Kramer scale for neonatal jaundice. Jaundice syndrome in the newborn period Jaundice of newborns severity according to Kramer

Neonatal jaundice occurs due to impaired bilirubin metabolism. Transient (also called physiological) can pass without a trace for the first few days of a child’s life. It will take a long time to cure some types of severe pathologies. The doctor, of course, determines why the baby became “golden” after birth. But expectant mothers need to know general information about the disease so as not to panic ahead of time.

Causes of the disease

Bilirubin is formed through the breakdown of red blood cells. These red blood cells contain hemoglobin, which provides oxygen to the body. The fetus receives oxygen from special fetal red blood cells, which disintegrate at the moment of birth. This produces a large amount of bilirubin, which in some cases the liver cannot cope with, since not all of the child’s enzyme systems have yet adapted to new living conditions. The removal of pigment through urine is difficult, and the skin of children turns golden.

After a week or a little more, these functions normalize their activity, and the remaining bilirubin leaves through the excretory system. This happens in healthy children.

These symptoms are included in the concept of “norm”. Jaundice in premature infants occurs more often and can be characterized as prolonged. Moreover, how long the “golden” period will last depends not on weight, but on the degree of maturity of the child’s body. It also affects how well a woman’s pregnancy progressed. Yellowness of the skin is more common in children who have suffered birth trauma, twins, or those whose mothers have diabetes.

Physiological jaundice of newborns does not imply any external manifestations, except for changes in skin tone, it is not contagious. Unless the baby becomes a little more lethargic and drowsy. Although some doctors use the Cramer scale to visually determine the severity of the disease. According to this system, the first degree of severity occurs when the bilirubin level is 80 µmol/l and is characterized by pigmentation on the baby’s face and neck. When the readings increase to 150 µmol/l, the yellow color extends to the level of the navel. The third degree of severity involves yellow coloration up to the knees and 200 µmol/l bilirubin. If only the palms and soles remain non-golden – fourth degree (300 µmol/l). The most severe stage on the Cramer scale is characterized by a complete change in pigmentation and a bilirubin level of 400 μmol/L.

The normal amount of dangerous pigment in the blood on the first day after birth is up to 60 µmol/l. This rate can increase for five days, and by the end of the first month it can decrease significantly. In premature infants, transient neonatal jaundice is characterized by an increase in indirect bilirubin levels to 103 µmol/l.

Postpartum jaundice of newborns occurs in 90 percent of these babies. But if the test values ​​jump up significantly, there is a danger of kernicterus.

It can cause damage to the subcortical parts of the brain, and its consequences can lead to deafness, paralysis, blindness, mental retardation and even death. A strong increase in bilirubin values ​​requires immediate help. Alarming data: 320 µmol/l in a full-term infant at the very beginning of life and 150–250 µmol/l in the blood of a premature infant a week after birth.

If the baby’s bilirubin level and the norm for this period differ significantly, stool and urine tests and an abdominal ultrasound are prescribed to determine the cause.

It differs from all other species by exhibiting the following symptoms:

1. Orangeish skin tone.
2. Pigmentation is uneven, with bright spots on the feet, palms, near the ears, nose and lips.
3. The whites of the eyes are not stained.
4. The normal color of stool and urine, as well as general condition.

In children in the first month of life, this type occurs infrequently, only when the mother eats a lot of vegetables and fruits containing carotene. The disease goes away on its own and has no consequences.

Sometimes postpartum jaundice in newborns can be a reaction to breast milk. This type is called pregnane jaundice (Aries syndrome), it manifests itself in an average of three percent of babies in the first month of life.

It is diagnosed by briefly weaning the baby and switching to pasteurized breast milk.

In most cases, the malaise goes away within two days, as a result of which the child is transferred back to breastfeeding.

Pathological forms of the disease

Jaundice of newborns can be in pathological and non-pathological forms. Transient jaundice of healthy infants must be differentiated from dangerous types of the disease. There are at least 50 conditions when children in the first month of life have yellowish pigmentation of the skin and sclera! Pathological jaundice of newborns is provoked by serious illnesses, as a result of which their treatment usually takes place in the hospital.

Signs of such jaundice in newborns:

• change in urine pigmentation towards darkening and light-colored feces;
• too bright jaundiced shade of the child’s skin and sclera;
• bruises and minor hemorrhages appearing from nowhere;
• childhood lethargy, drowsiness;
• reluctance to suckle milk;
• high temperature as normal;
• hysterical crying with throwing back of the head, convulsions;
• an increase in the volume of the liver and spleen, noticeable upon palpation;
• vomiting yellowish milk.

Signs such as heredity and the child’s behavior are taken into account. Pathological blood tests usually show increased breakdown of red blood cells and symptoms of anemia.

Types of the most common pathological conditions:

All these pathological manifestations require close medical supervision and medical and sometimes surgical treatment.

Modern methods of therapy

Previously, intravenous glucose, choleretic drugs, ascorbic acid, and other drugs were used to eliminate excess bilirubin. But this treatment was found to be insufficiently effective. Now there are new, non-drug methods in which neonatal jaundice is treated more effectively and at the same time more safely.

To reduce the amount of dangerous pigment in the blood, phototherapy (light therapy) is used. This technique reduces the toxicity of a dangerous pigment quickly and painlessly (the course of therapy includes several stages and lasts an average of 96 hours).

Under the influence of light from a special ultraviolet lamp, bilirubin is transformed into a harmless derivative - lumirubin, which naturally leaves the body through the excretory system.

Minor side effects of light therapy include:

• peeling, dry skin;
• frequent diarrhea;
• drowsiness.

But all of them go away completely after completing the course of therapy. Normal health is restored.

Phototherapy is usually carried out in a hospital setting; the doctor decides how long it will take. But in mild forms of physiological jaundice, when the symptoms are minor, mother and baby can be discharged. In this case, home therapy is carried out using a fiber optic blanket or bandage. These items also help reduce bilirubin, but more slowly than phototherapy. In some cases, both therapeutic methods are used simultaneously.

In the case of phototherapy in a hospital setting, mothers are allowed to stay with the baby and take care of him - the disease is not contagious.

What can parents do to ensure that the therapy is successful and the child is not nervous:

• During a phototherapy session, when the baby is lying in the incubator, you should touch the baby more often. This can be done through special holes on the sides of the structure.
• If the baby cries, you can take him out for a while to calm him down.
• It is worth talking to the little patient and singing songs to him.

When neonatal jaundice is treated at home, weekly monitoring of bilirubin levels and visits to the home nurse will likely be required. In good weather conditions, it’s a good idea to let your baby soak in the indirect rays of the sun.

Breastfeeding also contributes to the removal of dangerous pigment, because mother's milk contains special enzymes. Why is this especially important in the first days after birth? Colostrum during this period gives a laxative effect and promotes the rapid elimination of excess bilirubin. And in the future, the best therapy against neonatal jaundice will be early and frequent feedings.

Such a child is often overcome by drowsiness, so they need to be woken up during meals. A nursing mother is also required to exclude fatty, spicy, fried, smoked foods and fast food from her diet so as not to harm the baby’s liver. You should not eat a lot of orange and yellow fruits rich in carotene.

In all cases of home treatment, you need to carefully monitor the child: does the yellowish color spread, does the behavior change. And if alarming symptoms appear, consult a doctor.

If it is diagnostically confirmed that the jaundice of a newborn is pathological in nature, serious therapy will be required. Drugs are prescribed depending on the type of disease. Phototherapy and intravenous administration of glucose and saline solution are also used as additional procedures. With the hemolytic type of the disease, the baby will need immunoglobulin. If the effect of drug treatment is insufficient, a blood transfusion may be required in the hospital.

Each disease has its own methods of prevention. A disease such as newborn jaundice is no exception. Here parents must take care of the health of their offspring. In medical centers that deal with family planning, you can undergo tests for the presence of genetic diseases even before the baby is born. The expectant mother should also take into account discrepancies in the Rh factors between her and the child.

- a physiological or pathological condition caused by hyperbilirubinemia and manifested by icteric discoloration of the skin and visible mucous membranes in children in the first days of their life. Jaundice of newborns is characterized by an increase in the concentration of bilirubin in the blood, anemia, icterus of the skin, mucous membranes and sclera of the eyes, hepato- and splenomegaly, and in severe cases – bilirubin encephalopathy. Diagnosis of jaundice in newborns is based on visual assessment of the degree of jaundice using the Cramer scale; determining the level of red blood cells, bilirubin, liver enzymes, blood type of mother and child, etc. Treatment of jaundice in newborns includes breastfeeding, infusion therapy, phototherapy, and replacement blood transfusion.

General information

Neonatal jaundice is a neonatal syndrome characterized by a visible icteric discoloration of the skin, sclera and mucous membranes due to increased levels of bilirubin in the baby’s blood. According to observations, in the first week of life, neonatal jaundice develops in 60% of full-term and 80% of premature infants. In pediatrics, physiological jaundice of newborns is the most common, accounting for 60–70% of all cases of the syndrome. Neonatal jaundice develops when bilirubin levels increase above 80-90 µmol/l in full-term infants and more than 120 µmol/l in premature infants. Prolonged or severe hyperbilirubinemia has a neurotoxic effect, i.e. causes brain damage. The degree of toxic effects of bilirubin depends mainly on its concentration in the blood and the duration of hyperbilirubinemia.

Classification and causes of jaundice in newborns

First of all, neonatal jaundice can be physiological and pathological. Based on the origin, newborn jaundice is divided into hereditary and acquired. Based on laboratory criteria, i.e., an increase in one or another fraction of bilirubin, a distinction is made between hyperbilirubinemia with a predominance of direct (bound) bilirubin and hyperbilirubinemia with a predominance of indirect (unbound) bilirubin.

Conjugation jaundice of newborns includes cases of hyperbilirubinemia resulting from reduced clearance of bilirubin by hepatocytes:

• Physiological (transient) jaundice of full-term newborns
• Jaundice of premature newborns
• Hereditary jaundice associated with Gilbert, Crigler-Najjar syndromes types I and II, etc.
• Jaundice due to endocrine pathology (hypothyroidism in children, diabetes mellitus in the mother)
• Jaundice in newborns with asphyxia and birth trauma
• Pregnane jaundice of breastfed children
• Drug-induced jaundice of newborns caused by the administration of chloramphenicol, salicylates, sulfonamides, quinine, large doses of vitamin K, etc.

Jaundice of mixed origin (parenchymal) occurs in newborns with fetal hepatitis caused by intrauterine infections (toxoplasmosis, cytomegaly, listeriosis, herpes, viral hepatitis A), toxic-septic liver damage due to sepsis, hereditary metabolic diseases (cystic fibrosis, galactosemia).

Symptoms of newborn jaundice

Physiological jaundice of newborns

Transient jaundice is a borderline condition in the neonatal period. Immediately after the baby is born, excess red blood cells containing fetal hemoglobin are destroyed to form free bilirubin. Due to the temporary immaturity of the liver enzyme glucuronyl transferase and intestinal sterility, the binding of free bilirubin and its excretion from the newborn’s body in feces and urine is reduced. This leads to the accumulation of excess bilirubin in the subcutaneous fat and discoloration of the skin and mucous membranes yellow.

Physiological jaundice of newborns develops on days 2-3 after birth, reaching its maximum on days 4-5. The peak concentration of indirect bilirubin averages 77-120 µmol/l; urine and feces are normal in color; the liver and spleen are not enlarged.

With transient jaundice of newborns, a mild degree of jaundice of the skin does not extend below the umbilical line and is detected only with sufficient natural light. With physiological jaundice, the newborn’s well-being is usually not affected, but with significant hyperbilirubinemia, sluggish sucking, lethargy, drowsiness, and vomiting may occur.

In healthy newborns, the occurrence of physiological jaundice is associated with temporary immaturity of liver enzyme systems, and therefore is not considered a pathological condition. When monitoring the child, organizing proper feeding and care, the manifestations of jaundice subside on their own by the age of 2 weeks.

Jaundice of premature newborns is characterized by an earlier onset (1–2 days), reaching a peak of manifestations by the 7th day and subsiding by three weeks of the child’s life. The concentration of indirect bilirubin in the blood of premature infants is higher (137-171 µmol/l), its increase and decrease occurs more slowly. Due to the longer maturation of liver enzyme systems, premature babies are at risk of developing kernicterus and bilirubin intoxication.

Hereditary jaundice

The most common form of hereditary conjugative jaundice of newborns is constitutional hyperbilirubinemia (Gilbert's syndrome). This syndrome occurs in the population with a frequency of 2-6%; inherited in an autosomal dominant manner. Gilbert's syndrome is based on a defect in the activity of liver enzyme systems (glucuronyl transferase) and, as a consequence, a violation of the uptake of bilirubin by hepatocytes. Jaundice of newborns with constitutional hyperbilirubinemia occurs without anemia and splenomegaly, with a slight increase in indirect bilirubin.

Hereditary jaundice of newborns in Crigler-Najjar syndrome is associated with very low glucuronyl transferase activity (type II) or its absence (type I). In type I syndrome, newborn jaundice develops already in the first days of life and steadily increases; hyperbilirubinemia reaches 428 µmol/l and higher. The development of kernicterus is typical, and death is possible. Type II syndrome, as a rule, has a benign course: neonatal hyperbilirubinemia is 257-376 µmol/l; Kernicterus rarely develops.

Jaundice due to endocrine pathology

At the first stage, the clinical signs of bilirubin intoxication predominate: lethargy, apathy, drowsiness of the child, monotonous cry, wandering eyes, regurgitation, vomiting. Soon, newborns develop classic signs of kernicterus, accompanied by stiff neck, spasticity of the body muscles, periodic agitation, bulging of the large fontanel, extinction of sucking and other reflexes, nystagmus, bradycardia, and convulsions. During this period, which lasts from several days to several weeks, irreversible damage to the central nervous system occurs. Over the next 2-3 months of life, a deceptive improvement is observed in the children’s condition, but already at 3-5 months of life, neurological complications are diagnosed: cerebral palsy, mental retardation, deafness, etc.

Diagnosis of jaundice in newborns

Jaundice is detected while the child is in the maternity hospital by a neonatologist or pediatrician when visiting a newborn shortly after discharge.

The Cramer scale is used to visually assess the degree of jaundice in newborns.

• I degree – jaundice of the face and neck (bilirubin 80 µmol/l)
• II degree – jaundice extends to the level of the navel (bilirubin 150 µmol/l)
• III degree - jaundice extends to the level of the knees (bilirubin 200 µmol/l)
• IV degree - jaundice extends to the face, torso, extremities, with the exception of the palms and soles (bilirubin 300 µmol/l)
• V - total jaundice (bilirubin 400 µmol/l)

The necessary laboratory tests for the primary diagnosis of jaundice in newborns are: bilirubin and its fractions, complete blood count, blood group of the child and mother, Coombs test, IPT, general urine test, liver tests. If hypothyroidism is suspected, it is necessary to determine the thyroid hormones T3, T4, and TSH in the blood. Detection of intrauterine infections is carried out by ELISA and PCR.

As part of the diagnosis of obstructive jaundice, newborns undergo ultrasound of the liver and bile ducts, MR cholangiography, FGDS, plain radiography of the abdominal cavity, consultation with a pediatric surgeon and pediatric gastroenterologist.

Treatment of neonatal jaundice

To prevent jaundice and reduce the degree of hyperbilirubinemia, all newborns require early (from the first hour of life) and regular breastfeeding. In newborns with neonatal jaundice, the recommended frequency of breastfeeding is 8–12 times a day without a night break. It is necessary to increase the daily volume of fluid by 10-20% compared to the physiological need of the child, and take enterosorbents. If oral hydration is not possible, infusion therapy is performed: glucose drip, physical. solution, ascorbic acid, cocarboxylase, B vitamins. In order to increase the conjugation of bilirubin, phenobarbital may be prescribed to a newborn with jaundice.

The most effective treatment for indirect hyperbilirubinemia is continuous or intermittent phototherapy, which helps convert indirect bilirubin into a water-soluble form. Complications of phototherapy may include hyperthermia, dehydration, burns, and allergic reactions.

For hemolytic jaundice of newborns, exchange transfusion, hemosorption, are indicated. All pathological jaundice of newborns require immediate treatment of the underlying disease.

Prognosis of newborn jaundice

Transient jaundice of newborns in the vast majority of cases resolves without complications. However, disruption of adaptation mechanisms can lead to the transition of physiological jaundice in newborns to a pathological state. Observations and evidence indicate that there is no relationship between hepatitis B vaccination and neonatal jaundice. Critical hyperbilirubinemia can lead to the development of kernicterus and its complications.

Children with pathological forms of neonatal jaundice are subject to dispensary observation by a local pediatrician and

Yellowing of the skin of newborns is a well-known phenomenon. But pregnane jaundice remains unexplained: its cause is considered to be breast milk.

From a quarter to a half of all born children develop jaundice on the 3rd-4th day of their life. Among premature babies, the proportion of jaundiced children reaches 90%. The skin, mucous membranes, and sclera of the eyes of infants turn yellow. Jaundice syndrome of newborns - and pregnane jaundice as one of its manifestations - is a natural manifestation of the child’s body’s adaptation to life in the external environment. But in some cases, this can be a serious illness that threatens the baby with disability and even death. Therefore, young mothers need to have an idea of ​​what kind of jaundice in newborns there is, so as not to worry in vain or, on the contrary, to sound the alarm in time - in serious cases.

Transient jaundice of newborns - physiological, pregnane - passes without a trace

By the middle of the first week of the child’s life, the pediatrician examining him suddenly declares that the facial skin and mucous membrane of the newborn’s eyeball have become “icteric” - i.e. turned yellow. Is this a pathology or a normal physiological phenomenon? Doctors answer that infantile jaundice is a borderline process: being normal, it must be controlled, because there is a possibility of adverse development. Jaundice syndrome is associated with the appearance of a special substance in the blood - bilirubin.

Bilirubin metabolism in newborns

After birth, the breakdown of fetal hemoglobin - F (HbF), which carries out oxygen exchange inside the mother's womb, begins in the child's blood. This hemoglobin binds and carries oxygen better, but easily breaks down when the temperature and acidity of the blood changes. A new hemoglobin A (HbA) is formed, which is more resistant to environmental fluctuations. This will become the main one in the red blood cells of the new person.

The breakdown of fetal hemoglobin occurs as follows: iron is torn from its molecule, then globin protein, the remaining part turns into bilirubin - a red bile compound (bilirubin in translation is red bile).

The red residue of hemoglobin binds to albumin, a blood plasma protein. The resulting compound is called indirect, free bilirubin. It is poisonous to the baby's tissues, especially brain cells. It is insoluble in water, so it is not excreted through the kidneys. With the blood flow, indirect bilirubin enters the liver.

Here, the bilirubin-albumin complex is converted by enzymes into an easily soluble compound. For the process to proceed quickly, the following must be present in the baby’s liver in sufficient quantities:

• Y- and Z-proteins in the cytoplasm;
• the enzyme uridine diphosphate glucuronyl transferase (UDPGT);
• glucuronic acid;

Consistently combining with protein molecules (Y- and Z-proteins), glucuronic acid, bilirubin is converted into a soluble complex, passes through the bile ducts into the intestines, is processed by the flora living there and is excreted from the body with feces.

Transient jaundice of newborns

Transient or physiological jaundice is a natural phenomenon of the postpartum development of a child, the body’s adaptation to existence in the external environment.

The breakdown of hemoglobin and the formation of bilirubin begin on the first day of life and increase by 2-3 days. By this time, the baby’s liver has only 5% of the necessary substances and enzymes. The bile ducts are not spacious enough to remove bilirubin from the body. It remains in the blood, accumulates and causes yellowing of the outer skin of the child. The yellowness syndrome increases until the end of the first week of life, yellowing reaches the level of the navel - no further. There is no enlargement of the liver and spleen, no accelerated breakdown of red blood cells and no anemia. The bilirubin level rises to a maximum of 200 µmol/l.

According to this scenario, physiological jaundice develops in the majority of newly born children. This is transient jaundice, i.e. passing, temporary. In the second week of life, the activity of liver enzymes increases, the bile ducts return to normal, the body improves the removal of bilirubin, and after 10-14 days physiological jaundice disappears.

Jaundice of newborns

Bilirubin metabolism in newborns is complicated by many factors, taking into account which several types of newborn jaundice are distinguished.

Conjugative

Conjugation jaundice develops due to insufficient ability of the liver to remove bilirubin; This type includes:

• physiological;
• jaundice of seven-month-old children: premature babies also suffer physiological jaundice, only in a more pronounced form and it lasts longer;
• received with genes from parents (Gilbert syndrome, etc.).
• jaundice from breast milk (Aries syndrome);
• jaundice associated with oxygen starvation - asphyxia;
• drug jaundice;
• jaundice due to hypothyroidism - decreased thyroid function, which is associated with liver function.

Hemolytic

Accompanied by pathological destruction of the baby's hemoglobin by maternal antibodies. The reason is a discrepancy between the blood of the child and the mother according to the Rh factor.

Parenchymatous

Congenital damage to the liver tissue; hepatitis of various origins.

Obstructive

The presence of damaged ducts in the liver that interfere with the transport of bile.

Any jaundice is associated with hyperbilirubinemia - an increased level of bilirubin in the blood. The walls of blood vessels in newborns have a permeability barrier, but if the amount of free bilirubin exceeds a critical level, this tissue poison will leak out and begin to poison the body.

The central nervous system will be affected first. Brain poisoning with bilirubin is called kernicterus or encephalopathy. Even if the threat of death passes, the child after such a complication remains disabled with irreversible damage to the central nervous system.

In order to notice pathological development in time, there is a visual diagnostic method - the Cramer scale.

Cramer scale for neonatal jaundice

Examination of the child’s skin allows you to determine the degree of accumulation of bilirubin in the blood and take timely measures to reduce it when it becomes critical. Jaundice according to Kramer has a gradation indicated in the table below.

Note: µmol/l - micromoles per liter

If the first and second degrees do not cause alarm - this is transient jaundice of newborns, then the third and higher degrees of jaundice are pathological symptoms and require intensive treatment.

Pregnane jaundice in newborns

One of the variants of physiological transient jaundice is infant jaundice from mother's milk. 2 percent of breastfed infants develop high levels of bilirubin in the blood. Newborns on artificial nutrition do not have such symptoms. The cause of this phenomenon has not yet been established.

Some researchers believe that pregnanediol, contained in the mother's blood, interferes with the conversion of bilirubin in the liver and its removal from the body. Pregnanediol is a product of the interaction of two female hormones, progesterone and estrogen. From the name of the inhibitory hormone, a disease of this kind received the name pregnane jaundice.

Aries syndrome

For the first time, the connection between increased bilirubin levels and breastfeeding was established by I.M. Aries, and the syndrome of jaundice as a result of breastfeeding was named after him. Three factors influence this process:

• the presence of pregnanediol in mother's milk;
• insufficient ability of the liver to remove bilirubin in the first days of life;
• late passage of feces of a newborn (12 hours after birth) - bilirubin from the intestines has time to be reabsorbed into the blood.

Causes of pregnane jaundice

• I. Reason No. 1 - mother’s milk itself, its composition. In cases where the child was transferred to artificial nutrition and was deprived of mother's milk, the jaundice disappeared within 2 days; when breastfeeding was resumed, her symptoms returned.
• II. The cause could be initial starvation and weight loss after birth. It has been proven that frequent breastfeeding and increased nutrition reduce bilirubin levels. A breastfed baby receives fewer calories than a bottle-fed baby. Due to lack of food, bilirubin can be reabsorbed from the intestines into the blood.
• III. The use of drugs that induce labor may affect the liver's ability to bind and eliminate bilirubin.
• IV. Factors that cause an increase in bilirubin in all children: premature birth; genetic predisposition; thyroid diseases; oxygen starvation after childbirth. These factors increase the likelihood of developing breast milk jaundice.

Symptoms and diagnosis of infant jaundice from breast milk

• Pregnane jaundice begins on the first day of feeding and lasts from 3 weeks to one and a half months. If the yellowing does not go away within this time frame, it means that it was not caused by mother's milk. Jaundice due to hypothyroidism lasts up to 6 months. There is a risk of confusing it with pregnane and delaying treatment.
• Yellowing rarely reaches grade 3 on the Cramer scale. Basically, the skin turns yellow on the face, shoulders to the navel.
• Canceling breastfeeding for 2-3 days and decreasing the bilirubin level by 85 µmol/liter at this time is a clear symptom of pregnane jaundice.
• yellowing is accompanied by mild intoxication: lethargy, anemia, drowsiness.

Treatment methods and prognosis

1. The main method of preventing and treating jaundice is to breastfeed the baby as often as possible: 8–12 times a day, including at night.
2. Increase fluid intake, administer it using a dropper.
3. Phototherapy gives good results: the baby’s body with a protected face should be exposed to sunlight or lamps as often as possible.
4. Free bilirubin, when exposed to light, turns into a soluble form and is excreted through the kidneys.

The latter method has complications:

• burns on the child’s body;
• dehydration of his body due to overheating;
• allergy.

Aries syndrome, like physiological jaundice, disappears without a trace and does not cause complications. But this does not mean that the child’s condition at this time does not require strict medical supervision. There is always a risk of discovering pathological jaundice instead of a transient form, which must be urgently treated.

RCHR (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical protocols of the Ministry of Health of the Republic of Kazakhstan - 2014

Hemolytic disease of the fetus and newborn (P55), Neonatal jaundice due to other and unspecified liver cell damage (P59.2), Neonatal jaundice, unspecified (P59.9), Neonatal jaundice due to other specified causes (P59.8), Neonatal jaundice due to other specified forms of excessive hemolysis (P58.8), Neonatal jaundice due to ingestion of maternal blood (P58.5), Neonatal jaundice due to infection (P58.2), Neonatal jaundice due to bruising (P58.0), Neonatal jaundice due to drugs or toxins passed from the mother or administered to the newborn (P58.4), Neonatal jaundice due to polycythemia (P58.3), Neonatal jaundice due to lactation inhibitors (P59.3), Neonatal jaundice due to excessive hemolysis, unspecified (P58.9), Neonatal jaundice associated with preterm delivery (P59.0)

Neonatology, Pediatrics

general information

Short description

Approved by the Expert Commission

On health development issues

Ministry of Health of the Republic of Kazakhstan

Neonatal jaundice- the appearance of visible icteric staining of the skin and mucous membranes of the newborn due to an increase in the level of total bilirubin in the blood serum.

Jaundice becomes visible when the serum total bilirubin (TSB) level reaches ³ 80 µmol/L. In low birth weight newborns, icteric discoloration of the skin appears at lower serum bilirubin levels, which depends on the thickness of the subcutaneous fat layer.

I. INTRODUCTORY PART

Protocol code:

Protocol name: Neonatal jaundice

ICD-10 code(s):

P55 Hemolytic disease of the fetus and newborn

P58.0 Neonatal jaundice due to bruising.

P58.2 Neonatal jaundice due to infection

P58.3 Neonatal jaundice due to polycythemia.

P58.4 Neonatal jaundice caused by drugs or toxins passed from the mother's body or administered to the newborn

P58.5 Neonatal jaundice due to ingestion of maternal blood

P58.8 Neonatal jaundice due to other specified forms of excessive hemolysis

P58.9 Neonatal jaundice due to excessive hemolysis, unspecified.

P59.0 Neonatal jaundice associated with premature birth (hyperbilirubinemia of prematurity)

P59.2 Neonatal jaundice due to other and unspecified damage to liver cells (congenital viral hepatitis).

P59.3 ​​Neonatal jaundice caused by drugs that inhibit lactation.

P59.8 Neonatal jaundice due to other specified causes.

P59.9 Neonatal jaundice, unspecified (Physiological severe jaundice).

Abbreviations used in the protocol:

G6PDG - glycose-6-phosphate dehydrogenase

GUT - glucuronyl transferase

TBS - total serum bilirubin

PB - direct bilirubin

NB - indirect bilirubin

OZBK is an exchange blood transfusion operation.

Date of development of the protocol: year 2013.

Protocol users: neonatologists, pediatricians, GPs.

Classification

Clinical classification

Physiological jaundice of newborns:

Usually appears after 36 hours of a child's life.

Peak serum total bilirubin levels usually occur on days 3–4 in a full-term baby and on days 5–7 in a preterm baby.

The peak serum total bilirubin level is ±205 µmol/L (12 mg/dL).

The level of total serum bilirubin decreases in a full-term baby by the 14th day of life, in a premature baby - by the 21st day of life.

The child’s clinical condition is satisfactory: he is active, has clearly distinguishable periods of sleep and wakefulness, and has a good sucking reflex; the child has a stable temperature; the liver and spleen are of normal size, the urine is light, and the stool is normally colored.

Jaundice associated with breastfeeding can be accompanied by two peaks in the rise of bilirubin, the first on days 4-5 and the second on days 14-15, decreases slowly and can be detected at the age of 12 weeks.

Pathological jaundice of newborns:

Jaundice that appears during the first 24 hours of life or is detectable on the feet and palms at any time.

Jaundice that appeared after the 7th day of life.

The rate of increase in total serum bilirubin is > 85 µmol/l/day or 5 mg/dl/day.

Direct bilirubin level > 34 µmol/L (2 mg/dL), or ³ 20% of total serum bilirubin level.

The child's condition is unsatisfactory, regardless of the level of bilirubin, the presence of pathological clinical symptoms.

Hepatomegaly and/or splenomegaly.

Discolored stool or dark-colored urine.

Jaundice without a tendency to decrease after the 14th day of life in a full-term newborn or after the 21st day of life in a premature newborn is called “protracted jaundice.”

Diagnostics

II. METHODS, APPROACHES AND PROCEDURES FOR DIAGNOSIS AND TREATMENT

Diagnostic measures

Basic diagnostic measures:

A. In the antenatal period, determine the blood type and Rh factor in all pregnant women, as well as the presence of a burdened transfusion and vaccination history.

B. At birth:

In a child from a mother with Rh-negative cord blood, determine the group and Rh-ness, Coombs test;

In a child born from a mother with blood type O (1) Rh-positive, determine the blood type and Coombs test in the umbilical cord blood.

The level of total bilirubin in umbilical cord blood and its fraction (if necessary) over time.

Additional diagnostic measures:

A. If you suspect the presence of neonatal jaundice caused by excessive hemolysis (P 58-58.9), determine the level of hemoglobin, hematocrit, the number of red blood cells, reticulocytes, microcytes.

B. If you suspect the presence of jaundice due to hypothyroidism, examine the level of thyroid hormones.

B. If you suspect the presence of hereditary pathological jaundice:

Kigler-Najjar syndromes types I and II, Lucey-Driscol, Gilbert - genetic examination;

Hereditary hemolytic anemias (membranopathy, hemoglobinopathies, enzyme-deficiency anemia, etc.) - examine a peripheral blood smear with a Price-Jones curve, calculate the thickness, sphericity index, average volume of erythrocytes and average concentration of hemoglobin in an erythrocyte, osmotic stability of erythrocytes (for more details, see the protocol "Hemolytic anemia").

If you suspect the presence of diseases of the liver or bile ducts, see the protocols “Diseases of the liver and hepatobiliary system”.

Diagnostic criteria

Complaints and anamnesis

Medical history:

Mother's Rh and blood group.

Infections during pregnancy and childbirth.

Hereditary diseases (G6PD deficiency, hypothyroidism, other rare diseases).

Presence of jaundice in parents.

Presence of jaundice in a previous child.

Baby's weight and gestational age at birth

Feeding the baby (underfeeding and/or vomiting).

Was the baby given resuscitation at birth?

Risk factors:

Acute hemolysis.

Premature birth.

Bruising and cephalohematomas.

Loss of body weight (> 10%).

Neonatal asphyxia, acidosis.

Hypoglycemia.

Neonatal infection.

Hypoalbuminemia (< 30 г/л).

Physical examination:

Examine a fully undressed child in good lighting (preferably daylight).

Provide your child with thermal protection during the examination.

Assess the color of the skin after lightly pressing with your finger to the level of the subcutaneous fat.

Determine the location of jaundice using the modified Cramer scale.
Modified Cramer scale:

Assess the severity of detected jaundice:

Laboratory research

There are various causes of pathological jaundice, so it is necessary to determine, in addition to OBS, bilirubin fractions (direct/conjugated bilirubin and indirect/non-conjugated bilirubin).

For neonates with jaundice and stable hyperbilirubinemia that lasts more than 3 weeks, testing for galactosemia is recommended.

If there is a family history of jaundice associated with G6PD deficiency, it is necessary to determine the level of this enzyme

Newborns with high levels of direct bilirubin should be evaluated for sepsis (laboratory and bacteriological blood tests) [D].

In newborns with elevated direct bilirubin levels and hepatomegaly, ALT and AST transaminase levels should be determined to exclude hepatitis.

Indications for consultation with specialists:

Infectious disease specialist

Surgeon,

Neurologist.

Differential diagnosis

Differential diagnosis

In the vast majority of cases, newborn jaundice is physiological, but it is very important to monitor each case for timely detection and appropriate treatment of “dangerous” jaundice in order to prevent complications:

Bilirubin encephalopathy: acute damage to the central nervous system.

Kernicterus: irreversible chronic damage to the central nervous system.

For early detection of signs of dangerous jaundice, it is important to record the time of onset of any jaundice and its regular clinical assessment (with an interval of 8-12 hours).

Jaundice after day 7 may be a sign of cholestasis, glucose-6-phosphate dehydrogenase (G6PDH) deficiency, Crigler-Nayjar disease, or late-onset neonatal sepsis.

Dangerous jaundice appears within the first 24 hours of life or can be detected on the feet and palms at any time.

Algorithm for the differential diagnosis of neonatal jaundice not associated with isoimmunization

Treatment

Goal of treatment: providing interventions to help prevent potential side effects from occurring.

Treatment tactics

Non-drug treatment

A. Physiological jaundice

Babies with “physiological jaundice” do not require special treatment, but require exclusive breastfeeding. The child's mother must be explained that exclusive breastfeeding (as often and for as long as the child wants, day and night, but at least 8-12 times a day) helps the child overcome jaundice faster.

B. Pathological jaundice

Phototherapy is the most effective method of reducing bilirubin levels in neonatal jaundice [A].

Timely and correct phototherapy reduces the need for replacement blood transfusion by up to 4% and reduces the risk of complications of neonatal jaundice.

Indications for phototherapy and exchange transfusion in a full-term newborn(see appendix, chart 1):

If jaundice appears in the first 24 hours after birth, or it is “dangerous”, phototherapy should be started immediately and the indication for an exchange transfusion should be considered.

In other cases, it is necessary to plot the result of OBS on the chart in accordance with the age of the child and decide on the initiation of phototherapy.

In the presence of risk factors, the decision to initiate phototherapy should be based on lower serum total bilirubin (TSB) levels.

If the child experiences a deterioration in general condition, phototherapy should be started at lower RBS values.

The indications for starting phototherapy in a premature baby are the same as for newborns with hemolytic disease (see appendix graph 2).

Phototherapy is carried out using an incubator or in a warm crib, in a room protected from drafts

The child's eyes should be protected with a light-proof bandage.

Monitor the child’s body temperature and the air temperature under the lamp every 3 hours. Maintain your body temperature at 36.5-37.5 °C.

Turn your baby over every 3 hours.

Weigh your child at least once a day.

During phototherapy, your baby's stool may be loose and yellow in color. This is normal and does not require treatment.

Continue breastfeeding on demand, without night breaks, at least 8 times a day. [B]

If the baby is receiving expressed breast milk or intravenous fluids during phototherapy, it is recommended to increase the volume of milk/solutions to 10% of the total daily requirement.

Determine the OBS level every 12 hours.

Phototherapy in a newborn is stopped in the event of a sustained decrease in serum bilirubin concentration within 24-36 hours below the values ​​that became the basis for the initiation of phototherapy and the presence or absence of risk factors.

Indications for exchange blood transfusion:

In the event of the appearance of clinical symptoms of acute bilirubin encephalopathy (muscle hypertonicity, opisthotonus, fever, “brain scream”), an exchange transfusion is performed regardless of the bilirubin level. [D]

Replacement blood transfusion in the case of HDN caused by an isolated Rhesus conflict uses Rh-negative red blood cells and plasma of the same group as the child’s blood, but, if possible, AB (IV) blood group in a 2:1 ratio.

Replacement blood transfusion in the case of HDN caused by an isolated group conflict uses group O (I) red blood cells that match the Rhesus group of the child's red blood cells and single-group or AB (IV) group plasma in a 2:1 ratio.

Replacement blood transfusion in case of incompatibility of the mother's blood and the child's blood due to rare factors must use blood from individually selected donors.

It must be remembered that for children with tension-type headache, erythromass is used with a shelf life of no more than 72 hours.

Exchange transfusion, which is an unsafe procedure, should only be performed by trained personnel under strict indications after phototherapy.

Further management: neonatal care, breastfeeding.

Discharge criteria:

Discharge of a newborn with jaundice from the hospital should be carried out individually, taking into account the severity of jaundice and the results of treatment.

Every mother/family needs to be taught how to assess for jaundice and how to recognize “danger signs” in a baby and how to perform exclusive breastfeeding.

Surgical intervention: in cases of obstructive jaundice.

Emergency hospitalization of a newborn with pathological jaundice from obstetric organizations of the 1st and 2nd levels to the emergency department of obstetric organizations of the 3rd and 4th levels.

Information

Sources and literature

1. Minutes of meetings of the Expert Commission on Health Development of the Ministry of Health of the Republic of Kazakhstan, 2014
   1. 1)American Academy of Pediatrics. Clinical practical Guideline. Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation. 2004. 2)A guide for essential practice. World Health organization, Geneva 2006 3) Bhutany V, Gourley G, Adler S, Kreamer B, Dalin C, Johnson L. Non-invasive Measurement of Total Bilirubin in a Multiracial Predischarge Newborn Population to Assess the Risk of Severe Hyperbilirubinemia. J. Pediatrics (106) 2000, NO 2. 4) David E. Hertz. Care of the Newborn: A Handbook for Primary Care. Lippincott Williams, 2005. 60-72 5) Essential Newborn Care and Breastfeeding: Training Module. WHO Euro, Copenhagen, 2002. 6) Guidelines for detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants (35 or more weeks’ gestation), Canadian Pediatric Society, 2007. 7) Jaundice and hyperbilirubinemia in the newborn. In: Berhman RE, Kliegman RM, Jenson HB, eds. Nelson textbook of pediatrics. 16th ed. Philadelphia: Saunders, 2000:511-28. 8) Jaundice in the Healthy Term Newborn. British Columbia Reproductive Care Program Newborn Guideline 4. April 2002 (Revised). 9) Jaundice. RPA newborn care protocol book. Royal Prince Alfred Hospital. 2003. 10) Meredith L. Porter, Beth L. Dennis. Hyperbilirubinemia in the Term Newborns. American Family Physician, 2002, Volume 65, Number 4. 11) Managing Newborn Problems: A guide for doctors, nurses, and midwives. WHO, Geneva, 2003. 12) Maisels MJ, Watchko JF. Treatment of jaundice in low birth weight infants. Arch. Dis. Child. Fetal Neonatal Ed. 2003; 88; 459-463). 13) Mills JF, Tudehope D. Fibreoptic phototherapy for neonatal jaundice (Cochrane review). The Cochrane library. Issue 2, 2003. 14) Management of Hyperbilirubinemia in the newborn infants 35 or more weeks of gestation. American Academy of Paediatrics Subcommittee on Hyperbilirubinemia. Paediatrics 2004; 114:297-316. 15) Newborn Services Clinical Guideline. Protocol Management of Neonatal Jaundice. New Zealand. Reviewed by Peter Nobbs, May 2001. 16) Newborn Services Clinical Guideline, Assessment of Prolonged and Late-Onset Jaundice, 2004. 17) Shelly C Springer. Kernicterus. EMedicine. Meredith L. Porter, Beth L. Dennis. Hyperbilirubinemia in the Term Newborns. American Family Physician, 2002, Volume 65, Number 4.

   Schedule 2: Indications for phototherapy and exchange transfusion in a premature newborn or newborn with signs of hemolysis
   

   
   

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In most cases, jaundice in newborns manifests itself during the first 3 days of life, is physiological in nature, being a “borderline condition”, and does not require treatment.
The danger of jaundice lies in the fact that with a high level of free (unconjugated, indirect) bilirubin in the blood serum, there is a threat of developing bilirubin encephalopathy (kernicterus), which occurs exclusively during the newborn period and leads to the development of profound mental retardation and cerebral palsy (CP) . The degree of toxic effects of bilirubin mainly depends on its concentration in brain tissue and the duration of hyperbilirubinemia. The health care worker observing the child should be able to assess the individual “safe level” of bilirubin in a newborn with jaundice and anticipate its possible increase.

The mechanism of jaundice in newborns

Jaundice, or icterus, is yellow pigmentation of the skin and/or sclera with bilirubin. It, in turn, is caused by an increase in the level of bilirubin in the blood - hyperbilirubinemia. In total, there are about 50 diseases that are accompanied by the appearance of yellowness of the skin. In adults, skin staining occurs when the level of total bilirubin increases to more than 34 µmol/l, in full-term newborns - when the level of total bilirubin is approximately 70 µmol/l, in premature infants - at a level of 50 µmol/l.
The exchange of bilirubin in a newborn has a number of features.
Increased formation of bilirubin in newborns is associated with the destruction of fetal hemoglobin, which is released in large quantities into the child’s bloodstream during labor, and the transient inability of the newborn liver to conjugate (bind). The “launch” of the bilirubin conjugation system normally occurs within a period of several hours to several days after birth. The activity of the liver conjugating system increases slowly and reaches adult levels by the end of the 3-4th week of life.
Bilirubin is constantly being exchanged in the body of any person. Bilirubin is formed mainly due to the breakdown of hemoglobin in aging red blood cells. Normally, about 80-85% of bilirubin is formed per day. About 15-20% of bilirubin is formed from other sources (bone marrow, liver). With the destruction of red blood cells that have reached the end of their normal lifespan - approximately 120 days, hemoglobin is released, from which globin is first separated, and from the remaining non-protein part of the molecule (heme), as a result of biochemical transformations, bilirubin is formed, which is called free, or indirect, bilirubin. This form of bilirubin is fat soluble. Indirect bilirubin in the bloodstream binds to proteins (albumin) and in this form is transported to the liver. In the liver, indirect (free) bilirubin is taken up by liver cells (hepatocytes) and converted into another form of bilirubin bound to enzymes. This fraction of bilirubin changes its properties, becomes water-soluble, and is called the bound, or direct, fraction - direct bilirubin. This bilirubin is excreted from the liver into the gallbladder and subsequently enters the intestines, turning the stool dark. A small part of direct (bound) bilirubin enters the general bloodstream and is excreted from the body through the kidneys, giving the urine a yellowish tint. Bilirubin is a pigment, its free fraction is a bright shade of yellow, and its bound fraction is a dirty yellow color.
The excretory function of the liver at the birth of a child is significantly reduced due to the anatomical immaturity of the excretory system: the bile capillaries are narrow, their number is reduced. The excretory function of the liver of newborns reaches the eliminating capacity of the liver of adults by the end of the first month of life.
The peculiarity of the intestinal metabolism of bile pigments in newborns contributes to the partial return of unconjugated bilirubin into the blood and an increase or maintenance of hyperbilirubinemia.
In newborns, 80-90% of bilirubin is represented by the indirect (free) fraction.

Types of jaundice during the newborn period

Based on the content of bilirubin fractions in blood serum, they are distinguished:
- unconjugated (indirect) hyperbilirubinemia (the level of indirect bilirubin is more than 85% of total bilirubin);
- conjugated (direct) hyperbilirubinemia of newborns (characterized by a level of direct bilirubin greater than 15% of total bilirubin).
Indirect (unconjugated) bilirubinemia
Physiological jaundice of newborns, or jaundice of a healthy newborn, is also called transient. Physiological hyperbilirubinemia develops in all newborns in the first days of life, and jaundice of the skin, i.e. direct physiological jaundice, only in 60% of full-term and 80% of premature infants. Yellowness of the skin appears on the 2-3rd day of life, when the concentration of indirect bilirubin reaches 51-60 µmol/l in full-term newborns, and 85-103 µmol/l in premature newborns.
Physiological jaundice is caused by the peculiarities of bilirubin metabolism during this period of life:
- high release of red blood cells during childbirth;
- high content of fetal hemoglobin;
- its rapid destruction after childbirth;
- deficiency of conjugating enzymes in the liver. Main clinical characteristics of physiological jaundice:
- appears 48 hours after birth, sometimes on the second day of life;
- the rise in bilirubin levels does not reach critical values ​​that can cause kernicterus;
- the condition of the newborn remains satisfactory.
Physiological jaundice goes through two temporary phases. The first covers 5 days of life from the moment of birth and is characterized by a relatively rapid increase in the level of free (unconjugated, indirect) bilirubin. The second phase is characterized by a slow decrease in indirect bilirubin, which reaches a normal level (below 50 µmol/l) by the 11th-14th day of life, sometimes it lasts up to 1 month of life, depending on the functional maturity of the child and the type of feeding (breast milk can contribute to the delay jaundiced skin color in a child).
First of all, with physiological jaundice, the face is stained, then the neck, torso and limbs. The decrease in jaundice occurs in the reverse order: first the legs turn pale, then the torso and lastly the face.
Increased physiological jaundice and its prolongation can be caused by a lack of breast milk - breastfeeding jaundice and exposure to breast milk itself - breast milk jaundice.
Breastfeeding jaundice is observed in newborns with improper organization of breastfeeding, when, for various reasons, breast milk consumption is reduced. During the first few days of life, exclusively breastfed newborns typically receive less fluid and milk than those fed with breast milk substitutes, which results in higher bilirubin levels by 4–5 days of life. The basis for the prevention and treatment of breastfeeding jaundice is staying together in the postpartum ward, frequent breastfeeding day and night without observing exact intervals, but “at the request” of the child.
Breast milk jaundice (jaundice from mother’s milk) is also not a pathological condition, it is associated with the child’s body’s reaction to breast milk fats and is characterized by the following features:
- appears or sharply intensifies when the baby begins to receive a sufficient amount of mother’s milk (on the 3-7th day);
- the level of indirect (free) bilirubin in the serum is usually above 184 µmol/l (12%), but does not exceed 360 µmol/l; no cases of kernicterus have been described;
- cessation of breastfeeding (for 24-48 hours) leads to a sharp decrease in bilirubin and a decrease in jaundice;
- if the baby continues to receive breast milk, jaundice persists for 4-6 weeks, then begins to gradually decrease. Complete normalization of indirect bilirubin in the blood serum occurs by the 12-16th week of life.
Given the benign nature of hyperbilirubinemia associated with breast milk, once the diagnosis is confirmed, breastfeeding should not be stopped under any circumstances!
In addition to physiological, jaundice in newborns can be pathological in nature, associated with high levels of total and indirect bilirubin, which determines the risk of developing the most serious complication - kernicterus.
The following signs always indicate the pathological nature of jaundice(one or more signs may be present):
- present at birth or appears on the first day of life;
- combined with signs of hemolysis, pallor, splenomegaly;
- lasts more than a week in full-term infants and more than 2 weeks in premature infants;
- proceeds in waves (the yellowness of the skin and mucous membranes increases in intensity after a period of its decrease or disappearance);
- indirect bilirubin level more than 220 µmol/l;
- the maximum level of direct bilirubin is more than 25 µmol/l.
Hemolytic disease of the newborn- hyperproduction of bilirubin due to increased hemolysis of red blood cells - occurs with group (ABO) or Rh incompatibility of mother and fetus.
Blood type incompatibility: the mother has blood group 1, the child has blood group 2 or 3. Rh factor incompatibility: the mother has Rh negative blood, the child has Rh positive blood.
The basis of hemolytic disease of newborns is the penetration of maternal antibodies developed in a previous pregnancy to the Rh factor or to the blood group of the fetus to the fetus. Thanks to immunoprophylaxis carried out during pregnancy, the number of newborns with severe hemolytic disease due to Rh incompatibility has decreased significantly.
With hemolytic disease, jaundice occurs in the first 24 hours of life, there is a decrease in hemoglobin levels and an increase in liver size. With hemolytic disease of newborns, the risk of developing kernicterus is highest: 2-3 days for Rh incompatibility and 3-4 days for blood group incompatibility.
Clinical manifestations of kernicterus(bilirubin encephalopathy):
- the appearance of lethargy, drowsiness, lethargy and suppression of the sucking reflex;
- increased irritability, muscle hypertension, monotonous screaming;
- at irreversible stages, the child experiences convulsions, apnea, bradycardia, a high-pitched cry, stupor and coma.
The outcome of kernicterus is a severe neurological defect, delayed mental and motor development, cerebral palsy, deafness, decreased vision, severe asocial disability.
Jaundice associated with hemolysis can also occur during the acute course of a number of congenital infections (cytomegaly, herpes, rubella, toxoplasmosis, syphilis, listeriosis) or be a sign of the development of neonatal sepsis. Less common in the neonatal period are familial hemolytic anemias (microspherocytic anemia of Minkowski-Choffard), erythrocyte enzymopathies and hemoglobinopathies, accompanied by jaundice. Other (non-hemolytic) causes of bilirubin hyperproduction include severe neonatal polycythemia (increased hematocrit - that part of the blood volume that accounts for red blood cells - in venous blood above 70%), swallowed blood syndrome, massive hemorrhages, including extensive cephalohematomas.
Violation of bilirubin conjugation is observed in hereditary diseases (Gilbert and Crigler-Najjar syndromes). In Gilbert's syndrome, jaundice is usually moderately expressed (8-120 µmol/l), cases of kernicterus have not been described, and the general condition is little affected. Clinical manifestations can be observed from 2-3 days of life or at any age up to 10 years, and the intensity of jaundice can change every 3-5 weeks. Jaundice in Crigler-Nayjar syndrome appears at the age of 2-3 days of life, growing steadily by the 5-8th day (indirect bilirubin level more than 340 µmol/l), creating a risk of developing kernicterus.

Conjugated (direct) hyperbilirubinemia

Principles of treatment of hyperbilirubinemia

Principles of treatment of hyperbilirubinemia:
- elimination of the main cause of the pathological increase in the level of bilirubin in the blood serum;
- prevention of the increase in bilirubin in the blood serum;
- methods that promote the removal of bilirubin. The main treatments that follow these principles are exchange transfusion and phototherapy. Other treatment methods that were used previously are considered ineffective.
Although the main treatment for hyperbilirubinemia is carried out in the maternity hospital, every healthcare professional should have an understanding of these treatment methods.
Exchange blood transfusion. Replacement blood transfusion is usually performed for hemolytic disease of newborns caused by incompatibility of the Rh factor or blood group. The decision to conduct an exchange blood transfusion is made based on a set of facts:
- studying anamnesis;
- results of blood serum testing for antibodies;
- clinical manifestations when the rate of increase in bilirubin is more than 17 µmol/l/h;
- if the hemoglobin level in the umbilical cord blood is below 110 g/l.
Phototherapy. Phototherapy has been used for over 30 years to treat neonatal jaundice without any negative consequences. The positive effects of sunlight and artificial lighting on reducing the severity of jaundice in newborns were first described in 1958. Since then, phototherapy has been the mainstay of treatment for neonatal jaundice throughout the world. Under the influence of light, indirect bilirubin is converted from a fat-soluble substance, toxic to the brain, into a non-toxic water-soluble form. The larger the body surface that is illuminated, the greater the effect of reducing toxicity. In this regard, when carrying out phototherapy, the child should be as naked as possible, but warm (incubators are used for this). Eyes and genitals must be protected from the toxic effects of the blue fraction of light. The duration of phototherapy can be from 1 to 3 days, depending on the rate of decrease in bilirubin concentration.
Basic provisions for phototherapy: if the concentration of indirect bilirubin can reach a toxic level. In case of liver diseases and obstructive jaundice, phototherapy is contraindicated.
Infusion therapy for hyperbilirubinemia. Indirect fat-soluble bilirubin has a toxic effect, so its level in the blood serum cannot be reduced by administering glucose solutions. The prescription of parenteral fluid administration to a newborn with jaundice is determined by other indications:
- there is a syndrome of vomiting and regurgitation;
- fluid is lost during phototherapy;
- there are conditions that require infusion therapy (for example, sepsis, gastroenteritis).
Induction of liver enzymes with phenobarbital. Currently, newborns with jaundice are not treated with phenobarbital.
Treatments that reduce the enterohepatic circulation of bilirubin. There are no drugs or other drugs that reduce the enterohepatic circulation of bilirubin.
All studies described in the literature on the effectiveness of drugs that improve enterohepatic circulation, binding and absorption of bilirubin, such as activated carbon, agar, cholesterol, Essentiale Forte, etc., are not scientifically based, from the point of view of evidence-based medicine.
If the yellowness of the skin increases, the pallor of the skin increases or the shade of yellowness changes, the condition is disturbed, it is necessary to begin examining the child to clarify the genesis of hyperbilirubinemia.

Observation of a child with neonatal jaundice in an outpatient setting

If the child is discharged on the third day, it is necessary to examine him at home until he reaches the age of 120 hours (5 days) and then several times until the 10th day of life (after the 10th day of life, bilirubin staining of the brain practically does not occur, which is the most dangerous complication of neonatal jaundice). In addition, it is during this period that signs of hepatitis, cholestasis and other rarer diseases and conditions appear that occur with symptoms of hyperbilirubinemia that require special intervention. A trained nurse can conduct examinations of the child and report the results of her observations to the local doctor. This will make it possible to structure and make the first period of observation of the child more logical and reasonable.
When examining a newborn with jaundice, the following questions should be answered.
1. When did jaundice appear?
2. What is the general condition of the child?
3. What is the nature (shade) of jaundice?
4. How do the sizes of the liver and spleen change?
5. What color is urine and feces?
6. Are there any hemorrhagic manifestations?
At each visit (5, 7, 10 days of the child’s life), in the outpatient chart, the nurse should note the dynamics of the child’s well-being, type of feeding, presence and changes in the nature of regurgitation, size of the liver and spleen, color of urine and stool.
In physiological jaundice, bilirubin levels can be assessed on an outpatient basis using the modified Cramer scale. It is known that staining of the skin has a characteristic dynamics from top to bottom (the extinction of jaundice also occurs from bottom to top). In Fig. Table 7.1 shows the staining zones and their approximate correspondence to the level of bilirubin in the blood.
- If a child’s face and neck are only painted, and the rest of the body is pink (zone 1), then the level of total bilirubin does not exceed 100 µmol/l.
- If the degree of staining visually corresponds to the 2nd zone - face, neck and upper body to the umbilical line, then the level of total bilirubin corresponds to 150 µmol/l.
- When staining the 3rd zone - face, neck, entire torso to legs - the approximate level of total bilirubin in the blood serum corresponds to 200 µmol/l.
- Fourth zone - the child’s entire body is painted, only the feet and palms are not painted - the level of total bilirubin in the blood serum corresponds to 250 µmol/l.
- Fifth zone - the whole body, feet and palms are painted - the level of total bilirubin is above 250 µmol/l.
Detection of staining in zones 4 and 5 is an alarming factor, especially before the 10th day of life, when there is a risk of developing bilirubin encephalopathy.
A visual assessment of jaundice according to the Cramer scale must be carried out at each visit in order to monitor the dynamics of the disease - its decrease indicates the correct course of the process, and its increase requires urgent consultation with a doctor and a decision on therapy.
A visual assessment of the level of staining is carried out in daylight: the child must be completely undressed, light pressure is applied to the skin.

An example of a clinical description of a child’s condition in an outpatient card during patronage: “The child is 6 days old (3rd examination at home). Jaundice from the second day of life. The general state of health is satisfactory, the child is active, moves in full range, when unwrapped, he immediately wakes up and stretches, takes a flexion pose. Exclusively breastfed, fed on demand, applied to the breast correctly, regurgitates rarely, and did not increase in regurgitation during observation. The skin is yellow on a pink background, without growth, according to the Cramer scale - zone 2. The liver is +0.5 cm, the edge is soft, the spleen is not palpable, the stool is yellow with an admixture of greenery, after each feeding, the urine is light. Conclusion: physiological jaundice in the child proceeds normally.”
Such a record provides comprehensive information about the child and allows you to determine the tactics of its management and the need for any treatment.
An alternative to using the visual Cramer scale is to determine transcutaneous bilirubin by calculating the transcutaneous bilirubin index.
Transcutaneous method for determining bilirubin levels. The ability of bilirubin to change skin color with increasing concentration has made it possible to develop and introduce into medical practice non-invasive devices for transcutaneous (percutaneous) determination of bilirubin. Transcutaneous determination of bilirubin level in blood serum is based on the fact that there is a direct relationship between the concentration of bilirubin in the blood and in the skin. Bilirubin has a pronounced yellow color; the color of the skin changes depending on the bilirubin content in it. Since there are no standards for the concentration of bilirubin in the skin (and are unlikely to be created), instruments for the transcutaneous determination of bilirubin in the skin are calibrated in arbitrary units, which are named in accordance with international practice “transcutaneous bilirubin index”.
Advantages of the method:
- accessibility, ease of use, portability of the device;
- the ability to carry out multiple measurements during the day by any nursing staff (nurse, parents);
- non-invasive and painless for the child, regardless of his gestational age, body weight and day of life;
- the ability to control the course of jaundice, to objectively determine its increase and decrease;
- the ability to use the device in outpatient and home settings to monitor the course of jaundice.
Important!
Transcutaneous bilirubinmetry allows you to measure the level of bilirubin in the dermis, and not in the blood serum, therefore the value of the transcutaneous bilirubin index conveys only the dynamics of the course of hyperbilirubinemia.
Hospitalization of a child with prolonged hyperbilirubinemia is indicated only in the following cases:
- deterioration of the child’s condition;
- serum bilirubin more than 200 µmol/l without a tendency to decrease or with its increase (more than the 3rd zone on the Cramer scale after the 7-10th day of life);
- direct bilirubin fraction more than 20% of the total serum bilirubin level;
- enlarged liver and/or spleen;
- presence of dark urine and discolored stool.
Thus, the main difficulty in the management of newborn jaundice is, on the one hand, the high frequency of uncomplicated forms, which require optimal care and feeding, without the use of any medications. On the other hand, the risk of complications in case of missed pathological hyperbilirubinemia is so great that it places excessive responsibility on the medical professional when making a diagnosis and choosing a treatment method. In order not to miss dangerous symptoms and avoid possible mistakes, it is not advisable to rely on experience and intuition, but it is better to use a clear algorithm of actions, which is described above, accepted in world practice and allows you to perform all the necessary studies depending on the day of the child’s life and the symptoms that appear. This allows you to prevent bilirubin encephalopathy with complicated indirect hyperbilirubinemia of any nature, make a timely diagnosis and outline a plan for further treatment and observation. The use of a surveillance algorithm also allows a large number of newborns with physiological jaundice to avoid unnecessary and sometimes dangerous interventions such as infusions, medications (phenobarbital), and hospitalization for treatment, which separates him from his mother and limits breastfeeding.

Nursing care for a newborn in an outpatient setting. Ed. DI. Zelinskaya. 2010