Childhood Nephrotic Syndrome What is nephrotic syndrome? A child with nephrotic syndrome has these signs: ephrotic syndrome is a set of signs or syTremptomsatment that may Methigh levhodels of prosteinfor in the urine, a point to kidney problems. The condition called proteinuria kidneysN are two bean-shaped organs low levels of protein in the blood found in the lowerK bacidnek. Each isy aboutFa ilure in Children the size of a fist. They clean the blood swelling resulting from buildup of by filtering out excess waidneyster plaandy a snaltimpor andtan t part sinalt andProb waterlem s Specific to Children waste products from food.achild Hea’s growtlthyh andkid- health. Everyone who has kidney failure, adults less frequent urination neys keep proteinKin theThey blood, which and children alike, will experience med- ical complications, which may include helps the blood soakremovup wae waterste fsromand extis-tra water fromweight gain from excess water extreme fatigue, inability to concen- sues. But kidneys withthe bldamoodaged filters Nephrotictrat synde, wromeeak bo nesis no, nervet itse dalfmage, a dis- may leak protein intoregula thete urine. blood preAsssuare depression, and sleep problems. Addi- ease. But it can be the first sign of a result, not enough protein is left in the tional problems for children can include balance chemicals like sodiumdiandsease that damages the kidney’s tiny blood to soak up thep otassiumwater. The water blood-filtering units, called glomeruli, then moves from the blood into body make a hormone that signals bownehere urine is made. tissues and causes swemarrowlling. to make red blood cells Both children and admakeults acan hor monehave to help bones grow and keep them strong How is childhood nephrotic nephrotic syndrome. The causes of and treatments for nephroKidneytic failu synredrom can leade in directly tos yndromemore diagnosed? children are sometihealthmes pr diffoblemeres,nt like from swelling of Ttheo diagnose childhood nephrotic syn- body, bone deformities, and growthdrome fail- , the doctor may askKidneys for a urine the causes and treatmeure. Ants successful in adu kidnlts. eyF ortran splant can information aboutgive ne aphrchildotic wi syndroth chronmeic kid ney faisaluremple to check for protein. The doc- tor will dip a strip of chemically treated in adults, see the Ntheatio bestnal cha Institnce toutegrow of normally and Ureters Diabetes and Digestleadive a full, and a ctiveKidn lifeye. Dis- Dialysis capnaperhelp into the urine sample. Too much a child to survive an acute episode of eases (NIDDK) publication Nephrotic protein in the urine will make the paper kidney failure or to stay healthy untchangil a e color. Or the doctor may ask for Syndrome in Adultsdonat. ed kidney becomes available. a 24-hour collection of urine for a more Childhood nephrotFamiliesic synd carromeing fo rcana chi ld with kidnpreceyise measurement of the protein and occur at any age butdise aseis mo oftenst ncommoneed help—not just from doctors and nurses, but from a woholether substanceBladders in the urine. between the ages of 1½ and 5 years. team of pediatric specialists, including It seems to affect dboysietitia mons, socire oftal wenorke rs, and family than girls. counselors. Learning about treatments for kidney disease and getting to know The kidneys remove wastes and extra water from the the entire team can make life easier for blood to form urine. Urine flows from the kidneys to your child and your entire family. the bladder through the ureters. U.S. Department ofN Healthational Institute ofNATIONAL Diabetes and INST DigestiveITUTES and Kid ney Diseases U.S. Department of Health and Human ServicesNATI ONAL INSTIOFTUTES HEALTH OF HEALTH and Human Services Phone: 800.633.6628 • www.kidneyurology.org The doctor may take a blood sample to see What conditions are associated with how well the kidneys are removing wastes. Healthy kidneys remove creatinine and urea childhood nephrotic syndrome? nitrogen from the blood. If the blood contains Minimal Change Disease high levels of these waste products, some kid- The condition most commonly associated ney damage may have already occurred. But with childhood nephrotic syndrome is mini- most children with nephrotic syndrome do not mal change disease. Doctors do not know have permanent kidney damage. what causes it. The condition is called mini- In some cases, the doctor may want to examine mal change disease because children with this a small piece of kidney tissue with a micro- form of the nephrotic syndrome have normal scope to see if something specific is causing or nearly normal appearing kidney biopsies. the syndrome. The procedure of collecting a If a child is diagnosed with minimal change small tissue sample from the kidney is called a disease, the doctor will probably prescribe biopsy, and it is usually performed with a long prednisone, which belongs to a class of drugs needle passed through the skin. The child will called corticosteroids. Prednisone stops the be awake during the procedure and receive movement of protein from the blood into the calming drugs and a local painkiller at the site urine, but it does have side effects that the doc- of the needle entry. A child who is prone to tor will explain. Following the doctor’s direc- bleeding problems may require open surgery tions exactly is essential to protect the child’s for the biopsy. General anesthesia will be used health. The doctor may also prescribe another if surgery is required. For any biopsy proce- type of drug called a diuretic, which reduces dure, the child will stay overnight in the hos- the swelling by helping the child urinate more pital to rest and allow the health care team to frequently. address quickly any problems that might occur. When protein is no longer present in the urine, the doctor will begin to reduce the dosage of prednisone. This process takes several weeks. Some children never get sick again, but most experience a relapse, developing swelling and protein in the urine again, usually following a viral illness. However, as long as the child continues to respond to prednisone and the urine becomes protein free, the child has an excellent long-term outlook without kidney damage. Children who relapse frequently, or who seem to be dependent on prednisone or have side effects from it, may be given a second type of drug called a cytotoxic agent. The agents most frequently used are cyclophosphamide and chlorambucil. After reducing protein in the urine with prednisone, the doctor may pre- A strip of chemically treated paper will change color when scribe the cyclophosphamide or chlorambucil dipped in urine with too much protein. for 8 to 12 weeks. Alternatively, cyclosporine, 2 a drug also used in transplant patients, may be Congenital Nephropathy given. Treatment with cyclosporine frequently Rarely, a child may be born with congenital continues over an extended period. nephropathy, a condition that causes nephrotic In recent years, doctors have explored the syndrome. The most common form of this use of mycophenolate mofetil (MMF) instead condition is congenital nephropathy of the of cytotoxic agents for children who relapse Finnish type (CNF), inherited as an autosomal frequently. MMF is an immunosuppressant recessive trait—meaning the gene for CNF used to treat autoimmune diseases and to keep must be inherited from both parents. the body from rejecting a transplanted organ. Another condition that causes nephrotic syn- MMF has not been tested for treating minimal drome in the first months of life is diffuse change disease in large clinical trials, but doc- mesangial sclerosis (DMS). The pattern of tors report promising results with small num- inheritance for DMS is not as clearly under- bers of patients. MMF has milder side effects stood as the pattern for CNF, although the than cytotoxic agents, but taking immunosup- condition does appear to be genetic. pressants can raise the risk of infection and Since medicines have little effect on congeni- other diseases. The good news is that most tal nephropathy, transplantation is usually children outgrow minimal change disease by required by the second or third year of life, their late teens with no permanent damage to when the child has grown enough to receive a their kidneys. kidney. To keep the child healthy, the doctor may recommend infusions of the protein albu- Focal Segmental Glomerulosclerosis min to make up for the protein lost in urine (FSGS) and Membranoproliferative and prescribe a diuretic to help eliminate the Glomerulonephritis (MPGN) extra fluid that causes swelling. The child’s In about 20 percent of children with nephrotic immune system may be weakened, so antibiotics syndrome, a kidney biopsy reveals scarring or should be given at the first sign of infection. deposits in the glomeruli. The two most com- mon diseases that damage these tiny blood- Congenital nephropathy can disturb thyroid filtering units are FSGS and MPGN. activity, so the child may need the substitute hormone thyroxine to promote growth and Because prednisone is less effective in treat- help bones mature. Some children with con- ing these diseases than it is in treating minimal genital nephropathy have excessive blood change disease, the doctor may use additional clotting, or thrombosis, which must be treated therapies, including cytotoxic agents. Recent with a blood thinner like warfarin. experience with another class of drugs called ACE inhibitors, usually used to treat high A child with congenital nephropathy may need blood pressure, indicates these drugs can help tube feedings to ensure proper nutrition. In decrease the amount of protein leaking into some cases, the diseased kidneys may need to the urine and keep the kidneys from being be removed to eliminate proteinuria. Dialy- damaged in children with FSGS or MPGN. sis will then be required to replace kidney function until the child’s body is big enough to receive a transplanted kidney. Peritoneal dialysis is preferable to hemodialysis for young children.