Nephrology and kidney transplantation FAQs
- What is acute renal failure?
- What is Alport syndrome?
- What is chronic kidney disease (CKD)?
- What is end-stage kidney disease or end-stage renal disease (ESRD)?
- What is hematuria?
- What is hemodialysis?
- What is hemolytic-uremic syndrome?
- What is IgA nephropathy?
- What are kidney stones?
- What is lupus?
- What is multicystic kidney disease?
- What is nephritis?
- What is nephrotic syndrome?
- What is peritoneal dialysis?
- What is polycystic kidney disease?
- What is proteinuria?
- What is renal tubular acidosis?
- What is the urinary tract?
- What is vesicoureteral reflux (VUR)?
- What is Wilms tumor?
Acute renal failure is a sudden cessation of kidney function. This potentially life-threatening condition can result from dehydration, injury, obstruction to the kidney, infection, or other causes. Some acute kidney failures can be treated with medications, such as antibiotics if the kidney failure is due to infections. If kidney failure becomes very severe, dialysis maybe sometimes necessary to get rid of excess wastes that build up.
Alport syndrome, also known as “hereditary nephritis” is a rare inherited disorder that can affect not only the kidneys, but also hearing and vision. Named after the physician who first documented it, the syndrome is characterized by degeneration of kidney function and accumulation of fluids and wastes, leading to end-stage kidney disease at an early age. The condition generally affects males more severely than females. Children with Alport syndrome may initially experience leakage of small amounts of blood or protein into the urine. Although no specific treatment exists, Alport syndrome is best managed by controlling high blood pressure and using kidney protective medications called ACE (angiotensin converting enzyme) inhibitors and ARBs (angiotensin receptor blockers).
CKD refers to the permanent impairment of kidney function. The term CKD replaces older terms CRF (chronic renal failure) and CRI (chronic renal insufficiency), both of which were based on the severity of kidney impairment. When loss of renal function is severe and results in impairment of other organ systems, dialysis or transplantation may be considered and a patient is said to have progressed to end-stage renal disease (ESRD). Both CKD and ESRD are uncommon in children: the annual incidence for both is less than 15 per million population in North America. CKD in children typically results from congenital kidney defects, damage caused by kidney infections and other diseases including polycystic kidney disease, Alport syndrome, lupus, nephrotic syndrome and kidney stone formation. CKD, when advanced, allows waste products to accumulate in the body. Symptoms include weight loss or excess weight gain, fatigue, decrease in urine output, loss of appetite, swelling of the body, increased tendency to bruise or bleed, paleness, seizures, confusion or even coma. Sometimes ‘uremic poisoning’ is indicated by presence of white crystals on the skin. Although CKD has no cure, its symptoms may be controlled through careful management, using dietary measures, life style changes, and medications to control high blood pressure and the use of ACE inhibitors and ARBs.
A child whose kidney function has ceased is said to have end-stage kidney disease. To survive, the child either must undergo a kidney transplant or receive dialysis.
Hematuria is the medical term for the presence of blood in the urine—an abnormal condition that in children indicates kidney or urinary tract disease, other illnesses or injury. Visible blood in the urine is likely the result of a serious medical condition requiring immediate attention.
Hemodialysis is a process by which an artificial kidney called a “hemodialyzer” mechanically purifies the blood. Hemodialysis is performed in a medical facility. It removes impurities more efficiently than peritoneal dialysis. A hemodialysis treatment typically requires about 3 hours and is generally performed three times a week. The duration of hemodialysis episodes depend upon the degree of damage to the kidneys, amount of excess fluid present in the body and body weight.
Hemolytic-uremic syndrome (HUS) is the most common cause of acute (sudden) kidney failure in children. It most commonly occurs following a gastrointestinal infection by E. coli bacteria, which can contaminate undercooked foods. The disease destroys red blood cells, so patients with HUS become pale and anemic. Because the condition can be life-threatening, doctors may prescribe kidney dialysis or blood transfusions. With effective treatment, most patients recover.
IgA nephropathy, also known as “Berger’s disease” in honor of the physician who identified it, is a disorder that results in abnormal deposits of a type of protein called “IgA” within the kidneys. These IgA deposits damage the glomeruli, which are the filtering tissues within the kidney. It is often detected when children develop dark or bloody urine, and it can sometimes lead to nephrotic syndrome. Diagnosis is confirmed by a kidney biopsy that reveals the presence of IgA in the kidney tissue. While the disease in benign in most children, about 25% will develop end-stage renal failure within about 25 years. Treatment options include diet modification and medications (fish oil, ACE inhibitors, ARBs, and steroids).
Formation of kidney stones, a condition called nephrolithiasis, results from buildup of salts within the kidney or bladder. As they enlarge into stones these salt crystals can clog the ureter, a narrow duct between the bladder and the kidney, with severe, often intensely painful consequences. Patients may succeed in expelling the stone through the urinary tract. However, a stone that is blocking the flow of urine or causing an infection or damage to the kidney may require surgical removal. Since children who have formed 1 kidney stone are at risk of forming more kidney stones in the future, they all need to be evaluated by a kidney specialist. Simple measures, such as diet modification, increased fluid intake and occasionally the use of medications, can prevent recurrent stone formation.
Lupus is the term often used to refer to systemic lupus erythematosus (SLE), a chronic autoimmune disorder that can involve the skin, joints and internal organs. Females are more likely than males to develop lupus. The immune system in a child who has lupus cannot distinguish between invasive substances and the body’s own cells. Consequently the immune system triggers production of antibodies that target the patient’s own cells, resulting in rashes and pain in the joints. Lupus also can cause damage to the kidneys, which if persistent can lead to renal failure requiring dialysis or kidney transplantation. The effects of the disease can be mild and intermittent, or can be severe and potentially fatal. Lupus has no cure, but medical treatment can suppress the disease.
Multicystic kidney disease (MCDK) is a congenital kidney disease, most often diagnosed on a prenatal ultrasound, in which the kidney is non-functional and resembles a cluster of grapes, unlike the “kidney bean” shape of a normally developed organ. It is usually present only in 1 kidney. Children with MCDK on one side often have kidney abnormalities on the other side and therefore need to be seen by a kidney specialist. If the other kidney is normal, these children have a normal life with no need for medications.
Nephritis is an inflammation or bruising of tissues within the kidney, which is most commonly the result of a recent strep infection (of the throat or skin). Other less common causes include lupus, IgA nephropathy hepatitis B and hepatitis C. Children with nephritis present with ‘tea’ colored urine, puffy face and decreased urine output. Some may develop high blood pressure and acute renal failure, needing dialysis. Most children with strep-related nephritis get better on their own and have no long-term kidney damage or problems.
Childhood nephrotic syndrome, which also is called "nephrosis" is a condition characterized by loss of large amounts of protein through the urine. That results in a corresponding loss of necessary protein from the blood and a buildup of water within body tissues known as "edema". Edema becomes evident in swelling around the eyes, the abdomen or in the legs. Nephrotic syndrome is usually successfully treated with a medication called prednisone. Fortunately, treated nephrotic syndrome usually does not cause permanent kidney damage, and the condition may disappear at the onset of adolescence. If prednisone does not work, a kidney biopsy may be needed to find out exactly what is going on in the kidney so that the best treatment can be decided upon.
Peritoneal dialysis is a blood cleansing process which, unlike hemodialysis, can be performed at home or even during normal activities. The process involves passing fluid through an area in the abdomen called the peritoneal cavity, where the blood is cleansed. One approach, called continuous ambulatory peritoneal dialysis (CAPD), is performed without machines. The patient squeezes a special “dialysate” solution through a catheter into the cavity, where it remains for several hours while it attracts and collects contaminants. While the dialysate cleanses the blood, the patient can go about normal activities, including working and walking—which is why it’s called “ambulatory.” After the prescribed period of time for the “exchange”, the dialysate is drained out of the body into the bag and discarded. Another approach, called continuous cycling peritoneal dialysis (CCPD), is also performed at home but involves use of a machine called a “cycler,” which automatically completes several exchange cycles. That process typically is performed throughout the night while the patient is asleep.
Polycystic kidney diseases, known as PKD, are inherited disorders characterized by formation of large numbers of fluid-filled cysts within the kidneys. Symptoms include high blood pressure, pain in the back or sides, and presence of blood in the urine. The cysts can interfere with proper function of the kidneys, potentially leading to kidney failure. Although no cure for PKD exists, treatment of other conditions associated with it—such as high blood pressure or bladder infections—can help minimize damage and prolong life. Children with the ‘recessive’ form of PKD (ARPKD) also have liver problems and may need a liver transplant in addition to a kidney transplant.
Proteinuria is the medical term for excess protein in the urine. Protein structures are too large to pass through the normally functioning kidneys, so presence of protein in the urine may indicate an improperly functioning or damaged kidney.
Renal tubular acidosis (RTA) is a disease which affects the ability of the kidneys to regulate the amount of acid in the body. Left unchecked, excess acidity causes bone calcium to dissolve, resulting in rickets, growth impairment, skeletal deformities and muscular weakness. Complications of RTA can be life-threatening, but the condition can be very easily treated with potassium citrate, sodium bicarbonate or other alkaline medications to neutralize the acidity.
The urinary tract encompasses the organs and ducts involved in production and expulsion of urine from the body—the bladder, the two ureters (ducts) leading from the kidneys to the bladder, and the urethra (the tube through which urine is expelled from the body). The urinary tract can become infected by bacteria, causing irritation, swelling and pain that requires treatment by antibiotic medication. Girls are more likely than boys to contract a urinary tract infection (UTI). Treatment of UTIs is important to avoid spread of the infection to the kidneys, which can lead to permanent kidney damage.
VUR is a condition in which urine moves backwards from the bladder into the kidney. VUR is most often looked for in the setting of a kidney infection being diagnosed in a child. It is detected with a test called the voiding cystourethrogram (VCUG). This study involves putting a contrast material using a catheter into the urinary bladder. The catheter is then removed and the child is asked to urinate while x-rays are being taken. Children who have VUR have high risk of developing kidney infections. Most children with VUR, especially low grades of VUR improve on their own. Preventative antibiotic treatment is strongly recommended for all patients with VUR detected in the setting of a kidney infection. Once you have documented resolution of VUR, medications may be safely discontinued. Some children with reflux may be candidates for surgical correction, which corrects the reflux in more than 95% of cases.
What is Wilms tumor?
Wilms tumor is a cancerous growth that involves the kidney or other abdominal organs. Children under 3 years of age are most susceptible, and it is rarely seen in children older than age 8. These tumors often can be removed surgically, but treatment may include radiation or chemotherapy as well. The cure rate for this disease is about 90 percent with proper and timely treatment.