Nuclear neonatal jaundice - Newborn brain damage due to the direct cytotoxic effect of high concentrations of bilirubin. Manifested pronounced muscular hypertonicity: tilting the head back, Rolling Eyes. Characterized by frequent bouts of sleep apnea, can join convulsions. Nuclear neonatal jaundice leads to the development of persistent neurological disorders. Diagnosis is based on clinical and laboratory confirmation of hyperbilirubinemia. Treatment is aimed at the normalization of the level of bilirubin in the blood and elimination of neurological symptoms.
encephalopathy occurs even before the bilirubin colors the structure of the brain, so it can be argued that nuclear neonatal jaundice is a severe form of bilirubin encephalopathy. The exact incidence of the disease is unknown. The urgency in pediatrics incredibly high due to the high mortality rate, especially among premature infants and children with hemolytic disease of the newborn. In 80% of cases are reported persistent neurological effects, leading to a decrease of adaptation and disability.
neonatal jaundice, but normally handles baby and the symptoms disappear within a few days or weeks. Nuclear jaundice neonatal hyperbilirubinemia is characterized by excessive, resulting in bile pigment begins to pass through the blood-brain barrier, turning the basal ganglia, the nucleus of the cerebellum and the cranial nerves.
For the development of nuclear jaundice necessary set of conditions. In particular, the blood-brain barrier allows only unconjugated bilirubin, ie one that is not bound to albumin. So, one of the factors contributing to the development of this condition is hypoproteinemia. For this reason, the symptoms are more common in children with edema, and so extensive cephalohematoma. D. The hypoxic, infectious or traumatic brain injury, which occurred in utero or at birth, greatly increases the permeability of the blood-brain barrier. The same applies to prematurity, brain structures when the time of birth are immature.
Hemolytic disease of the newborn is always a factor of increased risk of kernicterus newborns, because in this case the concentration of unbound bilirubin most quickly reach the maximum permissible value and exceeds them, facilitating the penetration of bilirubin in the brain substance. In such cases, the condition develops rapidly, often with fatal or profound disability. Regardless of the factors contributing to the development of the disease, it is important to understand that there is no specific values of bilirubin, in which the symptoms of a nuclear encephalopathy. The number is always individual and depends on the child's compensatory systems: the liver, kidneys and brain with blood-brain barrier, etc.
sepsis, birth trauma in neonates with bleeding and so on. e., making it difficult to early detection of pathology.
Spastic stage of kernicterus infants presented severe gipertonusom extensor muscles until opisthotonos. The baby's head involuntarily sharply throws back, his eyes rolled up (a symptom of "the setting sun"). Breathing violated, frequent apnea episodes. There may be tonic convulsions and squawk associated with muscle tension and partly from the pain accompanying hypertonicity. In severe cases of kernicterus newborn muscle tension applied to the limb with their sharp straightening and pronation compressed cams.
Within a few weeks of the child's condition improves. Symptoms subside, spasms and hypertonicity observed less frequently. However, by the end of the first year of life in 80% of cases develop muscle rigidity, seizures are increasing again. Progressing symptoms of cranial nerve, which clinically manifested decrease in vision and hearing. Often develops strabismus and hearing loss. The massive loss of neurons inevitably affects brain development. In children undergoing nuclear jaundice, subsequently often diagnosed attention deficit hyperactivity disorder (ADHD), and mental retardation.
pediatrician suspected nuclear jaundice. On the other hand, increased muscle tone may be the consequence of brain damage of any other nature. It is therefore important to assess the level of bilirubin in the blood, and track it over time. The rate of concentration of bile pigment in the blood and for the adult and the child is about 20 mmol /l, but with neonatal jaundice, this value can be up to 200 mmol /l is acceptable for children first weeks of life.
Be sure to conduct full-scale blood analysis, direct and indirect Coombs test. It is believed that bilirubin begins to penetrate the blood brain barrier when the concentration of 400 mmol /l, but this figure is a schematic and can significantly decrease when exposed to a variety of factors listed above. Therefore, it is important to focus not only on the level of hyperbilirubinemia, but also on the condition of the child. Ideally, treatment of neonatal kernicterus should begin before the onset of symptoms. Typically, therapeutic activities are conducted upon reaching 50-70% of the maximum permissible level of bilirubin.
indirect bilirubin in an isomer that can be excreted by the liver and kidneys without binding to albumin blood. If background phototherapy bilirubin level is not reduced, it is showing a transfusion of blood plasma. Transfused with plasma binding proteins contribute to excess bilirubin followed by excretion from the body.
With the ineffectiveness of these treatments or the rapid progression of symptoms is carried out direct blood transfusion. It is also possible to use drugs that prevent the formation of bilirubin. This is especially true if the parents of a child for any reason, refuse to accept blood transfusions. This occurs in practice, because whole blood is associated with the development of life-threatening complications such as incompatibility reactions. Nuclear neonatal jaundice - a manifestation of a direct toxic effect of bile pigments in the neurons of the brain, so the treatment is always used neuroprotective. According to testimony carried anticonvulsant therapy.
Prediction and prevention of kernicterus newborn
Prognosis is poor condition. The cytotoxic effect of bilirubin in the brain cells permanently. In most cases, children who have had a nuclear jaundice develops bilateral choreoathetosis, there is a decrease of vision and hearing loss, sometimes significant. There is mental retardation up to the mental retardation of varying degrees. Preventing state is possible in the first days of life. Prophylactic phototherapy held all newborns with bilirubin levels in the blood above 200 mmol /l, as well as all preterm infants, children who have had intrauterine infection, fetal hypoxia, birth trauma, and others.