Pairing jaundice

Description:

So-called, pairing jaundice can arise not only at newborns. The matter is that this term is meant as not some specific disease and a form of jaundice. It is known that jaundice is caused by bilirubin which happens to the straight lines (connected or conjugated) and indirect (untied, or not conjugated). Depending on that what its fraction prevails and designate a type of jaundice.
Pairing jaundice can be several types:
      1 Physiological (tranzitorny) jaundice of newborns
      2. Jaundice of premature newborns
      3. Hereditary jaundice (Zhilbert, Kriglera-Najjar and Lyutseya-Driskop's syndromes)
      4. Jaundice of children who are on natural (chest) feeding (a pregnanov or Ariyes's syndrome)
      5. Jaundice at children with asphyxia
      6. Medicamentous jaundice
      7. Jaundice at children with endocrine pathology

Symptoms of Krnjyugatsionny jaundice:

1. The hyperbilirubinemia develops at all newborns in the first to Yana lives, however yellowness of integuments is noted only at 60-70%. Concentration of bilirubin (in the further called B) in blood serum in the first days of life increases with a speed of 1,7-2,6 µmol/l/h and reaches for 3-4 day on average 103-137 µmol/l (The B in serum of umbilical blood makes 26-34 µmol/l). Increase in level B goes at the expense of not conjugated its fraction - indirect.
Yellowness of integuments appears at tranzitorny jaundice of newborns for 2-3 day of life when concentration of the indirect bilirubin (IB) reaches at the full-term newborn 51-60 µmol/l. Does not demand treatment.

2. Jaundice of premature newborns is shown at the level of the indirect bilirubin (IB) within 85-103 µmol/l and is noted at 90-95% of premature children. More than at 20% of children the maximum concentration of NB in blood exceeds 171 µmol/l and development of a kernicterus is possible in such cases.

Unlike physiological jaundice at full-term, the maintenance of an indirect bilirubin in blood premature usually above, but its accumulation goes more slowly. If at premature the maximum concentration of NB in blood is reached by 5-8 day and averages 137-171 µmol/l, then at full-term the peak of level of an indirect bilirubin falls on the 3rd day and makes 77-120 µmol/l. Decrease in maintenance of NB at premature goes also in a slowed-up way - up to 3rd and more weeks. Slower development of fermental systems of a liver in the premature child creates threat of bilirubinovy intoxication.

3. Hereditary konyogatsioniy jaundice (Zhipbert's syndrome) - the most widespread. Its frequency in population makes 2-6%. It is transferred on autosomal dominantly type. Defect of capture of bilirubin hepatocytes owing to disturbance of their function and decrease of the activity of a glyukuroniltransferaza of a liver is the cornerstone of development.
Typical sign of this type of jaundice is absence of anemia, splenomegaly, a reticulocytosis and other signs of a cytolysis. Raising of an indirect bilirubin in blood serum is not high, cases of a kernicterus is not described. In diagnosis significance is attached it is long to the keeping indirect hyperbilirubinemia, the analysis of a family tree, an exception of other reasons of a hyperbilirubinemia.

4. Hereditary jaundice (Kriglera-Najjar's syndrome). It is characterized by lack of a glyukuroniltransferaza in a liver (the I type) or its very low activity (the II type). At the first type jaundice is shown in the first days of life and steadily grows on intensity. NB in blood serum reaches 428 µmol/l and more. Development of a kernicterus, lack of effect of treatment by phenobarbital is typical, but improvement from phototherapy is reached. According to indications make zamenny hemotransfusion.

5. Hereditary jaundice of Lyutseya-Driskol is inherited on an autosomal recessive noma to type and is caused by deep, but tranzitorny, neonatal defect of activity of a glyukuroniltransferaza.
The high hyperbilirubinemia is presented, generally the indirect fraction of bilirubin also notes in the first days of life. Development of a kernicterus is possible. Believe that the factor inhibiting bilirubin conjugation - one of pregnancy hormones since it is revealed in blood serum of mothers.

6. Jaundice at the children who are on natural (chest) feeding of an uyegnanov or Ariyes's syndrome). It is described for the first time in 1963 by I. Ariyes and соавт. The generalized data of literature on these questions such:
a) at the newborns who are on breastfeeding (including donor milk) the frequency of development of pathological hyperbilirubinemias within the first week of life is 3 times more, than at their age-mates transferred to artificial feeding by the adapted mixes from cow's milk;
b) value as causative factors - starvations, frequencies of feedings, expressivenesses of loss of initial body weight, components of women's milk (pregnandiol, activity of a lipase and level of fatty acids, etc.), the increased bilirubin reabsorption from intestines is discussed. It is established that early applying to a breast and 8-times feeding reduce the frequency of hyperbilirubinemias at newborns. However in the first days of life the child, at the unsteady lactation at mother, receives a smaller kalo-passion, than the baby from a horn at artificial feeding. Expressiveness of loss of initial body weight also does not define probability of development of this jaundice. Opinions and on an etiological role of level of pregnandiol in mother's milk, activity of a lipase and free fatty acids are contradictory (there are no signs of an increased hemolysis);
c) Hyperbilirubinemias at newborns a late otkhozhdeniye of meconium (after 12 h life), a delay of crossclamping of an umbilical cord, appointment of mother in labor of oxytocin, diazoxide, salicylates, etc. promote.
Thus, different newborns have a pathogeny of "jaundice from maternal milk") can be гетерогенен, but nevertheless disturbances of excretion and conjugation are considered leading

The NB level izheniye on 85 µmol/l and more at the feeding termination by maternal milk for 48-72 hours can be diagnostic indirect test for "jaundice from maternal milk".

7. Jaundice at children with asphyxia and a birth trauma. In the conditions of a hypoxia and asphyxia formation of glyukuroniltransferazy system is late, there occurs dissociation of a complex bilirubin-albumine, increases pronitsa-rst vessels and a blood-brain barrier owing to what at the newborn the hyperbilirubinemia and even a clinical picture of a kernicterus can develop.

The intracraneal hemorrhages and disturbances of cerebral circulation, considerable talogematoma arising in the complicated labor and childbirth in buttock presentation, and ке considerable hemorrhage in skin can be sources of formation of NB and its strengthened penetration into blood with development of an icteric prokrashivaniye of skin and vnut-them bodies.


The clinical picture will depend at the same time on weight of a hypoxemic-asfiksichesky syndrome of disturbance of cerebral circulation and level of bilirubin in blood serum.

8. Medicamentous jaundice. To medicines which can contact glucuronic acid carry levomycetinum, menthol, salicylates, streptocides, high doses Vit. To, quinine. Appointment as their newborn can lead to the expressed hemolysis of erythrocytes, a hyperbilirubinemia and development of jaundice. At the same time the anemization, decolouration of a chair and gepatosplenomegaliya does not happen. In clinic only against the background of an intensive hyperbilirubinemia slackness, a loss of appetite, late recovery of initial weight atsya.

9. Jaundice at children with endocrine pathology. Is one of symptoms of an inborn hypothyroidism and usually has a long current. Emergence it is explained: a carotenemia (the liver loses ability to turn carotene into Vit. A); disturbance of education and biliation; the increased ability of skin to detain bilirubin; deficit of thyroxine (products of glyukuronilt-ransferazny enzyme are slowed down and process of conjugation of an indirect bilirubin is broken.

Reasons of Krnjyugatsionny jaundice:

Pairing jaundice owing to disturbance in work of fermental systems of a liver develops – process of binding of free bilirubin therefore its level sharply increases is broken. Unfortunately, this bilirubin in high concentration has toxic effect on a nervous system and it is not desirable to allow it high values extremely.

If to speak about the reasons on which pairing jaundice, then it as a rule the states accompanied with temporary increase in formation of bilirubin (the strengthened erythrocytolysis) develops, then the liver is simply not capable at once will cope with such amount of toxin. In other cases pairing jaundice develops in view of severe damage of a liver or inborn insufficiency of fermental system of a liver (so imposed pigmental hepatoses)

Treatment of Krnjyugatsionny jaundice:

Treatment is caused by the main zubolevaniye at which pairing jaundice develops.
At Zhilbert and  Kriglera-Najjar's syndromes the good therapeutic effect gives phenobarbital - intensity of jaundice decreases or it disappears.
Also the phototherapy is applied. According to indications make zamenny hemotransfusion.
At Kriglera-Najjar's syndrome also purpose of the drugs reducing gepatoenterogenny circulation of bilirubin - enterosorbents, холестирамин, an agar-agar is shown. Also new approaches to treatment - liver transplantation and a defective gene are developed.
Treatment of jaundice at children with asphyxia and a birth trauma: elimination of a hypoxemic-asfiksichesky syndrome; purpose of the funds allocated for reduction of a hyperbilirubinemia (albumine, enterosorbents, etc.); frequent replacement of blood at critical levels of bilirubin.