Research Training Fellowship: Dr Sally Johnson.
This research was completed on 22 February 2008
Published on 22 October 2005
Helping children with kidney failure
Each year, Action Medical Research awards these prestigious grants to help the brightest and best doctors and scientists develop their career in medical research. Dr Johnson's grant of £131,633 will fund her two-year study into a devastating form of kidney failure in children.
Dr Sally Johnson is a talented young doctor committed to caring for children with kidney disease. She's made an outstanding start to her career, but a dire lack of treatment options limits how much she can help some of her young patients.
Many children are confined to a harsh regime of regular dialysis, others have kidney transplants that can turn out to be unsuccessful and, tragically, some children can lose their life to their illness. So Dr Johnson is thrilled that this fellowship will allow her to carry out some much-needed research.
With this fellowship, I hope to make some real steps towards finding a treatment for children with a devastating form of kidney disease, called atypical HUS, and making a difference to the lives of sufferers and their families.Dr Sally Johnson
Kidney failure devastates children's lives
In this fellowship, Dr Johnson is focusing on a rare but destructive kidney disease called haemolytic uraemic syndrome (HUS). This condition typically starts in very young children - babies and toddlers who are just starting out in life - with many going on to develop permanent kidney failure. Sadly, some children lose their life to HUS.
There is no cure for young patients with HUS. Even if a suitable kidney donor can be found, and a child has a transplant, the disease often returns in the new kidney.
‘For many children with this condition life is dominated by dialysis, harsh dietary restrictions and frequent hospital stays.’ explains Dr Johnson.
This strict regime complicates day-to-day life, yet many children face up to their illness well, bravely striving to live as normal a life as possible. Dr Johnson is just as determined as her young patients, and hopes her fellowship will ultimately lead to better treatment for children with kidney failure. 'I am glad that Action Medical Research has recognised that this is a tremendously important area of research,' says Dr Johnson.
Faults in the immune system can damage kidneys
Some people with HUS have faulty immune systems, which attack their own kidneys. Dr Johnson is studying people who have inherited a fault in particular protein, called factor H. Seventy percent of people who have HUS because of faulty factor H develop permanent kidney failure, and at least 30% of those who have transplants suffer a recurrence of the disease.1
Dr Johnson plans to find out how faults in factor H lead to kidney failure. She is thrilled that her fellowship will allow her to carry out these long-needed studies: 'I can now concentrate on the matter in hand - understanding a complex and interesting disease that devastates the lives of young children and their families.
|Project Leader||Dr Sally Johnson|
|Location||Department of Renal Immunobiology, University of Birmingham, Birmingham|
|Grant awarded||22 July 2005|
|Start date||1 October 2005|
|End date||22 February 2008|
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Working towards new treatments for children with kidney disease
Dr Johnson hopes this fellowship will lead to detailed new information on the disease process in HUS. The ultimate aim is to use this information to develop a new treatment for children with HUS that stops the immune system from damaging the kidneys.
This exciting new treatment could have the potential to improve children's lives dramatically - it might help stop them from developing kidney failure and free them from dialysis.
What's more, although HUS is rare, the knowledge gained from Dr Johnson's work may well help children with other forms of kidney disease. Problems with the immune system are a major cause of permanent kidney failure in children - causing around one quarter of cases 2 - so even more children might benefit in the future.
This fellowship will also help Dr Johnson achieve her long-term personal goal. 'I would like to be a clinician scientist in paediatric nephrology. That is, a doctor who looks after children with kidney disease who also leads research into the conditions that affect those children,' explains Dr Johnson. By giving such a bright and dedicated young doctor the chance to train, this fellowship should bring benefits that continue for many years to come.
People with HUS develop kidney failure because of damage caused by their own immune systems.
Our immune system normally attacks and destroys bugs and viruses that invade our body.
In HUS, one part of the immune system, called complement, becomes confused, and unleashes its attack on blood vessels in the patient’s kidneys.
Damage to the microscopic filters within the kidney causes kidney failure.
In healthy people, a protein called factor H binds to the lining of blood vessels in the kidney and protects them from attack by the immune system.
Previous studies suggest people with HUS have mutant factor H, which doesn’t stick properly to the lining of blood vessels and doesn’t stop the immune system’s attack.
But exactly how does this lead to HUS? And why are the blood vessels of the kidney targeted and not those in the rest of the body? I aim to address these questions in my fellowship.Dr Sally Johnson
In her search for answers, Dr Johnson is using blood vessel cells and faulty factor H to investigate how the lining of blood vessels is damaged.
She is investigating how well and where the faulty factor H sticks to blood vessels in comparison to normal factor H.
Dr Johnson is also comparing the blood vessels of the kidney with those from other parts of the body to see why the kidney is targeted by this disease.
- Loirat C, Niaudet P. The risk of recurrence of hemolytic uremic syndrome after renal transplantation in children. Pediatr Nephrol 2003; 18(11):1095-101.
- Loirat C ME. Haemolytic Uraemic Syndrome. In: P C, editor. European Society for Paediatric Nephrology Handbook. Lyon: Medcom, 2002:337-342.