Children and families |
Childhood nephrotic syndromeMost people have two kidneys. The kidneys main function is to clean the blood, getting rid of waste products and excess water. Blood filters through the kidneys, which act like a sieve, allowing waste products and excess water to be excreted as urine, but prevents loss of essential blood components such as protein and blood cells. In healthy kidneys this process controls the body’s water and salt balance and regulates blood pressure. In childhood nephrotic syndrome there is a problem affecting the sieve mechanism of the kidney. The holes of the sieve enlarge, allowing large amounts of protein to be lost from the body in the urine (proteinuria). Contents
What causes nephrotic syndrome?In most cases there is no known cause of nephrotic syndrome but research is beginning to provide us with more information about the actual changes in the kidney. Childhood nephrotic syndrome is an uncommon condition. Every year approximately two to seven children in every 100,000 develop nephrotic syndrome. It tends to be more common in the Arab and Asian populations and in families with a history of allergies. Nephrotic syndrome often starts between the ages of two to five years, affecting twice as many boys as girls. What are the symptoms of nephrotic syndrome?Abnormally large quantities of protein are lost in the urine so as a consequence, the level of protein in the blood drops. Proteins in the blood usually keep fluids within the blood stream. When they are lost in the urine, fluid can leak into the body tissues causing swelling (oedema). Swelling is noticeable especially around the eyes and lower legs, but can lead to swelling all over the body including inside the body around the intestines and stomach, which can make the abdomen swell and cause vomiting and diarrhoea. How is nephrotic syndrome diagnosed?A simple urine test will show high levels of protein present whilst low levels of protein will be identified in the blood. In a few cases it will be necessary for a kidney biopsy to take a very small piece of tissue from your child’s kidney to be looked at under a microscope to find a cause and make a diagnosis. Mostly this is not necessary especially if they respond well to treatment. How is nephrotic syndrome treated?Steroid medication (prednisolone) will be prescribed for your child. Most children with nephrotic syndrome respond well to steroid treatment. The protein disappears from the urine and then the swelling is lost within a week or two. This is called remission. Some children take longer than others to go into remission. The term remission is used rather than cure because protein can return in the urine and swelling can come back. This is known as a relapse. Most children will relapse once or several times. Each relapse requires more steroid treatments. Sometimes coughs and colds can trigger a relapse. Very occasionally water tablets (diuretics) and protein infusions are given to control the oedema before the steroids have worked. What should I know about the steroid treatment?When taking steroid treatment your child should carry a blue steroid card at all times. If the child becomes ill, requiring a doctor, the card gives essential treatment details. Your child will require a course of high dose steroids with each relapse. Not all children experience side-effects from steroid treatment and any effects are temporary and will stop when the course of steroids has finished or reduced to low alternate day doses. Some common side-effects are listed:
For more in-depth information ask the hospital pharmacy for the Short-term steroid treatment factsheet. What do I have to do at home?You need to test your child’s first urine for protein every morning unless told otherwise. Normally the testing strips will be Albustix®. There is no need to test the urine for other substances at home, as this will be done when you attend out-patient clinic. It is essential you keep a diary of all your child’s urine results for each day and bring it with you to each clinic visit. This is very important as it enables the doctor or nurse to review your child’s progress. A diary is available from out-patients clinic or if you have a computer, you may wish to make your own. The doses of prednisolone given each day and any other treatment should be recorded next to the daily urine result. You may also wish to include any other comments, for example, their appearance or how they are feeling. The urine results in the diary will show how your child is responding to treatment or when they have relapsed. Records that are complete and accurate give the doctor or nurse a good idea of your child’s general health and progress. If your child shows ‘protein 3+’ or more for three days in a row then this could mean there is a relapse and you should contact your doctor or nurse. If they have no protein (negative) or trace for three days in a row they are in remission and should be well. What other treatment may be necessary?Some children with nephrotic syndrome relapse more frequently and begin to have side effects from the steroids. Other treatments that may be considered are:
These treatments may help your child have longer periods of remission and therefore reducing the amount of steroids taken. There are a small group of children with nephrotic syndrome that do not respond to steroid treatment and continue to have lots of protein in their urine. It is these children who usually require a kidney biopsy to investigate what is happening inside the kidney. Cyclophosphamide may help these children go into remission. The doctor will always discuss the need for further treatment or investigations before a biopsy or other medications are suggested. Further information is also available on these medications from the hospital pharmacy factsheets or GOSH website www.goshfamilies.nhs.uk . Is there a special diet?You need to encourage your child to eat a balanced healthy diet. It is a good idea for all members of the family to also follow a healthier eating plan. A ‘no added salt’ diet is encouraged especially when they are in a relapse. Avoid adding salt to food at the table and try to reduce the intake of processed foods for example, crisps, tinned and packet foods. Information on this is available if required. Can my child lead a normal life?Yes. In the past parents have tended to keep children off school when they have relapsed. This leads to a lot of missed schooling and their education has suffered as a consequence. In nearly all cases you do not need to keep your child off school, even when they relapse. We actively encourage school attendance unless they are really unwell with symptoms. There is no need to restrict their activity. Exercise is important for all the family. A full level of activity is possible when in remission and many children continue to manage a full range of sports and activities even in a relapse. What about childhood illnesses?Proteins are used in the body for growth and repair and form part of the immune system. When your child has heavy protein loss in the urine they will be more susceptible to infections. It is therefore important when they are in a relapse, they take a preventative course of antibiotic (usually penicillin V) until they are in remission again. If your child develops diarrhoea and/or vomiting while taking any of the medications contact your doctor or nurse. The steroid, cyclophosphamide or ciclosporin treatment suppresses the child’s immunity. They could be very unwell if in contact with chicken pox or measles. Let the hospital know if your child has been in close contact with another child with chicken pox or measles. Speak to your child’s school about the possibility. What about immunisations?Steroids can reduce the effect of immunisations and your child may be unwell or relapse if immunisations are given without medical advice. If your child is due immunisations please discuss this with your doctor.
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UCL Institute of Child Health
Great Ormond Street Hospital for Children NHS Trust
