Cholestasis is a less commonly heard term, but it can be the root cause of various health issues. It’s a medical condition that affects bile flow from the liver, leading to a buildup of bile acids in the bloodstream. Cholestasis can occur during pregnancy, with medication intake, due to liver disease, or in neonates due to congenital problems. This condition can be harmful to both the mother and fetus during pregnancy. If left untreated, it can lead to significant complications, including liver failure or even death.
People diagnosed with cholestasis must undergo surgery and maintain a healthy lifestyle, changing their diet afterward to manage this condition effectively.
In this article, we will discuss everything patients need to know about cholestasis, including its symptoms, various types, diagnostic methods, and available medical treatments. If you or a loved one has been diagnosed with cholestasis, rest assured that this condition is manageable with proper medical treatment and a healthy lifestyle. We’ll provide you with valuable information on the various treatment options and how to ensure that your condition is well-managed.
What is cholestasis?
Cholestasis is a medical condition in which bile flow from the liver to the small intestine is blocked or reduced. Various factors, including liver damage, certain medications, or certain genetic conditions, can cause this. It is defined as a condition in which the flow of bile is reduced or blocked, and there is an imbalance in bile components. This can cause various symptoms, including jaundice, abdominal pain, itching, dark urine, and pale stools.
Cholestasis can be either acute or chronic. Acute cholestasis is a short-term condition that can sometimes be very severe. Chronic cholestasis is caused by long-term damage to the liver, such as cirrhosis, and it often requires ongoing medical care.
Cholestasis can have serious consequences, such as liver failure, so it is essential to be aware of the symptoms and to seek medical attention if they occur. Early diagnosis and treatment can help prevent complications and improve a patient’s quality of life.
Signs and symptoms of cholestasis
1) Jaundice
Jaundice is the yellowish coloration of the skin and whites of the eyes caused by elevated levels of bilirubin in the blood. This is typically caused by cholestasis when the biliary system is blocked and the bilirubin cannot be excreted properly. It usually starts to be noticeable in the white portion of the eyes when you reach 2 mg/dL of conjugated bilirubin levels, but symptoms may become more visible with higher levels.
This happens because the biliary system is blocked, impairing bilirubin excretion. This causes the bilirubin to build up in the blood, leading to the characteristic yellowish discoloration.
2) Dark urine
Dark urine is a common symptom of high bilirubin levels. The exact mechanism of cholestasis is a bit complicated, but it involves disruption of the normal flow of bile which leads to an accumulation of bile acids and bilirubin, which can eventually be excreted in the urine and can cause dark urine.
It is important to note that dark urine is an indirect sign of cholestasis, and it is important to seek medical attention to confirm the diagnosis. It can be caused by other factors that should also be considered. Dark urine usually happens in more severe cases when the damage to your liver is more pronounced.
3) Pale stools
Pale stools are an essential symptom of cholestasis. In more advanced stages of the disease, pale stools can be found in conjunction with dark urine. This is because of the body’s inability to break down bilirubin, a yellow-orange pigment found in bile secreted by the liver.
When bile cannot flow normally, bilirubin accumulates in the body tissues and is eventually excreted in the urine, resulting in the darkening of the urine, as mentioned above. The decreased flow of bile also results in the decreased release of bile acids and bile salts into the gut, resulting in pale stools.
4) Severe pruritus
Severe pruritus (itchiness) is a common symptom of jaundice, which occurs when bile acids accumulate in the body.
The mechanism of how bile acids cause pruritus is not entirely understood. However, it is believed to be related to the stimulation of nerve terminals. Bile acids are considered to activate nerve endings in the skin, leading to intense itching. Furthermore, bile acids can also reduce the skin’s ability to retain moisture, leading to further itching.
It is important to seek medical attention if you suffer from severe itching or jaundice, as it may be a sign of cholestasis or another underlying medical condition. It is also important to note that cholestasis can lead to other serious complications, such as liver damage, so early detection, and treatment are essential.
5) Sleep problems and scratch marks
Patients may experience severe pruritus, a feeling of intense itching in the skin. When bile acids reach five times the reference range, the symptoms can be incredibly debilitating, leading to sleep problems and scratch marks caused by the patient’s attempts to relieve the itching.
In severe cases, the itching can be accompanied by other symptoms such as fatigue and abdominal pain. However, seeking attention as soon as possible is important to avoid complications.
Types of cholestasis
A) Obstructive cholestasis
Obstructive cholestasis is a form of cholestasis caused by a blockage or obstruction of the common bile duct. The blockage prevents the normal flow of bile, a digestive fluid produced by the hepatocytes in the liver. This causes the bile to be retained in the liver, leading to a buildup of bile salts in the hepatocytes.
The retained bile salts cause inflammation and injury to the hepatocytes, leading to impaired bile salt uptake and excretion and decreased secretion of bile acids. This disruption of bile flow causes a decrease in the synthesis of bile salts, which in turn reduces the production of lipids, proteins, and vitamins essential for normal digestion.
Furthermore, the accumulation of bile salts in the liver leads to the formation of gallstones, which can cause severe abdominal pain and other complications.
B) Physiologic and neonate cholestasis
Physiologic cholestasis is a condition characterized by the impaired excretion of bile from the liver and subsequent jaundice. Various factors, including immature liver function in newborns, certain medications, and certain diseases, can cause this condition.
Physiologic cholestasis is most commonly observed in newborns and is often called neonatal jaundice. This condition is caused by an immature liver that does not efficiently produce and excrete bile, accumulating bile salts in the body. This results in the yellow discoloration of the skin and sclera, known as jaundice.
Physiologic or neonatal cholestasis is a condition that can potentially be serious if left untreated. It is essential to seek medical attention if a newborn infant is exhibiting signs of jaundice, as early diagnosis and treatment can help to reduce the risk of any long-term complications.
Diagnosis
1. Lab studies
Lab studies are essential for diagnosing cholestasis, as they provide physicians with insight into the biochemical mechanisms underlying the condition. Commonly used lab studies include serum bilirubin, serum lipoprotein X, serum alkaline phosphatase levels, serum 5′-nucleotidase levels, and serum gamma-glutamyl transferase.
Serum bilirubin measures the amount of bile that has been excreted and is usually elevated in cases of cholestasis as bile is retained in the liver. Serum lipoprotein X is a measure of cholesterol that has been excreted into the bile, and is usually decreased in cases of cholestasis. Serum alkaline phosphatase levels are typically elevated in cholestasis cases due to increased liver enzyme activity. Serum 5′-nucleotidase and serum gamma-glutamyl transferase are usually elevated in cases of cholestasis, as the enzymes are released from the biliary tree in response to bile retention.
By measuring these markers, physicians can gain insight into the underlying biochemical mechanism of cholestasis and make an informed diagnosis.
2. Imaging studies
When it comes to the diagnosis of cholestasis, imaging studies such as ultrasound scans and CT scans are commonly used. These diagnostic methods help visualize the structure of the bile ducts and detect any blockages or abnormalities that could be causing the symptoms.
Ultrasound and CT scans are useful for detecting gallstones, bile duct strictures, and other conditions causing cholestasis.
The other type of study used to diagnose cholestasis is biliary nuclear medicine studies. These studies are used to differentiate between obstructive and hepatocellular cholestasis.
Overall, imaging and biliary nuclear medicine studies are essential tools for diagnosing cholestasis and determining the underlying cause. They provide detailed information about the structure of the bile ducts and the levels of bile salts in the bile ducts, which can help diagnose and treat this condition.
3. Endoscopic retrograde cholangiography and Percutaneous transhepatic cholangiography
Endoscopic retrograde cholangiography (ERC) and Percutaneous transhepatic cholangiography (PTC) are two critical diagnostic techniques used to diagnose cholestasis. ERC is a procedure where small probes are inserted into the pancreatic and biliary ducts, which are then used to inject a contrast dye for visualization. On the other hand, PTC is a more aggressive procedure where a contrast dye is injected directly into the bile ducts using a needle. These procedures allow for visualizing any blockages or abnormalities of the bile ducts and are essential for diagnosing cholestasis.
In the case of ERC, a catheter is passed into the pancreatic and biliary ducts, and a contrast dye is injected. This dye helps in outlining the ductal structure and any blockages or abnormalities. For PTC, the contrast dye is injected directly into the bile ducts using a needle, allowing for more detailed visualization of the bile ducts. This allows the doctor to determine if a blockage, narrowing, or abnormal structure exists.
4. Exploratory surgery
Diagnosis of neonatal cholestasis typically involves a combination of clinical evaluation, laboratory testing, and imaging. In some cases, exploratory surgery is necessary to identify the cause of the condition and evaluate the extent of damage caused by the interruption of bile flow.
During the surgery, the surgeon examines the biliary tract, including the gallbladder and bile ducts, to determine the underlying cause of the cholestasis. In addition, imaging of the liver, gallbladder, and biliary tract may be used to assess the degree of obstruction. The surgeon may also take tissue samples for microscopic examination to evaluate the extent of damage and to look for abnormal cells or tissue.
After the exploratory surgery is complete, further treatment, such as medications or surgical repair of the biliary tract, can be pursued to correct the underlying cause of the condition.
Cholestasis treatment
1. Surgical treatment
The most common treatment for cholestasis is a biliary bypass, which creates a new pathway for bile to reach the intestines. This procedure is performed by making an incision in the patient’s abdomen and connecting the bile ducts to the intestines, bypassing the obstruction. Another surgical option is a cholecystostomy, which removes the gallbladder and the obstruction in the bile ducts. This procedure is typically used for an infection or tumor in the gallbladder.
A laparoscopic cholecystectomy may be performed if the patient is not a candidate for a biliary bypass or a standard cholecystostomy. This operation involves the insertion of a small camera and instruments into the abdomen, allowing the surgeon to view and remove the gallbladder.
Finally, a liver transplant may be necessary if there is irreparable liver damage caused by cholestasis. This procedure involves removing the diseased liver and replacing it with a healthy liver from a donor. In all cases, a multidisciplinary team of medical professionals is involved in diagnosing and treating cholestasis to ensure the best possible outcome for the patient.
2. Dietary advice
Patients with cholestasis should follow a diet focusing on high-fiber, low-fat foods. Eating fruits, vegetables, and whole grains can help reduce inflammation and promote bile flow. Other beneficial foods include lean proteins and complex carbohydrates. Avoiding fatty and greasy foods and limiting sugar intake can also help manage symptoms. Additionally, it is essential to drink plenty of water and limit alcohol consumption.
Patients with cholestasis should also be aware of food intolerances or sensitivities. Common triggers include caffeine, sulfur-containing foods, and high-fat foods. It is important to pay attention to any reactions to these foods, as they may worsen symptoms. A registered dietitian can help patients with cholestasis create an individualized eating plan that meets their nutritional needs. It is also important for patients to discuss any changes in diet with their healthcare providers before making any modifications.
3. Treatment of neonatal cholestasis
Treatment for neonatal cholestasis typically involves phototherapy and medications to help promote bile excretion. Phototherapy consists of the use of special lights to break down bile pigments in the skin, while medications, such as ursodeoxycholic acid, can help to reduce the production of bile acids.
Surgery may sometimes be needed to relieve any blockage of the bile ducts. Additional treatments may include dietary modifications and vitamin supplementation to help support the liver and its function.