Treatments for fever serve to alleviate the symptoms.
To find out if you have a fever, always use a thermometer. A digital thermometer is best. To use it, place it in the ill person’s armpit for approximately two minutes, until it beeps. In breastfeeding babies it can be placed in the rectum, but bear in mind that if done this way a fever is anything above 38°C.
If, despite having applied the above measures, the fever remains higher than 38ºC and the child is generally unwell, antipyretics may be administered.
The medication used most often is paracetamol or ibuprofen. Both can be administered in drops or syrup.
In summary, when we have an infection and the body raises our temperature, it causes what is known as a fever. Antipyretics do not cure the infection, but they can alleviate the symptoms.
Meningococcal disease (meningitis) is a serious infectious illness caused by a bacterium called “meningococcus”, which is transmitted through pharyngeal and nasal secretions.
The risk of contracting meningitis increases if you come into close contact with someone who has it (if you sleep in the same room, live in the same house, kiss on the cheek, etc.), but there is no need to disinfect objects or areas because meningococcus bacteria do not live long outside of the human body.
The measures to prevent this illness are:
With meningitis, the appropriate measure is to administer antibiotics to the family and other people who live with the affected person.
If the illness was caused by meningococcal group B, the only preventive measure, and the most common in Spain, is chemoprophylaxis, which aims to eliminate the microorganism from the pharynx. This stops it from spreading, in the space of a few days, between the people who have taken the medication.
If the illness was caused by meningococcal group C, as well as administering chemoprophylaxis, people close to the affected person will be vaccinated if they have not already been so.
If there is evidence that someone has had intimate contact with the patient in the ten days prior to the occurrence of the disease and they have not been administered chemoprophylaxis, healthcare staff must be informed.
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