PKD

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Nephrotic Syndrome

Nephrotic syndrome is a disorder of the kidneys which is characterized by high protein levels in the urine, high lipid (cholesterol and triglyceride) levels in the blood, low albumin (a blood protein) levels in the blood, and generalized edema (swelling). This clinical state can occur as a primary disease, as a secondary disorder resulting from damage to the glomeruli of the kidney, or as a congenital disorder resulting from an inherited gene.

Etiology or Causes

Nephrotic syndrome is characterized by increased glomerular membrane permeability to blood proteins. This means that the large protein molecules are able to pass thru the membrane and spill into the urine.  The nephrons,are tiny organs that are responsible for filtering the blood to form urine. Inside each nephron is the glomerulus. The glomerulus is a network of capillaries that form the first step of the filtering process. With nephrotic syndrome, that membrane becomes damaged. Proteins leak into the urine, which results in generalized swelling and this causes weight gain.  There are a number of conditions that cause that damage. However, in children, the most common condition is known as minimal change disease. Other conditions that may lead to nephrotic syndrome are diseases like diabetes, some cancers, genetic disorders, immune disorders, infections, certain drugs, and even certain kidney disorders.

Signs and Symptoms

            The most common symptom related to this disorder is generalized edema or swelling. You will see puffiness of the face, particularly around the eyes. It tends to be more obvious in the morning when the child wakes up, but it may subside as the day progresses. Other areas of the body that have a tendency to swell are the abdomen (ascites), genitals, legs, ankles and feet. Edema of the lining of the intestine, a more covert symptom, can lead to diarrhea, loss of appetite, and malabsorption of nutrients in the intestines. You may also notice your child experience weight gain, become more easily fatigued, lethargic, and irritable. Their urine output will decreased in amount and frequency, and may appear frothy and darker.

           

Diagnostic Test

            A physical examination is the first step in making a definitive diagnosis. After the exam, your doctor will order lab tests (blood and urine) to determine how well the kidneys are functioning. The urinalysis will show high levels of protein. The blood work will show elevated cholesterol and triglyceride levels and low albumin levels.  Serum (blood) creatinine level and creatinine clearance (a urine test) are routinely ordered.  A kidney biopsy may be ordered to find out the cause of the disorder. There are other tests your doctor may also order to make the diagnosis or to rule out other disorders.

Treatment

The goal for managing nephrotic syndrome is fourfold. It includes 1) reducing the excretion of urine protein, 2) preventing infection, 3) reducing the swelling which is a result of fluid retention and lastly, 4) minimizing the complications secondary to the treatment.  A low salt diet is highly recommended as well as restricting the amount of fluid your child drinks.  Medication such as Prednisone, (a corticosteroids),is the first line of therapy in treating this disorder. If your child does not respond to steroids or if the side effects outweigh the benefits, then other immunosuppressant medications will be considered.   Since these drugs suppress the immune system you will need to monitor your child for signs of infection. These kids are particularly vulnerable to upper respiratory infections. Some symptoms to be looking for are frequent cough, sore or reddened throat, runny nose, and fever.  Diuretics are used to get rid of excess fluids and reduce swelling.  The diuretics can be an adjunct therapy to albumin infusions. It is wise to closely monitor their intake and output because the use of diuretics can lead to dehydration. Some diuretics are known to deplete the blood potassium levels because potassium is excreted in the urine.  For that reason, a potassium supplement should be ordered by your doctor.  Some children experience high blood pressure with this disorder, so blood pressure medication may be necessary.  Checking your child’s urine for protein (by using dipsticks) is taught to the parents.

Prognosis

            The silver lining with this disease is that it is self-limiting and complete recovery is possible. It is estimated that as many as 80% of those affected have a favorable prognosis. However, it is dependent upon the underlying cause of the disease, the age of the person, and their response to treatment.  For children under the age of five, the prognosis is poorer than for the prepubescent child. For some children, dialysis or kidney transplantation may be necessary.   When detected early and treated promptly, damage to the kidney is minimized.  Although your child may experience periods where they relapse and have to resume treatment over time, the tendency to relapse will decrease.  Once any relapses have occurred and been treated, in time your child may well outgrow this condition and kidney function may return to normal or to near normal.

Hemolytic Uremic Syndrome

Hemolytic uremic syndrome (HUS) is an uncommon, acute kidney disorder. It occurs primarily in children between the ages of 6 months to 5 years. HUS is considered to be the most frequent cause of acute renal failure.  It is characterized by anemia, acute kidney failure and thrombocytopenia.The anemia is a result of the destruction of red blood cells. These damaged red blood cells will eventually clog the filtering system in the kidneys, which may result in the life-threatening kidney failure associated with hemolytic uremic syndrome.

Etiology or Causes

            There are a number of causes for this disorder, but in children it is more frequently associated with the E Coli bacteria. E Coli refers to a group of bacteria normally found in the intestines of healthy humans and animals. Although there are hundreds of strains of E Coli, most of the strains are harmless. E Coli can be found in contaminated meat and produce.  It can also be found in swimming pools or lakes contaminated with feces.  Certain medications and viruses have been known to cause this disorder, as well. There is also a genetic component to a certain type of HUS. Consequently, HUS can be acquired from a parent.

Signs and Symptoms

            The signs and symptoms of this disorder are irritability, fatigue, abdominal pain, vomiting, fever, and pale skin tone. The hemorrhagic manifestations will include bruising, petechiae, jaundice, and bloody diarrhea. Kidney involvement includes blood in the urine and decreased urination. Central nervous system involvement includes seizures, lethargy, stupor and coma.

Diagnostic Test

            A number of tests will be performed to obtain a definitive diagnosis.  However, the anemia, thrombocytopenia and renal failure are sufficient for diagnosis.  A complete blood cell count will reveal a low platelet count, a low red blood cell count and a high serum creatinine level.  A urinalysis is ordered to check for the presence of blood in the urine.  Your doctor may order a stool sample to check for the presence of bacteria.

Treatment and Prevention

            The primary goals of therapy are to diagnose this disease early and to provide contentious, supportive care.  Hemodialysis or peritoneal dialysis is the gold standard for treating the acute renal failure associated with this disease. It is usually implemented if the child has not produced urine for 24 hours or if the child is demonstrating oliguria. Any fluid and electrolyte replacement needed due to fluid loss from diarrhea and vomiting should be done cautiously in light of the fact the kidneys aren’t removing fluid and waste as efficiently as normal. The low red blood cells, hemoglobin and hematocrit levels are treated with red blood cell transfusions given through an IV (intravenous) needle.  Platelet transfusions are given if the child is bruising or bleeding easily because platelets can help your blood clot normally. In extreme cases, plasma exchange may be implemented. Plasma is the liquid component of blood that supports the circulation of blood cells and platelets. With plasma exchange, a machine is used to clear the blood of its own plasma and replace it with fresh donor plasma. This process is known as plasmapheresis.

While specific preventive measure for HUS are not known, it is recommended that everyone should take the following precautions against E. Coli and any other food-borne pathogens:

Good hand washing after using the toilet, after changing a diaper and before eating
Keep food surfaces and utensils clean
Defrost raw meats in the refrigerator or the microwave
Cook ground meat to at least 165 degrees F (71C)
Always wash your fruits and vegetables under running water
Do not drink unpasteurized milk, juice or cider
Keep raw food separated from ready-to-eat foods
Avoid pools of water that are potentially contaminated with feces
Don’t allow anyone to swim in a pool if they have diarrhea

Prognosis

            The recovery rate from this disease is 95%, if treatment is sought early. However, residual kidney impairment ranges from 10% to 50%. This may necessitate the need for supportive care in light of the chronic renal impairment, high blood pressure and central nervous system disorders.  If the kidneys are damaged significantly and the child develops permanent kidney failure, he or she may need to undergo long term dialysis and possibly kidney transplantation.

Acute Glomerulonephritis

Glomerulonephritis is a disorder in which the tiny filters in your kidneys, known as the glomeruli, becomes inflamed and injured due to the immune process. The glomeruli are the structures in the kidneys that remove excess fluid, electrolytes and waste from the bloodstream and pass them into your urine. Many cases occur after an infection. The most common bacteria associated with this condition are the pneumococcal and streptococcal bacteria. Acute post-streptococcal glomerulonephritis (APSGN) is the most common of the post-infectious renal diseases in childhood.

This disease, also known as, glomerular disease can be classified as acute (a sudden onset of the disease) or chronic, (coming on gradually). This disorder is seen more frequently in males than in females and its peak age of occurrence is around the 5 to 6 year olds.

Etiology and Causes

APSGN typically occurs a week or two after a streptococcal infection, like strep throat or a skin infection such as impetigo, although that etiology is fairly rare. With an infection, there is an antibody-antigen reaction. This causes inflammation to the glomeruli which results in altered structure and function of this filtering component. Some viruses may trigger glomerulonephritis, such as, the virus known as HIV, Hepatitis B and Hepatitis C virus. Other known causes are Bacterial Endocarditis, Lupus or Vasculitis, Goodpasture Syndrome, and IgA nephropathy.

Signs and Symptoms

The signs and symptoms of this disorder is fever, lethargy, irritability, fatigue (from anemia), hematuria ( blood in the urine), proteinuria (protein in the urine), scanty urine output, headache, high blood pressure, pallor, abdominal discomfort, loss of appetite and vomiting. Facial edema, or swelling, is common which tends to be more prominent in the morning. This edema tends to spread to the extremities and the abdomen during the day.

Diagnostic Test

Most children who develop this disorder are in good health until they experience a bacterial or a viral infection. The onset of this condition appears after a latency period of on the average, 10 days. The child will appear healthy during the latency period and because of that fact, many parents do not see the association between the previous infection and the disorder. The following test will be ordered to help make a definitive diagnosis:

Urinalysis– This test will result in red blood cells, red cell casts and protein in your urine, which is a sign of possible damage to the glomeruli. A finding of white blood cells in your urine is an indicator of infection or inflammation.

Blood tests– Serum creatinine and blood urea nitrogen levels can provide information about kidney impairment and damage. You can expect to see an elevated erythrocyte sedimentation rate and a depressed serum complement level. An elevated anti-streptolysin (ASO) titer and an elevated DNAase B antigen titer are laboratory test specifically designated to detect whether or not a streptococcus infection had occurred.

Imaging test– If your doctor determines that there is evidence of kidney damage, he or she may order diagnostic test that will allow them to visualize your kidneys. Test such as an x-ray, a kidney sonogram or a computerized tomography (CT) scan.

Kidney biopsy– It is common for a kidney biopsy to be ordered to confirm a diagnosis of glomerulonephritis. This test will allow the doctor to determine the cause of the inflammation.

Treatment

The treatment for glomerulonephritis is dependent upon several factors: 1) the underlying cause, 2) whether or not the condition is acute in nature or chronic, and 3) the severity of your signs and symptoms. The primary goal is to prevent further damage to the kidneys. In cases where glomerulonephritis was preceded by a strep infection, many times the body tends to heal itself and no specific treatment may be required.

One of the complications of this disorder is high blood pressure and keeping your blood pressure under control is so important in protecting your kidneys. Your doctor may order blood pressure medication and diuretics to manage your blood pressure and to aid in excreting excess fluid.

When your doctor determines the underlying cause then your treatment will be directed towards the underlying cause. If there is a bacterial infection involved, your doctor may prescribe an antibiotic. If lupus or vasculitis is involved, your doctor may prescribe a corticosteroid and immunosuppressing drugs to control the inflammation. In cases where IgA nephropathy is the underlying cause, doctors have been using fish oil and immunosuppressing drugs, concurrently and this has been successful in certain patients. There is research ongoing regarding fish oil supplements and its effectiveness in treating IgA nephropathy.

Diabetic kidney disease (Nephropathy) is a condition that can develop in patients with diabetes. Maintaining appropriate blood glucose levels and blood pressure can help prevent or slow down kidney damage. When Goodpasture Syndrome is the primary diagnosis that led to acute glomerulonephritis, a procedure known as plasmapheresis is used. This is a blood exchange process that removes antibodies from our blood by removing plasma and replacing the plasma with donated plasma and other fluids.

A complication that can result from glomerulonephritis is acute kidney failure and chronic kidney disease. Kidney failure occurs as the filtering component of your kidney loses its ability to remove waste products and fluids. If your kidney function deteriorates you may require dialysis. With chronic kidney disease, the kidney gradually loses its ability to function properly. When kidneys are functioning at less than 10 percent of normal ability, the patient is classified as having end-stage-kidney disease. At this point, dialysis or a kidney transplant is necessary to sustain life.

Prevention

Although, there might not be any way to prevent most forms of glomerulonephritis, we can offer some recommendations that may be beneficial:

  1. Avoiding high blood pressure can lessen the likeliness of damage to your kidneys from hypertension.
  2. Seek immediate medical attention, if you contract strep throat or impetigo which could have the streptococcus bacteria as the causative agent.
  3. Maintain proper blood glucose levels to prevent diabetic nephropathy
  4. Use of non-steroidal anti-inflammatory drugs (NSAID’s) should be avoided because the anti-prostaglandin action of NSAID’s may cause a decrease in the glomerular filtration rate (which is a precursor, as well as, a component of end-stage renal disease).