Specific kidney disorders
There are many causes of chronic kidney failure which can lead to the need for dialysis. These can be diagnosed by a variety of blood tests, X-rays and perhaps, a kidney biopsy. In all of these conditions, high blood pressure is a major factor causing progression of the problems and needs to be controlled very carefully (140/80 or less). All of the problems associated with kidney failure may develop and need to be managed carefully in the follow-up clinic.
This is a painless inflammation of the part of the kidney surrounding the renal tubules, rather than the glomerulus. It is related to the effects of urine infection spreading up to the kidney and the most damage is done by infections early in childhood. The problem tends to run in families and can be diagnosed by the presence of scars in the kidneys on an ultrasound scan. Not all patients with chronic pyelonephritis will progress to more severe kidney damage - high blood pressure and the passing out of large amounts of protein in the urine tend to be associated with progression.
Glomerulonephritis can develop in an acute (sudden, rapid onset) or a chronic (slow) form and is caused by a painless inflammation of the glomerulus. It can occur by itself eg IgA nephropathy or as part of a more general problem such as systemic lupus erythematosus (SLE) or ANCA associated vasculitis. Testing of the urine shows the presence of blood and protein. It is associated with high blood pressure and progressive decline of kidney function. It is diagnosed by blood tests and a kidney biopsy where the pathologist describes the appearances - many different types are described which have specific associated problems. Some of these include - proliferative glomerulonephritis, mesangio-capillary glomerulonephritis and focal segmental glomerulosclerosis. One of the commonest is IgA glomerulonephritis which presents often with blood in the urine, sometimes visibly at the time of a throat infection. Some patients with IgA glomerulonephritis will progress to more severe damage - once again, high blood pressure and presence of protein in the urine may predict the tendency to progression. Many patients with IgA glomerulonephritis will simply need to be followed up each year in the out-patient clinic.
Specific drugs may be used to try and dampen down the inflammation, particularly steroids (prednisone) and drugs such as azathioprine, mycophenolate, rituximab, cyclosporin, tacrolimus and cyclophosphamide.
Polycystic kidney disease
This is a very common (1 in 400 to 1 in 1000 people) genetic problem leading to the development of large kidneys which are enlarged by presence of cysts. These cysts can also develop in other organs such as the liver and the pancreas. There is a variable expression of the problem within families and not everybody with polycystic kidney disease will develop kidney failure - maybe 50-75% will progress. The genes responsible for this disease have been found. Often polycystic kidney disease will not cause anaemia as the kidneys still produce erythropoeitin. Polycystic kidney disease can present with loin pain, urinary infections and blood being observed in the urine.
Kidney damage develops in about 20% of patients with both type 1, young onset, insulin-dependent diabetes and type 2, older onset, non-insulin dependent diabetes. Problems start after 10 to 15 years of diabetes and the kidney problems are associated with diabetic eye disease. Poor control of the blood glucose and high blood pressure are factors which lead to this problem and make it worse once it starts. Blood pressure lowering, particularly using ACE inhibitors (drug names end in -pril)or angiotensin receptor blockers (drug names end in -sartan), at the earliest stages can have a major impact on reducing the progression of the kidney disease. In the diabetic clinic the urine will be tested for tiny amounts of protein and treatment begun at this stage.
Renal artery stenosis / renovascular disease
A narrowing of the kidney blood vessel will lead to the kidney being starved of a good enough blood supply. This leads to the kidney becoming smaller - often it presents with high blood pressure - and if in both kidneys will lead to kidney failure and sometimes a tendency to pulmonary oedema, fluid in the lungs causing breathlessness. The problem may be suggested by a kidney scan which shows one kidney is smaller than the other but can only be diagnosed with certainty by a special X-ray of the kidney blood vessels (an angiogram). Sometimes it may be felt that the narrowing should be stretched open using a fine, narrow balloon (angioplasty) and then kept open with a tube inserted into the blood vessel (a stent). All of this can be done from the "inside" during a procedure similar to an angiogram. In everybody with this problem, the blood pressure will be controlled, the cholesterol level checked and normalised and aspirin may be given.
If there is an obstruction to the kidneys then there could be development of kidney failure - this could occur at different levels of the drainage systems of the kidneys - from the junction of the kidney pelvis and the ureter (PUJ) to the outflow from the bladder. Often the problem is made worse by the tendency to get infections in association with the problem. It is diagnosed by an ultrasound scan demonstrating obstructed kidneys (hydronephrosis) and poor drainage on an IVU or an isotope renogram. Surgery may be needed to improve the drainage of the kidneys or remove kidney stones.
Unfortunately it is still not uncommon for patients to present with chronic kidney failure and usually hypertension - when the kidneys are scanned they are small. Something has been going on the kidneys for a long time but all of the tests, even a kidney biopsy cannot tell what the exact starting problem was and no specific treatment will make a difference. Blood pressure control and treatment of the problems associated with kidney failure are the keys to managing this condition.
This is not a single underlying disease but develops alongside other processes such as a glomerulonephritis or with diabetes. The kidneys pass out large amounts of protein (the urine may be "frothy") into the urine and this means the water in the blood stream passes into the tissues, particularly the feet and around the eyes. Usually it is caused by a glomerulonephritis such as minimal change nephropathy (the commonest in children) or membranous glomerulonephritis (commonest in adults). The kidney function may be absolutely normal and the only problem is the protein leak - minimal change nephropathy in particular, very very rarely leads to kidney failure. Treatment is by restricting the fluid intake, using diuretics and perhaps aspirin and cholesterol lowering drugs. Focal segmental glomerulosclerosis is a much more challenging cause and often leads to kidney failure. A kidney biopsy will be done to determine the exact nature of the problem and to decide if treatment with steroids or other drugs is needed.