Jaundice: a symptom of various conditions

Jaundice is not a disease. It is a symptom that can be associated with a number of different conditions, especially of the liver and gallbladder. The most noticeable symptom of jaundice is the yellow discoloration of the whites of the eyes. But the gums and skin can also turn yellowish. In severe jaundice, the urine is dark and the stools pale. It all has to do with too much bilirubin in the blood; a waste product resulting from the breakdown of hemoglobin from red blood cells. Jaundice (icterus) occurs when there is too much bilirubin in your blood. Bilirubin is the waste product left over after the breakdown of hemoglobin (the red blood dye) from red blood cells. In healthy people, red blood cells have a lifespan of about 120 days. After this, they are removed from the blood and broken down by the spleen. The bilirubin that is created as a waste product is transported to the liver via the blood.

Symptoms of jaundice

The liver contains enzymes that convert the insoluble bilirubin and ensure that it can be dissolved in water. Together with bile fluid, the bilirubin leaves the liver and is drained through the gallbladder to the small intestine. Ultimately, the waste leaves our body with urine and feces. Bilirubin has an intense yellow color. When the concentration of bilirubin in the blood is very high, you can literally turn yellow. The bilirubin accumulates in the blood and is partly stored in the skin and mucous membranes. This is especially visible in the whites of the eyes. But the gums and skin can also turn yellowish.
If the bilirubin cannot enter the intestines, for example due to a blockage of the bile ducts, it cannot be removed through the feces. The result is light, gray-white stools and dark , tea-colored urine. The bile fluids (containing bilirubin) normally provide the brown color of the stool.
If these fluids are missing, the stool loses its color. The bilirubin is now largely removed via the kidneys with the urine. Due to the greatly increased concentration of bilirubin in your urine, it turns very dark in contrast to the stool.

Jaundice occurs when there is too much bilirubin in the blood. This can have three causes: the supply of bilirubin is too high, the liver is unable to process the bilirubin supplied due to a disease or abnormality, or the converted bilirubin cannot be transported to the intestines.


Jaundice due to increased breakdown of red blood cells

Jaundice due to hemolytic anemia / Source: Sab3el3eish, Wikimedia Commons (CC BY-3.0)

Due to a greatly increased breakdown of red blood cells, the supply of bilirubin can become very high. This is, for example, the case with heavy bleeding. The liver can then no longer cope with the supply of bilirubin, causing too much bilirubin to remain in the blood. But also in certain forms of anemia (hemolytic anemia), red blood cells are broken down too quickly. Normally, red blood cells function for about 120 days. After this, they are removed from the blood and broken down by the spleen. The bilirubin that is created is transported to the liver. In hemolytic anemia, the lifespan of red blood cells is significantly shorter than normal. The blood cells are broken down too quickly and the liver has to process more bilirubin than it can handle. The concentration of bilirubin in the blood therefore remains too high, resulting in (hemolytic) jaundice. This condition usually also causes severe fatigue.

Jaundice due to a hereditary abnormality of the liver

The liver is a large and important organ. It is located in the upper right abdomen, just behind the ribs. Sometimes the liver is damaged by a disease or abnormality in such a way that the bilirubin cannot be processed and excreted properly. An increased concentration of the waste product then remains in the blood.
In Gilbert’s syndrome, the liver is unable to convert the bilirubin into a water-soluble substance because the enzyme required for this is partly missing or functions insufficiently. The bilirubin cannot therefore be properly removed through the urine and feces and remains present in the body in excessive quantities. The result is (mild) jaundice. Gilbert’s syndrome is a chronic, but harmless and benign liver disease. It is a hereditary condition that affects about 1 in 20 people. Sometimes, in addition to jaundice, there are complaints such as vague abdominal pain and fatigue. There is no treatment for this condition.
Crigler-Najjar disease is a rare, hereditary disorder of the liver. In this disease, the enzyme that is responsible for the conversion of bilirubin in the liver is completely missing. This causes a serious build-up of insoluble bilirubin in the blood. This accumulation can cause various complaints that, if left untreated, can eventually become life-threatening. A very high concentration of bilirubin in the blood is toxic to the nervous system in the long term and can cause permanent brain damage. The disease is hereditary and usually manifests itself shortly after birth. The diagnosis can be made based on blood tests. The treatment consists of very intensive light therapy, which can usually keep the bilirubin level under control.

Jaundice due to a disease of the liver

If the liver is affected, less bilirubin can be processed. Damage to liver cells can have various causes. The most common are liver inflammation (hepatitis), fatty liver disease and cirrhosis.

Jaundice due to Hepatitis A / Source: CDC/Dr. Thomas F. Sellers/Emory University, Wikimedia Commons (Public domain)

Sudden inflammation of the liver (acute hepatitis) is common. Most people are cured within two months, but the inflammation can sometimes last for months or even years (chronic hepatitis). Acute liver inflammation is usually caused by a hepatitis virus (A to E). However, bacteria (e.g. Leptospira, which causes Weil’s disease), parasites (e.g. Plasmodium, the cause of malaria), medications (e.g. anticonvulsants, the anesthetic gas halothane and an overdose of paracetamol) and alcohol can also cause liver inflammation. With liver inflammation, jaundice is often accompanied by nausea, vomiting, pain in the upper right side of the abdomen, poor appetite, fatigue, fever and a swollen abdomen. Hepatitis A, B and C are the most common. The Hepatitis A virus is the mildest form and is mainly transmitted through contaminated food and water. Hepatitis B is spread through contact with the body fluids of infected people, for example through sexual intercourse, when drug users share needles or during birth from mother to child. Hepatitis C affects approximately 3 percent of the world’s population every year. Infection with this virus usually occurs through contact with infected blood, often when sharing needles among drug users. In developed countries, excessive alcohol consumption is one of the leading non-viral causes. Severe hepatitis can cause liver failure, mental confusion, and sometimes a coma. The diagnosis can be made based on blood tests. Sometimes an additional ultrasound scan or liver biopsy is necessary. There is no treatment for most cases of acute hepatitis.
Fatty liver disease is the most common liver disease. The liver plays an important role in fat metabolism in the body. If fat metabolism is disrupted, fat can accumulate in the liver cells. This is called fatty liver disease. Chronic alcohol use, which causes liver cells to inflame and function less well, is one of the most common causes of fatty liver disease. But obesity (especially in people who have a lot of fat around their middle) and diabetes type 2 also often lead to fatty liver disease. In people with type 2 diabetes, the body is insensitive to insulin, which causes blood sugar levels to remain too high. The excess sugars are converted into fats in the liver. These are stored in the liver and cause fatty liver disease. Fatty liver disease sometimes causes symptoms such as vague pain in the upper right side of the abdomen, fatigue and jaundice. Fatty liver disease can be diagnosed using blood tests or an ultrasound scan. There is no treatment that can cure fatty liver disease. However, it is a reversible process; the fat accumulation disappears as soon as the cause is removed. If the cause remains present, the fat accumulation will expand and can lead to liver inflammation. This happens to 1 in 5 people with fatty liver disease. Inflammation of the liver can eventually progress to cirrhosis.

Jaundice due to a failing liver / Source: James Heilman, Wikimedia Commons (CC BY-3.0)

In liver cirrhosis or shriveling of the liver, the liver tissue is so severely damaged that it can no longer recover. Liver cirrhosis is the result of chronic liver disease, often hepatitis, or chronic liver damage due to long-term alcohol use. The liver cells become inflamed, die and are replaced by scar tissue. As a result, the liver is increasingly unable to perform its important functions. To compensate for this loss of function, healthy liver cells divide. For this reason, the liver often enlarges in early cirrhosis. Ultimately, however, the liver becomes smaller and more complaints arise: nausea, vomiting, loss of appetite, weight loss, weakness, abdominal pain, jaundice, itching, cessation of menstruation in women and breast formation in men. In severe liver cirrhosis, varicose veins may develop in the esophagus or stomach, fluid accumulates in the abdominal cavity and the spleen becomes enlarged. In an advanced stage, liver cirrhosis can lead to liver cancer. At the beginning of the disease, the process can often be stopped and remedied. In a later phase, the disease is irreversible and a liver transplant is the only possible treatment. Liver cirrhosis can be diagnosed by blood tests, ultrasound, liver puncture, CT scan or MRI scan.

Jaundice due to a disease of the gallbladder or bile ducts

Sometimes the bile fluid formed in the liver cannot be drained. This causes the bilirubin to return to your blood. The blockage is usually caused by gallstones or inflammation.

Gallstones / Source: George Chernilevsky, Wikimedia Commons (CC BY-SA-4.0)

The bile fluid, produced by the liver, is transported to the gallbladder via bile ducts. The gallbladder is a small, pear-shaped sac that lies in the upper right part of the abdomen, against the liver. The bile fluid sometimes remains in the gallbladder for a long time and can then thicken. If the fluid thickens too much, a gallstone is formed . It is not known why the bile fluid thickens too much. The composition of the fluid and the functioning of the gallbladder itself may play a role. As long as the gallstone is in the gallbladder, few or no complaints will occur. These only occur when the gallstone becomes stuck in the bile ducts and the flow of bile fluid is obstructed. The gallbladder tries to remove the blockage by contracting violently. This can cause severe pain in the upper right abdomen (biliary colic), which can last for hours. In addition, nausea, vomiting, jaundice, light stools and dark urine occur. If the blockage remains present for longer, inflammation of the gallbladder, bile ducts or pancreas may occur. Gallstones can be detected by ultrasound or MRI scan. Removal of the gallbladder is the most effective treatment. Medicines sometimes work for small gallstones, but cannot prevent gallstones from developing again.
An inflammation of the gallbladder or bile ducts is almost always caused by a narrowing or blockage of the bile ducts. The blockage reduces the flow of bile fluid. Bacteria from, for example, the small intestine can then easily settle in the gallbladder or bile ducts and cause inflammation. Sometimes the inflammation is the result of an infection with a parasite or an infection with bacteria that occurs after surgery, a serious injury or burn. The inflammation can be acute or chronic. In the beginning there is mainly a severe, persistent pain in the upper right abdomen that can radiate to the right shoulder. In addition, complaints such as fever and chills, jaundice, itching, discolored stools and dark urine often occur. Permanent untreated inflammation can eventually lead to liver cirrhosis. The diagnosis can be made using blood tests and possibly an ultrasound or MRI scan. Treatment usually consists of administering antibiotics. Additional treatment depends on the cause (e.g. a gallstone).
Occasionally, a congenital defect (biliary atresia or bile duct blockage) or a tumor in the gallbladder, bile ducts or pancreas (when the tumor presses against the gallbladder or bile duct ) causes a blockage, preventing the bilirubin from being properly drained.
Occasionally jaundice occurs as a side effect of certain medications. Sometimes jaundice is simply caused by taking an unusually large amount of yellow dyes, such as carotene found in carrots or tanning tablets.

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