Diseases

Bilirubin Encephalopathy – Causes, Symptoms & Treatment

Bilirubinenzephalopathie

Bilirubin encephalopathy is a serious complication of neonatal hyperbilirubinemia. It is damage to the central nervous system. Serious consequential damage or even death are possible.

What is bilirubin encephalopathy?

Bilirubin encephalopathy is characterized by severe damage to the central nervous system (CNS) caused by elevated bilirubin levels in neonates. Hyperbilirubinemia can trigger so-called kernicterus (jaundice with cerebral intoxication ) in the infant. Free unconjugated bilirubin is insoluble in water. It only dissolves in fats. However, it is normally bound by certain albumins in the blood and transported to the liver.

For various reasons, however, the binding capacity of the albumins can be overwhelmed, resulting in an accumulation of bilirubin in the blood. Newborn jaundice develops, which in rare cases can be dangerous. If bilirubin crosses the blood-brain barrier, it can enter the core areas of the brain and develop neurotoxic effects there. This is where the term kernicterus comes from.

The basal ganglia , which are composed of the putamen, globus pallidus and caudate nucleus, are particularly affected by the damage . Severe bilirubin encephalopathy is often fatal. This complication occurs in 0.4 to 2.7 cases in 100,000 live births in the western world. Because of less medical care, kernicterus is 100 times more common in some developing countries.

causes

Bilirubin encephalopathy is caused by damage to certain nuclear areas in the newborn’s brain by intoxication with unconjugated bilirubin. Unconjugated bilirubin is very commonly found in the blood of newborns. Around 60 percent of all infants show symptoms of neonatal jaundice, which usually heals within four days. Because the liver is still immature, bilirubin often cannot be broken down as quickly. However, these symptoms are usually not cause for concern.

However, in rare cases, the bilirubin concentration becomes so high that free unconjugated bilirubin can cross the blood-brain barrier. There it has a neurotoxic effect and damages important core areas of the brain. Normally, the unconjugated, fat-soluble bilirubin is bound to albumins, transported to the liver, and broken down there. If there is an increased formation of bilirubin through haemolysis in the case of blood group incompatibility with the mother, the binding capacity of albumin is overtaxed. The bilirubin concentration in the blood increases significantly and can cross the blood-brain barrier.

Various drugs also reduce the binding capacity of bilirubin to albumin through displacement processes. These include, for example, diazepam, sulfonamides, furosemide and others. Even with normal bilirubin concentrations in the blood, the blood-brain barrier can become permeable to bilirubin. This often occurs with a lack of oxygen ( hypoxia ), low blood sugar ( hypoglycemia ), hyperacidity of the blood ( acidosis ) or hypothermia . If the albumin concentration is too low (hypalbuminemia), bilirubin can also be exceeded by the blood-brain barrier.

Symptoms, Ailments & Signs

Acute bilirubin encephalopathy usually has three phases:

  • In a second phase, the newborn begins to cry shrill. Consciousness becomes increasingly clouded ( stupor ). There is also increased muscle tension, which leads to overstretching of the neck or spine.
  • Eventually, muscle tension may increase, with spasms appearing. The stupor can progress to a coma . The disease often ends fatally. If the infant survives the acute phase, however, late effects such as deafness , extrapyramidal motor movement disorders and psychosomatic developmental disorders often set in.

The extrapyramidal motor movement disorders are referred to as athetosis and are expressed in involuntarily, slowly extending, screwing movements of the feet and hands. The joints are overstretched. The gait is stumbling and overshooting. The cause of these bizarre movements lies in the disruption of the interaction between antagonists and agonists.

Diagnosis & History

Elevated bilirubin levels are very common in the first few days of life. For this reason, screenings are carried out in the maternity clinics in order to avert the risk of bilirubinencephalopathy as early as possible by detecting possible hyperbilirubinemia. When the infant turns yellow, there are early signs of elevated bilirubin levels. The bilirubin values ​​are already determined in the first 20 hours using a multispectral device through the skin.

If the values ​​are critical, a blood test for hyperbilirubinemia must be done. It has been shown that neurological disorders can already occur at a value above 20 mg/dl. At this level, if not treated in time, motor dysfunction can occur by the age of seven. At bilirubin concentrations above 25 mg/dl there is already a great risk of suffering from kernicterus.

complications

An increased bilirubin level in the child initially leads to a yellowish coloring of the child (newborn jaundice), which is usually not serious and subsides again without any complications. However, in the worst cases, bilirubin in the brain can accumulate in the basal ganglia, leading to kernicterus and bilirubin encephalopathy as a result. The infant is initially characterized by general weakness and muscle weakness.

This results in a reluctance to drink , which can dehydrate the baby (exsiccosis). This causes the skin to crack and makes the baby more susceptible to infections. In the worst case, the heart can also fail. In addition, the reflexes in the newborn are weakened.

In addition, the baby may suddenly start crying out loud in pain. In addition, there is a clouding of consciousness and cramping of the muscles, especially in the neck and spine (opisthotonus), so that the baby overstretches its head. In addition, the sunset phenomenon can be revealed in infants, which means that the eye turns downwards when it is opened and the visual range is thus restricted.

In the worst cases, the child suffers from cerebral failure, which can have various consequences, such as deafness. In addition, there are usually additional seizures and a mental development disorder. The disease can also lead to a coma and the death of the child.

When should you go to the doctor?

In most cases, bilirubin encephalopathy is diagnosed before birth or immediately after birth. For this reason, no additional diagnosis or treatment by another doctor is necessary. However, treatment in the hospital must take place immediately, since in the worst case the bilirubinencephalopathy can otherwise lead to the death of the person concerned.

In most cases, an examination should be done when the child has no muscle tone or is very sleepy and not moving. The child’s consciousness is also clouded and can thus indicate the disease. In severe cases, the children fall into a coma. To avoid complications or death later, a doctor should be alerted immediately if these symptoms occur.

In most cases, the diagnosis and treatment of bilirubin encephalopathy takes place directly in the hospital. As a rule, the parents do not have to consult an additional doctor. A positive course of the disease cannot be guaranteed in every case.

Treatment & Therapy

If bilirubin levels are very high, above 20 mg/dl, treatment must be initiated immediately to prevent bilirubin encephalopathy. The treatment is carried out within the first 72 hours by blue light phototherapy . The wavelength of blue light is between 425 and 475 nanometers.

During phototherapy, the unconjugated, water-insoluble bilirubin is converted to water-soluble lumirubin. This is then excreted from the body via the bile or the kidneys. If the bilirubin levels are above 30 mg/dl, phototherapy will no longer help. Then a blood transfusion should be done.

Outlook & Forecast

The prognosis of bilirubin encephalopathy depends on how soon after the onset of symptoms of the disease or when bilirubin levels are elevated, therapy is initiated.

Even before the onset of the disease, the infant must be constantly monitored in order to be able to react quickly if the concentration of unconjugated bilirubin exceeds 15 mg/dl. When unconjugated bilirubin crosses the blood-brain barrier into the brain, it destroys nerve cells by blocking phosphorylation reactions.

These processes are potentially irreversible. They can therefore no longer be undone or only partially undone. The water-insoluble unconjugated bilirubin is converted into water-soluble conjugated bilirubin during treatment by blue light and can thus be excreted from the body via blood exchange transfusions.

If no treatment is given, long-term effects can set in, the symptoms of which can only be alleviated by symptomatic therapies. These late effects include motor disorders, deafness, constant seizures and mental retardation. The motor disorders are expressed, among other things, by helical movements of the extremities. The late damage is all the more serious the later treatment begins. However, starting treatment immediately after the onset of the disease does not guarantee that there will be no long-term damage.

Since high bilirubin levels are very common in newborns, early screening after birth is very important to detect and treat hyperbilirubinemia (high levels of bilirubin in the blood) in good time.

prevention

Bilirubin encephalopathy can only be prevented by early screening after birth. In the case of greatly increased bilirubin values, blue light treatment must be carried out immediately or, in the case of extremely high values, a blood exchange transfusion. If jaundice appears after a few days at home and the child falls into lethargy, the doctor should be consulted immediately.

aftercare

As a rule, those affected with bilirubin encephalopathy have no special means of aftercare available. In the worst case, this disease can also be fatal, with the child dying. Early diagnosis and therapy have a very positive effect on the further course of the disease and can prevent various complications.

As a rule, the patient is dependent on irradiation with blue light to alleviate the symptoms. If there is no treatment, the child often dies immediately. In most cases, further follow-up care for bilirubin encephalopathy relates to the newborn child and not to the mother. The child needs special support to treat mental retardation and further delayed development.

Seizures can also be relieved with the help of various medications. Parents must ensure that they are taken regularly, and interactions with other medications should also be taken into account. After birth, the child is dependent on regular examinations. Since bilirubin encephalopathy can also lead to psychological problems in the parents and relatives of the child, intensive discussions and contact with other people affected by bilirubin encephalopathy are also very helpful.

You can do that yourself

The options for self-help are very limited in the case of bilirubin encephalopathy. The disease occurs in newborns. Naturally, they cannot take any measures to improve their situation. Therefore, the consequences of the disease are usually borne by relatives and parents. They find themselves helpless because of the circumstances and have to regulate their own emotional states.

If this cannot be achieved on your own, psychological support should be sought . Immediate medical care for the newborn is important. A close exchange with the treating doctors and nurses is necessary so that changes in the state of health can be reacted to as quickly as possible.

In addition, relatives should be informed about the disease to a sufficient extent and inform themselves. The consequences and disturbances are individual, but to a very life-threatening extent. It is important to remain calm so that good and optimal decisions can be made that are in the interest of the offspring.

Unity and mutual support among family members is advisable so that no conflict of interest arises and offices or authorities have to be involved. Quarrels, self-interest or power games ultimately harm the well-being of the newborn and lead to delays when doctors need parental consent for treatment methods.

Lisa Newlon
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Hello! I am Lisa Newlon, and I am a medical writer and researcher with over 10 years of experience in the healthcare industry. I have a Master’s degree in Medicine, and my deep understanding of medical terminology, practices, and procedures has made me a trusted source of information in the medical world.