Complications

Autosomal dominant polycystic kidney disease (ADPKD) can sometimes lead to potentially serious complications in other parts of the body besides the kidneys.

Some of the main complications associated with ADPKD are described below.

Liver cysts

Many people with ADPKD develop cysts in other organs, as well as in their kidneys.The liver is also often affected by ADPKD.

Cysts that develop in the liver don't usually disrupt normal liver function, but they can sometimes become infected and/or cause symptoms such as:

• abdominal (tummy) pain
• abdominal swelling and bloating
• in rare cases, Jaundice (yellowing of the skin and whites of the eyes from liver damage)

In most cases, these symptoms will pass without the need for treatment. In rare cases, where a larger cyst causes severe or persistent pain, surgery may be required to drain the cyst.

Very rarely, the liver can become so massively swollen that it stops working properly. In such cases itmay be necessary to surgically remove some of the liver or carry out a complete liver transplant .

Cardiovascular disease

As a result of high blood pressure , people with ADPKD also have an increased risk of developing cardiovascular disease (CVD) .

CVD is a general term that refers to conditions that affect the heart and blood vessels and includes:

• coronary heart disease where the blood supply to the heart becomes restricted
• stroke when the blood supply to part of the brain is blocked, causing irreversible brain damage
• heart attack when the blood supply to the heart is suddenly blocked, causing irreversible damage to the heart muscle

If you're at risk of developing CVD, you may be prescribed low-dose aspirin to help stop your blood clotting, and a medication called a statin to reduce your cholesterol levels.

Making healthy lifestyle changes, such as stopping smoking , reducing your alcohol intake , exercising regularly and eating a healthy, balanced diet ,can also reduce your risk of developing CVD.

As blood passes through the weakened part of the vessel, the blood pressure causes it to bulge outwards like a balloon.

Brain aneurysms are more common in people with ADPKD than those in the general population, probably because the high blood pressure affects the weakened blood vessel walls.

Abrain aneurysm doesn'tusually cause any noticeable symptoms unless it bursts (ruptures). A ruptured aneurysm causes bleeding over the surface of the brain. This is known as a subarachnoid haemorrhage .

Symptoms of a subarachnoid haemorrhage can include:

• a sudden agonising headache often described as being like being suddenly hit on the head, resulting in a blinding pain unlike anything experienced before
• stiff neck
• feeling and being sick
• sensitivity to light (photophobia)
• blurred or double vision
• confusion
• stroke-like symptoms such as slurred speech and weakness on one side of the body
• loss of consciousness or convulsions (uncontrollable shaking)

A subarachnoid haemorrhage is a medical emergency that requires immediate treatment to prevent serious complications, brain damage and death.

Dial 999 immediately and ask for an ambulance if you think that you or someone you know is having a subarachnoid haemorrhage.

Screening

It's estimated that around 10% of people with ADPKD will develop a brain aneurysm, but most won't have any symptoms and it will never cause a problem. People with ADKPD who also have a family history of subarachnoid haemorrhages are at greater risk.

If you have ADPKD and a family history of subarachnoid haemorrhages, you'll usually be offered amagnetic resonance angiography(MRA) scan to check for aneurysms in your brain. An MRA scan uses a magnetic field and radio waves to produce images of your arteries and the blood flow within them.

If no, or only small aneurysms are found, you'll be offered further scans at intervals of one to five years to check for new haemorrhages or an increase in the size of an existing one.

If an aneurysm of a particular size is detected, and your doctor thinks there's a risk it could rupture in the future, they may recommend that you have an operation or procedure to prevent this.

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