ARPKD| CHF Alliance
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Q & A, Portal Hypertension and Protection

Q. My 8-year-old daughter has portal hypertension and enlarged liver and spleen. Although she is eager to keep up with her classmates, I donít allow her to participate in soccer or other vigorous sports because Iím afraid she might injure her spleen. But what about gymnastics, playing on the monkey bars or other playground equipment?

A. The spleen is like a bag filled with blood. This is because the spleenís purpose is to filter the blood supply of bacteria and remove old red blood cells. Normally, the spleen is safely

tucked up under the ribs; but when the spleen is enlarged due to portal hypertension it can be felt below the ribs.

Whenever the spleen is felt below the ribs it is not protected from injury. Abdominal trauma such as a kick or direct blow with a blunt object, such as an elbow, stick, or bicycle handlebars may cause the spleen to rupture and leak blood into itself or into the abdominal cavity. This may occur rapidly and a child could die suddenly.

Vigorous exercise, including gymnastics, sit-ups, push-ups, and playground equipment are generally safe.  A child with an enlarged spleen should not participate in contact sports, such as kick boxing, boxing, karate, hockey, and football.  (Note:  Wrestling and Lacrosse may also not be considered safe with portal hypertension.)  On the other hand, sports like soccer, baseball, volleyball, and basketball are generally safe (although elbowing can be dangerous.)  In some cases, an abdominal binder can be fashioned to help protect the spleen from injury.

Dr. Berquist is Chief, Gastroenterology and Liver Transplantation at Lucile Salter Packard Childrenís Hospital at Stanford University and Chairman of the CLASS Scientific Advisory Board.
From Archived ARPKD/CHF Newsletter


Many of these questions and answers are ADPKD related, generally because not enough specific research has been done on ARPKD and CHF. These comments should not be taken as medical advice.  Please refer to your specialists for specific options.

Finding A Doctor
Your first step should be finding a local medical professional who has knowledge about the disease. To find a qualified professional, please visit:

What are typical treatment options?
Many, but not all infants/children need treatment to control high blood pressure; this is done with medication(s).  It is not unusual for dosages to be decreased or weaned with age.  (Kidneys generally do not grow in size, but stay the same with ARPKD.)  If needed, urinary tract infections (UTIís) are controlled with antibiotics.  Other symptoms are treated as they occur.

Will this affect growth?
Some children experience slow growth and stature ("failure to thrive").  In some cases, depending on the circumstances, supplements are required and growth hormones used when children consistently score below the 5th percentile of standard growth and development charts.

Will my child need a transplant?
It is likely at some point kidney transplantation will be needed, but this varies greatly to very early in life to later in life.  It all depends on the rate of decline of kidney function.

Can surgery help this problem?
Not usually.  It will not change the course of the disease.  Banding may help with the varcises and shunts may help with the blood flow.

How can diet help?
Eating a healthy diet is recommended.  A low sodium diet may be recommended (yet children may crave salt), depending on individual circumstances.  Generally children are NOT placed on a low protein diet, as protein is needed for growth. 

Top Do's:

  • Find a nephrologist to help manage renal function, high blood pressure and other clinical symptoms.
  • There will likely come a time to find a GI specialist who can assist with clinical liver manifestations.
  • Take your medication(s) as directed.
  • Normalize lifestyle as much as possible.
  • Eat a healthy diet.

Top Doníts:

  • Donít take medicines or vitamins without first speaking with your specialist.
  • Ibuprofen (Advil) is thought to be detrimental to PKD kidneys.
  • Watch your diet restrictions during different phrases of the disease- especially under dialysis.
  • Avoid contact sports

What tests can be done to monitor my kidney function?
There are 4 main tests used to determine kidney function:

A test that measures levels of creatine in your urine. Creatine is dependent on weight, age, physical activity and even weather.  If you are not drinking sufficient fluids, your creatinine will go up due to dehydration; inactivity breaks down muscle, creating more creatine.

Blood Urea Nitrogen (BUN)
A test that measures levels of urea nitrogen in your blood. Urea is the main waste product of protein metabolism.  Blood levels of it are referred to as blood urea nitrogen (BUN) and are used to
evaluate kidney disease.  A high BUN is the hallmark of kidney disease.  It is also high in heart failure, increased protein breakdown, diabetic acidosis, dehydration, intestinal bleeding, and muscle degeneration.  Certain drugs cause an elevated BUN, as will a recent meal heavy in protein. Low BUN is associated with excess fluid intake, malnutrition, liver disease, and pregnancy.

24 Hr. Creatinine Clearance
A 24 hour collection of urine samples that is measured for creatine levels.  Creatine is a waste product of normal metabolism.  Blood levels
óalong with blood urea nitrogenóare the most common kidney function tests.  High creatinine is associated with any of a number of kidney diseases, but provides only a rough approximation of the extent of disease and does not identify its cause.

Radionuclide studies (GFR)
A test that uses an isotope solution injected into your vein, with a gamma camera taking pictures to show the blood supply to each kidney.

What is GFR?
The filtering action of the kidneys processes about 120 milliliters of blood per minute.  Doctors measure this rate as an indication of how well the kidneys are working and call it the glomerular filtration rate or GFR for short.

Glomerular filtration rate (GFR) is considered by medical professionals to be the best measure of kidney function.

What is a normal GFR?
The chart below lists values for GFR:

Kidney Function

Glomerular Filtration Rate


120 ML/MIN: Normal Kidneys


60 ML/MIN: Failing Kidneys


30 ML/MIN: Chronic Renal Failure


6 ML/MIN: End-Stage Renal Failure

What is standard therapy?
In medicine, standard therapy is a treatment that experts agree is accepted, appropriate and widely used.  Healthcare providers are obligated to provide patients with standard therapy.  The terms standard of care or best practice are also used.



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